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  1. Regret and Responsibility Resolved? Evaluation Ordonez and Connolly's Conclusions.

    NARCIS (Netherlands)

    Zeelenberg, M.; van Dijk, W.W.; Manstead, A.S.R.

    2000-01-01

    T. Connolly, L. D. Ordo;aan;atez, and R. Coughlan (1997, Organizational Behavior and Human Decision Processes, 70, 73-85) argued, on the basis of 5 experiments, that regret need not be related to a sense of responsibility for the regretted outcome. We (M. Zeelenberg, W. W. van Dijk, & A. S. R.

  2. Regret and Responsibility Resolved? Evaluating Ordóñez and Connolly's (2000) Conclusions.

    Science.gov (United States)

    Zeelenberg; van Dijk WW; Manstead

    2000-01-01

    T. Connolly, L. D. Ordo;aan;atez, and R. Coughlan (1997, Organizational Behavior and Human Decision Processes, 70, 73-85) argued, on the basis of 5 experiments, that regret need not be related to a sense of responsibility for the regretted outcome. We (M. Zeelenberg, W. W. van Dijk, & A. S. R. Manstead, 1998, Organizational Behavior and Human Decision Processes, 74, 254-272) showed in 2 experiments that this conclusion was premature, because it was based on an indirect measure of regret (i.e., overall happiness with the decision outcome). When regret was directly measured, the predicted effects of responsibility were found. L. D. Ordo;aan;atez and T. Connolly (2000, Organizational Behavior and Human Decision Processes, 81, 132-142) replicated our findings in 2 experiments. Based on their findings they arrived at 4 conclusions. In this rejoinder we first discuss Ordóñez and Connolly's new studies and we then discuss the validity of their 4 conclusions. Copyright 2000 Academic Press.

  3. Democracy: Between the essentially contested concept and the agonistic practice: Connolly, Mouffe, Tully

    Directory of Open Access Journals (Sweden)

    Sladeček Michal

    2010-01-01

    Full Text Available The text considers points of view of theoreticians of the radical pluralism (democracy: Connolly (William Connolly, Mouffe (Chantal Mouffe and Tully (James Tully with regard to the status and the nature of concepts in the political discourse, as well as the consequences of these conceptual presumptions to understanding democracy. The three authors emphasize the essential contestability of political concepts, the paradox of liberal democracy and the need to revise standard rational consensus theories of democracy. Also, the three authors take over the specific interpretation of Vittgenstein to the direction of political theory the centre of which consists of everyday contingent practices of politics as well as dissent about their assessment. The text analyzes the extent to which this reading is compatible to Wittgenstein's position. The author defends the opinion that the essential contestability does not imply agonism and denial of the significance of rules and tries to indicate to the points of illegitimate transition from antiessentialism to unconsensus rules. Also, the text underlines the flaws of dissent conception of democracy and social integration.

  4. Sjögren syndrome

    Science.gov (United States)

    Xerostomia - Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome ... The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most ...

  5. LIGSITEcsc: predicting ligand binding sites using the Connolly surface and degree of conservation

    Directory of Open Access Journals (Sweden)

    Schroeder Michael

    2006-09-01

    Full Text Available Abstract Background Identifying pockets on protein surfaces is of great importance for many structure-based drug design applications and protein-ligand docking algorithms. Over the last ten years, many geometric methods for the prediction of ligand-binding sites have been developed. Results We present LIGSITEcsc, an extension and implementation of the LIGSITE algorithm. LIGSITEcsc is based on the notion of surface-solvent-surface events and the degree of conservation of the involved surface residues. We compare our algorithm to four other approaches, LIGSITE, CAST, PASS, and SURFNET, and evaluate all on a dataset of 48 unbound/bound structures and 210 bound-structures. LIGSITEcsc performs slightly better than the other tools and achieves a success rate of 71% and 75%, respectively. Conclusion The use of the Connolly surface leads to slight improvements, the prediction re-ranking by conservation to significant improvements of the binding site predictions. A web server for LIGSITEcsc and its source code is available at scoppi.biotec.tu-dresden.de/pocket.

  6. A Retrospective Audit of In-Hospital 30-day Mortality from Acute Myocardial Infarction in Connolly Hospital Blanchardstown

    LENUS (Irish Health Repository)

    Hensey, M

    2017-09-01

    In 2015, The Department of Health published the first annual report of the “National Healthcare Quality Reporting System.” Connolly Hospital was reported to a mortality rate within 30 days post-Acute Myocardial Infarction (AMI) of 9.87 per 100 cases which was statistically significantly higher than the national rate. We carried out a retrospective audit of patients who were HIPE-coded as having died within 30 days of AMI from 2011-2013 and identified 42 patients. On review, only 23 patients (54.8%) were confirmed as having had an AMI. We identified 12 patients who had AMI included on death certificate without any evidence for same. If the 22 patients incorrectly coded were excluded, the mortality rate within 30 days post-AMI in CHB would fall to 4.14 deaths per 100 cases, well below the national average. Inaccuracies of data collection can lead to erroneous conclusions when examining healthcare data.

  7. [Sjögren's syndrome].

    Science.gov (United States)

    Silvestre Donat, F J; Bagán Sebastián, J V

    1990-10-01

    We carry out an updated review of Sjögren's syndrome on occasion of the presentation of three new clinical cases whose oral manifestations led the patients to first seek help. Each patient was subjected to clinical exploration, together with complementary evaluations, aimed at establishing an early and precise diagnosis of the syndrome. We consider it important to adopt a series of standardized diagnostic criteria and revise treatment to maintain the orodental conditions as favorable as possible.

  8. Periodontal disease in primary Sjögren's Syndrome

    DEFF Research Database (Denmark)

    Schjødt, Morten; Christensen, Lisa Bøge; Petersen, P.E.

    2001-01-01

    Sjögren's syndrome, gingivitis, periodontitis, periodontal disease, xerostomia, oral manifestations......Sjögren's syndrome, gingivitis, periodontitis, periodontal disease, xerostomia, oral manifestations...

  9. Primary Sjögren's syndrome as a systemic disease

    DEFF Research Database (Denmark)

    Malladi, Arundathi S; Sack, Kenneth E; Shiboski, Stephen C

    2012-01-01

    To study the prevalence of extraglandular manifestations in primary Sjögren's syndrome (SS) among participants enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA) Registry.......To study the prevalence of extraglandular manifestations in primary Sjögren's syndrome (SS) among participants enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA) Registry....

  10. Karl Gustaf Torsten Sjögren and the Sjögren-Larsson syndrome

    Directory of Open Access Journals (Sweden)

    Khalid Al Aboud

    2011-09-01

    Full Text Available Karl Gustaf Torsten Sjögren (1896-1974 a Swedish psychiatrist and geneticist, was a pioneer of modern Swedish psychiatry. Sjögren studied medicine at the University of Uppsala. From 1932 to 1935, he was Head Physician and Director of Lillehagen Hospital in Gothenburg, and between 1935- 1945, he was physician-in-chief at the psychiatric department of Sahlgrenska Hospital in Gothenburg. Sjögren was professor of psychiatry at Karolinska Institutet from 1945 to 1961 and was elected to the Royal Swedish Academy of Sciences in 1951. Sjögren was an expert of psychiatry for the World Health Organization. Among his many contributions to medicine, he is credited for describing several medical conditions, which were later named after him, including Graefe- Sjögren syndrome, Marinesco-Sjögren syndrome, and Sjögren-Larsson syndrome (SLS. During his work on juvenile amaurotic idiocy, Sjögren forged a collaboration with Tage K.L. Larsson, a statistics lecturer at the University of Lund. Their study on the combination of oligophrenia, congenital ichthyosis, and spastic disorders in 1957 established the clinical and genetic profile of a new disease entity, later known as Sjogren-Larsson syndrome (SLS.

  11. Periodontal conditions of individuals with Sjögren's syndrome.

    Science.gov (United States)

    Antoniazzi, Raquel Pippi; Miranda, Letícia Algarves; Zanatta, Fabricio Batistin; Islabão, Alexandre Garcia; Gustafsson, Anders; Chiapinotto, Geraldo Augusto; Oppermann, Rui Vicente

    2009-03-01

    Sjögren's syndrome (SjS) is a systemic autoimmune disease that might lead to hyposalivation and negatively affect the oral environment. The evidence with regard to the periodontal conditions in this group of subjects is still controversial. This study aimed to evaluate the periodontal clinical conditions and inflammatory markers in gingival crevicular fluid (GCF) from patients with primary Sjögren's syndrome (SjS [P]) or secondary Sjögren's syndrome (SjS [S]) compared to a control group. Nineteen individuals with SjS (11 SjS [P] and eight SjS [S]) and 19 controls, matched for gender, age, and tobacco exposure, were selected from two private clinics and a hospital. The groups were compared for stimulated whole saliva (SWS) flow rate, plaque index (PI), gingival index (GI), probing depth (PD), bleeding on probing (BOP), clinical attachment level (CAL), and total amount of interleukin (IL)-1beta and total elastase activity in the GCF. Generalized estimating equations were used for data analysis. Individuals with SjS had a significantly lower SWS flow rate and higher mean PI, GI, PD, CAL, and BOP than controls. After adjustment for plaque, GI remained significantly higher in patients with SjS. Patients with SjS (S) had significantly higher mean CAL and PD than patients with SjS (P), and CAL and BOP remained significantly higher in this subgroup after adjustment. No differences were observed with regard to the GCF inflammatory markers. After adjusting for PD, subjects with SjS (P) showed lower levels of IL-1beta compared to controls. SjS seemed to negatively affect the periodontal condition because gingival inflammation was more evident in the individuals with SjS, particularly those with SjS (S).

  12. Syndrome de Sjögren-Larsson

    NARCIS (Netherlands)

    Misery, Laurent; Antoine, Jean-Christophe; Touraine, Renaud; Wanders, Ronald; Maitre, Séverine; Has, Christina; Perrot, Jean-Luc; Cambazard, Frédéric

    2002-01-01

    INTRODUCTION: Sjögren-Larsson syndrome (SJS) is an autosomal-recessive disorder. Patients suffer from congenital ichtyosis, mental retardation and symmetric spastic paralysis. Ichtyosis is usually pronounced and associated with erythroderma. Neurological manifestations occur usually between 4 and 13

  13. Cytokines in Sjögren's syndrome

    NARCIS (Netherlands)

    Roescher, N.; Tak, P. P.; Illei, G. G.

    2009-01-01

    Cytokines play a central role in the regulation of immunity and are often found to be deregulated in autoimmune diseases. Sjögren's syndrome is a chronic autoimmune disease characterized by inflammation and loss of secretory function of the salivary and lachrymal glands. This review highlights the

  14. Sjøforsvaret i ny drakt

    DEFF Research Database (Denmark)

    Vestvik, Marit; Bjerck, Mari

    Denne rapporten formidler resultater fra undersøkelser som er gjort på to av Sjøforsvarets fartøy i 2010 og 2011, som omfatter utprøving av ny niformsmodell for utendørs bruk. Undersøkelsene er en del av forskningsprosjektet Uni-Form: Arbeidsbekledning for kvinner i mannsdominerte yrker. I...... prosjektet utvikles arbeidsklær for Sjøforsvarets personell som har bedre funksjonalitet og komfort enn eksisterende uniformer. En målsetning er å bedre trivselen for personellet gjennom å utvikle forbedrete uniformbekledning. At uniformsbekledningen er tilfredsstillende i funksjonalitet, passform og...... tilpasset snitt for kvinner. Dette gjelder også Forsvarets uniformer....

  15. The lived experience of Sj?gren?s Syndrome

    OpenAIRE

    Ngo, Di Ying J.; Thomson, William M.; Nolan, Anita; Ferguson, Shelagh

    2016-01-01

    Background Sj?gren?s Syndrome is an autoimmune exocrinopathy characterised by lymphocytic infiltration of exocrine glands in multiple sites, with dry mouth as a primary presenting symptom. Although quantitative studies have shown the negative impact of both dry mouth and Sj?gren?s Syndrome on patients? quality of life, no qualitative diary and interview study has been undertaken to examine the lived experience of dry mouth for Sj?gren?s Syndrome sufferers. The aim of this qualitative study wa...

  16. Pain in Sjögren’s syndrome

    Directory of Open Access Journals (Sweden)

    C. Giacomelli

    2014-06-01

    Full Text Available Sjögren’s syndrome (SjS is an autoimmune disease that affects the salivary and lacrimal glands, but it can also have extra-glandular manifestations. Although pain has not yet been fully studied and characterized, it is a symptom that can be often found in patients with SjS, who mainly complain of neuropathic pain, followed by nociceptive pain. The latter when combined with widespread dysfunctional symptoms is defined fibromyalgia. The aim of this work is to analyze the scientific literature on the presence of pain in patients with primary Sjögren’s syndrome.

  17. Region Sjællands Økologiprofil

    DEFF Research Database (Denmark)

    Holm, Jesper; Lund Sørensen, Runa Cecilie

    Nærværende rapport bygger på erfaringer foretaget gennem en kortlægning af Region Sjællands økologiske sektor, som blev lavet i forbindelse med Region Sjællands store fødevareprojekt Grønne Regionale Madoplevelser (GRO) under delprojektet Økologisk Oplevelsesinnovation. Formålet med undersøgelsen...... har været at generere et overblik over Region Sjællands økologiske sektor, både hvad angår antallet og udbredelsen af økologiske landbrugsbedrifter, forarbejdningsvirksomheder, engrosvirksomheder og detailforretninger....

  18. Genetics Home Reference: Marinesco-Sjögren syndrome

    Science.gov (United States)

    ... insertion mutation in the SIL1 gene in 3 Japanese patients with Marinesco-Sjögren syndrome. J Neurol Sci. ... of Medicine Lister Hill National Center for Biomedical Communications 8600 Rockville Pike, Bethesda, MD 20894, USA HONCode ...

  19. The lived experience of Sjögren's Syndrome.

    Science.gov (United States)

    Ngo, Di Ying J; Thomson, William M; Nolan, Anita; Ferguson, Shelagh

    2016-02-01

    Sjögren's Syndrome is an autoimmune exocrinopathy characterised by lymphocytic infiltration of exocrine glands in multiple sites, with dry mouth as a primary presenting symptom. Although quantitative studies have shown the negative impact of both dry mouth and Sjögren's Syndrome on patients' quality of life, no qualitative diary and interview study has been undertaken to examine the lived experience of dry mouth for Sjögren's Syndrome sufferers. The aim of this qualitative study was to provide clinicians with insight into how dry mouth can impact on the daily lives of Sjögren's Syndrome patients. The American-European Consensus Group (AECG) Revised International Classification criteria were used to identify participants from patients seen in an oral medicine clinic. After pilot study work to test the approach, the 10 main study participants were recruited. Diary entries and semi-structured interviews were used to explore how dry mouth affects their lives. Owing to the exploratory nature of the research, thematic content analysis was applied, allowing the themes to arise naturalistically from the data without bias or elicitation. The data showed that it is unrealistic to understand the experience of a single symptom, but that the disease as a whole needs to be taken into perspective. The empirical evidence supported four main themes that depicted the lived experience of Sjögren's Syndrome. These included: (1) the journey to diagnosis; (2) disease impact spectrum (of dry mouth amid other symptoms); (3) interactions with healthcare professionals; and (4) the positive coping process. The findings revealed patients' perspectives on diagnosis, coping with dry mouth and Sjögren's Syndrome, and interaction with healthcare professionals. Dry mouth is not a trivial symptom for Sjögren's Syndrome sufferers; it has considerable impact on their day-to-day lives. Healthcare professionals need to understand patients as individuals in their environment in order to be part

  20. Management of dry mouth in Sjögren's syndrome

    OpenAIRE

    Yoichi Nakagawa

    2011-01-01

    The management of dry mouth is essential for patients with Sjögren's syndrome. The symptomatic treatment has included using air humidifiers, rinsing the mouth with water or mouthwash, the application of a salivary substitute and administration of secretagogues. There are three secretagogues suitable for the alleviation of dry mouth in Sjögren's syndrome patients in Japan; cevimeline hydrochloride hydrate (cevimeline), pilocarpine hydrochloride, and anetholtrithione. A relationship between the...

  1. Pancreatic function and morphology in Sjögren's syndrome

    DEFF Research Database (Denmark)

    Afzelius, Pia; Fallentin, Eva Marie; Larsen, Steen

    2010-01-01

    Sjögren's syndrome (SS) is considered to be a universal exocrinopathy most likely based on autoimmune mechanisms. The degree of exocrine involvement in SS with the exception of salivary and lachrymal glands is, however, not yet established.......Sjögren's syndrome (SS) is considered to be a universal exocrinopathy most likely based on autoimmune mechanisms. The degree of exocrine involvement in SS with the exception of salivary and lachrymal glands is, however, not yet established....

  2. Pulmonary manifestations of Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Thomas Flament

    2016-06-01

    Full Text Available In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

  3. The epidemiology of Sjögren’s syndrome

    Directory of Open Access Journals (Sweden)

    Patel R

    2014-07-01

    Full Text Available Ruchika Patel, Anupama Shahane Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA Abstract: Sjögren’s syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. It can present as an entity by itself, primary Sjögren's syndrome (pSS, or in addition to another autoimmune disease, secondary Sjögren's syndrome (sSS. pSS has a strong female propensity and is more prevalent in Caucasian women, with the mean age of onset usually in the 4th to 5th decade. Clinical presentation varies from mild symptoms, such as classic sicca symptoms of dry eyes and dry mouth, keratoconjunctivitis sicca, and xerostomia, to severe systemic symptoms, involving multiple organ systems. Furthermore, a range of autoantibodies can be present in Sjögren's syndrome (anti-SSA/Ro and anti-SSB/La antibodies, rheumatoid factor, cryoglobulins, antinuclear antibodies, complicating the presentation. The heterogeneity of signs and symptoms has led to the development of multiple classification criteria. However, there is no accepted universal classification criterion for the diagnosis of Sjögren's syndrome. There are a limited number of studies that have been published on the epidemiology of Sjögren's syndrome, and the incidence and prevalence of the disease varies according to the classification criteria used. The data is further confounded by selection bias and misclassification bias, making it difficult for interpretation. The aim of this review is to understand the reported incidence and prevalence on pSS and sSS, the frequency of autoantibodies, and the risk of malignancy, which has been associated with pSS, taking into account the different classification criteria used. Keywords: Sjögren's syndrome, incidence, prevalence, classification criteria, autoantibodies, lymphoma

  4. Cutaneous and Mucosal Manifestations of Sjögren's Syndrome.

    Science.gov (United States)

    Generali, Elena; Costanzo, Antonio; Mainetti, Carlo; Selmi, Carlo

    2017-12-01

    Sjögren's syndrome is currently considered an "autoimmune epithelitis," as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren's syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases. Further, xerophtalmia (also known as dry eye) frequently associated with keratoconjunctivitis sicca cumulatively affects approximately 10-30% of the general population with increasing incidence with age and is more frequently secondary to non-autoimmune diseases. On the other hand, numerous patients with Sjögren's syndrome manifest signs of systemic dryness involving the nose, the trachea, the vagina, and the skin, suggesting that other glands are also affected beyond the exocrine epithelia. Skin involvement in Sjögren's syndrome is relatively common, and various manifestations may be present, in particular xeroderma, eyelid dermatitis, annular erythema, and cutaneous vasculitis. Additional skin non-vasculitic manifestations include livedo reticularis which may occur in the absence of vasculitis, and localized nodular cutaneous amyloidosis possibly representing lymphoproliferative diseases related to Sjögren's syndrome. The treatment of skin and mucosal manifestations in Sjögren's syndrome is similar regardless of the cause, starting from patient education to avoid alcohol and tobacco smoking and to pursue dental hygiene. In conclusion, a strict collaboration between the dermatologist and the rheumatologist is essential in the adequate management of Sjögren's syndrome skin and mucosal manifestations.

  5. Síndrome seco - Síndrome de Sjögren

    OpenAIRE

    Cañas Dávila, Carlos Alberto

    2002-01-01

    ¿Qué es el "síndrome seco"?/ ¿Qué es el síndrome Sjögren?/ ¿Qué es un síndrome de Sjögren primario y qué es un síndrome de Sjögren secundario?/ ¿Cuáles son las causas del síndrome de Sjögren?/ ¿Qué otras manifestaciones distintas a los síntomas secos puede ocasionar el síndrome de Sjögren?/ ¿Cómo se diagnostica un síndrome de Sjögren?/ ¿Cuál es el tratamiento del síndrome de Sjögren?/ ¿Qué tan frecuente es el síndrome de Sjögren?

  6. Dry Mouth and Sjögren's Syndrome: An Overview.

    Science.gov (United States)

    Khan, Abgeena; Shirlaw, Penelope J

    2016-02-01

    Dry mouth is a common condition presenting to a GP or general dental practitioner. The most common cause of a dry mouth is related to medication use, however patients with Sjögren's syndrome, a multisystem autoimmune condition, may present to their dentist rather than their GP complaining of dry mouth and dry eyes. This article explores the causes of dry mouth and how a patient can be investigated to find the cause of their dry mouth. An overview of Sjögren's syndrome, the relevant diagnostic criteria, presenting signs and symptoms, investigations and management principles are outlined.

  7. Síndrome de Sjögren: relato de caso = Sjögren’s syndrome: case report

    OpenAIRE

    Alencar, Cristiane

    2007-01-01

    Objetivos: Apresentar um caso clínico enfatizando a abordagem terapêutica realizada pelo cirurgião dentista com a finalidade de melhorar a qualidade de vida dos pacientes acometidos pela Síndrome de Sjögren Descrição do caso: Paciente feminina com Síndrome de Sjögren encaminhada para reabilitação protética apresentava ressecamento da face, olhos, lábios e toda a mucosa bucal. Queixava-se de dificuldade para falar, mastigar e engolir. Foi realizada a adequação bucal e foram dadas instruções...

  8. EULAR Sjögren's syndrome disease activity index (ESSDAI)

    DEFF Research Database (Denmark)

    Seror, Raphaèle; Bowman, Simon J; Brito-Zeron, Pilar

    2015-01-01

    The EULAR Sjögren's syndrome (SS) disease activity index (ESSDAI) is a systemic disease activity index that was designed to measure disease activity in patients with primary SS. With the growing use of the ESSDAI, some domains appear to be more challenging to rate than others. The ESSDAI is now i...

  9. Polymorphism and association of microsatellite SJ01 with birth ...

    African Journals Online (AJOL)

    PRECIOUS

    2009-12-15

    Dec 15, 2009 ... 2College of Life Science, Linyi Normal University, Linyi 276005, China. Accepted 9 ... breeds, Duroc population had the highest heterozygosity and deviated from Hardy-Weinberg equilibrium (P < 0.05). ... suggest that microsatellite SJ01 is a potential DNA marker for early growth trait selection in Yorkshire.

  10. Management of dry mouth in Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Yoichi Nakagawa

    2011-08-01

    Full Text Available The management of dry mouth is essential for patients with Sjögren's syndrome. The symptomatic treatment has included using air humidifiers, rinsing the mouth with water or mouthwash, the application of a salivary substitute and administration of secretagogues. There are three secretagogues suitable for the alleviation of dry mouth in Sjögren's syndrome patients in Japan; cevimeline hydrochloride hydrate (cevimeline, pilocarpine hydrochloride, and anetholtrithione. A relationship between the effect of cevimeline on saliva secretion and the degree of salivary gland destruction evaluated by sialography and histopathological findings in the labial minor salivary glands has been reported. These diagnostic approaches could provide useful prognostic information on the efficacy of cevimeline in Sjögren's syndrome patients. Concomitantly, a bite guard was suggested as an effective lubricating device because it maintains the lubricants in the proper location. In addition, the management of the complications of dry mouth, such as tooth caries, periodontitis and oral candidiasis, which all lead to a reduction in the QOL, is also important. Both the prevention and treatment of erythematous candidiasis is especially important in the management of Sjögren's syndrome.

  11. Síndrome de Sjögren: relato de caso = Sjögren’s syndrome: case report

    Directory of Open Access Journals (Sweden)

    Alencar, Cristiane

    2007-01-01

    Conclusões: A Síndrome de Sjögren compreende anormalidades em diversos setores do organismo, incluindo a saliva, que desempenha importante papel na fisiologia e biologia do ser humano. Portanto, a interdisciplinaridade, com envolvimento do cirurgião dentista, é de extrema importância para o sucesso terapêutico desta doença

  12. [Expression and characterization of recombinant Sj23HD-HSA fusion protein in Saccharomyces cerevisiae].

    Science.gov (United States)

    Zhang, Wei; Yu, Chuan-Xin; Yang, Jian-Linag; Feng, Wei; Yin, Xu-Ren; Song, Li-Jun; Wang, Jie; Qian, Chun-Yan; Ke, Xue-Dan

    2011-12-01

    To prepare the fusion protein of large hydrophilic domain of 23 kDa membrane protein of Schistosoma japonicum and the mature peptide of human serum albumin (Sj23HD-HSA) and investigate its immunoreactivity. A fusion protein gene encoding Sj23HD-HSA fusion protein was prepared by overlapping PCR, which was confirmed by TA cloning and DNA sequencing. The fusion gene of Sj23HD-HSA was directionally subcloned into yeast expression plasmid pWX530 to construct a recombinant plasmid Sj23HD-HSA/pWX530. The transformants of Saccharomyces cerevisiae containing the recombinant plasmid Sj23HD-HSA/pWX530 were screened on leu deficient SD medium after yeast competent cells were transformed with recombinant plasmid. The excretive Sj23HD-HSA protein was expressed by culturing the yeast transformants at 30 degrees C for 1 week, and the protein component of culture supernatant was analyzed by SDS-PAGE. Sj23HD-HSA fusion protein was purified through Ion Exchange Chromatography. The immunoreactivity of recombinant Sj23HD-HSA fusion protein was determined by Western blotting with sera of schistosomiasis, clonorchiasis and healthy. The gene encoding the Sj23HD-HSA fusion protein was constructed successfully, which was confirmed by DNA sequencing. The yeast transformants containing plasmid Sj23HD-HSA/pWX530 could express the excretive Sj23HD-HSA fusion protein without inducing. The results of Western blotting indicated Sj23HD-HSA could be recognized by the sera of schistosomiasis, but could not be recognized by the sera of clonorchiasis and healthy respectively. Sj23HD-HSA fusion protein with good immune reactivity is prepared successfully, which will be a potential antigen for schistosomiasis immunodiagnosis.

  13. Atypical Presentation of Sjögren-Larsson Syndrome

    Directory of Open Access Journals (Sweden)

    D. Papathemeli

    2017-01-01

    Full Text Available Sjögren-Larsson syndrome is a rare neurocutaneous disorder characterized by ichthyosis, spastic diplegia or tetraplegia, and intellectual disability. Herein, we describe a case of a Greek patient with ichthyosis and spasticity of the legs but with normal intelligence (IQ 95. This syndrome should be suspected when a child presents with ichthyosis and spastic diplegia or tetraplegia, even if intelligence is normal.

  14. Radiation Measured for Chinese Satellite SJ-10 Space Mission

    Science.gov (United States)

    Zhou, Dazhuang; Sun, Yeqing; Zhang, Binquan; Zhang, Shenyi; Sun, Yueqiang; Liang, Jinbao; Zhu, Guangwu; Jing, Tao; Yuan, Bin; Zhang, Huanxin; Zhang, Meng; Wang, Wei; Zhao, Lei

    2018-02-01

    Space biological effects are mainly a result of space radiation particles with high linear energy transfer (LET); therefore, accurate measurement of high LET space radiation is vital. The radiation in low Earth orbits is composed mainly of high-energy galactic cosmic rays (GCRs), solar energetic particles, particles of radiation belts, the South Atlantic Anomaly, and the albedo neutrons and protons scattered from the Earth's atmosphere. CR-39 plastic nuclear track detectors sensitive to high LET are the best passive detectors to measure space radiation. The LET method that employs CR-39 can measure all the radiation LET spectra and quantities. CR-39 detectors can also record the incident directions and coordinates of GCR heavy ions that pass through both CR-39 and biosamples, and the impact parameter, the distance between the particle's incident point and the seed's spore, can then be determined. The radiation characteristics and impact parameter of GCR heavy ions are especially beneficial for in-depth research regarding space radiation biological effects. The payload returnable satellite SJ-10 provided an excellent opportunity to investigate space radiation biological effects with CR-39 detectors. The space bio-effects experiment was successfully conducted on board the SJ-10 satellite. This paper introduces space radiation in low Earth orbits and the LET method in radiation-related research and presents the results of nuclear tracks and biosamples hitting distributions of GCR heavy ions, the radiation LET spectra, and the quantities measured for the SJ-10 space mission. The SJ-10 bio-experiment indicated that radiation may produce significant bio-effects.

  15. Oral involvement in primary Sjögren syndrome.

    Science.gov (United States)

    Fox, Philip C; Bowman, Simon J; Segal, Barbara; Vivino, Frederick B; Murukutla, Nandita; Choueiri, Karen; Ogale, Sarika; McLean, Lachy

    2008-12-01

    In small studies, investigators have described oral features and their sequelae in primary Sjögren syndrome (PSS), but they have not provided a full picture of the aspects and implications of oral involvement. The authors describe what is, to their knowledge, the first large-scale evaluation to do so. In addition, they report data regarding utilization and cost of dental care among patients with PSS. The authors surveyed patients with primary Sjögren syndrome as identified by their physicians (PhysR-PSS), patient-members of the Sjögren's Syndrome Foundation (SSF-PSS) and control subjects who did not have PSS. They made comparisons between the three groups. Subjects were 277 patients with PhysR-PSS, 1,225 patients with SSF-PSS and 606 control subjects. More than 96 percent of those in the patient groups experienced oral problems. An oral complaint was the initial symptom in more than one-half of the patients. Xerostomia-associated signs and symptoms were common and severe, as evidenced by scores on an inventory of sicca symptoms. These patients' rate of dental care utilization was high, and the care was costly. Oral and dental disease in PSS is extensive and persistent and represents a significant burden of illness. Oral symptoms and signs are common in patients with PSS. Early recognition of the significance of these findings by oral specialists could accelerate diagnosis and minimize oral morbidities.

  16. Síndrome Sjögren - Larsson

    Directory of Open Access Journals (Sweden)

    Leonardo Portela Rabello

    2011-12-01

    Full Text Available Relatam-se os casos de dois irmãos consanguíneos com síndrome de Sjögren- Larsson, enfatizando a importância clínica do exame oftalmológico. BPLS, masculino, 11 anos e MBLS, feminino, 10 anos, irmãos de pais não-consanguíneos, apresentando ictiose congênita, diplegia espástica e retardo mental. Ao exame oftalmológico, apresentavam miopia, fotofobia, baixa acuidade visual. A fundoscopia, presença de cristais branco-amarelados em área foveal e parafoveal em ambos os olhos. Aconselhamento genético foi realizado. O manejo foi de suporte. A Síndrome de Sjögren-Larsson é uma rara doença autossômica recessiva em que há 100% de penetrância. Síndrome de Sjögren-Larsson é classicamente caracterizada por ictiose, espasticidade e deficiência mental. A doença é causada por mutações no gene aldeído desidrogenase. As alterações oculares observadas são geralmente bilaterais, cristais branco-amarelados em área retiniana, que aparecem nos dois primeiros anos de vida e que vão aumentando em número com a idade. As anormalidades oculares não têm relação com a severidade da ictiose ou com as anormalidades neurológicas. Acredita-se que as lesões oftalmológicas sejam um sinal patognomônico da síndrome. É necessário enfatizar a importância do diagnóstico precoce e possibilidades de tratamento dietético.

  17. Primary Sjögren's syndrome: oral aspects on pathogenesis, diagnostic criteria, clinical features and approaches for therapy

    DEFF Research Database (Denmark)

    Pedersen, A.M.; Nauntofte, Birgitte

    2001-01-01

    diagnostic criteria, labial salivary gland histopathology, primary Sjögren's syndrome, salivary gland function, therapy, xerostomia......diagnostic criteria, labial salivary gland histopathology, primary Sjögren's syndrome, salivary gland function, therapy, xerostomia...

  18. Självkänsla och idrottslig kompetens hos idrottare inom Special Olympics

    OpenAIRE

    Bengtsson, Sandra; Dalsmyr, Malin

    2015-01-01

    Individer med intellektuella funktionsnedsättningar (IF), IK<70, tenderar att ha lägre självkänsla än individer utan IF. Låg självkänsla korrelerar med psykisk ohälsa, såsom depression, ångest och ätstörningar. Global självkänsla, en individs generella uppfattning av sig själv och sitt värde, påverkar och påverkas av undergrupper, såsom idrottslig kompetens (Shavelson, Hubner, & Stanton, 1976). Studier utanför Sverige visar att deltagande i organiserad idrottsverksamhet ökar global sjä...

  19. Primary Sjögren′s syndrome without ocular involvement: A rare case report

    Directory of Open Access Journals (Sweden)

    Tushar Phulambrikar

    2014-01-01

    Full Text Available Sjögren′s Syndrome (SS is a chronic systemic autoimmune disorder, characterized by the lymphocytic infiltration of lacrimal and salivary glands, giving rise to dry eyes (keratoconjunctivitis sicca and dry mouth (xerostomia. Primary Sjögren′s Syndrome commonly presents only with sicca manifestations; whereas, secondary Sjögren′s syndrome occurs in connection with other autoimmune rheumatic diseases. Primary Sjögren′s syndrome without ocular manifestation is rarely reported in the literature. Here we report a case of a 45-year-old female, who presented to us with complaints of dryness of mouth and dysphagia, without any ocular and systemic manifestations. On further evaluation she was diagnosed as a case of Primary Sjögren′s syndrome. With this case report, we intend to emphasize the importance of an early diagnosis of this disorder, along with a brief review of various diagnostic criteria.

  20. Primary Sjögren’s syndrome with polymyositis, a rare amalgamation

    Directory of Open Access Journals (Sweden)

    Harpreet Singh

    2018-01-01

    Full Text Available Sjögren’s syndrome is characterized by diminished lacrimal and salivary gland secretory function. This disorder is not strictly confined to the exocrine glands and its manifestations may extend to extraglandular sites, such as the lungs, kidneys, reticuloendothelial system, and the musculoskeletal system. Although muscular manifestations are very common with Sjögren’s syndrome, true myopathy is very rare. Here, we report a case of a 45-year-old woman who presented with complaints of bilateral progressive weakness of upper and lower limbs associated with difficulty in neck holding with a history of dryness of the mouth and the eyes. The diagnosis of polymyositis associated with Sjögren’s syndrome was established on the basis of clinical picture and diagnostic tests. True polymyositis is very rare in primary Sjögren syndrome and there are scarcely any cases of primary Sjögren’s syndrome with polymyositis reported in the literature.

  1. Xerostomia secondary to Sjögren's syndrome in the elderly: recognition and management.

    Science.gov (United States)

    Al-Hashimi, Ibtisam

    2005-01-01

    Xerostomia is a common symptom in the elderly population. Studies have suggested that the underlying cause of approximately 40% of xerostomia in the elderly is Sjögren's syndrome. Although it is highly prevalent among middle-aged individuals, elderly patients account for up to 20% of Sjögren's syndrome cases. Sjögren's syndrome is a multisystem exocrinopathy characterised by dry mouth and dry eyes with wide-ranging extraglandular involvement. The exocrine manifestations of Sjögren's syndrome affect the mouth, eyes, nose, ears, skin, vagina and the entire respiratory and gastrointestinal systems. The nonexocrine involvement may include the joints, thyroid gland, liver, kidneys and the musculoskeletal, vascular and central nervous systems. Currently, the mechanism(s) of development and progression of Sjögren's syndrome is/are not clear. Inflammation and lymphocytic infiltration of the exocrine glands is a classical feature of Sjögren's syndrome. During the progression of the disease, the acinar cells of the exocrine glands are replaced by fibrosis, rendering the glands nonfunctional. Sjögren's syndrome remains one of the most underdiagnosed conditions, particularly in the elderly population, because the cardinal sicca symptoms, which are the hallmark of the disease, are frequently attributed to aging and/or medications, which consequently delays the diagnosis. This delay in diagnosis imposes significant physical, psychological and economic burdens on elderly patients. The diagnosis of Sjögren's syndrome requires evaluation of both the exocrine and nonexocrine components of the disease. Management of Sjögren's syndrome requires collaboration by the primary-care physician, rheumatologist, ophthalmologist and dentist. This article reviews current understanding of the clinical manifestations, diagnosis and treatment of Sjögren's syndrome with special emphasis on the oral component of the disease.

  2. Plant-derived chimeric virus particles for the diagnosis of primary Sjögren syndrome

    Directory of Open Access Journals (Sweden)

    Elisa eTinazzi

    2015-12-01

    Full Text Available Plants are ideal for the production of protein-based nanomaterials because they synthesize and assemble complex multimeric proteins that cannot be expressed efficiently using other platforms. Plant viruses can be thought of as self-replicating proteinaceous nanomaterials generally stable and easily produced in high titers. We used Potato virus X (PVX chimeric virus particles (CVPs and Cowpea mosaic virus (CPMV empty virus-like particles (eVLPs to display a linear peptide (lipo derived from human lipocalin , which is immunodominant in Sjögren’s syndrome (SjS and is thus recognized by autoantibodies in SjS patient serum. These virus-derived nanoparticles (VNPs were thus used to develop a diagnostic assay for SjS based on a direct enzyme linked immunosorbent assay (ELISA format. We found that PVX-lipo formulations were more sensitive than the chemically synthesized immunodominant peptide and equally specific when used to distinguish between healthy individuals and SjS patients. Our novel assay therefore allows the diagnosis of SjS using a simple, low-invasive serum test, contrasting with the invasive labial biopsy required for current tests. Our results demonstrate that nanomaterials based on plant viruses can be used as diagnostic reagents for SjS, and could also be developed for the diagnosis of other diseases.

  3. Plant-Derived Chimeric Virus Particles for the Diagnosis of Primary Sjögren Syndrome.

    Science.gov (United States)

    Tinazzi, Elisa; Merlin, Matilde; Bason, Caterina; Beri, Ruggero; Zampieri, Roberta; Lico, Chiara; Bartoloni, Elena; Puccetti, Antonio; Lunardi, Claudio; Pezzotti, Mario; Avesani, Linda

    2015-01-01

    Plants are ideal for the production of protein-based nanomaterials because they synthesize and assemble complex multimeric proteins that cannot be expressed efficiently using other platforms. Plant viruses can be thought of as self-replicating proteinaceous nanomaterials generally stable and easily produced in high titers. We used Potato virus X (PVX), chimeric virus particles, and Cowpea mosaic virus, empty virus-like particles to display a linear peptide (lipo) derived from human lipocalin, which is immunodominant in Sjögren's syndrome (SjS) and is thus recognized by autoantibodies in SjS patient serum. These virus-derived nanoparticles were thus used to develop a diagnostic assay for SjS based on a direct enzyme linked immunosorbent assay format. We found that PVX-lipo formulations were more sensitive than the chemically synthesized immunodominant peptide and equally specific when used to distinguish between healthy individuals and SjS patients. Our novel assay therefore allows the diagnosis of SjS using a simple, low-invasive serum test, contrasting with the invasive labial biopsy required for current tests. Our results demonstrate that nanomaterials based on plant viruses can be used as diagnostic reagents for SjS, and could also be developed for the diagnosis of other diseases.

  4. Sjögren syndrome: what and where are we looking for?

    Science.gov (United States)

    de Paiva, Cintia S; Rocha, Eduardo Melani

    2015-11-01

    Sjögren's syndrome affects exocrine glands leading to a dry mouth and dry eyes. Dry eye manifestations can precede the diagnosis of Sjögren's syndrome by many years. Innumerous spontaneous and inducible Sjögren's syndrome models have been used to study the pathogenesis of Sjögren's syndrome. This review focuses on recent human data, ocular and extraglandular manifestations of animal models, what is known, what is still unknown and how we need to look, and their correlation correspondence to human disease. Hallmarks of dry eye in Sjögren's syndrome include increased corneal staining, goblet cell loss and low tear volume. Confocal microscopy and impression cytology are able to clarify new markers of the ocular disease. Extraglandular manifestations should be an alert more severe complications in the eye. Some models have strong sex and exocrine gland predilection, whereas aging generally worsens the disease phenotype. Although most models do not display a significant increase in corneal staining or tear secretion impairment, conjunctival infiltration and decrease in goblet cells are frequently seen. We have seen great advances in the role of inflammation in ocular, oral and extra-glandular manifestations of Sjögren's syndrome. Several mechanisms and mediators of Sjögren's syndrome have been elucidated in animal model studies.

  5. Hammarby Sjöstad in Stockholm a model case?

    DEFF Research Database (Denmark)

    Grønlund, Bo

    2010-01-01

    early test case in the 1990s. The result was very low crime rates providing some evidence that planning and design mattered to reduce crime and feelings of un-safety. This work resulted in the thoroughly worked-out major guide for housing areas 'Bo Tryggt' in 2001, revised in 2005 with inclusion......Starting in Denmark in the 1980s and followed by initiatives in Sweden since the 1990s, several guidelines for Crime Prevention through urban planning and building design have been developed in the Nordic welfare countries. Standardisation institutes, national crime prevention councils and some...... of a process approach. The presentation will discuss the challenges of putting in practice the guidelines of Crime Prevention Through Urban Planning and Building Design in Hammarby Sjöstad, Stockholm....

  6. Facilitering i de sjællandske lokale energispareudvalg

    DEFF Research Database (Denmark)

    Christensen, Hanne Broen

    2001-01-01

    Formålet med denne rapport er på baggrund af en kortlægning af de sjællandske lokale energispareudvalg samt en SWOT-analyse, en aktøranalyse og en teoretisk diskussion at belyse, hvordan de lokale energispareudvalg ved hjælp af en facilitator kan støttes i deres arbejde med at opfylde målene, der...... er på baggrund af SWOT-analysen identificeret seks problemer hos udvalgene. Det er udvalgets manglende identitet, manglende konkrete projektforslag, uenighed om geografiske områder, at deltagerne ikke kender hinandenn, frygt for skæve magtforhold i udvalgene og manglende visioner. Aktøranalysen har...

  7. Vitamin D and Sjögren syndrome.

    Science.gov (United States)

    Garcia-Carrasco, Mario; Jiménez-Herrera, Erick Alejandro; Gálvez-Romero, Jose Luis; de Lara, Luis Vázquez; Mendoza-Pinto, Claudia; Etchegaray-Morales, Ivet; Munguía-Realpozo, Pamela; Ruíz-Argüelles, Alejandro; Jose, Rosas; Vera-Recabarren, Mauricio; Cervera, Ricard

    2017-06-01

    The immunomodulatory effects of vitamin D have been extensively studied in the context of autoimmunity. Multiple studies have demonstrated a high prevalence of vitamin D deficiency in autoimmune diseases. Recently, a possible protective role of vitamin D in autoimmunity has been described; however, this function remains controversial. Few studies have investigated the role of vitamin D in patients with Sjögren syndrome (SS). In this review, we compiled the main features of SS pathogenesis, the vitamin D immunomodulatory effects and the possible interaction between both. Data suggests that vitamin D may play a role in the SS pathogenesis. In addition, vitamin D low levels have been found in SS patients, which are associated with extra-glandular manifestations, such as lymphoma or neuropathy, suggesting a possible benefit effect of vitamin D in SS. Copyright © 2017 Elsevier B.V. All rights reserved.

  8. Diagnostic markers of Sjögren's syndrome.

    Science.gov (United States)

    Witte, Torsten

    2010-01-01

    Establishing a diagnosis of Sjögren's syndrome (SS) has been difficult without sensitive laboratory markers, and in light of the non-specificity of the symptoms of dry eyes and mouth. Rather than complaints about dry eyes or dry mouth, objective symptoms and extraglandular manifestations should raise suspicion of SS. This evaluation requires the detection of antibodies against Ro (SSA) and La (SSB) or a pathologic salivary gland biopsy. Since an invasive biopsy is not always performed, further diagnostic markers are required. Recently antibodies against alpha-fodrin have been shown to be present in the majority of untreated patients, and can be used in the screening process of SS as an additional marker. Copyright 2010 S. Karger AG, Basel.

  9. Innate immunity in Sjögren's syndrome.

    Science.gov (United States)

    Kiripolsky, Jeremy; McCabe, Liam G; Kramer, Jill M

    2017-09-01

    Sjögren's syndrome (SS) is an autoimmune disease of exocrine tissue that primarily affects women. Although patients typically experience xerostomia and xerophthalmia, numerous systemic disease manifestations are seen. Innate immune hyperactivity is integral to many autoimmune diseases, including SS. Results from SS mouse models suggest that innate immune dysregulation drives disease and this is a seminal event in SS pathogenesis. Findings in SS patients corroborate those in mouse models, as innate immune cells and pathways are dysregulated both in exocrine tissue and in peripheral blood. We will review the role of the innate immune system in SS pathogenesis. We will discuss the etiology of SS with an emphasis on innate immune dysfunction. Moreover, we will review the innate cells that mediate inflammation in SS, the pathways implicated in disease, and the potential mechanisms governing their dysregulation. Finally, we will discuss emerging therapeutic approaches to target dysregulated innate immune signaling in SS. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Manliga och kvinnliga teknologers självbilder och deras stereotypbilder av teknologer

    Directory of Open Access Journals (Sweden)

    Charlotte Soneson

    2013-01-01

    Full Text Available Självbild och bilden av en typisk teknolog undersöktes hos civilingenjörsstudenter på LTH med personlighetstestet Adjective Check List. Vi fann stora skillnader mellan självbild och stereotypbild. Undersökningen visade också att dessa skillnader till viss del såg olika ut bland manliga och kvinnliga studenter, och skillnaden mellan kvinnornas genomsnittliga självbild och deras genomsnittliga bild av en typisk teknolog var då större än motsvarande skillnad bland männen.

  11. Juvenile Sjögren’s syndrome: Case report

    Directory of Open Access Journals (Sweden)

    Sušić Gordana

    2013-01-01

    Full Text Available Introduction. Sjögren’s syndrome (SS is an autoimmune disease of unknown etiology, clinically manifested by dry eyes (xerophthalmia and dry mouth (xerostomia. In childhood SS is a rare disease, clinically atypically or asymptomatic and is often unrecognized. We report a girl with asymptomatic, juvenile form of primary Sjögren’s syndrome (JSS. Case Outline. A 13­year­old girl was initially observed for several months due to elevated sedimentation rate (ESR 75­90 mm/h without signs of inflammation or other symptoms and disease signs. Subjective symptoms of dryness of the eyes and mouth were absent at the beginning. Ophthalmologic examination demonstrated hypolacrimia although the patients had no subjective signs of xerophthalmia. Ultrasonography (US revealed mild enlargement and heterogeneity of large salivary glands parenchyma. Increased rheumatoid factor (RF, anti SS­A/Ro, anti SS­B/La antibodies were found in serum. Ophthalmologic examination demonstrated decreased lacrimation. JSS was confirmed on the basis of ophthalmologic examination, immunological tests, histological findings of biopsy of small and US of major salivary glands. During a 12­years follow­up period systemic or extraglandular manifestations of JSS and other autoimmune diseases were not observed. Conclusion. Our experience suggests that in the differential diagnosis of unexplained elevated ESR the primary form of JSS should be also taken into consideration. Ultrasonographic changes of major salivary glands in the absence of symptoms of xerostomia point out that this noninvasive method has an important role in the diagnosis and management of patients with JSS.

  12. Virtual Touch Quantification of the Salivary Glands for Diagnosis of Primary Sjögren Syndrome.

    Science.gov (United States)

    Chen, Shaoqi; Wang, Yukai; Chen, Shaoxing; Wu, Qiulin; Chen, Shigao

    2016-12-01

    To investigate the value of salivary gland stiffness measured by Virtual Touch quantification (VTQ; Siemens Medical Solutions, Erlangen, Germany) for assessment of primary Sjögren syndrome. Fifty-four patients with primary Sjögren syndrome, 35 patients without primary Sjögren syndrome (patients with dry mouth and dry eye symptoms), and 52 healthy control volunteers were included in this study. Patients with primary Sjögren syndrome were classified as early or advanced stage by labial gland biopsies. All participants underwent B-mode sonography, on which the salivary glands (parotid and submandibular) were identified and VTQ measurements of shear wave velocity (SWV) were obtained. The diagnostic performance of SWV was evaluated by sensitivity and specificity at the optimum cutoff point and the area under the receiver operating characteristic curve. For submandibular glands, the mean SWV ± SD values were 2.25 ± 0.34 m/s in patients with early-stage primary Sjögren, 1.84 ± 0.20 m/s in patients without primary Sjögren syndrome, and 1.82 ± 0.27 m/s in healthy controls (Psyndrome from those without Sjögren syndrome and healthy controls, the sensitivity and specificity were 77.1% and 85.4% and 79.2% and 83.9%, respectively. For parotid glands, the SWV values were 2.78 ± 0.82 m/s in patients with early-stage primary Sjögren syndrome, 1.93 ± 0.33 m/s, in patients without primary Sjögren syndrome, and 1.85 ± 0.31 m/s in healthy controls (P syndrome from those without Sjögren syndrome and healthy controls, the sensitivity and specificity were 89.3 % and 75.3% and 91.4% and 80.0%. The VTQ technique might be a useful noninvasive strategy for assessment of salivary glands in the early stage of primary Sjögren syndrome. © 2016 by the American Institute of Ultrasound in Medicine.

  13. Schistosoma japonicum egg specific protein SjE16.7 recruits neutrophils and induces inflammatory hepatic granuloma initiation.

    Directory of Open Access Journals (Sweden)

    Chenyun Wu

    2014-02-01

    Full Text Available Neutrophils are known to play a major role in the egg granulomatous lesions caused by Schistosoma japonicum, but the precise mechanism by which eggs recruit or active neutrophil is unknown. Here we report S. japonicum egg specific EF-hand protein-SjE16.7 is a potent neutrophil recruiter and initiates the egg associated inflammatory granuloma in schistosomiasis. We show that the expression of SjE16.7 at level of both mRNA and protein is restricted to the egg stage. It locates in the miracidium and subshell area of the egg and can be secreted by the egg. The antigenic properties of SjE16.7 strongly suggest a role for SjE16.7 as an egg-derived molecule involved in host-parasite interactions. To study SjE16.7 functions in vivo, we challenged murine air pouch with recombinant SjE16.7. The results showed SjE16.7 trigged more inflammatory cell infiltration than vehicle or control protein. Using peritoneal exudate neutrophils from mice, we found that SjE16.7 significantly induced neutrophil chemotaxis in vitro, and the observed phenotypes were associated with enhanced Rac GTPase activation in SjE16.7 treated cells. Finally, in vivo hepatic granuloma formation model showed SjE16.7 coupled beads recruited more inflammatory cell infiltration than control beads. Our findings suggest SjE16.7 is an important pathogenic factor derived from egg. By recruiting neutrophils and inducing local inflammation, SjE16.7 facilitates eggs to be excreted through gut tissues and also initiates pathology in the liver; therefore SjE16.7 is a possible target for the prevention and treatment of schistosomiasis.

  14. Sjögren-like pluriglandular exocrine insufficiency after drug-induced toxic epidermal necrolysis.

    OpenAIRE

    Sabán, J.; Pais, J. R.; Rodríguez, J. L.; Boixeda, D.

    1991-01-01

    We present the case of a patient that progressively developed xerophthalmia, xerostomia, cutaneous xerosis and exocrine pancreatic insufficiency 3 months after metamizole-induced toxic epidermal necrolysis. Though the association of Sjögren's syndrome and exocrine pancreatic impairment is well established, the Sjögren-like syndrome after drug-induced toxic epidermal necrolysis in association with such a wide exocrine glandular insufficiency has not been previously described, to our knowledge.

  15. Oral mucosal manifestations in primary and secondary Sj?gren syndrome and dry mouth syndrome

    OpenAIRE

    B?ochowiak, Katarzyna; Olewicz-Gawlik, Anna; Pola?ska, Adriana; Nowak-Gabryel, Michalina; Koci?cki, Jaros?aw; Witmanowski, Henryk; Sokalski, Jerzy

    2016-01-01

    Introduction : One of the most important symptoms of Sjögren syndrome is xerostomia. The oral cavity deprived of saliva and its natural lubricative, protective and antibacterial properties is prone to a number of unfavourable consequences. Aim : To present the most important lesions on the oral mucosa in primary and secondary Sjögren syndrome and in dry mouth syndrome. Material and methods: The study group comprised 55 patients including 52 women and 3 men aged 20–72 years (aver...

  16. Crohn's Disease Associated with Sweet's Syndrome and Sjögren's Syndrome Treated with Infliximab

    Directory of Open Access Journals (Sweden)

    Erina N. Foster

    2005-01-01

    Full Text Available The association of Crohn's disease (CD and Sweet's syndrome is rare and the presence of Sjögren's syndrome in Crohn's disease is even rarer, with only three reports found in the literature. We describe two cases of Crohn's disease associated with Sweet's syndrome, one of which is the first case of CD and Sweet's concomitantly associated with Sjögren's syndrome. Both cases responded rapidly to Infliximab therapy with complete resolution of the skin lesions.

  17. Sjögren's syndrome. Controversies and progress.

    Science.gov (United States)

    Fox, R I

    1997-09-01

    Diagnostic criteria for Sjögren's syndrome (SS) are required by both physicians and patients to (1) provide a rational basis for their symptoms, assess their prognosis, and guide therapy; (2) identify a group of patients who are most likely to share a common etiopathogenesis, in order to identify those genetic and environmental factors that are crucial in pathogenesis; (3) fill out the myriad medical insurance forms that require a diagnosis code; and (4) serve as a "shorthand" code that alerts specialists in different fields (oral medicine, ophthalmology, and a variety of specialists in internal medicine) to search for particular clinical problems found in the SS patient. The key question in this article is whether the term "Sjögren's syndrome" should apply to a rather restricted group of individuals (those with an autoimmune basis for exocrinopathy) or to a rather large group of individuals who share a similar symptom complex of dry eyes and mouth. Primary SS, as defined by San Diego criteria, is a systemic autoimmune disease that is characterized by keratoconjunctivitis sicca and xerostomia resulting from lymphocytic infiltrates of the lacrimal and salivary glands. The criteria for the diagnosis of SS continues to be controversial, leading to confusion in the clinical and research literature. It is important to distinguish SS (an idiopathic autoimmune process) from other processes including hepatitis C infection, retroviral infection, lymphoma, autonomic neuropathy, depression, primary fibromyalgia, and drug side effects that can result in sicca symptoms. Recent studies on pathogenesis of SS in human and animal models have examined the clonality of the T-cell infiltrates, the production of cytokines by lymphocytes and glandular epithelial cells, neuroendocrine and hormonal factors that affect glandular secretion, and the fine structure of antigens recognized by T cells and B cells. Studies in SS have allowed comparison of lymphocytes in blood and in the

  18. Sjögren, Wiedemann ja liivi keele sõnaraamat. Panus 19. sajandi teadusajalukku / Eberhard Winkler

    Index Scriptorium Estoniae

    Winkler, Eberhard, 1955-

    2009-01-01

    Uuritakse, milline võis olla kummagi teadlase osa esimese liivi keele sõnaraamatu koostamisel: Sjögren, Andreas Johan. Gesammelte Schriften. Band II. Theil I., Joh. Andreas Sjögren's Livische Grammatik nebst Sprachproben ; Band II. Theil II., Joh. Andreas Sjögren's livisch-deutsches und deutsch-livisches Wörterbuch / bearbeitet von Ferdinand Joh. Wiedemann. St. Petersburg, 1861

  19. Diagnostic Approaches to Sjögren’s Syndrome: a Literature Review and Own Clinical Experience

    Directory of Open Access Journals (Sweden)

    Pedro de Sousa Gomes

    2012-03-01

    Full Text Available Objectives: The purpose of present paper is to critically address the recent advances on diagnostic procedures of Sjögren’s syndrome, taking into account the attained local and systemic features of the disease. Material and Methods: A comprehensive review of the available literature regarding to the diagnostic approaches to Sjögren’s syndrome was conducted. Eligible studies were identified by searching the electronic literature PubMed, Medline, Embase, and ScienceDirect databases for relevant reports (last search update January 2012 combining the MESH heading term “Sjögren’s syndrome”, with the words "diagnosis, diagnostic procedures, salivary gland function, ocular tests, histopathology, salivary gland imaging, serology". The authors checked the references of the selected articles to identify additional eligible publications and contacted the authors, if necessary. Results: Presented article addresses the established diagnostic criteria for Sjögren’s syndrome and critically evaluates the most commonly used diagnostic procedures, presenting data from author’s own clinical experience. Diagnostic criteria for Sjögren’s syndrome are required both by healthcare professionals and patients, namely in order to provide a rational basis for the assessment of the symptoms, establish an individual disease prognosis, and orientate the therapeutic intervention. Conclusions: Sjögren’s syndrome is quite a common autoimmune disease of which the diagnosis and treatment are not easily established. Due to its systemic involvement, it can exhibit a wide range of clinical manifestations that contribute to confusion and delay in diagnosis. The use of proper diagnostic modalities will help to reduce the time to diagnosis and preserve the health and quality of life of patients with Sjögren’s syndrome.

  20. Sjögren's syndrome: An underdiagnosed condition in mixed connective tissue disease

    Directory of Open Access Journals (Sweden)

    Fany Solange Usuba

    2014-03-01

    Full Text Available OBJECTIVE: To determine the prevalence of sicca symptoms, dry eye, and secondary Sjögren's syndrome and to evaluate the severity of dry eye in patients with mixed connective tissue disease. METHODS: In total, 44 consecutive patients with mixed connective tissue disease (Kasukawa's criteria and 41 healthy controls underwent Schirmer's test, a tear film breakup time test, and ocular surface staining to investigate dry eye. In addition, the dry eye severity was graded. Ocular and oral symptoms were assessed using a structured questionnaire. Salivary gland scintigraphy was performed in all patients. Classification of secondary Sjögren's syndrome was assessed according to the American-European Consensus Group criteria. RESULTS: The patients and controls had comparable ages (44.7±12.4 vs. 47.2±12.2 years and frequencies of female gender (93 vs. 95% and Caucasian ethnicity (71.4 vs. 85%. Ocular symptoms (47.7 vs. 24.4% and oral symptoms (52.3 vs. 9.7% were significantly more frequent in patients than in controls. Fourteen (31.8% patients fulfilled Sjögren's syndrome criteria, seven of whom (50% did not have this diagnosis prior to study inclusion. A further comparison of patients with mixed connective tissue disease with or without Sjögren's syndrome revealed that the former presented significantly lower frequencies of polyarthritis and cutaneous involvement than did the patients without Sjögren's syndrome. Moderate to severe dry eye was found in 13 of 14 patients with mixed connective tissue disease and Sjögren's syndrome (92.8%. CONCLUSIONS: Sjögren's syndrome, particularly with moderate to severe dry eye, is frequent in patients with mixed connective tissue disease. These findings alert the physician regarding the importance of the appropriate diagnosis of this syndrome in such patients.

  1. Oral mucosal manifestations in primary and secondary Sjögren syndrome and dry mouth syndrome

    Directory of Open Access Journals (Sweden)

    Katarzyna Błochowiak

    2016-02-01

    Full Text Available Introduction : One of the most important symptoms of Sjögren syndrome is xerostomia. The oral cavity deprived of saliva and its natural lubricative, protective and antibacterial properties is prone to a number of unfavourable consequences. Aim : To present the most important lesions on the oral mucosa in primary and secondary Sjögren syndrome and in dry mouth syndrome. Material and methods: The study group comprised 55 patients including 52 women and 3 men aged 20–72 years (average: 28.25 years. Results : Basing on the accepted criteria, primary Sjögren syndrome was diagnosed in 22 (40% patients, secondary Sjögren syndrome in 18 (32.7% patients, and dry mouth syndrome in 15 (27.27% patients. The physical examination and the examination of the mouth were performed and history was elicited from every patient. Conclusions : The most common pathologies appearing on the oral mucosa in primary and secondary Sjögren syndrome are angular cheilitis, cheilitis, increased lip dryness as well as non-specific ulcerations, aphthae and aphthoid conditions.

  2. Oral mucosal manifestations in primary and secondary Sjögren syndrome and dry mouth syndrome.

    Science.gov (United States)

    Błochowiak, Katarzyna; Olewicz-Gawlik, Anna; Polańska, Adriana; Nowak-Gabryel, Michalina; Kocięcki, Jarosław; Witmanowski, Henryk; Sokalski, Jerzy

    2016-02-01

    One of the most important symptoms of Sjögren syndrome is xerostomia. The oral cavity deprived of saliva and its natural lubricative, protective and antibacterial properties is prone to a number of unfavourable consequences. To present the most important lesions on the oral mucosa in primary and secondary Sjögren syndrome and in dry mouth syndrome. The study group comprised 55 patients including 52 women and 3 men aged 20-72 years (average: 28.25 years). Basing on the accepted criteria, primary Sjögren syndrome was diagnosed in 22 (40%) patients, secondary Sjögren syndrome in 18 (32.7%) patients, and dry mouth syndrome in 15 (27.27%) patients. The physical examination and the examination of the mouth were performed and history was elicited from every patient. The most common pathologies appearing on the oral mucosa in primary and secondary Sjögren syndrome are angular cheilitis, cheilitis, increased lip dryness as well as non-specific ulcerations, aphthae and aphthoid conditions.

  3. Posttranslational Protein Modification in the Salivary Glands of Sjögren’s Syndrome Patients

    Directory of Open Access Journals (Sweden)

    Rafael Herrera-Esparza

    2013-01-01

    Full Text Available The present study investigated posttranslational reactions in the salivary glands of patients with Sjögren’s syndrome. We analysed the biopsies of primary Sjögren’s patients using immunohistochemistry and a tag-purified anticyclic citrullinated protein (CCP antibody to detect citrullinated peptides, and the presence of peptidylarginine deiminase 2 (PAD2 was assessed simultaneously. The present work demonstrated the weak presence of the PAD2 enzyme in some normal salivary glands, although PAD2 expression was increased considerably in Sjögren’s patients. The presence of citrullinated proteins was also detected in the salivary tissues of Sjögren’s patients, which strongly supports the in situ posttranslational modification of proteins in this setting. Furthermore, the mutual expression of CCP and PAD2 suggests that this posttranslational modification is enzyme dependent. In conclusion, patients with Sjögren’s syndrome expressed the catalytic machinery to produce posttranslational reactions that may result in autoantigen triggering.

  4. Aging and Immunopathology in Primary Sjögren's Syndrome.

    Science.gov (United States)

    Bouma, Hjalmar R; Bootsma, Hendrika; van Nimwegen, Jolien F; Haacke, Erlin A; Spijkervet, Fred K; Vissink, Arjan; Kroese, Frans G M

    2015-01-01

    Sicca complaints (sensation of dry mouth and/or eyes) are present in about a quarter of the individuals above the age of 65 years old and are mainly due to medication. However, physiological changes that occur during aging might also lead to a diminished glandular function. These age-related changes are, at least in part, to be the consequence of decreased androgen levels. In addition to these physiological effects that occur during normal aging, sicca complaints can also be caused by Sjögren's syndrome (SS): a systemic auto-inflammatory disorder mainly affecting exocrine glands. Genetic factors, lowered levels of gonadal hormones and (viral) infections appear to contribute to the etiology of the syndrome. The incidence of SS is higher among aged individuals, which might be due to earlier diagnosis, as the onset of SS in an individual with age-related exocrine gland dysfunction lowers the threshold for sicca complaints. On the other hand, physiological aging might be considered as a risk factor for development of SS, resulting in a faster development of the syndrome. Differentiating physiological sicca complaints from SS in the elderly can be challenging, since apparently healthy individuals might present with auto-antibodies and lymphocytic infiltrates in salivary glands might be present as well. The drop in the level of androgens and estrogens upon aging, immunosenescence and pro-inflammatory features of the aging immune system may all contribute to the etiology of pSS in the elderly. In this review, we describe the physiological effects of aging and the influence of SS on exocrine gland morphology and function.

  5. Magnetic Resonance Spectroscopy in Sjögren-Larsson Syndrome

    Directory of Open Access Journals (Sweden)

    Fesih Aktar

    2016-06-01

    Full Text Available Sjögren-Larsson syndrome (SLS is a rare neurocutane­ous disease showing an autosomal recessive transmis­sion due to a lack of fatty acid aldehyde dehydrogenase. Spastic diplegia or triplegia, mental retardation and con­genital lamellar ichthyosis are the major findings of the disease. The syndrome may be accompanied by various eye and teeth features, skeletal system anomaly, speak­ing defects, hypertelorism and epilepsy. A 9-month male patient has been hospitalized for convulsion and flaking on body. The patient history showed that flaking skin thickening and peeling was started at the birth, and he suffered a right-side focused seizure when he was three month-old and he was treated with phenobarbital and car­bamazepine upon the epilepsy diagnosis. Wide ichthyo­sis, hypertelorism and bilateral simian line were observed in the physical examination. Bilateral punctuate lesions in cornea, pigment epithelial atrophy in the right eye and esotropia in the left eye have been determined during the eye examination. An epiteliform anomaly has been ob­served in the left hemisphere by electroencephalography. In brain magnetic resonance imaging (MRI, an increase in cerebral-cerebellar brain parenchyma and T1-T2 relax­ation time and in the signal in corpus callosum (delayed myelination have been determined. With the observa­tion of the white matter in centrum semi oval using brain MRI spectroscopy, signs of a sphingolipid peak at 1.3 ppm have been observed. An SLS diagnosis has been proposed upon clinical and laboratory observations. We want to emphasize on the fact that in epilepsy cases with ichthyosis, SLS should be considered.

  6. Primary biliary cirrhosis complicated by transverse myelitis in a patient without Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Micheli A

    2005-01-01

    Full Text Available Transverse myelitis is an acute inflammatory process, affecting one or more segments of the spinal cord. Its association with primary biliary cirrhosis has been documented in only four cases - all along with Sjögren's syndrome. Herein, we report for the first time, a patient who developed recurrent acute transverse myelitis in association with primary biliary cirrhosis without any clinical or histological indication of Sjögren's syndrome. A 42-year-old woman with primary biliary cirrhosis developed acute onset quadriparesis and urinary retention. Diagnostic evaluation excluded the presence of Sjögren's syndrome, other autoimmune syndromes, infections and multiple sclerosis. Magnetic resonance imaging of the spinal cord disclosed signal intensity abnormalities from C1 to T2 after gadolinium enhancement. As diagnosis of acute transverse myelitis was prominent, the patient was treated with intravenous methylprednisolone. The patient had a fair outcome despite an early recurrence of the symptoms after treatment withdrawal.

  7. Intrathecal Baclofen Therapy for the Treatment of Spasticity in Sjögren-Larsson Syndrome.

    Science.gov (United States)

    Hidalgo, Eveline Teresa; Orillac, Cordelia; Hersh, Andrew; Harter, David H; Rizzo, William B; Weiner, Howard L

    2017-01-01

    Intrathecal baclofen therapy is widely accepted as a treatment option for patients with severe spasticity. The current treatment of spasticity in patients with Sjögren-Larsson syndrome is largely symptomatic, given that no effective causal therapy treatments are available. We report the outcome of 2 patients with Sjögren-Larsson syndrome who had pump implantation for intrathecal baclofen. We observed a positive response, with a decrease of spasticity, reflecting in the Modified Ashworth Scale, and parents and caregivers observed a functional improvement in both patients. One patient experienced skin irritation 15 months after surgery, necessitating pump repositioning. No infection occurred. Our report shows that intrathecal baclofen therapy can have a positive therapeutic effect on spasticity in patients with Sjögren-Larsson syndrome, and therefore may be a promising addition to current treatments.

  8. [Stevens-Johnson syndrome followed by Gougerot-Sjögren syndrome].

    Science.gov (United States)

    de Roux Serratrice, C; Serratrice, J; Granel, B; Weiller-Merli, C; Rey, J; Lepidi, H; Disdier, P; Weiller, P J

    2001-03-24

    Sicca syndrome after Stevens-Johnson syndrome is classically described. However, to our knowledge, authentic Sjögren's syndrome following epidermal necrolysis has not been described previously. A 36-year-old woman with Steven-Johnson syndrome developed transient hepatitis and a persistent sicca syndrome. Fourteen years later an authentic Sjögren's syndrome was identified with presence of antinuclear and anti-SSA antibodies and lymphocytes infiltration of the accessory salivary glands, i.e. grade IV disease in the Chisholm classification. The initial mucosal destruction observed in our patients may have laid the ground for Sjögren's syndrome via abnormal antigen presentation in a predisposed dysimmune context.

  9. Histoplasmosis diseminada, linfopenia, y síndrome de Sjögren Disseminated histoplasmosis, lymphopenia and Sjögren´s syndrome

    Directory of Open Access Journals (Sweden)

    Horacio F. Rodrigo

    2012-10-01

    Full Text Available El síndrome de Sjögren es una enfermedad autoinmune caracterizada por disminución de las secreciones de las glándulas exocrinas; puede presentar también diversas alteraciones hemáticas, entre ellas linfopenia. Presentamos el caso de un varón de 28 años que consultó por cefalea de un mes de evolución a la que se agregaron en las últimas 48 horas vómitos y fiebre. Presentaba lesiones en piel de tronco y cara; no tenía rigidez de nuca. Se demostró infección por Histoplasma capsulatum var. capsulatum en piel y líquido cefalorraquídeo, linfopenia, anticuerpos anti Ro-SSA positivos, baja concentración del trazador en centellograma de glándulas salivales e infiltración linfocitaria en glándulas salivales, lo que permitió confirmar al diagnóstico de síndrome de Sjögren. El tratamiento con anfotericina liposomal e itraconazol mejoró el cuadro clínico. Comunicamos este caso para referir que una infección oportunista, como la histoplasmosis diseminada, puede ser una forma poco común de presentación del síndrome de Sjögren.Sjögren's syndrome is an autoimmune disease characterized by decreased exocrine gland secretions; patients may also present several hematological abnormalities, like lymphopenia. We describe the case of a 28 year old man who complained of headache a month of duration, with fever and vomiting in the last 48 hours. He also presented skin lesions on trunk and face, without neck stiffness. The diagnosis of duration infection was confirmed by culture from the skin biopsy and spinal fluid specimens; in addition, the presence of lymphopenia, positive anti Ro-SSA antibodies, poor concentration of the tracer in scintigraphy and lymphocytic infiltration in salivary glands confirmed the diagnosis of Sjögren's syndrome. The patient was successfully treated with liposomal amphotericin and itraconazole. We report this case to emphasize that opportunistic infections, such as disseminated histoplasmosis, may be an

  10. 75 FR 10245 - S.J. Energy Partners, Inc.; Supplemental Notice That Initial Market-Based Rate Filing Includes...

    Science.gov (United States)

    2010-03-05

    ... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ER10-735-000] S.J. Energy Partners, Inc.; Supplemental Notice That Initial Market-Based Rate Filing Includes Request for Blanket... proceeding of S.J. Energy Partners, Inc.'s application for market-based rate authority, with an accompanying...

  11. Physical activity and physical activity cognitions are potential factors maintaining fatigue in patients with primary Sjögren's syndrome

    NARCIS (Netherlands)

    Van Leeuwen; Bossema; Kruize; Geenen; Bijlsma; Bootsma; MD E.J.M. Wouters

    2012-01-01

    Background Fatigue is a prevalent and debilitating problem in Sjögren's syndrome. It has been suggested that physical activity and cognitions about physical activity can influence fatigue. Objective The aim of this study was to examine fatigue and physical activity levels in patients with Sjögren's

  12. SjTPdb: integrated transcriptome and proteome database and analysis platform for Schistosoma japonicum

    Directory of Open Access Journals (Sweden)

    Wang Zhi-Qin

    2008-06-01

    Full Text Available Abstract Background Schistosoma japonicum is one of the three major blood fluke species, the etiological agents of schistosomiasis which remains a serious public health problem with an estimated 200 million people infected in 76 countries. In recent years, enormous amounts of both transcriptomic and proteomic data of schistosomes have become available, providing information on gene expression profiles for developmental stages and tissues of S. japonicum. Here, we establish a public searchable database, termed SjTPdb, with integrated transcriptomic and proteomic data of S. japonicum, to enable more efficient access and utility of these data and to facilitate the study of schistosome biology, physiology and evolution. Description All the available ESTs, EST clusters, and the proteomic dataset of S. japonicum are deposited in SjTPdb. The core of the database is the 8,420 S. japonicum proteins translated from the EST clusters, which are well annotated for sequence similarity, structural features, functional ontology, genomic variations and expression patterns across developmental stages and tissues including the tegument and eggshell of this flatworm. The data can be queried by simple text search, BLAST search, search based on developmental stage of the life cycle, and an integrated search for more specific information. A PHP-based web interface allows users to browse and query SjTPdb, and moreover to switch to external databases by the following embedded links. Conclusion SjTPdb is the first schistosome database with detailed annotations for schistosome proteins. It is also the first integrated database of both transcriptome and proteome of S. japonicum, providing a comprehensive data resource and research platform to facilitate functional genomics of schistosome. SjTPdb is available from URL: http://function.chgc.sh.cn/sj-proteome/index.htm.

  13. Sex differential association of dermatomyositis with Sjögren syndrome.

    Science.gov (United States)

    Tseng, Chia-Chun; Chang, Shun-Jen; Tsai, Wen-Chan; Ou, Tsan-Teng; Wu, Cheng-Chin; Sung, Wan-Yu; Hsieh, Ming-Chia; Yen, Jeng-Hsien

    2017-02-06

    Although dermatomyositis and Sjögren syndrome share serologic autoantibodies and genetic polymorphisms, population data about the incidence of Sjögren syndrome in patients with dermatomyositis is unavailable. We performed a nationwide cohort study to explore the potential relation between dermatomyositis and Sjögren syndrome and, if an association exists, to elucidate whether it varies by sex. We identified all patients with newly diagnosed dermatomyositis from the Registry of Catastrophic Illness Database in Taiwan between Jan. 1, 1998, and Dec. 31, 2011. Each patient was matched to, at most, 5 control patients from the National Health Insurance Research Database by age, sex and entry date. Cox regression was used to calculate the hazard ratio (HR) and 95% confidence interval (CI) of Sjögren syndrome after adjusting for age, sex, rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis. A total of 1602 patients with dermatomyositis and 7981 control patients were enrolled in the study. There was a positive association of having Sjögren syndrome among patients with dermatomyositis after adjusting for age, sex, rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis (HR 2.67, 95% CI 2.01-3.54). The association was more pronounced in the male cohort (HR 2.69, 95% CI 1.19-6.09). We found a sex differential association of Sjögren syndrome among patients with dermatomyositis independent of age and concomitant autoimmune disease. Further studies are required to determine the clinical importance of this association for both outcomes and therapeutic options. © 2017 Canadian Medical Association or its licensors.

  14. SjAPI, the First Functionally Characterized Ascaris-Type Protease Inhibitor from Animal Venoms

    Science.gov (United States)

    Yang, Weishan; Cao, Zhijian; Zhuo, Renxi; Li, Wenxin; Wu, Yingliang

    2013-01-01

    Background Serine protease inhibitors act as modulators of serine proteases, playing important roles in protecting animal toxin peptides from degradation. However, all known serine protease inhibitors discovered thus far from animal venom belong to the Kunitz-type subfamily, and whether there are other novel types of protease inhibitors in animal venom remains unclear. Principal Findings Here, by screening scorpion venom gland cDNA libraries, we identified the first Ascaris-type animal toxin family, which contains four members: Scorpiops jendeki Ascaris-type protease inhibitor (SjAPI), Scorpiops jendeki Ascaris-type protease inhibitor 2 (SjAPI-2), Chaerilus tricostatus Ascaris-type protease inhibitor (CtAPI), and Buthus martensii Ascaris-type protease inhibitor (BmAPI). The detailed characterization of Ascaris-type peptide SjAPI from the venom gland of scorpion Scorpiops jendeki was carried out. The mature peptide of SjAPI contains 64 residues and possesses a classical Ascaris-type cysteine framework reticulated by five disulfide bridges, different from all known protease inhibitors from venomous animals. Enzyme and inhibitor reaction kinetics experiments showed that recombinant SjAPI was a dual function peptide with α-chymotrypsin- and elastase-inhibiting properties. Recombinant SjAPI inhibited α-chymotrypsin with a Ki of 97.1 nM and elastase with a Ki of 3.7 μM, respectively. Bioinformatics analyses and chimera experiments indicated that SjAPI contained the unique short side chain functional residues “AAV” and might be a useful template to produce new serine protease inhibitors. Conclusions/Significance To our knowledge, SjAPI is the first functionally characterized animal toxin peptide with an Ascaris-type fold. The structural and functional diversity of animal toxins with protease-inhibiting properties suggested that bioactive peptides from animal venom glands might be a new source of protease inhibitors, which will accelerate the development of

  15. Draft genome sequence of a caprolactam degrader bacterium: Pseudomonas taiwanensis strain SJ9.

    Science.gov (United States)

    Hong, Sung-Jun; Park, Gun-Seok; Khan, Abdur Rahim; Jung, Byung Kwon; Shin, Jae-Ho

    Pseudomonas taiwanensis strain SJ9 is a caprolactam degrader, isolated from industrial wastewater in South Korea and considered to have the potential for caprolactam bioremediation. The genome of this strain is approximately 6.2 Mb (G+C content, 61.75%) with 6,010 protein-coding sequences (CDS), of which 46% are assigned to recognized functional genes. This draft genome of strain SJ9 will provide insights into the genetic basis of its caprolactam-degradation ability. Copyright © 2016 Sociedade Brasileira de Microbiologia. Published by Elsevier Editora Ltda. All rights reserved.

  16. Draft genome sequence of a caprolactam degrader bacterium: Pseudomonas taiwanensis strain SJ9

    Directory of Open Access Journals (Sweden)

    Sung-Jun Hong

    Full Text Available Abstract Pseudomonas taiwanensis strain SJ9 is a caprolactam degrader, isolated from industrial wastewater in South Korea and considered to have the potential for caprolactam bioremediation. The genome of this strain is approximately 6.2 Mb (G + C content, 61.75% with 6,010 protein-coding sequences (CDS, of which 46% are assigned to recognized functional genes. This draft genome of strain SJ9 will provide insights into the genetic basis of its caprolactam-degradation ability.

  17. Genetic control of disease in an experimental model for Sjögren's syndrome

    DEFF Research Database (Denmark)

    Andersson, Åsa Inga Maria

    2009-01-01

    susceptibility in Sjögren's syndrome, studies of disease phenotypes have been performed in the non-obese diabetic (NOD) mouse. By the identification of genetic regions controlling development of autoimmune exocrinopathy in the NOD mouse and by reducing one of these regions considerably, Nguyen et al. in a recent......Sjögren's syndrome is an autoimmune disease with a complex etiology depending on hereditary and environmental factors. The disease is characterized by lymphocytic infiltration and inflammation in the salivary and lacrimal glands, leading to oral and ocular dryness. To understand the genetic...

  18. SjAPI, the first functionally characterized Ascaris-type protease inhibitor from animal venoms.

    Directory of Open Access Journals (Sweden)

    Zongyun Chen

    Full Text Available BACKGROUND: Serine protease inhibitors act as modulators of serine proteases, playing important roles in protecting animal toxin peptides from degradation. However, all known serine protease inhibitors discovered thus far from animal venom belong to the Kunitz-type subfamily, and whether there are other novel types of protease inhibitors in animal venom remains unclear. PRINCIPAL FINDINGS: Here, by screening scorpion venom gland cDNA libraries, we identified the first Ascaris-type animal toxin family, which contains four members: Scorpiops jendeki Ascaris-type protease inhibitor (SjAPI, Scorpiops jendeki Ascaris-type protease inhibitor 2 (SjAPI-2, Chaerilus tricostatus Ascaris-type protease inhibitor (CtAPI, and Buthus martensii Ascaris-type protease inhibitor (BmAPI. The detailed characterization of Ascaris-type peptide SjAPI from the venom gland of scorpion Scorpiops jendeki was carried out. The mature peptide of SjAPI contains 64 residues and possesses a classical Ascaris-type cysteine framework reticulated by five disulfide bridges, different from all known protease inhibitors from venomous animals. Enzyme and inhibitor reaction kinetics experiments showed that recombinant SjAPI was a dual function peptide with α-chymotrypsin- and elastase-inhibiting properties. Recombinant SjAPI inhibited α-chymotrypsin with a Ki of 97.1 nM and elastase with a Ki of 3.7 μM, respectively. Bioinformatics analyses and chimera experiments indicated that SjAPI contained the unique short side chain functional residues "AAV" and might be a useful template to produce new serine protease inhibitors. CONCLUSIONS/SIGNIFICANCE: To our knowledge, SjAPI is the first functionally characterized animal toxin peptide with an Ascaris-type fold. The structural and functional diversity of animal toxins with protease-inhibiting properties suggested that bioactive peptides from animal venom glands might be a new source of protease inhibitors, which will accelerate the

  19. Search for D{sub (sJ)}(2632) at BaBar

    Energy Technology Data Exchange (ETDEWEB)

    Aubert, B.

    2004-08-17

    The authors performed a search for the D{sub sJ}*(2632){sup +} state recently reported by the SELEX Collaboration at FNAL. This preliminary analysis makes use of an integrated luminosity of 125 fb{sup -1} collected by the BABAR detector at the PEP-II asymmetric-energy e{sup +}e{sup -} collider. The resulting D{sub s}{sup +}{eta} and D{sup 0}K{sup +} mass spectra show no evidence for the D{sub sJ}*(2632){sup +} state. In addiition, no signal is observed in the D*{sup +} K{sub S} mass spectrum.

  20. Contemporary management and full mouth rehabilitation of a patient with Sjögren syndrome.

    Science.gov (United States)

    Fisselier, Francois; Comut, A Alper

    2017-12-16

    Sjögren syndrome is a chronic autoimmune disorder with xerostomia, increased tooth wear, high rates of caries, and repeated failure of dental restorations as the main oral symptoms. These render the prosthetic treatment challenging. This clinical report describes a contemporary approach to the treatment of a patient with Sjögren syndrome using translucent multilayered monolithic zirconia restorations and focuses on the treatment protocols before, during, and after the prosthetic treatment. Copyright © 2017 Editorial Council for the Journal of Prosthetic Dentistry. Published by Elsevier Inc. All rights reserved.

  1. Kulturelle Fyrtårne i Region Sjælland

    DEFF Research Database (Denmark)

    Sørensen, Flemming; Kjølbæk, Anita Schou; Bærenholdt, Jørgen Ole

    2010-01-01

    Rapporten analyserer Region Sjællands kulturelle attraktioners betydning socialt og økonomisk. Analysen er foretaget på baggrund af 1) en definitionsmæssig afklaring af begreberne 'kulturelle attraktioner' og 'kulturelle fyrtårne' og en teoretisk diskussion af kulturelle attraktioners betydning; 2......) en kortlægning og analyse af kulturelle attraktioner i Region Sjælland; 3) en case analyse af forskellige typer kulturelle attraktioner; samt 4) en sammenfattende analyse. Analysen indikerer at regionens kulturelle attraktioners potentialer knytter sig til forskellige geografiske og administrative...

  2. Diagnóstico e tratamento da síndrome de Sjögren Sjögren's syndrome: diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Sergio Felberg

    2006-12-01

    Full Text Available A síndrome de Sjögren (SS é doença sistêmica inflamatória crônica, de provável etiologia auto-imune, com distribuição mundial. Seu quadro clínico é responsável por considerável impacto na qualidade de vida de seus portadores. O objetivo deste artigo é descrever seus principais sintomas, os critérios atualmente usados para seu diagnóstico e as modalidades terapêuticas disponíveis até o momento.Sjögren's syndrome is a systemic inflammatory autoimmune disease with worldwide distribution, responsible for considerable impact on the patient's quality of life. The aim of this article is to describe its main symptoms, the currently used different diagnostic criteria and the available treatment for the syndrome.

  3. Pediatric primary Sjögren syndrome presenting with bilateral ranulas: A case report and systematic review of the literature.

    Science.gov (United States)

    Means, Casey; Aldape, Mark A; King, Ericka

    2017-10-01

    Primary Sjögren syndrome is uncommon in children, and the standard clinical criteria used in diagnosis of adult Sjögren syndrome will miss many children with the disease. Floor of mouth ranulas have not been described in Sjögren syndrome. This study aims to describe a novel presentation of juvenile primary Sjögren syndrome, and to present a comprehensive systematic review of the literature regarding the presentation and diagnosis of Sjögren syndrome in children. Ovid MEDLINE. A MEDLINE literature search was performed using the following search terms: primary, Sjögren, disease, and children. Results were limited to human subjects and articles written in English between 1981 and 2014. Applicable articles were reviewed and qualitatively summarized. Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PRIMA). Initial MEDLINE search yielded 146 articles, 80 of which were excluded as not clinically pertaining to Sjögren syndrome. An additional 25 were excluded due to lack of pediatric-specific data. Systematic review of the literature revealed no reports of ranula in association with Sjögren syndrome. 6 papers were manually included from review of reference lists of included articles. Our review indicated that recurrent parotitis is the most commonly reported presenting symptom in children, followed by ocular and oral symptoms, musculoskeletal, and renal symptoms. Compared to adults, children are less likely to present with dry eyes and mouth. All studies were retrospective chart reviews, case series or case reports. This is the first report of a child presenting with floor of mouth ranulas in association with Sjögren syndrome. While recurrent parotitis is the most common presentation in children, other salivary gland and extra-salivary manifestations may be seen, and the clinician must maintain a high index of suspicion for underlying Sjögren syndrome. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. A novel assay for the prenatal diagnosis of Sjögren-Larsson syndrome

    NARCIS (Netherlands)

    van den Brink, D. M.; van Miert, J. M.; Wanders, R. J. A.

    2005-01-01

    Sjögren-Larsson syndrome (SLS) is a metabolic disorder characterized by ichthyosis, mental retardation and spastic diplegia or tetraplegia. The biochemical defect has been identified as a deficiency of fatty aldehyde dehydrogenase (FALDH), which is part of an enzyme complex that converts fatty

  5. Dry Eyes and Mouth? You May Have Sjögren's Syndrome

    Science.gov (United States)

    ... Unexplained Cases of Allergic Reactions Linked to Red Meat Reducing Children’s Chances of Asthma Surviving Sepsis Looking at Lupus Wise Choices Easing Sjögren’s Symptoms Take sips of water for dry mouth. Use sugar-free candies and gums. Use artificial tears for dry eyes. Use a humidifier for ...

  6. The burden of Sjögren's syndrome, dryness and fatigue

    NARCIS (Netherlands)

    van Leeuwen, N.

    2014-01-01

    Dryness and fatigue are prevalent and debilitating symptoms in Sjögren’s syndrome. In this thesis, consequences of dryness for patients’ well-being and daily life functioning, and possible targets for treatment were described. In the first part of this thesis, an encompassing overview of the

  7. Pelvic floor dysfunction in female Sjögren's syndrome: an 8-year audit.

    Science.gov (United States)

    Budden, Aaron K; Te West, Nèvine I D; Sturgess, Allan D; Moore, Kate H

    2016-09-01

    The classic triad of dry eyes, mouth and vagina is known to most gynaecologists as pathognomonic of Sjögren's syndrome, but rheumatologists seldom consider vaginal symptoms. Our hypothesis was that women with Sjögren's syndrome would have an increased likelihood of postoperative voiding dysfunction, severe vaginal stenosis or poor response to anticholinergics compared with the general urogynaecology patient. All patients with Sjögren's syndrome were prospectively recorded from July 2007 to June 2015. Presenting complaint, pelvic examination findings, previous/subsequent pelvic surgery, voiding dysfunction and response to anticholinergics were noted. The denominator, all new urogynaecology patients, was prospectively recorded. Fifteen patients were identified over 8 years (0.5 % of 2794 new presentations). Of the seven patients who had previously undergone surgery elsewhere, all had demonstrable pelvic tissue fibrosis; five had such severe fibrosis that no speculum could be passed. Anticholinergic medications were completely intolerable in 10/11 (91 %) women, and severe postoperative voiding dysfunction occurred in 6/9 (67 %) women. Only 2/15 (13 %) women were unaffected by fibrosis, postoperative voiding dysfunction or intolerance to anticholinergics. This audit demonstrates a substantial risk of vaginal stenosis, postoperative voiding dysfunction or severe intolerance to anticholinergics in women with Sjögren's syndrome.

  8. Clinical practice guidelines for oral management of Sjögren disease: Dental caries prevention.

    Science.gov (United States)

    Zero, Domenick T; Brennan, Michael T; Daniels, Troy E; Papas, Athena; Stewart, Carol; Pinto, Andres; Al-Hashimi, Ibtisam; Navazesh, Mahvash; Rhodus, Nelson; Sciubba, James; Singh, Mabi; Wu, Ava J; Frantsve-Hawley, Julie; Tracy, Sharon; Fox, Philip C; Ford, Theresa Lawrence; Cohen, Stephen; Vivino, Frederick B; Hammitt, Katherine M

    2016-04-01

    Salivary dysfunction in Sjögren disease can lead to serious and costly oral health complications. Clinical practice guidelines for caries prevention in Sjögren disease were developed to improve quality and consistency of care. A national panel of experts devised clinical questions in a Population, Intervention, Comparison, Outcomes format and included use of fluoride, salivary stimulants, antimicrobial agents, and nonfluoride remineralizing agents. The panel conducted a systematic search of the literature according to pre-established parameters. At least 2 members extracted the data, and the panel rated the strength of the recommendations by using a variation of grading of recommendations, assessment, development, and evaluation. After a Delphi consensus panel was conducted, the experts finalized the recommendations, with a minimum of 75% agreement required. Final recommendations for patients with Sjögren disease with dry mouth were as follows: topical fluoride should be used in all patients (strong); although no study results link improved salivary flow to caries prevention, the oral health community generally accepts that increasing saliva may contribute to decreased caries incidence, so increasing saliva through gustatory, masticatory, or pharmaceutical stimulation may be considered (weak); chlorhexidine administered as varnish, gel, or rinse may be considered (weak); and nonfluoride remineralizing agents may be considered as an adjunct therapy (moderate). The incidence of caries in patients with Sjögren disease can be reduced with the use of topical fluoride and other preventive strategies. Copyright © 2016 American Dental Association. Published by Elsevier Inc. All rights reserved.

  9. Management of xerostomia and other complications of Sjögren's syndrome.

    Science.gov (United States)

    Pinto, Andres

    2014-02-01

    This article provides an overview of the published literature in English in the past 63 years involving the management of xerostomia and other oral complications of Sjögren's syndrome. The evidence supporting studied interventions was evaluated using published criteria. Copyright © 2014 Elsevier Inc. All rights reserved.

  10. Salivary gland dysfunction and xerostomia in Sjögren's syndrome

    DEFF Research Database (Denmark)

    Jensen, Siri Beier; Vissink, Arjan

    2014-01-01

    In this article, salivary gland dysfunction and xerostomia in Sjögren's syndrome (SS) are discussed, with a focus on the pathophysiology of salivary dysfunction in SS, the clinical presentation of dry mouth in SS, how to assess salivary gland hypofunction and xerostomia in SS, and the impact...

  11. Optimizing dry mouth treatment for individuals with Sjögren's syndrome.

    Science.gov (United States)

    Wu, Ava J

    2008-11-01

    A hallmark of the oral component of Sjögren's syndrome (SS) is the complaint of dry mouth thought to be secondary to dysfunction of the salivary glands. This article describes how treatment may be optimized for individuals who have dry mouth.

  12. Pilot clinical trial of dehydroepiandrosterone (DHEA) versus placebo for Sjögren's syndrome

    NARCIS (Netherlands)

    Pillemer, Stanley R.; Brennan, Michael T.; Sankar, Vidya; Leakan, Rose Anne; Smith, Janine A.; Grisius, Margaret; Ligier, Sophie; Radfar, Lida; Kok, Marc R.; Kingman, Albert; Fox, Philip C.

    2004-01-01

    To screen for potential efficacy and assess feasibility and safety of dehydroepiandrosterone (DHEA) as a treatment for Sjögren's syndrome (SS). A 24-week randomized, double-blinded, pilot trial of oral DHEA (200 mg/day) versus placebo was conducted. The primary comparison was to a hypothesized 20%

  13. Sjögren's syndrome and acquired splenic atrophy with septic shock: a case report.

    Science.gov (United States)

    Santos, Natacha; Silva, Rui; Rodrigues, Joana; Torres-Costa, José

    2014-01-06

    The most frequent causes of adult-onset recurrent infections are human immunodeficiency virus infection, malignancy, and autoimmune diseases, while acquired non-surgical hyposplenism is rare. Although acquired asplenia/hyposplenism have been described in association with celiac disease and, less frequently, with autoimmune diseases such as Sjögren's syndrome, the manifestations in this context are usually only detectable in the laboratory setting, with Howell-Jolly bodies or thrombocytosis. To the best of our knowledge, no previous case of pneumococcal septic shock in a patient with acquired hyposplenism and co-morbid Sjögren's syndrome has been reported. We report a case of a 45-year-old Caucasian woman who developed pneumococcal pneumonia at age 42 years, pneumococcal meningitis at age 44 years and septic shock with Streptococcus agalactiae bacteremia at age 45 years and was subsequently diagnosed with radiological splenic atrophy and functional asplenia, as well as primary Sjögren's syndrome. After appropriate immunizations, the patient has been free from clinically important infections. Hyposplenism should be suspected in patients with adult-onset infections caused by encapsulated bacteria, especially if autoantibodies are present. Early diagnosis can help prevent potentially life-threatening infections. Possible associations between splenic atrophy and Sjögren's syndrome are discussed.

  14. Sjögren’s syndrome and acquired splenic atrophy with septic shock: a case report

    Science.gov (United States)

    2014-01-01

    Introduction The most frequent causes of adult-onset recurrent infections are human immunodeficiency virus infection, malignancy, and autoimmune diseases, while acquired non-surgical hyposplenism is rare. Although acquired asplenia/hyposplenism have been described in association with celiac disease and, less frequently, with autoimmune diseases such as Sjögren’s syndrome, the manifestations in this context are usually only detectable in the laboratory setting, with Howell-Jolly bodies or thrombocytosis. To the best of our knowledge, no previous case of pneumococcal septic shock in a patient with acquired hyposplenism and co-morbid Sjögren’s syndrome has been reported. Case presentation We report a case of a 45-year-old Caucasian woman who developed pneumococcal pneumonia at age 42 years, pneumococcal meningitis at age 44 years and septic shock with Streptococcus agalactiae bacteremia at age 45 years and was subsequently diagnosed with radiological splenic atrophy and functional asplenia, as well as primary Sjögren’s syndrome. After appropriate immunizations, the patient has been free from clinically important infections. Conclusion Hyposplenism should be suspected in patients with adult-onset infections caused by encapsulated bacteria, especially if autoantibodies are present. Early diagnosis can help prevent potentially life-threatening infections. Possible associations between splenic atrophy and Sjögren’s syndrome are discussed. PMID:24393211

  15. What have we learned from clinical trials in primary Sjögren's syndrome about pathogenesis?

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.; Vissink, Arjan; Kroese, Frans G. M.; Abdulahad, Wayel H.; Bootsma, Hendrika

    2011-01-01

    In vitro and in vivo experimental data have pointed to new immunopathogenic mechanisms in primary Sjögren's syndrome (pSS). The availability of targeted treatment modalities has opened new ways to selectively target these mechanistic pathways in vivo. This has taught us that the role of

  16. L'atteinte nerveuse au cours du syndrome de gougerot - sjögren, a ...

    African Journals Online (AJOL)

    -Sjögren se voient dans 8,5 à 70 % des cas. Les données de la littérature concernant le système nerveux périphérique sont nombreuses et assez concordantes. En revanche les données concernant les manifestations centrales sont ...

  17. Sjældne medfødte sygdomme og deres orale manifestationer

    DEFF Research Database (Denmark)

    Lauridsen, Eva; Gjørup, Hans

    2018-01-01

    Behandlingen af patienter med medfødte sjældne sygdomme kan have orale, dentale eller kraniofaciale associerede afvigelser og være meget kompleks. Her beskrives kliniske og radiologiske karakteristika og principper for behandling af patienter med ektodermal dysplasi, cleidocranial dysplasi, osteo...

  18. OCD og orofaciale dyskinesier forårsaget af sjælden basalganglielidelse

    DEFF Research Database (Denmark)

    Elmgreen, Søren Bruno; Danielsen, Erik Hvid

    2014-01-01

    Bevægeforstyrrelser hos psykiatriske patienter skyldes sædvanligvis ekstrapyramidale bivirkninger til antipsykotisk medicin. Her beskrives en sjælden årsag til orofaciale dyskinesier, tungedystoni og koreiforme bevægelser hos en 31-årig mand i behandling for obsessiv-kompulsiv lidelse (OCD)....

  19. Physical fatigue characterises patient experience of primary Sjögren's syndrome

    NARCIS (Netherlands)

    Arends, Suzanne; Meiners, Petra M; Moerman, Rada V; Kroese, Frans G M; Brouwer, Elisabeth; Spijkervet, Frederik K L; Vissink, Arjan; Bootsma, Hendrika

    2017-01-01

    OBJECTIVES: Besides ocular and oral dryness, fatigue is a major symptom in patients with primary Sjögren's syndrome (pSS). Our aim was to investigate the importance of fatigue in relation to other symptoms experienced as well as to evaluate the effect of rituximab treatment on fatigue in pSS

  20. Characterization and risk estimate of cancer in patients with primary Sjögren syndrome

    Directory of Open Access Journals (Sweden)

    Pilar Brito-Zerón

    2017-04-01

    Full Text Available Abstract Background The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren syndrome (SjS. Methods We had analyzed the development of cancer in 1300 consecutive patients fulfilling the 2002 SjS classification criteria. The baseline clinical and immunological characteristics and systemic activity (ESSDAI scores were assessed at diagnosis as predictors of cancer using Cox proportional hazards regression analysis adjusted for age at diagnosis and gender. The sex-and age-specific standardized incidence ratios (SIR of cancer were estimated from 2012 Spanish mortality data. Results After a mean follow-up of 91 months, 127 (9.8% patients developed 133 cancers. The most frequent type of cancer was B-cell lymphoma (including 27 MALT and 19 non-MALT B-cell lymphomas. Systemic activity at diagnosis of primary SjS correlated with the risk of hematological neoplasia and cryoglobulins with a high risk of either B-cell or non-B-cell lymphoma subtypes. Patients with cytopenias had a high risk of non-MALT B-cell and non-B-cell cancer, while those with low C3 levels had a high risk of MALT lymphomas and those with monoclonal gammopathy and low C4 levels had a high risk of non-MALT lymphomas. The estimated SIR for solid cancer was 1.13 and 11.02 for hematological cancer. SIRs for specific cancers were 36.17 for multiple myeloma and immunoproliferative diseases, 19.41 for Hodgkin lymphoma, 6.04 for other non-Hodgkin lymphomas, 5.17 for thyroid cancer, 4.81 for cancers of the lip and oral cavity, and 2.53 for stomach cancer. Conclusions One third of cancers developed by patients with primary SjS are B-cell lymphomas. The prognostic factors identified at SjS diagnosis differed according to the subtype of B-cell lymphoma developed. Primary SjS is also associated with the development of some non-hematological cancers (thyroid, oral cavity, and stomach.

  1. [Necrotizing vasculitis of the panarteritis nodosa type in a long-course primary Sjögren's syndrome].

    Science.gov (United States)

    Lahoz Rallo, C; Arribas López, J R; Arnalich Fernández, F; Monereo Alonso, A; Llanos Chavarri, M C; Camacho Siles, J

    1990-10-01

    Vasculitis is a complication happening in a third of patients with primary Sjögren's Syndrome. It can be of 2 types: neutrophilic or mononuclear and sometimes mixed. Very occasionally, a necrotizing vasculitis polyarteritis nodosa type during the evolution of Sjögren's Syndrome has appeared. A case of a female patient with a Sjögren's Syndrome of large evolution, who suddenly showed a poly-systemic affliction (nervous system, kidneys, muscles and digestive system) secondary to a necrotizing vasculitis type polyarteritis nodosa with a good response to immunosuppressors, is presented.

  2. Bronquiectasias associadas à síndrome de Sjögren Bronchiectasis associated to Sjögren syndrome: case report

    Directory of Open Access Journals (Sweden)

    SIMONE APARECIDA CÂMARA TECCHIO

    2000-08-01

    Full Text Available Diversas formas de comprometimento pulmonar têm sido descritas na síndrome de Sjögren, incluindo raros casos em que bronquiectasias foram o achado predominante. Relata-se o caso de uma paciente com história de infecções respiratórias de repetição e dispnéia progressiva de longa evolução, cuja tomografia computadorizada de alta resolução evidenciava inúmeras bronquiectasias. A revisão de uma biópsia pulmonar realizada no início dos sintomas mostrava alterações bronquiolares de natureza inflamatória e fibrótica. Apesar da presença de sintomas de síndrome seca há anos, o diagnóstico de SS foi feito apenas tardiamente. São discutidos os possíveis mecanismos patogenéticos que possam ter levado ao desenvolvimento das bronquiectasias e a necessidade de um apurado senso clínico para o diagnóstico precoce de tais condições.Different kinds of pulmonary impairment have been described in Sjögren syndrome, including rare cases of bronchiectasis. The authors report a female patient with a history of episodes of respiratory infections and progressive breathlessness whose high resolution computerized tomography revealed bronchiectasis. A former open lung biopsy showed bronchiolar inflammatory and fibrotic changes. The diagnosis of Sjögren syndrome was made only late in the evolution, although sicca syndrome symptoms had been present for years. The authors discuss the potential pathogenic mechanisms involved in the development of the bronchiectasis and the need for a high degree of clinical medical skill for the early diagnosis of such conditions.

  3. Characterization of VAMP2 in Schistosoma japonicum and the Evaluation of Protective Efficacy Induced by Recombinant SjVAMP2 in Mice.

    Directory of Open Access Journals (Sweden)

    Qian Han

    Full Text Available The outer-tegument membrane covering the schistosome is believed to maintain via the fusion of membranous vesicles. Fusion of biological membranes is a fundamental process in all eukaryotic cells driven by formation of trans-SNARE (soluble N-ethylmaleimide-sensitive factor attachment protein receptor complexes through pairing of vesicle associated v-SNAREs (VAMP with complementary t-SNAREs on target membranes. The purpose of this study was to characterize Schistosoma japonicum vesicle-associated membrane protein 2 (SjVAMP2 and to investigate its potential as a candidate vaccine against schistosomiasis.The sequence of SjVAMP2 was analyzed, cloned, expressed and characterized. SjVAMP2 is a member of the synaptobrevin superfamily harboring the v-SNARE coiled-coil homology domain. RT-PCR analysis revealed that significantly higher SjVAMP2 levels were observed in 14-, 28- and 42-day-old worms, and SjVAMP2 expression was much higher in 42-day-old female worms than in those male worms. Additionally, the expression of SjVAMP2 was associated with membrane recovery in PZQ-treated worms. Immunostaining assay showed that SjVAMP2 was mainly distributed in the sub-tegument of the worms. Western blotting revealed that rSjVAMP2 showed strong immunogenicity. Purified rSjVAMP2 emulsified with ISA206 adjuvant induced 41.5% and 27.3% reductions in worm burden, and 36.8% and 23.3% reductions in hepatic eggs in two independent trials. Besides, significantly higher rSjVAMP2-specific IgG, IgG1, IgG2a levels were detected in rSjVAMP2-vaccinated mice.Our study indicated that SjVAMP2 is a potential vaccine candidate against S. japonicum and provided the basis for further investigations into the biological function of SjVAMP2.

  4. The Importance of Minor Salivary Gland Biopsy in Sjögren Syndrome Diagnosis and the Clinicopathological Correlation.

    Science.gov (United States)

    Serin, Gürdeniz; Karabulut, Gonca; Kabasakal, Yasemin; Kandiloğlu, Gülşen; Akalin, Taner

    2016-01-01

    Minor salivary gland biopsy is one of the objective tests used in the diagnosis of Sjögren syndrome. The aim of our study was to compare the clinical and laboratory data of primary and secondary Sjögren syndrome cases with a lymphocyte score 3 and 4 in the minor salivary gland biopsy. Data from a total of 2346 consecutive minor salivary gland biopsies were retrospectively evaluated in this study. Clinical and autoantibody characteristics of 367 cases with lymphocyte score 3 or 4 and diagnosed with primary or secondary Sjögren syndrome were compared. There was no difference between lymphocyte score 3 and 4 primary Sjögren syndrome patients in terms of dry mouth, dry eye symptoms and Schirmer test results but Anti-Ro and Antinuclear Antibody positivity was statistically significantly higher in cases with lymphocyte score 4 (p= 0.025, p= 0.001). Anti-Ro test results were also found to be statistically significantly higher in secondary Sjögren syndrome patients with lymphocyte score 4 (p= 0.048). In this study, the high proportion of cases with negative autoantibody but positive lymphocyte score is significant in terms of showing the contribution of minor salivary gland biopsy to Sjögren syndrome diagnosis. Lymphocyte score 3 and 4 cases were found to have similar clinical findings but a difference regarding antibody positivity in primary Sjögren syndrome. We believe that cases with lymphocyte score 4 may be Sjögren syndrome cases whose clinical manifestations are relatively established and higher autoantibody levels are therefore found.

  5. Rectal mucosal prolapse syndrome as an unusual gastrointestinal manifestation of Sjögren's syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Koga Hideki

    2009-10-01

    Full Text Available Abstract Introduction Rectal mucosal prolapse syndrome, histologically characterized by fibromuscular obliteration in the lamina propria, hyperplastic glands and thickened muscularis mucosa, causes rectal bleeding. Sjögren's syndrome is an autoimmune exocrinopathy that chiefly destroys the salivary and lacrimal glands by lympho-plasmacytic infiltration. Although various gastrointestinal manifestations have been reported in patients with Sjögren's syndrome, there have not been to our knowledge any case reports to date of rectal mucosal prolapse syndrome in association with Sjögren's syndrome. Case presentation A 68-year-old Japanese woman with Sjögren's syndrome and long-term constipation consulted our hospital because of rectal bleeding. Because of dysphagia and xerostomia, she had consistently refused recommendations to take oral medicines including cathartics. Therefore, she frequently strained excessively during defecation. Colonoscopy and radiological examinations disclosed eroded flat protrusions of the rectum. Microscopic examination demonstrated inflamed mucosa with elongated tortuous glands and fibromuscular obliteration. Based on these findings, a diagnosis of rectal mucosal prolapse syndrome was made. Prohibition of straining during defecation and sulfasalazine suppository use were effective. Conclusion This case highlights the importance of defecation control in patients with Sjögren's syndrome. In the case presented, rectal mucosal prolapse syndrome following long-term excessive straining during defecation caused rectal bleeding. Clinicians should consider rectal mucosal prolapse syndrome as a gastrointestinal manifestation of Sjögren's syndrome.

  6. Samband mellan övervikt och självkänsla bland ungdomar

    OpenAIRE

    Fallenius, Katarina

    2012-01-01

    Barnens och ungdomarnas fysiska och psykiska välmående är ett viktigt delområde inom hälsovården. Inom den förebyggande vården finns utmaningar i och med barnens och ungdomarnas ökande övervikt och försämrade psykiska hälsa. Syftet med detta examensarbete var att kartlägga sambandet mellan ungdomars övervikt och självkänsla. Den centrala frågeställningen i arbetet var, precis som syftet, om det finns ett samband mellan ungdomars övervikt och självkänsla. Arbetet avgränsades till 11-15 åriga u...

  7. Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome.

    Science.gov (United States)

    Borie, Raphael; Schneider, Sophie; Debray, Marie-Pierre; Adle-Biasssette, Homa; Danel, Claire; Bergeron, Anne; Mariette, Xavier; Aubier, Michel; Papo, Thomas; Crestani, Bruno

    2011-01-01

    Sjögren's syndrome is a frequent auto-immune disorder with a pulmonary location in almost 10% of the patients. Although bronchial involvement is very common, most patients only complain of cough and this involvement rarely results in severe symptoms or chronic respiratory failure are rarely observed. We describe here 5 patients with severe chronic bronchiolitis revealing primary Sjögren's syndrome. The lung involvement resulted in chronic bronchorrhea, recurrent sinusitis, diffuse bronchiolar nodules with bronchiectasis on the CT scan, and a severe obstructive airway pattern on lung function tests. Improvement was obtained in 4 patients with combination of inhaled corticosteroids, inhaled long acting beta-agonists, and a low dose of erythromycin. Copyright © 2010 Elsevier Ltd. All rights reserved.

  8. The Sjögren’s syndrome – an interdisciplinary problem viewed by a dentist

    Directory of Open Access Journals (Sweden)

    Agata Barczyk

    2014-11-01

    Full Text Available Sjögren’s syndrome is common and chronic disease. So far there is no effective therapy. In 90% of cases, it affects women, usually in the peri-menopausal period. Many systems and organs may be involved in the course of the disease, thus it is interesting for many medical specialists. There are primary and secondary Sjögren’s syndromes, both characterized mainly by xerostomia and keratoconjunctivitis. The paper reviews basic symptoms in the oral cavity involving the oral mucosa, lips, tongue, gingiva, teeth and periodontium. Treatment and prevention in a dental clinic are also described. Lifestyle and pharmacotherapy to avoid symptom aggravation are additionally considered.

  9. Sjögren Syndrome and Other Causes of Sicca in Older Adults.

    Science.gov (United States)

    Baer, Alan N; Walitt, Brian

    2017-02-01

    Dry eye and dry mouth symptoms are each reported by up to 30% of persons more than 65 years of age, particularly in women. Medication side effects are the most common contributing factors. The evaluation of these symptoms requires measures of ocular and oral dryness. Sjögren syndrome is the prototypic disease associated with dryness of the eyes and mouth and predominantly affects women in their perimenopausal and postmenopausal years. In addition to topical treatment of the mucosal dryness, patients with Sjögren syndrome may require treatment with systemic immunomodulatory and immunosuppressive agents to manage a variety of extraglandular manifestations. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Solution to trouble of D{sub sJ} particles

    Energy Technology Data Exchange (ETDEWEB)

    Matsuki, Takayuki [Tokyo Kasei University, Itabashi, Tokyo (Japan)]. E-mail: matsuki@tokyo-kasei.ac.jp; Morii, Toshiyuki [Kobe University, Nada, (Japan). Faculty of Human Development]. E-mail: morii@kobe-u.ac.jp; Sudoh, Kazutaka [High Energy Accelerator Research Organization, Tsukuba, Ibaraki (Japan). Institute of Particle and Nuclear Studies]. E-mail: kazutaka.sudoh@kek.jp

    2005-07-01

    Recent discovery of D{sub sJ} particles, which are considered to be a great trouble by experimentalists as well as theorists, had already been resolved by our potential model proposed some time ago by two of us (T.M. and T.M.), in which the Hamiltonian and wave functions are expanded in 1/(heavy quark mass) respecting heavy quark symmetry. Using our model, we explain how narrow states like D{sub sJ} can be realized, predict properties of 0{sup +} and 1{sup +} states of B and B{sub s} heavy mesons, and interpret how global SU(3) symmetry seems to be recovered for these 0{sup +} and 1{sup +} heavy mesons. (author)

  11. Design and modeling of an SJ infrared solar cell approaching upper limit of theoretical efficiency

    Science.gov (United States)

    Sahoo, G. S.; Mishra, G. P.

    2018-01-01

    Recent trends of photovoltaics account for the conversion efficiency limit making them more cost effective. To achieve this we have to leave the golden era of silicon cell and make a path towards III-V compound semiconductor groups to take advantages like bandgap engineering by alloying these compounds. In this work we have used a low bandgap GaSb material and designed a single junction (SJ) cell with a conversion efficiency of 32.98%. SILVACO ATLAS TCAD simulator has been used to simulate the proposed model using both Ray Tracing and Transfer Matrix Method (under 1 sun and 1000 sun of AM1.5G spectrum). A detailed analyses of photogeneration rate, spectral response, potential developed, external quantum efficiency (EQE), internal quantum efficiency (IQE), short-circuit current density (JSC), open-circuit voltage (VOC), fill factor (FF) and conversion efficiency (η) are discussed. The obtained results are compared with previously reported SJ solar cell reports.

  12. Sjögren-Larsson syndrome: biochemical defects and follow up in three cases.

    Science.gov (United States)

    Auada, Mariam Patrícia; Taube, Maria Beatriz Puzzi; Collares, Edgard F; Tanaka, Ana Maria Uthida; Cintra, Maria Letícia

    2002-01-01

    Sjögren-Larsson syndrome is a rare disorder that consists of congenital ichthyosis and neurological symptoms due to an enzymatic defect of fatty aldehyde dehydrogenase in the fatty alcohol cycle. We report three cases of Sjögren-Larsson syndrome in which the primary biochemical defect was established in two patients (patient 2: 175 pmol/min/mg; patient 3: 103 pmol/min/mg protein enzimatic activity in fibroblasts in skin, while normal controls were 8,860 +/- 1,624, n: 22 ). A dietary program was proposed: reduced total fatty intake at 30% of total calories, n-3 and n-6 acids (canola oil) as well as unsaturated fatty acids (Milupan milk). Topical keratolytic agents were used too. Good clinical course was observed in one of the patients in whom dietary intervention was started in early infancy. In the other two patients, who started the therapy later (five and three years old), cutaneous symptoms were improved after treatment.

  13. Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren’s Syndrome

    Directory of Open Access Journals (Sweden)

    Go Makimoto

    2012-01-01

    Full Text Available Sjögren’s syndrome (SS is a systemic autoimmune disease characterized by sicca symptoms. Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common symptoms of pulmonary involvement in primary SjS, and they are rarely accompanied by serositis such as pleuritis or pericarditis. We report a case of SS presenting initially with bilateral pleural effusions. A 63-year old man was admitted to our hospital with a one-month history of cough, dyspnea, and right chest pain. Chest-computed tomography revealed bilateral pleural effusions. Serum anti-SS-A antibody titer was 1 : 256. Ophthalmological examination revealed a positive Schirmer test. Lip biopsy showed atrophy and plasmacytic infiltration of the salivary gland. Corticosteroid treatment was initiated. Pleural effusions were almost completely resolved by day 30. The patient has not experienced any recurrence.

  14. Detailed Analysis of the Articular Domain in Patients with Primary Sjögren Syndrome.

    Science.gov (United States)

    Moerman, Rada V; Arends, Suzanne; Meiners, Petra M; Vissink, Arjan; Spijkervet, Frederik K L; Kroese, Frans G M; Brouwer, Elisabeth; Bootsma, Hendrika

    2017-03-01

    We used the 28-joint Disease Activity Score (DAS28) and the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (ESSDAI) articular domain to assess the effect of rituximab (RTX) and abatacept (ABA) on articular involvement in primary Sjögren syndrome (pSS). Patients with pSS treated with RTX (n = 18) or ABA (n = 13) and having a DAS28 erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) level ≥ 3.2 at baseline were selected. Generalized estimating equations were used to analyze the DAS28 and ESSDAI articular domain over time. In the RTX group, DAS28-ESR/CRP decreased significantly up to 48 weeks. In the ABA group, DAS28-ESR/CRP decreased significantly up to 24 weeks. DAS28 correlated significantly with ESSDAI articular domain. DAS28 is useful to evaluate the effect of biologicals on articular involvement in patients with pSS.

  15. Undiagnosed Sjögren’s Syndrome Presenting as Mesenteric Panniculitis

    Directory of Open Access Journals (Sweden)

    Rebecca L. Burns

    2016-01-01

    Full Text Available Mesenteric panniculitis is a rare inflammatory and fibrotic process that affects the small intestine mesentery. It may occur following abdominal surgery or in association with a variety of conditions, including malignancy, infection, and certain autoimmune and inflammatory conditions. Herein, an unusual case of mesenteric panniculitis in a patient with primary Sjögren’s syndrome will be presented. The patient presented with abdominal pain, weight loss, sicca symptoms, fatigue, and arthralgia. An abdominal CT revealed mesenteric fat stranding and prominent lymph nodes of the small intestine mesentery. She was found on laboratory workup to have positive antinuclear and anti-SSa antibodies. Minor salivary gland lip biopsy revealed focal lymphocytic sialadenitis. The patient’s symptoms and CT findings improved with corticosteroids. This case suggests that Sjögren’s syndrome should be considered as an underlying disease process in the evaluation of patients with mesenteric panniculitis.

  16. Bronchial hyperresponsiveness to methacholine in patients with primary Sjögren's syndrome.

    OpenAIRE

    Gudbjörnsson, B; Hedenström, H; Stålenheim, G; Hällgren, R

    1991-01-01

    The prevalence of bronchial hyperresponsiveness (BHR) to methacholine inhalation in a consecutive series of 21 patients with primary Sjögren's syndrome was studied prospectively. Slight to severe BHR was seen in 12/20 (60%) of the patients. Ten of 12 patients with BHR (83%) had a non-productive cough, wheezing, or intermittent breathlessness. Bronchial hyperresponsiveness was more common in patients with extraglandular symptoms (10/14, 71%) than in those with only glandular symptoms (29%). Sp...

  17. Purpura vasculaire récidivant et syndrome de Sjögren: quel lien ...

    African Journals Online (AJOL)

    Le purpura hypergammaglobulinémique de Waldenstrom (PHGGW) est un syndrome caractérisé par un purpura vasculaire récidivant associé à une hypergammaglobulinémie. Il peut être isolé ou plus fréquemment associé à une hémopathie maligne ou à une connectivite essentiellement le syndrome de Gougerot Sjögren ...

  18. Draft Genome Sequence of Caprolactam-Degrading Pseudomonas putida Strain SJ3.

    Science.gov (United States)

    Hong, Sung-Jun; Park, Gun-Seok; Khan, Abdur Rahim; Jung, Byung Kown; Park, Yeong-Jun; Yoo, Na-Kyung; Lee, Changhee; Park, Choi Kyu; Shin, Jae-Ho

    2015-07-23

    Pseudomonas putida strain SJ3, which possesses caprolactam-degrading ability, was isolated from dyeing industry wastewater in Daegu, Republic of Korea. Here, we describe the draft genome sequence and annotation of the strain. The 5,596,765-bp-long genome contains 4,293 protein-coding genes and 68 RNA genes with 61.70% G+C content. Copyright © 2015 Hong et al.

  19. Salivary eicosanoid concentration in patients with Sjögren's syndrome.

    OpenAIRE

    Tishler, M; Yaron, I; Raz, A; Meyer, F A; Yaron, M

    1996-01-01

    OBJECTIVE: To investigate eicosanoid concentrations in the saliva of patients with primary Sjögren's syndrome (SS). METHODS: Whole mixed saliva of 36 subjects was assayed for eicosanoid concentrations using a radioimmunoassay. Patients with primary SS having positive lip biopsy served as the study group; their results were compared with data from patients with dry mouth and negative lip biopsy (dry mouth group), and with a group of normal healthy controls. RESULTS: Concentrations of thromboxa...

  20. Serologic features of primary Sjögren’s syndrome: clinical and prognostic correlation

    Science.gov (United States)

    García-Carrasco, Mario; Mendoza-Pinto, Claudia; Jiménez-Hernández, César; Jiménez-Hernández, Mario; Nava-Zavala, Arnulfo; Riebeling, Carlos

    2013-01-01

    Sjögren’s syndrome (SS) is a chronic inflammatory systemic autoimmune disease. The disease spectrum extends from sicca syndrome to systemic involvement and extraglandular manifestations, and SS may be associated with malignancies, especially non-Hodgkin’s lymphoma. Patients with SS present a broad spectrum of serologic features. Certain serological findings are highly correlated with specific clinical features, and can be used as prognostic markers. PMID:23525186

  1. A Case of Idiopathic Retroperitoneal Fibrosis Associated With Sjögren's Syndrome.

    Science.gov (United States)

    Droz, Nicole C; Mathew, Stephanie D

    2016-10-01

    After presenting with nonspecific symptoms, a 52-year-old female was found to have a 3.4 × 2.6 cm retroperitoneal mass resulting in hydronephrosis on abdominal computed tomography scan. After extensive workup including surgical debulking, excisional biopsy, and autoimmune panel, she was found to have idiopathic retroperitoneal fibrosis (IRF) with negative staining for immunoglogulin G4 (IgG4). Two years after presentation, the patient developed dyspnea, cough, and xerostomia. Repeat anti-Sjögren Syndrome A and anti-Sjögren Syndrome B titers and labial gland biopsy confirmed the diagnosis of concomitant Sjögren's syndrome (SS). The patient was treated with mycophenolate mofetil with improvement in her symptoms. Most cases of retroperitoneal fibrosis are associated with IgG4-related disease or other autoimmune disease. To our knowledge, this is only the second reported case of SS associated with IRF. Because symptoms of IRF are nonspecific, there is often a delay in diagnosis resulting in end-organ disease such as ureteral obstruction or hydronephrosis. Although IRF is uncommon, it should be considered in patients presenting with abdominal or flank pain, especially in patients with concomitant autoimmune disorders. Early recognition of disease can prevent end-organ damage and, as more cases are diagnosed, its relationship to SS may be elucidated leading to further advances in treatment and surveillance. Reprint & Copyright © 2016 Association of Military Surgeons of the U.S.

  2. Primary Biliary Cirrhosis and Primary Sjögren's Syndrome: Insights for the Stomatologist

    Directory of Open Access Journals (Sweden)

    Liliane Lins

    2014-08-01

    Full Text Available Primary biliary cirrhosis (PBC is a chronic progressive autoimmune disease characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Primary Sjögren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands, mainly the lachrymal and salivary glands, in the absence of other definitively diagnosed rheumatologic disease. We report a diagnosed case of primary Sjögren's syndrome associated with PBC. A 59-year-old Caucasian woman went to oral evaluation reporting dry mouth, difficulty in eating associated with burning mouth syndrome, dysgeusia and dysphagia. Intraoral examination revealed extensive cervical caries, gingivitis, gingival retraction, angular cheilitis and atrophic tongue. Hyposalivation was detected by salivary flow and Schirmer's test was positive. Antinuclear and antimitochondrial antibodies were both positive. Anti-Ro/SSA and anti-La/SSB antibodies were negative. A minor salivary gland biopsy of the lower lip was performed. Histopathologic analysis revealed lymphocytic infiltrate with destruction of salivary gland architecture in some areas and replacement of glandular tissues by mononuclear cells. Optimal management of PBC associated with Sjögren's syndrome requires a multidisciplinary approach as the key to optimal patient care. Dental practitioners should be able to recognize the clinical features of this associated condition. Appropriate dental care may prevent tooth decay, periodontal disease and oral infections as well as improve the patient's quality of life.

  3. Histoplasmosis diseminada, linfopenia, y síndrome de Sjögren

    Directory of Open Access Journals (Sweden)

    Horacio F. Rodrigo

    2012-10-01

    Full Text Available El síndrome de Sjögren es una enfermedad autoinmune caracterizada por disminución de las secreciones de las glándulas exocrinas; puede presentar también diversas alteraciones hemáticas, entre ellas linfopenia. Presentamos el caso de un varón de 28 años que consultó por cefalea de un mes de evolución a la que se agregaron en las últimas 48 horas vómitos y fiebre. Presentaba lesiones en piel de tronco y cara; no tenía rigidez de nuca. Se demostró infección por Histoplasma capsulatum var. capsulatum en piel y líquido cefalorraquídeo, linfopenia, anticuerpos anti Ro-SSA positivos, baja concentración del trazador en centellograma de glándulas salivales e infiltración linfocitaria en glándulas salivales, lo que permitió confirmar al diagnóstico de síndrome de Sjögren. El tratamiento con anfotericina liposomal e itraconazol mejoró el cuadro clínico. Comunicamos este caso para referir que una infección oportunista, como la histoplasmosis diseminada, puede ser una forma poco común de presentación del síndrome de Sjögren.

  4. The Image Processing of Droplet for Evaporation Experiment in SJ-10

    Science.gov (United States)

    Xue, Changbin; Feng, Yanhui; Yu, Qiang

    2017-06-01

    We have completed an experiment for droplet evaporation processing using Young-Laplace fitting, exponent fitting, polynomial fitting and ellipse fitting, which could be used for multiple shapes of droplets. The droplet evaporation experiment test was an important science experiment in SJ-10. In order to get the change process of the physical parameter, such as the touching edges and the droplet evaporation rate, we had gained the contour edge image of the droplet and used mathematic method to do the fitting analysis. The accuracy of the physical parameter was depended on the accuracy of the mathematic fitting. Using the original Young-Laplace fitting method could not process all the images of evaporation and liquid interface from the space experiment facility of SJ-10, especially the smaller droplet images. We could get more accurate contour fitting and result using the new method described in this article. This article proposes a complete solution, including edge detecting and contour fitting. In edge detecting, Canny detector was applied to extract droplet edge. In contour fitting, Young-Laplace fitting, exponent fitting, polynomial fitting and ellipse fitting are designed to fit the contour of droplets, which make the solution apply to all of droplets in SJ-10.

  5. A successful use of a new shuttle cloning vector pA13 for the cloning of the bacteriocins BacSJ and acidocin 8912

    Directory of Open Access Journals (Sweden)

    Kojić Milan

    2010-01-01

    Full Text Available The aim of this paper was to research the molecular cloning of genes encoding the novel bacteriocin BacSJ from Lactobacillus paracasei subsp. paracasei BGSJ2-8 by using a newly constructed shuttle cloning vector pA13. A new shuttle-cloning vector, pA13, was constructed and successfully introduced into Escherichia coli, Lactobacillus and Lactococcus strains, showing a high segregational and structural stability in all three hosts. The natural plasmid pSJ2-8 from L. paracasei subsp. paracasei BGSJ2-8 was cloned in the pA13 using BamHI, obtaining the construct pB5. Sequencing and in silico analysis of the pB5 revealed 15 open reading frames (ORF. Plasmid pSJ2-8 harbors the genes encoding the production of two bacteriocins, BacSJ and acidocin 8912. The combined N-terminal amino acid sequencing of BacSJ in combination with DNA sequencing of the bacSJ2-8 gene enabled the determination of the primary structure of a bacteriocin BacSJ. The production and functional expression of BacSJ in homologous and heterologous hosts suggest that bacSJ2-8 and bacSJ2-8i together with the genes encoding the ABC transporter and accessory protein are the minimal requirement for the production of BacSJ. Biochemical and genetic analyses showed that BacSJ belongs to the class II bacteriocins. The shuttle cloning vector pA13 could be used as a tool for genetic manipulations in lactobacilli and lactococci.

  6. Regenerating Gene Protein as a Novel Autoantigen in the Pathogenesis of Sjögren’s Syndrome

    Directory of Open Access Journals (Sweden)

    Takashi Fujimoto

    2015-12-01

    Full Text Available Sjögren’s syndrome, an autoimmune disease characterized by exocrine gland dysfunction leading to dry mouth and dry eye diseases, is typified by lymphoplasmacytic infiltrations and a progressive destruction of the salivary and lacrimal glands. Despite an ever-increasing focus on identifying the underlying etiology of Sjögren’s syndrome, the factors that initiate this autoimmune disease and the mechanisms that cause the subsequent exocrine gland dysfunction remain a mystery. The original explanatory concept for the pathogenesis of Sjögren’s syndrome proposed a specific, self-perpetuating, immune-mediated loss of acinar and ductal cells as the principal cause of salivary gland dysfunction. We highlight the possible involvement of regenerating gene (Reg in the regeneration and destruction of salivary gland acinar and ductal cells in Sjögren’s syndrome. The Reg gene was originally isolated as a gene specifically overexpressed in regenerating pancreatic islets and constitutes a growth factor family (Reg family. We describe how salivary gland dysfunction is initiated and maintained and how it can be regenerated or progressed, mediated by the Reg gene, Reg protein, and anti-REG autoantibodies in Sjögren’s syndrome.

  7. Matriptase Deletion Initiates a Sjögren’s Syndrome-Like Disease in Mice

    Science.gov (United States)

    Liu, Xibao; Swaim, William D.; Lai, Zhennan; Cabrera-Perez, Javier; Di Pasquale, Giovanni; Ambudkar, Indu S.; Bugge, Thomas H.; Chiorini, John A.

    2014-01-01

    Objective The objective of this study was to determine the effect of epithelial barrier disruption, caused by deficiency of the membrane-anchored serine protease, matriptase, on salivary gland function and the induction of autoimmunity in an animal model. Methods Embryonic and acute ablation of matriptase expression in the salivary glands of mice was induced, leading to decreased epithelial barrier function. Mice were characterized for secretory epithelial function and the induction of autoimmunity including salivary and lacrimal gland dysfunction, lymphocytic infiltration, serum anti-Ro/SSA, anti-La/SSB and antinuclear antibodies. Salivary glands immune activation/regulation, barrier function as well as tight junction proteins expression also were determined. Expression of matriptase in minor salivary gland biopsies was compared among pSS patients and healthy volunteers. Results Embryonic ablation of matriptase expression in mice resulted in the loss of secretory epithelial cell function and the induction of autoimmunity similar to that observed in primary Sjögren’s syndrome. Phenotypic changes included exocrine gland dysfunction, lymphocytic infiltrates, production of Sjögren’s syndrome-specific autoantibodies, and overall activation of the immune system. Acute ablation of matriptase expression resulted in significant salivary gland dysfunction in the absence of overt immune activation. Analysis of the salivary glands indicates a loss of electrical potential across the epithelial layer as well as altered distribution of a tight junction protein. Moreover, a significant decrease in matriptase gene expression was detected in the minor salivary glands of pSS patients compared with healthy volunteers. Conclusions Our findings demonstrate that local impairment of epithelial barrier function can lead to loss of exocrine gland dysfunction in the absence of inflammation while systemic deletion can induce a primary Sjögren’s syndrome like phenotype with

  8. Matriptase deletion initiates a Sjögren's syndrome-like disease in mice.

    Directory of Open Access Journals (Sweden)

    Hongen Yin

    Full Text Available The objective of this study was to determine the effect of epithelial barrier disruption, caused by deficiency of the membrane-anchored serine protease, matriptase, on salivary gland function and the induction of autoimmunity in an animal model.Embryonic and acute ablation of matriptase expression in the salivary glands of mice was induced, leading to decreased epithelial barrier function. Mice were characterized for secretory epithelial function and the induction of autoimmunity including salivary and lacrimal gland dysfunction, lymphocytic infiltration, serum anti-Ro/SSA, anti-La/SSB and antinuclear antibodies. Salivary glands immune activation/regulation, barrier function as well as tight junction proteins expression also were determined. Expression of matriptase in minor salivary gland biopsies was compared among pSS patients and healthy volunteers.Embryonic ablation of matriptase expression in mice resulted in the loss of secretory epithelial cell function and the induction of autoimmunity similar to that observed in primary Sjögren's syndrome. Phenotypic changes included exocrine gland dysfunction, lymphocytic infiltrates, production of Sjögren's syndrome-specific autoantibodies, and overall activation of the immune system. Acute ablation of matriptase expression resulted in significant salivary gland dysfunction in the absence of overt immune activation. Analysis of the salivary glands indicates a loss of electrical potential across the epithelial layer as well as altered distribution of a tight junction protein. Moreover, a significant decrease in matriptase gene expression was detected in the minor salivary glands of pSS patients compared with healthy volunteers.Our findings demonstrate that local impairment of epithelial barrier function can lead to loss of exocrine gland function [corrected] in the absence of inflammation while systemic deletion can induce a primary Sjögren's syndrome like phenotype with autoimmunity and loss of

  9. Oral findings in patients with primary Sjögren's syndrome and oral lichen planus - a preliminary study on the effects of bovine colostrum-containing oral hygiene products

    DEFF Research Database (Denmark)

    Pedersen, A.M.; Torpet, L.A.; Reibel, J.

    2002-01-01

    Primary Sjögren's syndrome, oral lichen planus, bovine colostrum, saliva, xerostomia, oral mucosa......Primary Sjögren's syndrome, oral lichen planus, bovine colostrum, saliva, xerostomia, oral mucosa...

  10. [Neurological manifestations of Gougerot and Sjögren disease: a Black African case].

    Science.gov (United States)

    Belo, M; Sama, H D; Nagbé, Y; Kumako, V; Bissa, H; Agoda-Koussema, L K; Balo, K P; Grunitzky, E K

    2015-10-01

    Primitive Gougerot-Sjögren syndrome (PGSS) is a systemic autoimmune exocrinopathy in which twenty percent of affected patients develop neurological manifestations. It has been little described in the Black African literature. We report a case of PGSS in a 64-year-old Black woman as revealed by neurological manifestations in an inflammatory context. Central neuropathy was present and the patient fulfilled at least four of the six Vitali American-European diagnostic criteria. There were abnormal aspects on the sylvian artery and hyperintense lesions of periventricular predominance. PGSS is a serious disease which we must consider when we encounter any unexplained neurological manifestations especially those associated with vasculitic lesions.

  11. Developing new behavior strategies of robot soccer team SjF TUKE Robotics

    Directory of Open Access Journals (Sweden)

    Mikuláš Hajduk

    2016-09-01

    Full Text Available There are too many types of robotic soccer approaches at present. SjF TUKE Robotics, who won robot soccer world tournament for year 2010 in category MiroSot, is a team with multiagent system approach. They have one main agent (master and five agent players, represented by robots. There is a point of view, in the article, for code programmer how to create new behavior strategies by creating a new code for master. There is a methodology how to prepare and create it following some rules.

  12. Cerebral Venous Thrombosis Revealing Primary Sjögren Syndrome: Report of 2 Cases

    Directory of Open Access Journals (Sweden)

    A. Mercurio

    2013-01-01

    Full Text Available Sjögren syndrome (SS is an autoimmune disease of the exocrine glands, characterized by focal lymphocytic infiltration and destruction of these glands. Neurologic complications are quite common, mainly involving the peripheral nervous system (PNS. The most common central nervous system (CNS manifestations are myelopathy and microcirculation vasculitis. However, specific diagnostic criteria for CNS SS are still lacking. We report two cases of primary SS in which the revealing symptom was cerebral venous thrombosis (CVT in the absence of genetic or acquired thrombophilias.

  13. Fatal Tuberculous Myositis in an Immunocompromised Adult With Primary Sjögren's Syndrome

    Directory of Open Access Journals (Sweden)

    Chi-Chang Huang

    2010-09-01

    Full Text Available Tuberculous myositis, which mimics rheumatic symptoms, is an extremely rare disease. Clinical ambiguity easily leads to misdiagnosis and delayed initial treatment. We present the case of a 55-year-old man who had primary Sjögren's syndrome and active cutaneous vasculitis treated with steroid and immunosuppressive drugs. He presented with a swollen, painful, hot left thigh. Although anti-tuberculosis medications were administered soon after a positive acid-fast stain of incisional muscular tissue, he died of rapidly progressive tuberculous myositis and multiorgan failure following 18 days of hospitalization. This case is presented to increase the awareness of this rare entity in clinical practice.

  14. Sjögren's syndrome in a 25-year-old female: A case study

    Directory of Open Access Journals (Sweden)

    Bacem A.E.O. Khalele

    2016-12-01

    Full Text Available Sjögren syndrome (SS is an autoimmune epithelitis characterized by lymphocytic glandular infiltration and various extraglandular manifestations. SS is usually encountered in middle-aged females (>50 years. Immunological, viral, hereditary, environmental and hormonal etiologic factors are controversially proposed with regard to the pathogenesis of SS with no upper hand given. The present study reports an atypical case of SS in a 25-year-old female who was closely followed up for three years. Being pregnant in 2015, SS ran a total remission course but did relapse more aggressively after delivery. Immunologically implicating, the possible interpretation, which may account for such a pathological fluctuation, is also tackled.

  15. Making the diagnosis of Sjögren’s syndrome in patients with dry eye

    Directory of Open Access Journals (Sweden)

    Beckman KA

    2015-12-01

    Full Text Available Kenneth A Beckman,1,2 Jodi Luchs,3,4 Mark S Milner5,6 1Comprehensive EyeCare of Central Ohio, Westerville, OH, 2The Ohio State University, Columbus, OH, 3Hofstra University School of Medicine, Hempstead, NY, 4South Shore Eye Care, Wantagh, NY, 5The Eye Center of Southern Connecticut, PC, Hamden, CT, 6Yale University School of Medicine, New Haven, CT, USA Abstract: Sjögren’s syndrome (SS is a chronic and progressive systemic autoimmune disease that often presents initially with symptoms of dry eye and dry mouth. Symptoms are often non-specific and develop gradually, making diagnosis difficult. Patients with dry eye complaints warrant a step-wise evaluation for possible SS. Initial evaluation requires establishment of a dry eye diagnosis using a combination of patient questionnaires and objective ocular tests, including inflammatory biomarker testing. Additional work-up using the Schirmer test and tear film break-up time can differentiate between aqueous-deficient dry eye (ADDE and evaporative dry eye. The presence of ADDE should trigger further work-up to differentiate between SS-ADDE and non-SS-ADDE. There are numerous non-ocular manifestations of SS, and monitoring for SS-related comorbid findings can aid in diagnosis, ideally in collaboration with a rheumatologist. The clinical work-up of SS can involve a variety of tests, including tear function tests, serological tests for autoantibody biomarkers, minor salivary gland and lacrimal gland biopsies. Examination of classic SS biomarkers (SS-A/Ro, SS-B/La, antinuclear antibody, and rheumatoid factor is a convenient and non-invasive way of evaluating patients for the presence of SS, even years prior to confirmed diagnosis, although not all SS patients will test positive, particularly those with early disease. Recently, newer biomarkers have been identified, including autoantibodies to salivary gland protein-1, parotid secretory protein, and carbonic anhydrase VI, and may allow for earlier

  16. Oral Rehabilitation and Management for Secondary Sjögren’s Syndrome in a Child

    OpenAIRE

    Fidalgo, Tatiana Kelly da Silva; Nogueira, Carla; Andrade, Marcia Rejane Thomas Canabarro; Valente, Andrea Graciene Lopez Ramos; Tannure, Patricia Nivoloni

    2016-01-01

    The aim of this paper is to describe a rare case report of a pediatric patient with secondary Sjögren’s syndrome (SSS). A 12-year-old female child was referred to the Pediatric Dentistry Clinic with the chief complaint of tooth pain, dry mouth, and tooth sensibility. The patient was submitted to orthodontic treatment prior to syndrome diagnosis. The clinical treatment consisted of the interruption of orthodontic treatment and restoring the oral condition with dental treatment and the use of a...

  17. Characterization and genomic analysis of chromate resistant and reducing Bacillus cereus strain SJ1

    Directory of Open Access Journals (Sweden)

    He Minyan

    2010-08-01

    Full Text Available Abstract Background Chromium is a toxic heavy metal, which primarily exists in two inorganic forms, Cr(VI and Cr(III. Chromate [Cr(VI] is carcinogenic, mutational, and teratogenic due to its strong oxidizing nature. Biotransformation of Cr(VI to less-toxic Cr(III by chromate-resistant and reducing bacteria has offered an ecological and economical option for chromate detoxification and bioremediation. However, knowledge of the genetic determinants for chromate resistance and reduction has been limited so far. Our main aim was to investigate chromate resistance and reduction by Bacillus cereus SJ1, and to further study the underlying mechanisms at the molecular level using the obtained genome sequence. Results Bacillus cereus SJ1 isolated from chromium-contaminated wastewater of a metal electroplating factory displayed high Cr(VI resistance with a minimal inhibitory concentration (MIC of 30 mM when induced with Cr(VI. A complete bacterial reduction of 1 mM Cr(VI was achieved within 57 h. By genome sequence analysis, a putative chromate transport operon, chrIA1, and two additional chrA genes encoding putative chromate transporters that likely confer chromate resistance were identified. Furthermore, we also found an azoreductase gene azoR and four nitroreductase genes nitR possibly involved in chromate reduction. Using reverse transcription PCR (RT-PCR technology, it was shown that expression of adjacent genes chrA1 and chrI was induced in response to Cr(VI but expression of the other two chromate transporter genes chrA2 and chrA3 was constitutive. In contrast, chromate reduction was constitutive in both phenotypic and gene expression analyses. The presence of a resolvase gene upstream of chrIA1, an arsenic resistance operon and a gene encoding Tn7-like transposition proteins ABBCCCD downstream of chrIA1 in B. cereus SJ1 implied the possibility of recent horizontal gene transfer. Conclusion Our results indicate that expression of the chromate

  18. Review of autoantigens in Sjögren’s syndrome: an update

    Directory of Open Access Journals (Sweden)

    Tong L

    2017-08-01

    Full Text Available Louis Tong,1–4 Vanessa Koh,3 Bernard Yu-Hor Thong5 1Department of Ophthalmology, Yong Loo Lin School of Medicine, National University of Singapore, 2Corneal and External Eye Disease, Singapore National Eye Centre, 3Ocular Surface Research Group, Singapore Eye Research Institute, 4Eye Academic Clinical Program, Duke-NUS Medical School, 5Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore Abstract: Primary Sjögren’s syndrome (pSS is an autoimmune disease characterized by inflammation in exocrine glands, resulting in reduced secretion of tears and saliva, manifesting as xerophthalmia and xerostomia, respectively. It is commonly associated with Sjögren’s syndrome type A (Ro and Sjögren’s syndrome type B (La antigens. However, in most patients, the identity of the triggering antigen is not known. Factors such as genetics of histocompatibility, dysregulation of T-cells, B-cells and viral infections have been implicated. Several important studies on autoantigens in pSS have been published since a review in 2012, and the aim of this review is to provide an update on further peer-reviewed original articles in this field. Oxidative damage of Ro60 antigen may explain the epitope spreading during the immune activation in pSS. Immune-mediated destruction of the muscarinic receptor-3-expressing cells has been associated with a reduction in parasympathetic function, which could cause reduced secretory function of exocrine glands. Such a process also activates reactive oxidative species and antioxidants, which are linked to the triggering of inflammatory responses. Elevated levels of kallikrein, yet another antigen present in the lacrimal gland and other tissues, are similarly involved in triggering an autoimmune T-cell response against target glands. Studying additional antigens, the platelet-selectin and vasoactive intestinal peptides, in patients with pSS can help to elucidate the origin and process of

  19. Is Hammarby Sjöstad a model case? Crime prevention through environmental design in Stockholm, Sweden

    DEFF Research Database (Denmark)

    Grønlund, Bo

    2012-01-01

    Crime Prevention Through Environmental Design (CPTED) claims to reduce crime and fear of crime through urban design and planning. If it does, it ought to be part of the sustainable planning and design of cities. This chapter gives an overview of the development of CPTED principles and then uses...... the European Committee for Standardization (CEN) guidelines to assess the case study of Hammarby Sjöstad – a residential area in Stockholm, the capital of Sweden. Urban planning and urban design principles are assessed from a CPTED point of view. Crime statistics as well as data on perceived safety...... of residents of Hammarby Sjöstad are compared with other areas in Stockholm. This chapter concludes that CPTED guidelines are met to a large degree: crime rates are relatively lower and perceived safety is higher in Hammarby Sjöstad than Stockholm’s average. Finally, the role of CPTED versus other factors when...

  20. Effect of omega-3 and vitamin E supplementation on dry mouth in patients with Sjögren's syndrome.

    Science.gov (United States)

    Singh, Medha; Stark, Paul C; Palmer, Carole A; Gilbard, Jeffrey P; Papas, Athena S

    2010-01-01

    To determine whether omega-3 (n-3) increases saliva production in patients with Sjögren's syndrome, 61 patients with Sjögren's received either wheat germ oil (n = 23) or n-3 supplement (TheraTears Nutrition®) (n = 38) in a prospective, randomized, double-masked trial. The outcomes assessed were salivary secretion and markers for oral inflammation. The differences between the n-3 group and wheat germ oil group were not statistically significant for either unstimulated (US) or stimulated (SS) salivary secretion (p= 0.38 and p= 0.346, respectively) nor for the number of sites with probing depth (PD) ≥ 4 mm (p= 0.834). In this pilot study, supplementation with n-3 was not found to be significantly better than wheat germ oil in stimulating saliva production in patients with Sjögren's syndrome. ©2010 Special Care Dentistry Association and Wiley Periodicals, Inc.

  1. Självkänsla hos daghemsbarn : En kvalitativ undersökning om hur barnträdgårdslärare bekräftar och stärker barnets självkänsla på daghem

    OpenAIRE

    Fant, Sofia

    2017-01-01

    Syftet med detta examensarbete är att ta reda på hur en barnträdgårdslärare kan stärka och bekräfta barnets självkänsla på daghem. Vilken syn och uppfattning finns det om självkänslan på daghem och hur kan man stödja barnen på bästa sätt när det gäller självkänslan? I detta examensarbete har jag genom litteratur tagit fram vad självkänsla är, men även olika definitioner kring självkänslan och hur miljön runt om kan påverka självkänslan hos barnet. I intervjuerna som blev gjorda framkom ...

  2. Histopathological changes in exocrine glands of murine transplantation chimeras. I: The development of Sjögren's syndrome-like changes secondary to GVH induced lupus syndrome

    DEFF Research Database (Denmark)

    Sørensen, Inger; Ussing, Anne Phaff; Prause, J.U.

    1992-01-01

    Autoimmune disease, systemic lupus erythematosus, chronic graft-versus-host reaction, renal insufficiency, Sjögren's syndrome, inbred mouse strains......Autoimmune disease, systemic lupus erythematosus, chronic graft-versus-host reaction, renal insufficiency, Sjögren's syndrome, inbred mouse strains...

  3. Increased frequency of CD16+ monocytes and the presence of activated dendritic cells in salivary glands in primary Sjögren syndrome

    NARCIS (Netherlands)

    Wildenberg, M. E.; Welzen-Coppens, J. M. C.; van Helden-Meeuwsen, C. G.; Bootsma, H.; Vissink, A.; van Rooijen, N.; van de Merwe, J. P.; Drexhage, H. A.; Versnel, M. A.

    2009-01-01

    In the salivary glands of patients with primary Sjögren Syndrome (pSjS) an accumulation of dendritic cells (DCs) is seen, which is thought to play a role in stimulating local inflammation. Aberrancies in subsets of monocytes, generally considered the blood precursors for DCs, may play a role in this

  4. Enhancement of protective efficacy through adenoviral vectored vaccine priming and protein boosting strategy encoding triosephosphate isomerase (SjTPI) against Schistosoma japonicum in mice.

    Science.gov (United States)

    Dai, Yang; Wang, Xiaoting; Tang, Jianxia; Zhao, Song; Xing, Yuntian; Dai, Jianrong; Jin, Xiaolin; Zhu, Yinchang

    2015-01-01

    Schistosomiasis japonica is a zoonotic parasitic disease; developing transmission blocking veterinary vaccines are urgently needed for the prevention and control of schistosomiasis in China. Heterologous prime-boost strategy, a novel vaccination approach, is more effective in enhancing vaccine efficacy against multiple pathogens. In the present study, we established a novel heterologous prime-boost vaccination strategy, the rAdV-SjTPI.opt intramuscular priming and rSjTPI subcutaneous boosting strategy, and evaluated its protective efficacy against Schistosoma japonicum in mice. Adenoviral vectored vaccine (rAdV-SjTPI.opt) and recombinant protein vaccine (rSjTPI) were prepared and used in different combinations as vaccines in a mouse model. The specific immune responses and protective efficacies were evaluated. Furthermore, the longevity of protective efficacy was also determined. Results showed that the rAdV-SjTPI.opt priming-rSjTPI boosting strategy elicited higher levels of specific IgG responses and broad-spectrum specific cellular immune responses. The protective efficacy could reach up to nearly 70% and 50% of protection could be observed at 10 weeks after the last immunization in mice. The rAdV-SjTPI.opt intramuscular priming-rSjTPI subcutaneous boosting vaccination strategy is a novel, highly efficient, and stable approach to developing vaccines against Schistosoma japonicum infections in China.

  5. Enhancement of protective efficacy through adenoviral vectored vaccine priming and protein boosting strategy encoding triosephosphate isomerase (SjTPI against Schistosoma japonicum in mice.

    Directory of Open Access Journals (Sweden)

    Yang Dai

    Full Text Available Schistosomiasis japonica is a zoonotic parasitic disease; developing transmission blocking veterinary vaccines are urgently needed for the prevention and control of schistosomiasis in China. Heterologous prime-boost strategy, a novel vaccination approach, is more effective in enhancing vaccine efficacy against multiple pathogens. In the present study, we established a novel heterologous prime-boost vaccination strategy, the rAdV-SjTPI.opt intramuscular priming and rSjTPI subcutaneous boosting strategy, and evaluated its protective efficacy against Schistosoma japonicum in mice.Adenoviral vectored vaccine (rAdV-SjTPI.opt and recombinant protein vaccine (rSjTPI were prepared and used in different combinations as vaccines in a mouse model. The specific immune responses and protective efficacies were evaluated. Furthermore, the longevity of protective efficacy was also determined. Results showed that the rAdV-SjTPI.opt priming-rSjTPI boosting strategy elicited higher levels of specific IgG responses and broad-spectrum specific cellular immune responses. The protective efficacy could reach up to nearly 70% and 50% of protection could be observed at 10 weeks after the last immunization in mice.The rAdV-SjTPI.opt intramuscular priming-rSjTPI subcutaneous boosting vaccination strategy is a novel, highly efficient, and stable approach to developing vaccines against Schistosoma japonicum infections in China.

  6. [Sjögren's syndrome (SS), a review of the subject and saliva as a diagnostic method].

    Science.gov (United States)

    Riega-Torres, Janett Carmen Luzmila; Villarreal-Gonzalez, Antonio Jaime; Ceceñas-Falcon, Luis Ángel; Salas-Alanis, Julio Cesar

    2016-01-01

    Sjögren's syndrome is a chronic autoimmune disease whose main clinical manifestation is oral dryness (xerostomia) and ocular dryness (xerophthalmia). It is characterized by progressive mononuclear infiltration of the exocrine glands and can affect a variety of organ systems. The prevalence of primary Sjögren's syndrome varies from 0.01 up to 4.8%; this variability reflects differences in definition, application of diagnostic criteria, and geographic differences in age groups. The etiology of primary Sjögren's syndrome is unknown, but the interaction between genetic and environmental factors (viruses, hormones, vitamins, stress) is important. There are few reported cases of concordance in monozygotic twins, and it is common for patients with primary Sjögren's syndrome to have relatives with other autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, thyroid disease, psoriasis, and multiple sclerosis. Among the most common findings is hypergammaglobulinemia. Elevated levels of γ-globulins contain autoantibodies directed against nonspecific antigens such as rheumatoid factor, antinuclear antibodies, and cellular antigens SS-A/Ro and SS-B/La. Regarding diagnosis, there have been 11 different published criteria for Sjögren's syndrome since 1965; none have been approved by the American College of Rheumatology or the European League Against Rheumatism. The current criteria were published in 2012 jointly with the progressive advance in the knowledge of the human salivary proteome that has gained wide acceptance in Sjögren's syndrome, with the possibility of using saliva as a useful tool in both diagnosis and prognosis in this field because the analysis of salivary proteins may reflect the state of locally underlying disease of the salivary glands, which are the target organs in this disease.

  7. Loss of PKCδ results in characteristics of Sjögren’s syndrome including salivary gland dysfunction

    OpenAIRE

    Banninger, Gregg P.; Cha, Seunghee; Said, M Sherif; Pauley, Kaleb M.; Carter, Cristan J.; Ornate, Mairelys; Pauley, Brad A.; Anderson, Steven M.; Reyland, Mary E.

    2011-01-01

    Chronic infiltration of lymphocytes into the salivary and lacrimal glands of Sjögren’s Syndrome patients leads to destruction of acinar cells and loss of exocrine function. Protein kinase C-delta (PKCδ) is known to play a critical role in B cell maintenance. Mice in which the PKCδ gene has been disrupted have a loss of B cell tolerance, multiple organ lymphocytic infiltration, and altered apoptosis. To determine if PKCδ contributes to the pathogenesis of Sjögren’s Syndrome, we quantified chan...

  8. Reduced sulfation of muc5b is linked to xerostomia in patients with Sjögren syndrome

    DEFF Research Database (Denmark)

    Alliende, C; Kwon, Y-J; Brito, M

    2008-01-01

    OBJECTIVES: MUC5B contains sulfated and sialylated oligosaccharides that sequester water required for moisturising the oral mucosa. Xerostomia, in patients with Sjögren syndrome, is generally associated with reduced quantities, rather than altered properties, of saliva. Here, we determined...... disorganised basal lamina. CONCLUSION: Disorganisation of the basal lamina observed in patients with Sjögren syndrome may lead to dedifferentiation of acinar mucous cells and, as a consequence, alter sulfation of MUC5B. These changes are suggested to represent a novel mechanism that may explain xerostomia...

  9. Clasp/SJ Observation of Time Variations of Lyman-Alpha Emissions in a Solar Active Region

    Science.gov (United States)

    Ishikawa, S.; Kubo, M.; Katsukawa, Y.; Kano, R.; Narukage, N.; Ishikawa, R.; Bando, T.; Winebarger, A.; Kobayashi, K.; Trujillo Bueno, J.; hide

    2016-01-01

    The Chromospheric Lyman-alpha SpectroPolarimeter (CLASP) is a sounding rocket experiment launched on September 3, 2015 to investigate the solar chromosphere, and the slit-jaw (SJ) optical system took Lya images with the high time cadence of 0.6 s. By the CLASP/SJ observation, many time variations in the solar chromosphere with the time scale of region and investigated the short (regions. As the result, we found the regions. On the other hand, the <30 s time variations had no dependency on the temperature of the loop.

  10. Therapy of Sjögren's syndrome. New aspects and future directions.

    Science.gov (United States)

    Vlachoyiannopoulos, P G

    1998-02-01

    Therapy of Sjögren's syndrome remains empirical and symptomatic. The main goals are to treat the disease related features, especially sicca manifestations, since the immunosuppressive therapy has not given promising results. For the treatment of keratoconjunctivitis sicca: local stimulators of tear secretion, protective bicarbonate buffered solutions, replacement therapy or supportive operative procedures should be tried. For oral manifestations: stimulators of salivary secretion such as pilocarpine, or agents changing the consistency of saliva such as bromhexine orally should be tried. Saliva substitutes have a transient effect. Frequent ingestion of sugar free liquids may help. Oral hygiene is important to avoid oral candidiasis and dental caries. Treatment of parotid gland swelling is not necessary. Pulmonary manifestations include pulmonary infiltrates in perialveolar areas, nodular or cavitary lesions which may represent lymphoma. Hilar adenopathy, solid or cavitary lesions should be biopsied. In case of vasculitis prednisolone 1 mg/kg/day with progressive tapering should be tried. Renal involvement is manifested mainly as interstitial disease. Administration of NaHCO3 or sodium citrate is important to prevent acidosis and nephrocalcinosis. Vasculitis, when it is of the leukoclasic form, does not need therapy; when it is manifested with severe major organ involvement corticosteroids and/or cytotoxic therapy should be tried. Lymphoma is treated as in the patients without Sjögren's in close collaboration with the oncology department.

  11. Dental perspective on Sjögren’s syndrome: literature review.

    Directory of Open Access Journals (Sweden)

    Jesús Rodríguez

    2015-06-01

    Full Text Available The human body releases around 500-600mL of saliva daily, however when values of unstimulated whole saliva range from 0.1 to 0.2mL/min, there is a condition called Hyposalivation or hyposialia. Hyposalia is characterized by a large number of systemic conditions, including Sjögren’s syndrome, a chronic autoimmune disease that affects between 0.1 and 3% of the world population and is characterized by exocrinopathy of the salivary glands leading to glandular hypofunction and thus decreasing the normal salivary flow. Saliva is part of innate immunity, when there is a decrease in protein secretion, numerous oral manifestations occur such as dental caries, candidiasis, gingival disease, angular cheilitis, lymphomas of the salivary glands, dysphagia, erythematous and fissured tongue, among others. Currently there is no defined dental treatment, however there are alternative treatments by sialogogues and salivary substitutes, plus non-pharmacological therapies, which seek to maintain the ecology and oral conditions stable, in addition to preventive and restorative dental treatment for lesions already established as a consequence of the disease. The aim of this study is to conduct a literature review on the characteristics, classification, oral manifestations and dental management of Sjögren’s syndrome.

  12. [A case of sarcoidosis associated with Sjögren's syndrome].

    Science.gov (United States)

    Akiyama, Y; Suzuki, T; Tanaka, M; Katagiri, T; Ishibashi, T; Imai, F; Ohno, S; Doi, Y

    1992-10-01

    A 49-year-old woman, who has had a past history of Raynaud's phenomenon since 1981, dry mouth since 1987 and dry eyes since 1990, was admitted to our hospital complaining of general fatigue, bilateral gonalgia, and shortness of breath in April 1991. Physical examinations revealed bilateral parotid gland enlargement and bilateral uveitis. Her family history included mixed connective tissue disease in her eldest daughter. Laboratory findings indicated an elevated erythrocyte sedimentation rate, hypergammaglobulinemia, and elevation of serum lysozyme and angiotensin converting enzyme levels. Antinuclear antibodies, anti-SSA antibodies, anti-SSB antibodies were positive, while tuberculin test was negative. Chest X-rays showed bilateral hilar lymphadenopathy and small nodular shadows in both lung fields. Increases in lymphocytes and the CD4/CD8 ratio of T cells were noted in alveolar lavage fluid. Renal biopsy revealed non-caseous granulomas. Other remarkable findings included positive Schirmer's test, apple tree lesions by sialography and chronic sialoadenitis by biopsy of the labial minor salivary gland. On the basis of all these findings, we diagnosed her as suffering from sarcoidosis with Sjögren's syndrome. After oral administration of prednisolone, her shortness of breath, hilar lymphadenopathy and small nodular shadows in the lung field disappeared, but the patient was transferred to the department of urology due to the onset of hydronephrosis. This case is noteworthy because sarcoidosis associated with Sjögren's syndrome has been reported in only 11 cases in the literature.

  13. Aspectos do diagnóstico na Síndrome de Sjögren

    Directory of Open Access Journals (Sweden)

    Liquidato Bianca M.

    2002-01-01

    Full Text Available Introdução: A Síndrome de Sjögren é uma doença auto-imune que envolve principalmente as glândulas salivares e lacrimais e que pode cursar apenas com xerostomia e diminuição do lacrimejamento ou mesmo com outras manifestações sistêmicas. Vários estudos tentaram estabelecer critérios diagnósticos, já que não existe um exame de certeza. Objetivo: Nosso estudo tem como objetivo a avaliação dos métodos utilizados para o diagnóstico da doença. Forma de estudo: Clínico prospectivo. Material e Método: Foram avaliadas 15 pacientes com suspeita de Síndrome de Sjögren no Ambulatório de Estomatologia do Depto. de Otorrinolaringologia da Santa Casa de São Paulo. Resultados: Das 15 pacientes avaliadas, o diagnóstico foi confirmado apenas em 6 casos. Conclusões: Existe grande dificuldade em se estabelecer o diagnóstico desta doença e, por este motivo, técnicas de exame pouco invasivas devem ser desenvolvidas com a participação do otorrinolaringologista.

  14. Sjögren syndrome and other causes of sicca in the older adult

    Science.gov (United States)

    Baer, Alan N.; Walitt, Brian

    2016-01-01

    Synopsis Dry eye and dry mouth symptoms are each reported by up to 30% of persons over the age of 65 years, particularly in women. Multiple factors contribute to these symptoms, but medication side effects are the most common. The evaluation of these symptoms requires measures of ocular and oral dryness, some of which can be done by the geriatrician in a clinic setting. Sjögren syndrome is the prototypic disease associated with dryness of the eyes and mouth and predominantly affects women in their peri- and postmenopausal years of life. The diagnosis requires substantive evidence of autoimmune-induced inflammation targeting the salivary or lacrimal glands. With current classification criteria, the diagnosis can only be established if the patient has antibodies to the SSA (Ro) and/or SSB (La) antigens or a labial gland biopsy showing a characteristic pattern and severity of inflammation, in concert with at least one measure of ocular or oral dryness. A diagnosis is important, since this disease is associated with a substantial increased risk of lymphoma and other forms of morbidity. In addition to topical treatment of the mucosal dryness, patients with Sjögren's syndrome may require treatment with systemic immunomodulatory and immunosuppressive agents to manage a variety of “extraglandular” manifestations. PMID:27886700

  15. Effect of Systemic Infliximab Therapy in Patients with Sjögren’s Syndrome

    Directory of Open Access Journals (Sweden)

    Elif Betül Türkoğlu

    2015-08-01

    Full Text Available Objectives: To investigate the effect of systemic infliximab therapy on tear function tests and the ocular surface in patients with Sjögren’s syndrome secondary to various autoimmune diseases. Materials and Methods: This prospective study included 22 eyes of 22 patients with Sjögren’s syndrome who began treatment with systemic infliximab. Tear film break-up time (TBUT, anesthetized Schirmer’s 1 test, fluorescein staining test, and Ocular Surface Disease Index (OSDI scores were recorded before treatment and in the 3rd and 6th months of treatment. Results: In the 3rd month of infliximab therapy, no significant changes were observed in Schirmer’s values, TBUT, fluorescein staining, or OSDI scores (p=0.260, p=0.357, p=0.190 and p=0.07, respectively. In the 6th month of infliximab therapy, no significant changes were observed in TBUT, fluorescein staining, Schirmer’s value or OSDI scores (p=0.510, p=0.320, p=0.220 and p=0.344, respectively. Conclusion: Infliximab therapy, which is commonly used in systemic autoimmune diseases such as rheumatoid arthritis, Crohn’s disease, ulcerative colitis, and ankylosing spondylitis, did not show a positive effect on ocular surface and tear function tests. (Turk J Ophthalmol 2015; 45: 138-141

  16. Genome-Wide Association Analysis Reveals Genetic Heterogeneity of Sjögren's Syndrome According to Ancestry

    DEFF Research Database (Denmark)

    Taylor, Kimberly E; Wong, Quenna; Levine, David M

    2017-01-01

    OBJECTIVE: The Sjögren's International Collaborative Clinical Alliance (SICCA) is an international data registry and biorepository derived from a multisite observational study of participants in whom genotyping was performed on the Omni2.5M platform and who had undergone deep phenotyping using co...

  17. Rapamycin Eye Drops Suppress Lacrimal Gland Inflammation In a Murine Model of Sjögren's Syndrome

    Science.gov (United States)

    Shah, Mihir; Edman, Maria C.; Reddy Janga, Srikanth; Yarber, Frances; Meng, Zhen; Klinngam, Wannita; Bushman, Jonathan; Ma, Tao; Liu, Siyu; Louie, Stan; Mehta, Arjun; Ding, Chuanqing; MacKay, J. Andrew; Hamm-Alvarez, Sarah F.

    2017-01-01

    Purpose To evaluate the efficacy of topical rapamycin in treating autoimmune dacryoadenitis in a mouse model of Sjögren's syndrome. Methods We developed rapamycin in a poly(ethylene glycol)-distearoyl phosphatidylethanolamine (PEG-DSPE) micelle formulation to maintain solubility. Rapamycin or PEG-DSPE eye drops (vehicle) were administered in a well-established Sjögren's syndrome disease model, the male nonobese diabetic (NOD) mice, twice daily for 12 weeks starting at 8 weeks of age. Mouse tear fluid was collected and tear Cathepsin S, a putative tear biomarker for Sjögren's syndrome, was measured. Lacrimal glands were retrieved for histological evaluation, and quantitative real-time PCR of genes associated with Sjögren's syndrome pathogenesis. Tear secretion was measured using phenol red threads, and corneal fluorescein staining was used to assess corneal integrity. Results Lymphocytic infiltration of lacrimal glands from rapamycin-treated mice was significantly (P = 0.0001) reduced by 3.8-fold relative to vehicle-treated mice after 12 weeks of treatment. Rapamycin, but not vehicle, treatment increased tear secretion and decreased corneal fluorescein staining after 12 weeks. In rapamycin-treated mice, Cathepsin S activity was significantly reduced by 3.75-fold in tears (P eye. PMID:28122086

  18. A taxonomy of disease experiences of women with Sjögren’s syndrome from the perspective of the patient

    NARCIS (Netherlands)

    Visser-Noordegraaf, M.Y.; Hiensch, V.; Rojas-Alcayaga, G.; Geenen, R.|info:eu-repo/dai/nl/087017571

    Background In earlier studies of experiences of patients with Sjögren's Syndrome (SS), professionals interpreted the data and drew conclusions and implications. In the current study, patients had a major share in the interpretation and structuring of experiences, and a patient research partner was

  19. The Toll of too much Interferon : The systemic interferon signature in the pathogenesis of Sjögren’s syndrome

    NARCIS (Netherlands)

    N.I. Maria (Naomi)

    2015-01-01

    markdownabstractAbstract Primary Sjögren's Syndrome (pSS) is an autoimmune disease characterized by accumulation of white blood cells in the salivary and lachrymal glands. Characteristic symptoms are dry eyes and mouth. Other manifestations frequently present in pSS involve among others the

  20. Immunogenetics of rheumatoid arthritis and primary Sjögren's syndrome: DNA polymorphism of HLA class II genes

    DEFF Research Database (Denmark)

    Morling, Niels; Andersen, V; Fugger, L

    1992-01-01

    We investigated the DNA restriction fragment length polymorphism (RFLP) of the Major Histocompatability Complex (MHC) class II genes: HLA-DRB, -DQA, -DQB, DPA, and -DFB in 24 patients with rheumatoid arthritis (RA), in 19 patients with primary Sjögren's syndrome (primary SS), and healthy Danes. T...

  1. Understanding fetal factors that contribute to preterm birth: Sjögren-Larsson syndrome as a model.

    Science.gov (United States)

    Staps, Pippa; Hogeveen, Marije; Fuijkschot, Joris; van Drongelen, Joris; Willemsen, Michèl A A P

    2017-09-15

    Preterm birth is the world's leading cause of neonatal death. Unfortunately, the pathophysiology of preterm birth remains poorly understood. Sjögren-Larsson syndrome is a rare, neurometabolic disorder caused by a fatty aldehyde dehydrogenase deficiency. A majority of patients with Sjögren-Larsson syndrome is born preterm. Data of all known Dutch patients with Sjögren-Larsson syndrome and all cases reported in literature were analyzed to learn from preterm birth in context of this rare disease. Exact gestational age was known in 33 Dutch patients; 24 (73%) of them were born preterm, with a median gestational age of 36 weeks. The literature search confirmed our findings: 13 (59%) of 22 cases was born preterm. Preterm birth is a hallmark of Sjögren-Larsson syndrome, presumably caused by the abnormal lipid metabolism of the fetus. At least five additional rare genetic disorders (namely Ehlers-Danlos syndrome, ichthyosis prematurity syndrome, congenital analbuminemia, osteogenesis imperfecta type II and restrictive dermopathy) were found in literature that lead to preterm birth of the affected fetus. These disorders are in fact "experiments of nature" and as such they shed new lights on the mechanisms causing preterm birth.

  2. The Significance of Dehydroepiandrosterone for Fatigue in primary Sjögren’s Syndrome and Systemic Lupus Erythematosus

    NARCIS (Netherlands)

    Hartkamp, A.

    2014-01-01

    Fatigue is a prevalent and debilitating symptom in patients with the chronic inflammatory autoimmune diseases primary Sjögren’s syndrome (pSS) and systemic lupus erythematosus (SLE). Both diseases have a female preponderance (women to men ratio 9:1). Doctors recognize the existence of fatigue and

  3. Connolly Hospital (Silver Birch & Woodland Units), Blanchardstown, Dublin 15.

    LENUS (Irish Health Repository)

    Hickey, Ann

    2012-12-13

    Patient documentation is time consuming and can detract from care. The authors report a novel computer programme that manipulates routinely collected information to quantify nursing workload, along with the reason for admission, functional status, estimates of in-hospital mortality and life expectancy. The programme stores information in a database, and produces a print-out in a situation\\/background\\/assessment\\/recommendation (SBAR) format. The average time taken to enter 629 patient encounters was 6.6 minutes. Pain was the most common presentation for low workload patients, while high workload patients often presented with altered mental status and reduced mobility. There was only a modest correlation between the risk of death and nursing workload. The programme measures nursing workload without further paperwork, and improves routine documentation with a legible brief report that is automatically generated. This report can be shared and provides data that is immediately available for day-to-day care, audit, quality control and service planning.

  4. Connolly Hospital (Silver Birch & Woodland Units), Blanchardstown, Dublin 15.

    LENUS (Irish Health Repository)

    Semchenko, Evgeny A

    2010-11-30

    Abstract Background Campylobacter jejuni is a major bacterial cause of food-borne enteritis, and its lipooligosaccharide (LOS) plays an initiating role in the development of the autoimmune neuropathy, Guillain-Barré syndrome, by induction of anti-neural cross-reactive antibodies through ganglioside molecular mimicry. Results Herein we describe the existence and heterogeneity of multiple LOS forms in C. jejuni strains of human and chicken origin grown at 37°C and 42°C, respectively, as determined on sodium dodecyl sulphate-polyacrylamide electrophoresis gels with carbohydrate-specific silver staining and blotting with anti-ganglioside ligands, and confirmed by nuclear magnetic resonance (NMR) spectroscopy. The C. jejuni NCTC 11168 original isolate (11168-O) was compared to its genome-sequenced variant (11168-GS), and both were found to have a lower-Mr LOS form, which was different in size and structure to the previously characterized higher-Mr form bearing GM1 mimicry. The lower-Mr form production was found to be dependent on the growth temperature as the production of this form increased from ~5%, observed at 37°C to ~35% at 42°C. The structure of the lower-Mr form contained a β-D-Gal-(1→3)-β-D-GalNAc disaccharide moiety which is consistent with the termini of the GM1, asialo-GM1, GD1, GT1 and GQ1 gangliosides, however, it did not display GM1 mimicry as assessed in blotting studies but was shown in NMR to resemble asialo-GM1. The production of multiple LOS forms and lack of GM1 mimicry was not a result of phase variation in the genes tested of NCTC 11168 and was also observed in most of the human and chicken isolates of C. jejuni tested. Conclusion The presence of differing amounts of LOS forms at 37 and 42°C, and the variety of forms observed in different strains, indicate that LOS form variation may play a role in an adaptive mechanism or a stress response of the bacterium during the colonization of different hosts.

  5. Connolly Hospital (Silver Birch & Woodland Units), Blanchardstown, Dublin 15.

    LENUS (Irish Health Repository)

    Garvey, P

    2013-01-01

    Salmonella Typhimurium DT8 was a very rare cause of human illness in Ireland between 2000 and 2008, with only four human isolates from three patients being identified. Over a 19-month period between August 2009 and February 2011, 34 confirmed cases and one probable case of Salmonella Typhimurium DT8 were detected, all of which had an MLVA pattern 2-10-NA-12-212 or a closely related pattern. The epidemiological investigations strongly supported a linkbetween illness and exposure to duck eggs. Moreover, S. Typhimurium with an MLVA pattern indistinguishable (or closely related) to the isolates from human cases, was identified in 22 commercial and backyard duck flocks, twelve of which were linked with known human cases. A range of control measures were taken at farm level, and advice was provided to consumers on the hygienic handling and cooking of duck eggs. Although no definitive link was established with a concurrent duck egg-related outbreak of S. Typhimurium DT8 in the United Kingdom, it seems likely that the two events were related. It may be appropriate for other countries with a tradition of consuming duck eggs to consider the need for measures to reduce the risk of similar outbreaks.

  6. EBV-positive diffuse large B-cell lymphoma in a patient with primary Sjögren’s syndrome and membranous glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Kim Chang

    2012-11-01

    Full Text Available Abstract Background Sjögren’s syndrome is a systemic autoimmune disease in which lymphatic cells destroy the salivary and lacrimal glands. Glomerulonephritis is thought to be a rare occurrence in primary Sjögren’s syndrome. Furthermore, concurrent glomerular involvement and lymphoma in patients with Sjögren’s syndrome has seldom been reported. Case presentation A 52-year-old woman with primary Sjögren’s syndrome developed membranous glomerulonephritis and Epstein-Barr virus-positive diffuse large B-cell lymphoma (DLBCL. She was diagnosed with Sjögren’s syndrome based on the dry eyes, dry mouth, positive anti-nuclear antibody test, anti-Ro (SS-A antibody, salivary gland biopsy, and salivary scintigraphy. Moreover, renal biopsy confirmed the diagnosis of membranous glomerulonephritis. Three months later, her small bowel was perforated with pneumoperitoneum, and the biopsy revealed Epstein-Barr virus-positive DLBCL. Conclusions We observed the first case of primary Sjögren’s syndrome associated with Epstein-Barr Virus-positive DLBCL and membranous glomerulonephritis. Because of the possibility of malignancy-associated membranous glomerulonephritis in patients with primary Sjögren’s syndrome, we should be careful and examine such patients for hidden malignancy.

  7. GÉNERO, PODER Y ESPIRITUALIDAD EN LIMA COLONIAL: LA REFORMA CONVENTUAL DEL MÍSTICO DIEGO MARTÍNEZ, SJ 0609-1626

    Directory of Open Access Journals (Sweden)

    Alexandre Coello de la Rosa

    2013-02-01

    Full Text Available Este ensayo tiene como objetivo estudiar la figura del jesuita Diego Martínez (15421626, SJ, como místico y maestro espiritual de religiosas y beatas de los conventos de Lima. Sobre la mística jesuita y peruana del siglo XVII se ha escrito poco, a pesar de contar con ilustres místicos, como Diego Álvarez de Paz (1562-1620, SJ, Antonio Ruiz de Montoya (1585-1652, SJ, Juan de Alloza (1597-1666, SJ, Francisco del Castillo, SJ (1615-1673, o el mismo Diego Martínez, SJ. y poco, o muy poco, sobre el contexto social e histórico que favoreció la espiritualidad de la Compañía deJesús en el Perú (161025. Durante el provincialato del padre Diego Álvarez de Paz (1616-1620, SJ, los jesuitas habían explorado individualmente la mística y la espiritualidad en su vertiente contemplativa, pero su espacio de difusión no fue la universidad sino los conventos de mujeres.

  8. Capilaroscopia en el Síndrome de Sjögren Primario

    OpenAIRE

    Alonso Vila, Serafin

    2013-01-01

    La Síndrome de Sjögren primària (SSp) és una malaltia inflamatòria crònica autoimmune que afecta a les glàndules exocrines. Cursa amb símptomes de sequedat generalitzada i un terç dels pacients presenten afecció extraglandular, incloent Fenomen de Raynaud. Per investigar l'existència d'un patró capilaroscòpic típic o suggestiu de SSp es va practicar videocapilaroscòpia a 107 pacients, analitzant posteriorment les possibles relacions entre les alteracions trobades i el perfil immunològic i /o ...

  9. Atrophic pityriasis versicolor occurring in a patient with Sjögren's syndrome.

    Science.gov (United States)

    Marinello, Elena; Piaserico, Stefano; Alaibac, Mauro

    2017-01-18

    Pityriasis versicolor is one of the most frequent epidermal mycotic infections in the world, but its atrophic variant is rarely described. The aetiology of the atrophy is still unknown, and two main hypotheses have been formulated, one suggesting a correlation with long-term use of topical steroids and the other a delayed type hypersensitivity to epicutaneous antigens derived from components of the fungus. Atrophic pityriasis versicolor is a benign disease, but needs to be distinguished from other more severe skin diseases manifesting with cutaneous atrophy. The diagnosis can be easily confirmed by direct microscopic observation of the scales soaked in 15% potassium hydroxide, which reveals the typical 'spaghetti and meatball' appearance, or by a skin biopsy in doubtful cases. Here, we describe a case of extensive atrophic pityriasis versicolor occurring in a woman affected by Sjögren's syndrome which completely resolved after topical antifungal treatment. 2017 BMJ Publishing Group Ltd.

  10. Hypokalemic paralysis: the first presentation of primary Sjögren’s syndrome

    OpenAIRE

    Martinho, A; Capela, A; Duarte, F

    2012-01-01

    O Síndrome de Sjögren é uma doença auto-imune sistémica caracterizada por inflamação crónica das glândulas exócrinas. As manifestações extraglandulares têm uma incidência até 25%. O envolvimento renal ocorre em 18,4-67% dos casos, e nestes a nefrite tubulointersticial é a lesão mais frequente. Apresenta-se o caso clínico de uma mulher de 37 anos, internada por fraqueza muscular generalizada de grau 2 com uma semana de evolução. Objectivou-se hipocaliémia, rabdomiólise, pH urinário 6,5, protei...

  11. Oral Rehabilitation and Management for Secondary Sjögren’s Syndrome in a Child

    Directory of Open Access Journals (Sweden)

    Tatiana Kelly da Silva Fidalgo

    2016-01-01

    Full Text Available The aim of this paper is to describe a rare case report of a pediatric patient with secondary Sjögren’s syndrome (SSS. A 12-year-old female child was referred to the Pediatric Dentistry Clinic with the chief complaint of tooth pain, dry mouth, and tooth sensibility. The patient was submitted to orthodontic treatment prior to syndrome diagnosis. The clinical treatment consisted of the interruption of orthodontic treatment and restoring the oral condition with dental treatment and the use of artificial saliva in an innovative apparatus. Dental therapy involved the control of dental caries, periodontal disease, and opportunistic fungal infections and the use of fluoride-rich solutions. The present clinical case describes clinical and laboratory aspects of SSS in pediatric patients. The management of the oral findings promoted an improvement in the oral health status and quality of life of the child.

  12. Primary Sjögren’s syndrome with minimal change disease—A case report

    Directory of Open Access Journals (Sweden)

    Mei-Li Yang

    2011-05-01

    Full Text Available Glomerular involvement in patients with primary Sjögren’s syndrome (pSS has rarely been reported. Among them, membranoproliferative glomerulonephritis and membranous nephropathy are the more common types. We report a middle-aged female presenting concurrently with nephrotic syndrome and microscopic hematuria, and her pSS was diagnosed by positive anti-Ro (SSA/anti-La (SSB autoantibodies, dry mouth, severely diffuse impaired function of both bilateral parotid and submandibular glands, and a positive Schirmer test. Renal pathology revealed minimal change disease and thin basement membrane nephropathy. The patient’s nephrotic syndrome resolved after treatment with corticosteroids. To our knowledge, this is the first report of minimal change disease in a patient with pSS.

  13. Oral Rehabilitation and Management for Secondary Sjögren's Syndrome in a Child.

    Science.gov (United States)

    Fidalgo, Tatiana Kelly da Silva; Nogueira, Carla; Andrade, Marcia Rejane Thomas Canabarro; Valente, Andrea Graciene Lopez Ramos; Tannure, Patricia Nivoloni

    2016-01-01

    The aim of this paper is to describe a rare case report of a pediatric patient with secondary Sjögren's syndrome (SSS). A 12-year-old female child was referred to the Pediatric Dentistry Clinic with the chief complaint of tooth pain, dry mouth, and tooth sensibility. The patient was submitted to orthodontic treatment prior to syndrome diagnosis. The clinical treatment consisted of the interruption of orthodontic treatment and restoring the oral condition with dental treatment and the use of artificial saliva in an innovative apparatus. Dental therapy involved the control of dental caries, periodontal disease, and opportunistic fungal infections and the use of fluoride-rich solutions. The present clinical case describes clinical and laboratory aspects of SSS in pediatric patients. The management of the oral findings promoted an improvement in the oral health status and quality of life of the child.

  14. Potential Therapy for Rheumatoid Arthritis and Sjögren Syndrome With Human Chorionic Gonadotropin.

    Science.gov (United States)

    Rao, C V

    2016-05-01

    Autoimmune diseases such as rheumatoid arthritis (RA) and Sjögren syndrome (SS) ameliorate during pregnancy, through dampening (immunotolerance) of the maternal immune system which protects the fetus from rejection. A large number of studies have shown that human chorionic gonadotropin (hCG) contributes to this tolerance. Studies on animal models have reaffirmed that hCG treatment mimics the benefits of pregnancy. Based on the scientific evidence, randomized clinical trials comparing hCG with current therapies and/or placebo are recommended for RA, SS, and for other autoimmune diseases such as, type 1 diabetes and ankylosing spondylitis, which also get better during pregnancy and hCG treatment seems to help. © The Author(s) 2015.

  15. Primary Sjögren's Syndrome and Celiac Disease: Causal Association or Serendipity?

    Science.gov (United States)

    Alvarez-Celorio, M D; Angeles-Angeles, A; Kraus, A

    2000-08-01

    Primary Sjögren's syndrome (pSS) is an autoimmune disease associated with other autoimmune diseases like thymoma, hypothyroidism or mucosa-associated lymphoid tissue lymphomas. Celiac disease (CD) is a gluten sensitive enteropathy that causes small bowel mucosal damage in genetically susceptible individuals. Several immunologic alterations and immune-associated diseases, for example, insulin dependent diabetes mellitus, autoimmune thyroid disease, and connective tissue disorders, have been described in CD. We report 3 of 141 pSS patients who also had CD and review literature that suggests that CD is increased in patients with pSS. Successful treatment of CD did not relieve sicca symptoms or signs; thus, the two diseases must be treated independently. Although intestinal inflammation can occur in patients with pSS, diarrhea should be evaluated for CD and other causes. Because of the occasional severity of CD, clinicians must be aware of the possible association with pSS.

  16. Primary Sjögren's syndrome and bronchiolitis. An unusual association.

    Science.gov (United States)

    Medina-Paz, Lourdes; Che-Morales, José Luis; Alonso Moshe Barrera-Pérez, Hirian; Cortes-Telles, Arturo

    2017-03-03

    Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects the exocrine glands. It has been reported that the association between SS and respiratory involvement occurs in 11% and 45% of cases, and can be the initial manifestation of the disease. The diagnosis is based on the integration of clinical, immunological and histopathological criteria. This paper examines a 58-year-old man with SS, severe dyspnea and recurrent exacerbations associated with bronchiolitis-bronchiectasis. The absence of anti-Ro and anti-La antibodies associated with severe bronchiolitis is a clinical presentation that is poorly characterized in this group of patients; moreover, how to treat them remains unclear. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  17. Distal renal tubular acidosis and quadriparaesis in Sjögren′s syndrome: A cunning congregate

    Directory of Open Access Journals (Sweden)

    Arundhati G Diwan

    2014-01-01

    Full Text Available Sjögren′s syndrome (SS is a chronic autoimmune disease, chiefly affecting the exocrine glandular function of salivary glands and lacrimal glands. Rarely, it involves the kidneys, central and peripheral nervous system, muscloskeletal apparatus and lungs. We report a rare constellation of SS with distal renal tubular acidosis and quadriparaesis in a young female. History of quadriparaesis was acute, with rapid progression. Supplementary treatment for severe hypokalemia was instituted at the earliest, lest the patient develop respiratory muscle weakness. Concomitantly, metabolic acidosis with alkaline urine was suspected and subsequently investigated. Eventually, this was attributed to impaired renal acidification of urine in the distal tubules. History of dryness of eyes and mouth since 6 months justified salivary gland biopsy. The results yielded a lymphocytic infiltrative pathology strongly favoring SS. The patient benefited from prompt potassium replacement therapy and had complete resolution over the next week. Supportive treatment for predictable manifestations was continued along with potassium supplements.

  18. Patient burden of Sjögren’s: a comprehensive literature review revealing the range and heterogeneity of measures used in assessments of severity

    OpenAIRE

    Hammitt, Katherine M; Naegeli, April N; van den Broek, Remon W M; Birt, Julie A

    2017-01-01

    Context The severity of Sjögren’s syndrome has been evaluated using a wide variety of clinical measures and patient-reported outcomes (PROs). This may contribute to the lack of clarity concerning the burden of Sjögren’s from the patient perspective. Objective To perform a comprehensive peer-reviewed literature analysis of the patient aspects of Sjögren’s, focusing on PROs, to investigate the complexity underlying the evaluation of the syndrome and to elucidate the discordance between the diff...

  19. A Transcriptional Signature of Fatigue Derived from Patients with Primary Sjögren's Syndrome.

    Directory of Open Access Journals (Sweden)

    Katherine James

    Full Text Available Fatigue is a debilitating condition with a significant impact on patients' quality of life. Fatigue is frequently reported by patients suffering from primary Sjögren's Syndrome (pSS, a chronic autoimmune condition characterised by dryness of the eyes and the mouth. However, although fatigue is common in pSS, it does not manifest in all sufferers, providing an excellent model with which to explore the potential underpinning biological mechanisms.Whole blood samples from 133 fully-phenotyped pSS patients stratified for the presence of fatigue, collected by the UK primary Sjögren's Syndrome Registry, were used for whole genome microarray. The resulting data were analysed both on a gene by gene basis and using pre-defined groups of genes. Finally, gene set enrichment analysis (GSEA was used as a feature selection technique for input into a support vector machine (SVM classifier. Classification was assessed using area under curve (AUC of receiver operator characteristic and standard error of Wilcoxon statistic, SE(W.Although no genes were individually found to be associated with fatigue, 19 metabolic pathways were enriched in the high fatigue patient group using GSEA. Analysis revealed that these enrichments arose from the presence of a subset of 55 genes. A radial kernel SVM classifier with this subset of genes as input displayed significantly improved performance over classifiers using all pathway genes as input. The classifiers had AUCs of 0.866 (SE(W 0.002 and 0.525 (SE(W 0.006, respectively.Systematic analysis of gene expression data from pSS patients discordant for fatigue identified 55 genes which are predictive of fatigue level using SVM classification. This list represents the first step in understanding the underlying pathophysiological mechanisms of fatigue in patients with pSS.

  20. Making the diagnosis of Sjögren’s syndrome in patients with dry eye

    Science.gov (United States)

    Beckman, Kenneth A; Luchs, Jodi; Milner, Mark S

    2016-01-01

    Sjögren’s syndrome (SS) is a chronic and progressive systemic autoimmune disease that often presents initially with symptoms of dry eye and dry mouth. Symptoms are often nonspecific and develop gradually, making diagnosis difficult. Patients with dry eye complaints warrant a step-wise evaluation for possible SS. Initial evaluation requires establishment of a dry eye diagnosis using a combination of patient questionnaires and objective ocular tests, including inflammatory biomarker testing. Additional work-up using the Schirmer test and tear film break-up time can differentiate between aqueous-deficient dry eye (ADDE) and evaporative dry eye. The presence of ADDE should trigger further work-up to differentiate between SS-ADDE and non-SS-ADDE. There are numerous non-ocular manifestations of SS, and monitoring for SS-related comorbid findings can aid in diagnosis, ideally in collaboration with a rheumatologist. The clinical work-up of SS can involve a variety of tests, including tear function tests, serological tests for autoantibody biomarkers, minor salivary gland and lacrimal gland biopsies. Examination of classic SS biomarkers (SS-A/Ro, SS-B/La, antinuclear antibody, and rheumatoid factor) is a convenient and non-invasive way of evaluating patients for the presence of SS, even years prior to confirmed diagnosis, although not all SS patients will test positive, particularly those with early disease. Recently, newer biomarkers have been identified, including autoantibodies to salivary gland protein-1, parotid secretory protein, and carbonic anhydrase VI, and may allow for earlier diagnosis of SS. A diagnostic test kit is commercially available (Sjö®), incorporating these new biomarkers along with the classic autoantibodies. This advanced test has been shown to identify SS patients who previously tested negative against traditional biomarkers only. All patients with clinically significant ADDE should be considered for serological assessment for SS, given the

  1. Making the diagnosis of Sjögren's syndrome in patients with dry eye.

    Science.gov (United States)

    Beckman, Kenneth A; Luchs, Jodi; Milner, Mark S

    2016-01-01

    Sjögren's syndrome (SS) is a chronic and progressive systemic autoimmune disease that often presents initially with symptoms of dry eye and dry mouth. Symptoms are often nonspecific and develop gradually, making diagnosis difficult. Patients with dry eye complaints warrant a step-wise evaluation for possible SS. Initial evaluation requires establishment of a dry eye diagnosis using a combination of patient questionnaires and objective ocular tests, including inflammatory biomarker testing. Additional work-up using the Schirmer test and tear film break-up time can differentiate between aqueous-deficient dry eye (ADDE) and evaporative dry eye. The presence of ADDE should trigger further work-up to differentiate between SS-ADDE and non-SS-ADDE. There are numerous non-ocular manifestations of SS, and monitoring for SS-related comorbid findings can aid in diagnosis, ideally in collaboration with a rheumatologist. The clinical work-up of SS can involve a variety of tests, including tear function tests, serological tests for autoantibody biomarkers, minor salivary gland and lacrimal gland biopsies. Examination of classic SS biomarkers (SS-A/Ro, SS-B/La, antinuclear antibody, and rheumatoid factor) is a convenient and non-invasive way of evaluating patients for the presence of SS, even years prior to confirmed diagnosis, although not all SS patients will test positive, particularly those with early disease. Recently, newer biomarkers have been identified, including autoantibodies to salivary gland protein-1, parotid secretory protein, and carbonic anhydrase VI, and may allow for earlier diagnosis of SS. A diagnostic test kit is commercially available (Sjö(®)), incorporating these new biomarkers along with the classic autoantibodies. This advanced test has been shown to identify SS patients who previously tested negative against traditional biomarkers only. All patients with clinically significant ADDE should be considered for serological assessment for SS, given the

  2. A Transcriptional Signature of Fatigue Derived from Patients with Primary Sjögren’s Syndrome

    Science.gov (United States)

    James, Katherine; Al-Ali, Shereen; Tarn, Jessica; Cockell, Simon J.; Gillespie, Colin S.; Hindmarsh, Victoria; Locke, James; Mitchell, Sheryl; Lendrem, Dennis; Bowman, Simon; Price, Elizabeth; Pease, Colin T.; Emery, Paul; Lanyon, Peter; Hunter, John A.; Gupta, Monica; Bombardieri, Michele; Sutcliffe, Nurhan; Pitzalis, Costantino; McLaren, John; Cooper, Annie; Regan, Marian; Giles, Ian; Isenberg, David; Saravanan, Vadivelu; Coady, David; Dasgupta, Bhaskar; McHugh, Neil; Young-Min, Steven; Moots, Robert; Gendi, Nagui; Akil, Mohammed; Griffiths, Bridget; Wipat, Anil; Newton, Julia; Jones, David E.; Isaacs, John; Hallinan, Jennifer; Ng, Wan-Fai

    2015-01-01

    Background Fatigue is a debilitating condition with a significant impact on patients’ quality of life. Fatigue is frequently reported by patients suffering from primary Sjögren’s Syndrome (pSS), a chronic autoimmune condition characterised by dryness of the eyes and the mouth. However, although fatigue is common in pSS, it does not manifest in all sufferers, providing an excellent model with which to explore the potential underpinning biological mechanisms. Methods Whole blood samples from 133 fully-phenotyped pSS patients stratified for the presence of fatigue, collected by the UK primary Sjögren’s Syndrome Registry, were used for whole genome microarray. The resulting data were analysed both on a gene by gene basis and using pre-defined groups of genes. Finally, gene set enrichment analysis (GSEA) was used as a feature selection technique for input into a support vector machine (SVM) classifier. Classification was assessed using area under curve (AUC) of receiver operator characteristic and standard error of Wilcoxon statistic, SE(W). Results Although no genes were individually found to be associated with fatigue, 19 metabolic pathways were enriched in the high fatigue patient group using GSEA. Analysis revealed that these enrichments arose from the presence of a subset of 55 genes. A radial kernel SVM classifier with this subset of genes as input displayed significantly improved performance over classifiers using all pathway genes as input. The classifiers had AUCs of 0.866 (SE(W) 0.002) and 0.525 (SE(W) 0.006), respectively. Conclusions Systematic analysis of gene expression data from pSS patients discordant for fatigue identified 55 genes which are predictive of fatigue level using SVM classification. This list represents the first step in understanding the underlying pathophysiological mechanisms of fatigue in patients with pSS. PMID:26694930

  3. Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort

    Science.gov (United States)

    Carvajal Alegria, Guillermo; Guellec, Dewi; Mariette, Xavier; Gottenberg, Jacques-Eric; Dernis, Emmanuelle; Dubost, Jean-Jacques; Trouvin, Anne-Priscille; Hachulla, Eric; Larroche, Claire; Le Guern, Veronique; Cornec, Divi; Devauchelle-Pensec, Valérie; Saraux, Alain

    2016-01-01

    Objectives Neurological manifestations seem common in primary Sjögren's syndrome (pSS) but their reported prevalences vary. We investigated the prevalence and epidemiology of neurological manifestations in a French nationwide multicentre prospective cohort of patients with pSS, the Assessment of Systemic Signs and Evolution in Sjögren's syndrome (ASSESS) cohort. Methods The ASSESS cohort, established in 2006, includes 395 patients fulfilling American–European Consensus Group criteria for pSS. Demographic and clinical data were compared between patient groups with and without neurological manifestations, and across patient groups with peripheral nervous system (PNS) manifestations, central nervous system (CNS) manifestations and no neurological manifestations. Results Data at inclusion were available for 392 patients, whose mean age was 58±12 years. Mean follow-up was 33.9 months. Neurological manifestations were present in 74/392 (18.9%) patients, including 63 (16%) with PNS manifestations and 14 (3.6%) with CNS manifestations. Prevalences were 9.2% for pure sensory neuropathy, 5.3% for sensorimotor neuropathy, 1.3% for cerebral vasculitis and 1.0% for myelitis. Neurological manifestations were associated with greater pSS activity as assessed using the ESSDAI (9.4±6.8 vs 4.3±4.8; p<0.001) and proportion of patients taking immunomodulatory/immunosuppressive drugs (32.4% (24/74) versus 13.8% (44/318), p=0003). New neurological symptoms were more common in patients with than without prior neurological manifestations (RR=3.918 (95% CI 1.91 to 8.05); p<0.001). Conclusions Prevalences of peripheral and central neurological manifestations in pSS are about 15% and 5%, respectively. Neurological manifestations are associated with greater pSS activity. New neurological manifestations are more common in patients with prior neurological involvement. PMID:27110384

  4. Is YouTube useful as a source of information for Sjögren's syndrome?

    Science.gov (United States)

    Delli, K; Livas, C; Vissink, A; Spijkervet, F K L

    2016-04-01

    To quantitatively and qualitatively assess the characteristics of YouTube videos dealing with Sjögren's syndrome. A comprehensive electronic search was performed for 'Sjögren's syndrome' in YouTube. After excluding duplicates, irrelevant videos, and non-English-language domains, 70 videos were included for analysis. Videos were classified as useful, misleading, or personal experience. The overall quality of videos was scored according to the Global Quality Scale (GQS). Useful videos were assessed for reliability and comprehensiveness based on two 5-point scales. Key points of the misleading videos were explored and patients' personal experiences were further investigated. Thirty-six videos (51.4%) were classified as useful, 6 (8.6%) as misleading, and 28 (40%) as patient's personal experience. Independent users tend to upload videos with personal experience, while university channels/professional organizations share useful videos with evidence-based information. Significant difference was observed in GQS among useful, misleading videos, and patient's experiences. The mean reliability, comprehensiveness, and GQS scores of useful videos were 2.5 (s.d.:1.2), 2.6 (s.d.:1.4), and 3.4 (s.d.:1.0), respectively, whereas only six videos (16.7%) were rated as complete vis-à-vis content. The most frequently misleading topics were etiology and treatment. Specialists should refer their patients to validated e-information resources and actively participate in the development of video-sharing platforms. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  5. The usefulness of ultrasound in the diagnostics of Sjögren’s syndrome

    Directory of Open Access Journals (Sweden)

    Fadhil Saied

    2013-06-01

    Full Text Available Sjögren’s syndrome is an autoimmune exocrinopathy which manifests itself with dryness of the eyes and the oral cavity. These symptoms comprise a so-called sicca syndrome (xerostomia and xerophthalmia. Two forms of this disease may be distinguished: primary Sjögren’s syndrome which affects salivary glands and secondary Sjögren’s syndrome with other autoimmune diseases present such as rheumatoid arthritis, systemic lupus erythematosus or systemic scleroderma. The diagnosis is based on the classification criteria established in 2002 by a group of American and European scientists (American-European Consensus Group, which involve the interview and physical examination as well as serological, histopathological and radiological tests. Most of these examinations show some limitations such as invasiveness, expensiveness or limited accessibility. The latest research suggests that ultrasound examination may appear promising in the diagnostics of the main salivary glands: submandibular and parotid glands. It is an accessible and relatively cheap examination with high sensitivity and specificity values which are comparable to those obtained via conventional means used in the diagnostics of this disease, i.e. biopsy of the minor salivary glands, sialography and scintigraphy, as well as superior to those obtained in sialometry and Schirmer’s test. Additionally, ultrasonography correlates with the results of magnetic resonance imaging. Therefore, a number of authors claim that US examination should be included in the classification criteria of Sjögren’s syndrome. The aim of this article is to present the diagnostic capacity of the US examination in Sjögren’s syndrome using the current ultrasound classification systems based on the grey-scale, Doppler and contrast-enhanced examinations. The latest research confirms that the most valuable diagnostic criterion in Sjögren’s syndrome is the heterogeneity of the

  6. Patient burden of Sjögren’s: a comprehensive literature review revealing the range and heterogeneity of measures used in assessments of severity

    Science.gov (United States)

    Hammitt, Katherine M; Naegeli, April N; van den Broek, Remon W M; Birt, Julie A

    2017-01-01

    Context The severity of Sjögren’s syndrome has been evaluated using a wide variety of clinical measures and patient-reported outcomes (PROs). This may contribute to the lack of clarity concerning the burden of Sjögren’s from the patient perspective. Objective To perform a comprehensive peer-reviewed literature analysis of the patient aspects of Sjögren’s, focusing on PROs, to investigate the complexity underlying the evaluation of the syndrome and to elucidate the discordance between the different measures. Methods We searched Embase for articles published between January 2005 and September 2015. Research articles, clinical and diagnostic reviews, and validation studies with a focus on patient aspects of Sjögren’s were selected as the primary information source. Results 157 articles met the eligibility criteria. A wide variety of assessment measures used to evaluate glandular, extraglandular and functional domains were observed. Many different, non-validated Visual Analogue Scales, with a wide range of anchor words, were used in the quantification of Sjögren’s disease burden, impeding comparisons between studies. Relatively few clinical trials of drug therapies used validated scales: European League Against Rheumatism Sjögren’s Syndrome Patient Reported Index was used most often for symptom assessment and 36 Item Short Form Survey for quality of life (QoL). Conclusion A wide range and diversity of measures are used to evaluate the patient burden of Sjögren’s; most are not validated for use in this disease. PRO endpoints, validated specifically in Sjögren’s, that demonstrate improvement are needed. These measures should focus on QoL aspects important to patients and will most likely involve gauging change in function rather than patient-reported symptoms. PMID:28955493

  7. Increase of Intracellular BAFF in B Cells of Sjögren’s Patients Is Not Affected by Decrease of BAFFR

    Directory of Open Access Journals (Sweden)

    Jan Krejsek

    2015-07-01

    Full Text Available The presence of a broad spectrum of autoantibodies in Sjögren’s syndrome (SjS patients is the result of abnormal B-cell regulation that can be at least partially explained by abnormal BAFF/BAFFR regulation. The objective of this study was to determine both membrane and intracellular expression of BAFF/BAFFR in monocytes and B-cells in peripheral blood of 19 primary Sjögren’s syndrome patients and 20 healthy controls using flow cytometry. We also measured sBAFF in serum. Compared to healthy controls, both surface and intracellular expression of BAFF was significantly increased in monocytes and B-cells of SjS patients. Also serum sBAFF level was elevated. Expression of BAFFR on B-cells of SjS patients was surprisingly decreased, but there was no clear increase or decrease within monocytes. Our results indicate that activated monocytes communicate with B-cells via BAFF and BAFFR, so that B-cells are stimulated, but BAFF is also produced to stimulate cells in autocrine way. The decrease of BAFFR expression in SjS patients suggests that there is the mechanism that attempts to take over in order to balance the high level of BAFF.

  8. SjAPI-2 is the first member of a new neurotoxin family with Ascaris-type fold and KCNQ1 inhibitory activity.

    Science.gov (United States)

    Chen, Jing; Zhang, Chuangeng; Yang, Weishan; Cao, Zhijian; Li, Wenxin; Chen, Zongyun; Wu, Yingliang

    2015-08-01

    Peptides with Ascaris-type fold are a new kind of toxins founded from venomous animals recently. Functionally, these unique toxin peptides had been identified as potent protease inhibitors, which was similar to other known Ascaris-type peptides from non-venomous animals. Whether Ascaris-type peptides from venom animals have neurotoxin activities remains unclear. Here, a scorpion toxin SjAPI-2 with Ascaris-type fold was characterized to have a neurotoxin activity, which can selectively inhibit KCNQ1 potassium channel. SjAPI-2 had 62 amino acid residues, including 10 cysteine residues. Charged residue analyses showed that two acidic residues of SjAPI-2 were regionally distributed, and 10 basic residues were distributed widely throughout the whole peptide, which was similar to classical potassium channel toxins. Pharmacological studies confirmed that SjAPI-2 was a selective KCNQ1 potassium channel inhibitor with weak effects on other potassium channels, such as Kv1.1, Kv1.2, Kv1.3, SKCa2, SKCa3, and IKCa channels. Concentration-dependent studies showed that SjAPI-2 inhibited the KCNQ1 potassium channel with an IC50 of 771.5±169.9 nM. To the best of our knowledge, SjAPI-2 is the first neurotoxin with a unique Ascaris-type fold, providing novel insights into the divergent evolution of neurotoxins from venomous animals. Copyright © 2015 Elsevier B.V. All rights reserved.

  9. Sexual dimorphism in an animal model of Sjögren's syndrome: a potential role for Th17 cells

    Directory of Open Access Journals (Sweden)

    Alexandria Voigt

    2015-11-01

    Full Text Available Sjögren's syndrome is a complex autoimmune disease with an array of diverse immunological, genetic and environmental etiologies, making identification of the precise autoimmune mechanism difficult to define. One of the most distinctive aspects of Sjögren's syndrome is the high sexual dimorphism with women affected 10-20 times more than men. It is nearly impossible to study the sexual dimorphic development of Sjögren's syndrome in human patients; therefore it is pertinent to develop an appropriate animal model which resembles human disease. The data indicated that female C57BL/6.NOD-Aec1Aec2 mice developed an earlier onset of sialadenitis with a higher composition of CD3+ T cells and a 10-fold increase in glandular infiltration of Th17 cells at the onset of clinical disease compared to male mice. Inflammatory Th17 cells of female mice exhibited a stronger proliferation in response to disease-specific antigen than their male counterpart. At the clinical disease stage, altered autoantibody patterns can be detected in females whereas they are seldom observed in male C57BL/6.NOD-Aec1Aec2 mice. Interestingly, male C57BL/6.NOD-Aec1Aec2 mice developed an earlier loss of secretory function, despite the fact that female C57BL/6.NOD-Aec1Aec2 mice exhibited a more rapid secretory loss. This data indicates the strong sexual dimorphism in the SjS-susceptible C57BL/6.NOD-Aec1Aec2 animal model, making it an appropriate animal model to examine human disease.

  10. Uppsatsetik – om etisk problematik i studenters självständiga arbeten

    Directory of Open Access Journals (Sweden)

    Nina Nikku

    2013-04-01

    Full Text Available Studenternas självständiga arbeten på grund- och avancerad nivå kan inom vissa utbildningar innefatta studier av och med individer. Arbeten på denna nivå omfattas inte av forskningsetisk prövning. Det blir därför lärosätets, utbildningarnas och de enskilda handledarnas uppgift att förmedla ett forskningsetiskt förhållningssätt. Den här artikeln fokuserar på samhällsvetenskapliga och humanistiska utbildningar och bygger på en enkät till ett urval utbildningar; intervjuer, samtal och seminarier med erfarna handledare samt intervjuer med studenter. Därutöver har ett antal uppsatser granskats utifrån ett etiskt perspektiv. Den teoretiska referensramen utgörs av forskningsetisk vägledning, principer och lagstiftning. Utifrån materialet och referensramen görs en kartläggning av de etiska frågeställningar som aktualiseras för studenter och handledare med avseende på insamling, analys och presentation av data och empiri. Artikeln problematiserar i vilken mån forskningsetisk vägledning är tillämplig och tillräcklig för studenters självständiga arbeten. Resultatet visar att de intervjuade handledarna inte har en enhetlig inställning till vilka former av datainsamling studenterna bör få göra och att det finns en osäkerhet angående regler och beslutsordning kring detta. Framförallt aktualiseras detta i samband med studenternas val av informatörer och studerade grupper, bland annat vid studier av det som brukar benämnas svaga grupper. Det är dock viktigt att uppmärksamma att det är kombinationen av syfte, frågeställningar och metod som sammanvägt konstituerar den etiska känsligheten. 

  11. Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjögren's syndrome.

    Science.gov (United States)

    Lessard, Christopher J; Li, He; Adrianto, Indra; Ice, John A; Rasmussen, Astrid; Grundahl, Kiely M; Kelly, Jennifer A; Dozmorov, Mikhail G; Miceli-Richard, Corinne; Bowman, Simon; Lester, Sue; Eriksson, Per; Eloranta, Maija-Leena; Brun, Johan G; Gøransson, Lasse G; Harboe, Erna; Guthridge, Joel M; Kaufman, Kenneth M; Kvarnström, Marika; Jazebi, Helmi; Cunninghame Graham, Deborah S; Grandits, Martha E; Nazmul-Hossain, Abu N M; Patel, Ketan; Adler, Adam J; Maier-Moore, Jacen S; Farris, A Darise; Brennan, Michael T; Lessard, James A; Chodosh, James; Gopalakrishnan, Rajaram; Hefner, Kimberly S; Houston, Glen D; Huang, Andrew J W; Hughes, Pamela J; Lewis, David M; Radfar, Lida; Rohrer, Michael D; Stone, Donald U; Wren, Jonathan D; Vyse, Timothy J; Gaffney, Patrick M; James, Judith A; Omdal, Roald; Wahren-Herlenius, Marie; Illei, Gabor G; Witte, Torsten; Jonsson, Roland; Rischmueller, Maureen; Rönnblom, Lars; Nordmark, Gunnel; Ng, Wan-Fai; Mariette, Xavier; Anaya, Juan-Manuel; Rhodus, Nelson L; Segal, Barbara M; Scofield, R Hal; Montgomery, Courtney G; Harley, John B; Sivils, Kathy L

    2013-11-01

    Sjögren's syndrome is a common autoimmune disease (affecting ∼0.7% of European Americans) that typically presents as keratoconjunctivitis sicca and xerostomia. Here we report results of a large-scale association study of Sjögren's syndrome. In addition to strong association within the human leukocyte antigen (HLA) region at 6p21 (Pmeta = 7.65 × 10(-114)), we establish associations with IRF5-TNPO3 (Pmeta = 2.73 × 10(-19)), STAT4 (Pmeta = 6.80 × 10(-15)), IL12A (Pmeta = 1.17 × 10(-10)), FAM167A-BLK (Pmeta = 4.97 × 10(-10)), DDX6-CXCR5 (Pmeta = 1.10 × 10(-8)) and TNIP1 (Pmeta = 3.30 × 10(-8)). We also observed suggestive associations (Pmeta < 5 × 10(-5)) with variants in 29 other regions, including TNFAIP3, PTTG1, PRDM1, DGKQ, FCGR2A, IRAK1BP1, ITSN2 and PHIP, among others. These results highlight the importance of genes that are involved in both innate and adaptive immunity in Sjögren's syndrome.

  12. Mixed cryoglobulinemia-associated Sjögren's syndrome leading to spontaneous rupture of the kidney: a case report

    Directory of Open Access Journals (Sweden)

    Haddiya I

    2016-03-01

    Full Text Available Intissar Haddiya,1 Hakim Hamzaoui,1 Zitouna Alhamany,2 Fatime-zohra Berkchi,1 Hakima Rhou,1 Loubna Benamar,1 Naima Ouzeddoun,1 Rabea Bayahia1 1Department of Nephrology-Dialysis-Renal Transplantation, Ibn Sina University Hospital, Rabat, Morocco; 2Department of Pathology, Rabat Children's Hospital, Ibn Sina University Hospital, Rabat, Morocco Background: Spontaneous rupture of the kidney is uncommon and is mainly caused by renal tumors. Only a few cases are caused by vasculitis. We report here the first case of spontaneous rupture of kidney resulting from mixed cryoglobulinemia. Case presentation: A 44-year-old man presented with sudden onset of fever, acute pulmonary edema, left flank abdominal pain unassociated with trauma, and rapidly progressive renal failure requiring dialysis. Computed tomography of the abdomen revealed a large perirenal hematoma of the left kidney. During conservative surgery, the patient underwent renal biopsy that showed renal vasculitis and membranoproliferative glomerulonephritis with intracapillary microthrombi. Tests were positive for mixed cryoglobulinemia caused by Sjögren's syndrome. The patient was better after immunosuppressive therapy, with the disappearance of clinical symptoms and the recovery of baseline renal function. Conclusion: We report on this case and discuss a possible link between spontaneous rupture of kidney and mixed cryoglobulinemia-associated Sjögren's syndrome. Keywords: mixed cryoglobulinemia, Sjögren's syndrome, spontaneous rupture of kidney, renal hematoma, Wünderlich syndrome

  13. Dysfunction of Lacrimal and Salivary Glands in Sjögren's Syndrome: Nonimmunologic Injury in Preinflammatory Phase and Mouse Model

    Directory of Open Access Journals (Sweden)

    Toshiharu Hayashi

    2011-01-01

    Full Text Available Sjögren's syndrome (SjS is a chronic autoimmune disorder characterized by dry eyes and dry mouth due to dacryoadenitis and sialoadenitis with SS-A/Ro and/or SS-B/La autoantibodies in genetically predisposed individuals. Destruction of lacrimal and salivary glands by autoimmune reactions may lead to clinical manifestation. However, the mechanisms behind the decreased volume of secretions in tears and saliva are complex and are not fully understood. Exocrine gland dysfunction may precede autoimmunity (acquired immunity or represent a process independent from inflammation in the pathogenesis of SjS. The preceded functional and morphologic changes of those tissues by nonimmunologic injury before the development of inflammation at the sites of target organs have been implicated. This paper focuses on the several factors and components relating to glandular dysfunction and morphologic changes by nonimmunologic injury during the preinflammatory phase in mouse model, including the factors which link between innate immunity and adaptive immunity.

  14. Correlação entre sinais e sintomas de olho seco em pacientes portadores da síndrome de Sjögren Correlation between signals and symptoms of dry eye in Sjögren's syndrome patients

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    Marcello Novoa Colombo Barboza

    2008-08-01

    Full Text Available OBJETIVO: Estudar, numa determinada população de portadores da síndrome de Sjögren, a correlação entre os sinais e sintomas de olho seco. MÉTODOS: Foi constituído o Grupo Caso, formado por 17 pacientes portadores da síndrome de Sjögren e o Grupo Controle, formado por 25 pacientes normais. Para avaliação dos sintomas foi aplicado o questionário "Ocular Surface Disease Index (OSDI" nos pacientes de ambos os grupos e, em seguida, todos os indivíduos foram submetidos à realização dos testes de Schirmer I e II, coloração da superfície ocular com corante rosa bengala, paquimetria e estesiometria. Para análise das correlações entre sinais e sintomas foi utilizado o teste de correlação de Spearman e para comparação das médias dos valores encontrados no questionário "Ocular Surface Disease Index (OSDI" e nos testes clínicos entre os pacientes dos Grupos, foi utilizado teste t de Student para amostras independentes. RESULTADOS: Os resultados deste estudo evidenciaram fraca correlação entre os sintomas avaliados pelo questionário "Ocular Surface Disease Index (OSDI" e os testes oculares, o que indica que nem todos os pacientes que apresentavam sintomas exuberantes, exibiam testes proporcionalmente alterados. A média da sensibilidade da córnea de ambos os olhos dos pacientes do Grupo Caso esteve estatisticamente reduzida quando comparada à do Grupo Controle. Todos os parâmetros estudados no Grupo Caso apresentaram diferenças estatisticamente significantes (pPURPOSE: To study the correlation between the signals and symptoms of dry eye in Sjögren's syndrome patients. METHODS: We formed the case group with 17 Sjögren's syndrome patients and the control group with 25 normal patients. For evaluation of the symptoms the "Ocular Surface Disease Index (OSDI" questionnaire was applied to both groups and, after that, all the individuals were submitted to the ocular tests: Schirmer I and II, coloration of the ocular surface with

  15. Speech-language performance in Sjögren-Larsson syndrome.

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    Fuijkschot, Joris; Maassen, Ben; Gorter, Jan Willem; Gerven, Marjo van; Willemsen, Michèl

    2009-04-01

    To describe speech-language pathology in patients with Sjögren-Larsson syndrome (SLS) in relation to their cognitive and motor impairment. Observational case series. Cognitive functioning was assessed in 16 patients with SLS (nine males; seven females) using different neuropsychological tests. Speech-language pathology was studied focusing on dysarthria, oral motor functioning, speech intelligibility and language development. Potential correlations between speech-language pathology and other neurological symptoms (e.g. spasticity) were studied. The median cognitive developmental age was 5;8 (n = 13; range 3;5-8;0) years. A variable degree of mainly pseudobulbar dysarthria was found. Speech intelligibility was influenced by dysarthria, but was also related to language pathology. No correlation between motor functioning and dysarthria or cognitive development was observed. Dysarthria and language problems are important factors in daily life functioning of patients with SLS. Based upon the clinical profile found, early speech-language therapy is recommended in order to optimize their speech-language development.

  16. Voice, speech, and laryngeal features of primary Sjögren's syndrome.

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    Heller, Amanda; Tanner, Kristine; Roy, Nelson; Nissen, Shawn L; Merrill, Ray M; Miller, Karla L; Houtz, Daniel R; Ellerston, Julia; Kendall, Katherine

    2014-11-01

    This study examined voice, speech, and laryngeal characteristics in primary Sjögren's syndrome (pSS). Eleven patients (10 female, 1 male; mean [SD] age = 57 [14] years) from The University of Utah Division of Rheumatology provided connected speech and sustained vowel samples. Analyses included the Multi-Dimensional Voice Profile, the Analysis of Dysphonia in Speech and Voice, and dysphonia severity, speech clarity, and videolaryngostroboscopy ratings. Shimmer, amplitude perturbation quotient, and average fundamental frequency differed significantly from normative values (P speech (mean [SD] = 20.26 [8.36]) and sustained vowels (mean [SD] = 16.91 [11.08]). Ratings of dysphonia severity and speech clarity using 10-cm visual analog scales suggested mild-to-moderate dysphonia in connected speech (mean [SD] = 2.11 [1.72]) and sustained vowels (mean [SD] = 3.13 [2.20]) and mildly reduced speech clarity (mean [SD] = 1.46 [1.36]). Videolaryngostroboscopic ratings indicated mild-to-moderate dryness and mild reductions in overall laryngeal function. Voice Handicap Index scores indicated mild-to-moderate voice symptoms (mean [SD] = 43 [23]). Individuals with pSS may experience dysphonia and articulatory imprecision, typically in the mild-to-moderate range. These findings have implications for diagnostic and referral practices in pSS. © The Author(s) 2014.

  17. Sjögren's syndrome: the diagnostic potential of early oral manifestations preceding hyposalivation/xerostomia.

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    Mignogna, M D; Fedele, S; Lo Russo, L; Lo Muzio, L; Wolff, A

    2005-01-01

    Sjögren's syndrome (SS) is a systemic autoimmune exocrinopathy that affects mainly the salivary and lacrimal glands, leading to progressive reduction in saliva and tear flow. Although the underlying immuno-mediated glandular destruction is thought to develop slowly over several years, a long delay from the start of the symptoms to final diagnosis has been frequently reported. A limited knowledge concerning SS natural history is among the major causes of the actual diagnostic delay. Although very few studies have been focused on the analysis of SS early clinical onset, a series of oral features preceding xerostomia/hyposalivation development in patients eventually diagnosed as having SS have been reported. Sialochemistry alterations, salivary gland swelling, early dental loss and sialorrhea have been observed before the onset of typical signs and symptoms (namely xerostomia and/or hyposalivation), which usually lead to SS clinical presentation and diagnosis. Here we suggest, after evaluating available data, that the traditional 'untouchable' association between SS and xerostomia/hyposalivation might probably be reconsidered, and that astute clinicians should not underestimate the possible presence or development of SS in patients without xerostomia/hyposalivation and presenting these atypical early oral features.

  18. Present and future of biologic drugs in primary Sjögren's syndrome.

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    Sambataro, Domenico; Sambataro, Gianluca; Dal Bosco, Ylenia; Polosa, Riccardo

    2017-01-01

    Primary Sjögren's (pSS) syndrome is a chronic, autoimmune, and systemic disease characterized by xerostomia, xerophthalmia, muscle pain and fatigue. The disease may be complicated by a systemic involvement, such as a pulmonary fibrosis or the development of lymphoma which severely worsens the prognosis. Actually, there are no recommendations for the management of pSS. However, recent advances in the understanding of its pathogenesis have uncovered some pathways that have potential as therapeutic targets. Areas covered: In this review, the authors present the biologic drugs potentially valuable to the treatment of pSS in light of its physiopathology with a 'bird's eye' view of future prospects. The authors took into account relevant studies published from 2004 to 2016. Expert opinion: Biological treatment in pSS is a promising opportunity to potentially control disease activity and prevent its complication. Currently, inhibition of B-cell and IL-17 pathways seem to be the most promising avenues. New achievements in the knowledge of pSS pathophysiology are necessary in order to try to simultaneously predict the predominant pathogenic pathway, the kind of patients at major risk to develop a more severe disease, and the appropriate biological therapy to use.

  19. Evaluation of Swallowing Functions in Patients with Sjögren's Syndrome.

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    Eyigör, Sibel; Sezgin, Baha; Karabulut, Gonca; Öztürk, Kerem; Göde, Sercan; Kirazlı, Tayfun

    2017-04-01

    Patients with Sjögren's syndrome (SS) manifest symptoms such as dry eyes, dry mouth, and dysphagia. This study aims to evaluate the swallowing functions of the patients with SS. 69 patients with SS (65 females, 4 males) and 40 healthy individuals (33 females, 7 males) were included as study and control groups, respectively. Mean ages were 52.86 and 48.25 years for study and control groups, respectively. Swallowing functions were evaluated by fiberoptic endoscopic evaluation of swallowing (FEES). All the patients underwent FEES and were given 3, 5, and 10 ml water, yogurt, and fish-shaped crackers twice, respectively. The presence of bolus control, residue, penetration, and aspiration were evaluated. Additionally, certain types of foods triggering the dysphagia, any difficulties in bolus control, need to clean the throat, sensation of having a lump in the throat, sensation of choking, and xerostomia were included in the questionnaire. The MD Anderson Dysphagia Inventory and the Beck Depression Inventory were administered to patients. Considering the presence of residue with yogurt and fish cracker, there was a significant difference between groups (P  0.05). Regarding the MD Anderson Dysphagia Inventory, the average scores were 48.18 ± 13.21 and 87.6 ± 10.67 for study and control groups, respectively, and a statistically significant difference was detected (P quality of life in patients with SS.

  20. Saliva as a potential tool for diagnosis of dry mouth including Sjögren's syndrome.

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    Ohyama, K; Moriyama, M; Hayashida, J-N; Tanaka, A; Maehara, T; Ieda, S; Furukawa, S; Ohta, M; Imabayashi, Y; Nakamura, S

    2015-03-01

    Recently, the use of saliva as a diagnostic tool has gained considerable attention because it is non-invasive and easy to perform repeatedly. In this study, we focused on soluble molecules in saliva to establish a new diagnostic method for xerostomia. Saliva was obtained from 90 patients with Sjögren's syndrome (SS), 22 patients with xerostomia associated with neurogenic/neuropsychiatric disorders and drugs (XND), 30 patients with radiation-induced xerostomia (RX), and 36 healthy controls. Concentrations of helper T (Th) cytokines in saliva were measured by flow cytometric analysis. Concentrations of secretory IgA (SIgA) and chromogranin A (CgA) were measured by ELISA. Unstimulated and stimulated whole saliva from patients with SS, XND, and RX was significantly reduced compared with controls. Th1 and Th2 cytokines from SS patients were significantly higher than controls. Furthermore, Th2 cytokines were closely associated with strong lymphocytic accumulation in salivary glands from SS patients, while Th1 and Th17 cytokines were negatively associated. SIgA levels were not significantly different between all patient groups and controls. CgA levels from XND patients were significantly higher than controls. The measurement of cytokines, CgA, and SIgA in saliva is suggested to be useful for the diagnosis of xerostomia and also to reveal disease status. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. A short neuropsychological evaluation of patients with primary Sjögren's syndrome

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    Dóra-Neide Rodrigues

    2014-01-01

    Full Text Available Objective: To investigate cognitive deficits in patients with primary Sjögren’s syndrome (PSS. Method: Eighteen patients with PSS, aged between 25 and 61 years, were subjected to a short neuropsychological battery and compared with 18 patients with multiple sclerosis and 18 healthy controls. Results: The analysis of variance (ANOVA revealed that the clinical groups had significantly worse performance than the control group on the Rey Auditory Verbal Learning Test 3; (F 2,53 =3.500, p=0.038 and 7 (F 2,53 =5.068, p=0.010. The clinical groups had elevated levels of depression on the Beck Depression Inventory (BDI; (p=0.003. The analysis of the data from the Trail Making Test B-A revealed a significant difference between the clinical and control groups (p=0.023. The analysis of covariance with BDI score as a covariate, did not change the outcome. Conclusion: Our study revealed cognitive deficits in patients with PSS detectable by a short neuropsychological battery.

  2. Interferons in Sjögren’s syndrome: genes, mechanisms, and effects

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    He eLi

    2013-09-01

    Full Text Available Sjögren’s syndrome (SS is a common, progressive autoimmune exocrinopathy distinguished by dry eyes and mouth and affects ~0.7% of European population. Overexpression of transcripts induced by interferons (IFN, termed as an ‘IFN signature’, has been found in SS patients. Four microarray studies have been published in SS that identified dysregulated genes within type I IFN signaling in either salivary glands or peripheral blood of SS patients. The mechanism of this type I IFN activation is still obscure, but several possible explanations have been proposed, including virus infection-initiated and immune-complex-initiated type I IFN production by plasmacytoid dendritic cells (pDCs. Genetic predisposition to increased type I IFN signaling is supported by candidate gene studies showing evidence for association of variants within IFN-related genes. Once activated, IFN signaling may contribute to numerous aspects of SS pathophysiology, including lymphocyte infiltration into exocrine glands, autoantibody production, and glandular cell apoptosis. Thus, dysregulation of IFN pathways is an important feature that can be potentially used as a serum biomarker for diagnosis and targeting of new treatments in this complex autoimmune disease.

  3. Neurologic Complications Associated with Sjögren’s Disease: Case Reports and Modern Pathogenic Dilemma

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    Michele Colaci

    2014-01-01

    Full Text Available Objectives. Sjögren’s syndrome (SS may be complicated by some neurological manifestations, generally sensory polyneuropathy. Furthermore, involvement of cranial nerves was described as rare complications of SS. Methods. We reported 2 cases: the first one was a 40-year-old woman who developed neuritis of the left optic nerve as presenting symptom few years before the diagnosis of SS; the second was a 54-year-old woman who presented a paralysis of the right phrenic nerve 7 years after the SS onset. An exhaustive review of the literature on patients with cranial or phrenic nerve involvements was also carried out. Results. To the best of our knowledge, our second case represents the first observation of SS-associated phrenic nerve mononeuritis, while optic neuritis represents the most frequent cranial nerve involvement detectable in this connective tissue disease. Trigeminal neuropathy is also frequently reported, whereas neuritis involving the other cranial nerves is quite rare. Conclusions. Cranial nerve injury is a harmful complication of SS, even if less commonly recorded compared to peripheral neuropathy. Neurological manifestations may precede the clinical onset of SS; therefore, in patients with apparently isolated cranial nerve involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable.

  4. Loss of PKCδ results in characteristics of Sjögren's syndrome including salivary gland dysfunction.

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    Banninger, G P; Cha, S; Said, M S; Pauley, K M; Carter, C J; Onate, M; Pauley, B A; Anderson, S M; Reyland, M E

    2011-09-01

    Chronic infiltration of lymphocytes into the salivary and lacrimal glands of patients with Sjögren's syndrome (SS) leads to destruction of acinar cells and loss of exocrine function. Protein kinase C-delta (PKCδ) is known to play a critical role in B-cell maintenance. Mice in which the PKCδ gene has been disrupted have a loss of B-cell tolerance, multiple organ lymphocytic infiltration, and altered apoptosis. To determine whether PKCδ contributes to the pathogenesis of SS, we quantified changes in indicators of SS in PKCδ-/- mice as a function of age. Salivary gland histology, function, the presence of autoantibodies, and cytokine expression were examined. Submandibular glands were examined for the presence of lymphocytic infiltrates, and the type of infiltrating lymphocyte and cytokine deposition was evaluated by immunohistochemistry. Serum samples were tested by autoantibody screening, which was graded by its staining pattern and intensity. Salivary gland function was determined by saliva collection at various ages. PKCδ-/- mice have reduced salivary gland function, B220+ B-cell infiltration, anti-nuclear antibody production, and elevated IFN-γ in the salivary glands as compared to PKCδ+/+ littermates. PKCδ-/- mice have exocrine gland tissue damage indicative of a SS-like phenotype. © 2011 John Wiley & Sons A/S.

  5. Controversies on Rituximab Therapy in Sjögren Syndrome-Associated Lymphoproliferation

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    Luca Quartuccio

    2009-01-01

    Full Text Available Sjögren's syndrome (SS is a systemic autoimmune disease characterized by chronic inflammation of salivary and lachrymal glands, and frequently accompanied by systemic symptoms. A subgroup of SS patients develops malignant B cell non-Hodgkin's lymphoma (NHL, usually of the mucosa-associated lymphoid tissue (MALT type and very often located in the major salivary glands. Currently, there is a lack of evidence-based intervention therapy which may influence SS-related chronic inflammation and lymphoproliferation. B cells are involved in the pathogenesis of SS, and B cell downregulation may lead to a decrease of disease activity. Rituximab (RTX, a chimeric monoclonal antibody targeting the CD20 antigen on the B cell surface, has been successfully investigated in other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, ANCA-associated vasculitis, and mixed cryoglobulinemic syndrome. Preliminary experiences of RTX therapy in SS patients with or without a lymphoproliferative disorder suggest that SS patients with more residual exocrine gland function might better benefit from RTX. Efficacy of RTX in SS-associated B-cell lymphoma, mainly in low-grade salivary gland lymphomas, remains an open issue.

  6. A monoclonal antibody (SJ-9A4) to P24 present on common alls, neuroblastomas and platelets - I. Characterization and development of a unique radioimmunometric assay.

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    Komada, Y; Peiper, S C; Melvin, S L; Metzger, D W; Tarnowski, B H; Green, A A

    1983-01-01

    We report the development and characterization of SJ-9A4, a monoclonal antibody (MoAb) produced against common acute lymphoblastic leukemia (C-ALL) cell lines. SJ-9A4 reacted with C-ALL, B-cell chronic lymphocytic leukemia (B-CLL), platelets and C-ALL neuroblastoma (NB) and the K562 cell lines. It had no significant reactivity with erythrocytes, granulocytes, circulating T or B lymphocytes, monocytes, granulocytic cell lines or a Ewing's sarcoma cell line. SJ-9A4 was shown to recognize the same region as two other MoAb to the p24 antigen, BA-2 and DU-ALL-1, as demonstrated by their ability to inhibit the binding of labeled SJ-9A4 to NALM-1 and NB cells. Other MoAb: J5, PI 153/3 and monoclonal anti-HLA-DR antibodies gave no inhibition. A solid phase indirect radioimmunometric assay (IRA) was developed which enabled the detection of P24 from C-ALL cells, utilizing its ability to bind the Ricinus communis agglutinin (RCA1) or wheat germ agglutinin (WGA) and SJ-9A4 simultaneously. When BA-2 and DU-ALL-1 were used in place of SJ-9A4, similar IRA results were obtained. Using the RCA1/SJ-9A4-IRA, P24 from as few as 1.6 X 10(4) cells of a C-ALL cell line could be detected; however, similar extracts of NB cell lines were negative despite high levels of SJ-9A4 binding to intact cells. The presence of P24 in NB extracts was demonstrated by (1) preincubation of NB extracts with SJ-9A4 which blocked MoAb binding to P24 and (2) immunoadsorption of P24 from solubilized membranes of 35S-methionine (met) labeled NB cells. Treatment of NB cells with neuraminidase did not result in IRA binding when either RCA1 or WGA were used as the solid phase lectin indicating that the differences in lectin affinity are not due to over sialation of NB membrane glycoproteins. These findings demonstrate a difference in the glycosylation of P24 from C-ALL and NB cells.

  7. Are the women with Sjögren's Syndrome satisfied with their sexual activity?

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    Hatice Isik

    Full Text Available ABSTRACT Objective: Females with Sjögren's Syndrome (SS often experience vaginal dryness and dyspareunia, along with glandular and extraglandular symptoms. We aimed to evaluate sexual function and life quality in women with SS. Methods: Forty-six premenopausal women with SS and 47 age-matched controls were studied. Age, duration of the disease, medications, and comorbid diseases were noted. Participants completed 36-Item Short Form Health Survey (SF-36 and Female Sexual Function Index (FSFI. Patients were asked about vaginal discharge and itching in the last month, and if they informed their rheumatologists about any sexual problems. Gynecologic examinations were performed and vaginal smears were taken on each participant. Results: The median total scores of FSFI were significantly lower in the SS group than the controls [17.12 (2.4-27.8 and 27.4 (16.9-36.0, respectively, p < 0.001]. In the SS group, 37 (80.4% and in the control group 18 (38.3% of patients were sexually dissatisfied (p < 0.001. Vaginal dryness and lubricant use were significantly increased in patients with SS compared to controls (p < 0.001. Life quality scores were significantly lower in patients with SS than the controls (p < 0.001. Vaginal dryness was negatively correlated with FSFI total (r = −0.312, p = 0.035 and subscores except desire and arousal. Physical functioning, role physical and role emotional scores were positively correlated with total FSFI scores (r = 0.449, p = 0.002, r = 0.371, p = 0.011, r = 0.299, p = 0.043, respectively. Conclusions: Women with SS experience less satisfaction with sexual activity, which can be affected by age, vaginal dryness, physical pain, and impaired function due to the disease. Therefore, rheumatologists should pay attention to these symptoms and management.

  8. Standardisation of labial salivary gland histopathology in clinical trials in primary Sjögren's syndrome.

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    Fisher, Benjamin A; Jonsson, Roland; Daniels, Troy; Bombardieri, Michele; Brown, Rachel M; Morgan, Peter; Bombardieri, Stefano; Ng, Wan-Fai; Tzioufas, Athanasios G; Vitali, Claudio; Shirlaw, Pepe; Haacke, Erlin; Costa, Sebastian; Bootsma, Hendrika; Devauchelle-Pensec, Valerie; Radstake, Timothy R; Mariette, Xavier; Richards, Andrea; Stack, Rebecca; Bowman, Simon J; Barone, Francesca

    2017-07-01

    Labial salivary gland (LSG) biopsy is used in the classification of primary Sjögren's syndrome (PSS) and in patient stratification in clinical trials. It may also function as a biomarker. The acquisition of tissue and histological interpretation is variable and needs to be standardised for use in clinical trials. A modified European League Against Rheumatism consensus guideline development strategy was used. The steering committee of the ad hoc working group identified key outstanding points of variability in LSG acquisition and analysis. A 2-day workshop was held to develop consensus where possible and identify points where further discussion/data was needed. These points were reviewed by a subgroup of experts on PSS histopathology and then circulated via an online survey to 50 stakeholder experts consisting of rheumatologists, histopathologists and oral medicine specialists, to assess level of agreement (0-10 scale) and comments. Criteria for agreement were a mean score ≥6/10 and 75% of respondents scoring ≥6/10. Thirty-nine (78%) experts responded and 16 points met criteria for agreement. These points are focused on tissue requirements, identification of the characteristic focal lymphocytic sialadenitis, calculation of the focus score, identification of germinal centres, assessment of the area of leucocyte infiltration, reporting standards and use of prestudy samples for clinical trials. We provide standardised consensus guidance for the use of labial salivary gland histopathology in the classification of PSS and in clinical trials and identify areas where further research is required to achieve evidence-based consensus. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  9. Activation of Stimulator of Interferon Genes (STING) and Sjögren Syndrome.

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    Papinska, J; Bagavant, H; Gmyrek, G B; Sroka, M; Tummala, S; Fitzgerald, K A; Deshmukh, U S

    2018-03-01

    Sjögren syndrome (SS), a chronic autoimmune disorder causing dry mouth, adversely affects the overall oral health in patients. Activation of innate immune responses and excessive production of type I interferons (IFNs) play a critical role in the pathogenesis of this disorder. Recognition of nucleic acids by cytosolic nucleic acid sensors is a major trigger for the induction of type I IFNs. Upon activation, cytosolic DNA sensors can interact with the stimulator of interferon genes (STING) protein, and activation of STING causes increased expression of type I IFNs. The role of STING activation in SS is not known. In this study, to investigate whether the cytosolic DNA sensing pathway influences SS development, female C57BL/6 mice were injected with a STING agonist, dimethylxanthenone-4-acetic acid (DMXAA). Salivary glands (SGs) were studied for gene expression and inflammatory cell infiltration. SG function was evaluated by measuring pilocarpine-induced salivation. Sera were analyzed for cytokines and autoantibodies. Primary SG cells were used to study the expression and activation of STING. Our data show that systemic DMXAA treatment rapidly induced the expression of Ifnb1, Il6, and Tnfa in the SGs, and these cytokines were also elevated in circulation. In contrast, increased Ifng gene expression was dominantly detected in the SGs. The type I innate lymphoid cells present within the SGs were the major source of IFN-γ, and their numbers increased significantly within 3 d of treatment. STING expression in SGs was mainly observed in ductal and interstitial cells. In primary SG cells, DMXAA activated STING and induced IFN-β production. The DMXAA-treated mice developed autoantibodies, sialoadenitis, and glandular hypofunction. Our study demonstrates that activation of the STING pathway holds the potential to initiate SS. Thus, apart from viral infections, conditions that cause cellular perturbations and accumulation of host DNA within the cytosol should also be

  10. Management of primary Sjögren's syndrome: recent developments and new classification criteria.

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    Del Papa, Nicoletta; Vitali, Claudio

    2018-02-01

    For many years primary Sjögren's syndrome (pSS) has been considered an orphan disease, since no specific therapies were recognized as being capable of contrasting the development and progression of this disorder. The treatment of oral and ocular features, as well as of the systemic organ involvement, has been entrusted to the joint management of different subspecialty physicians, like ophthalmologists, otolaryngologists, dentists and rheumatologists. These latter subspecialty doctors are usually more involved in the treatment of systemic extraglandular involvement and, to do it, they have long been using the conventional therapies borrowed by the treatment schedules adopted in other systemic autoimmune diseases. The increasing knowledge of the biological pathways that are operative in patients with pSS, and the parallel development of molecular biology technology, have allowed the production and availability of a number of biological agents able to positively act on different disease mechanisms, and thus are candidates for testing in therapeutic trials. Meanwhile, the scientific community has made a great effort to develop new accurate and validated classification criteria and outcome measures to be applied in the selection of patients to be included and monitored in therapeutic studies. Some of the new-generation biotechnological agents have been tested in a number of open-label and randomized controlled trials that have produced in many cases inconclusive or contradictory results. Behind the differences in trial protocols, adopted outcome measures and predefined endpoints, reasons for such unsatisfactory results can be found in the large heterogeneity of clinical subtypes in the examined cohorts. The future challenge for a substantial advancement in the therapeutic approach to pSS could be to identify the pathologic mechanisms, outcome tools and biomarkers that characterize the different subsets of the disease in order to test carefully selected target therapies

  11. Sjögren's syndrome complicated by interstitial cystitis: A case series and literature review.

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    Darrieutort-Laffite, Christelle; André, Vincent; Hayem, Gilles; Saraux, Alain; Le Guern, Véronique; Le Jeunne, Claire; Puéchal, Xavier

    2015-07-01

    To characterize the interstitial cystitis (IC) associated with Sjögren's syndrome (SS). Report of three new cases. Only cases fulfilling the American-European consensus criteria for SS and the European Society for the Study of Interstitial Cystitis criteria with positive histological findings for IC were included. Thirteen cases of SS and IC have been reported in women, including the three reported here, with a mean age of 54 years. SS appeared first in 77% (n=10) of cases, a mean of 6.6 years before IC. The symptoms of IC included pollakiuria (n=11), lower abdominal pain (n=8), urinary urgency (n=5), painful micturition (n=6), hematuria (n=3) and dysuria (n=3). Urinary dilatation occurred in three cases, leading to acute renal failure in two patients. The diagnosis of IC was confirmed by anatomical evidence of cystitis inflammation on bladder biopsy in all (n=13) patients. Treatment was reported for nine patients, seven of whom (78%) received corticosteroid treatment, which was partially or completely effective in six cases. Immunosuppressive treatment was added in three cases (cyclosporine, n=2; azathioprine, n=1; cyclophosphamide, n=1). Local bladder treatments were performed, with hydraulic distension in five cases and DMSO instillation in one patient. A urinary catheter was inserted in the two cases of acute obstructive renal failure. Urinary symptoms without infection should lead the physician to consider a diagnosis of IC in SS patients. Urinary dilatation may occur, leading to acute obstructive renal failure. Corticosteroid treatment may be effective and local treatments have been tried. Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  12. Predominant Glandular Cholinergic Dysautonomia in Patients With Primary Sjögren's Syndrome.

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    Imrich, Richard; Alevizos, Ilias; Bebris, Lolita; Goldstein, David S; Holmes, Courtney S; Illei, Gabor G; Nikolov, Nikolay P

    2015-05-01

    The autonomic nervous system (ANS) modulates exocrine gland function. Available data show poor correlation between the degree of function and destruction of the exocrine glands in primary Sjögren's syndrome (SS), suggesting that other mechanisms, such as autonomic dysfunction, may be important in these patients. The aim of this study was to perform a comprehensive analysis of sympathoneural and sympathetic cholinergic function in well-characterized patients with primary SS. Twenty-one patients with primary SS (mean ± SEM age 44.2 ± 2.8 years) and 13 healthy control subjects (mean ± SEM age 50.8 ± 1.9 years) were assessed during orthostasis and intravenous injection of edrophonium (10 mg). The postganglionic sympathetic cholinergic system was evaluated by assessing sweat production by means of the Quantitative Sudomotor Axon Reflex Test (QSART). Tests of gastric emptying were used to assess the gastrointestinal ANS in primary SS patients. The velocity index and the acceleration index were significantly higher (P < 0.05) in patients with primary SS as compared to controls, both before and during the orthostatic and edrophonium tests. Findings of other hemodynamic and neurochemical parameters did not differ between primary SS patients and controls during the orthostasis and edrophonium test; however, the edrophonium-induced saliva increment was lower in primary SS patients (P = 0.002). Abnormally low sweat production was found in 4 primary SS patients but in none of the controls, as determined by the QSART. Gastric empting was delayed in 53% of primary SS patients. We observed subtle differences in several ANS domains, including the gastrointestinal and sympathocholinergic systems, suggesting the presence of a complex ANS dysfunction in primary SS. The impact was greatest on the exocrine glands, with subtle differences in the cardiac parasympathetic function that were independent of glandular inflammation and atrophy, suggesting an alternative mechanism

  13. Predominant Glandular Cholinergic Dysautonomia in Patients with Primary Sjögren’s Syndrome

    Science.gov (United States)

    Imrich, Richard; Alevizos, Ilias; Bebris, Lolita; Goldstein, David S.; Holmes, Courtney S.; Illei, Gabor G.; Nikolov, Nikolay P.

    2015-01-01

    Objectives The autonomic nervous system (ANS) modulates exocrine gland function. Available data show poor correlation between the degree of exocrine gland function and destruction in primary Sjögren’s syndrome (pSS) suggesting other mechanisms, such as autonomic dysfunction may be important in these patients. We performed a comprehensive analysis of sympathoneural and sympathetic cholinergic function in well-characterized patients with pSS. Methods 21 pSS patients (mean±SE age 44±3 years) and in 13 healthy controls (51±2 years) were assessed during orthostasis and intravenous injection of edrophonium (10 mg). The postganglionic sympathetic cholinergic system was evaluated by assessing sweat production by the quantitative sudomotor axon reflex test (QSART). Gastric empting testing assessed the gastro-intestinal ANS in pSS patients. Results Velocity index and acceleration index were significantly higher (p<0.05) in pSS compared to controls before and during the orthostatic and edrophonium tests. Other hemodynamic and neurochemical parameters did not differ between pSS patients and controls during the orthostasis and edrophonium test, however, edrophonium-induced saliva increment was lower in pSS (p=0.002). Abnormally low sweat production was found in four (N=4) pSS patients but in none of the controls in the QSART. Gastric empting was delayed in 53 % of pSS patients. Conclusion We observed subtle differences in several ANS domains, including gastrointestinal and sympathocholinergic system suggesting a complex ANS dysfunction in pSS. The impact was the largest on the exocrine glands with subtle differences in the cardiac parasympathetic function independent of glandular inflammation and atrophy, suggesting an alternative pathogenesis mechanism of the disease in pSS. PMID:25622919

  14. Primary Sjögren's syndrome and pregnancy: A report of 18 cases.

    Science.gov (United States)

    Demarchi, Julia; Papasidero, Silvia B; Klajn, Diana; Alba, Paula; Babini, Alejandra M; Durigan, Virginia; Gobbi, Carla; Raiti, Laura

    2017-08-24

    Primary Sjögren's syndrome (pSS) is a condition that predominantly affects women. Reports of pregnancy outcome in these patients are limited and contradictory. To describe pregnancy characteristics and outcomes and newborn morbidity in women with pSS. We included women with pSS who became pregnant after the onset of the symptoms of the disease. Clinical and serological characteristics, risk factors and previous maternal comorbidities are described. For each pregnancy in a woman with pSS, we recorded pregnancy course and outcome and newborn condition. We assessed 11 patients with 18 pregnancies after the onset of pSS symptoms. All of them presented FAN +; 10 anti-Ro / SSA + and 7 anti-La / SSB +. The mean age in years at the onset of symptoms was 24.9 (SD 6.9) and at the time of pregnancy was 30.3 (SD 5.4). Thirteen pregnancies happened before the diagnosis, reporting only one miscarriage. Two preterm births, 1 case of oligohydramnios, 2 of premature membrane rupture and 2 low birthweight babies were reported after the onset of pSS symptoms. There was 1 newborn with congenital atrioventricular block and another with neonatal cutaneous lupus. All the women with pregnancy complications (n=6) had anti-Ro/SSA antibodies. Almost half of the pregnancies assessed in women with pSS were associated with complications not attributable to factors other than the disease. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  15. Effect of laser acupuncture on salivary flow rate in patients with Sjögren's syndrome.

    Science.gov (United States)

    Cafaro, Adriana; Arduino, Paolo Giacomo; Gambino, Alessio; Romagnoli, Ercole; Broccoletti, Roberto

    2015-08-01

    Sjögren's syndrome (SS) is a multisystem autoimmune disease characterized by hypofunction of the salivary and lacrimal glands, frequently relieved with symptomatic treatments, such as saliva substitutes, eye lubricants, and cholinergic stimulators. The aim of this pilot randomized placebo-controlled study was to estimate the effects of laser acupuncture on salivary flow rates in patients with severe hyposalivation due to SS. A prospective cohort of 26 female patients affected by SS has been evaluated. The laser therapy equipment used was the Pointer Pulse, emitting light in the red visible spectrum (650 nm), with a power of 5 mW and an irradiation time of 120 s per acupoint, in an area of 3.14 mm(2) (fluence = 19.2 J/cm(2), power density = 0.16 W/cm(2), total dose = 0.6 J). The following acupuncture points were stimulated bilaterally: LI 2 Erjian, ST 5 Daying, ST 6 Jiache, ST 7 Xiaguan, SI 19 Tinggong, and BL 13 Feishu. True laser acupuncture led to a significantly higher amount of saliva production, measured after the end of the protocol (5 weeks), and during the 6-month follow-up period. The results are stable from the end of the protocol until the 3rd month of follow-up; during the last control, a slight but significant decrease in production has also been shown. This preliminary study proposes laser acupuncture as a possible treatment for improving salivary flow rates in patients with SS, but further validation on a larger sample is still necessary.

  16. Thrombospondin-derived peptide attenuates Sjögren's syndrome-associated ocular surface inflammation in mice.

    Science.gov (United States)

    Contreras Ruiz, L; Mir, F A; Turpie, B; Masli, S

    2017-04-01

    Sjögren's syndrome is the second most common rheumatic disease in which autoimmune response targets exocrine glands (salivary and lacrimal glands) result in clinical symptoms of dry mouth and dry eye. Inflammation of the lacrimal gland induces tear abnormalities that contribute to the inflammation of the ocular surface, which includes ocular mucosa. Thrombospondin-1 (TSP-1) plays a critical regulatory role in the ocular mucosa and as such TSP-1 -/- mice develop spontaneously chronic ocular surface inflammation associated with Sjögren's syndrome. The autoimmune pathology is also accompanied by a peripheral imbalance in regulatory (T reg ) and inflammatory Th17 effectors. In this study, we demonstrate an in-vitro effect of a CD47-binding TSP-derived peptide in the induction of transforming growth factor (TGF)-β1-secreting forkhead box protein 2 (Foxp3 + ) T regs from activated CD4 + CD25 - T cells and the inhibition of pathogenic T helper type 17 (Th17)-promoting interleukin (IL)-23 derived from antigen-presenting cells. The in-vivo administration of this peptide promotes Foxp3 + T reg induction and inhibition of Th17 development. Consistent with these results, topical administration of CD47-binding TSP peptide, both before and after the onset of the disease, attenuates clinical symptoms of SS-associated dry eye in TSP-1 -/- mice. Augmented expression of Foxp3 detected in the draining lymph nodes of TSP peptide -treated mice compared to those treated with control peptide suggests the ability of TSP peptide to restore peripheral immune imbalance. Thus, our results suggest that TSP-derived peptide attenuates Sjögren's syndrome-associated dry eye and autoimmune inflammation by preventing Th17 development while promoting the induction of T regs . Collectively, our data identify TSP-derived peptide as a novel therapeutic option to treat autoimmune diseases. © 2017 British Society for Immunology.

  17. CLASP/SJ Observations of Rapid Time Variations in the Lyα Emission in a Solar Active Region

    Science.gov (United States)

    Ishikawa, Shin-nosuke; Kubo, Masahito; Katsukawa, Yukio; Kano, Ryouhei; Narukage, Noriyuki; Ishikawa, Ryohko; Bando, Takamasa; Winebarger, Amy; Kobayashi, Ken; Trujillo Bueno, Javier; Auchère, Frédéric

    2017-09-01

    The Chromospheric Lyα SpectroPolarimeter (CLASP) is a sounding rocket experiment launched on 2015 September 3 to investigate the solar chromosphere and transition region. The slit-jaw (SJ) optical system captured Lyα images with a high time cadence of 0.6 s. From the CLASP/SJ observations, many variations in the solar chromosphere and transition region emission with a timescale of <1 minute were discovered. In this paper, we focus on the active region within the SJ field of view and investigate the relationship between short (<30 s) temporal variations in the Lyα emission and the coronal structures observed by Solar Dynamics Observatory/Atmospheric Imaging Assembly (AIA). We compare the Lyα temporal variations at the coronal loop footpoints observed in the AIA 211 Å (≈2 MK) and AIA 171 Å (≈0.6 MK) channels with those in the regions with bright Lyα features without a clear association with the coronal loop footpoints. We find more short (<30 s) temporal variations in the Lyα intensity in the footpoint regions. Those variations did not depend on the temperature of the coronal loops. Therefore, the temporal variations in the Lyα intensity at this timescale range could be related to the heating of the coronal structures up to temperatures around the sensitivity peak of 171 Å. No signature was found to support the scenario that these Lyα intensity variations were related to the nanoflares. Waves or jets from the lower layers (lower chromosphere or photosphere) are possible causes for this phenomenon.

  18. Evaluation of water-stress tolerance of Acala SJ 2 and Auburn 2 cotton cultivars in a phenotyping platform

    Directory of Open Access Journals (Sweden)

    Cleber Morais Guimarães

    2017-08-01

    Full Text Available Due to the current scarcity of water, which may be aggravated by climate changes, it is important to develop cultivars tolerant to water stress. For this, information regarding the variability of the tolerance of cultivars to this stress is required. This information can be obtained by using phenotyping platforms, which allow a large-scale and automated assessment of crop traits related to productivity under water stress. This study took place in a greenhouse and used a phenotyping platform to evaluate some agronomic and physiological traits of two cotton cultivars with differing tolerances to this stress. The experiment was performed in a randomized block design, in a split-plot scheme with four replicates. The main plots were composed of five water regimes and the subplots of two cultivars, Acala SJ 2 and Auburn 2. The water regimes consisted of a well-irrigated treatment (daily replacement of 100% of evapotranspired water and four water restriction regimes (20%, 40%, 60% and 80% of evapotranspired water at the well-irrigated treatment. The phenotyping platform and protocol (different percentages of daily replacement of evapotranspired water employed were suitable to the evaluated cotton cultivars for water-stress tolerance, and allowed the determination of the cultivar most tolerant (Acala SJ 2 and of the cultivar most sensitive (Auburn 2 to water stress. ‘Acala SJ 2’ displayed better physiological and agronomic traits in all water regimes, including higher root density in the upper soil layer, as well as higher water use than ‘Auburn 2’, which explained its higher seed yield under the conditions employed.

  19. A Turkish family with Sjögren-Larsson syndrome caused by a novel ALDH3A2 mutation

    Directory of Open Access Journals (Sweden)

    Faruk Incecik

    2013-01-01

    Full Text Available Sjögren-Larsson syndrome (SLS is an inherited neurocutaneous disorder caused by mutations in the aldehyde dehydrogenase family 3 member A2 (ALDH3A2 gene that encodes fatty aldehyde dehydrogenase. Affected patients display ichthyosis, mental retardation, and spastic diplegia. More than 70 mutations in ALDH3A2 have been discovered in SLS patients. We diagnosed two brothers age of 12 and 20 years with characteristic features of this rare syndrome. Magnetic resonance imaging showed demyelinating disease in both of them. We described a novel homozygous, c. 835 T > A (p.Y279N mutation in exon 6 in two patients.

  20. Papel patogénico de la interleuquina-10 en el síndrome de Sjögren

    OpenAIRE

    Juan Manuel Anaya; Mónica Herrera; Paula Correa

    2001-01-01

    La interleuquina (IL)-10 es una citoquina con múltiples actividades
    sobre linfocitos B y T. Fisiológicamente es considerada como inmunosupresora; sin embargo, en patología este papel no ha sido confirmado en todos los casos (1). El síndrome de Sjögren primario (SSp) es una enfermedad autoinmune caracterizada por la hiperactividad de los linfocitos B y un infiltrado mononuclear en las glándulas exocrinas responsable de su hipofunción (2). En el presente estudio se investigó simultán...

  1. Marcadores del sexo en un conjunto osteológico del Formativo de Tilcara (sitio SJ Til. 20)

    OpenAIRE

    Barboza, María Carolina; Bordach, María A.; Mendonça, Osvaldo Juan

    2003-01-01

    El sitio SJ Til. 20 (Tilcara, Jujuy) corresponde al Período Formativo (AMS Calibrado de 2 Sigma, Prob. 95%, Cal AD 545 a 660; Cal BP 1405 -1290). Desde el punto de vista de los antecedentes, constituye un hallazgo funerario excepcional para el área por la forma en que se recuperaron y trataron los materiales óseos recuperados. El tratamiento de estos materiales proporcionó información relacionada con marcadores biológicos, tanto métricos como morfológicos, del sexo. El objetivo de este trabaj...

  2. Oral Nodular Lesions in Patients with Sjögren’s Syndrome: Unusual Oral Implications of a Systemic Disorder

    OpenAIRE

    Pinheiro, Juliana Barchelli; Tirapelli, Camila; Silva, Claudia Helena Lovato da; Komesu, Marilena Chinali; Petean, Flávio Calil; Louzada Junior, Paulo; León, Jorge Esquiche; Motta, Ana Carolina Fragoso

    2017-01-01

    Abstract Sjögren’s syndrome (SS) is a systemic chronic autoimmune disorder affecting the lacrimal and salivary glands. SS may manifest as primary SS (pSS) or secondary SS (sSS), the latter occurring in the context of another autoimmune disorder. In both cases, the dry eyes and mouth affect the patient’s quality of life. Late complications may include blindness, dental tissue destruction, oral candidiasis and lymphoma. This paper reports two cases of SS, each of them presenting unusual oral no...

  3. Olho seco e Sjögren secundário na artrite reumatóide

    OpenAIRE

    Gehlen, Marcelo Luiz; Skare, Thelma Larocca; Silva, Marilia Barreto; Antero, Daniel Casagrande; Miyazaki, Fernando; Parra, Andreo Garcia Morante

    2012-01-01

    OBJETIVO: Examinar a prevalência de ceratoconjuntivite seca e de Síndrome de Sjögren (SS) secundária em pacientes com AR do sul do Brasil e analisar suas relações com duração e atividade da AR e classe funcional do paciente. Pacientes com artrite reumatóide (AR) podem ter ceratoconjuntivite seca, que é considerada uma manifestação extra-articular dessa doença. MÉTODOS: Estudaram-se 82 pacientes com AR para sintomas de secura, Schirmer teste, DAS-28 ( índice de atividade da AR) e classe funcio...

  4. Is Hammarby Sjöstad a model case? Crime prevention through environmental design in Stockholm, Sweden

    DEFF Research Database (Denmark)

    Grønlund, Bo

    2012-01-01

    the European Committee for Standardization (CEN) guidelines to assess the case study of Hammarby Sjöstad – a residential area in Stockholm, the capital of Sweden. Urban planning and urban design principles are assessed from a CPTED point of view. Crime statistics as well as data on perceived safety......Crime Prevention Through Environmental Design (CPTED) claims to reduce crime and fear of crime through urban design and planning. If it does, it ought to be part of the sustainable planning and design of cities. This chapter gives an overview of the development of CPTED principles and then uses...

  5. [Clinical significance of cevimeline hydrochloride in the treatment of dry mouth in patients with Sjögren's syndrome].

    Science.gov (United States)

    Ogawa, Noriyoshi; Shimoyama, Kumiko; Karasawa, Hiromi; Fukushima, Toshihiro; Masaki, Yasufumi; Wano, Yuji; Hirose, Yuko; Sugai, Susumu

    2004-10-01

    To evaluate the efficacy and safety of cevimeline hydrochloride for the treatment of dry mouth in patients with Sjögren's syndrome (SS), eight SS patients received 30 mg of cevimeline twice or three times daily for 24 weeks. Six out of the eight patients had improvement in dry mouth. Five patients had more than 20% increase in saliva secretion. In the assessment of salivary gland scintigraphy, three patients showed improvement. There was a significant negative correlation between the improvement of saliva secretion and the severity of tissue damage assessed by MR sialography (r= - 0.754, pdry mouth in patients with SS, in particular, with less severe salivary gland destruction.

  6. Autoimmune myelofibrosis accompanied by Sjögren's syndrome in a 47, XXX/46, XX mosaic woman.

    Science.gov (United States)

    Takahashi, Tohru

    2014-01-01

    This report describes a patient with autoimmune myelofibrosis accompanied by Sjögren's syndrome (SS). A 36-year-old woman was admitted due to petechiae, purpura, gingival bleeding, dyspnea on exertion, and a lack of concentration. She had pancytopenia and was diagnosed with SS. A bone marrow study showed hypercellular marrow with reticulin fibrosis. Lymphocytic infiltrates and aggregates composed of a mixture of T and B cells in the marrow were also observed. A chromosomal analysis of the marrow cells showed 47, XXX and an analysis of peripheral lymphocytes revealed 47, XXX/46, XX mosaic results. The patient's cytopenia resolved following treatment with oral prednisolone.

  7. H1N1 vaccination in Sjögren's syndrome triggers polyclonal B cell activation and promotes autoantibody production.

    Science.gov (United States)

    Brauner, Susanna; Folkersen, Lasse; Kvarnström, Marika; Meisgen, Sabrina; Petersen, Sven; Franzén-Malmros, Michaela; Mofors, Johannes; Brokstad, Karl A; Klareskog, Lars; Jonsson, Roland; Westerberg, Lisa S; Trollmo, Christina; Malmström, Vivianne; Ambrosi, Aurelie; Kuchroo, Vijay K; Nordmark, Gunnel; Wahren-Herlenius, Marie

    2017-10-01

    Vaccination of patients with rheumatic disease has been reported to result in lower antibody titres than in healthy individuals. However, studies primarily include patients on immunosuppressive therapy. Here, we investigated the immune response of treatment-naïve patients diagnosed with primary Sjögren's syndrome (pSS) to an H1N1 influenza vaccine. Patients with Sjögren's syndrome without immunomodulatory treatment and age-matched and gender-matched healthy controls were immunised with an H1N1 influenza vaccine and monitored for serological and cellular immune responses. Clinical symptoms were monitored with a standardised form. IgG class switch and plasma cell differentiation were induced in vitro in purified naïve B cells of untreated and hydroxychloroquine-treated patients and healthy controls. Gene expression was assessed by NanoString technology. Surprisingly, treatment-naïve patients with Sjögren's syndrome developed higher H1N1 IgG titres of greater avidity than healthy controls on vaccination. Notably, off-target B cells were also triggered resulting in increased anti-EBV and autoantibody titres. Endosomal toll-like receptor activation of naïve B cells in vitro revealed a greater propensity of patient-derived cells to differentiate into plasmablasts and higher production of class switched IgG. The amplified plasma cell differentiation and class switch could be induced in cells from healthy donors by preincubation with type 1 interferon, but was abolished in hydroxychloroquine-treated patients and after in vitro exposure of naïve B cells to chloroquine. This comprehensive analysis of the immune response in autoimmune patients to exogenous stimulation identifies a mechanistic basis for the B cell hyperactivity in Sjögren's syndrome, and suggests that caution is warranted when considering vaccination in non-treated autoimmune patients. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights

  8. Dealing with emotions when the ability to cry is hampered: emotion processing and regulation in patients with primary Sjögren's syndrome.

    Science.gov (United States)

    van Leeuwen, Ninke; Bossema, Ercolie R; van Middendorp, Henriët; Kruize, Aike A; Bootsma, Hendrika; Bijlsma, Johannes W J; Geenen, Rinie

    2012-01-01

    The hampered ability to cry in patients with Sjögren's syndrome may affect their ways of dealing with emotions. The aim of this study was to examine differences in emotion processing and regulation between people with and without Sjögren's syndrome and correlations of emotion processing and regulation with mental well-being. In 300 patients with primary Sjögren's syndrome and 100 demographically matched control participants (mean age 56.8 years, 93% female), emotion processing (affect intensity and alexithymia, i.e. difficulty identifying and describing feelings), emotion regulation (cognitive reappraisal, suppression and expression of emotions), and mental well-being were assessed. Criteria for clinical alexithymia applied to 22% of the patients and 12% of the control participants; patients had significantly more difficulty identifying feelings than control participants. No other significant differences in emotion processing and emotion regulation were found. In patients, the emotion processing styles affect intensity and alexithymia (0.32emotion regulation strategy suppression of emotions (r=0.13) significantly correlated with worse mental well-being, which is about similar to control participants. Processing and regulating emotions in patients with Sjögren's syndrome does not deviate from normal with one exception: a relatively large number of patients is alexithymic. As in the general population, in patients with Sjögren's syndrome the more intense and deficient processing and regulation of emotions is associated with worse mental well-being. This study indicates that, except for selected patients, processing and regulation of emotions is not a key therapeutic issue for the majority of patients with Sjögren's syndrome.

  9. MALT Lymphoma of Minor Salivary Glands in a Sjögren’s Syndrome Patient: a Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Savvas Titsinides

    2017-03-01

    Full Text Available Objectives: Sjögren’s syndrome is a chronic systemic disease, characterized by lymphocytic infiltration and destruction mainly of the salivary and lacrimal glands, resulting in xerostomia and xeropthalmia. Sjögren’s syndrome patients have a 44-fold excess risk for the development of non-Hodgkin’s lymphoma particularly mucosa-associated lymphoid tissue (MALT lymphoma, prevalently affecting the major salivary glands. In this report, a rare case of MALT lymphoma of minor salivary glands in a patient with Sjögren’s syndrome is described. A review of the published cases of MALT lymphoma located in the minor salivary glands of patients with Sjögren’s syndrome is provided. Material and Methods: In a 64-year-old female patient previously diagnosed with Sjögren’s syndrome, an asymptomatic soft tissue mass at the palate was noticed, exhibiting rapid enlargement within one month. With a main differential diagnosis of salivary gland neoplasm or lymphoproliferative lesion, a partial biopsy was performed accompanied by proper immunohistochemical analysis. Results: A final diagnosis of MALT lymphoma was rendered and the patient was referred for further multidisciplinary evaluation. Gastric endoscopy and biopsy revealed a Helicobacter pylori-negative gastric MALT lymphoma, while spleen involvement and bone marrow infiltration were also identified. Patient was classified as having stage IV disseminated disease and a standard chemotherapy protocol was administered; the treatment was well tolerated and resulted in complete remission. Conclusions: This case emphasizes the need for close monitoring of patients with Sjögren’s syndrome by oral medicine specialists, which, besides ensuring proper management of xerostomia and its sequelae, may also lead to early recognition of lymphoma development.

  10. Offshore vindenergianlegg - sjøfugl, havørn, hubro og vadere. En screening av potensielle konfliktområde

    OpenAIRE

    Christensen-Dalsgaard, Signe; Lorentsen, Svein-Håkon; Dahl, Espen Lie; Follestad, Arne; Hanssen, Frank Ole; Systad, Geir Helge

    2010-01-01

    Christensen-Dalsgaard, S., Lorentsen, S.-H., Dahl, E. L., Follestad, A., Hanssen, F. & Systad, G. H. 2010. Offshore vindenergianlegg - sjøfugl, havørn, hubro og vadere. En screening av potensielle konfliktområder - NINA Rapport 557. 100 s. Målet for foreliggende studie var å utarbeide en beslutningsrelevant, storskala screening av områder som kan være aktuelle for etablering av offshore vindenergianlegg med lavest mulig konfliktrisiko i forhold til forekomster av sjøfugl, vader...

  11. Persistence and selection of an expanded B-cell clone in the setting of rituximab therapy for Sjögren’s syndrome

    Science.gov (United States)

    2014-01-01

    Introduction Subjects with primary Sjögren’s syndrome (SjS) have an increased risk of developing B-cell lymphoma and may harbor monoclonal B-cell expansions in the peripheral blood. Expanded B-cell clones could be pathogenic, and their persistence could exacerbate disease or predispose toward the development of lymphoma. Therapy with anti-CD20 (rituximab) has the potential to eliminate expanded B-cell clones and thereby potentially ameliorate disease. This study was undertaken to identify and track expanded B-cell clones in the blood of subjects with primary SjS who were treated with rituximab. Methods To determine whether circulating B-cell clones in subjects with primary SjS emerge or remain after B cell-depleting therapy with rituximab, we studied the antibody heavy-chain repertoire. We performed single-memory B-cell and plasmablast sorting and antibody heavy-chain sequencing in six rituximab-treated SjS subjects over the course of a 1-year follow-up period. Results Expanded B-cell clones were identified in four out of the six rituximab-treated SjS subjects, based upon the independent amplification of sequences with identical or highly similar VH, DH, and JH gene segments. We identified one SjS subject with a large expanded B-cell clone that was present prior to therapy and persisted after therapy. Somatic mutations in the clone were numerous but did not increase in frequency over the course of the 1-year follow-up, suggesting that the clone had been present for a long period of time. Intriguingly, a majority of the somatic mutations in the clone were silent, suggesting that the clone was under chronic negative selection. Conclusions For some subjects with primary SjS, these data show that (a) expanded B-cell clones are readily identified in the peripheral blood, (b) some clones are not eliminated by rituximab, and (c) persistent clones may be under chronic negative selection or may not be antigen-driven. The analysis of sequence variation among members of an

  12. Persistence and selection of an expanded B-cell clone in the setting of rituximab therapy for Sjögren's syndrome.

    Science.gov (United States)

    Hershberg, Uri; Meng, Wenzhao; Zhang, Bochao; Haff, Nancy; St Clair, E William; Cohen, Philip L; McNair, Patrice D; Li, Ling; Levesque, Marc C; Luning Prak, Eline T

    2014-02-11

    Subjects with primary Sjögren's syndrome (SjS) have an increased risk of developing B-cell lymphoma and may harbor monoclonal B-cell expansions in the peripheral blood. Expanded B-cell clones could be pathogenic, and their persistence could exacerbate disease or predispose toward the development of lymphoma. Therapy with anti-CD20 (rituximab) has the potential to eliminate expanded B-cell clones and thereby potentially ameliorate disease. This study was undertaken to identify and track expanded B-cell clones in the blood of subjects with primary SjS who were treated with rituximab. To determine whether circulating B-cell clones in subjects with primary SjS emerge or remain after B cell-depleting therapy with rituximab, we studied the antibody heavy-chain repertoire. We performed single-memory B-cell and plasmablast sorting and antibody heavy-chain sequencing in six rituximab-treated SjS subjects over the course of a 1-year follow-up period. Expanded B-cell clones were identified in four out of the six rituximab-treated SjS subjects, based upon the independent amplification of sequences with identical or highly similar VH, DH, and JH gene segments. We identified one SjS subject with a large expanded B-cell clone that was present prior to therapy and persisted after therapy. Somatic mutations in the clone were numerous but did not increase in frequency over the course of the 1-year follow-up, suggesting that the clone had been present for a long period of time. Intriguingly, a majority of the somatic mutations in the clone were silent, suggesting that the clone was under chronic negative selection. For some subjects with primary SjS, these data show that (a) expanded B-cell clones are readily identified in the peripheral blood, (b) some clones are not eliminated by rituximab, and (c) persistent clones may be under chronic negative selection or may not be antigen-driven. The analysis of sequence variation among members of an expanded clone may provide a novel means

  13. Sexuellt riskbeteende och självkänsla hos ungdomar

    OpenAIRE

    Unis, Brian

    2010-01-01

    Sammanfattning     Författare (Tillnamn, förnamn)                                                                                                                 Årtal                     Unis, Brian                                                                                                       2010              Arbetets titel   Sexuellt riskbeteende och självkänsla hos ungdomar Opublicerad uppsats för magisterexamen.                                                                      ...

  14. A new MHC-linked susceptibility locus for primary Sjögren's syndrome: MICA.

    Science.gov (United States)

    Carapito, Raphael; Gottenberg, Jacques-Eric; Kotova, Irina; Untrau, Meiggie; Michel, Sandra; Naegely, Lydie; Aouadi, Ismail; Kwemou, Marius; Paul, Nicodème; Pichot, Angélique; Locke, James; Bowman, Simon J; Griffiths, Bridget; Sivils, Kathy L; Sibilia, Jean; Inoko, Hidetoshi; Micelli-Richard, Corinne; Nocturne, Gaétane; Ota, Masao; Ng, Wan-Fai; Mariette, Xavier; Bahram, Seiamak

    2017-07-01

    The association of primary Sjögren's syndrome (pSS) with Major Histocompatibility Complex (MHC) alleles is quintessential of MHC-disease associations. Indeed, although disease associations with classical HLA class I and II alleles/haplotypes are amply documented, further dissection is often prevented by the strong linkage disequilibrium across the entire MHC complex. Here we study the association of pSS, not with HLA genes, but with the non-conventional MHC encoded class I gene, MICA (MHC class I chain-related gene A). MICA is selectively expressed within epithelia, and is the major ligand for the activatory receptor, NKG2D, both attributes relevant to pSS' etiology. MICA-pSS association was studied in two independent (French and UK) cohorts representing a total of 959 cases and 1,043 controls. MICA*008 allele was shown to be significantly associated with pSS (pcor=2.61 × 10-35). A multivariate logistic regression showed that this association was independent of all major known MHC-linked risk loci/alleles, as well as other relevant candidate loci that are in linkage disequilibrium with MICA*008 i.e. HLA-B*08:01, rs3131619 (T), MICB*008, TNF308A, HLA-DRB1*03:01 and HLA-DRB1*15:01 (P = 1.84 × 10-04). Furthermore, independently of the MICA*008 allele, higher levels of soluble MICA proteins were detected in sera of pSS patients compared to healthy controls. This study hence defines MICA as a new, MHC-linked, yet HLA-independent, pSS risk locus and opens a new front in our understanding of the still enigmatic pathophysiology of this disease. The fact that the soluble MICA protein is further amplified in MICA*008 carrying individuals, might also be relevant in other auto-immune diseases and cancer. © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  15. A BAFF receptor His159Tyr mutation in Sjögren's syndrome-related lymphoproliferation.

    Science.gov (United States)

    Papageorgiou, Aristea; Mavragani, Clio P; Nezos, Andrianos; Zintzaras, Elias; Quartuccio, Luca; De Vita, Salvatore; Koutsilieris, Michael; Tzioufas, Athanasios G; Moutsopoulos, Haralampos M; Voulgarelis, Michael

    2015-10-01

    To study the prevalence, clinical associations, and functional implications of the His159Tyr mutation of the BAFF receptor (BAFF-R) in patients with Sjögren's syndrome (SS). The BAFF-R His159Tyr mutation was evaluated using polymerase chain reaction (PCR)-based assays in 247 patients with SS (of whom 70 had SS complicated by lymphoma [SS-lymphoma]), 145 with systemic lupus erythematosus (SLE), and 101 with rheumatoid arthritis (RA), as well as 180 healthy controls. Real-time PCR and Western blotting were performed for the quantification of both NF-κB1 and NF-κB2 messenger RNA (mRNA) transcript and protein levels in isolated B cells from patients with SS-lymphoma carrying the mutation (SS-lymphoma-BAFF-RHis159Tyr -derived B cells) compared to B cells from patients with SS-lymphoma who were not carriers of the mutation and healthy controls. Both the SS-lymphoma and SS-nonlymphoma patient subgroups exhibited significantly higher frequencies of the His159Tyr BAFF-R mutation compared to healthy controls (8.6% of SS-lymphoma patients and 6.2% of SS-nonlymphoma patients versus 1.7% of healthy controls; P = 0.02 and P = 0.04, respectively). The corresponding frequencies of the His159Tyr BAFF-R mutation in SLE and RA patients were 3.5% and 3%, respectively. Of interest, 71.4% of the SS patients with mucosa-associated lymphoid tissue (MALT) lymphoma who were between the ages of 31 and 40 years at disease onset were mutation carriers. The generalized odds ratio for the development of SS-related MALT lymphoma in the younger age at onset (age BAFF-R mutation was 6.1 (95% confidence interval 2.0-18.7) (P BAFF-RHis159Tyr -derived B cells. This study identifies an increased prevalence of the BAFF-R His159Tyr mutation in patients with SS, particularly in those with SS complicated by MALT lymphoma whose disease onset occurred at a younger age. BAFF-RHis159Tyr -mediated activation of the alternate NF-κB pathway might contribute to the pathogenesis of SS

  16. Lupus anticoagulant: a marker for stroke and venous thrombosis in primary Sjögren's syndrome.

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    Pasoto, Sandra Gofinet; Chakkour, Henrique Pires; Natalino, Renato Romera; Viana, Vilma S T; Bueno, Cleonice; Lianza, Alessandro Cavalcanti; de Andrade, José Lázaro; Neto, Mauricio Levy; Fuller, Ricardo; Bonfa, Eloisa

    2012-09-01

    Antiphospholipid antibodies (aPL) and antiphospholipid syndrome (APS) have been described in primary Sjögren's syndrome (pSS) with controversial findings regarding aPL prevalence and their association with thrombotic events. We evaluated 100 consecutive pSS patients (American-European criteria) and 89 age-gender-ethnicity-matched healthy controls for IgG/IgM anticardiolipin (aCL), IgG/IgM anti-beta2-glycoprotein-I (aβ2GPI), and lupus anticoagulant (LA) (positivity according to APS Sydney's criteria). Clinical analysis followed standardized interview and physical examination assessing thrombotic and nonthrombotic APS manifestations and thrombosis risk factors. aPLs were detected in 16 % patients and 5.6 % controls (p = 0.035). LA was the most common aPL in patients (9 %), followed by aβ2GPI (5 %) and aCL (4 %). Thrombotic events occurred in five patients [stroke in two, myocardial infarction in one and deep-vein thrombosis (DVT) in four], but in none of controls (p = 0.061). Mean age at time of stroke was 35 years. Three patients with thrombotic events (including the two with stroke) had APS (Sydney's criteria) and were positive exclusively for LA. Comparison of patients with (n = 16) and without (n = 84) aPL revealed similar mean age, female predominance, and ethnicity (p > =0.387). Frequencies of livedo reticularis (25 vs. 4.8 %, p = 0.021), stroke (12.5 vs. 0 %, p = 0.024), and DVT (18.8 vs. 1.2 %, p = 0.013) were significantly higher in APL + patients. Conversely, frequencies of hypertension, dyslipidemia, diabetes, obesity, smoking, sedentarism, and hormonal contraception were similar in patients with or without aPL (p ≥ 0.253). Our study identified LA as an important marker for APS in pSS, particularly for stroke in young patients, warranting routine evaluation of these antibodies and rigorous intervention in modifiable risk factors.

  17. Joint Involvement in Primary Sjögren’s Syndrome: An Ultrasound “Target Area Approach to Arthritis”

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    Luis M. Amezcua-Guerra

    2013-01-01

    Full Text Available Objective. To characterize the ultrasound (US pattern of joint involvement in primary Sjögren’s syndrome (pSS. Methods. Seventeen patients with pSS, 18 with secondary Sjögren’s syndrome (sSS, and 17 healthy controls underwent US examinations of various articular regions. Synovitis (synovial hypertrophy/joint effusion, power Doppler (PD signals, and erosions were assessed. Results. In patients with pSS, synovitis was found in the metacarpophalangeal joints (MCP, 76%, wrists (76%, and knees (76%, while the proximal interphalangeal joints, elbows, and ankles were mostly unscathed. Intra-articular PD signals were occasionally detected in wrists (12%, elbows (6%, and knees (6%. Erosions were evident in the wrists of three (18% patients with pSS, one of these also having anti-cyclic citrullinated peptide (anti-CCP antibodies. While US synovitis does not discriminate between sSS and pSS, demonstration of bone erosions in the 2nd MCP joints showed 28.8% sensitivity and 100% specificity for diagnosing sSS; in comparison, these figures were 72.2 and 94.1% for circulating anti-CCP antibodies. Conclusions. In pSS, the pattern of joint involvement by US is polyarticular, bilateral, and symmetrical. Synovitis is the US sign most commonly found in patients with pSS, especially in MCP joints, wrists, and knees, and bone erosions also may occur.

  18. Validity of the saliva ferning test for the diagnosis of dry mouth in Sjögren's syndrome.

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    el-Miedany, Y M; el-Hady, S M; el-Baddin, M A

    1999-02-01

    To study the validity of saliva ferning patterns as a diagnostic test for dry mouth in primary or secondary Sjogren's Syndrome (SS). Salivary smears were collected from 25 patients with Sjögren's syndrome in the fasting and nonfasting state. All 25 patients had symptomatic xerostomia and xerophthalmia and a positive Shirmer's test. Smears were taken from four sites, the cheek, lower lip, tongue, and saliva. Tests were done for rheumatoid factor, antinuclear antibodies, and anti-Ro(SS-A) antibodies. The salivary smears were air-dried and examined under a light and a polarizing microscope. Smears from 25 healthy subjects were also examined as controls. Three patterns of salivary secretion were identified, namely normal geometric ferning, reindeer antler ferning, and thick branching ferning. All Sjögren's syndrome patients had abnormal salivary smears, usually with a combination of reindeer antler ferning, thick branching ferning, and mucosal squames. This combination was seen in six of the 25 fasting specimens (24%); most of the remaining fasting samples showed the reindeer antler ferning. Reindeer antler ferning alone was found in five fasting and four nonfasting samples: this pattern was absent from five fasting and five nonfasting samples in which mucosal squames were the only abnormal finding. All nonfasting control samples exhibited normal geometric ferning. Smears from the cheek and saliva provided the most illustrative findings. The saliva ferning test is a simple, reproducible, and useful diagnostic aid in autoimmune xerostomia, approximately equivalent to Shirmer's test in xerophthalmia.

  19. Increase in acrolein-conjugated immunoglobulins in saliva from patients with primary Sjögren's syndrome.

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    Hirose, Tadao; Saiki, Ryotaro; Uemura, Takeshi; Suzuki, Takehiro; Dohmae, Naoshi; Ito, Satoshi; Takahashi, Hoyu; Ishii, Itsuko; Toida, Toshihiko; Kashiwagi, Keiko; Igarashi, Kazuei

    2015-10-23

    We previously reported that the level of protein-conjugated acrolein (PC-Acro), a marker of cell or tissue damage, was increased in saliva from patients with primary Sjögren's syndrome (pSS), and that the level of PC-Acro was well correlated with the severity of pSS. Acrolein-conjugated immunoglobulins were measured in saliva from pSS patients. The activities of autoantibodies recognizing Sjögren's syndrome SSA (Ro) and SSB (La) proteins in saliva from pSS patients were approximately 3- to 5-fold higher than those from control subjects. We also found that autoantibody activities recognizing SSA (Ro) and SSB (La) proteins increased after acrolein treatment of saliva from control subjects. When an antibody against human serum albumin was treated with acrolein, the ability to recognize albumin was reduced but the ability to recognize other proteins was increased. Twenty-four and eleven kinds of acrolein-conjugated amino acids were found at the variable and constant regions of peptides, respectively, obtained from the immunoglobulins in saliva from pSS patients. The altered recognition patterns of immunoglobulins due to acrolein conjugation are at least partially involved in autoimmune diseases. Copyright © 2015 Elsevier B.V. All rights reserved.

  20. Valoración médico-laboral del trabajador con Síndrome de Sjögren

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    Maider Usarbarrena Ekiza

    2014-03-01

    Full Text Available El Síndrome de Sjögren (SS es una patología inflamatoria crónica, infradiagnosticada y de etiología desconocida, que se caracteriza por la infiltración linfocitaria de las glándulas exocrinas. La sintomatología puede ser muy variada. Inicialmente, lo más común es que se presente sequedad de mucosas (principalmente oculares y bucales; también se puede asociar a otras enfermedades autoinmunes, y hasta en el 65% de los casos, se pueden presentar manifestaciones extraglandulares, pudiéndose afectar cualquier parte del organismo. En muchos casos, la sintomatología inicial es leve, achacándose habitualmente a un posible disconfort en el puesto de trabajo. A esto hay que añadir que a nivel laboral no existe literatura específica en la que poder basarse para realizar una adecuada valoración médico-laboral. El objetivo de este trabajo ha sido crear una herramienta que englobe, por una parte, la vigilancia de la salud específica, y por otra, definir (aunque sea de forma orientativa, los criterios para poder realizar una correcta valoración de la capacidad laboral en los trabajadores con Síndrome de Sjögren.

  1. Prevalence of interferon type I signature in CD14 monocytes of patients with Sjögren's syndrome and association with disease activity and BAFF gene expression

    NARCIS (Netherlands)

    Z. Brkic (Zana); M. de Maria; C.G. van Helden-Meeuwsen; J.P. van de Merwe (Joop); P.L.A. van Daele (Paul); V.A.S.H. Dalm (Virgil); M.E. Wildenberg; W. Beumer (Wouter); H.A. Drexhage (Hemmo); M.A. Versnel (Marjan)

    2013-01-01

    textabstractObjective To determine the prevalence of upregulation of interferon (IFN) type I inducible genes, the so called "IFN type I signature", in CD14 monocytes in 69 patients with primary Sjögren's syndrome (pSS) and 44 healthy controls (HC) and correlate it with disease manifestations and

  2. Complete genome sequence of the caprolactam-degrading bacterium Pseudomonas mosselii SJ10 isolated from wastewater of a nylon 6 production plant.

    Science.gov (United States)

    Park, Gun-Seok; Chu, Ji-Hun; Hong, Sung-Jun; Kwak, Yunyoung; Khan, Abdur Rahim; Jung, Byung Kwon; Ullah, Ihsan; Shin, Jae-Ho

    2014-12-20

    Pseudomonas mosselii strain SJ10 is a caprolactam-degrading bacterium belonging to the class Gammaproteobacteria, which was isolated from wastewater of the nylon 6 producing Seongseo industrial complex in Daegu, Republic of Korea. Here, we report the complete genome sequence of the strain, providing genetic information for biodegradation of aromatic compounds.

  3. Successful Treatment of Dry Mouth and Dry Eye Symptoms in Sjögren's Syndrome Patients With Oral Pilocarpine: A Randomized, Placebo-Controlled, Dose-Adjustment Study.

    Science.gov (United States)

    Papas, Athena S; Sherrer, Yvonne S; Charney, Michael; Golden, Harvey E; Medsger, Thomas A; Walsh, Bridget T; Trivedi, Madhu; Goldlust, Barry; Gallagher, Susan C

    2004-08-01

    : Sjögren's syndrome is characterized by the presence of xerostomia and/or xerophthalmia. Pilocarpine, a muscarinic cholinergic agonist, has been proven to be efficacious in treating radiation-induced xerostomia (up to 30 mg/day) and symptoms of dry mouth in Sjögren's patients (up to 20 mg/day). : To compare the safety and efficacy of oral pilocarpine (dose-adjusted) versus placebo in the treatment of dry eye and dry mouth symptoms in Sjögren's syndrome at 6 and 12 weeks. : In this 11-center, 256-patient placebo-controlled study, the safety and efficacy of oral pilocarpine (20 mg to 30 mg daily) for relief of Sjögren's-related dry mouth and dry eye symptoms was assessed. Changes in symptoms and salivary flow were measured over 12 weeks. : Compared with placebo, salivary flow was significantly increased in the pilocarpine group (Pdry mouth (Pdry eyes (Pdry mouth symptoms was noted at 20 mg/day, and significant relief in ocular symptoms, including lower artificial tear requirement, was noted after the dose was increased to 30 mg/day.

  4. Distrofia macular cristalina em paciente com síndrome de Sjögren-Larsson: relato de caso Macular crystalline dystrophy in Sjögren-Larsson syndrome: case report

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    David Leonardo Cruvinel Isaac

    2009-04-01

    Full Text Available Apresentação de um caso de distrofia macular cristalina em paciente do sexo feminino com síndrome de Sjögren-Larsson. A doença caracteriza-se pela tríade: ictiose congênita, diplegia ou tetraplegia espástica e retardo mental. Os olhos são acometidos em até 100% dos casos sendo característica a presença de maculopatia cristalina como observado na paciente relatada. À fundoscopia observou-se a presença de depósitos esbranquiçados perifoveais. A tomografia de coerência óptica evidenciou pontos hiper-reflexivos correspondentes aos depósitos intrarretinianos, atrofia macular com redução na espessura macular em ambos os olhos. Os achados relacionados à síndrome, à tomografia de coerência óptica foram apresentados de maneira inédita em nosso meio.Presentation of a case of crystalline macular distrophy diagnosed in a female patient with Sjögren-Larsson syndrome. The disease consists of clinical findings of spastic diplegia or tetraplegia, mental retardation, and congential ichthyosis. The eyes are affected in up to 100% of cases, and crystalline maculopathy is the main finding as described in this case report. On fundus examination multiple white dots were observed at ophthalmoscopy. The optical coherence tomography has shown not only the hipereflexive intraretinal spots but also macular atrophy with macular thickness reduction. The tomographic findings were first described in our country.

  5. Papel patogénico de la interleuquina-10 en el síndrome de Sjögren

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    Juan Manuel Anaya

    2001-04-01

    Full Text Available La interleuquina (IL-10 es una citoquina con múltiples actividades
    sobre linfocitos B y T. Fisiológicamente es considerada como inmunosupresora; sin embargo, en patología este papel no ha sido confirmado en todos los casos (1. El síndrome de Sjögren primario (SSp es una enfermedad autoinmune caracterizada por la hiperactividad de los linfocitos B y un infiltrado mononuclear en las glándulas exocrinas responsable de su hipofunción (2. En el presente estudio se investigó simultáneamente el nivel sérico de la IL-10 en pacientes con SSp y su correlación con los títulos de autoanticuerpos, manifestaciones clínicas, actividad de la enfermedad y polimorfismo del gen de la IL-10.

  6. On the mechanism of action of SJ-172550 in inhibiting the interaction of MDM4 and p53.

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    Michal Bista

    Full Text Available SJ-172550 (1 was previously discovered in a biochemical high throughput screen for inhibitors of the interaction of MDMX and p53 and characterized as a reversible inhibitor (J. Biol. Chem. 2010; 285:10786. Further study of the biochemical mode of action of 1 has shown that it acts through a complicated mechanism in which the compound forms a covalent but reversible complex with MDMX and locks MDMX into a conformation that is unable to bind p53. The relative stability of this complex is influenced by many factors including the reducing potential of the media, the presence of aggregates, and other factors that influence the conformational stability of the protein. This complex mechanism of action hinders the further development of compound 1 as a selective MDMX inhibitor.

  7. Oral Nodular Lesions in Patients with Sjögren's Syndrome: Unusual Oral Implications of a Systemic Disorder.

    Science.gov (United States)

    Pinheiro, Juliana Barchelli; Tirapelli, Camila; Silva, Claudia Helena Lovato da; Komesu, Marilena Chinali; Petean, Flávio Calil; Louzada Junior, Paulo; León, Jorge Esquiche; Motta, Ana Carolina Fragoso

    2017-01-01

    Sjögren's syndrome (SS) is a systemic chronic autoimmune disorder affecting the lacrimal and salivary glands. SS may manifest as primary SS (pSS) or secondary SS (sSS), the latter occurring in the context of another autoimmune disorder. In both cases, the dry eyes and mouth affect the patient's quality of life. Late complications may include blindness, dental tissue destruction, oral candidiasis and lymphoma. This paper reports two cases of SS, each of them presenting unusual oral nodular lesion diagnosed as relapsed MALT lymphoma and mucocele. The importance of the diagnosis, treatment and management of the oral lesions by a dentist during the care of SS patients is emphasized, as the oral manifestations of SS may compromise the patient's quality of life.

  8. Hypokalemic paralysis as a presenting manifestation of primary Sjögren′s syndrome: A report of two cases

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    Deepak Khandelwal

    2012-01-01

    Full Text Available Primary Sjögren′s syndrome (pSS is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement. Overt or latent renal tubular acidosis (RTA, caused by tubulointerstitial nephropathy, is a common extraglandular manifestation of pSS. Hypokalemic paralysis is a well known, albeit rare complication of severe distal RTA from any cause. Cases of pSS manifesting for the first time as hypokalemic paralysis caused by distal RTA have been rarely reported. We herein present our experience of two cases, who presented to us for evaluation of hypokalemic paralysis and on work up found evidence of distal RTA, which on further work up found to be secondary to pSS. A high index of suspicion for pSS should be kept in all patients with hypokalemic paralysis.

  9. Laser as a therapy for dry mouth symptoms in a patient with Sjögren's syndrome: a case report.

    Science.gov (United States)

    Simões, Alyne; Platero, Maira Duque; Campos, Luana; Aranha, Ana Cecília; Eduardo, Carlos de Paula; Nicolau, José

    2009-01-01

    This clinical case study reports on dry mouth symptoms in a patient with Sjögren's syndrome (SS) who was treated with laser phototherapy (LPT). A 60-year-old woman diagnosed with SS was referred to the laboratory for lasers in dentistry to treat her severe xerostomia. A diode laser (780 nm, 3.8 J/cm2, 15 mW) was used to irradiate the parotid, submandibular, and sublingual glands, three times per week, for a period of 8 months. The salivary flow rate and xerostomia symptoms were measured before, during, and after LPT. Dry mouth symptoms improved during LPT. After LPT, the parotid salivary gland pain and swelling were no longer present. Treatment with LPT was an effective method to improve the quality of life of this patient with SS.

  10. Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome.

    Science.gov (United States)

    Collado, Paz; Kelada, Aml; Cámara, Maria; Zeft, Andrew; Flagg, Aron

    2017-03-08

    Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined. We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Fine needle aspiration of the salivary glands (SG) showed nonspecific findings that led to delayed diagnosis of SS. The diagnosis of B-cell lymphoma associated with JSS was based on morphologic and immunohistochemical staining done during the biopsy. To highlight awareness of EMZL as a timely and appropriate update of an unusual complication in children with SS. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  11. Genome-Wide Association Analysis Reveals Genetic Heterogeneity of Sjögren's Syndrome According to Ancestry.

    Science.gov (United States)

    Taylor, Kimberly E; Wong, Quenna; Levine, David M; McHugh, Caitlin; Laurie, Cathy; Doheny, Kimberly; Lam, Mi Y; Baer, Alan N; Challacombe, Stephen; Lanfranchi, Hector; Schiødt, Morten; Srinivasan, M; Umehara, Hisanori; Vivino, Frederick B; Zhao, Yan; Shiboski, Stephen C; Daniels, Troy E; Greenspan, John S; Shiboski, Caroline H; Criswell, Lindsey A

    2017-06-01

    The Sjögren's International Collaborative Clinical Alliance (SICCA) is an international data registry and biorepository derived from a multisite observational study of participants in whom genotyping was performed on the Omni2.5M platform and who had undergone deep phenotyping using common protocol-directed methods. The aim of this study was to examine the genetic etiology of Sjögren's syndrome (SS) across ancestry and disease subsets. We performed genome-wide association study analyses using SICCA subjects and external controls obtained from dbGaP data sets, one using all participants (1,405 cases, 1,622 SICCA controls, and 3,125 external controls), one using European participants (585, 966, and 580, respectively), and one using Asian participants (460, 224, and 901, respectively) with ancestry adjustments via principal components analyses. We also investigated whether subphenotype distributions differ by ethnicity, and whether this contributes to the heterogeneity of genetic associations. We observed significant associations in established regions of the major histocompatibility complex (MHC), IRF5, and STAT4 (P = 3 × 10 -42 , P = 3 × 10 -14 , and P = 9 × 10 -10 , respectively), and several novel suggestive regions (those with 2 or more associations at P ancestry (P = 4 × 10 -15 and P = 4 × 10 -5 , respectively), but that subphenotype differences did not explain most of the ancestry differences in genetic associations. Genetic associations with SS differ markedly according to ancestry; however, this is not explained by differences in subphenotypes. © 2017, The Authors. Arthritis & Rheumatology published by Wiley Periodicals, Inc. on behalf of American College of Rheumatology.

  12. Abdominal ultrasonogram of autoimmune pancreatitis: Five cases of pancreatic lesions accompanied by Sjögren syndrome.

    Science.gov (United States)

    Yoshizaki, Hideo; Takeuchi, Kazuo; Okuda, Chikao; Honjyo, Hajime; Yamamoto, Takatugu; Kora, Tetuo; Takamori, Yoriyuki

    2002-09-01

    The concept of autoimmune pancreatitis has recently been established, and ultrasonographic findings we obtained from five cases consistent with autoimmune pancreatitis are reported here. Case 1, a 77-year-old man, was admitted complaining of loss of body weight. Serum hepatobiliary enzymes and γ-globulin levels were elevated, and antinuclear antibody was positive, Abdominal ultrasonography showed dilatation of the intrahepatic bile duct, wall thickening of the common bile duct and hypoechoic swelling of the pancreatic head and body. ERCP revealed multiple stenosis of the intra-and extra-hepatic bile ducts, and diffuse irregular narrowing of the main pancreatic duct. The patient complained of thirst, and the minor salivary gland was examined histologically. Our diagnosis was Sjögren syndrome accompanied by sclerosing cholangitis and a pancreatic lesion. Obstructive jaundice also developed, and PTCD was therefore performed. Both the pancreatic swelling and multiple stenosis of the bile duct improved after steroids were administered. Case 2, a 71-year-old man, was admitted with jaundice. Abdominal ultrasonography showed hypoechoic swelling of the pancreas. ERCP showed stenosis of the common bile duct in the pancreatic head region and diffuse irregular narrowing of the main pancreatic duct. Histological examination of the minor salivary gland suggested Sjögren syndrome. Steroids were therefore administered because the presence of both hyper-γ-globulinemia and positive antinuclear antibody suggested involvement of the autoimmune mechanism. Steroid therapy improved the jaundice as well as the findings from the cholangiograms and pancreatograms. We also encountered three similar cases, all consistent with the concept of autoimmune pancreatitis. The ultrasonographic findings of the pancreatic lesion (1) showed them as homogeneous and markedly hypoechoic areas and, (2) visualized the main pancreatic duct in the lesion, which facilitated a differential diagnosis of the

  13. Characterization of the serological biomarkers associated with Sjögren’s syndrome in patients with recalcitrant dry eye disease

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    Matossian C

    2016-07-01

    Full Text Available Cynthia Matossian,1,2 Joan Micucci1 1Matossian Eye Associates, Doylestown, PA, USA; 2Department of Ophthalmology, Temple University School of Medicine, Philadelphia, PA, USA Purpose: The purpose was to characterize the biomarkers associated with Sjögren’s syndrome (SS identified in the serological samples of patients with recalcitrant dry eye disease; additionally, the modalities utilized in the treatment of dry eye disease were evaluated for subsets of patients with and without SS. Patients and methods: Data for this retrospective, single-center, pilot study were based on a chart review of 48 sequential patients with recalcitrant dry eye who were evaluated for SS via serological analysis. Data presented include the presence of the autoantibodies identified through the serological biomarker analysis and identification of the concurrent dry eye treatment modalities. Results: Eleven out of 48 patients (23% tested positive for biomarkers associated with SS. Autoantibodies for salivary protein-1, parotid secretory protein 1, and carbonic anhydrase VI, markers associated with the early development of SS, were detected in 91% (ten out of eleven of the patients who tested positive for SS, whereas 27% (three out of eleven of patients tested positive for the traditional SS markers, SS-A and/or SS-B. Common treatment modalities utilized in SS patients included omega-3 supplements (82%, topical cyclosporine (74%, and artificial tear solutions (64%, as compared to omega-3 supplements (80%, hot-mask therapy (77%, and artificial tear solutions (77%, in SS-negative patients. Conclusion: Evaluation for salivary protein-1, parotid secretory protein 1, and carbonic anhydrase VI biomarkers allows for identification of a subset of patients with biomarkers associated with SS that may not be identified through the traditional assessments (SS-A/SS-B. Earlier recognition of SS biomarkers allows for a confirmatory diagnosis and appropriate management of this

  14. Semi-quantitative analysis of salivary gland scintigraphy in Sjögren's syndrome diagnosis: a first-line tool.

    Science.gov (United States)

    Angusti, Tiziana; Pilati, Emanuela; Parente, Antonella; Carignola, Renato; Manfredi, Matteo; Cauda, Simona; Pizzigati, Elena; Dubreuil, Julien; Giammarile, Francesco; Podio, Valerio; Skanjeti, Andrea

    2017-09-01

    The aim of this study was the assessment of semi-quantified salivary gland dynamic scintigraphy (SGdS) parameters independently and in an integrated way in order to predict primary Sjögren's syndrome (pSS). Forty-six consecutive patients (41 females; age 61 ± 11 years) with sicca syndrome were studied by SGdS after injection of 200 MBq of pertechnetate. In sixteen patients, pSS was diagnosed, according to American-European Consensus Group criteria (AECGc). Semi-quantitative parameters (uptake (UP) and excretion fraction (EF)) were obtained for each gland. ROC curves were used to determine the best cut-off value. The area under the curve (AUC) was used to estimate the accuracy of each semi-quantitative analysis. To assess the correlation between scintigraphic results and disease severity, semi-quantitative parameters were plotted versus Sjögren's syndrome disease activity index (ESSDAI). A nomogram was built to perform an integrated evaluation of all the scintigraphic semi-quantitative data. Both UP and EF of salivary glands were significantly lower in pSS patients compared to those in non-pSS (p quantitative parameters and ESSDAI. The proposed nomogram accuracy was 87%. SGdS is an accurate and reproducible tool for the diagnosis of pSS. ESSDAI was not shown to be correlated with SGdS data. SGdS should be the first-line imaging technique in patients with suspected pSS.

  15. Minor Salivary Gland Biopsy To Detect Primary Sjögren Syndrome in Patients With Interstitial Lung Disease

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    Jeffrey J. Swigris; du Bois, Roland M.; Groshong, Steve D.; Cool, Carlyne D.; Sahin, Hakan; Lynch, David A.; Gillis, JoAnn Z.; Cohen, Marc D.; Meehan, Richard T.; Brown, Kevin K.

    2009-01-01

    Purpose: To describe a cohort of patients who presented with interstitial lung disease (ILD) of unknown cause, features of primary Sjögren syndrome (pSS), and a positive minor salivary gland biopsy (MSGB). Methods: Thirty-eight patients with ILD evaluated at our center underwent an MSGB to confirm a diagnosis of pSS. All of the samples were reviewed by pathologists experienced in the evaluation of salivary gland histology. We defined a positive MSGB finding as a lymphocyte focus score of >1. Results: At presentation, all patients had ILD, and symptoms of cough and dyspnea. None had a definable connective tissue disease (CTD) or known cause for their ILD. Thirteen patients (34%) had positive MSGB findings. Of these, the median age was 61 years (age range, 33 to 75 years); 7 patients (54%) were women; 8 patients (62%) had a smoking history; and 10 patients (77%) had sicca symptoms. In all patients, a thoracic high-resolution CT scan evaluation demonstrated bibasilar, peripheral-predominant, ground-glass, and reticular opacities. Four patients (31%) were negative for both antinuclear autoantibody (ANA) and rheumatoid factor (RF) autoantibody, and three patients (23%) were negative for ANA, RF, Sjögren syndrome (SS)-A, and SS-B autoantibodies. No patients experienced any complications from the MSGB. The identification of underlying pSS did not affect the management of ILD in these patients. Conclusions: Confirming a diagnosis of pSS-related ILD by performing MSGB allows for a moreprecise CTD classification. This study provides evidence that CTD may exist subclinically, andlongitudinal studies are needed to determine whether identifying occult CTD impacts on management, longitudinal changes in lung function, or survival. PMID:19429722

  16. Clinical, histopathological and immunohistological study of lymphoid disorders in the parotid gland of patients with Sjögren's syndrome

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    Trenkić-Božinović Marija

    2009-01-01

    Full Text Available Bacground/Aim. Sjögren's syndrome is a chronic autoimmune systemic disease characterized by polyglandular tissue destruction, leading to keratoconjunctivitis sicca and xerostomia. These patients have 44-fold increased risk of developing salivary gland lymphoma, of which 80% are marginal zone (MALT type. Having in mind that criteria for distinguishing benign lymphoepithelial lesions from MALT lymphoma are obscure, the aim of this study was to provide practical information that could be integrated into diagnostic practice. Methods. Among 32 parotidectomies, 27 cases were identified as having benign lymphoepithelial disorders and 5 cases low grade MALT lymphoma. Histological sections were stained routinely with hematoxylin and eosin (H&E and special stains. Immunohistochemical study was performed by LSAB2 method, by using primary antibodies for CD20, CD3, Kappa and Lambda light chains and Cytokeratin (Dako Denmark. Results. The 27 patients with Sjögren's sialoadenitis (22 women and 5 men, and 5 patients with MALT lymphoma (only women were included in this analysis. According to the Ann Harbor Classification, all patients with MALT lymphoma had stage IE. Both groups of patients had an indolent clinical course, except permanent, rapid parotid enlargement in the patients with MALT lymphoma. Histologically, the periductal lymphoid infiltrate, gradually extended to the acini, completely replacing them by a sea of polyclonal lymphocytes, immunoblasts, germinal centers and plasma cells (confirmed immunohistochemically, but sparing the ducts and preserving lobular appearance. The histological feature of salivary gland MALT lymphoma included heterogeneous B-cell infiltrate that totally or subtotally had effaced the normal glandular structure. Malign lymphoepithelial lesions, representing infiltration of the ductal and epithelial structures by monoclonal neoplastic Bcells, positive for CD20, were highlighted by antibody to cytokeratin. Conclusion. The

  17. Effect of liposomal local therapy on salivary glands in acoustic radiation force impulse imaging in Sjögren's syndrome.

    Science.gov (United States)

    Hofauer, Benedikt; Mansour, Naglaa; Heiser, Clemens; Straßen, Ulrich; Bas, Murat; Knopf, Andreas

    2016-10-01

    The purpose of this study was to evaluate the utility of acoustic radiation force impulse (ARFI) imaging as a monitoring tool for the effect of a liposomal local therapy in patients with dry mouth symptoms due to primary Sjögren's syndrome (pSS). Fifty patients with pSS, diagnosed according to the American-European Consensus Group (AECG) criteria, were included. Clinical data were collected, and sonographic examination including ARFI imaging of the parotid and submandibular glands was performed. Subjective symptoms were evaluated via visual analogue scales (VAS), and the unstimulated whole salivary flow was measured. After a two-month period of liposomal local therapy, sonographic examination was repeated and both subjective and objective symptoms were re-evaluated. Before local treatment, the mean ARFI value of parotid glands was 2.96 m/s (SD 0.97). Mean ARFI value of the submandibular glands was 2.09 m/s (SD 0.71). After the two-month treatment period, a significant decline of ARFI values in the parotid glands to a value of 2.34 m/s (SD 0.70, p dry mouth symptoms could be observed (p = 0.0001). With the application of ARFI imaging, a decline in parotid gland stiffness could be observed in patients with primary Sjögren's Syndrome accompanied by a significant improvement of the subjective sensation of dry mouth symptoms. The seromucous submandibular glands did not show any changes compared to the serous parotid glands.

  18. CP-25 Alleviates Experimental Sjögren's Syndrome Features in NOD/Ltj Mice and Modulates T Lymphocyte Subsets.

    Science.gov (United States)

    Gu, Fang; Xu, Shixia; Zhang, Pengying; Chen, Xiaoyun; Wu, Yujing; Wang, Chun; Gao, Mei; Si, Min; Wang, Xinming; Heinrich, Korner; Wu, Huaxun; Wei, Wei

    2018-04-17

    Primary Sjögren's syndrome (pSS) is a chronic inflammatory autoimmune illness of the moisture-producing glands such as salivary glands that is characterized by various immune abnormalities. The aetiology of pSS remains unclear and there is no curative agent. In this study, we investigated the putative therapeutic effects on a NOD/Ltj mouse model of Sjögren's syndrome-like disorders of an ester derivative of paeoniflorin, Paeoniflorin-6'O-benzene (termed CP-25). Our study showed that CP-25 alleviated effectively clinical manifestations in NOD/Ltj mice resulting for example in increased salivary flow and reduced histopathological scores. Furthermore, CP-25 decreased lymphocyte viability in NOD/Ltj mice and attenuated the infiltration of Th1 cells and Th2 cells into the salivary glands of NOD/Ltj mice. In the spleen on NOD/Ltj mice, CP-25 skewed the ratio of Th17 and regulatory T cells towards regulatory T cells. After treatment, concentrations of anti-La/SSB and IgG antibodies were reduced and the titer of the inflammatory cytokines IFN-γ, IL-4, IL-6 and IL-17A in the serum on NOD/Ltj mice was alleviated. Thus, we define CP-25 as a novel compound that is a potent therapeutic agent for pSS by modulating T lymphocyte subsets. Future studies will validate the use of CP-25 as a therapeutic strategy for the treatment of pSS. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  19. Pulmonary cryptococcosis in childhood systemic lupus erythematosus and Sjögren syndrome overlap: a rare opportunistic infection.

    Science.gov (United States)

    Marques, V L S; Gomes, R C; Viola, G R; Maia, M M; Durigon, G S; Aikawa, N E; Artur Silva, C

    2013-11-01

    Meningitis is the main manifestation of cryptococcosis in adult systemic lupus erythematosus (SLE) patients, and other organs and systems, such as the lungs, are rarely affected in this fungal infection. To our knowledge, no case of pulmonary cryptococcosis has been described in the pediatric lupus population. Therefore, we report herein one patient with childhood SLE (C-SLE) and Sjögren's syndrome overlap that presented encapsulated Cryptococcus yeast cells in lung tissue. A 14-year-old girl was diagnosed with C-SLE. At the age of 16 years and 5 months, she presented with fever, cough and dyspnea, without headache, vomiting, and also without signs of meningeal irritation or other clinical manifestations. She was being treated with mycophenolate mofetil, hydroxychloroquine and prednisone. Chest radiography and chest computer tomography showed a single nodule in the left posterior apex and three nodular lesions in the left hemithorax respectively. Bronchoalveolar lavage and transbronchial biopsy were normal and without isolation of bacteria or fungi. Voriconazole was empirically introduced for 21 days. Fifteen days after the first biopsy, she underwent open thoracotomy with surgical left lung biopsy and was diagnosed with pulmonary cryptococcosis. Voriconazole was replaced with oral fluconazole and this antifungal therapy was maintained with improvement of clinical manifestations and without marked alteration of radiological images. In conclusion, we report the first case of pulmonary cryptococcosis in Sjögren's and C-SLE patient with a satisfactory clinical response to antifungal therapy. Fungal infections should be excluded in the presence of lung nodules and etiological identification is required for proper treatment.

  20. Stick-Jump (SJ) Evaporation of Strongly Pinned Nanoliter Volume Sessile Water Droplets on Quick Drying, Micropatterned Surfaces.

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    Debuisson, Damien; Merlen, Alain; Senez, Vincent; Arscott, Steve

    2016-03-22

    We present an experimental study of stick-jump (SJ) evaporation of strongly pinned nanoliter volume sessile water droplets drying on micropatterned surfaces. The evaporation is studied on surfaces composed of photolithographically micropatterned negative photoresist (SU-8). The micropatterning of the SU-8 enables circular, smooth, trough-like features to be formed which causes a very strong pinning of the three phase (liquid-vapor-solid) contact line of an evaporating droplet. This is ideal for studying SJ evaporation as it contains sequential constant contact radius (CCR) evaporation phases during droplet evaporation. The evaporation was studied in nonconfined conditions, and forced convection was not used. Micropatterned concentric circles were defined having an initial radius of 1000 μm decreasing by a spacing ranging from 500 to 50 μm. The droplet evaporates, successively pinning and depinning from circle to circle. For each pinning radius, the droplet contact angle and volume are observed to decrease quasi-linearly with time. The experimental average evaporation rates were found to decrease with decreasing pining radii. In contrast, the experimental average evaporation flux is found to increase with decreasing droplet radii. The data also demonstrate the influence of the initial contact angle on evaporation rate and flux. The data indicate that the total evaporation time of a droplet depends on the specific micropattern spacing and that the total evaporation time on micropatterned surfaces is always less than on flat, homogeneous surfaces. Although the surface patterning is observed to have little effect on the average droplet flux-indicating that the underlying evaporation physics is not significantly changed by the patterning-the total evaporation time is considerably modified by patterning, up to a factor or almost 2 compared to evaporation on a flat, homogeneous surface. The closely spaced concentric circle pinning maintains a large droplet radius and

  1. Prevalência da síndrome de Sjögren primária em importante área metropolitana no Brasil Primary Sjögren's syndrome prevalence in a major metropolitan area in Brazil

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    Valéria Valim

    2013-02-01

    Full Text Available Não há estudo anterior sobre a prevalência de síndrome de Sjögren (SS no Brasil. O objetivo deste estudo foi avaliar a prevalência de SS na população geral de Vitória, estado do Espírito Santo, Brasil. Trata-se de estudo epidemiológico, observacional e transversal com 1.205 indivíduos randomizados, cujas idades variaram de 18 a 65 anos, residentes em Vitória. Os indivíduos foram triados para xerostomia e xeroftalmia por meio de entrevista domiciliar. Aqueles com sintomas "sicca" foram convidados a comparecer ao hospital para realizar avaliação médica adicional, avaliação do fluxo salivar não estimulado, teste de Schirmer I, exame de sangue e biopsia de glândula salivar labial menor. Os sintomas "sicca" foram identificados em 18% (217 indivíduos da amostra. Dos 217 indivíduos com sintomas "sicca", 127 (58% estavam disponíveis para exame. Nessa amostra, 61,7% eram mulheres e 46,8% usavam medicação. A síndrome "sicca" foi confirmada em 12% deles por pelo menos um exame (fluxo salivar ou teste de Schirmer I. Dois pacientes (0,17% preencheram quatro critérios classificatórios americano-europeus (95% IC = 0,020-0,5983.There has been no previous prevalence study about of Sjögren's syndrome (SS in Brazil. The aim was to evaluate the SS prevalence in a general population in Vitória, ES, Brazil. This was an epidemiological, observational, and cross-sectional study conducted on 1,205 randomized people, aged 18-65 years, who lived in Vitória. The subjects were screened for xerostomia and xerofphthalmia through home interviews. Those with sicca symptoms were asked to report to a hospital for further medical evaluation, unstimulated salivary flow, Schirmer I test, blood analysis and minor labial salivary biopsy. Sicca symptoms were found in 18% (217 subjects of the sample. Of the 217 subjects with sicca symptoms, 127 (58% were available for examination. In this sample, 61.7% were female and 46.8% were under medication. Sicca

  2. Sexual dimorphic function of IL-17 in salivary gland dysfunction of the C57BL/6.NOD-Aec1Aec2 model of Sjögren's syndrome.

    Science.gov (United States)

    Voigt, Alexandria; Esfandiary, Lida; Wanchoo, Arun; Glenton, Patricia; Donate, Amy; Craft, William F; Craft, Serena L M; Nguyen, Cuong Q

    2016-12-13

    Interleukin (IL)-17 is one of the critical inflammatory cytokines that plays a direct role in development of Sjögren's syndrome (SjS), a systemic autoimmune disease characterized by a progressive chronic attack against the exocrine glands. The expression levels of IL-17 are correlated with a number of essential clinical parameters such as focus score and disease duration in human patients. Significantly immunological differences of Th17 cells were detected at the onset of clinical disease in female SjS mice compared to males. To further define the role of IL-17 in SjS and elucidate its involvement in the sexual dimorphism, we examined the systemic effect of IL-17 by genetically ablating Il-17 in the C57BL/6.NOD-Aec1Aec2, spontaneous SjS murine model. The results indicate that IL-17 is a potent inflammatory molecule in the induction of chemoattractants, cytokines, and glandular apoptosis in males and females. Elimination of IL-17 reduced sialadenitis more drastically in females than males. IL-17 is highly involved in modulating Th2 cytokines and altering autoantibody profiles which has a greater impact on changing plasma cells and germinal center B cell populations in females than males. The result supports a much more important role for IL-17 and demonstrates the sexual dimorphic function of IL-17 in SjS.

  3. Polyneuropathy as Novel Initial Manifestation in a Case of “Nonsecretory” POEMS Syndrome with Sjögren’s Syndrome

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    Minrui Liang

    2017-01-01

    Full Text Available POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes is a paraneoplastic syndrome driven by plasma cell dyscrasias. We report a patient with novel initial manifestation of polyneuropathy, which was considered due to Sjögren’s syndrome but with poor response to methylprednisolone (120 mg/d and intravenous immunoglobulin (IVIg. Further investigation by imaging tests and following biopsy eventually confirmed the diagnosis of POEMS syndrome secondary to solitary plasmocytoma. To our knowledge, this is the first reported case of POEMS syndrome with Sjögren’s syndrome occurring in the absence of a peripheral monoclonal gammopathy, highlighting the diagnostic challenges posed by this disease and reviewing the diagnostic role of (18 F-FDG PET/CT in POEMS syndrome.

  4. Marginal zone B-cell lymphoma with multiple extranodal locations in a patient with Sjögren’s syndrome – a diagnostic problem

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    Marta Domżalska

    2014-09-01

    Full Text Available Sjögren’s syndrome is a chronic autoimmune disease characterized by the presence of lymphocytic infiltrates in exocrine glands, mainly salivary and lacrimal glands, which result in xerophthalmia and xerostomia. About half of the patients develop systemic complications, including lymphoproliferative disorders. We report a case of a 27-year-old woman with a diagnosis of Sjögren’s syndrome and a suspicion of respiratory system involvement in the course of granulomatosis with polyangiitis. Histopathological examination of a skin lesion suggested marginal zone B-cell lymphoma. After pathological and immunohistochemical evaluation of all available previous biopsy samples and the medical documentation the diagnosis of extranodal marginal zone B-cell lymphoma stage IV according to the Ann Arbor classification was rendered. The patient was referred to the Department of Haematology and was treated with R-CVP (cyclophosphamide, vincristine, prednisone, rituximab.

  5. Acupuncture at Waiguan (SJ5) and sham points influences activation of functional brain areas of ischemic stroke patients: a functional magnetic resonance imaging study

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    Qi, Ji; Chen, Junqi; Huang, Yong; Lai, Xinsheng; Tang, Chunzhi; Yang, Junjun; Chen, Hua; Qu, Shanshan

    2014-01-01

    Most studies addressing the specificity of meridians and acupuncture points have focused mainly on the different neural effects of acupuncture at different points in healthy individuals. This study examined the effects of acupuncture on brain function in a pathological context. Sixteen patients with ischemic stroke were randomly assigned to true point group (true acupuncture at right Waiguan (SJ5)) and sham point group (sham acupuncture). Results of functional magnetic resonance imaging revea...

  6. A third study on the use of orally administered anhydrous crystalline maltose for relief of dry mouth in primary Sjögren's syndrome.

    Science.gov (United States)

    Fox, Philip C; Cummins, Martin J; Cummins, Joseph M

    2002-10-01

    To examine the safety and efficacy of anhydrous crystalline maltose for treatment of dry mouth and other symptoms of dryness in patients with primary Sjögren's syndrome. Anhydrous crystalline maltose was delivered orally as a 200-mg lozenge given three times daily over a 24-week period to a total of 100 subjects. All participants had prominent complaints of persistent dry mouth associated with primary Sjögren's syndrome. Patients were examined at baseline and every 6 weeks of treatment. Patients were seen in outpatient clinics at a total of 27 sites within the United States. Unstimulated whole saliva output, a measure of basal salivary gland function, was determined at each visit. Symptoms associated with oral and ocular dryness were assessed at the same time with the use of 100-mm visual analogue scales. Safety was assessed by physical examination and laboratory studies. During this clinical trial, a majority of evaluable subjects (39/76) demonstrated an increase in unstimulated whole saliva output, and the treatment exhibited an excellent safety profile. The anhydrous crystalline maltose treatment led to significant improvement in several subjective measures of oral and ocular comfort. In this study, anhydrous crystalline maltose lozenges administered three times daily for 24 weeks improved salivary output and decreased complaints of dry mouth and eyes in patients with primary Sjögren's syndrome. Side-effects were minimal, and treatment was without significant adverse events. These results are similar to the benefits observed in two prior studies reported by the authors. This safe and simple intervention appears to provide clinical benefit to primary Sjögren's syndrome patients with distressing dry mouth symptoms.

  7. Crying Without Tears: Dimensions of Crying and Relations With Ocular Dryness and Mental Well-Being in Patients With Sjögren's Syndrome.

    Science.gov (United States)

    van Leeuwen, N; Bossema, E R; Vermeer, R R; Kruize, A A; Bootsma, H; Vingerhoets, A J J M; Bijlsma, J W J; Geenen, R

    2016-03-01

    This study examined dimensions of crying and its relations with ocular dryness and mental well-being in patients with Sjögren's syndrome, a systemic autoimmune disease with dryness as primary symptom. Three-hundred patients with Sjögren's syndrome completed questionnaires on crying, dryness, and well-being. The crying questionnaire revealed four dimensions: "Cryability" (comprising both crying sensibility and ability to cry), Somatic consequences, Frustration, and Suppression. Compared to 100 demographically-matched control participants from the general population, patients scored low on Cryability and high on Somatic consequences and Frustration. The crying dimensions generally showed significant but weak associations with ocular dryness and mental well-being in patients. This is the first quantitative study indicating that crying problems are more common in patients with Sjögren's syndrome than in the general population. Perhaps, patients who experience problems with crying could be helped to rely on other ways of expressing emotions than crying in tear-inducing situations.

  8. Comparison of the NEI-VFQ and OSDI questionnaires in patients with Sjögren's syndrome-related dry eye

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    Smith Janine A

    2004-09-01

    Full Text Available Abstract Background To examine the associations between vision-targeted health-related quality of life (VT-HRQ and ocular surface parameters in patients with Sjögren's syndrome, a systemic autoimmune disease characterized by dry eye and dry mouth. Methods Forty-two patients fulfilling European / American diagnostic criteria for Sjögren's syndrome underwent Schirmer testing without anesthesia, ocular surface vital dye staining; and measurement of tear film breakup time (TBUT. Subjects were administered the Ocular Surface Disease Index (OSDI and the 25-item National Eye Institute Vision Functioning Questionnaire (NEI-VFQ. Main outcome measures included ocular surface parameters, OSDI subscales describing ocular discomfort (OSDI-symptoms, vision-related function (OSDI-function, and environmental triggers, and NEI-VFQ subscales. Results Participants (aged 31–81 y; 95% female all had moderate to severe dry eye. Associations of OSDI subscales with the ocular parameters were modest (Spearman r (ρ Conclusions Associations between conventional objective measures of dry eye and VT-HRQ were modest. The generic NEI-VFQ was similar to the disease-specific OSDI in its ability to measure the impact of Sjögren's syndrome-related dry eye on VT-HRQ.

  9. Oral conditions in patients with Sjögren’s Syndrome: a Systematic Review - doi:10.5020/18061230.2006.p234

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    Marina Fernandes de Sena

    2012-01-01

    Full Text Available The aim of this study was to investigate through a systematic review, the oral manifestations of Sjögren’s syndrome. It had as research sources: manual searches in publications, sites and electronic data bases such as MEDLINE, LILACS and BBO. As its inclusion criteria: cross-sectional, case-control and cohort studies which data collection was done by means of clinical indexes for dental caries, periodontal disease and oral mucosa. The selected idioms were: Portuguese, English and Spanish; in the period of 1990 to 2003. Searching strategies used included the following words: Sjögren, dmf, caries, decay, periodontal, plaque and gingivitis. Thirteen studies were selected, one of these in Spanish and the others in English. All delineations were case-control, 54% of these aimed at evaluating the relationship between patients with the syndrome and caries presence, 85% with periodontal disease and 32% relating to the alterations of oral mucosa. The analyzed studies showed that the main symptom of Sjögren’s syndrome is xerostomy and that exist a slight association between syndromic patients and dental caries index and some alterations of oral mucosa and a weak association with periodontal diseases.

  10. Współwystępowanie zespołu Sjögrena i twardziny układowej

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    Justyna Morawska

    2010-12-01

    Full Text Available Zespół Sjögrena jest przewlekłą chorobą zapalną o podłożu autoimmunologicznymcharakteryzującą się upośledzeniem czynnościgruczołów wydzielania zewnętrznego. Skutkuje to powstaniemobjawów, nazywanych zespołem suchości. Tylko w ok. 40% jest tozespół pierwotny, a w pozostałej cz��ści towarzyszy innym schorzeniom,najczęściej również o podłożu autoimmunologicznym,takim jak twardzina układowa. Ponad połowa chorych z twardzinąukładową ma charakterystyczne dla zespołu suchości objawy,natomiast tylko niewielki procent tych osób spełnia kryteria rozpoznaniazespołu Sjögrena. Na podstawie dostępnego piśmiennictwaprzeanalizowano częstość występowania zespołu Sjögrenaw grupie osób z rozpoznaną twardziną układową oraz przebiegobu chorób w przypadku ich współistnienia.

  11. Correlação entre o grau de xerostomia e o resultado da sialometria em pacientes com Síndrome de Sjögren Correlation between xerostomic level and the result of the sialometry in patients with Sjögren Syndrome

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    Gustavo Polacow Korn

    2002-10-01

    Full Text Available Introdução: Xerostomia é a sensação subjetiva de boca seca, que pode estar associada à várias doenças ou alterações sistêmicas, incluindo a Síndrome de Sjögren. Forma de estudo: Clínico prospectivo. Material e método: Realizamos um estudo com 8 pacientes portadores de Síndrome de Sjögren e que se queixavam de xerostomia. Esses pacientes foram submetidos a um questionário que abordava sinais e sintomas comumente relacionados à xerostomia e realizaram uma sialometria a fim de se tentar estabelecer algum tipo de associação entre ambos (questionário e sialometria. Resultado: Observamos que apenas 3 pacientes apresentaram um fluxo salivar diminuído (anormal, enquanto todos eles referiam sintomas orais, em graus variáveis. Conclusão: Assim, concluímos que a intensidade/quantidade de queixas orais não está diretamente relacionada ao fluxo salivar dos pacientes.Introduction: Xerostomia, a subjective dry mouth feeling, may be associated to several diseases and systemic disorders incluiding Sjögren syndrome. Study design: Clinical prospective. Material and method: We conducted an eight Sjögren syndrome patients study with xerostomia complains. They were asked to answer a group of questions related to commonly xerostomia signs and symptoms and had, in addition, a sialometry performed in order to assess wether any correlation could be found between both kinds of observations. Results: In spite of variable degrees of oral symptoms present in all eight patients, saliva flow decrease could only be documented in three. Conclusion: Therefore, in our observation, no correlation could be found between oral symptoms magnitude and amount of saliva secreted in Sjögren patients.

  12. Avaliação da sialometria e biópsia de glândula salivar menor na classificação de pacientes com Síndrome de Sjögren Evaluation of sialometry and minor salivary gland biopsy in classification of Sjögren's Syndrome patients

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    Bianca Maria Liquidato

    2005-06-01

    Full Text Available A Síndrome de Sjögren é afecção auto-imune das glândulas exócrinas, que envolve particularmente as glândulas salivares e lacrimais. Não existe exame de certeza para diagnóstico. OBJETIVO: Avaliação da importância do papel da biópsia de glândula salivar menor e da sialometria, isoladamente ou associadas, como métodos utilizados para a classificação da Síndrome de Sjögren. FORMA DE ESTUDO: Coorte transversal. CASUÍSTICA E MÉTODO: Todos os 72 pacientes com queixa de boca seca, de janeiro de 1997 a setembro de 2003, foram submetidos à investigação diagnóstica e classificação com base nos critérios estabelecidos. A sialometria não-estimulada foi realizada com a técnica do swab. Os exames histopatológicos foram avaliados quanto à presença de focos inflamatórios. RESULTADOS: A sialometria não-estimulada e a biópsia de glândula salivar menor apresentaram sensibilidades diferentes para Síndrome de Sjögren primária e Síndrome de Sjögren secundária. A sialadenite focal com maior número de focos foi característica da Síndrome de Sjögren primária. Compararam-se biópsia e sialometria e observou-se que a especificidade e o valor preditivo positivo da biópsia foram maiores. Entre biópsia e biópsia associada à sialometria, a biópsia teve maior sensibilidade e maior valor preditivo negativo. A especificidade da biópsia associada à sialometria foi maior. Entre sialometria e biópsia associada à sialometria, a biópsia associada à sialometria apresentou maior valor preditivo positivo e maior especificidade. A sensibilidade da sialometria foi maior. CONCLUSÕES: Os testes sialometria e biópsia apresentaram desempenhos diferentes nos pacientes com Síndrome de Sjögren primária e secundária; a positividade dos dois critérios em conjunto aumenta muito a especificidade para Síndrome de Sjögren (95%.Sjögren's Syndrome is an autoimmune disease of the exocrine glands, mainly salivary and lachrymal glands

  13. Total glucosides of peony ameliorates Sjögren's syndrome by affecting Th1/Th2 cytokine balance.

    Science.gov (United States)

    Wu, Guolin; Wu, Nayuan; Li, Tianyi; Lu, Wenwen; Yu, Guoyou

    2016-03-01

    The present study aimed to investigate the molecular mechanisms underlying the effects of total glucosides of peony (TGP) in the treatment of Sjögren's syndrome (SS). A total of 40 mice with SS were evenly assigned into four groups, including: Control group; TGP group, receiving 1 mg TGP daily; hydroxychloroquine (HCQ) group, receiving 0.25 mg HCQ daily; and a combined group, receiving 1 mg TGP and 0.25 mg HCQ daily. After 8 weeks, quantitative polymerase chain reaction and an enzyme-linked immunosorbent assay were used to detect the levels of interferon-γ (IFN-γ), interleukin-4 (IL-4), Fas and FasL in each group of mice. In addition, immunohistochemical analysis was used to determine the expression levels of IFN-γ and IL-4. IFN-γ, IL-4, Fas and FasL levels were significantly increased in the control group compared with the other three groups (PTGP and combined groups compared with the control group (PTGP ameliorates SS by affecting the Th1/Th2 cytokine balance and decreasing the expression levels of IFN-γ, IL-4, Fas and FasL. Therefore, TGP may represent a potential novel therapeutic agent for the treatment of SS.

  14. Xerostomia, xerophthalmia, and plasmacytic infiltrates of the salivary glands (Sjögren's-like syndrome) in a cat.

    Science.gov (United States)

    Canapp, S O; Cohn, L A; Maggs, D J; Miller, M A; Kerl, M E; O'Brien, D P

    2001-01-01

    A 2.5-year-old domestic shorthair cat was evaluated because of dysphagia and weight loss of 4 weeks' duration. MIld blepharospasm and conjunctival hyperemia were evident in both eyes, oral mucous membranes were tacky on palpation, and salivary glands were enlarged. Results of a Schirmer tear test were 0 mm/min for both eyes. Administration of atropine did not cause salivation or caused secretion fo thick rope-like saliva. Examination of biopsy specimens of salivary glands revealed a plasmacytic infiltrate. Sjögren's syndrome (SS) was diagnosed. Oral administration of prednisone was instituted but was discontinued after a minimal positive response was evident 6 weeks after initiation of treatment. Palliative treatment with a 6% solution of pilocarpine 4 to 5 times/d, cyclosporine, hylan A, and neomycin-polymyxin-bacitracin ophthalmic ointment resulted in clinical improvement in the cat. Although reported rarely in animals, SS may be more common than currently is recognized. Most treatment regimens for SS are aimed at alleviating clinical signs.

  15. Periodontitis prevalence and serum antibody reactivity to periodontal bacteria in primary Sjögren's syndrome: a pilot study.

    Science.gov (United States)

    Lugonja, Bozo; Yeo, Lorraine; Milward, Michael R; Smith, Diana; Dietrich, Thomas; Chapple, Iain L C; Rauz, Saaeha; Williams, Geraint P; Barone, Francesca; de Pablo, Paola; Buckley, Chris; Hamburger, John; Richards, Andrea; Poveda-Gallego, Ana; Scheel-Toellner, Dagmar; Bowman, Simon J

    2016-01-01

    The aims of this study were as follows: (i) To assess the prevalence of periodontitis among patients with primary Sjögren's syndrome (pSS) and comparator groups of patients with rheumatoid arthritis (RA) and osteoarthritis (OA). (ii) To perform a pilot study to compare serum antibody responses to 10 oral/periodontal bacteria in these patient groups and a historical comparator group of patients with periodontitis. Standard clinical periodontal assessments were performed on 39 pSS, 36 RA and 23 OA patients and "In-house" antibody ELISAs for serum antibodies against 10 oral/periodontal bacteria were performed in these groups. Forty-six percent of the pSS group, 64% of the RA group and 48% of the OA group had moderate/severe periodontitis. These frequencies did not reach statistical significance between groups. Raised antibody levels to Prevotella denticola were found in the pSS, RA and periodontitis groups compared to the OA group. Significant between group differences were seen for Aggregatibacter actinomycetemcomitans, Prevotella intermedia and Campylobacter showae. None of these differences were specifically associated with pSS. This study showed no increase in periodontitis in pSS patients. Although the P. denticola data are of interest, identifying bacterial triggering factors for pSS will likely require alternative strategies including modern techniques such as microbiome analysis. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  16. Immunogenetics of rheumatoid arthritis and primary Sjögren's syndrome: DNA polymorphism of HLA class II genes

    DEFF Research Database (Denmark)

    Morling, Niels; Andersen, V; Fugger, L

    1992-01-01

    We investigated the DNA restriction fragment length polymorphism (RFLP) of the Major Histocompatability Complex (MHC) class II genes: HLA-DRB, -DQA, -DQB, DPA, and -DFB in 24 patients with rheumatoid arthritis (RA), in 19 patients with primary Sjögren's syndrome (primary SS), and healthy Danes....... The frequencies of DNA fragments associated with the following HLA class II genes were increased in RA when compared to normal controls: DRB1*04 (DR4) (relative risk, RR = 7.4, P less than 10(-3), DRB4*0101 (DRw53) (RR = 9.6, P less than 10(-3), DQA1*0301 (RR = 9.6, P less than 10(-3), DQB1*0301 (DQw7) (RR = 2.......05). The frequencies in RA of other HLA class II associated DNA fragments including DPA and DPB and the antigens DPw1-w6 defined by primed lymphocyte stimulation, did not differ significantly from those in controls. In primary SS, the frequency of HLA-B8 was significantly increased (RR = 9.0, P less than 10...

  17. A functional variant in the OAS1 gene is associated with Sjögren's syndrome complicated with HBV infection.

    Science.gov (United States)

    Liu, Xianjun; Xing, Hongcun; Gao, Wenjing; Yu, Di; Zhao, Yuming; Shi, Xiaoju; Zhang, Kun; Li, Pingya; Yu, Jiaao; Xu, Wei; Shan, Hongli; Zhang, Kaiyu; Bao, Wanguo; Fu, Xueqi; Yang, Sirui; Wang, Shafeng

    2017-12-14

    Hepatitis B virus (HBV) has been suspected to contribute to several autoimmune diseases, including Sjögren's syndrome (SS), although the exact mechanism is unknown. The 2'-5' oligoadenylate synthetase (OAS1) is one of the most important components of the immune system and has significant antiviral functions. We studied a polymorphism rs10774671 of OAS1 gene in Han Chinese descent. The minor allele G was significantly associated with a decreased risk for SS, anti-SSA-positive SS, and anti-SSA-positive SS complicated with HBV infection, which have not been seen in anti-SSA-negative SS and HBcAb-negative SS patients. Gene expression analysis showed that the risk-conferring A allele was correlated with lower expression of p46 and increased expression of p42, p48, and p44. A functional study of enzymatic activities revealed that the p42, p44, and p48 isoforms display a reduced capacity to inhibit HBV replication in HepG2 cells compared to the normal p46 isoform. Our data demonstrated that the functional variant, rs10774671, is associated with HBV infection and anti-SSA antibody-positive SS. The SAS variant switches the primary p46 isoform to three alternatives with decreased capacities to inhibit HBV replication. These data indicated that individuals harboring the risk allele might be susceptible to hepatitis B infection and SS development.

  18. Arterial Spin Labeling Imaging for the Parotid Glands of Patients with Sjögren’s Syndrome

    Science.gov (United States)

    Kami, Yukiko N.; Sumi, Misa; Takagi, Yukinori; Sasaki, Miho; Uetani, Masataka; Nakamura, Takashi

    2016-01-01

    Sjögren’s syndrome (SS) is characterized by hypofunction of the salivary and lacrimal glands. The salivary function is largely dependent upon the blood supply in the glands. However, the diseased states of the gland perfusion are not well understood. The arterial spin labeling (ASL) technique allows noninvasive quantitative assessment of tissue perfusion without the need for contrast agent. Here, we prospectively compared the perfusion properties of the parotid glands between patients with SS and those with healthy glands using ASL MR imaging. We analyzed salivary blood flow (SBF) kinetics of 22 healthy parotid glands from 11 volunteers and 28 parotid glands from 14 SS patients using 3T pseudo-continuous ASL imaging. SBF was determined in resting state (base SBF) and at 3 sequential segments after gustatory stimulation. SBF kinetic profiles were characterized by base SBF level, increment ratio at the SBF peak, and the differences in segments where the peak appeared (SBF types). Base SBFs of the SS glands were significantly higher than those of healthy glands (59.2 ± 22.8 vs. 46.3 ± 9.0 mL/min/100 g, p = 0.01). SBF kinetic profiles of the SS glands also exhibited significantly later SBF peaks (p parotid glands were mostly hyperemic and the SS gland responses to gustatory stimulation were stronger and more prolonged than those of the healthy glands. The ASL may be a promising technique for assessing the diseased salivary gland vascularization of SS patients. PMID:26959680

  19. Oral distress in primary Sjögren's syndrome: implications for health-related quality of life.

    Science.gov (United States)

    Enger, Tone B; Palm, Øyvind; Garen, Torhild; Sandvik, Leiv; Jensen, Janicke L

    2011-12-01

    The aims of the study were to evaluate oral distress in patients with primary Sjögren's syndrome (pSS) compared with age- and sex-matched Norwegian normative data, to estimate the occurrence of oral symptoms in pSS, and to evaluate the impact of oral distress on health-related quality of life (HRQoL). The Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) was used to assess HRQoL, and the Oral Health Impact Profile 14 (OHIP-14) was used to measure oral distress. Of the 246 pSS patients invited to participate in the study, 177 (72%) responded. Data were analysed for the female participants (n = 163). Significant deviations from normative estimates were found in all OHIP-14 item results, and the findings indicated a high level of oral distress among the pSS patients. Health-related quality of life was decreased among pSS patients, with the largest deviations from normative estimates related to general health and role physical. The patients with high levels of oral distress scored significantly lower than patients with low levels of oral distress in five of the SF-36 subscales, indicating that oral conditions have a marked impact on general quality of life. In conclusion, oral distress in pSS is pronounced and severe, and should receive increased attention with a view to improving the quality of life for these patients. © 2011 Eur J Oral Sci.

  20. Primary Sjögren syndrome that initially presented with repeated hypergammaglobulinemic purpura after prolonged sitting: A case report.

    Science.gov (United States)

    Zhou, Zhihua; Jiang, Weiqiang; Wang, Ming; Liu, Yongyuan; Zhang, Wei; Huang, Manping; Liang, Donghui

    2017-12-01

    Purpura is a common dermatologic manifestation in Sjögren syndrome (SS). When a patient presents with sicca symptoms, the diagnosis of SS is not difficult. Here, we reported a case of a 52-year-old Chinese woman who initially presented with nonpalpable purpura on both lower extremities, and these lesions had developed soon after prolonged sitting. In the past 2 years, she had repeated cutaneous nonpalpable purpura 4 times. She had no sicca symptoms, dry eyes, or dry mouth. Combining the laboratory findings, Schirmer test, and labial gland biopsy, primary SS was confirmed. The patient was placed on a trial of hydroxychloroquine (200 mg once daily). The purpura on both lower extremities had faded at the sixth day after onset and at the third day after hydroxychloroquine treatment. These case was not easy to diagnosis primary SS because she had no sicca symptoms. A patient with primary SS who initially presented with recurrent purpura associated with prolonged sitting. Prolonged sitting had been a possible aggravating factor for the cutaneous purpura of this patient with primary SS. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  1. Translation and Validation of a Korean Version of the Xerostomia Inventory in Patients with Primary Sjögren's Syndrome.

    Science.gov (United States)

    Lee, Jennifer; Koh, Jung Hee; Kwok, Seung-Ki; Park, Sung-Hwan

    2016-05-01

    This study was conducted to generate and validate a cross-culturally adapted Korean version of the xerostomia inventory (XI), an 11-item questionnaire designed to measure the severity of xerostomia. The original English version of the XI was translated into Korean according to the guidelines for cross-cultural adaptation of health-related quality-of-life measures. Among a prospective cohort of primary Sjögren's syndrome (pSS) in Korea, 194 patients were analyzed. Internal consistency was evaluated by using Cronbach's alpha, and test-retest reliability was obtained by using an intraclass correlation coefficient (ICC) analysis. Construct validity was investigated by performing a correlation analysis between XI total score and salivary flow rate (SFR). Cronbach's alpha for internal consistency was 0.868, and the ICC for test-retest reliability ranged from 0.48 to 0.827, with a median value of 0.72. Moderate negative correlations between XI score and stimulated SFR, unstimulated SFR, and differential (stimulated minus unstimulated) SFR were observed (Spearman's rho, ρ = -0.515, -0.447, and -0.482, respectively; P xerostomia in the pSS patients. In conclusion, the Korean version of the XI is a reliable tool to estimate the severity of xerostomia in patients with pSS.

  2. Swallowing Disorders in Sjögren's Syndrome: Prevalence, Risk Factors, and Effects on Quality of Life.

    Science.gov (United States)

    Pierce, Jenny L; Tanner, Kristine; Merrill, Ray M; Miller, Karla L; Kendall, Katherine A; Roy, Nelson

    2016-02-01

    This epidemiological investigation examined the prevalence, risk factors, and quality-of-life effects of swallowing disorders in Sjögren's syndrome (SS). One hundred and one individuals with primary or secondary SS (94 females, 7 males; mean age 59.4, SD = 14.1) were interviewed regarding the presence, nature, and impact of swallowing disorders and symptoms. Associations among swallowing disorders and symptoms, select medical and social history factors, SS disease severity, and the M.D. Anderson Dysphagia Inventory (MDADI) and Short Form 36 Health Survey (SF-36) were examined. The prevalence of a current self-reported swallowing disorder was 64.4 %. SS disease severity was the strongest predictor of swallowing disorders, including significant associations with the following swallow symptoms: taking smaller bites, thick mucus in the throat, difficulty placing food in the mouth, and wheezing while eating (p quality of life. Among those with swallowing disorders, 42 % sought treatment, with approximately half reporting improvement. Patient-perceived swallowing disorders are relatively common in SS and increase with disease severity. Specific swallowing symptoms uniquely and significantly reduce swallow and health-related quality of life, indicating the need for increased identification and management of dysphagia in this population.

  3. Four cases of atopic dermatitis complicated by Sjögren's syndrome: link between dry skin and autoimmune anhidrosis.

    Science.gov (United States)

    Kitaba, Shun; Matsui, Saki; Iimuro, Eriko; Nishioka, Megumi; Kijima, Akiko; Umegaki, Noriko; Murota, Hiroyuki; Katayama, Ichiro

    2011-09-01

    We report four adult cases of atopic dermatitis (AD) complicated by Sjögren's syndrome (SS). The patients fulfilled diagnostic criteria for AD and SS. All cases showed persistent itchy dry skin and eczematous lesions complicated by sicca symptoms including dry eyes and dry mouth with moderate joint pain. One case manifested annular erythema and another manifested widespread discoid erythema. To investigate the underlying cause of dry skin in these cases, sweating function was evaluated using a quantitative sudomotor axon reflex test (QSART) in which the axon reflex is stimulated by acetylcholine iontophoresis. The sweating latency time was significantly prolonged in eczematous skin of AD and AD/SS compared to normal controls. Axon reflex (AXR) sweat volume was also significantly reduced in AD (normal and eczematous skin) and AD/SS (normal and eczema) compared to normal control. In contrast, the direct sweat volume of lesional or non-lesional AD skin induced by direct stimulation with acetylcholine was only slightly reduced compared to that in normal controls, but not in SS and lesional skin of AD/SS patients. These results suggest that the impaired sweat response in AD is attributable to an abnormal sudomotor axon reflex, which is accelerated and modulated when complicated by SS resulting in dry skin in the present cases.

  4. A validated method of labial minor salivary gland biopsy for the diagnosis of Sjögren's syndrome.

    Science.gov (United States)

    Kim, Jean; Sun, Daniel; Ozl, Rebecca; Grader-Beck, Thomas; Birnbaum, Julius; Akpek, Esen K; Baer, Alan N

    2016-09-01

    To validate a technique and outcomes of labial minor salivary gland biopsy (LSGB) used for the diagnosis of Sjögren's syndrome (SS). Prospective cohort study. Clinical data were prospectively obtained pre- and postbiopsy using patient-reported questionnaires. LSGB was performed using described methods. Specimens were analyzed by a pathologist with expertise in SS and assessed using established criteria to determine the focus score. Data were analyzed using cross-tabulations. Among the 58 patients in the study, 52 (90%) presented with sicca symptoms of dry eyes and/or mouth. Eight patients (14%) had histopathologic findings supportive of a diagnosis of SS. At 1 month postbiopsy, greater than 71% of patients denied any complaints of pain, swelling, numbness. or tingling. Sixteen patients (28%) had only a minor level of complaints. Only one patient complained of severe numbness at the biopsy site. Greater than 70% of patients would consider rebiopsy after the procedure, if requested by their physician. We present a safe and effective method of LSGB for the diagnosis of SS. 4 Laryngoscope, 126:2041-2046, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  5. Risk factors for positive minor salivary gland biopsy findings in Sjögren's syndrome and dry mouth patients.

    Science.gov (United States)

    Brennan, Michael T; Sankar, Vidya; Leakan, Rose Anne; Kleiner, David; Atkinson, Jane C; Wilkinson, William E; Baum, Bruce J; Pillemer, Stanley R

    2002-04-15

    To investigate risk factors for positive minor salivary gland biopsy results in Sjögren's syndrome (SS) and dry mouth patients. A total of 289 patients with dry mouth symptoms were evaluated. Potential risk factors for positive minor salivary gland biopsy results (>1 focus of lymphocytes) were studied in 2 phases. In phase 1, predictor variable candidates were identified for the test study (phase 2). Odds ratios were calculated for predictor variables. IgG, IgA, keratoconjunctivitis sicca, and sex, identified as the best predictor variables from phase 1 data, were included in a logistic regression model using phase 2 data. Only IgG demonstrated association with biopsy results (chi(2) = 20.4, P = 0.0001). An elevated IgG level (>1,482 mg/dl) had a high specificity (97% and 97%), high positive predictive value (PPV) (97% and 97%), but poor sensitivity (40% and 45%) in predicting positive biopsy results and SS, respectively. Elevated serum IgG levels best predicted a positive biopsy result and SS with high PPV and specificities.

  6. Primary Sjögren's syndrome: fatigue is an ever-present, fluctuating, and uncontrollable lack of energy.

    Science.gov (United States)

    Mengshoel, Anne Marit; Norheim, Katrine B; Omdal, Roald

    2014-08-01

    To examine how fatigue may differ from ordinary tiredness in patients with primary Sjögren's syndrome (SS). A purposive, heterogeneous sample of individuals with primary SS who had participated in a study that examined the effects of medication on fatigue was asked to participate in individual interviews. Patients were asked about their fatigue before and after the onset of illness, changes due to medications, and how fatigue was experienced in daily life. An inductive thematic analysis founded on a social constructionist perspective was performed. Five women and 4 men ages 27-76 years participated. Two themes were identified. "A heavy, resistant body and ever-present lack of vitality" represented a profound, ever-present lack of energy along with an unfamiliar bodily heaviness quite different from the experience after alleviation by the drug and being healthy. The patients had scaled down their everyday life in different ways to manage this lack of energy. "Unpredictable and uncontrollable fluctuations in fatigue" expressed how the level of fatigue fluctuated from day to day and even within a particular day. The informants tried to portion out their energy without knowing exactly how much energy they would have at any time. On bad days, patients felt that their "batteries were flat," and they had to put their life on hold. Fatigue in primary SS clearly differs from ordinary tiredness. Patients describe it as an ever-present, fluctuating, and nonrelievable lack of vitality being beyond one's own control. Copyright © 2014 by the American College of Rheumatology.

  7. Circulating immune complexes, immunoglobulin G, salivary proteins and salivary immunoglobulin A in patients with Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Hadži-Mihailović Miloš

    2009-01-01

    Full Text Available Introduction. Sjögren's syndrome (SS is a chronic autoimmune disorder, with its major clinical manifestations resulting from changes in exocrine glands. Objective The aim of this study was to evaluate serum concentrations of circulating immune complexes (CIC and immunoglobulin G (IgG, and salivary proteins (SP and salivary immunoglobulin A (sIgA in 40 patients with SS, and to correlate these values among themselves, as well as with the unstimulated salivary flow rate (USFR and the duration of disease. Methods. The total of 40 patients were included in this research. CIC was determined using the solution of polyethylene glycol and IgG with the standard procedure of radial immunodiffusion. SP was investigated by the method of Lowry and sIgA was separated from the whole saliva using the method of immune chromatography. Results. The values of most of the studied parameters exceeded the normal range in a high degree: CIC 72.5%, IgG 70%, SP 80%. The concentrations of CIC were significantly higher in the patients with the duration of disease less than 10 years. With the decrease of USFR, the concentration of sIgA and IgG were increased with statistical significance. Conclusion The increased prevalence of abnormal values of CIC, IgG and SP indicate that the patients with SS have developed a higher level of immune reactivity. These results could be useful in diagnosis and disease activity monitoring.

  8. Biopsy-proven renal involvement and prognosis in 13 hispanic patients with primary Sjögren syndrome.

    Science.gov (United States)

    Carrillo-Pérez, Diego Luis; Tejeda-Maldonado, Javier; Garza-García, Carlos; Soto-Abraham, Virgilia; Hernández-Molina, Gabriela; Molina-Paredes, Giovanni Arnoldo; Uribe-Uribe, Norma O; Morales-Buenrostro, Luis E

    2018-01-23

    The aim of this study was to describe a case series of 13 Hispanic patients with primary Sjögren syndrome (pSS) and biopsy-proven renal involvement. We describe the clinical, serological and histological characteristics as well as the prognosis in a group of patients with pSS and biopsy-proven renal involvement, treated in 2 referral nephrology units in Mexico City. Thirteen patients with pSS underwent kidney biopsy (KB) over a period of 27 years. The median duration from pSS diagnosis to KB was 13.9 months. Seven patients (54%) had glomerulonephritis and 6 patients (46%) had tubulointerstitial nephritis. All patients were treated with corticosteroids and/or immunosuppressants. Eight patients (62%) remained stable or their renal function improved after a median follow-up of 12 months. This case series reflects the broad spectrum of renal involvement in pSS. We observed that in our Hispanic population, glomerular involvement was the most frequent abnormality, mainly membranous glomerulopathy, followed by tubulointerstitial disease. Tubular atrophy and interstitial fibrosis were also common biopsy findings. Treatment with corticosteroids or other immunosuppressive agents appear to slow renal disease progression. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  9. A link between interferon and augmented plasmin generation in exocrine gland damage in Sjögren's syndrome.

    Science.gov (United States)

    Gliozzi, Maria; Greenwell-Wild, Teresa; Jin, Wenwen; Moutsopoulos, Niki M; Kapsogeorgou, Efstathia; Moutsopoulos, Haralampos M; Wahl, Sharon M

    2013-02-01

    Sjögren's syndrome is an autoimmune disease that targets exocrine glands, but often exhibits systemic manifestations. Infiltration of the salivary and lacrimal glands by lymphoid and myeloid cells orchestrates a perpetuating immune response leading to exocrine gland damage and dysfunction. Th1 and Th17 lymphocyte populations and their products recruit additional lymphocytes, including B cells, but also large numbers of macrophages, which accumulate with disease progression. In addition to cytokines, chemokines, chitinases, and lipid mediators, macrophages contribute to a proteolytic milieu, underlying tissue destruction, inappropriate repair, and compromised glandular functions. Among the proteases enhanced in this local environment are matrix metalloproteases (MMP) and plasmin, generated by plasminogen activation, dependent upon plasminogen activators, such as tissue plasminogen activator (tPA). Not previously associated with salivary gland pathology, our evidence implicates enhanced tPA in the context of inflamed salivary glands revolving around lymphocyte-mediated activation of macrophages. Tracking down the mechanism of macrophage plasmin activation, the cytokines IFNγ and to a lesser extent, IFNα, via Janus kinase (JAK) and signal transducer and activator of transcription (STAT) activation, were found to be pivotal for driving the plasmin cascade of proteolytic events culminating in perpetuation of the inflammation and tissue damage, and suggesting intervention strategies to blunt irreversible tissue destruction. Published by Elsevier Ltd.

  10. Manifestações de linfoma na síndrome de Sjögren: existe relação? Lymphoma's manifestations in Sjögren's syndrome: is there a relation?

    Directory of Open Access Journals (Sweden)

    Fabrícia Dias Colombano Limares

    2005-06-01

    Full Text Available A Síndrome de Sjögren (SS é considerada uma afecção multissistêmica, crônica, que se caracteriza pela infiltração linfocítica nas glândulas exócrinas e a produção de auto-anticorpos. OBJETIVO: Vários estudos têm notado um aumento na incidência de linfomas malignos em pacientes com SS. Em nosso estudo tentamos descrever esta relação. FORMA DE ESTUDO: Coorte transversal. MATERIAL E MÉTODO: Pacientes com Síndrome Sicca acompanhados no ambulatório de Estomatologia do Departamento de Otorrinolaringologia da Santa Casa de Misericórdia de São Paulo, no período de julho de 1999 a abril de 2002. RESULTADOS: Dos 39 pacientes, 24 foram classificados com SS. A idade variou de 19 a 83 anos, com predominância do sexo feminino (69,7%. O intervalo de tempo entre o início dos sintomas e o diagnóstico de SS variou de 3,77 anos. Não foi observado desenvolvimento de linfoma em nenhum dos pacientes avaliados. CONCLUSÃO: O diagnóstico de SS e o aumento do risco de desenvolvimento de Linfoma ao longo dos anos são importantes, então um longo período de seguimento destes pacientes é fundamental. Observamos que nossos achados foram diferentes quando comparados com a literatura. Nós não encontramos nenhum linfoma em nossos pacientes.Sjögren's Syndrome (SS is considered a multisystemic chronic disorder, which is characterized by a lymphocytic infiltration of exocrine glands and autoantibodies production. AIM: Several studies have demonstrated increased incidence of Lymphoma in SS patients. Our study tries to determine this relation. STUDY DESIGN: Transversal cohort. MATERIAL AND METHOD: Patients with Sicca's Syndrome from the Stomathology service, Santa Casa de Misericórdia Hospital, in Sao Paulo, ENT Department, from July 1999 to April 2002. RESULTS: Out of 39 patients, 24 were diagnose as SS. Ages ranged from 19 to 83 years old, with predominance of women (69.7%. Time between the first symptoms and SS diagnosis were variable

  11. Primary Sjögren syndrome: report of a 10 years old girl with local edema and positivity of anti SS-A and anti SS-B autoantibodies

    Directory of Open Access Journals (Sweden)

    V. Gerloni

    2011-09-01

    Full Text Available Sjögren-Larsson syndrome (SLS is an autoimmune disease, uncommon in childhood. We report a case of SLS in a 10-year-old girl with a history of tumor, calor and rubor in the back of her toes almost every month, which resolved in 4-5 days without therapy. She did not complain of dry mouth or dry eyes. The laboratory fi ndings showed high infl ammation markers, rheumatoid factor 128 IU, Waaler-Rose 256 IU, anti nuclear antibody (ANA 1/640, SSA (anti Sjögren antigen A and SSB (anti Sjögren antigen B positive and hypergammaglobulinemia. The Schirmer’s test resulted to be pathologic, the ultrasonography images and biopsy of minor salivary glands revealed focal periductal lymphocytic infi ltrate and sialoduct ectasia class IV of juvenile Sjögren syndrome. The juvenile Sjögren syndrome is frequently under-diagnosed. Clinical manifestations in children might be different from the adult form, although laboratory fi ndings may be similar to those found in adults.

  12. Clinical Efficacy and Safety of Total Glucosides of Paeony for Primary Sjögren’s Syndrome: A Systematic Review

    Directory of Open Access Journals (Sweden)

    Liang Jin

    2017-01-01

    Full Text Available Objective. To evaluate the clinical efficacy and safety of total glucosides of paeony (TGP for primary Sjögren’s syndrome (pSS. Methods. Eight electronic databases were searched from their inception to July 2016. Clinical randomized controlled trials (RCTs were included. The study quality was evaluated according to the standard suggested in the Cochrane Handbook. RevMan 5.1 was used for statistical analysis. Results. Seven RCTs involving 443 patients were included. The results showed that TGP combined with an immunosuppressant (IS showed greater efficacy for improving the saliva flow test of pSS compared to immunosuppressant alone (WMD −6.88, 95% CI −9.02 to −4.74, and P<0.00001. And the same trend favouring TGP-IS dual combination was found in Schirmer test (WMD 1.63, 95% CI 0.26 to 3.01, and P=0.02, ESR (WMD 7.33, 95% CI −10.08 to −4.59, and P<0.00001, CRP (WMD −6.00, 95% CI −7.17 to −4.83, and P<0.00001, IgM (WMD = −0.42, 95% CI −0.70 to 0.13, and P=0.004, and IgG (WMD −3.22, 95% CI −4.32 to −2.12, and P<0.00001 analysis. However, TGP did not affect IgA (WMD 0.53, 95% CI −1.34 to −0.29, and P=0.20. The adverse events manifested no significant differences between the two groups. Conclusions. The TGP-IS combination is superior to IS alone in the treatment of pSS. However, due to the low quality of included studies, high-quality RCTs are needed to confirm the beneficial effects of TGP.

  13. Anti-centromere antibody-seropositive Sjögren's syndrome differs from conventional subgroup in clinical and pathological study

    Directory of Open Access Journals (Sweden)

    Ida Hiroaki

    2010-07-01

    Full Text Available Abstract Background To clarify the clinicopathological characteristics of primary Sjögren's syndrome (pSS with anti-centromere antibody (ACA. Methods Characteristics of 14 patients of pSS with ACA were evaluated. All patients were anti-SS-A/Ro and SS-B/La antibodies negative (ACA+ group without sclerodactyly. The prevalence of Raynaud's phenomenon (RP, titer of IgG and focus score (FS in the minor salivary glands (MSGs were determined. Quantification analysis of Azan Mallory staining was performed to detect collagenous fiber. Forty eight patients in whom ACA was absent were chosen as the conventional (ACA- pSS group. Results Prevalence of ACA+ SS patients was 14 out of 129 (10.85% pSS patients. RP was observed in 61.5% of the patients with ACA. The level of IgG in the ACA+ group was significantly lower than that of the ACA- group (p = 0.018. Statistical difference was also found in the FS of MSGs from the ACA+ group (1.4 ± 1.0 as compared with the ACA- group (2.3 ± 1.6 (p = 0.035. In contrast, the amount of fibrous tissue was much higher in the ACA+ group (65052.2 ± 14520.6 μm2 versus 26251.3 ± 14249.8 μm2 (p = 1.3 × 10-12. Conclusions Low cellular infiltration but with an increase in fibrous tissues may explain the clinical feature of a high prevalence of RP and normal IgG concentration in ACA+ pSS.

  14. Low-level laser therapy for xerostomia in primary Sjögren's syndrome: a randomized trial.

    Science.gov (United States)

    Fidelix, Tania; Czapkowski, Adriano; Azjen, Sergio; Andriolo, Adagmar; Neto, Pedro Horvath; Trevisani, Virgínia

    2018-03-01

    To evaluate the effectiveness of low-level laser therapy (LLLT) in the treatment of xerostomia in primary Sjögren's syndrome (SS), a randomized clinical trial of patients with dry mouth symptoms associated with primary SS receiving care at a university hospital was conducted. Sixty-six patients were randomly assigned with a 1:1 allocation ratio to receive LLLT (laser group, n = 33) or placebo treatment (placebo group, n = 33). Patients in the laser group received LLLT twice a week for 6 weeks, for a total of 12 treatment sessions. Laser irradiation was performed with an aluminum-gallium-arsenide laser diode at a wavelength of 808 nm, 100-mW output power, and energy density of 4.0 J/cm 2 per irradiation point per session. Placebo treatment was performed following the same protocol used for irradiated patients and using the same laser device to mimic a real irradiation, but with no active laser emission and the tip of the laser probe covered with aluminum foil. The outcomes of interest were xerostomia inventory scores, salivary flow rate, salivary beta-2 microglobulin levels, and salivary sodium and chlorine concentrations. Patients in both groups showed no improvement in xerostomia. Likewise, there was no significant improvement in xerostomia inventory scores (p = 0.301) or salivary flow rate (p = 0.643) in either group. There was no difference in salivary beta-2 microglobulin levels, sodium concentration, and chlorine concentration before and after intervention or between the two groups. The LLLT protocol used in this study effected no improvement in xerostomia or salivary flow rate in patients with primary SS. ClinicalTrials.gov Identifier: NCT02066896.

  15. Diagnostic contribution of quantitative analysis of salivary scintigraphy in patients with suspected Sjögren's syndrome.

    Science.gov (United States)

    Infante, J R; García, L; Rayo, J I; Serrano, J; Domínguez, M L; Moreno, M

    2016-01-01

    The evaluation of the salivary scintigraphy is part of the classification criteria of Sjögren's syndrome (SS). The aim of the study was to determine a method of quantitative evaluation of this technique with easy application and high diagnostic accuracy. A review was carried out on a total of 111 patients with clinical suspicion of SS, referred to our department over the last 4 years (94 women, range 14-82 years). Thirty-minute dynamic studies were performed after injection of (99m)Tc-pertechnetate, with secretory stimulus at 15 minutes. After drawing regions of interest in both parotids, submandibular glands, and in the background, quantitative parameters were determined. These included the ejection fraction, uptake ratio at 15 min, and the percentage uptake (PC). Based on the definitive diagnosis, the subjects were classified into patients with SS, with sicca syndrome, and healthy subjects. Significant differences were found between the SS group and healthy subjects in the 3 quantitative parameters for the 4 glands. Significant differences in the PC parameter were observed between the group with sicca syndrome and healthy subjects. ROC analysis showed that the best differentiation parameter for the 3 groups was the PC in both parotid and submandibular glands. The quantitative analysis of salivary scintigraphy has proved to be a useful method and easy to apply in daily practice to differentiate patients with SS from healthy subjects, with the PC, both in parotid and submandibular glands, being the parameter with highest diagnostic accuracy. Copyright © 2015 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

  16. A qualitative exploration of physical, mental and ocular fatigue in patients with primary Sjögren's Syndrome.

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    Rebecca J Stack

    Full Text Available Primary Sjögren's Syndrome (pSS affects exocrine glands such as those producing the tear film, leading to dry and painful eyes, but is also associated with fatigue. The experience of fatigue in pSS, and its relationship with sicca symptoms, is poorly understood.Twenty people diagnosed with pSS were recruited to participate in a semi-structured qualitative interview about their symptoms experience. Interviews were audio-recorded, transcribed verbatim and analysed using thematic analysis.People with pSS described physical tiredness, mental fatigue and ocular fatigue. Mental fatigue was characterised by difficulties in attention, particularly, the ability to follow conversations and short-term memory problems. Participants linked their experience of fatigue to feeling of depression, frustration, irritation and anxiety, and therefore, fatigue was suggested to have had a large impact on their psychological well-being. People with pSS also described a range of ocular symptoms including pain, dryness, and itching, which were compounded by fatigue. For some, eye fatigue was pervasive, and daily activities involving the eyes such as reading, using the computer and driving were impaired. In some cases, the level of ocular discomfort was so severe it prevented sleep, which in turn impacted on general fatigue levels.People with pSS experience fatigue in a range of ways; physical, mental and ocular fatigue were described. Fatigue was suggested to exacerbate other ocular symptoms, posed serious physical limitations and caused psychological distress. Further research into the nature of fatigue and ocular symptoms in pSS is required.

  17. [Liposomal local therapy as treatment for sicca symptoms in patients with primary Sjögren's syndrome].

    Science.gov (United States)

    Hofauer, B; Bas, M; Manour, N; Knopf, A

    2013-11-01

    Primary Sjögren's syndrome (pSS) represents the most common rheumatologic disease in the head and neck area. Due to various nonspecific symptoms, many different medical specialities are involved in diagnostic and therapeutic regimes of patients with pSS. Therefore broad knowledge of symptoms and therapeutic options is important. Effective management of xerostomia, keratoconjunctivis sicca (KCS), and rhinitis sicca (RS) is essential in every patient to alleviate symptoms and prevent local complications. Various substitutes with different ingredients are available for topical and symptomatic therapy, but there are no generally accepted treatment suggestions due to the lack of studies proving effective relief of symptoms. Between October 2007 and August 2012, pSS was diagnosed in 73 patients according to the American European Consensus Group (AECG) criteria. Before and after a 2-month period of treatment with liposomal agents for oral, nasal, and ocular complaints (LipoSaliva®, LipoNasal®, and Tears Again®,) AECG criteria and subjective symptoms were evaluated using visual analogue scales. Xerostomia (92 %), KCS (92 %), and recurrent salivary gland swelling (40 %) were the leading clinical symptoms. Mean salivary flow rate was 1.89 g (Saxon test), mean lacrimal flow 12.7 mm (Schirmer I test). SS-A and -B antibodies could be detected in 47 patients (29 %). An average histologic focus score of 3 was assigned (Chisholm and Mason). Extraglandular manifestations were observed in 17 %, while severe local complications due to ineffective treatment of sicca symptoms were diagnosed in 70.5 %. Liposomal local therapy caused a significant reduction of xerostomia (p = 0.0001), KCS (p = 0.004), and RS (p = 0.004). Local therapy with liposomal agents is effective in the symptomatic treatment of xerostomia, KCS, and RS in patients with pSS.

  18. Association between primary Sjögren's syndrome, cardiovascular and cerebrovascular disease: a systematic review and meta-analysis.

    Science.gov (United States)

    Yong, Wai Chung; Sanguankeo, Anawin; Upala, Sikarin

    2018-03-19

    Acute systemic inflammation and chronic systemic vasculitis are associated with endothelial dysfunction and atherosclerotic plaque formation. Studies on cardiovascular or cerebrovascular events in primary Sjögren's syndrome (pSS) are limited, with conflicting results. This meta-analysis aimed to explore the risk of cardiovascular and cerebrovascular disease in pSS. A comprehensive search of the MEDLINE and EMBASE databases was performed from date of inception through August 2017. The inclusion criterion was observational studies evaluating the association between pSS and cardiovascular disease or cerebrovascular event. Outcomes are diagnosis of ischaemic heart disease, myocardial infarction, ischaemic stroke or haemorrhagic stroke. The pooled odds ratio (OR) of the cerebrovascular event or cardiovascular disease and their 95% confidence interval (CI) were calculated using a random-effect meta-analysis to compare risk between patients with pSS and controls. The between-study heterogeneity of effect-size was quantified using the Q statistic and I2. Data were extracted from 10 observational studies involving 165,291 subjects. Pooled result demonstrated a significant increase in risk of having cardiovascular disease or cerebrovascular event in pSS patients compared with controls (OR=1.28; 95% CI: 0.11-1.46, p valuecerebrovascular event (OR=1.31; 95% CI: 0.96-1.79, p value=0.09, I2=71%), but an increased risk of cardiovascular disease (OR=1.30; 95% CI: 1.09-1.55, p value=0.003, I2=74%). Our study has shown an increased risk of cardiovascular or cerebrovascular disease in patients with pSS. These results support multiple studies' finding of increased arterial stiffness in patients with pSS.

  19. Olho seco e Sjögren secundário na artrite reumatóide

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    Marcelo Luiz Gehlen

    2012-02-01

    Full Text Available OBJETIVO: Examinar a prevalência de ceratoconjuntivite seca e de Síndrome de Sjögren (SS secundária em pacientes com AR do sul do Brasil e analisar suas relações com duração e atividade da AR e classe funcional do paciente. Pacientes com artrite reumatóide (AR podem ter ceratoconjuntivite seca, que é considerada uma manifestação extra-articular dessa doença. MÉTODOS: Estudaram-se 82 pacientes com AR para sintomas de secura, Schirmer teste, DAS-28 ( índice de atividade da AR e classe funcional. RESULTADOS: Sintomas de secura ocular estavam presentes em 57,3%, Schirmer positivo em 41,2% e SS secundária em 24.3%. A presença de um teste positivo para o Schirmer ou da SS secundária não teve associação com duração de doença (p=0,65 and 0,80, atividade da AR (p=0,42 and 0,25 nem com a classe funcional do paciente (p= 0.84 and 0,79. CONCLUSÃO: Existe uma alta prevalência de síndrome sicca nos pacientes com AR; ¼ deles tem SS secundário. O aparecimento de SS secundário e da síndrome sicca é independentes de atividade, tempo de duração e do dano cumulativo gerado pela AR subjacente .

  20. Glutathione protects against hepatic injury in a murine model of primary Sjögren’s syndrome

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    Shuhua Jiang

    2016-08-01

    Full Text Available Primary Sjögren’s syndrome (pSS is a systemic autoimmune disease which may cause complications such as hepatic dysfunction and injury. As an important antioxidant, reduced glutathione (GSH has been reported protecting against hepatic injury induced by some diseases, but the role of GSH in pSS is poorly understood. This study aims at investigating the role of GSH in hepatic injury during pSS. A murine model of pSS, non-obese diabetic (NOD mice, was used for GSH administration via tail intravenous injection. Enzyme-linked immunosorbent assay (ELISA was performed to detect serum levels of aspartate aminotransferase (AST and alanine aminotransferase (ALT, as well as the levels of GSH, tumor necrosis factor, interleukin (IL 10, integrin alpha M, IL1B, malondialdehyde, nicotinamide adenine dinucleotide phosphate oxidase 4, and superoxide dismutases in hepatocyte homogenates. Hematoxylin-eosin staining was performed to observe hepatic histology. The results showed that serum AST and ALT levels were up-regulated in the NOD mice (p = 0.0021 and 0.0048, but were significantly recovered after the GSH administration (p = 0.0081 and 0.0263. The NOD mice exhibited disturbed hepatic tissue structure, which was attenuated by GSH. The GSH administration could also promote the production of GSH in the hepatocytes (p = 0.0264, and control the levels of inflammatory factors and oxidative stress-related factors. These results indicate that GSH has significant effects on protecting against the hepatic injury during pSS, which may be associated with its regulation of the inflammatory factors and oxidative stress-related factors. This study suggests that GSH is a promising therapeutic strategy for controlling hepatic injury during pSS and offers valuable information for further research.

  1. Glutathione protects against hepatic injury in a murine model of primary Sjögren’s syndrome

    Science.gov (United States)

    Jiang, Shuhua; Hu, Liwei; Ping, Lifeng; Sun, Fengyan; Wang, Xiaolei

    2016-01-01

    Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease which may cause complications such as hepatic dysfunction and injury. As an important antioxidant, reduced glutathione (GSH) has been reported protecting against hepatic injury induced by some diseases, but the role of GSH in pSS is poorly understood. This study aims at investigating the role of GSH in hepatic injury during pSS. A murine model of pSS, non-obese diabetic (NOD) mice, was used for GSH administration via tail intravenous injection. Enzyme-linked immunosorbent assay (ELISA) was performed to detect serum levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT), as well as the levels of GSH, tumor necrosis factor, interleukin (IL) 10, integrin alpha M, IL1B, malondialdehyde, nicotinamide adenine dinucleotide phosphate oxidase 4, and superoxide dismutases in hepatocyte homogenates. Hematoxylin-eosin staining was performed to observe hepatic histology. The results showed that serum AST and ALT levels were up-regulated in the NOD mice (p = 0.0021 and 0.0048), but were significantly recovered after the GSH administration (p = 0.0081 and 0.0263). The NOD mice exhibited disturbed hepatic tissue structure, which was attenuated by GSH. The GSH administration could also promote the production of GSH in the hepatocytes (p = 0.0264), and control the levels of inflammatory factors and oxidative stress-related factors. These results indicate that GSH has significant effects on protecting against the hepatic injury during pSS, which may be associated with its regulation of the inflammatory factors and oxidative stress-related factors. This study suggests that GSH is a promising therapeutic strategy for controlling hepatic injury during pSS and offers valuable information for further research. PMID:27186973

  2. Biochemical deficiency of pyridoxine does not affect interleukin-2 production of lymphocytes from patients with Sjögren's syndrome.

    Science.gov (United States)

    Tovar, A R; Gómez, E; Bourges, H; Ortíz, V; Kraus, A; Torres, N

    2002-11-01

    There is evidence that pyridoxine deficiency may alter the immune response. It is not known whether a deficiency of this vitamin is evident in subjects with primary Sjögren's syndrome (SS). We studied whether subjects with primary SS showed a biochemical deficiency of pyridoxine, and if it is associated with abnormal production of interleukin-2 from lymphocytes stimulated in vitro with phytohemagglutinin (PHA). Two studies were conducted, (i) biochemical and nutritional assessments were performed in a cross-over study in subjects with primary SS, who were supplemented with 25 mg/day of pyridoxine or placebo for 3 months. After 1 month washout, they were supplemented for 3 months with placebo, (ii) patients with SS and matched controls received pyridoxine or placebo for 45 days, and a blood sample was obtained to study IL-2 production and expression in T-lymphocytes stimulated with PHA. Subjects with primary SS showed limited dietary intake of pyridoxine and biochemical deficiency of this vitamin assessed through the activation coefficient of the erythrocyte aspartate aminotransferase. The biochemical deficiency did not affect production nor mRNA expression of IL-2 from T-lymphocytes stimulated in vitro with PHA compared with the control group. Supplementation of subjects with primary SS with 25 mg/day with pyridoxine for 45 days did not produce any significant change as compared to those patients supplemented with placebo. Subjects with primary SS showed biochemical deficiency of pyridoxine, possibly due to limited intake of this vitamin which was corrected by supplementation with pyridoxine. However, IL-2 production and mRNA expression from stimulated lymphocytes were unaffected by supplementation, probably because the deficiency was not severe enough to affect the immune system. This work was supported by the National Council of Science and Technology (CONACYT), Mexico, grant no. 212226-5-0902PM.

  3. Aire-deficient mice provide a model of corneal and lacrimal gland neuropathy in Sjögren's syndrome.

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    Feeling Y Chen

    Full Text Available Sjögren's syndrome (SS is a chronic, autoimmune exocrinopathy that leads to severe dryness of the mouth and eyes. Exocrine function is highly regulated by neuronal mechanisms but little is known about the link between chronic inflammation, innervation and altered exocrine function in the diseased eyes and exocrine glands of SS patients. To gain a better understanding of neuronal regulation in the immunopathogenesis of autoimmune exocrinopathy, we profiled a mouse model of spontaneous, autoimmune exocrinopathy that possess key characteristics of peripheral neuropathy experienced by SS patients. Mice deficient in the autoimmune regulator (Aire gene developed spontaneous, CD4+ T cell-mediated exocrinopathy and aqueous-deficient dry eye that were associated with loss of nerves innervating the cornea and lacrimal gland. Changes in innervation and tear secretion were accompanied by increased proliferation of corneal epithelial basal cells, limbal expansion of KRT19-positive progenitor cells, increased vascularization of the peripheral cornea and reduced nerve function in the lacrimal gland. In addition, we found extensive loss of MIST1+ secretory acinar cells in the Aire -/- lacrimal gland suggesting that acinar cells are a primary target of the disease, Finally, topical application of ophthalmic steroid effectively restored corneal innervation in Aire -/- mice thereby functionally linking nerve loss with local inflammation in the aqueous-deficient dry eye. These data provide important insight regarding the relationship between chronic inflammation and neuropathic changes in autoimmune-mediated dry eye. Peripheral neuropathies characteristic of SS appear to be tightly linked with the underlying immunopathological mechanism and Aire -/- mice provide an excellent tool to explore the interplay between SS-associated immunopathology and peripheral neuropathy.

  4. Aire-deficient mice provide a model of corneal and lacrimal gland neuropathy in Sjögren's syndrome.

    Science.gov (United States)

    Chen, Feeling Y; Lee, Albert; Ge, Shaokui; Nathan, Sara; Knox, Sarah M; McNamara, Nancy A

    2017-01-01

    Sjögren's syndrome (SS) is a chronic, autoimmune exocrinopathy that leads to severe dryness of the mouth and eyes. Exocrine function is highly regulated by neuronal mechanisms but little is known about the link between chronic inflammation, innervation and altered exocrine function in the diseased eyes and exocrine glands of SS patients. To gain a better understanding of neuronal regulation in the immunopathogenesis of autoimmune exocrinopathy, we profiled a mouse model of spontaneous, autoimmune exocrinopathy that possess key characteristics of peripheral neuropathy experienced by SS patients. Mice deficient in the autoimmune regulator (Aire) gene developed spontaneous, CD4+ T cell-mediated exocrinopathy and aqueous-deficient dry eye that were associated with loss of nerves innervating the cornea and lacrimal gland. Changes in innervation and tear secretion were accompanied by increased proliferation of corneal epithelial basal cells, limbal expansion of KRT19-positive progenitor cells, increased vascularization of the peripheral cornea and reduced nerve function in the lacrimal gland. In addition, we found extensive loss of MIST1+ secretory acinar cells in the Aire -/- lacrimal gland suggesting that acinar cells are a primary target of the disease, Finally, topical application of ophthalmic steroid effectively restored corneal innervation in Aire -/- mice thereby functionally linking nerve loss with local inflammation in the aqueous-deficient dry eye. These data provide important insight regarding the relationship between chronic inflammation and neuropathic changes in autoimmune-mediated dry eye. Peripheral neuropathies characteristic of SS appear to be tightly linked with the underlying immunopathological mechanism and Aire -/- mice provide an excellent tool to explore the interplay between SS-associated immunopathology and peripheral neuropathy.

  5. Condição oral dos pacientes com síndrome de Sjögren: uma revisão sistemática = Oral conditions in patients with Sjögren’s syndrome: a systematic review

    Directory of Open Access Journals (Sweden)

    Lima Junior, José Ferreira

    2005-01-01

    Full Text Available Introdução: A Síndrome de Sjögren é uma doença auto-imune crônica, na qual o sistema imune do paciente ataca as glândulas salivares e lacrimais. No meio ambiente oral, ela provoca uma série de alterações dentre as quais se sobressai a xerostomia. Objetivo: Este estudo teve por objetivo investigar as manifestações orais da Síndrome de Sjögren e suas conseqüências na estrutura dentária. Materiais e métodos: Foram utilizados como fonte de pesquisa consultas manuais em periódicos especializados da área, sites e nas bases de dados eletrônicas MEDLINE, LILACS e BBO. Foram incluídos estudos transversais, caso-controle e coorte com avaliação clínica realizada através dos índices de cárie dentária e de doença periodontal. Os idiomas selecionados foram português, inglês e espanhol, considerando o período de 1990 a 2003. As estratégias de busca foram realizadas utilizando-se as seguintes palavras: sjögren, dmf, carie, decay, periodontal, plaque, gingivite. Resultados: No total, 78 estudos foram encontrados, sendo que destes, apenas 10 foram selecionados (todos da MEDLINE por estarem de acordo com aos critérios de inclusão. Conclusões: Os estudos analisados demonstram que o principal sintoma da síndrome de Sjögren é a xerostomia e que existe uma fraca relação entre os pacientes sindrômicos e os índices de cárie e nenhuma relação com a doença periodontal

  6. Growth Arrest-Specific 6 Protein in Patients with Sjögren Syndrome: Determination of the Plasma Level and Expression in the Labial Salivary Gland.

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    Chen-Hung Chen

    Full Text Available Growth arrest-specific protein 6 (Gas6 is a vitamin K-dependent protein expressed by endothelial cells and leukocytes that are involved in cell survival, migration, and proliferation in response to inflammatory processes. The aim of this study was to assess the implications of Gas6 in Sjögren syndrome (SS and its expression in the labial salivary gland.A total of 254 adults, including 159 with primary Sjögren syndrome (pSS, 34 with secondary Sjögren syndrome (sSS, and 61 normal controls, were recruited. Plasma Gas6 concentrations were determined, and Gas6 expressions in labial salivary gland (LSG tissues from controls and pSS and sSS patients were also evaluated. Plasma Gas6 concentrations were significantly lower among patients with pSS than normal controls (13.5 ± 8.6 vs. 19.9 ± 13.4 ng/ml, p < 0.001. There were, however, no significant differences in plasma Gas6 levels between pSS and sSS patients (13.5 ± 8.6 vs. 16.9 ± 11.2 ng/ml, p = 0.068. In multivariate logistic regression analysis, after adjustment for white blood cell count, hemoglobin level, platelet count, lymphocyte count, and C3 and C4 levels, lower plasma Gas6 concentrations were significantly associated with an increased risk of SS. Moreover, by using a semi-quantitative scale to evaluate Gas6 expression in LSG tissues, Gas6 expression was found to be markedly lower in LSG tissues from pSS patients than in tissues from normal controls.Decreased plasma Gas6 concentration and LSG expression were associated with pSS. As such, Gas6 may represent a novel independent risk factor for pSS, with a potential role in salivary gland inflammation and dysfunction.

  7. Effects of subconjunctival administration of anti-high mobility group box 1 on dry eye in a mouse model of Sjӧgren's syndrome.

    Science.gov (United States)

    Kim, Kyeong Hwan; Kim, Dong Hyun; Jeong, Hyun Jeong; Ryu, Jin Suk; Kim, Yu Jeong; Oh, Joo Youn; Kim, Mee Kum; Wee, Won Ryang

    2017-01-01

    Extracellular high mobility group box 1 (HMGB1) acts as a damage associated molecular pattern molecule through the Toll-like receptor to promote autoreactive B cell activation, which may be involved in the pathogenesis of Sjӧgren's syndrome. The aim of this study was to investigate the effect of subconjunctival administration of anti-HMGB1 on dry eye in a mouse model of Sjӧgren's syndrome. Ten weeks-old NOD.B10.H2b mice were subconjunctivally injected with 0.02 to 2 μg of anti-HMGB1 antibodies or PBS twice a week for two consecutive weeks. Tear volume and corneal staining scores were measured and compared between before- and after-treatment. Goblet cell density was counted in PAS stained forniceal conjunctiva and inflammatory foci score (>50 cells/focus) was measured in extraorbital glands. Flow cytometry was performed to evaluate the changes in BrdU+ cells, IL-17-, IL-10-, or IFNγ-secreting cells, functional B cells, and IL-22 secreting innate lymphoid cells (ILC3s) in cervical lymph nodes. The level of IL-22 in intraorbital glands was measured by ELISA. Injection of 2 μg or 0.02 μg anti-HMGB1 attenuated corneal epithelial erosions and increased tear secretion (peye. The improvement of dry eye may involve an increase of ILC3s, rather than modulation of B or plasma cells, as shown using a mouse model of Sjӧgren's syndrome.

  8. Sjögren's syndrome associated dry eye in a mouse model is ameliorated by topical application of integrin α4 antagonist GW559090.

    Science.gov (United States)

    Contreras-Ruiz, Laura; Mir, Fayaz A; Turpie, Bruce; Krauss, Achim H; Masli, Sharmila

    2016-02-01

    Sjögren's syndrome is an autoimmune disease associated with inflammation of exocrine glands with clinical manifestations of dry eye and dry mouth. Dry eye in this disease involves inflammation of the ocular surface tissues - cornea and conjunctiva. While systemic blockade of adhesion molecules has been used to treat autoimmune diseases, the purpose of this study was to determine the therapeutic efficacy of topical application of an integrin α4 adhesion molecule antagonist in a mouse model of dry eye associated with Sjögren's syndrome. To assess this spontaneously developed ocular surface inflammation related to Sjögren's syndrome in TSP-1null mice (12 wks) was evaluated. Mice were treated with topical formulations containing 0.1% dexamethasone or 30 mg/ml GW559090 or vehicle control. Corneal fluorescein staining and conjunctival goblet cell density were assessed. Real-time PCR analysis was performed to assess expression of the inflammatory marker IL-1β in the cornea and Tbet and RORγt in the draining lymph nodes. Ocular surface inflammation was detectable in TSP-1null mice (≥12 wk old), which resulted in increased corneal fluorescein staining indicative of corneal barrier disruption and reduced conjunctival goblet cell density. These changes were accompanied by increased corneal expression of IL-1β as compared to WT controls and an altered balance of Th1 (Tbet) and Th17 (RORγt) markers in the draining lymph nodes. Topically applied dexamethasone and GW559090 significantly reduced corneal fluorescein staining compared to vehicle treatment (p = 0.023 and p syndrome. Copyright © 2015 Elsevier Ltd. All rights reserved.

  9. Oral delivery of the Sj23LHD-GST antigen by Salmonella typhimurium type III secretion system protects against Schistosoma japonicum infection in mice.

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    Guo Chen

    2011-09-01

    Full Text Available BACKGROUND: Schistosomiasis japonica is a zoonotic parasitic disease and oral vaccine delivery system would be benefit for prevention of this disease. Although attenuated salmonella has been used as an antigen expression vector for oral vaccine development, the membrane-bound vacuoles in which bacteria reside hinders the presentation of expressed heterologous antigens to the major histocompatibility complex (MHC molecules. The present work used an attenuated Salmonella typhimurium strain VNP20009 to secretory expression of Sj23LHDGST bivalent antigen from Schistosoma japonicum and tested the protective efficacy against S. japonicum infection in orally immunized mice. METHODOLOGY/PRINCIPAL FINDINGS: Promoters (nirB or pagC were used to express the antigen (Sj23LHDGST and the Salmonella type III or α-hemolysin secretion system was employed to secrete it. The immunoblotting analysis and fluorescent microscopy revealed that the antigen was effectively expressed and delivered to the cytosol of macrophages in vitro. Among recombinant vaccine strains, an engineered VNP20009 which expressed the antigen by nirB promoter and secreted it through type III secretion system (nirB-sopE(1-104-Sj23LHD-GST efficiently protected against S. japonicum infection in a mouse model. This strain elicited a predominantly IgG(2a antibody response and a markedly increase in the production of IL-12 and IFN-γ. The flow cytometric analysis demonstrated that this strain caused T cell activation as evidenced by significantly increased expression of CD44 and CD69. CONCLUSION/SIGNIFICANCE: Oral delivery of antigen by nirB-driven Salmonella typhimurium type III secretion system is a novel, safe, inexpensive, efficient and convenient approach for schistosome vaccine development.

  10. Vínculos entre teatro y cine en La señorita Julia. De August Strindberg a Alf Sjöberg y Liv Ullmann

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    Daniela Oulego

    2016-03-01

    Full Text Available El propósito del presente artículo es abordar comparativamente el texto dramático La señorita Julia, escrito por el dramaturgo August Strindberg, con las versiones fílmicas dirigidas por los cineastas Alf Sjöberg y Liv Ullmann. Si bien la obra responde al naturalismo, consideramos que Strindberg debatió con el carácter determinista de la herencia. En este sentido, elementos de la mitología cristiana, como el festejo de la Noche de San Juan, y la presencia de lo dionisíaco formarán parte del análisis. Asimismo, las divisiones jerárquicas ancestrales entre los sexos, por un lado, y amos y esclavos, por otro, serán vinculadas con la religión desde una mirada crítica del cristianismo. En su película Sjöberg se distanció de Strindberg acentuando en el factor determinista al indagar en la prehistoria de los personajes mediante el recurso técnico del flashback y apelando a metáforas audiovisuales en consonancia con el cine sueco experimental de su tiempo. Del mismo modo, Liv Ullmann, continuó examinando la influencia del determinismo y profundizó la construcción de los personajes desde la animalización planteada por Sjöberg. Por último, las referencias metateatrales del texto original retomadas en esta transposición permitirán enriquecer el estudio interdisciplinario entre teatro y cine.

  11. Musikern som varumärke : En kvalitativ studie om hur frilansande folkmusiker   lanserar sig själva

    OpenAIRE

    Lindblom, Hanna

    2010-01-01

    Denna uppsats syftar till att få en inblick i hur frilansande folkmusiker i Sverige gör för att lansera sig själva som artister, vilka redskap som används samt vilken funktion dessa redskap fyller. Detta har undersökts genom kvalitativa intervjuer med fyra frilansmusiker inom den folkmusikaliska genren. Musikbranschens förändrade förutsättningar, ställda i relation till Lundberg, Malm & Ronströms (2000) begrepp görare, vetare och makare, utgör de teoretiska utgångspunkterna för uppsatsen....

  12. Avaliação dos fatores tradicionais e não tradicionais de risco cardiovascular na síndrome de Sjögren.

    OpenAIRE

    BALARINI, G. M.

    2015-01-01

    Pacientes com a Síndrome de Sjögren primária (SSp) apresentam maior prevalência de alguns fatores tradicionais de risco, mas relação entre a placa de aterosclerose carotídea e fatores tradicionais e não tradicionais de risco é pouco estudada. Identifica-se a associação da placa de aterosclerose carotídea com os fatores de risco tradicionais, características da doença, perfil de citocinas e calprotectina em pacientes com a SSp. Utiliza-se o estudo de caso-controle com 63 pacientes com a SSp...

  13. Manejo clínico odontológico integral del paciente con síndrome de Sjögren: una propuesta

    OpenAIRE

    G. Sturla Rojas; F. Romo Ormazábal; M.A. Torres-Quintana

    2014-01-01

    El síndrome de Sjögren (SS) es una enfermedad autoinmune, crónica e inflamatoria caracterizada por infiltración de células plasmáticas y linfocitos en las glándulas exocrinas, particularmente en las salivales y oculares. La patogénesis del SS está relacionada con factores inmunológicos, neurológicos, genéticos, virales y hormonales. La deficiente calidad y cantidad de saliva trae consecuencias devastadoras para la salud dental y bucal, alteraciones del esmalte, caries en las superficies denta...

  14. The Potential Role for Early Biomarker Testing as Part of a Modern, Multidisciplinary Approach to Sjögren's Syndrome Diagnosis.

    Science.gov (United States)

    Beckman, Kenneth A; Luchs, Jodi; Milner, Mark S; Ambrus, Julian L

    2017-04-01

    Sjögren's syndrome (SS) is a chronic and progressive multisystem autoimmune disease typically managed by rheumatologists. Diagnostic delays are common, due in large part to the non-specific and variable nature of SS symptoms and the slow progression of disease. The hallmark characteristics of SS are dry eye and dry mouth, but there are a broad range of other possible symptoms such as joint and muscle pain, skin rashes, chronic dry cough, vaginal dryness, extremity numbness or tingling, and disabling fatigue. Given that dry eye and dry mouth are typically the earliest presenting complaints, eye care clinicians and dental professionals are often the first point of medical contact and can provide critical collaboration with rheumatologists to facilitate both timely diagnosis and ongoing care of patients with SS. Current diagnostic criteria advocated by the American College of Rheumatology are predicated on the presence of signs/symptoms suggestive of SS along with at least two objective factors such as traditional biomarker positivity, salivary gland biopsy findings, and/or presence of keratoconjunctivitis sicca. Traditional biomarkers for SS include the autoantibodies anti-Sjögren's syndrome-related antigen A (SS-A/Ro), anti-Sjögren's syndrome-related antigen B (SS-B/La), antinuclear antibody (ANA) titers, and rheumatoid factor (RF). While diagnostically useful, these biomarkers have low specificity for SS and are not always positive, especially in early cases of SS. Several newly-identified biomarkers for SS include autoantibodies to proteins specific to the salivary and lacrimal glands [SP-1 (salivary gland protein-1), PSP (parotid secretory protein), CA-6 (carbonic anhydrase VI)]. Data suggest that these novel biomarkers may appear earlier in the course of disease and are often identified in cases that test negative to traditional biomarkers. The Sjö ® test is a commercially available diagnostic panel that incorporates testing for traditional SS biomarkers

  15. Long-term follow-up in primary Sjögren's syndrome reveals differences in clinical presentation between female and male patients

    DEFF Research Database (Denmark)

    Ramírez Sepúlveda, Jorge I; Kvarnström, Marika; Eriksson, Per

    2017-01-01

    BACKGROUND: Despite men being less prone to develop autoimmune diseases, male sex has been associated with a more severe disease course in several systemic autoimmune diseases. In the present study, we aimed to investigate differences in the clinical presentation of primary Sjögren's syndrome (p...... (ns). Clinical data including serological and hematological parameters and glandular and extraglandular manifestations were compared between men and women. RESULTS: Male patient serology was characterized by more frequent positivity for anti-Ro/SSA and anti-La/SSB (p = 0.02), and ANA (p = 0...

  16. Endogenous programmed death ligand-1 restrains the development and onset of Sjӧgren’s syndrome in non-obese diabetic mice

    OpenAIRE

    Jing Zhou; Jun-O. Jin; Toshihisa Kawai; Qing Yu

    2016-01-01

    Programmed death-ligand 1 (PD-L1) down-modulates various immune responses by engaging the co-inhibitory receptor programmed death-1. Expression of PD-L1 and programmed death-1 is elevated in the salivary glands of patients with Sj?gren?s syndrome (SS). The objective of this study is to define the role of endogenous PD-L1 in SS pathogenesis in non-obese diabetic (NOD) mouse model of this disease. We inhibited endogenous PD-L1 function by intraperitoneal administration of a blocking antibody to...

  17. Efficacy and safety of rituximab treatment in early primary Sjögren's syndrome: a prospective, multi-center, follow-up study

    OpenAIRE

    Carubbi, Francesco; Cipriani, Paola; Marrelli, Alessandra; Benedetto, Paola Di; Ruscitti, Piero; Berardicurti, Onorina; Pantano, Ilenia; Liakouli, Vasiliki; Alvaro, Saverio; Alunno, Alessia; Manzo, Antonio; Ciccia, Francesco; Gerli, Roberto; Triolo, Giovanni; Giacomelli, Roberto

    2013-01-01

    Introduction Primary Sjögren’s syndrome (pSS) is an autoimmune disorder affecting exocrine glands; however, a subgroup of pSS patients experience systemic extra-glandular involvement leading to a worsening of disease prognosis. Current therapeutic options are mainly empiric and often translated by other autoimmune diseases. In the last few years growing evidence suggests that B-cell depletion by rituximab (RTX) is effective also in pSS. Patients with early active disease appear to be those wh...

  18. EPS-SJ exopolisaccharide produced by the strain Lactobacillus paracasei subsp. paracasei BGSJ2-8 is involved in adhesion to epithelial intestinal cells and decrease on E. coli association to Caco-2 cells

    Directory of Open Access Journals (Sweden)

    Milica eZivkovic

    2016-03-01

    Full Text Available The aim of this study was to determine the role of an exopolysaccharide produced by natural dairy isolate Lactobacillus paracasei subsp. paracasei BGSJ2-8, in the adhesion to intestinal epithelial cells and a decrease in E. coli’s association with Caco-2 cells. Annotation of the BGSJ2-8 genome showed the presence of a gene cluster, epsSJ, which encodes the biosynthesis of the strain-specific exopolysaccharide EPS-SJ, detected as two fractions (P1 and P2 by size exclusion chromatography (SEC coupled with multi-angle laser light scattering (MALLS detection. SEC-MALLS analysis revealed that an EPS-SJ‒ mutant (EPS7, obtained by insertion mutagenesis of the glps_2198 gene encoding primary glycosyltransferase does not produce the P2 fraction of EPS-SJ. Transmission electron microscopy showed that EPS7 mutant has a thinner cell wall compared to the EPS-SJ+ strain BGSJ2-83 (a plasmid free-derivative of BGSJ2-8. Interestingly, strain BGSJ2-83 showed higher adhesion to Caco-2 epithelial intestinal cell line than the EPS7 mutant. Accordingly, BGSJ2-83 effectively reduced E. coli ATCC25922’s association with Caco-2 cells, while EPS7 did not show statistically significant differences. In addition, the effect of EPS-SJ on the proliferation of lymphocytes in gastrointestinal associated lymphoid tissue (GALT was tested and the results showed that the reduction of GALT lymphocyte proliferation was higher by BGSJ2-83 than by the mutant. To the best of our knowledge this is the first report indicating that the presence of EPS (EPS-SJ on the surface of lactobacilli can improve communication between bacteria and intestinal epithelium, implying its possible role in gut colonization.

  19. Antischistosomal activity of acridanone- hydrazones in Cebus monkeys experimentally infected with the SJ strain of Schistosoma mansoni

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    Paulo Marcos Zech Coelho

    1995-09-01

    Full Text Available In this study, four compounds were utilized at the dose of 12.5mg/kg body weight, p.o., to treat Cebus monkeys experimentally infected with about 200 cercariae of Schistosoma mansoni (SJ strain, via transcutaneous route. The oograms performed with rectal snips, as well as stool examinations carried out periodically, showed no viable eggs of the parasite, from day 29 to 226post-treatment. The perfusion undertaken after killing the animals showed absence of worms in the treated monkeys, whereas 83 worms were recovered from the control, thus corroborating the results obtained by means of oograms and coproscopy. These results confirm the efficacy of 9-acridanone- hydrazones previously tested against the LE strain of S. mansoni. The low curative dose and apparent absence of toxicity render these dmgs an important therapeutic reserve, taking into consideration the reports on the resistance of S. mansoni to the modern drugs oxamniquine and praziquantel.No presente trabalho, quatro compostos foram utilizados na dose de 12,5mg/kg de peso, por via oral, em macacos infectados transcutaneamente com cerca de 200 cercárias de Schistosoma mansoni. Os oogramas realizados com fragmentos de mucosa retal e os exames de fezes realizados, periodicamente, demonstraram a ausência de ovos viáveis do parasito a partir do 29- até o 226a dia pós-tratamento. A perfusão, apôs sacrifício dos animais tratados, não detectou vermes, enquanto que do macaco cotztrole 83 vermes foram recuperados, confirmando assim os resultados dos oogramas e da coproscopia. Estes resultados confirmam a eficácia das 9-acridanonas- hydrazonas já observada anteriormente contra a cepa LE de S. mansoni. A baixa dosagem curativa e aparente ausência de toxicidade colocam estas drogas como uma reserva terapêutica importante, tendo em vista o relato de resistência do S. mansoni às drogas modernas oxamniquína e praziquantel.

  20. Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjögren’s syndrome

    Science.gov (United States)

    Lessard, Christopher J.; Li, He; Adrianto, Indra; Ice, John A.; Rasmussen, Astrid; Grundahl, Kiely M.; Kelly, Jennifer A.; Dozmorov, Mikhail G.; Miceli-Richard, Corinne; Bowman, Simon; Lester, Sue; Eriksson, Per; Eloranta, Maija-Leena; Brun, Johan G.; Gøransson, Lasse G.; Harboe, Erna; Guthridge, Joel M.; Kaufman, Kenneth M.; Kvarnström, Marika; Jazebi, Helmi; Graham, Deborah S. Cunninghame; Grandits, Martha E.; Nazmul-Hossain, Abu N. M.; Patel, Ketan; Adler, Adam J.; Maier-Moore, Jacen S.; Farris, A. Darise; Brennan, Michael T.; Lessard, James A.; Chodosh, James; Gopalakrishnan, Rajaram; Hefner, Kimberly S.; Houston, Glen D.; Huang, Andrew J.W.; Hughes, Pamela J.; Lewis, David M.; Radfar, Lida; Rohrer, Michael D.; Stone, Donald U.; Wren, Jonathan D.; Vyse, Timothy J.; Gaffney, Patrick M.; James, Judith A.; Omdal, Roald; Wahren-Herlenius, Marie; Illei, Gabor G.; Witte, Torsten; Jonsson, Roland; Rischmueller, Maureen; Rönnblom, Lars; Nordmark, Gunnel; Ng, Wan-Fai; Mariette, Xavier; Anaya, Juan-Manuel; Rhodus, Nelson L.; Segal, Barbara M.; Scofield, R. Hal; Montgomery, Courtney G.; Harley, John B.; Sivils, Kathy L. Moser

    2013-01-01

    Sjögren’s syndrome is a common autoimmune disease (~0.7% of European Americans) typically presenting as keratoconjunctivitis sicca and xerostomia. In addition to strong association within the HLA region at 6p21 (Pmeta=7.65×10−114), we establish associations with IRF5-TNPO3 (Pmeta=2.73×10−19), STAT4 (Pmeta=6.80×10−15), IL12A (Pmeta =1.17×10−10), FAM167A-BLK (Pmeta=4.97×10−10), DDX6-CXCR5 (Pmeta=1.10×10−8), and TNIP1 (Pmeta=3.30×10−8). Suggestive associations with Pmeta<5×10−5 were observed with 29 regions including TNFAIP3, PTTG1, PRDM1, DGKQ, FCGR2A, IRAK1BP1, ITSN2, and PHIP amongst others. These results highlight the importance of genes involved in both innate and adaptive immunity in Sjögren’s syndrome. PMID:24097067

  1. Síndrome de Sjögren: revisão de literatura e acompanhamento de um caso clínico

    Directory of Open Access Journals (Sweden)

    Freitas Tarsila M. C.

    2004-01-01

    Full Text Available A síndrome de Sjögren é uma doença inflamatória sistêmica, de natureza auto-imune, caracterizada pela infiltração linfocitária progressiva de vários órgãos exócrinos e não exócrinos. Acomete preferencialmente as glândulas salivares e lacrimais determinando prejuízo estrutural e disfunção secretória destes órgãos. A produção de auto-anticorpos e a hipergamaglobulinemia policlonal indicam que anormalidades na imunidade humoral desempenham um papel importante na patogenia desta afecção e o seu diagnóstico é baseado na combinação de vários achados clínicos e laboratoriais. A proposta deste trabalho é apresentar um caso clínico de síndrome de Sjögren enfatizando a sua importância clínica e a necessidade do diagnóstico precoce na tentativa de melhorar a qualidade de vida dos pacientes acometidos.

  2. A Systematic Review of Peripheral and Central Nervous System Involvement of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Primary Sjögren’s Syndrome, and Associated Immunological Profiles

    Directory of Open Access Journals (Sweden)

    Anastasia Bougea

    2015-01-01

    Full Text Available Both central (CNS and peripheral nervous system (PNS complications are frequent and varied in connective tissue diseases. A systematic review was conducted between 1989 and 2014 in the databases Medline, Scopus, and Cochrane Library using the search terms, peripheral and central nervous complications and immunological profiles, to identify studies in specific connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosus, and primary Sjögren’s syndrome. A total of 675 references were identified, of which 118 were selected for detailed analysis and 22 were included in the final review with a total of 2338 participants. Our search focused only on studies upon connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosus, and primary Sjögren’s syndrome associated with seroimmunological data. The reported prevalence of CNS involvement ranges from 9 to 92% across the reported studies. However, the association between CNS and PNS manifestations and seroimmunological profiles remains controversial. Τo date, no laboratory test has been shown as pathognomonic neither for CNS nor for PNS involvement.

  3. Optimization of nutritional and non-nutritional factors involved for production of antimicrobial compounds from Lactobacillus pentosus SJ65 using response surface methodology

    Directory of Open Access Journals (Sweden)

    Appukuttan Saraniya

    2014-01-01

    Full Text Available Bacteriocins from lactic acid bacteria are ribosomal synthesized antibacterial proteins/ peptides having wide range of applications. Lactobacillus pentosus SJ65, isolated from fermented Uttapam batter (used to prepare south Indian pan cake, produces bacteriocin having a broad spectrum of activity against pathogens. Optimization studies are of utmost important to understand the source of utilization and the conditions to enhance the production of metabolites. In the present study, an attempt was made to identify the parameters involved for maximal production of antimicrobial compounds especially bacteriocin from the isolate L. pentosus SJ65. Initially, optimal conditions, such as incubation period, pH, and temperature were evaluated. Initial screening was done using methodology onevariable-at-a-time (OVAT for various carbon and nitrogen sources. Further evaluation was carried out statistically using Plackett-Burman design and the variables were analyzed using response surface methodology using central composite design. The optimum media using tryptone or soy peptone, yeast extract, glucose, triammonium citrate, MnSO4, dipotassium hydrogen phosphate and tween 80 produced maximum bacteriocin activity.

  4. Isolation, Characterization, and Molecular Modeling of a Rheumatoid Factor from a Hepatitis C Virus Infected Patient with Sjögren’s Syndrome

    Directory of Open Access Journals (Sweden)

    Yu-Ching Lee

    2013-01-01

    Full Text Available We have previously isolated several IgG rheumatoid factors (RFs from patients with both rheumatoid arthritis and idiopathic thrombocytopenia purpura using phage display system. To study IgG RFs in patients with other autoimmune diseases, phage display antibody libraries from a hepatitis C virus infected patient with Sjögren’s syndrome were constructed. After panning, a specific clone RFL11 was isolated for characterization in advance. The binding activity and specificity of RFL11 to IgG Fc fragment were comparable to those of RFs previously isolated. The analysis with existed RF-Fc complex structures indicated the homology model of RFL11 is similar to IgM RF61 complex with high binding affinity of about 6×10-8 M. This effect resulted from longer complementarity-determining region (CDR combining key somatic mutations. In the RFL11-Fc interfaces, the CDR-H3 loop forms a finger-like structure extending into the bottom of Fc pocket and resulting in strong ion and cation-pi interactions. Moreover, a process of antigen-driven maturation was proven by somatically mutated VH residues on H2 and H3 CDR loops in the interfaces. Taken together, these results suggested that high affinity IgG RFs can be generated in patients with Sjögren’s syndrome and may play an important role in the pathogenesis of this autoimmune disease.

  5. Increased Interleukin-17F is Associated with Elevated Autoantibody Levels and More Clinically Relevant Than Interleukin-17A in Primary Sjögren’s Syndrome

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    Yuzhou Gan

    2017-01-01

    Full Text Available Th17 related immune response is pathogenic in primary Sjögren’s syndrome (pSS. However, the role of IL-17F, one potent inflammatory member of IL-17 family cytokines in pSS, has not been specifically defined. We recruited one hundred and nine pSS patients and forty-two healthy controls and their serum levels of IL-17A and IL-17F were determined by multiplex cytokine assays. White blood cell, red blood cell, neutrophil, lymphocyte, IgM, IgG, C3, C4, RF, ANA, anti-SSA antibody, and anti-SSB antibody were measured by standard laboratory techniques. EULAR Sjögren’s syndrome disease activity index (ESSDAI score was also evaluated accordingly. We found that IL-17F was significantly increased in pSS patients. Elevated levels of IL-17F were associated with increased IgG and IgM, higher titers of ANA and anti-SSA antibodies, and reduction of C3 and C4. Patients with higher disease activity also showed higher serum IL-17F levels. However, serum IL-17A was only increased in patients with longer disease duration and showed few correlation with clinical and laboratory features in pSS patients. In conclusion, IL-17F was correlated with increased autoantibody levels and disease activity in pSS and is more clinically relevant than IL-17A.

  6. The anatomy and phylogeny of the African land snail Limicena Connolly, 1925 (Pulmonata: Cerastidae)

    NARCIS (Netherlands)

    Mordan, P.

    1998-01-01

    An investigation of the anatomy of Limicena confirms its position as a monotypic genus in the Cerastidae. Phylogenetic analysis places Limicena as the most plesiomorphic member of a clade characterised by a pseudosigmurethrous excretory system, the more advanced members of which are Rhachistia,

  7. Editor’s Pick: TYRO3, AXL, and MERTK Receptor Tyrosine Kinases: Is There Evidence of Direct Involvement in Development and Onset of Sjögren’s Syndrome?

    Directory of Open Access Journals (Sweden)

    Arun Wanchoo

    2016-07-01

    Full Text Available Sjögren’s syndrome (SjS is a chronic, progressive, systemic, human autoimmune disease in which an auto-inflammatory process within the salivary and lacrimal glands results in loss of saliva and tear production, respectively. In-depth analyses of the autoimmune process in humans and animal models of SjS substantiates one of the more important pathoaetiological pathways: an increased level of glandular apoptosis and/or cell lysis. We have hypothesised that failure in clearance of dying cells by macrophages, dendritic cells, and neighbouring tissues results in a sustained innate inflammatory response that transitions to autoimmunity. Since the intrinsic inhibition of inflammation following phagocytosis of dying cells is a function of a family of three receptor tyrosine kinases (RTKs known as the TAM (Tyro3, Axl, and Mertk, we put forward the following hypothesis: based on published information and analysis of our public microarray data, the failure of TAM RTK signalling, specifically in activating suppressor of cytokine signalling (SOCS 1 and SOCS3 (which are inhibitors of immune responses, may lead to autoimmunity, and specifically, to SjS-like disease.

  8. Efficacy and safety of rebamipide for the treatment of dry mouth symptoms in patients with Sjögren's syndrome: a double-blind placebo-controlled multicenter trial.

    Science.gov (United States)

    Sugai, Susumu; Takahashi, Hiroki; Ohta, Shuji; Nishinarita, Makoto; Takei, Masami; Sawada, Shigemasa; Yamaji, Ken; Oka, Hiroshi; Umehara, Hisanori; Koni, Ichiro; Sugiyama, Eiji; Nishiyama, Susumu; Kawakami, Atsushi

    2009-01-01

    The effects of rebamipide on dry mouth and salivary secretion in Sjögren's syndrome patients were investigated in a double-blind placebo-controlled study. Rebamipide (100 mg TID) or placebo was administered for eight weeks and patient-assessed improvement of dry mouth and increase in salivary secretion measured by the Saxon test were evaluated. At two, four, and eight weeks, dry mouth improvement rates were, respectively, 26.0, 44.0, and 46.9% for rebamipide and 20.0, 27.1, and 39.1% for placebo, and mean increases in salivary secretion were, respectively, 0.14, 0.24, and 0.35 g for rebamipide and 0.03, 0.09, and 0.17 g for placebo, indicating higher values in the rebamipide group for both parameters at all timepoints but no significant differences between the two groups. Analysis by baseline characteristics suggested a statistically significant salivary secretion increasing effect of rebamipide in cases of primary Sjögren's syndrome. No difference in the incidence of adverse events was seen between the two groups, confirming the safety of rebamipide. As a salivary secretion increasing effect was strongly suggested in cases of primary Sjögren's syndrome, further study on the administration of rebamipide for the treatment of dry mouth in patients with Sjögren's syndrome is required.

  9. Rétoři na moři a pomeranč v refektáři neboli Umění elokvence P. Johanna Krause SJ

    Czech Academy of Sciences Publication Activity Database

    Svatoš, Martin

    2010-01-01

    Roč. 50, č. 1 (2010), s. 169-191 ISSN 0323-0562 Institutional research plan: CEZ:AV0Z90090514 Keywords : P. Johann Kraus SJ * conceptual sermons * rhetoric * Jesuits in Bohemia Subject RIV: AJ - Letters, Mass-media, Audiovision

  10. Uso oral do óleo de linhaça (Linum usitatissimum no tratamento do olhoseco de pacientesportadores da síndrome de Sjögren Oral flaxseed oil (Linum usitatissimum in the treatment for dry-eye Sjögren's syndrome patients

    Directory of Open Access Journals (Sweden)

    Manuel Neuzimar Pinheiro Jr.

    2007-08-01

    Full Text Available OBJETIVO: Avaliar se a utilização por via oral do óleo de linhaça (Linum usitatissimum, que diminui a inflamação na artrite reumatóide, pode auxiliar no tratamento da ceratoconjuntivite seca de portadores da síndrome de Sjögren. MÉTODOS: Em estudo clínico randomizado, 38 pacientes do sexo feminino, com diagnóstico de artrite reumatóide ou lúpus eritematoso sistêmico associadas à ceratoconjuntivite seca e síndrome de Sjögren, provenientes do ambulatório de Reumatologia do Hospital Universitário da Universidade Federal do Amazonas, foram consecutivamente selecionadas. O diagnóstico de ceratoconjuntivite seca foi baseado em questionário para olho seco (Ocular Surface Disease Index - OSDI®, Teste I de Schirmer, tempo de quebra do filme lacrimal com fluoresceína e instilação do corante rosa bengala a 1%, com intensidade da impregnação da superfíce ocular quantificada pela escala de van Bijsterveld. Todas as pacientes tiveram a inflamação da superfície conjuntival avaliada e quantificada por interpretação de exame de citologia de impressão conjuntival antes do início e ao final do estudo. As pacientes foram divididas em três grupos: Grupo I (n=13, Grupo II (n=12 e Grupo III (n=13. O Grupo I recebeu cápsulas com dose final de 1 g/dia de óleo de linhaça, o Grupo II recebeu cápsulas com dose final de 2 g/dia de óleo de linhaça e o Grupo III - controle - recebeu cápsulas com placebo, por 180 dias. RESULTADOS: Comparando os resultados no início e no final do tratamento, foram verificadas mudanças estatisticamente significantes (pPURPOSE: To evaluate if oral flaxseed oil (Linum usitatissimum, which reduces the inflammation in rheumatoid arthritis, may help keratoconjunctivitis sicca's treatment in Sjögren's syndrome patients. METHODS: In a randomized clinical trial, 38 female patients with rheumatoid arthritis or systemic lupus erithematosus associated with keratoconjunctivitis sicca and Sjögren's syndrome

  11. Oxadiazole-2-oxides may have other functional targets, in addition to SjTGR, through which they cause mortality in Schistosoma japonicum.

    Science.gov (United States)

    Song, Li-Jun; Luo, Huan; Fan, Wen-Hua; Wang, Gu-Ping; Yin, Xu-Ren; Shen, Shuang; Wang, Jie; Jin, Yi; Zhang, Wei; Gao, Hong; Liu, Qian; Wang, Wen-Long; Feng, Bainian; Yu, Chuan-Xin

    2016-01-20

    Schistosomiasis is one of the world's major public health problems. Besides praziquantel (PZQ), there is currently no other effective treatment against schistosomiasis. The development of new antischistosomal agents to curb the emergence of PZQ resistance should be a high priority. Oxadiazole-2-oxides have been identified as potential antischistosomal reagents, with thioredoxin glutathione reductase (TGR) being one of their molecular targets. To develop novel treatment reagents against Schistosoma japonicum, 30 novel oxadiazole-2-oxides were synthesised and their antischistosomal activities on juvenile and adult S. japonicum were evaluated in vitro and in vivo. Their inhibitory activities against S. japonicum thioredoxin glutathione reductase (SjTGR) were also analysed. Most of the oxadiazole-2-oxides showed good juvenile and adult S. japonica killing activities in vitro. However, the antischistosomal effects of these compounds were not positively correlated with either their inhibition of SjTGR, or with nitric oxide (NO) release. Compounds 4a, 4b, 7c, 13, 16 and 20 resulted in 87.7%, 83.1%, 87.1%, 84.6%, 90.8% and 69.5%, respectively, mortality in the adult worms, when used to treat infected mice at schistosomula stage. These mortality rates were similar to or higher than that of artemisinin. Furthermore, compounds 4a and 16 resulted in 66.7% and 69.4% reductions in the worm burdens, respectively, when infected mice were treated at the adult worm stage. These treatment effects were similar to PZQ. No differences in activity of the oxadiazole-2-oxides against female and male adult worms were observed. The toxicity of the oxadiazole-2-oxides on mammalian cells appeared to be similar to, or less than, that of PZQ. The antischistosomal activity of the oxadiazole-2-oxides does not depend on NO production or the inhibition of SjTGR activity. There may be other functional targets of the oxadiazole-2-oxides in S. japonicum. Several of the novel oxadiazole-2-oxides

  12. Agranulocytosis and mixed-type autoimmune hemolytic anemia in primary sjögren's syndrome: a case report and review of the literature.

    Science.gov (United States)

    Qiao, Lin; Chen, Jing; Leng, Xiao-Mei; Zhang, Wen; Han, Bing; Zhao, Yan; Zeng, Xiao-Feng

    2016-12-01

    Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that presents with sicca symptoms of the main mucosal surfaces. Patients with pSS have a broad spectrum of laboratory features, such as cytopenias and hypergammaglobulinemia. Although hematological abnormalities are usually seen in pSS patients, agranulocytosis and autoimmune hemolytic anemia (AIHA) are rare. Here we describe a 40-year-old woman with pSS who developed both agranulocytosis and mixed-type AIHA. An increased risk of malignancies has also been reported in pSS patients with hematological changes. Although there is no evidence of malignancies, this patient should be closely followed up in case of developing lymphoma. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  13. Interstitial cystitis associated with primary Sjögren's syndrome successfully treated with a combination of tacrolimus and corticosteroid: A case report and literature review.

    Science.gov (United States)

    Ueda, Yo; Tomoe, Hikaru; Takahashi, Hiroyuki; Takahashi, Yuko; Yamashita, Hiroyuki; Kaneko, Hiroshi; Kano, Toshikazu; Mimori, Akio

    2016-01-01

    We report a case of interstitial cystitis (IC) associated with primary Sjögren's syndrome (SS) successfully controlled with combination therapy of tacrolimus and a corticosteroid. In 2011, a 69-year-old female, who had been diagnosed with primary SS 23 years ago, developed IC and was successfully treated with tacrolimus and prednisolone combination therapy. The mechanism of IC, including the involved autoimmunity, has not been elucidated. Clinical observation studies suggest a potential association between SS and IC. However, IC is currently thought to be underdiagnosed in patients with SS as well as in the general population. Based on our case and others reported previously, IC associated with SS responds well to immunosuppressive therapy. In particular, a combination of a calcineurin inhibitor (tacrolimus or cyclosporine) with a corticosteroid seems to be highly effective. The possibility of IC in patients with SS complaining of lower urinary tract symptoms without features of infection or other identifiable causes should be given attention.

  14. A Review on InGaSb Growth under Microgravity and Terrestrial Conditions Towards Future Crystal Growth Project Using Chinese Recovery Satellite SJ-10

    Science.gov (United States)

    Yu, Jianding; Liu, Yan; Pan, Xiuhong; Zhao, Hongyang; Kumar, Velu Nirmal; Arivanandhan, Mukannan; Momose, Yoshimi; Hayakawa, Yasuhiro; Zhang, Xingwang; Luo, Xinghong; Okano, Yasuhiro; Inatomi, Yuko

    2016-05-01

    The paper reviewed the previous microgravity experiment using Chinese recovery satellite, the in-situ measurement of composition profile in the solution by X-ray penetration method and homogeneous growth of InGaSb by temperature freezing method under terrestrial condition for making clear the effect of gravity on the growth of InGaSb ternary alloy semiconductor crystals. The previous experimental results showed that the shape of solid/liquid interfaces and composition profile in the solution were significantly affected by gravity. Based on the previous microgravity experimental results, experimental conditions were investigated to grow homogeneous In xGa 1-xSb with higher indium composition at Chinese recovery satellite SJ-10 in near future.

  15. Fler perspektiv, höjd vetenskaplig nivå, ökad trygghet: grupphandledning av självständiga arbeten i laboratoriemiljö.

    Directory of Open Access Journals (Sweden)

    Helen Setterud

    2013-01-01

    Full Text Available Syftet med denna artikel är att diskutera formerna för hur grupphandledning kan arrangeras inom utbildningar med starkt fokus på laborativ verksamhet. Jag presenterar data från vårt projekt om stöd för handledning, varav delar tidigare har beskrivits i slutrapporten. Jag argumenterar för att nätbaserade grupper är ett på många sätt bättre alternativ än grupper som möts fysiskt då projekten utförs i laborativ miljö. Detta är ett sätt att anpassa grupphandledningen till de förhållanden som råder inom en utbildningsmiljö med närvarokrävande laborativ verksamhet, där både studenter och handledare har mycket svårt att planera in fysiska möten. Handledningsgrupper skapades med studenter som tillsammans med en grupphandledare, frikopplad från deras egna projekt, diskuterade det självständiga arbetet samt fick möjlighet att ge och ta återkoppling på varandras texter vid ett flertal tillfällen under processens gång. Slutsatsen är att grupphandledning på nätet kan vara ett bra komplement till individuell handledning av självständiga arbeten i laborativ miljö. Studenterna i vår studie kände en ökad trygghet i arbetet, kvaliteten på deras skriftliga rapporter ökade och deras lärande förbättrades. Handledarna som deltog utvecklade sin pedagogiska kompetens och fick ökad insikt i handledningsprocessen

  16. A Rare Case of Necrotizing Myopathy and Fibrinous and Organizing Pneumonia with Anti-EJ Antisynthetase Syndrome and Sjögren’s Syndrome (SSA) Antibodies

    Science.gov (United States)

    Kashif, Muhammad; Arya, Divya; Niazi, Masooma; Khaja, Misbahuddin

    2017-01-01

    Patient: Male, 34 Final Diagnosis: Necrotizing myopathy • fibrinous • organizing pneumonia Symptoms: Short of breath • weakness in limbs Medication: — Clinical Procedure: — Specialty: Rheumatology Objective: Rare co-existance of disease or pathology Background: Idiopathic inflammatory myopathies are autoimmune disorders that can involve the skin, joints, muscles, and lungs. The most common of these disorders are dermatomyositis, polymyositis, overlap syndrome, and inclusion body myositis. Necrotizing autoimmune myopathy is an idiopathic inflammatory myopathy that is rarely associated with Sjögren’s syndrome. The most common lung findings associated with anti-EJ antisynthetase syndrome are nonspecific interstitial pneumonia and usual interstitial pneumonia; this condition is rarely associated with fibrinous and organizing pneumonia. Case Report: Here, we present a rare case of necrotizing myopathy and fibrinous and organizing pneumonia in a 34-year-old African American man with Sjögren’s syndrome and anti-EJ antibodies. The patient’s presenting symptoms were cough and proximal muscle weakness of the extremities. He had elevated serum creatine kinase level, aldolase level, and erythrocyte sedimentation rate. Myositis panel was positive for anti-EJ antibodies. Chest radiography was consistent with bilateral interstitial infiltrates. CT chest showed patchy bilateral infiltrates. Quadriceps muscle biopsy revealed widespread necrotic fibers and lung biopsy showed fibrinous and organizing pneumonia. The patient responded well to immunoglobulin therapy, mycophenolate, and prednisone, which resulted in complete resolution of bilateral infiltrates and improved muscle pain and weakness. Conclusions: Myopathies are characterized by myalgia and muscle weakness due to muscle fiber dysfunction and are associated with autoimmune diseases. Histopathological features may differ in idiopathic inflammatory myopathies. It is important to recognize the rare association

  17. The orthologue of Sjögren's syndrome nuclear autoantigen 1 (SSNA1 in Trypanosoma brucei is an immunogenic self-assembling molecule.

    Directory of Open Access Journals (Sweden)

    Helen P Price

    Full Text Available Primary Sjögren's Syndrome (PSS is a highly prevalent autoimmune disease, typically manifesting as lymphocytic infiltration of the exocrine glands leading to chronically impaired lacrimal and salivary secretion. Sjögren's Syndrome nuclear autoantigen 1 (SSNA1 or NA14 is a major specific target for autoantibodies in PSS but the precise function and clinical relevance of this protein are largely unknown. Orthologues of the gene are absent from many of the commonly used model organisms but are present in Chlamyodomonas reinhardtii (in which it has been termed DIP13 and most protozoa. We report the functional characterisation of the orthologue of SSNA1 in the kinetoplastid parasite, Trypanosoma brucei. Both TbDIP13 and human SSNA1 are small coiled-coil proteins which are predicted to be remote homologues of the actin-binding protein tropomyosin. We use comparative proteomic methods to identify potential interacting partners of TbDIP13. We also show evidence that TbDIP13 is able to self-assemble into fibril-like structures both in vitro and in vivo, a property which may contribute to its immunogenicity. Endogenous TbDIP13 partially co-localises with acetylated α-tubulin in the insect procyclic stage of the parasite. However, deletion of the DIP13 gene in cultured bloodstream and procyclic stages of T. brucei has little effect on parasite growth or morphology, indicating either a degree of functional redundancy or a function in an alternative stage of the parasite life cycle.

  18. Immune response against the coiled coil domain of Sjögren's syndrome associated autoantigen Ro52 induces salivary gland dysfunction.

    Science.gov (United States)

    Sroka, Magdalena; Bagavant, Harini; Biswas, Indranil; Ballard, Abigail; Deshmukh, Umesh S

    2018-01-31

    The structural domains of Ro52, termed the RING, B-box, coiled coil (CC) and B30.2/SPRY are targets of anti-Ro52 in multiple autoimmune disorders. In Sjögren's syndrome patients, the presence of anti-Ro52 is associated with higher disease severity, and in mice, they induce salivary gland hypofunction. This study was undertaken to investigate whether immune responses against different domains of Ro52, influences salivary gland disease in mice. Female NZM2758 mice were immunised with Ro52 domains expressed as recombinant fusion proteins with maltose binding protein (MBP) [MBP-RING-B-box, MBP-CC, MBP-CC(ΔC19), MBP-B30.2/SPRY]. Sera from immunised mice were studied for IgG antibodies to Ro52 by immunoprecipitation, and to salivary gland cells by immunofluorescence. Pilocarpine-induced saliva production was measured to evaluate salivary gland function. Submandibular glands were investigated by histopathology for inflammation and by immune-histochemistry for IgG deposition. Mice immunised with different Ro52-domains had comparable reactivity to Ro52 and to salivary gland cells. However, only mice immunised with the CC domain and its C-terminal truncated version CC(ΔC19) showed a significant drop in saliva production. None of the mice developed severe salivary gland inflammation. The salivary gland hypofunction significantly correlated with increased intra-lobar IgG deposits in the submandibular salivary glands. Our data demonstrate that epitope specificity of anti-Ro52 antibodies plays a critical role in the induction of glandular dysfunction. Clearly, screening Sjögren's syndrome patients for relative levels of Ro52 domain specific antibodies will be more informative for associating anti-Ro52 with clinical measures of the disorder.

  19. Impact of a sodium carbonate spray combined with professional oral hygiene procedures in patients with Sjögren's syndrome: an explorative study.

    Science.gov (United States)

    Gambino, Alessio; Broccoletti, Roberto; Cafaro, Adriana; Cabras, Marco; Carcieri, Paola; Arduino, Paolo G

    2017-06-01

    The aim of this study was to make an initial estimation on the effects of a sodium bicarbonate and xylitol spray (Cariex ® ), associated with non-surgical periodontal therapy, in participants with primary Sjögren's syndrome. Sjögren's syndrome (SS) is a multisystem autoimmune disease that predominantly involves salivary and lachrymal glands, with the clinical effect of dry eyes and mouth. A prospective cohort of 22 women and two men has been evaluated. They were randomized into three groups (eight patients each): Group A) those treated once with non-surgical periodontal therapy, education and motivation to oral hygiene, associated with the use of Cariex ® ; Group B) treated only with Cariex ® ; Group C) treated only with non-surgical periodontal therapy, education and motivation to oral hygiene. Clinical variables described after treatment were unstimulated whole salivary flow, stimulated whole salivary flow, salivary pH, reported pain (using Visual Analogue Scale) and the Periodontal Screening and Recording index. Salivary flow rate improved in all groups, but the difference was statistically significant only in those treated with Cariex ® , alone or in combination with periodontal therapy. Gingival status improved in participants who underwent periodontal non-surgical therapy while remained unchanged in those only treated with Cariex ® . Reported pain decreased in all groups, showing the best result in participants treated with periodontal therapy together with Cariex ® . We propose a practical approach for improving gingival conditions and alleviating oral symptoms in patients with SS. Future randomized and controlled trials are however required to confirm these results as well as larger population, and also assessing other parameters due to oral dryness, possible oral infections and more comprehensive periodontal indices. © 2016 John Wiley & Sons A/S and The Gerodontology Association. Published by John Wiley & Sons Ltd.

  20. Immunohistochemical Analysis of Interleukin-17 Producing T Helper Cells and Regulatory T Cells Infiltration in Annular Erythema Associated with Sjögren's Syndrome

    Science.gov (United States)

    Itoi, Saori; Tani, Mamori; Kitaba, Shun; Terao, Mika; Murota, Hiroyuki; Oiso, Naoki; Katayama, Ichiro

    2014-01-01

    Background Peculiar erythema known as annular erythema associated with Sjögren's syndrome (AESS) can be differentiated from autoimmune annular erythema and subacute cutaneous lupus erythematosus, both clinically and histologically. However, there are no detailed investigations on immune competent cells infiltration. Objective Preferential infiltration of interleukin-17-producing T helper (Th17) cells and regulatory T (Treg) cells into the labial salivary gland is reported to play a role in maintaining mucoepithelitis in patients with Sjögren's syndrome. In this study, we evaluated Th17 and Treg cell infiltration into the lesional skin of AESS. Methods We analyzed the numbers and infiltration patterns of Th17 and FoxP3 (+) Treg cells in seven cases of AESS using immunohistochemistry. Seven patients with systemic lupus erythematosus (SLE), atopic dermatitis (AD) and psoriasis vulgaris (PV), which are representatives of Th17 cell-involved skin disorders, were enrolled as disease controls. Results Periappendageal and epidermal changes, such as follicular plugging and liquefaction, were evident in the annular erythema of SLE, not AESS, tissue samples. In AESS tissue samples, dense perivascular and periappendageal infiltration of lymph cells was observed in the middle-to-deep dermis, as previously described, in contrast to the superficial infiltration pattern observed in both AD and PV samples. While the total number of infiltrated lymphocytes was similar between AESS and SLE tissue samples, Th17 cells were found to be preferentially infiltrated in the middle-to-deep dermis in AESS samples. Conclusion These results suggest that an increased number and distribution of infiltration of Th17 cells is a preferential feature of AESS, rather than a characteristic feature of annular erythema of SLE. PMID:24882975

  1. Autodisplay of the La/SSB protein on LPS-free E. coli for the diagnosis of Sjögren's syndrome.

    Science.gov (United States)

    Yoo, Gu; Dilkaute, Carina; Bong, Ji-Hong; Song, Hyun-Woo; Lee, Misu; Kang, Min-Jung; Jose, Joachim; Pyun, Jae-Chul

    2017-05-01

    The objective of this study was to present an immunoassay for the diagnosis of Sjögren's syndrome based on the autodisplayed La/SSB protein on the outer membrane of intact E. coli (strain UT-5600) and LPS-free E. coli (ClearColi™). As the first step, an autodisplay vector (pCK002) was transfected into intact E. coli and LPS-free E. coli for comparison of efficiency of autdisplay of La/SSB. The maximal level of La/SSB expression was estimated to be similar for LPS-free E. coli and intact E. coli at different optimal induction periods. Intact E. coli was found to grow twofold faster than LPS-free E. coli, and the maximal level of expression for LPS-free E. coli was obtained with a longer induction period. When the zeta potential was measured, both intact E. coli and LPS-free E. coli showed negative values, and the autodisplay of negatively charged La/SSB protein (pIE. coli and LPS-free E. coli resulted in a slight change in zeta potential values. E. coli with autodisplayed La/SSB protein was used for an immunoassay of anti-La/SSB antibodies for the diagnosis of Sjögren's syndrome. The surface of E. coli with the autodisplayed antigen was modified with rabbit serum and papain to prevent false positive signals because of nonspecific binding of unrelated antibodies from human serum. LPS-free E. coli with autodisplayed La/SSB protein yielded sensitivity and selectivity of 81.6% and 78.6%, respectively. The Bland-Altman test showed that the immunoassays based on LPS-free E. coli and intact E. coli with autodisplayed La/SSB protein were statistically equivalent to a clinical immunoassay for detection of anti-La/SSB antibodies (confidence coefficient 95%). Copyright © 2017 Elsevier Inc. All rights reserved.

  2. "Att leva ett meningsfullt liv med skuggan" : en kvantitativ, longitudinell och jämförande undersökelse av självbemästringsprogrammet "Att leva ett friskare liv"

    OpenAIRE

    Engell, Lena Cristina

    2005-01-01

    Fler och fler får kroniska sjukdomar, också yngre människor. Om man tar i betraktning den belastning som kronisk sjukdom kan ge, ser man att det är behov för en välfungerande utbildning för denna växande grupp av människor. Det amerikanska självhjälpsprogrammet Att leva ett friskare liv placerar personer med olika kroniska tillstånd och kombinationer av tilläggsjukdomar i samma program. Programmet söker att ge kursdeltagarna bistånd till att bli självhjälpta i sin strävan att bemästra sitt ...

  3. Condição oral dos pacientes com síndrome de Sjögren: uma revisão sistemática: uma revisão sistemática

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    Ferreira, Maria Angela

    2005-01-01

    Full Text Available Introdução: A Síndrome de Sjögren é uma doença auto-imune crônica, na qual o sistema imune do paciente ataca as glândulas salivares e lacrimais. No meio ambiente oral, ela provoca uma série de alterações dentre as quais se sobressai a xerostomia. Objetivo: Este estudo teve por objetivo investigar as manifestações orais da Síndrome de Sjögren e suas conseqüências na estrutura dentária. Materiais e métodos: Foram utilizados como fonte de pesquisa consultas manuais em periódicos especializados da área, sites e nas bases de dados eletrônicas MEDLINE, LILACS e BBO. Foram incluídos estudos transversais, caso-controle e coorte com avaliação clínica realizada através dos índices de cárie dentária e de doença periodontal. Os idiomas selecionados foram português, inglês e espanhol, considerando o período de 1990 a 2003. As estratégias de busca foram realizadas utilizando-se as seguintes palavras: sjögren, dmf, carie, decay, periodontal, plaque, gingivite. Resultados: No total, 78 estudos foram encontrados, sendo que destes, apenas 10 foram selecionados (todos da MEDLINE por estarem de acordo com aos critérios de inclusão. Conclusões: Os estudos analisados demonstram que o principal sintoma da síndrome de Sjögren é a xerostomia e que existe uma fraca relação entre os pacientes sindrômicos e os índices de cárie e nenhuma relação com a doença periodontal.

  4. Is primary Sjögren's syndrome a risk factor for malignancies different from lymphomas? What does the literature highlight about it?

    Science.gov (United States)

    Manzo, Ciro; Kechida, Melek

    2017-01-01

    Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease with an elevated risk of developing lymphoproliferative malignancies (LM). Whether pSS is a risk factor or not for non-lymphoma malignancies (NLM) has been scarcely evaluated in the literature. Age is per se a risk factor for malignancies: patients over 70 years old have 4 times higher risk for cancers than adults. Even if the mean age of pSS onset usually is in the 4 th and 5 th decade, its onset in patients aged over 65 years (Elderly Onset pSS - EOpSS) is not uncommon. To evaluate pSS as a risk factor for NLM we performed a systematic electronic search on PubMed in the period 2006-2016 to identify all the publications on this topic. The studies were eligible for inclusion if they reported specific Standardized Incidence Ratio (SIR) with 95% CI. Studies that did not report sufficient published and/or original data were excluded. Only 7 articles of 494 that we found in PubMed fulfilled the inclusion criterion. In the vast majority of these, SIR values were not statistically significant for NLM. The occurrence of NLM after LM was statistically significant in some studies and a NLM represented the most frequent cause of death. The possibility that NLM may represent a paraneoplastic syndrome seems much more frequent than LM, the risk of which increases with time after the diagnosis. Data regarding the neoplastic weight of EOpSS are mainly pointed out by case reports. Primary Sjögren's syndrome is not associated with an increased risk for NLM. However the possibility that NLM may appear after recovery from lymphoma should be carefully considered because it could be cause of the patient's death. Similarly the possibility that NLM may represent a paraneoplastic syndrome must be highlighted. The relationship between EOpSS and SIRs for NLM should be deepened with studies on ad hoc cohorts.

  5. Is primary Sjögren’s syndrome a risk factor for malignancies different from lymphomas? What does the literature highlight about it?

    Directory of Open Access Journals (Sweden)

    Ciro Manzo

    2017-07-01

    Full Text Available Background : Primary Sjögren’s syndrome (pSS is a chronic systemic autoimmune disease with an elevated risk of developing lymphoproliferative malignancies (LM. Whether pSS is a risk factor or not for non-lymphoma malignancies (NLM has been scarcely evaluated in the literature. Age is per se a risk factor for malignancies: patients over 70 years old have 4 times higher risk for cancers than adults. Even if the mean age of pSS onset usually is in the 4th and 5th decade, its onset in patients aged over 65 years (Elderly Onset pSS – EOpSS is not uncommon. Material and methods : To evaluate pSS as a risk factor for NLM we performed a systematic electronic search on PubMed in the period 2006–2016 to identify all the publications on this topic. The studies were eligible for inclusion if they reported specific Standardized Incidence Ratio (SIR with 95% CI. Studies that did not report sufficient published and/or original data were excluded. Results : Only 7 articles of 494 that we found in PubMed fulfilled the inclusion criterion. In the vast majority of these, SIR values were not statistically significant for NLM. The occurrence of NLM after LM was statistically significant in some studies and a NLM represented the most frequent cause of death. The possibility that NLM may represent a paraneoplastic syndrome seems much more frequent than LM, the risk of which increases with time after the diagnosis. Data regarding the neoplastic weight of EOpSS are mainly pointed out by case reports. Conclusions : Primary Sjögren’s syndrome is not associated with an increased risk for NLM. However the possibility that NLM may appear after recovery from lymphoma should be carefully considered because it could be cause of the patient’s death. Similarly the possibility that NLM may represent a paraneoplastic syndrome must be highlighted. The relationship between EOpSS and SIRs for NLM should be deepened with studies on ad hoc cohorts.

  6. MRI and ¹H-MRS findings of three patients with Sjögren-Larsson syndrome Síndrome de Sjögren-Larsson: achados à ressonância magnética e espectroscopia de prótons em três pacientes

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    Mauro Nakayama

    2006-06-01

    Full Text Available Sjögren-Larsson syndrome (SLS is a rare autosomal recessive neurocutaneous disorder caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. Patients present the classical triad of congenital ichthyosis, mental retardation and spastic di- or tetraplegia. Magnetic resonance imaging (MRI of the brain usually shows hypomyelination involving the periventricular white matter. Cerebral proton MR spectroscopy (¹H-MRS reveals a characteristic abnormal lipid peak. We report three cases of SLS from different families with the typical clinical triad. The MRI and ¹H-MRS findings are discussed.A síndrome de Sjögren-Larsson (SJL é distúrbio raro, autossômico recessivo, caracterizado pela tríade clássica de ictiose congênita, retardo mental e tetraplegia ou diplegia espástica. Trata-se de um erro inato do metabolismo dos lipídios, causado pela deficiência da enzima microssômica aldeído graxo desidrogenase. Os achados de imagem do encéfalo na SJL demonstram atrofia cerebral e alteração da substância branca. A espectroscopia de prótons, com poucos casos relatados, caracteriza-se pelo elevado pico de lipídios e redução de N-acetil-aspartato. Apresentamos três casos de SJL, com ênfase nos achados da ressonância magnética e da espectroscopia de prótons.

  7. The psychological defensive profile of primary Sjögren's syndrome patients and its relationship to health-related quality of life.

    Science.gov (United States)

    Hyphantis, T; Mantis, D; Voulgari, P V; Tsifetaki, N; Drosos, A A

    2011-01-01

    We aimed to assess the defensive profile of primary Sjögren's syndrome (SS) patients and to investigate the independent associations of psychological distress and personality variables with health-related quality of life (HRQOL). In 40 primary SS patients we assessed psychological distress (SCL-90-R), ego defense mechanisms (Defense Style Questionnaire), hostility features (HDHQ) and HRQOL (WHOQOL-BREF). Fifty-six patients with Systemic Lupus Erythematosous (SLE) and 80 healthy participants matched for age and sex served as controls. Primary SS patients presented higher rates of general psychological distress compared to SLE and healthy participants. Symptoms of somatisation were more prominent in SS than SLE or healthy controls. SS patients presented less use of humour defense and more help-rejecting complains and delusional guilt hostility, compared to controls. Primary SS patients' HRQOL was more impaired than healthy participants and comparable to SLE. Psychological distress was a constant independent correlate of SS patients' HRQOL, while less use of humour (ppsychological distress; more use of schizoid fantasy was also independently associated with impaired Environment HRQOL (p=0.005). Primary SS patients exhibit several specific psychological difficulties in adaptation to life stressors, and clinicians and consultation-liaison psychiatrists, apart from the early assessment and treatment of psychological distress and somatisation symptoms, should consider the patients' underlying defensive profile and coping capacities, since such personality traits, although usually underestimated, are also independently associated with the disease outcome.

  8. Effectiveness of low-dose doxycycline (LDD on clinical symptoms of Sjögren's Syndrome: a randomized, double-blind, placebo controlled cross-over study

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    Vuotila Tuija

    2007-12-01

    Full Text Available Abstract Background Matrix metalloproteinases (MMPs are proteolytic enzymes that may contribute to tissue destruction in Sjögren's syndrome (SS. Low-dose doxycycline (LDD inhibits MMPs. We evaluated the efficacy of LDD for the subjective symptoms in primary SS patients. This was a randomized, double blind, placebo controlled cross-over study. 22 patients were randomly assigned to receive either 20 mg LDD or matching placebo twice a day for 10 weeks. The first medication period was followed by 10-week washout period, after which the patient received either LDD or placebo, depending on the first drug received, followed by the second washout period. Stimulated saliva flow rates and pH were measured before and after one and ten weeks of each medication and after washout periods. VAS scale was used to assess the effect of LDD and placebo on following six subjective symptoms: xerostomia; xerophtalmia; difficulty of swallowing; myalgia; arthralgia; and fatigue. The effect was evaluated for each medication and washout period separately. Results Overall, the effects of medications on subjective symptoms were minor. Wilcoxon test demonstrated increased fatigue with LDD during medication (p Conclusion LDD may not be useful in reducing the primary SS symptoms.

  9. Proteomic analysis revealed the altered tear protein profile in a rabbit model of Sjögren's syndrome-associated dry eye.

    Science.gov (United States)

    Zhou, Lei; Wei, Ruihua; Zhao, Ping; Koh, Siew Kwan; Beuerman, Roger W; Ding, Chuanqing

    2013-08-01

    Sjögren's syndrome (SS) is an autoimmune disease that results in pathological dryness of mouth and eye. The diagnosis of SS depends on both clinical evaluation and specific antibodies. The goal of this study was to use quantitative proteomics to investigate changes in tear proteins in a rabbit model of SS-associated dry eye, induced autoimmune dacryoadenitis (IAD). Proteomic analysis was performed by iTRAQ and nano LC-MS/MS on tears collected from the ocular surface, and specific proteins were verified by high resolution MRM. It was found that in the tears of IAD rabbits at 2 and 4 weeks after induction, S100 A6, S100 A9, and serum albumin were upregulated, whereas serotransferrin (TF), prolactin-inducible protein (PIP), polymeric immunoglobulin receptor (pIgR), and Ig gamma chain C region were downregulated. High resolution MRM with mTRAQ labeling verified the changes in S100 A6, TF, PIP, and pIgR. Our results indicated significant changes of tear proteins in IAD rabbits, suggesting these proteins could potentially be used as biomarkers for the diagnosis and prognosis of dry eye. Several of these proteins were also found in the tears of non-SS dry eye patients indicating a common basis of ocular surface pathology, however, pIgR appears to be unique to SS. © 2013 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  10. BAFF overexpression increases lymphocytic infiltration in Sjögren's target tissue, but only inefficiently promotes ectopic B-cell differentiation.

    Science.gov (United States)

    Ding, Jian; Zhang, Wei; Haskett, Scott; Pellerin, Alex; Xu, Shanqin; Petersen, Britta; Jandreski, Luke; Hamann, Stefan; Reynolds, Taylor L; Zheng, Timothy S; Mingueneau, Michael

    2016-08-01

    B-cell activating factor (BAFF) levels are increased in rheumatoid arthritis, lupus and primary Sjögren's syndrome (pSS). However, BAFF contribution to pathogenesis is not completely understood. In pSS, immune infiltration of the salivary and lacrimal glands leads to xerostomia and xerophtalmia. Glandular B cell hyperactivation, differentiation into germinal center (GC)-like structures and plasma cell accumulation are histopathological hallmarks that were attributed to increased BAFF. Here, we experimentally tested this hypothesis by overexpressing BAFF in a mouse model of pSS. BAFF overexpression enhanced lymphocytic infiltration and MHCII expression on B cells. Increased BAFF also induced B cell differentiation into GC B cells within the autoimmune target tissue. However, even in these conditions, GC B cells only accounted for BAFF is not efficiently promoting ectopic GC formation in pSS and warranting further investigation of therapeutics targeting both BAFF and the related TNF-family member APRIL. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Fatigue and widespread pain in systemic lupus erythematosus and Sjögren's syndrome: symptoms of the inflammatory disease or associated fibromyalgia?

    Science.gov (United States)

    Iannuccelli, Cristina; Spinelli, Francesca Romana; Guzzo, Maria Paola; Priori, Roberta; Conti, Fabrizio; Ceccarelli, Fulvia; Pietropaolo, Mario; Olivieri, Marta; Minniti, Antonina; Alessandri, Cristiano; Gattamelata, Angelica; Valesini, Guido; Di Franco, Manuela

    2012-01-01

    Fatigue and generalised pain are debilitating symptoms that negatively impact the quality of life in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS). Chronic widespread musculoskeletal pain and fatigue are the clinical hallmarks of fibromyalgia (FM), a clinical entity which can be associated to connective tissue disease. The aim of the present study was to assess the prevalence of FM syndrome, fatigue and widespread pain in SLE and pSS patients and to evaluate the contribution of inflammatory disease and FM on those constitutional symptoms. Fifty SLE and 50 pSS patients were enrolled in the study. Patients rated fatigue, pain, and disease activity using a 100-mm visual analogue scale and completed the Health Assessment Questionnaire and the Fibromyalgia Impact Questionnaire. Zung depression and anxiety scales were used to quantify mood disorders. Tender points were evaluated using an algometer. Disease activity score as evaluated for each SLE and pSS patient. Fibromyalgia has been diagnosed in a significantly higher percentage of SLE patients than pSS patients (32% vs. 18%, p=0.022) even if the percentage of patients reporting fatigue and pain was higher among pSS patients. No correlation with disease activity was observed in either group of patients. FM seems to contribute to constitutional symptoms more in SLE than in pSS, suggesting a different underlying cause of fatigue and widespread pain in these two different connective tissue diseases.

  12. Metabolic bone disease as a presenting manifestation of primary Sjögren′s syndrome: Three cases and review of literature

    Directory of Open Access Journals (Sweden)

    Deepak Khandelwal

    2011-01-01

    Full Text Available Primary Sjögren′s syndrome (pSS is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement. Chronic inflammation compromises the glands′ function that leads to dry symptoms in the mouth/eyes. Renal involvement is a well recognized extraglandular manifestation of pSS. Metabolic bone disease (MBD, however, rarely occurs as the primary manifestation of a renal tubule disorder due to pSS. To the best of our knowledge there are only 6 reported cases of metabolic bone disease as the primary manifestation of pSS to date. Four of these had distal renal tubular acidosis (RTA, and 2 had a combined picture of distal and proximal tubular dysfunction. We herein present our experience of 3 cases who presented to us with a clinical picture suggestive of MBD. While investigating these patients, we found evidence of RTA, which was found to be secondary to pSS.

  13. Acupuncture at Waiguan (SJ5) and sham points influences activation of functional brain areas of ischemic stroke patients: a functional magnetic resonance imaging study.

    Science.gov (United States)

    Qi, Ji; Chen, Junqi; Huang, Yong; Lai, Xinsheng; Tang, Chunzhi; Yang, Junjun; Chen, Hua; Qu, Shanshan

    2014-02-01

    Most studies addressing the specificity of meridians and acupuncture points have focused mainly on the different neural effects of acupuncture at different points in healthy individuals. This study examined the effects of acupuncture on brain function in a pathological context. Sixteen patients with ischemic stroke were randomly assigned to true point group (true acupuncture at right Waiguan (SJ5)) and sham point group (sham acupuncture). Results of functional magnetic resonance imaging revealed activation in right parietal lobe (Brodmann areas 7 and 19), the right temporal lobe (Brodmann area 39), the right limbic lobe (Brodmann area 23) and bilateral occipital lobes (Brodmann area 18). Furthermore, inhibition of bilateral frontal lobes (Brodmann area 4, 6, and 45), right parietal lobe (Brodmann areas 1 and 5) and left temporal lobe (Brodmann area 21) were observed in the true point group. Activation in the precuneus of right parietal lobe (Brodmann area 7) and inhibition of the left superior frontal gyrus (Brodmann area 10) was observed in the sham group. Compared with sham acupuncture, acupuncture at Waiguan in stroke patients inhibited Brodmann area 5 on the healthy side. Results indicated that the altered specificity of sensation-associated cortex (Brodmann area 5) is possibly associated with a central mechanism of acupuncture at Waiguan for stroke patients.

  14. Cholinergic autoantibodies from primary Sjögren′s syndrome inhibit mucin production via phospholipase C and cyclooxygenase-2 in the rat submandibular gland

    Directory of Open Access Journals (Sweden)

    Daniela Passafaro

    2011-01-01

    Full Text Available Background: Patients with primary Sjögren′s syndrome (pSS produce functional IgG against cholinoreceptor of exocrine glands modifying their activity. The aim of the present work was to demonstrate pSS IgG antibodies (pSS IgG interacting with M 3 muscarinic acetylcholine receptors (mAChR of rats submandibular glands that alter mucin release and production via phospholipase C (PLC and cyclooxigenase-2 (COX-2 pathways. Methods:Mucin release and production of prostaglandin E2 (PGE2, and total inositol phosphates (InsP were measured in rat submandibular gland in the presence of pSS IgG auto antibodies. Results: The auto antibodies interacting with M3 mAChR decreased mucin release and production through stimulation of PLC and COX-2. This stimulation leads to an incremental increase in InsP production and in PGE2 generation, inducing signalling through the prostaglandin membrane receptors subtype 2 (EP2. Moreover, the decrease in mucin production had negative correlation with PGE 2 generation and InsP accumulation. Conclusion: IgG in patients with pSS could play an important role in the pathoetiology of dry mouth, decreasing the salivary mucin through the production of proinflammatory substances and leading to the reduction in the protection of the oral tissues.

  15. Serum CXCL4 increase in primary Sjögren's syndrome characterizes patients with microvascular involvement and reduced salivary gland infiltration and lymph node involvement.

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    Vettori, Serena; Irace, Rosaria; Riccardi, Antonella; Iacono, Daniela; Pellecchia, Luciana; Vicedomini, Lucia; Valentini, Gabriele

    2016-10-01

    CXCL4 is an antiangiogenic and immunomodulatory chemokine. We aimed to investigate serum levels of CXCL4 in primary Sjögren's syndrome (pSS), looking for associations with disease features. Thirty-nine consecutive pSS patients underwent clinical-serological assessment and nailfold videocapillaroscopy (NVC). Thirty-six patients and 30 controls affected by osteoarthritis were also investigated for serum levels of CXCL4 and soluble E-selectin (sE-selectin). CXCL4 was higher in pSS patients than in controls (1.79 [0.2-11.18] vs 1.023 ng/ml [0.02-14.45], p disease duration (r = 0.35, p Raynaud's phenomenon (RP) had more frequently abnormal CXCL4 levels than patients without RP (11/15 vs 3/21, p disease-specific autoantibodies, a low focus score, and the absence of lymphadenopathy) suggest clarifying the possible implication of this chemokine in pSS pathogenesis in larger studies.

  16. Functional Analysis of Dendritic Cells Generated from T-iPSCs from CD4+ T Cell Clones of Sjögren's Syndrome.

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    Iizuka-Koga, Mana; Asashima, Hiromitsu; Ando, Miki; Lai, Chen-Yi; Mochizuki, Shinji; Nakanishi, Mahito; Nishimura, Toshinobu; Tsuboi, Hiroto; Hirota, Tomoya; Takahashi, Hiroyuki; Matsumoto, Isao; Otsu, Makoto; Sumida, Takayuki

    2017-05-09

    Although it is important to clarify the pathogenic functions of T cells in human samples, their examination is often limited due to difficulty in obtaining sufficient numbers of dendritic cells (DCs), used as antigen-presenting cells, especially in autoimmune diseases. We describe the generation of DCs from induced pluripotent stem cells derived from T cells (T-iPSCs). We reprogrammed CD4+ T cell clones from a patient with Sjögren's syndrome (SS) into iPSCs, which were differentiated into DCs (T-iPS-DCs). T-iPS-DCs had dendritic cell-like morphology, and expressed CD11c, HLA-DR, CD80, CD86, and also BDCA-3. Compared with monocyte-derived DCs, the capacity for antigen processing was similar, and T-iPS-DCs induced the proliferative response of autoreactive CD4+ T cells. Moreover, we could evaluate T cell functions of the patient with SS. In conclusion, we obtained adequate numbers of DCs from T-iPSCs, which could be used to characterize pathogenic T cells in autoimmune diseases such as SS. Copyright © 2017 The Author(s). Published by Elsevier Inc. All rights reserved.

  17. Inhibitory Effects of iPSC-MSCs and Their Extracellular Vesicles on the Onset of Sialadenitis in a Mouse Model of Sjögren’s Syndrome

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    Bo Hai

    2018-01-01

    Full Text Available No effective treatment for Sjögren’s syndrome (SS, a chronic autoimmune disease affecting mainly salivary and lacrimal glands, is available now. Systemic infusion of allogeneic mesenchymal stem cells (MSCs isolated from tissues such as bone marrow (BM alleviated SS in mouse models and a small clinical trial, but further research and application of this MSC therapy were hindered by limited expandability, significant donor variations, and safety concerns of tissue-derived MSCs. To circumvent these issues, we derived MSCs from human iPSCs using an optimized protocol that can be easily scaled up to produce a huge amount of standardized MSCs. Our iPSC-MSCs inhibited the onset of lymphocyte infiltration into salivary glands in the NOD mouse model of SS in the same way as BM-MSCs. Extracellular vesicles (EVs carry bioactive molecules in the same way as their originating cells and are more stable and considered much safer than cells for therapies. We found that EVs derived from BM-MSCs and iPSC-MSCs suppressed activation of immune cells and expression of proinflammation factors essential for SS progression in vitro and that infusion of iPSC-MSC EVs at the predisease stage decreased the lymphocyte infiltration in salivary glands and serum autoantibody levels in the same way as infusion of BM-MSCs and iPSC-MSCs. These data suggested that iPSC-MSC EVs have the potential to prevent the progression of SS before the onset of sialadenitis.

  18. Functional Analysis of Dendritic Cells Generated from T-iPSCs from CD4+ T Cell Clones of Sjögren's Syndrome

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    Mana Iizuka-Koga

    2017-05-01

    Full Text Available Although it is important to clarify the pathogenic functions of T cells in human samples, their examination is often limited due to difficulty in obtaining sufficient numbers of dendritic cells (DCs, used as antigen-presenting cells, especially in autoimmune diseases. We describe the generation of DCs from induced pluripotent stem cells derived from T cells (T-iPSCs. We reprogrammed CD4+ T cell clones from a patient with Sjögren's syndrome (SS into iPSCs, which were differentiated into DCs (T-iPS-DCs. T-iPS-DCs had dendritic cell-like morphology, and expressed CD11c, HLA-DR, CD80, CD86, and also BDCA-3. Compared with monocyte-derived DCs, the capacity for antigen processing was similar, and T-iPS-DCs induced the proliferative response of autoreactive CD4+ T cells. Moreover, we could evaluate T cell functions of the patient with SS. In conclusion, we obtained adequate numbers of DCs from T-iPSCs, which could be used to characterize pathogenic T cells in autoimmune diseases such as SS.

  19. The TRACTISS Protocol: a randomised double blind placebo controlled clinical TRial of Anti-B-Cell Therapy In patients with primary Sjögren’s Syndrome

    Science.gov (United States)

    2014-01-01

    Background Primary Sjögren’s Syndrome (PSS) mainly affects women (9:1 female:male ratio) and is one of the commonest autoimmune diseases with a prevalence of 0.1 – 0.6% of adult women. For patients with PSS there is currently no effective therapy that can alter the progression of the disease. The aim of the TRACTISS study is to establish whether in patients with PSS, treatment with rituximab improves clinical outcomes. Methods/design TRACTISS is a UK multi-centre, double-blind, randomised, controlled, parallel group trial of 110 patients with PSS. Patients will be randomised on a 1:1 basis to receive two courses of either rituximab or placebo infusion in addition to standard therapy, and will be followed up for up to 48 weeks. The primary objective is to assess the extent to which rituximab improves symptoms of fatigue and oral dryness. Secondary outcomes include ocular dryness, salivary flow rates, lacrimal flow, patient quality of life, measures of disease damage and disease activity, serological and peripheral blood biomarkers, and glandular histology and composition. Discussion The TRACTISS trial will provide direct evidence as to whether rituximab in patients with PSS leads to an improvement in patient symptoms and a reduction in disease damage and activity. Trial registration UKCRN Portfolio ID: 9809 ISRCTN65360827. PMID:24438039

  20. Plasmid-free CRISPR/Cas9 genome editing in Plasmodium falciparum confirms mutations conferring resistance to the dihydroisoquinolone clinical candidate SJ733.

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    Emily D Crawford

    Full Text Available Genetic manipulation of the deadly malaria parasite Plasmodium falciparum remains challenging, but the rise of CRISPR/Cas9-based genome editing tools is increasing the feasibility of altering this parasite's genome in order to study its biology. Of particular interest is the investigation of drug targets and drug resistance mechanisms, which have major implications for fighting malaria. We present a new method for introducing drug resistance mutations in P. falciparum without the use of plasmids or the need for cloning homologous recombination templates. We demonstrate this method by introducing edits into the sodium efflux channel PfATP4 by transfection of a purified CRISPR/Cas9-guide RNA ribonucleoprotein complex and a 200-nucleotide single-stranded oligodeoxynucleotide (ssODN repair template. Analysis of whole genome sequencing data with the variant-finding program MinorityReport confirmed that only the intended edits were made, and growth inhibition assays confirmed that these mutations confer resistance to the antimalarial SJ733. The method described here is ideally suited for the introduction of mutations that confer a fitness advantage under selection conditions, and the novel finding that an ssODN can function as a repair template in P. falciparum could greatly simplify future editing attempts regardless of the nuclease used or the delivery method.

  1. Functional effects of proinflammatory factors present in Sjögren's syndrome salivary microenvironment in an in vitro model of human salivary gland.

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    Arce-Franco, Mayte; Dominguez-Luis, María; Pec, Martina K; Martínez-Gimeno, Carlos; Miranda, Pablo; Alvarez de la Rosa, Diego; Giraldez, Teresa; García-Verdugo, José María; Machado, José David; Díaz-González, Federico

    2017-09-19

    Primary Sjögren's syndrome (pSS) is an autoimmune exocrinopathy in which the role that the immune response plays in reducing exocrine gland function, including the glandular microenvironment of cytokines, has not been fully understood. Epithelial cells from biopsies of human parotid gland (HPG) were used to establish a model of human salivary gland in vitro. In this model, the functional consequences of several proinflammatory soluble factors present in the pSS glandular microenvironment were assessed. Stimulation with isoproterenol and calcium produced a significant increase in the basal activity of amylase in the HPG cell supernatants. Under these conditions, the presence of TNF-α and CXCL12 increased amylase mRNA cellular abundance, but reduced the amylase activity in the cell-free supernatant in a dose-dependent manner. IL-1β and IFN-γ, but not TGF-β, also diminished amylase secretion by HPG cells. These results suggest that the glandular microenvironment of cytokine, by acting post-transcriptionally, may be responsible, at least in part, for the reduced exocrine function observed in pSS patients. These data may help to a better understanding of the pathogenesis of SS, which in turn would facilitate the identification of new therapeutic targets for this disorder.

  2. Joseph Knabenbauer SJ (1839–1911 a otázka evolučního vzniku člověka

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    Ctirad V. Pospíšil

    2017-08-01

    Full Text Available The paper is a part of the research that focuses on the Catholic theologians and biologists between 1871 and 1910 who accepted Mivart’s thesis of the evolutionary origin of the human body, or who, on the contrary, tended to reject it. The paper presents J. Knabenbauer SJ (1839–1911. In the 1930s, Czech theologian J. Miklik asserted that Knabenauer accepted Mivart’s position. Knabenbauer’s study on evolution is analysed. Already in 1877, Knabenbauer shows, in the first two parts of his work, an openness to the reality of evolutionary origin of species in the fauna and flora. In the third part, he deals with Mivart’s thesis. From the philosophical point of view, Knabenbauer admits the possibility of the creation of human body by way of evolution. From the exegetical point of view, he refuses the reading of Gen 2:7 that considered this verse to be an allusion of the origin of the human body from the animal base. Because of this later view, Miklik’s information is to be considered imprecise. Knnabenbauer’s study contains, though, much relevant information that testifies to the Catholic theology and exegesis of his era.

  3. Significance and Implications of Patient-reported Xerostomia in Sjögren's Syndrome: Findings From the National Institutes of Health Cohort.

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    Billings, Monisha; Dye, Bruce A; Iafolla, Timothy; Baer, Alan N; Grisius, Margaret; Alevizos, Ilias

    2016-10-01

    Xerostomia is a chief complaint of patients with Sjögren's syndrome (SS). However, newer proposals for SS classification remove xerostomia and hyposalivation from the criteria list. Given these developments and the importance of patient-centered research outcomes, we sought to evaluate the utility of patient-reported xerostomia with implications for classification criteria, and clinical trials targeting SS treatment modalities. A nested case-control study was designed within The National Institute of Dental and Craniofacial Research/National Institutes of Health (NIDCR/NIH) SS Cohort - one of the largest SS cohorts in the US. Clinical characteristics of those with and without xerostomia in SS and other salivary gland dysfunctions were compared. Several analytical methods were employed, including multivariable logistic regression modeling. The NIDCR/NIH Sjögren's Syndrome Clinic has an open cohort with ongoing enrollment since 1984. This open cohort comprised of 2046 participants by August 27, 2015. Baseline data of 701 SS, 355 Sicca, and 247 ISS participants within the source cohort were analyzed. Xerostomia was highest among SS participants (87.4%, 95% CI: 84.8%-89.8%) compared to Sicca (72.4%, 95% CI: 67.4%-77.0%, pxerostomia were more likely to have SS than Sicca/ISS (OR=4.98, 95% CI: 3.78-6.56). The ability of xerostomia to screen for SS among those with salivary gland dysfunction was higher than screening for Sicca/ISS. Screening diagnostics of xerostomia were of greater utility compared to hyposalivation. After adjusting for confounding in multivariable modeling, SS participants with xerostomia were more likely to be White (Black/African Americans (OR: 0.40, 95% CI: 0.23-0.68, p-value=0.001) and Asians (OR: 0.49, 95% CI: 0.25-0.96, p-value=0.038) were less likely to have xerostomia compared to Whites), have dry eye symptoms for >3months (OR: 5.80, 95% CI: 3.62-9.28, p-value 2 (OR: 1.92, 95% CI: 1.20-3.09, p-value=0.007), and salivary gland swelling (OR: 49

  4. Assessment of interstitial lung disease in Sjögren's syndrome by lung ultrasound: a pilot study of correlation with high-resolution chest tomography.

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    Vasco, Pablo Guisado; de Luna Cardenal, Gonzalo; Garrido, Isabel Martín; Pinilla, José Manuel Luque; Rodríguez, Guadalupe Fraile; Mateo, Juan José Nava; Ruiz, Daniel Carnevalli

    2017-04-01

    The background of this study is to assess the accuracy of lung ultrasound (LUS) to diagnose interstitial lung disease (ILD) in Sjögren's syndrome (Sjs), in patients who have any alterations in pulmonary function tests (PFT) or respiratory symptoms. LUS was correlated with chest tomography (hrCT), considering it as the imaging gold standard technique to diagnose ILD. This is a pilot, multicenter, cross-sectional, and consecutive-case study. The inclusion criteria are ≥18 years old, Signs and symptoms: according to ACEG 2002 criteria, respiratory symptoms (dyspnea, cough), or any alterations in PFR. LUS was done following the International Consensus Conference on Lung Ultrasound protocol for interstitial syndrome (B pattern). Of the 50 patients in follow-up, 13 (26%) met the inclusion criteria. All were women with age 63.62 years (range 39-88). 78.6% of the cases had primary Sjs (SLE, RA, n = 2). The intra-rater reliability k is 1, according to Gwet's Ac1 and GI index (probability to concordance-e(K)-, by Cohen, of 0.52). LUS has a sensitivity of 1 (95% CI 0.398-1.0), specificity of 0.89 (95% CI 0.518-0.997), and a positive probability reason of 9.00 (95% CI 7.1-11.3) to detect ILD. The correlation of Pearson is r = 0.84 (p < 0.001). To check the accuracy of LUS to diagnose ILD, a completely bilateral criterion of yes/no for interstitial pattern was chosen, AUC reaches significance, 0.94 (0.07) (95% CI 0.81-1.0, p = 0.014). LUS reaches an excellent correlation to hrCT in Sjs affected with ILD, and might be a useful technique in daily clinical practice for the assessment of pulmonary disease in the sicca syndrome.

  5. An in silico approach reveals associations between genetic and epigenetic factors within regulatory elements in B cells from primary Sjögren’s syndrome patients

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    Orsia D. Konsta

    2015-08-01

    Full Text Available Recent advances in genetics have highlighted several regions and candidate genes associated with primary Sjögren's syndrome (SS, a systemic autoimmune epithelitis that combines exocrine gland dysfunctions, and focal lymphocytic infiltrations. In addition to genetic factors, it is now clear that epigenetic deregulations are present during SS and restricted to specific cell type subsets such as lymphocytes and salivary gland epithelial cells. In this study, 72 single nucleotide polymorphisms (SNPs associated with 43 SS gene risk factors were selected from publicly available and peer reviewed literature for further in silico analysis. SS risk variant location was tested revealing a broad distribution in coding sequences (5.6%, intronic sequences (55.6%, upstream/downstream genic regions (30.5%, and intergenic regions (8.3%. Moreover, a significant enrichment of regulatory motifs (promoter, enhancer, insulator, DNAse peak and eQTL characterizes SS risk variants (94.4%. Next, screening SNPs in high linkage disequilibrium (r2 ≥ 0.8 in Caucasians revealed 645 new variants including 5 SNPs with missense mutations, and indicated an enrichment of transcriptionally active motifs according to the cell type (B cells > monocytes > T cells >> A549. Finally, we looked at SS risk variants for histone markers in B cells (GM12878, monocytes (CD14+ and epithelial cells (A548. Active histone markers were associated with SS risk variants at both promoters and enhancers in B cells, and within enhancers in monocytes. In conclusion and based on the obtained in silico results, that need further confirmation, associations were observed between SS genetic risk factors and epigenetic factors and these associations predominate in B cells such as those observed at the FAM167A-BLK locus.

  6. Isolated congenital heart block in undifferentiated connective tissue disease and in primary Sjögren’s syndrome: a clinical study of 81 pregnancies in 41 patients

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    S. Todesco

    2011-09-01

    Full Text Available Objective: To study the incidence and the features of congenital heart block (CHB in patients with undifferentiated connective tissue disease (UCTD and primary Sjögren’s syndrome (pSS. Methods: We studied 81 pregnancies of 41 women attending the Outpatients’ Clinic of the Rheumatology Unit of University Hospital of Padova from July 1989 to March 2004. Twenty five of these (61% were affected with UCTD and 16 (39% with pSS. Serologic inclusion criteria was anti-Ro/La positivity, assessed by counterimmunoelectrophoresis and ELISA. Results: CHB was found in 2 out of the 46 (4,3% pregnancies followed by our Staff and in 2 out of the 35 (5,7% included in the retrospective part of the study. In 3 cases CHB was a 3rd degree block, causing pregnancy termination in 2. The only 2nd degree block was identified in one patient at the 22nd week of gestation and treated with dexamethasone and plasma-exchange. All of the women were positive to 52 kd and 60 kd Ro autoantibodies. CHB mothers had higher titer antibodies to 52 kd Ro protein than did the mothers with healthy infants (P = 0,026. Electrocardiographic abnormalities at birth were found in 3 out of 29 asymptomatic infants. One presented sinus bradycardia, the second abnormalities of ventricular repolarization, both regressed spontaneously, while the third ventricular extrasystoles which continue even now at 5 months. Conclusion: These results showed that in UCTD and pSS there is a higher incidence of CHB than that reported in Systemic Lupus Erythematosus. Electrocardiographic screening in all infants born to mothers with anti-Ro/La antibodies would seem an important measure to identify those with irreversible heart conduction abnormalities.

  7. The Prevalence of Dental Implants and Related Factors in Patients with Sjögren Syndrome: Results from a Cohort Study.

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    Albrecht, Katinka; Callhoff, Johanna; Westhoff, Gisela; Dietrich, Thomas; Dörner, Thomas; Zink, Angela

    2016-07-01

    To investigate prevalence and patient-reported outcomes of dental implants in patients with Sjögren syndrome (SS). A total of 205 female patients from an observational cohort study answered oral health questionnaires about periodontal signs and symptoms, dentures, dental implants, comorbidities, and therapies that may interfere with bone remodeling. Data were compared with the reports of 87 female healthy controls. The patients were older than the controls (58 ± 12 and 54 ± 14 yrs, respectively) and differed substantially in the prevalence of self-reported gingivitis (60% and 35%), self-reported periodontitis (19% and 8%), and in the numbers of remaining teeth (21 ± 7 and 24 ± 5). Patients more frequently had removable prostheses (36% compared with 23%) and dental implants (16% compared with 7%). The 32 patients with SS with dental implants had a mean number of 3.1 ± 2.0 implants. Notably, for patients with implants, their oldest existing implant survived for a mean period of 4.9 ± 5.4 years. A total of 5 of 104 (4.8%) implants in the patients and none of the 14 implants in the controls had to be removed. A total of 75% of the patients were highly satisfied with the implants and 97% would recommend them to other patients with SS. A substantial portion of patients with SS have dental complications and require subsequent implants. The majority were satisfied with the implants and would recommend them to other patients. The high implant survival rate may encourage patients, rheumatologists, and dentists to consider dental implants for the treatment of patients with SS.

  8. Characteristics of primary Sjögren's syndrome patients with IgG4 positive plasma cells infiltration in the labial salivary glands.

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    Liu, Chang; Zhang, Huayong; Yao, Genhong; Hu, Yunxia; Qi, Jingjing; Wang, Yan; Chen, Weiwei; Tang, Xiaojun; Li, Wenchao; Lu, Liwei; Gu, Luo; Sun, Lingyun

    2017-01-01

    The purpose of this study was to investigate the characteristics of primary Sjögren's syndrome (pSS) patients with IgG4 positive (IgG4 + ) plasma cell infiltration in labial salivary glands (LSGs). Paraffin sections of LSGs from 336 pSS patients were stained with IgG4 and IgG monoclonal antibodies. According to the infiltration of IgG4 + plasma cells, patients were divided and clinical and serological characteristics were analyzed and compared. Based on the infiltration of IgG4 + plasma cells in the LSGs, patients were divided into three subgroups, low IgG4, moderate IgG4, and high IgG4 groups. A negative association between the number of infiltrated IgG4 + plasma cells and the disease characteristics was observed. We found that the higher the IgG4 + expression in plasma cells, the lower the positive rates of serum anti-SSA antibodies, anti-SSB antibodies, antinuclear antibodies (ANA), and rheumatoid factor (RF). Besides, patients from the high IgG4 group had the highest frequency of interstitial lung disease (ILD, 30.6%) and tubulointerstitial nephritis (TIN, 13.9%), but the lowest frequency of leucopenia (13.9%), thrombocytopenia (11.1%), and abnormal thyroidal function (0%). PSS patients with different IgG4 + plasma cells infiltration in the LSGs had distinctive clinical and laboratory characteristics. It may help us to further understand the role of IgG4 + plasma cells in pSS.

  9. Identification of a Sjögren's syndrome susceptibility locus at OAS1 that influences isoform switching, protein expression, and responsiveness to type I interferons.

    Science.gov (United States)

    Li, He; Reksten, Tove Ragna; Ice, John A; Kelly, Jennifer A; Adrianto, Indra; Rasmussen, Astrid; Wang, Shaofeng; He, Bo; Grundahl, Kiely M; Glenn, Stuart B; Miceli-Richard, Corinne; Bowman, Simon; Lester, Sue; Eriksson, Per; Eloranta, Maija-Leena; Brun, Johan G; Gøransson, Lasse G; Harboe, Erna; Guthridge, Joel M; Kaufman, Kenneth M; Kvarnström, Marika; Cunninghame Graham, Deborah S; Patel, Ketan; Adler, Adam J; Farris, A Darise; Brennan, Michael T; Chodosh, James; Gopalakrishnan, Rajaram; Weisman, Michael H; Venuturupalli, Swamy; Wallace, Daniel J; Hefner, Kimberly S; Houston, Glen D; Huang, Andrew J W; Hughes, Pamela J; Lewis, David M; Radfar, Lida; Vista, Evan S; Edgar, Contessa E; Rohrer, Michael D; Stone, Donald U; Vyse, Timothy J; Harley, John B; Gaffney, Patrick M; James, Judith A; Turner, Sean; Alevizos, Ilias; Anaya, Juan-Manuel; Rhodus, Nelson L; Segal, Barbara M; Montgomery, Courtney G; Scofield, R Hal; Kovats, Susan; Mariette, Xavier; Rönnblom, Lars; Witte, Torsten; Rischmueller, Maureen; Wahren-Herlenius, Marie; Omdal, Roald; Jonsson, Roland; Ng, Wan-Fai; Nordmark, Gunnel; Lessard, Christopher J; Sivils, Kathy L

    2017-06-01

    Sjögren's syndrome (SS) is a common, autoimmune exocrinopathy distinguished by keratoconjunctivitis sicca and xerostomia. Patients frequently develop serious complications including lymphoma, pulmonary dysfunction, neuropathy, vasculitis, and debilitating fatigue. Dysregulation of type I interferon (IFN) pathway is a prominent feature of SS and is correlated with increased autoantibody titers and disease severity. To identify genetic determinants of IFN pathway dysregulation in SS, we performed cis-expression quantitative trait locus (eQTL) analyses focusing on differentially expressed type I IFN-inducible transcripts identified through a transcriptome profiling study. Multiple cis-eQTLs were associated with transcript levels of 2'-5'-oligoadenylate synthetase 1 (OAS1) peaking at rs10774671 (PeQTL = 6.05 × 10-14). Association of rs10774671 with SS susceptibility was identified and confirmed through meta-analysis of two independent cohorts (Pmeta = 2.59 × 10-9; odds ratio = 0.75; 95% confidence interval = 0.66-0.86). The risk allele of rs10774671 shifts splicing of OAS1 from production of the p46 isoform to multiple alternative transcripts, including p42, p48, and p44. We found that the isoforms were differentially expressed within each genotype in controls and patients with and without autoantibodies. Furthermore, our results showed that the three alternatively spliced isoforms lacked translational response to type I IFN stimulation. The p48 and p44 isoforms also had impaired protein expression governed by the 3' end of the transcripts. The SS risk allele of rs10774671 has been shown by others to be associated with reduced OAS1 enzymatic activity and ability to clear viral infections, as well as reduced responsiveness to IFN treatment. Our results establish OAS1 as a risk locus for SS and support a potential role for defective viral clearance due to altered IFN response as a genetic pathophysiological basis of this complex autoimmune disease.

  10. Identification of a Sjögren's syndrome susceptibility locus at OAS1 that influences isoform switching, protein expression, and responsiveness to type I interferons.

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    He Li

    2017-06-01

    Full Text Available Sjögren's syndrome (SS is a common, autoimmune exocrinopathy distinguished by keratoconjunctivitis sicca and xerostomia. Patients frequently develop serious complications including lymphoma, pulmonary dysfunction, neuropathy, vasculitis, and debilitating fatigue. Dysregulation of type I interferon (IFN pathway is a prominent feature of SS and is correlated with increased autoantibody titers and disease severity. To identify genetic determinants of IFN pathway dysregulation in SS, we performed cis-expression quantitative trait locus (eQTL analyses focusing on differentially expressed type I IFN-inducible transcripts identified through a transcriptome profiling study. Multiple cis-eQTLs were associated with transcript levels of 2'-5'-oligoadenylate synthetase 1 (OAS1 peaking at rs10774671 (PeQTL = 6.05 × 10-14. Association of rs10774671 with SS susceptibility was identified and confirmed through meta-analysis of two independent cohorts (Pmeta = 2.59 × 10-9; odds ratio = 0.75; 95% confidence interval = 0.66-0.86. The risk allele of rs10774671 shifts splicing of OAS1 from production of the p46 isoform to multiple alternative transcripts, including p42, p48, and p44. We found that the isoforms were differentially expressed within each genotype in controls and patients with and without autoantibodies. Furthermore, our results showed that the three alternatively spliced isoforms lacked translational response to type I IFN stimulation. The p48 and p44 isoforms also had impaired protein expression governed by the 3' end of the transcripts. The SS risk allele of rs10774671 has been shown by others to be associated with reduced OAS1 enzymatic activity and ability to clear viral infections, as well as reduced responsiveness to IFN treatment. Our results establish OAS1 as a risk locus for SS and support a potential role for defective viral clearance due to altered IFN response as a genetic pathophysiological basis of this complex autoimmune disease.

  11. Sjögren's syndrome: managed care data from a large United States population highlight real-world health care burden and lack of treatment options.

    Science.gov (United States)

    Birt, Julie A; Tan, YingMeei; Mozaffarian, Neelufar

    2017-01-01

    To better understand the real-world characteristics and costs of Sjögren's syndrome (SS). Analysing the MarketScan Commercial Claims database from Jan. 1, 2006 to Dec. 31, 2011, we identified 10,414 patients ≥18 years old newly diagnosed with SS. Patient characteristics, drugs (commonly used for SS), resource utilisation, and medical costs were evaluated for 12 months pre- and post-diagnosis. Mean age was 55 years; 90% were female. At diagnosis, SS patients were most often seen by rheumatologists (39%) or internists (14.2%); the most common concurrent autoimmune conditions were rheumatoid arthritis (17.9%) and systemic lupus erythematosus (14.6%). Other common comorbidities were hypertension (37.6%), osteoarthritis (31.4%), and hyperlipidaemia/dyslipidaemia (30.3%). Post diagnosis of SS, claims for myocardial infarction and coronary artery bypass graft doubled. Medications of interest prescribed post-diagnosis were eye/mouth drugs (32.2%) and synthetic immunosuppressants (32.1%). Biologic drugs were prescribed to a minority (TNF inhibitors, ~5.0%; non-TNF inhibitors, 1%). Of note, prescriptions for all systemic immunotherapies (synthetic and biologic) were significantly lower in the subgroup without concurrent autoimmune disease, and 15.1% of the overall population had no SS-related prescriptions. Post diagnosis, total medical resource utilisation and total medical costs increased (1.2 and 1.4-fold, respectively). In this retrospective, real-world analysis, medical claims in the first year after SS diagnosis revealed that cardiovascular (CV) events increased and all-cause healthcare costs grew by 40%. Pharmacologic management consisted primarily of low potency immunomodulation and symptomatic treatments. Systemic disease-modifying therapies were used mostly in patients who had another concurrent autoimmune disease, suggesting a lack of treatment options for SS.

  12. Salivary gland ultrasonography as a primary imaging tool for predicting efficacy of xerostomia treatment in patients with Sjögren's syndrome.

    Science.gov (United States)

    Takagi, Yukinori; Sumi, Misa; Nakamura, Hideki; Sato, Shuntaro; Kawakami, Atsushi; Nakamura, Takashi

    2016-02-01

    To evaluate ultrasonography (US) grading of salivary gland disease as a predictor of treatment efficacy for impaired salivary function in xerostomia patients with or without Sjögren's syndrome (SS). We retrospectively analysed the prognostic importance of salivary US grading in 317 patients (168 with SS and 149 without SS). US images of the parotid and submandibular glands in each patient were individually categorized into grades 0-4 based on the extent of damage to the gland; and the sum total grade of the two gland types on either side was assigned a US score of 0-8 for each patient. The relative importance of US score and demographic and clinical variables was assessed using stepwise multiple regression analysis after various durations of xerostomia treatment. Multiple regression analysis indicated that the baseline US score before treatment was the most important factor [standardized regression coefficient (β) = -0.523, t-statistic (t) = -7.967, P < 0.001] in predicting negative outcomes in SS patients. Treatment duration (β = 0.277, t = 4.225, P < 0.001) was also a significant but less important positive variable. On the other hand, US grading did not effectively predict treatment outcomes in non-SS patients, with treatment duration (β = 0.199, t = 2.486, P = 0.014) and baseline salivary flow rate before treatment (β = -0.172, t = -2.159, P = 0.032) being significant but weak predictors of positive and negative outcome, respectively. Salivary gland US grading may help to predict outcomes of treatment for impaired salivary function in patients with SS. © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  13. New low-dose liquid pilocarpine formulation for treating dry mouth in Sjögren's syndrome: clinical efficacy, symptom relief, and improvement in quality of life.

    Science.gov (United States)

    Watanabe, Machiko; Yamada, Chisato; Komagata, Yoshinori; Kikuchi, Hirotoshi; Hosono, Hiroyuki; Itagaki, Fumio

    2018-01-01

    Patients with Sjögren's syndrome (SS) typically present clinically with xerostomia (dry mouth) because of progressive damage to the exocrine glands. We developed a new, low-dose pilocarpine/sodium alginate (LPA) solution with pilocarpine hydrochloride to inhibit systemic adverse effects by administering via the oral mucosa. The purpose of this study was to assess its stability, safety, and efficacy. The pilocarpine concentration in an LPA liquid formulation was measured 3, 7, 14, and 28 days after preparation to assess its stability. A prospective clinical trial was undertaken to assess the efficacy and safety of the LPA solution as a symptomatic treatment for dry mouth in SS. Patients ( n  = 24) with clinically significant xerostomia were enrolled after providing written informed consent. Whole-mouth salivary flow rate was measured twice; immediately before and 60 min after LPA application. Symptoms were assessed by questionnaire with visual analog scales or checkboxes before the first application (baseline), and then once daily for 7 days. The pilocarpine content 3, 7, 14, and 28 days after preparation showed no marked change, confirming its stability. Salivary flow was significantly increased from 0.076 ± 0.092 g/30 s to 0.122 ± 0.140 g/30 s 60 min after LPA administration ( P  Dry mouth and thirstiness showed significant improvement compared with that of baseline ( P  ≤ 0.01). The only adverse effect was sweating, and no serious drug-related adverse events were reported. This new, low-dose pilocarpine formulation was well-tolerated and resulted in significant improvements in symptoms of dry mouth and other xerostomic conditions in patients with SS. The study approval number in the institution; 08-068-2. Registered January 19, 2009. UMIN000029307. Registered 27 September 2017 (retrospectively registered).

  14. The efficacy of topical 0.05 % cyclosporine A in patients with dry eye disease associated with Sjögren's syndrome.

    Science.gov (United States)

    Devecı, Hülya; Kobak, Senol

    2014-10-01

    Sjögren's syndrome (SS) is an autoimmune epithelitis which usually presents with mouth and eye dryness. Although the place of systemic drugs in keratoconjunctivitis sicca treatment has been discussed, the efficacy of some topical drugs has also been demonstrated; however, there are contradictory results related to topical cyclosporine A. We aimed to investigate the efficacy of 0.05 % topical cyclosporine A in patients with keratoconjunctivitis sicca due to primary and secondary SS. This prospective study included 26 patients with a diagnosis of primary and secondary SS who visited our rheumatology outpatient clinic. Keratoconjunctivitis sicca was diagnosed in all patients after they were examined at the outpatient clinic. Patients were given topical 0.05 % cyclosporine A emulsions for both eyes. We used another 20 patients with SS who were treated with saline solution as a control group. Subjective symptoms reported after 1-week and 1-month follow-up were complaints of burning and pricking sensation, light sensitivity and pain. Objective signs included redness, Schirmer test and tear break-up time. A total of 26 patients (19 female) were enrolled in the study with a mean age of 47.5 years and mean disease duration of 5.2 years. In the first physical examination of patients, 23 patients had burning and pricking sensation, 24 had pain, 23 had light sensitivity, and 24 had red eyes. All subjective symptoms (burning and pricking sensation, light sensitivity and pain) were statistically significantly improved after 1-week and 1-month follow-up examinations (p = 0.0001). All objective signs (Schirmer test, tear break-up time, and redness) were statistically significantly improved after 1-week and 1-month follow-up examinations (p = 0.0001). Compared with the control group, there was significant improvement in all parameters. It is concluded that topical 0.05 % cyclosporine A is an effective treatment option for keratoconjunctivitis sicca due to SS after a 1-month follow

  15. Managing the care of patients with Sjögren syndrome and dry mouth: comorbidities, medication use and dental care considerations.

    Science.gov (United States)

    Donaldson, Mark; Epstein, Joel; Villines, Dana

    2014-12-01

    As North Americans live longer, have more chronic conditions and take more medications, adverse oral events are likely to increase and aggravate the symptoms of Sjögren syndrome (SS). A total of 151 adults who self-reported having SS and who had a mean (standard deviation [SD]) age of 65.8 (11.5) years completed a survey that included questions about basic demographic information, current medical conditions, medications used (prescription and over the counter [OTC]) and the use of oral products to manage SS symptoms. Owing to the self-reporting process in our survey, the term "SS" in our study population represented a mixture of people with SS and people with dry mouth symptoms. The mean (SD) number of daily medications recorded as prescription, OTC and oral care products were 4.9 (3.5), 4.5 (2.8) and 4.6 (1.4), respectively. Participants with four or more comorbid medical conditions (n = 74; 49.0 percent) had significant differences (P < .05) in oral symptoms compared with those who had fewer than four (n = 75; 49.7 percent). Participants who were taking fewer than four prescription and OTC medications daily (n = 61; 40.4 percent) has significant differences (P < .05) in voice hoarseness compared with those taking four or more prescription and OTC medications daily (n = 54; 35.8 percent). The survey results indicated that medication use and comorbid medical conditions demonstrated significant differences and may have had a substantial impact on the oral symptoms in adults who self-reported having SS.

  16. Estudio de marcadores biológicos y culturales del sexo en un conjunto hispano-indígena de Tilcara (sitio SJ Til. 43

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    Barboza, María Carolina

    2002-01-01

    Full Text Available En la investigación arqueológica, el potencial para la interpretación de los restos óseos humanos está directamente vinculado con las condiciones de hallazgo, recuperación y asociación contextual. La excavación del Cementerio de La Falda (SJ Til. 43 Tilcara, Jujuy, proporcionó un conjunto de treinta y un individuos con alto grado de integridad y con asociaciones culturales que, entre otros aspectos, permiten la identificación de atributos propios de cada sexo. Desde el punto de vista bioantropológico, la manifestación de muchos rasgos anatómicos, tanto métricos como morfológicos puede resultar específica para cada población. Advertidos de que los patrones de dimorfismo sexual pueden variar entre grupos, el objetivo de este trabajo ha sido estudiar el comportamiento del patrón general de variabilidad sexual en los individuos adultos de La Falda. Para ello se realizó un estudio comparativo en el que se confronta la información cultural que permite identificar sexo, con aquella proporcionada por los marcadores óseos para determinación sexual. Nuestros resultados permiten comprobar la existencia de concordancias y discordancias en las asignaciones sexuales de índole osteológica, según el complejo o estructura anatómica considerada, las cuales son discutidas e interpretadas a la luz de la información tanto biológica como cultural disponible. Se presentan los marcadores osteológicos considerados más efectivos para esta muestra en particular.

  17. X Chromosome Dose and Sex Bias in Autoimmune Diseases: Increased 47,XXX in Systemic Lupus Erythematosus and Sjögren’s Syndrome

    Science.gov (United States)

    Liu, Ke; Kurien, Biji T.; Zimmerman, Sarah L.; Kaufman, Kenneth M.; Taft, Diana H.; Kottyan, Leah C.; Lazaro, Sara; Weaver, Carrie A.; Ice, John A.; Adler, Adam J.; Chodosh, James; Radfar, Lida; Rasmussen, Astrid; Stone, Donald U.; Lewis, David M.; Li, Shibo; Koelsch, Kristi A.; Igoe, Ann; Talsania, Mitali; Kumar, Jay; Maier-Moore, Jacen S.; Harris, Valerie M.; Gopalakrishnan, Rajaram; Jonsson, Roland; Lessard, James A.; Lu, Xianglan; Gottenberg, Jacques-Eric; Anaya, Juan-Manuel; Cunninghame-Graham, Deborah S.; Huang, Andrew J. W.; Brennan, Michael T.; Hughes, Pamela; Illei, Gabor G.; Miceli-Richard, Corinne; Keystone, Edward C.; Bykerk, Vivian P.; Hirschfield, Gideon; Xie, Gang; Ng, Wan-Fai; Nordmark, Gunnel; Eriksson, Per; Omdal, Roald; Rhodus, Nelson L.; Rischmueller, Maureen; Rohrer, Michael; Segal, Barbara M.; Vyse, Timothy J.; Wahren-Herlenius, Marie; Witte, Torsten; Pons-Estel, Bernardo; Alarcon-Riquelme, Marta E.; Guthridge, Joel M.; James, Judith A.; Lessard, Christopher J.; Kelly, Jennifer A.; Thompson, Susan D.; Gaffney, Patrick M.; Montgomery, Courtney G.; Edberg, Jeffrey C; Kimberly, Robert P; Alarcón, Graciela S.; Langefeld, Carl L.; Gilkeson, Gary S.; Kamen, Diane L.; Tsao, Betty P.; McCune, W. Joseph; Salmon, Jane E.; Merrill, Joan T.; Weisman, Michael H; Wallace, Daniel J; Utset, Tammy O; Bottinger, Erwin P.; Amos, Christopher I.; Siminovitch, Katherine A.; Mariette, Xavier; Sivils, Kathy L.

    2016-01-01

    Objective More than 80% of autoimmune disease is female dominant, but the mechanism for this female bias is poorly understood. We suspected an X chromosome dose effect and hypothesized that trisomy X (47,XXX , 1 in ~1,000 live female births) would be increased in female predominant diseases (e.g. systemic lupus erythematosus [SLE], primary Sjögren’s syndrome [SS], primary biliary cirrhosis [PBC] and rheumatoid arthritis [RA]) compared to diseases without female predominance (sarcoidosis) and controls. Methods We identified 47,XXX subjects using aggregate data from single nucleotide polymorphism (SNP) arrays and confirmed, when possible, by fluorescent in situ hybridization (FISH) or quantitative polymerase chain reaction (q-PCR). Results We found 47,XXX in seven of 2,826 SLE and three of 1,033 SS female patients, but only in two of the 7,074 female controls (p=0.003, OR=8.78, 95% CI: 1.67-86.79 and p=0.02, OR=10.29, 95% CI: 1.18-123.47; respectively). One 47,XXX subject was present for ~404 SLE women and ~344 SS women. 47,XXX was present in excess among SLE and SS subjects. Conclusion The estimated prevalence of SLE and SS in women with 47,XXX was respectively ~2.5 and ~2.9 times higher than in 46,XX women and ~25 and ~41 times higher than in 46,XY men. No statistically significant increase of 47,XXX was observed in other female-biased diseases (PBC or RA), supporting the idea of multiple pathways to sex bias in autoimmunity. PMID:26713507

  18. X Chromosome Dose and Sex Bias in Autoimmune Diseases: Increased Prevalence of 47,XXX in Systemic Lupus Erythematosus and Sjögren's Syndrome.

    Science.gov (United States)

    Liu, Ke; Kurien, Biji T; Zimmerman, Sarah L; Kaufman, Kenneth M; Taft, Diana H; Kottyan, Leah C; Lazaro, Sara; Weaver, Carrie A; Ice, John A; Adler, Adam J; Chodosh, James; Radfar, Lida; Rasmussen, Astrid; Stone, Donald U; Lewis, David M; Li, Shibo; Koelsch, Kristi A; Igoe, Ann; Talsania, Mitali; Kumar, Jay; Maier-Moore, Jacen S; Harris, Valerie M; Gopalakrishnan, Rajaram; Jonsson, Roland; Lessard, James A; Lu, Xianglan; Gottenberg, Jacques-Eric; Anaya, Juan-Manuel; Cunninghame-Graham, Deborah S; Huang, Andrew J W; Brennan, Michael T; Hughes, Pamela; Illei, Gabor G; Miceli-Richard, Corinne; Keystone, Edward C; Bykerk, Vivian P; Hirschfield, Gideon; Xie, Gang; Ng, Wan-Fai; Nordmark, Gunnel; Eriksson, Per; Omdal, Roald; Rhodus, Nelson L; Rischmueller, Maureen; Rohrer, Michael; Segal, Barbara M; Vyse, Timothy J; Wahren-Herlenius, Marie; Witte, Torsten; Pons-Estel, Bernardo; Alarcon-Riquelme, Marta E; Guthridge, Joel M; James, Judith A; Lessard, Christopher J; Kelly, Jennifer A; Thompson, Susan D; Gaffney, Patrick M; Montgomery, Courtney G; Edberg, Jeffrey C; Kimberly, Robert P; Alarcón, Graciela S; Langefeld, Carl L; Gilkeson, Gary S; Kamen, Diane L; Tsao, Betty P; McCune, W Joseph; Salmon, Jane E; Merrill, Joan T; Weisman, Michael H; Wallace, Daniel J; Utset, Tammy O; Bottinger, Erwin P; Amos, Christopher I; Siminovitch, Katherine A; Mariette, Xavier; Sivils, Kathy L; Harley, John B; Scofield, R Hal

    2016-05-01

    More than 80% of autoimmune disease predominantly affects females, but the mechanism for this female bias is poorly understood. We suspected that an X chromosome dose effect accounts for this, and we undertook this study to test our hypothesis that trisomy X (47,XXX; occurring in ∼1 in 1,000 live female births) would be increased in patients with female-predominant diseases (systemic lupus erythematosus [SLE], primary Sjögren's syndrome [SS], primary biliary cirrhosis, and rheumatoid arthritis [RA]) compared to patients with diseases without female predominance (sarcoidosis) and compared to controls. All subjects in this study were female. We identified subjects with 47,XXX using aggregate data from single-nucleotide polymorphism arrays, and, when possible, we confirmed the presence of 47,XXX using fluorescence in situ hybridization or quantitative polymerase chain reaction. We found 47,XXX in 7 of 2,826 SLE patients and in 3 of 1,033 SS patients, but in only 2 of 7,074 controls (odds ratio in the SLE and primary SS groups 8.78 [95% confidence interval 1.67-86.79], P = 0.003 and odds ratio 10.29 [95% confidence interval 1.18-123.47], P = 0.02, respectively). One in 404 women with SLE and 1 in 344 women with SS had 47,XXX. There was an excess of 47,XXX among SLE and SS patients. The estimated prevalence of SLE and SS in women with 47,XXX was ∼2.5 and ∼2.9 times higher, respectively, than that in women with 46,XX and ∼25 and ∼41 times higher, respectively, than that in men with 46,XY. No statistically significant increase of 47,XXX was observed in other female-biased diseases (primary biliary cirrhosis or RA), supporting the idea of multiple pathways to sex bias in autoimmunity. © 2016, American College of Rheumatology.

  19. Endogenous programmed death ligand-1 restrains the development and onset of Sjӧgren's syndrome in non-obese diabetic mice.

    Science.gov (United States)

    Zhou, Jing; Jin, Jun-O; Kawai, Toshihisa; Yu, Qing

    2016-12-14

    Programmed death-ligand 1 (PD-L1) down-modulates various immune responses by engaging the co-inhibitory receptor programmed death-1. Expression of PD-L1 and programmed death-1 is elevated in the salivary glands of patients with Sjögren's syndrome (SS). The objective of this study is to define the role of endogenous PD-L1 in SS pathogenesis in non-obese diabetic (NOD) mouse model of this disease. We inhibited endogenous PD-L1 function by intraperitoneal administration of a blocking antibody to 6 week-old female NOD/ShiLtJ mice repeatedly during a 9-day period. PD-L1 blockade accelerated leukocyte infiltration and caspase-3 activation in the submandibular gland (SMG), production of antinuclear and anti-M3 muscarinic acetylcholine receptor (M3R) autoantibodies and impairment of saliva secretion, indicative of accelerated development and onset of SS. The effect of PD-L1 blockade was associated with increased T- and B cells and T helper 1 cytokine IFN-γ in the SMG. Local administration of exogenous IFN-γ to the SMG led to impaired salivary secretion accompanied by down-regulation of aquaporin 5 and an increase in anti-M3R autoantibodies. Conversely, neutralization of IFN-γ markedly improved salivary secretion and aquaporin 5 expression in anti-PD-L1-treated NOD/ShiLtJ mice. Hence, endogenous PD-L1 hinders the development and onset of SS in NOD mice, in part by suppressing IFN-γ production.

  20. Health-related quality of life and psychological status of women with primary Sjögren's syndrome: A cross-sectional study of 304 Chinese patients.

    Science.gov (United States)

    Liu, Zhaoxiang; Dong, Zhenhua; Liang, Xiaochun; Liu, Jinhe; Xuan, Lei; Wang, Jing; Zhang, Gaili; Hao, Weixin

    2017-12-01

    Patients with primary Sjögren syndrome (pSS) always suffer from dryness, pain, and fatigue caused by the involvement of multiple different systems or organs. The uncomfortable disease symptoms, the consequent disability, and the side effects of therapeutic drugs decrease the quality of life and lead to emotional problems. We investigated the health-related quality of life and psychological status of a large cohort of women patients with pSS and associated factors.A total of 304 women with pSS referred to Peking Union Medical College Hospital during 2011 and 2014 were included. The internationally recognized Short Form (36) Health Survey (SF-36) was used to assess patients' quality of life; a higher score indicated a better quality of life. Patients' psychological status was assessed by the Hospital Anxiety and Depression Scale (HADS), and higher scores predicted more anxiety or depression.Patients with pSS had remarkably lower SF-36 scores. The Hospital Anxiety Scale (HAS) and Hospital Depression Scale (HDS) scores of the pSS patients (7 [4,10] and 6 [3,10], respectively) were significantly higher than that of patients with other internal diseases (3.37 ± 2.81 and 3.83 ± 3.14; both P quality of life were: pain (physical condition, β = -0.225; P quality of life with anxiety and depression. Pain and fatigue are primary factors for lower quality of life, which cause more anxiety and depression. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  1. Activation of MMP-9 activity by acrolein in saliva from patients with primary Sjögren's syndrome and its mechanism.

    Science.gov (United States)

    Uemura, Takeshi; Suzuki, Takehiro; Saiki, Ryotaro; Dohmae, Naoshi; Ito, Satoshi; Takahashi, Hoyu; Toida, Toshihiko; Kashiwagi, Keiko; Igarashi, Kazuei

    2017-07-01

    We have recently reported that the altered recognition patterns of immunoglobulins due to acrolein conjugation are at least partially responsible for autoimmune diseases in patients with primary Sjögren's syndrome (pSS). In the current study, it was found that the specific activity (activity/ng protein) of metalloproteinase-9 (MMP-9) in saliva was elevated about 2.4-fold in pSS patients. Accordingly, it was examined whether MMP-9 is activated by acrolein. It was found that the MMP-9 with 92kDa molecular weight was activated by acrolein. Under the conditions studied, Cys99, located in the propeptide, was conjugated with acrolein together with Cys230, 244, 302, 314, 329, 347, 361, 373, 388 and 516, which are located in fibronectin repeats and glycosyl domains, but not on the active site of MMP-9. In addition, 82 and 68kDa constructs of MMP-9s, lacking the NH 2 -terminal domain that contains Cys99, were not activated by acrolein. The results suggest that acrolein conjugation at Cys99 caused the active site of MMP-9 to be exposed. Activation of MMP-9 by acrolein was inhibited by cysteine, and slightly by lysine, because these amino acids inhibited acrolein conjugation with MMP-9. Conversely, MMP-9 activity in the presence of 50μM acrolein was enhanced by 100μM histidine. This was due to the inhibition of acrolein conjugation with His405 and 411 located at the Zn 2+ binding site of MMP-9. These results suggest that activation of 92kDa MMP-9 by acrolein is involved in tissue damage in pSS patients and is regulated by cysteine and histidine, and slightly by lysine. Activated 82 and 68kDa MMP-9s were not detected in saliva of pSS patients by Western blotting. Copyright © 2017 Elsevier Ltd. All rights reserved.

  2. Low Levels of IgG Recognizing the α-1-Antitrypsin Peptide and Its Association with Taiwanese Women with Primary Sjögren’s Syndrome

    Directory of Open Access Journals (Sweden)

    Yu-Sheng Chang

    2017-12-01

    Full Text Available The aim of this study was to examine oxidative stress and low level of α-1-antitrypsin (A1AT in primary Sjögren’s syndrome (pSS, and evaluate the associated autoreactivity against unmodified and their 4-hydroxy-2-nonenal (HNE-modified peptides with pSS. Two differentially expressed proteins, α-1-acid glycoprotein 1 (A1AG1 and A1AT, exhibited 2-fold differences, and their HNE modifications were identified by depleted-albumin and immunoglobulin G (IgG serum protein, in-solution digestion, in-gel digestion, and nano-liquid chromatography–tandem mass spectrometry (nano-LC-MS/MS from pSS patients and age-matched healthy controls (HCs. Furthermore, levels of proteins, confirmation of HNE modifications, HNE-protein adducts and autoreactivity against unmodified and their HNE-modified peptides were further validated. Levels of the HNE-protein adduct and A1AG1 were significantly higher in pSS patients than HCs, but levels of A1AT were significantly lower in pSS patients compared to HCs. Only the HNE modification of A1AT was confirmed. Our study suggests that elevated HNE-protein adduct, oxidative stress, level (odds ratio (OR 4.877, p = 0.003, lowered A1AT level (OR 3.910, p = 0.010 and a decreased level of anti-A1AT50–63 IgG (OR 3.360, p = 0.010 showed an increased risk in pSS patients compared to HCs, respectively.

  3. Challenges for Planning and Maintaining of Urban Rain Harvesting Systems- the case of Hammarby Sjöstad and Årstafältet in Stockholm

    Science.gov (United States)

    Suleiman, Lina

    2017-04-01

    Planners and policymakers' concern escalates over conventional systems dealing with rains in cities based on domination and control of nature rather than harmony and design with nature. A new spatial planning paradigm is needed to put in place systems which mimic natural water systems and promise multiple values instead of systems consider rain as a source of problem. However, such approach embodies significant planning challenges. Urban rain harvesting systems (URHs) are inherently viewed as 'sociotechnical' systems. As such, planning processes should consider the interdependence of 'social' and 'technical' aspects as essential elements if a transition towards sustainable urban water systems is to be realised. Drawing on a common understanding for what urban rain harvesting systems should deliver in terms of 'functions' and 'added values', a generic planning framework is developed to inform practitioners on how the 'socio' and 'technical' elements should be assimilated in a long-term and integrated planning processes of URHs. Using the developed framework, the paper examines the planning and maintenance processes of urban rain harvesting systems in Årstafältet and Hammarby Sjöstad respectively. Results show that planners lack a common operational understanding on how these systems should be designed holistically in a long term and integrated planning processes creating working gabs or positional conflicts. In practice, urban planners and water engineers look at these systems as either urban design component or water drainage system to deal with technical functions hindering a smooth transition path towards urban rain harvesting systems. The paper concludes on the urgency for reordering roles and relations within a new set-up organisation to incubate these systems in long-term planning and maintenance processes. Key words: 'Sociotechnical' system, Water, Planning, Urban Rain Harvesting systems (URHs), Hammarby Sjostad and Årstafältet

  4. Marcadores morfológicos y métricos del sexo en un conjunto osteológico del formativo de Tilcara (Sitio SJ Til. 20

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    Barboza, María Carolina

    2003-01-01

    Full Text Available En este trabajo se presentan los resultados obtenidos al aplicar un conjunto de técnicas basadas en el uso de marcadores biológicos (morfológicos y métricos del sexo en los individuos adultos de una colección osteológica correspondiente al Período Formativo (AMS Calibrado de 2 Sigma, Prob. 95%, Cal AD 545 a 660; Cal BP 1405 -1290 de la Quebrada de Humahuaca (Mendonça et al., 2002. Los restos óseos fueron recuperados en el transcurso de excavaciones sistemáticas en el así denominado "Sitio Mulqui" (SJ Til. 20, de la ciudad de Tilcara (Pcia. de Jujuy y constituyen una colección de 16 individuos, única de estas características para el período considerado (Bordach et al., 1999. El objetivo de este trabajo ha sido estudiar el comportamiento del patrón general de variabilidad sexual en los individuos adultos del Sitio Mulqui. Para ello se realizó un estudio comparativo en el que se confronta la información proporcionada por los distintos marcadores óseos para determinación sexual. Nuestros resultados permiten comprobar la existencia de concordancias y discordancias en las asignaciones sexuales de índole osteológica, según el complejo o estructura anatómica considerada, las cuales son discutidas e interpretadas a la luz de la información tanto biológica como cultural disponible. Se presentan los marcadores osteológicos considerados más efectivos para esta muestra en particular.

  5. A mixed-methods study identifying key intervention targets to improve participation in daily living activities in primary Sjögren's syndrome patients.

    Science.gov (United States)

    Hackett, Katie L; Deane, Katherine H O; Newton, Julia L; Deary, Vincent; Bowman, Simon; Rapley, Tim; Ng, Wan-Fai

    2018-02-06

    Functional ability and participation in life situations are compromised in many primary Sjögren's syndrome (PSS) patients. This study aims to identify the key barriers and priorities to participation in daily living activities, in order to develop potential future interventions. Group concept mapping (GCM), a semi-quantitative, mixed-methods, approach was used to identify and structure ideas from UK PSS patients, adults living with a PSS patient (AHMs) and health care professionals (HCPs). Brainstorming generated ideas, which were summarised into a final set of statements. Participants individually arranged these statements into themes and rated each statement for importance. Multidimensional scaling and hierarchical cluster analysis were applied to sorted and rated data to produce visual representations of the ideas (concept maps), enabling identification of agreed priority areas for interventions. 121 patients, 43 AHMs and 67 HCPs took part. 463 ideas were distilled down to 94 statements. These statements were grouped into seven clusters; 'Patient empowerment', 'Symptoms', 'Wellbeing', 'Access and coordination of healthcare', 'Knowledge and support', 'Public awareness and support' and 'Family and friends'. Patient empowerment and Symptoms were rated as priority conceptual themes. Important statements within priority clusters indicate patients should be taken seriously and supported to self-manage symptoms of oral and ocular dryness, fatigue, pain and poor sleep. Our data highlighted that in addition to managing PSS symptoms; interventions aiming to improve patient empowerment, general wellbeing, access to healthcare, patient education and social support are important to facilitate improved participation in daily living activities. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  6. Association of bone morphogenetic protein 6 with exocrine gland dysfunction in patients with Sjögren's syndrome and in mice.

    Science.gov (United States)

    Yin, Hongen; Cabrera-Perez, Javier; Lai, Zhenan; Michael, Drew; Weller, Melodie; Swaim, William D; Liu, Xibao; Catalán, Marcelo A; Rocha, Eduardo M; Ismail, Nevien; Afione, Sandra; Rana, Noreen A; Di Pasquale, Giovanni; Alevizos, Ilias; Ambudkar, Indu; Illei, Gabor G; Chiorini, John A

    2013-12-01

    Primary Sjögren's syndrome (SS) is characterized by autoimmune activation and loss of function in secretory epithelia. The present study was undertaken to investigate and characterize changes in the epithelia associated with the loss of gland function in primary SS. To identify changes in epithelial gene expression, custom microarrays were probed with complementary RNA (cRNA) isolated from minor salivary glands (MSGs) of female patients with primary SS who had low focus scores and low salivary flow rates, and the results were compared with those obtained using cRNA from the MSGs of sex-matched healthy volunteers. The effect of bone morphogenetic protein 6 (BMP-6) on salivary gland function was tested using adeno-associated virus-mediated gene transfer to the salivary glands of C57BL/6 mice. A significant increase in expression of BMP-6 was observed in RNA isolated from SS patients compared with healthy volunteers. Overexpression of BMP-6 locally in the salivary or lacrimal glands of mice resulted in the loss of fluid secretion as well as changes in the connective tissue of the salivary gland. Assessment of the fluid movement in either isolated acinar cells from mice overexpressing BMP-6 or a human salivary gland cell line cultured with BMP-6 revealed a loss in volume regulation in these cells. Lymphocytic infiltration in the submandibular gland of BMP-6 vector-treated mice was increased. No significant changes in the production of proinflammatory cytokines or autoantibodies associated with SS (anti-Ro/SSA and anti-La/SSB) were found after BMP-6 overexpression. In addition to identifying BMP-6 expression in association with xerostomia and xerophthalmia in primary SS, the present results suggest that BMP-6-induced salivary and lacrimal gland dysfunction in primary SS is independent of the autoantibodies and immune activation associated with the disease. Published 2013. This article is a U.S. Government work and is in the public domain in the USA.

  7. Association of Bone Morphogenetic Protein 6 With Exocrine Gland Dysfunction in Patients With Sjögren’s Syndrome and in Mice

    Science.gov (United States)

    Yin, Hongen; Cabrera-Perez, Javier; Lai, Zhenan; Michael, Drew; Weller, Melodie; Swaim, William D.; Liu, Xibao; Catalán, Marcelo A.; Rocha, Eduardo M.; Ismail, Nevien; Afione, Sandra; Rana, Noreen A.; Di Pasquale, Giovanni; Alevizos, Ilias; Ambudkar, Indu; Illei, Gabor G.; Chiorini, John A.

    2014-01-01

    Objective Primary Sjögren’s syndrome (SS) is characterized by autoimmune activation and loss of function in secretory epithelia. The present study was undertaken to investigate and characterize changes in the epithelia associated with the loss of gland function in primary SS. Methods To identify changes in epithelial gene expression, custom microarrays were probed with complementary RNA (cRNA) isolated from minor salivary glands (MSGs) of female patients with primary SS who had low focus scores and low salivary flow rates, and the results were compared with those obtained using cRNA from the MSGs of sex-matched healthy volunteers. The effect of bone morphogenetic protein 6 (BMP-6) on salivary gland function was tested using adeno-associated virus–mediated gene transfer to the salivary glands of C57BL/6 mice. Results A significant increase in expression of BMP-6 was observed in RNA isolated from SS patients compared with healthy volunteers. Overexpression of BMP-6 locally in the salivary or lacrimal glands of mice resulted in the loss of fluid secretion as well as changes in the connective tissue of the salivary gland. Assessment of the fluid movement in either isolated acinar cells from mice overexpressing BMP-6 or a human salivary gland cell line cultured with BMP-6 revealed a loss in volume regulation in these cells. Lymphocytic infiltration in the submandibular gland of BMP-6 vector–treated mice was increased. No significant changes in the production of proinflammatory cytokines or autoantibodies associated with SS (anti-Ro/SSA and anti-La/SSB) were found after BMP-6 overexpression. Conclusion In addition to identifying BMP-6 expression in association with xerostomia and xerophthalmia in primary SS, the present results suggest that BMP-6–induced salivary and lacrimal gland dysfunction in primary SS is independent of the autoantibodies and immune activation associated with the disease. PMID:23982860

  8. Neuroimmune interactions in Sjögren's syndrome: relationship of exocrine gland dysfunction with autoantibodies to muscarinic acetylcholine receptor-3 and mental health status parameters.

    Science.gov (United States)

    Deák, Magdolna; Szvetnik, Attila; Balog, Attila; Sohár, Nicolette; Varga, Renáta; Pokorny, Gyula; Tóth, Gábor; Kiss, Mária; Kovács, László

    2013-01-01

    Antimuscarinic acetylcholine receptor-3 (m3AChR) autoantibodies have been described in primary Sjögren's syndrome (pSS). The aim of this study was to compare various methods for their detection and to assess the contributions of anti-m3AChR and other immunological and psychosocial factors to the pathomechanism of secondary SS (sSS). Sixty-five rheumatoid arthritis (RA) patients, 103 systemic lupus erythematosus (SLE) patients, 76 pSS patients and 50 controls were compared. Three immunodominant epitopes of m3AChR were synthesized and used in ELISA. Two extracellular epitopes were also prepared in fusion with glutathione-S-transferase and one in conjugation with bovine serum albumin. Mental health status was assessed with the 36-item Short-Form Health Survey and Functional Assessment of Chronic Illness Therapy fatigue scale. Correlations were evaluated between glandular function and anti-m3AChR positivities and specificities, features of SLE and RA, and mental health parameters. Fourteen RA and 27 SLE patients had sSS. The autoantibody levels to all epitopes of m3AChR were significantly higher in pSS and SLE patients than in the controls. The fusion protein forms discriminated RA from pSS and SLE; furthermore, the YNIP fusion protein also distinguished pSS from SLE. The prevalence and the mean levels of all autoantibodies did not differ statistically between sicca and non-sicca SLE or RA patients. Glandular dysfunction correlated with higher age in SLE and RA and an impaired health-related quality of life in SLE. The second and third extracellular loops of m3AChR are antigenic in pSS. Immunoassays with antigens as fusion peptides demonstrate the best performance. Sicca SLE patients have worse mental health status. Anti-m3AChR antibodies represent a peculiar example of neuroimmune interactions. Copyright © 2012 S. Karger AG, Basel.

  9. Reversible lacrimal gland-protective regulatory T-cell dysfunction underlies male-specific autoimmune dacryoadenitis in the non-obese diabetic mouse model of Sjögren syndrome

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    Lieberman, Scott M; Kreiger, Portia A; Koretzky, Gary A

    2015-01-01

    CD4+ CD25+ Foxp3+ regulatory T (Treg) cells are required to maintain immunological tolerance; however, defects in specific organ-protective Treg cell functions have not been demonstrated in organ-specific autoimmunity. Non-obese diabetic (NOD) mice spontaneously develop lacrimal and salivary gland autoimmunity and are a well-characterized model of Sjögren syndrome. Lacrimal gland disease in NOD mice is male-specific, but the role of Treg cells in this sex-specificity is not known. This study aimed to determine if male-specific autoimmune dacryoadenitis in the NOD mouse model of Sjögren syndrome is the result of lacrimal gland-protective Treg cell dysfunction. An adoptive transfer model of Sjögren syndrome was developed by transferring cells from the lacrimal gland-draining cervical lymph nodes of NOD mice to lymphocyte-deficient NOD-SCID mice. Transfer of bulk cervical lymph node cells modelled the male-specific dacryoadenitis that spontaneously develops in NOD mice. Female to female transfers resulted in dacryoadenitis if the CD4+ CD25+ Treg-enriched population was depleted before transfer; however, male to male transfers resulted in comparable dacryoadenitis regardless of the presence or absence of Treg cells within the donor cell population. Hormone manipulation studies suggested that this Treg cell dysfunction was mediated at least in part by androgens. Surprisingly, male Treg cells were capable of preventing the transfer of dacryoadenitis to female recipients. These data suggest that male-specific factors promote reversible dysfunction of lacrimal gland-protective Treg cells and, to our knowledge, form the first evidence for reversible organ-protective Treg cell dysfunction in organ-specific autoimmunity. PMID:25581706

  10. Correlation of Increased Blood Levels of GITR and GITRL with Disease Severity in Patients with Primary Sjögren’s Syndrome

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    Xiaoxia Gan

    2013-01-01

    Full Text Available Glucocorticoid-induced tumor necrosis factor receptor family-related protein (GITR is a type I transmembrane protein belonging to the TNFR superfamily. After activated by its ligand GITRL, GITR could influence the activity of effector and regulatory T cells, participating in the development of several autoimmune and inflammatory diseases included rheumatoid arthritis and autoimmune thyroid disease. We previously reported that serum GITRL levels are increased in systemic lupus erythematosus (SLE patients compared with healthy controls (HC. Here, we tested serum soluble GITR (sGITR and GITRL levels in 41 primary Sjögren’s syndrome (pSS patients and 29 HC by ELISA and correlated sGITR and GITRL levels with clinical and laboratory variables. GITR and GITRL expression in labial salivary glands was detected by immunohistochemistry. pSS patients had significantly increased serum levels of sGITR and GITRL compared with controls (GITR: 5.66 ± 3.56 ng/mL versus 0.50 ± 0.31 ng/mL; P<0.0001; GITRL: 6.17 ± 7.10 ng/mL versus 0.36 ± 0.28 ng/mL; P<0.0001. Serum sGITR and GITRL levels were positively correlated with IgG (GITRL: r=0.6084, P<0.0001; sGITR: r=0.6820, P<0.0001 and ESR (GITRL: r=0.8315,P<0.0001; sGITR: r=0.7448, P<0.0001. Moreover, GITR and GITRL are readily detected in the lymphocytic foci and periductal areas of the LSGs. In contrast, the LSGs of HC subjects did not express GITR or GITRL. Our findings indicate the possible involvement of GITR-GITRL pathway in the pathogenesis of pSS. Further studies may facilitate the development of targeting this molecule pathway for the treatment of pSS.

  11. Deregulation of microRNA expression in purified T and B lymphocytes from patients with primary Sjögren’s syndrome

    Science.gov (United States)

    Wang-Renault, Shu-Fang; Boudaoud, Saida; Nocturne, Gaétane; Roche, Elodie; Sigrist, Nelly; Daviaud, Christian; Bugge Tinggaard, Andreas; Renault, Victor; Deleuze, Jean-François; Mariette, Xavier; Tost, Jörg

    2018-01-01

    Objective MicroRNAs (miRNAs) play an important role in the pathogenesis of autoimmune diseases such as primary Sjögren’s syndrome (pSS). This study is the first to investigate miRNA expression patterns in purified T and B lymphocytes from patients with pSS using a high-throughput quantitative PCR (qPCR) approach. Methods Two independent cohorts of both patients with pSS and controls, one for discovery and one for replication, were included in this study. CD4+ T cells and CD19+ B cells were isolated from peripheral blood mononuclear cells by magnetic microbeads and expression of miRNAs was profiled using the Exiqon Human miRNome panel I analysing 372 miRNAs. A selection of differentially expressed miRNAs was replicated in the second cohort using specific qPCR assays. Results A major difference in miRNA expression patterns was observed between the lymphocyte populations from patients with pSS and controls. In CD4 T lymphocytes, hsa-let-7d-3p, hsa-miR-155–5 p, hsa-miR-222–3 p, hsa-miR-30c-5p, hsa-miR-146a-5p, hsa-miR-378a-3p and hsa-miR-28–5 p were significantly differentially expressed in both the discovery and the replication cohort. In B lymphocytes, hsa-miR-378a-3p, hsa-miR-222–3 p, hsa-miR-26a-5p, hsa-miR-30b-5p and hsa-miR-19b-3p were significantly differentially expressed. Potential target mRNAs were enriched in disease relevant pathways. Expression of B-cell activating factor (BAFF) mRNA was inversely correlated with the expression of hsa-miR-30b-5p in B lymphocytes from patients with pSS and functional experiments showed increased expression of BAFF after inhibiting hsa-miR-30b-5p. Conclusions This study demonstrates major miRNAs deregulation in T and B cells from patients with pSS in two independent cohorts, which might target genes known to be involved in the pathogenesis of pSS. PMID:28916716

  12. Rituximab Effectiveness and Safety for Treating Primary Sjögren’s Syndrome (pSS): Systematic Review and Meta-Analysis

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    Souza, Francine Bertolais do Valle; Porfírio, Gustavo José Martiniano; Andriolo, Brenda Nazaré Gomes; de Albuquerque, Julia Vajda; Trevisani, Virginia Fernandes Moça

    2016-01-01

    Background Primary Sjögren’s Syndrome (pSS) is a systemic autoimmune disease that involves the exocrine glands and internal organs. pSS leads to destruction and loss of secretory function due to intense lymphoplasmacytic infiltration. Therapeutic options include mainly symptomatic and supportive measures, and traditional immunosuppressant drugs have shown no effectiveness in randomized trials. Rituximab (RTX) is a chimeric antibody anti-CD20 that leads to B cell depletion by diverse mechanisms. There is evidence that this drug may be effective for treating pSS. The objective of this systematic review was to evaluate Rituximab effectiveness and safety for treating pSS. Methods and Findings We conducted a systematic review of RCTs published until December 2015, with no language restriction. We registered a protocol on Plataforma Brasil (40654814.6.0000.5505) and developed search strategies for the following scientific databases: MEDLINE, EMBASE, CENTRAL and LILACS. We included adults with established pSS diagnosis and considered the use of Rituximab as intervention and the use of other drugs or placebo as control. Four studies met our eligibility criteria: three with low risk of bias and one with uncertain risk of bias. The total number of participants was 276 (145 RTX, 131 placebo). We assessed the risk of bias of each included study and evaluated the following as primary outcomes: lacrimal gland function, salivary gland function, fatigue improvement and adverse events. We found no significant differences between the groups in the Schirmer test at week 24 meta-analysis (MD 3.59, 95% CI -2.89 to 10.07). Only one study evaluated the lissamine green test and reported a statistically significant difference between the groups at week 24 (MD -2.00, 95% CI -3.52 to -0.48). There was a significant difference between the groups regarding salivary flow rate (MD 0.09, 95% CI 0.02 to 0.16) and improvement in fatigue VAS at weeks 6 (RR 3.98, 95% CI 1.61 to 9.82) and week 16

  13. Rituximab Effectiveness and Safety for Treating Primary Sjögren's Syndrome (pSS): Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Souza, Francine Bertolais do Valle; Porfírio, Gustavo José Martiniano; Andriolo, Brenda Nazaré Gomes; Albuquerque, Julia Vajda de; Trevisani, Virginia Fernandes Moça

    2016-01-01

    Primary Sjögren's Syndrome (pSS) is a systemic autoimmune disease that involves the exocrine glands and internal organs. pSS leads to destruction and loss of secretory function due to intense lymphoplasmacytic infiltration. Therapeutic options include mainly symptomatic and supportive measures, and traditional immunosuppressant drugs have shown no effectiveness in randomized trials. Rituximab (RTX) is a chimeric antibody anti-CD20 that leads to B cell depletion by diverse mechanisms. There is evidence that this drug may be effective for treating pSS. The objective of this systematic review was to evaluate Rituximab effectiveness and safety for treating pSS. We conducted a systematic review of RCTs published until December 2015, with no language restriction. We registered a protocol on Plataforma Brasil (40654814.6.0000.5505) and developed search strategies for the following scientific databases: MEDLINE, EMBASE, CENTRAL and LILACS. We included adults with established pSS diagnosis and considered the use of Rituximab as intervention and the use of other drugs or placebo as control. Four studies met our eligibility criteria: three with low risk of bias and one with uncertain risk of bias. The total number of participants was 276 (145 RTX, 131 placebo). We assessed the risk of bias of each included study and evaluated the following as primary outcomes: lacrimal gland function, salivary gland function, fatigue improvement and adverse events. We found no significant differences between the groups in the Schirmer test at week 24 meta-analysis (MD 3.59, 95% CI -2.89 to 10.07). Only one study evaluated the lissamine green test and reported a statistically significant difference between the groups at week 24 (MD -2.00, 95% CI -3.52 to -0.48). There was a significant difference between the groups regarding salivary flow rate (MD 0.09, 95% CI 0.02 to 0.16) and improvement in fatigue VAS at weeks 6 (RR 3.98, 95% CI 1.61 to 9.82) and week 16 (RR 3.08, 95% CI 1.21 to 7

  14. New epitopes and function of anti-M3 muscarinic acetylcholine receptor antibodies in patients with Sjögren's syndrome.

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    Tsuboi, H; Matsumoto, I; Wakamatsu, E; Nakamura, Y; Iizuka, M; Hayashi, T; Goto, D; Ito, S; Sumida, T

    2010-10-01

    M3 muscarinic acetylcholine receptor (M3R) plays a crucial role in the secretion of saliva from salivary glands. It is reported that some patients with Sjögren's syndrome (SS) carried inhibitory autoantibodies against M3R. The purpose of this study is to clarify the epitopes and function of anti-M3R antibodies in SS. We synthesized peptides encoding the extracellular domains of human-M3R including the N-terminal region and the first, second and third extracellular loops. Antibodies against these regions were examined by enzyme-linked immunosorbent assay in sera from 42 SS and 42 healthy controls. For functional analysis, human salivary gland (HSG) cells were preincubated with immunoglobulin G (IgG) separated from sera of anti-M3R antibody-positive SS, -negative SS and controls for 12 h. After loading with Fluo-3, HSG cells were stimulated with cevimeline hydrochloride, and intracellular Ca(2+) concentrations [(Ca(2+) )i] were measured. Antibodies to the N-terminal, first, second and third loops were detected in 42·9% (18 of 42), 47·6% (20 of 42), 54·8% (23 of 42) and 45·2% (19 of 42) of SS, while in 4·8% (two of 42), 7·1% (three of 42), 2·4% (one of 42) and 2·4% (one of 42) of controls, respectively. Antibodies to the second loop positive SS-IgG inhibited the increase of (Ca(2+) )i induced by cevimeline hydrochloride. Antibodies to the N-terminal positive SS-IgG and antibodies to the first loop positive SS-IgG enhanced it, while antibodies to the third loop positive SS-IgG showed no effect on (Ca(2+) )i as well as anti-M3R antibody-negative SS-IgG. Our results indicated the presence of several B cell epitopes on M3R in SS. The influence of anti-M3R antibodies on salivary secretion might differ based on these epitopes. © 2010 The Authors. Clinical and Experimental Immunology © 2010 British Society for Immunology.

  15. [Analysis of epitopes and function of anti-M3 muscarinic acetylcholine receptor antibodies in patients with Sjögren's syndrome].

    Science.gov (United States)

    Tsuboi, Hiroto; Matsuo, Naomi; Iizuka, Mana; Nakamura, Yumi; Matsumoto, Isao; Sumida, Takayuki

    2010-01-01

    Sjögren's syndrome (SS) is an autoimmune disease that affects exocrine glands including salivary and lacrimal glands. It is characterized by lymphocytic infiltration into exocrine glands, leading to dry mouth and eyes. A number of auto-antibodies, such as anti-SS-A and SS-B antibodies, are detected in patients with SS. However, no SS-specific pathologic auto-antibodies have yet been found in this condition. M3 muscarinic acetylcholine receptor (M3R) plays a crucial role in the secretion of saliva from salivary glands. It is reported that some patients with SS carried inhibitory auto-antibodies against M3R. To clarify the epitopes and function of anti-M3R antibodies in SS, we examined antibodies to the extracellular domains (N terminal region, the first, second, and third extracellular loop) of M3R by ELISA using synthesized peptide antigens encoding these domains in 42 SS and 42 healthy controls (HC). Titers and positivity of anti-M3R antibodies to every extracellular domain of M3R were significantly higher in SS than in HC. For functional analysis, human salivary gland (HSG) cells were pre-cultured with IgG from anti-M3R antibodies positive SS, negative SS, and HC. HSG cells were stimulated with cevimeline hydrochloride and intracellular calcium concentration ([Ca(2+)](i)) was measured. IgG from anti-M3R antibodies to the second loop positive SS inhibited the increase of [Ca(2+)](i), but IgG from antibodies to the N terminal or the first loop positive SS enhanced it, while IgG from antibodies to the third loop positive SS showed no effect on [Ca(2+)](i) as well as IgG from anti-M3R antibodies negative SS and HC. These findings indicated the presence of several B cell epitopes on M3R in SS and effect of anti-M3R antibodies on the salivary secretion might differ with these epitopes.

  16. Diagnostic validity of dynamic salivary gland scintigraphy with ascorbic acid stimulation in patients with Sjögren's syndrome: Comparation with unstimulated whole sialometry

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    Dugonjić Sanja

    2008-01-01

    Full Text Available Background/Aim. Beside many actual groups of classification criteria, uniform classification criteria for Sjögren’s syndrome (SS are still missing. The ophtalmic component of SS is well defined. Criteria for classifying its oral component remain controversial. The fifth item of the European Union and the United States of America (EU-US revised diagnostic classification criteria in 2002, is an objective evidence of xerostomia, diagnosed by one of the tests: unstimulated whole sialometry (UWS, parotid sialography, and dynamic salivary gland scintigraphy (DSGS. The aim of this study was to evaluate senstitivity, specificity, positive and negative predictive value and accuracy of DSGS with ascorbic acid stimulation in detecting xerostomia in SS patients and to compare DSGS findings with UWS values. Methods. Tests DSGS and UWS were done in 20 patients with SS and in 10 of the control subjects. The findings of DSGS were graded from 1 to 4 scintigraphie (SCT grade 1 - normal finding; SCT grade 2 - moderate function damage; SCT grade 3 - serious function damage, SCT grade 4 - very serious function damage. UWS measured 1.5 hour after the breakfast lasted 15 minutes. UWS bellow 2.5 ml/15min min. considered pathological. Results. All SS patients had pathological SCT findings. Comparing SCT grade between the patients and the control group, high statistical significance was found (p < 0.001. The estimated sensitivity of DSGS was 100%, specificity 80%, positive predictive value 91%, negative predictive value 100% and accuracy 93%. The calculated sensitivity of UWS was 75%. Salivary function damage detected by scintigraphy was in positive correlation with UWS findings. Conclusion. DSGS is a diagnostic test with high sensitivity, specificity, accuracy and positive and negative predictive values in detecting salivary function damage in SS patients. DSGS and UWS are very sensitive diagnostic tests for objective evidence of xerostomia, and have to be ones of

  17. Identification of potential saliva and tear biomarkers in primary Sjögren's syndrome, utilising the extraction of extracellular vesicles and proteomics analysis.

    Science.gov (United States)

    Aqrawi, Lara A; Galtung, Hilde Kanli; Vestad, Beate; Øvstebø, Reidun; Thiede, Bernd; Rusthen, Shermin; Young, Alix; Guerreiro, Eduarda M; Utheim, Tor Paaske; Chen, Xiangjun; Utheim, Øygunn Aass; Palm, Øyvind; Jensen, Janicke Liaaen

    2017-01-25

    There is a long-lasting need for non-invasive, more accurate diagnostic techniques when evaluating primary Sjögren's syndrome (pSS) patients. Incorporation of additional diagnostics involving screening for disease-specific biomarkers in biological fluid is a promising concept that requires further investigation. In the current study we aimed to explore novel disease biomarkers in saliva and tears from pSS patients. Liquid chromatography-mass spectrometry (LC-MS) was performed on stimulated whole saliva and tears from 27 pSS patients and 32 healthy controls, and salivary and tear proteomic biomarker profiles were generated. LC-MS was also combined with size exclusion chromatography to isolate extracellular vesicles (EVs) from both fluids. Nanoparticle tracking analysis was conducted on joint fractions from the saliva and tears to determine size distribution and concentration of EVs. Further EV characterisation was performed by immunoaffinity capture of CD9-positive EVs using magnetic beads, detected by flow cytometry. The LC-MS data were analysed for quantitative differences between patient and control groups using Scaffold, and the proteins were further analysed using the Database for Annotation, Visualization and Integrated Discovery (DAVID), for gene ontology overrepresentation, and the Search Tool for the Retrieval of Interacting Genes/Proteins for protein-protein interaction network analysis. Upregulation of proteins involved in innate immunity (LCN2), cell signalling (CALM) and wound repair (GRN and CALML5) were detected in saliva in pSS. Saliva EVs also displayed biomarkers critical for activation of the innate immune system (SIRPA and LSP1) and adipocyte differentiation (APMAP). Tear analysis indicated overexpression of proteins involved in TNF-α signalling (CPNE1) and B cell survival (PRDX3). Moreover, neutrophil gelatinase-associated lipocalin was upregulated in saliva and tears in pSS. Consistently, DAVID analysis demonstrated pathways of the adaptive

  18. Original paper Prevalence of selected organ-specific autoantibodies in rheumatoid arthritis and primary Sjögren’s syndrome patients

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    Arkadiusz Koszarny

    2015-05-01

    Full Text Available Objectives : The aim of the study was to investigate the prevalence of selected organ-specific autoantibodies in rheumatoid arthritis (RA and primary Sjögren’s syndrome (pSS patients, and discuss their clinical significance. Material and methods : The study included 121 RA and 30 pSS patients. Sera were tested for the presence of autoantibodies to thyroid peroxidase (anti-TPO, thyroglobulin (anti-TG, TSH receptor (TRAbs, mitochondrial antigen M2 (AMA-M2-3E and gliadin-analogous fusion peptides (anti-GAF(3X using the ELISA method. Non-organ-specific antibodies were determined: rheumatoid factor in IgM class, anti-citrullinated peptide antibodies and antinuclear antibodies. The occurrence of antibodies was also examined with regards to RA activity. Results : The following autoantibodies were detected in RA patients: anti-TPO – 13 (10.7%, anti-TG – 6 (5%, AMA-M2-3E – 3 (2.5%, anti-GAF(3X – 5 (4.1%. The respective levels of these autoantibodies in pSS patients were 3 (10%, 2 (6.7%, 4 (13.3% and 2 (6.7%. Polyautoimmunity was confirmed in 34 RA patients (including 20 cases of autoimmune thyroid disease [AITD] and in 6 pSS patients (6 cases of AITD. When RA patients were divided into anti-TPO positive and anti-TPO negative groups, we found a statistically significant relationship between groups regarding age and hemoglobin concentration. In pSS patients the anti-TPO positive group was less likely to use immunosuppressive drugs as compared with the anti-TPO negative group. Anti-TPO was significantly more frequently detected in RA + AITD vs. RA, RA + SS + AITD vs. RA and in pSS + AITD vs. pSS patients. Conclusions : Organ-specific autoantibodies are relatively frequently observed in patients with RA and pSS. Their presence is connected with the clinical picture of the diseases.

  19. Pro-inflammatory cytokines enhance ERAD and ATF6α pathway activity in salivary glands of Sjögren's syndrome patients.

    Science.gov (United States)

    Barrera, María-José; Aguilera, Sergio; Castro, Isabel; Cortés, Juan; Bahamondes, Verónica; Quest, Andrew F G; Molina, Claudio; González, Sergio; Hermoso, Marcela; Urzúa, Ulises; Leyton, Cecilia; González, María-Julieta

    2016-12-01

    Salivary gland (SG) acinar-cells are susceptible to endoplasmic reticulum (ER) stress related to their secretory activity and the complexity of synthesized secretory products. SGs of Sjögren's syndrome patients (SS)-patients show signs of inflammation and altered proteostasis, associated with low IRE1α/XBP-1 pathway activity without avert increases in apoptosis. Acinar-cells may avoid apoptosis by activation of the ATF6α pathway and ER-associated protein degradation (ERAD). The aim of this study was to evaluate the role of pro-inflammatory cytokines in ATF6α pathway/ERAD activation and cell viability in labial salivary glands (LSG) of SS-patients. In biopsies from SS-patients increased ATF6α signaling pathway activity, as evidenced by generation of the ATF6f cleavage fragment, and increased expression of ERAD machinery components, such as EDEM1, p97, SEL1L, gp78, UBE2J1, UBE2G2, HERP and DERLIN1, were observed compared to controls. Alternatively, for pro- (active-caspase-3) and anti-apoptotic (cIAP2) markers no significant difference between the two experimental groups was detected. Increased presence of ATF6f and ERAD molecules correlated significantly with increased expression of pro-inflammatory cytokines. These observations were corroborated in vitro in 3D-acini treated with TNF-α and/or IFN-γ, where an increase in the expression and activation of the ATF6α sensor and ERAD machinery components was detected under ER stress conditions, while changes in cell viability and caspase-3 activation were not observed. Cytokine stimulation protected cells from death when co-incubated with an ERAD machinery inhibitor. Alternatively, when cytokines were eliminated from the medium prior to ERAD inhibition, cell death increased, suggesting that the presence of pro-inflammatory cytokines in the medium is essential to maintain cell viability. In conclusion, the ATF6α pathway and the ERAD machinery are active in LSG of SS-patients. Both were also activated by TNF

  20. Rituximab Effectiveness and Safety for Treating Primary Sjögren's Syndrome (pSS: Systematic Review and Meta-Analysis.

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    Francine Bertolais do Valle Souza

    Full Text Available Primary Sjögren's Syndrome (pSS is a systemic autoimmune disease that involves the exocrine glands and internal organs. pSS leads to destruction and loss of secretory function due to intense lymphoplasmacytic infiltration. Therapeutic options include mainly symptomatic and supportive measures, and traditional immunosuppressant drugs have shown no effectiveness in randomized trials. Rituximab (RTX is a chimeric antibody anti-CD20 that leads to B cell depletion by diverse mechanisms. There is evidence that this drug may be effective for treating pSS. The objective of this systematic review was to evaluate Rituximab effectiveness and safety for treating pSS.We conducted a systematic review of RCTs published until December 2015, with no language restriction. We registered a protocol on Plataforma Brasil (40654814.6.0000.5505 and developed search strategies for the following scientific databases: MEDLINE, EMBASE, CENTRAL and LILACS. We included adults with established pSS diagnosis and considered the use of Rituximab as intervention and the use of other drugs or placebo as control. Four studies met our eligibility criteria: three with low risk of bias and one with uncertain risk of bias. The total number of participants was 276 (145 RTX, 131 placebo. We assessed the risk of bias of each included study and evaluated the following as primary outcomes: lacrimal gland function, salivary gland function, fatigue improvement and adverse events. We found no significant differences between the groups in the Schirmer test at week 24 meta-analysis (MD 3.59, 95% CI -2.89 to 10.07. Only one study evaluated the lissamine green test and reported a statistically significant difference between the groups at week 24 (MD -2.00, 95% CI -3.52 to -0.48. There was a significant difference between the groups regarding salivary flow rate (MD 0.09, 95% CI 0.02 to 0.16 and improvement in fatigue VAS at weeks 6 (RR 3.98, 95% CI 1.61 to 9.82 and week 16 (RR 3.08, 95% CI 1.21 to

  1. Characteristics of Labial Gland Mesenchymal Stem Cells of Healthy Individuals and Patients with Sjögren's Syndrome: A Preliminary Study.

    Science.gov (United States)

    Wang, Shi-Qin; Wang, Yi-Xiang; Hua, Hong

    2017-08-15

    Sjögren's syndrome (SS) is a systemic autoimmune disease that is characterized by focal lymphocytic infiltration into exocrine organs such as salivary and lacrimal glands, resulting in dry mouth and eyes, and other systemic injuries. There is no curative clinical therapy for SS, and stem cell therapy has shown great potential in this area. The mesenchymal stem cells (MSCs) in the salivary glands of healthy individuals and in patients with SS have not been extensively studied. The aim of this study was to elucidate the characteristics of MSCs from the labial glands of healthy controls and of those from patients with SS to elucidate the related pathogenesis and to uncover potential avenues for novel clinical interventions. Labial glands from patients with SS and healthy subjects were obtained, and MSCs were isolated and cultured by using the tissue adherent method. The MSC characteristics of the cultured cells were confirmed by using morphology, proliferation, colony forming-unit (CFU) efficiency, and multipotentiality, including osteogenic, adipogenic, and salivary gland differentiation. The MSCs from the healthy controls and SS patients expressed characteristic MSC markers, including CD29, CD44, CD73, CD90, and CD105; they were negative for CD34, CD45, and CD106, and also negative for the salivary gland epithelium markers (CD49f and CD117). Labial gland MSCs from both groups were capable of osteogenic and adipogenic differentiation. The CFU efficiency and adipogenic differentiation potential of MSCs were significantly lower in the SS group compared with the healthy controls. Cells from both groups could also be induced into salivary gland-like cells. Real-time polymerase chain reaction and immunofluorescence staining showed that the gene and protein expression of AMY1, AQP5, and ZO-1 in cells from the SS group was lower than that in cells from the healthy group. Thus, MSCs from the labial glands in patients with SS could lack certain characteristics and functions

  2. Mariología, espíritu misionero y sentir con la Iglesia: Caras permanentes del legado de Erich Przywara S.J. (1889-1972

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    Aníbal Edwards

    2010-01-01

    Full Text Available Este trabajo intenta complementar lo expuesto por el autor en El enigma Erich Prywara (Gregorianum, Roma 2009, 723-783, en tres facetas fundamentales en Przywara. Dado que zlas tesis doctorales alemanas acerca de Przywara, aprobadas en la 2ª mitad del s. XX no enfocan la unidad interna coherente de su enfoque metafísico, aludo brevemente a la unidad abierta de su «analogia entis» respecto de «analogia fidei» inseparada del dato revelado. La Mariología es temática recurrente, incluso en sus diálogos ecuménicos con K. Barth en Münster 1928, según deja constancia Przywara: «Si una "analogia entis" (y en ella, como pensaba Barth, lo mariológico ha de ser "principio fundamental católico", entonces jamás en tanto "principio", sino como "contextura coherente de algo pura y libremente fáctico"»; es decir, como contextura de un hecho religioso originario, según expliqué en el trabajo mencionado. En sus Escritos Religiosos, Przywara abunda en citas a San Ireneo, antes que A. Orbe rescatara su coherencia teológica. En su última época, Przywara enfatiza la centralidad de las nupcias en la redención en Cristo, así como antes en-fatizó la centralidad antropológica de una metafísica de lo sexuado, con María Virgen, Madre de esta Iglesia. El sentir con la Iglesia -manifiesto en toda su obra-, se patentiza en su intelección honda del Concilio Vaticano I y de la obra de Josef Kleutgen S.J., a quien se encomendó redactar la Constit. Dei Filius. De aquí repercutió en la Neo-Escolástica católica en Francia, Inglaterra y Alemania. Se ejemplifica también con la insistencia del Papado en el estudio a fondo de Tomás de Aquino, desde Leon XIII a Pío XI. El espíritu misionero subyace al Presupuesto que San Ignacio pone en quien da los Ejercicios Espirituales. Przywara inició el diálogo ecuménico en Alemania. Su respeto a la alteridad confesional a lo largo de su tarea como escritor libre, obtuvo su triunfo epocal en el Vaticano II

  3. Fortabte sjæle?

    DEFF Research Database (Denmark)

    Thyssen, Ole

    2017-01-01

    Anmeldelse af: Jamie Bartlett: Radicals. Outsiders Changing the World. William Heinemann, London, 382 sider,......Anmeldelse af: Jamie Bartlett: Radicals. Outsiders Changing the World. William Heinemann, London, 382 sider,...

  4. Sjögren's Syndrome

    Science.gov (United States)

    ... American College of Rheumatology Committee on Communications and Marketing. This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical ...

  5. Análise do pH salivar de indivíduos com Síndrome de Sjögren e refluxo laringofaríngeo pH salivary analysis of subjects suffering from Sjögren's Syndrome and laryngopharyngeal reflux

    Directory of Open Access Journals (Sweden)

    Marco Antonio dos Anjos Corvo

    2012-02-01

    Full Text Available A saliva é fundamental para a homeostase digestiva. Alterações no seu volume e composição são correlacionadas com o refluxo laringofaríngeo (RLF. Estudos recentes demonstraram que pacientes com RLF apresentam acidificação do pH salivar. A síndrome de Sjögren (SS foi eleita como modelo de hiposalivação, com objetivo de analisar as repercussões da diminuição salivar na laringe e faringe. CASUÍSTICA E MÉTODO: Estudo clínico transversal de 19 sujeitos com SS e RLF comparados com 12 indivíduos saudáveis. Obtidas amostras de saliva total não estimulada (STNE e saliva total estimulada por mastigação de parafilm M® (STE. RESULTADOS: Todos os indivíduos eram mulheres, média de idade de 60 anos (estudo e 44 anos. A prevalência de RLF foi 100%. O pH médio na STNE foi 7,53 (grupo estudo e 7,57(controle, aumentando para 7,87 e 7,93, respectivamente, após estimulação mecânica. Não houve diferença significante de pH entre os grupos. O volume salivar médio dos pacientes com SS foi 1,27mL (STNE e 3,78mL (STE, sendo o volume salivar do grupo controle significativamente maior (4,02mL na STNE e 11,96mL na STE. CONCLUSÕES: Todos os indivíduos estudados apresentaram RLF, fato provavelmente relacionado à queda uniforme observada no volume salivar e de todos seus componentes, não sendo observadas mudanças no pH salivar no grupo estudado. Isso sugere fisiopatogenias diferentes do RLF para indivíduos com SS.Saliva is one of the components for the digestive homeostasis. Recent studies have shown that patients with laryngopharyngeal reflux (LPR present a drop in salivary pH. Patients with Sjögren's syndrome (SS are a potential clinical research model for xerostomia and its laryngeal and pharyngeal consequences. The aim was to evaluate the characteristics of saliva of patients with SS and LPR. METHODS: 19 patients with SS plus LPR, and 12 healthy controls had their saliva studied prospectively for volume and pH. Two salivary

  6. Women in agriculture. A gender perspective Staða kvenna í landbúnaði. Kynjafræðilegur sjónarhóll

    Directory of Open Access Journals (Sweden)

    Hjördís Sigursteinsdóttir

    2009-06-01

    Full Text Available Abstract in English is unavailable.Meginmarkmið greinarinnar er að leita svara við því hvort til staðar sé kynjahalli á lögbýlum hér á landi þegar skoðaðir eru tilteknir þættir sem viðkoma félags- og efnahagslegri stöðu kynjanna. Erlendir femínískir fræðimenn hafa fært rök fyrir því að innan landbúnaðargeirans lifi feðraveldishugsunin enn góðu lífi og að félagsleg uppbygging hans ali á kynjaójafnrétti. Fáar kynjafræðilegar rannsóknir eru hins vegar til um stöðu kvenna í landbúnaði hér á landi ef frá er talin meistaranámsrannsókn Hjördísar Sigursteinsdóttur sem þessi grein byggist á. Þátttakendur í rannsókninni voru á aldrinum 18-70 ára og búsettir á lögbýlum á Íslandi. Notast var við lögbýlaskrá, útgefna af landbúnaðarráðuneytinu árið 2006, og þjóðskrá Hagstofu Íslands frá 1. desember 2006. Úrtakið var 1.985 manns, 49% karlar og 51% konur. Spurningalistinn var sendur til þátttakenda í janúar 2007. Í úrvinnslunni voru tengsl breyta ýmist skoðuð með fylgniútreikningum eða samanburði á meðaltölum. Svarhlutfallið var 53,5%. Þátttakendur endurspegluðu úrtakið vel með tilliti til kynferðis og landsvæða. Niðurstöðurnar sýndu tiltekinn kynjahalla á lögbýlum, konum í óhag. Þær voru almennt ekki eins ánægðar og karlar með búsetuskilyrði sín og barna sinna. Félags- og efnahagsleg staða kvenna var almennt lakari en karla þegar spurt var um þætti sem snertu tengsl við ættingja og vini, búrekstur, búsetuskilyrði, eignarhald og tekjur. Þetta má teljast áhyggjuefni, bæði út frá sjónarhóli kynjajafnréttis og byggðastefnu.

  7. Correlation between intravoxel incoherent motion MR parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome: A pilot study

    International Nuclear Information System (INIS)

    Chu, Chen; Zhou, Nan; Zhang, Huayong; Dou, Xin; Li, Ming; Liu, Song; Zhu, Yun; Chen, Weibo; Chan, Queenie; He, Jian; Sun, Lingyun; Zhou, Zhengyang

    2017-01-01

    Highlights: • Parotid glands at grade 0–3 had significant values from those in healthy glands. • Parotid D and f values were correlated with MR nodule grade significantly. • There were significant differences of D, f, and D * values among glands at grade 0–3. - Abstract: Purpose: To explore the correlation between intravoxel incoherent motion (IVIM) magnetic resonance (MR) parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome (SS). Materials and methods: A total of 31 consecutive patients with SS and 28 gender- and age-matched healthy volunteers underwent bilateral parotid 3.0T MR examination including the IVIM sequence (9 b values, 0–800 s/mm 2 ). The apparent diffusion coefficient (ADC), diffusion coefficient D, pseudo-diffusion coefficient D * , and perfusion fraction f of bilateral parotid glands were obtained, and the nodular grade of each parotid gland was evaluated according to the MR morphological appearance. Results: Sixty-two parotid glands in 31 patients with SS consisted of 32, 14, 8, and 8 parotid glands at MR nodular grades 0, 1, 2, and 3, respectively. In parotid glands of grade 0, 1, 2, 3 and healthy volunteers, the ADC values were (1.13 ± 0.25, 1.11 ± 0.17, 1.05 ± 0.24, 0.89 ± 0.04 and 1.00 ± 0.21) × 10 −3 mm 2 /s, D values were (0.92 ± 0.13, 0.90 ± 0.19, 0.90 ± 0.03, 0.67 ± 0.03, 0.81 ± 0.03) × 10 −3 mm 2 /s, f values were 0.20 ± 0.04, 0.18 ± 0.02, 0.15 ± 0.01, 0.11 ± 0.01, 0.15 ± 0.06, and D * values were (53.89 ± 28.26, 41.78 ± 16.35, 51.24 ± 18.69, 31.83 ± 18.03, 36.83 ± 16.14) × 10 −3 mm 2 /s respectively. The ADC, D, f, and D * values of parotid glands in patients with SS at grade 0 were significantly higher than those in healthy volunteers (all P < 0.05). Significant differences were observed in the D and f values of parotid glands in patients with SS among different grades (P = 0.003, < 0.001, respectively). The IVIM parameters (D, f) of parotid glands at early

  8. Correlation between intravoxel incoherent motion MR parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome: A pilot study

    Energy Technology Data Exchange (ETDEWEB)

    Chu, Chen, E-mail: chuchen19920905@163.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Zhou, Nan, E-mail: snscorpion@163.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Zhang, Huayong, E-mail: 13770560567@163.com [Department of Rheumatology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Dou, Xin, E-mail: douxin125@126.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Li, Ming, E-mail: lm069393@163.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Liu, Song, E-mail: liusongnj@126.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Zhu, Yun, E-mail: qqt111@hotmail.com [Department of Rheumatology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Chen, Weibo, E-mail: Weibo.Chen@philips.com [Philips Healthcare, Shanghai, 200233 (China); Chan, Queenie, E-mail: queenie.chan@philips.com [Philips Healthcare, Hong Kong (China); He, Jian, E-mail: hjxueren@126.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Sun, Lingyun, E-mail: lysun_nju@163.com [Department of Rheumatology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Zhou, Zhengyang, E-mail: zyzhou@nju.edu.cn [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China)

    2017-01-15

    Highlights: • Parotid glands at grade 0–3 had significant values from those in healthy glands. • Parotid D and f values were correlated with MR nodule grade significantly. • There were significant differences of D, f, and D{sup *} values among glands at grade 0–3. - Abstract: Purpose: To explore the correlation between intravoxel incoherent motion (IVIM) magnetic resonance (MR) parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome (SS). Materials and methods: A total of 31 consecutive patients with SS and 28 gender- and age-matched healthy volunteers underwent bilateral parotid 3.0T MR examination including the IVIM sequence (9 b values, 0–800 s/mm{sup 2}). The apparent diffusion coefficient (ADC), diffusion coefficient D, pseudo-diffusion coefficient D{sup *}, and perfusion fraction f of bilateral parotid glands were obtained, and the nodular grade of each parotid gland was evaluated according to the MR morphological appearance. Results: Sixty-two parotid glands in 31 patients with SS consisted of 32, 14, 8, and 8 parotid glands at MR nodular grades 0, 1, 2, and 3, respectively. In parotid glands of grade 0, 1, 2, 3 and healthy volunteers, the ADC values were (1.13 ± 0.25, 1.11 ± 0.17, 1.05 ± 0.24, 0.89 ± 0.04 and 1.00 ± 0.21) × 10{sup −3} mm{sup 2}/s, D values were (0.92 ± 0.13, 0.90 ± 0.19, 0.90 ± 0.03, 0.67 ± 0.03, 0.81 ± 0.03) × 10{sup −3} mm{sup 2}/s, f values were 0.20 ± 0.04, 0.18 ± 0.02, 0.15 ± 0.01, 0.11 ± 0.01, 0.15 ± 0.06, and D{sup *}values were (53.89 ± 28.26, 41.78 ± 16.35, 51.24 ± 18.69, 31.83 ± 18.03, 36.83 ± 16.14) × 10{sup −3} mm{sup 2}/s respectively. The ADC, D, f, and D{sup *} values of parotid glands in patients with SS at grade 0 were significantly higher than those in healthy volunteers (all P < 0.05). Significant differences were observed in the D and f values of parotid glands in patients with SS among different grades (P = 0.003, < 0.001, respectively). The IVIM

  9. Degradation of proteoglycan 4/lubricin by cathepsin S: Potential mechanism for diminished ocular surface lubrication in Sjögren's syndrome.

    Science.gov (United States)

    Regmi, Suresh C; Samsom, Michael L; Heynen, Miriam L; Jay, Gregory D; Sullivan, Benjamin D; Srinivasan, Sruthi; Caffery, Barbara; Jones, Lyndon; Schmidt, Tannin A

    2017-08-01

    Sjögren's syndrome (SS) is an autoimmune disease affecting the lacrimal and salivary glands with hallmark clinical symptoms of dry eye and dry mouth. Recently, markedly increased cathepsin S (CTSS) activity has been observed in the tears of SS patients. Proteoglycan 4 (PRG4), also known as lubricin, is an effective boundary lubricant that is naturally present on the ocular surface. While PRG4 is susceptible to proteolytic digestion, the potential effect of CTSS on PRG4 remains unknown. The objective of this study was to assess the ability of CTSS to enzymatically degrade purified PRG4, and PRG4 naturally present in human tears, and alter ocular surface boundary lubricating properties. To assess the potential time course and dose-dependency of PRG4 digestion by CTSS, full-length recombinant human PRG4 (rhPRG4) was incubated at 37 °C with or without CTSS in an enzymatic digestion buffer. Digestion of PRG4 by CTSS was also examined within normal human tear samples, both with and without supplementation by rhPRG4. Finally, digestion of endogenous PRG4 by CTSS, and the effect of a CTSS inhibitor, was examined in SS tears on Schirmer strips. Digestion products were separated on 3-8% SDS-PAGE and visualized by protein staining and western blotting. The boundary lubricating ability of rhPRG4 samples was assessed using an in vitro human eyelid-cornea friction test. Finally, SDS-PAGE protein stain bands resulting from rhPRG4 digestion were submitted for tandem mass spectrometry analysis to confirm their identity as PRG4 and identify non-tryptic cleavage sites. CTSS digested rhPRG4 in a time and dose dependent manner. CTSS digestion of rhPRG4 at 1% (where % is the mass ratio of CTSS to rhPRG4) resulted in a time dependent decrease in the full-length, ∼460 kDa, monomeric rhPRG4 band, and an appearance of lower MW fragments. After 20 h, no full-length rhPRG4 was observed. Furthermore, with an increased relative enzyme concentration of 3%, no protein bands were observed

  10. Serum levels of beta2-microglobulin and free light chains of immunoglobulins are associated with systemic disease activity in primary Sjögren's syndrome. Data at enrollment in the prospective ASSESS cohort.

    Directory of Open Access Journals (Sweden)

    Jacques-Eric Gottenberg

    Full Text Available To analyze the clinical and immunological characteristics at enrollment in a large prospective cohort of patients with primary Sjögren's syndrome (pSS and to investigate the association between serum BAFF, beta2-microglobulin and free light chains of immunoglobulins and systemic disease activity at enrollment.Three hundred and ninety five patients with pSS according to American-European Consensus Criteria were included from fifteen centers of Rheumatology and Internal Medicine in the "Assessment of Systemic Signs and Evolution of Sjögren's Syndrome" (ASSESS 5-year prospective cohort. At enrollment, serum markers were assessed as well as activity of the disease measured with the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI.Patient median age was 58 (25(th-75(th: 51-67 and median disease duration was 5 (2-9 years. Median ESSDAI at enrollment was 2 (0-7 with 30.9% of patients having features of systemic involvement. Patients with elevated BAFF, beta2-microglobulin and kappa, lambda FLCS had higher ESSDAI scores at enrollment (4 [2]-[11] vs 2 [0-7], P = 0.03; 4 [1]-[11] vs 2 [0-7], P< 0.0001; 4 [2]-[10] vs 2 [0-6.6], P< 0.0001 and 4 [2-8.2] vs 2 [0-7.0], P = 0.02, respectively. In multivariate analysis, increased beta2-microglobulin, kappa and lambda FLCs were associated with a higher ESSDAI score. Median BAFF and beta2-microglobulin were higher in the 16 patients with history of lymphoma (1173.3(873.1-3665.5 vs 898.9 (715.9-1187.2 pg/ml, P = 0.01 and 2.6 (2.2-2.9 vs 2.1 (1.8-2.6 mg/l, P = 0.04, respectively.In pSS, higher levels of beta2-microglobulin and free light chains of immunoglobulins are associated with increased systemic disease activity.

  11. Serum Levels of Beta2-Microglobulin and Free Light Chains of Immunoglobulins Are Associated with Systemic Disease Activity in Primary Sjögren’s Syndrome. Data at Enrollment in the Prospective ASSESS Cohort

    Science.gov (United States)

    Gottenberg, Jacques-Eric; Seror, Raphaèle; Miceli-Richard, Corinne; Benessiano, Joelle; Devauchelle-Pensec, Valerie; Dieude, Philippe; Dubost, Jean-Jacques; Fauchais, Anne-Laure; Goeb, Vincent; Hachulla, Eric; Hatron, Pierre Yves; Larroche, Claire; Le Guern, Véronique; Morel, Jacques; Perdriger, Aleth; Puéchal, Xavier; Rist, Stephanie; Saraux, Alain; Sene, Damien; Sibilia, Jean; Vittecoq, Olivier; Nocturne, Gaétane; Ravaud, Philippe; Mariette, Xavier

    2013-01-01

    Objectives To analyze the clinical and immunological characteristics at enrollment in a large prospective cohort of patients with primary Sjögren's syndrome (pSS) and to investigate the association between serum BAFF, beta2-microglobulin and free light chains of immunoglobulins and systemic disease activity at enrollment. Methods Three hundred and ninety five patients with pSS according to American-European Consensus Criteria were included from fifteen centers of Rheumatology and Internal Medicine in the “Assessment of Systemic Signs and Evolution of Sjögren's Syndrome” (ASSESS) 5-year prospective cohort. At enrollment, serum markers were assessed as well as activity of the disease measured with the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI). Results Patient median age was 58 (25th–75th: 51–67) and median disease duration was 5 (2–9) years. Median ESSDAI at enrollment was 2 (0–7) with 30.9% of patients having features of systemic involvement. Patients with elevated BAFF, beta2-microglobulin and kappa, lambda FLCS had higher ESSDAI scores at enrollment (4 [2]–[11] vs 2 [0–7], P = 0.03; 4 [1]–[11] vs 2 [0–7], P< 0.0001); 4 [2]–[10] vs 2 [0–6.6], P< 0.0001 and 4 [2–8.2] vs 2 [0–7.0], P = 0.02, respectively). In multivariate analysis, increased beta2-microglobulin, kappa and lambda FLCs were associated with a higher ESSDAI score. Median BAFF and beta2-microglobulin were higher in the 16 patients with history of lymphoma (1173.3(873.1–3665.5) vs 898.9 (715.9–1187.2) pg/ml, P = 0.01 and 2.6 (2.2–2.9) vs 2.1 (1.8–2.6) mg/l, P = 0.04, respectively). Conclusion In pSS, higher levels of beta2-microglobulin and free light chains of immunoglobulins are associated with increased systemic disease activity. PMID:23717383

  12. Traditional Chinese medicine compound ShengJinRunZaoYangXue granules for treatment of primary Sjögren's syndrome: a randomized, double-blind, placebo-controlled clinical trial.

    Science.gov (United States)

    Hu, Wei; Qian, Xian; Guo, Feng; Zhang, Miaojia; Lyu, Chengyin; Tao, Juan; Gao, Zhong'en; Zhou, Zhengqiu

    2014-01-01

    Traditional Chinese medical treatment of primary Sjögren's syndrome has advantages over Western medicine in terms of fewer side effects and improved patient conditions. This study was a multicenter, randomized, double-blind, placebo-controlled clinical trial of the efficacy and safety of ShengJinRunZaoYangXue granules for the treatment of primary Sjögren's syndrome, including the symptoms of dry mouth and dry eye. We undertook a 6-week, double-blind, randomized trial involving 240 patients with primary Sjögren's syndrome at five centers in East China. A computer-generated randomization schedule assigned patients at a 2:1 ratio to receive either ShengJinRunZaoYangXue granules or placebo once daily. Patients and investigators were blinded to treatment allocation. The primary endpoints were the salivary flow rate, Schirmer test results, and sugar test results. Intention-to-treat and per-protocol analyses were performed. All 240 patients were randomly allocated to either the treatment group (n = 160, ShengJinRunZaoYangXue granules) or placebo group (n = 80) and were included in the intention-to-treat analysis. After program violation and loss to follow-up, a total of 199 patients were included in the per-protocol analysis. At six week, intention-to-treat and per-protocol analyses of the left-eye Schirmer I test results showed an improved difference of 1.36 mm/5 min (95% CI: 0.03 to 2.69 mm/5 min) and 1.35 mm/5 min (95% CI: 0.04 to 2.73 mm/5 min), respectively, and those of the right-eye Schirmer I test results showed an improved difference of 1.12 mm/5 min (95% CI: 0.02 to 2.22 mm/5 min) and 1.12 mm/5 min (95% CI: -0.02 to 2.27 mm/5 min), respectively. There was no significant difference between the two groups before treatment. After treatment, the between-group and within-group before-and-after paired comparison results were statistically significant (P dry eye and dry mouth after six weeks of treatment. The incidence of adverse events was 15.6% in the treatment

  13. Impact of double positive for anti-centromere and anti-SS-a/Ro antibodies on clinicopathological characteristics of primary Sjögren's syndrome: a retrospective cohort study.

    Science.gov (United States)

    Suzuki, Yasunori; Fujii, Hiroshi; Nomura, Hideki; Mizushima, Ichiro; Yamada, Kazunori; Yamagishi, Masakazu; Kawano, Mitsuhiro

    2018-01-09

    The purpose of our study was to define the clinical characteristics of anti-centromere antibody and anti-SS-A/Ro antibody (ACA/SS-A) double positive Sjögren's syndrome (SS) and to clarify the clinical impact of these antibodies. We examined 108 patients (6 males, mean age 57.9 years) with SS who underwent labial salivary gland biopsy. The patients were divided into four groups by ACA and anti-SS-A/Ro antibody positivity. Symptoms, laboratory and pathological data, and scleroderma-related data were compared among the groups. The cohort consisted of 16 ACA/SS-A double positive, 20 ACA single positive, 67 SS-A single positive, and 5 ACA/SS-A double negative SS. ACA/SS-A double positive SS were significantly older than SS-A single positive SS (mean age 71.1 vs. 53.1 years). They had higher EULAR Sjögren's syndrome disease activity index (ESSDAI) at diagnosis (mean 3.81 vs. 0.50) and higher serum IgG (mean 2009 vs. 1389 mg/dL) than ACA single positive SS. No patients developed skin sclerosis during a mean follow-up period of 45.6 months (range: 1-178). These results demonstrate that ACA/SS-A double positive SS is distinct from ACA single positive and SSA single positive SS. The combination of ACA and anti-SS-A/Ro antibody in SS should deserve greater attention in clinical practice.

  14. Sjögren’s syndrome is associated with negatively variable impacts on domains of health-related quality of life: evidence from Short Form 36 questionnaire and a meta-analysis

    Directory of Open Access Journals (Sweden)

    Zhang Q

    2017-05-01

    Full Text Available Qiuxiang Zhang,1,2 Xulin Wang,3 Haoyang Chen,1,2 Biyu Shen1 1Department of Nursing, The Second Affiliated Hospital of Nantong University, 2School of Nursing, Nantong University, 3Department of Epidemiology and Medical Statistics, Nantong University, Nantong, Jiangsu, People’s Republic of China Purpose: The purpose of this article was to systematically review the literature to identify the impact of primary Sjögren’s syndrome (pSS on specific health-related quality of life (HRQoL domains.Methods: A meta-analysis was performed, and the related articles were searched in Medline, Embase, Cochrane Library, China National Knowledge Infrastructure, China Biology Medicine, and Web of Science databases and in reference lists of articles and systematic reviews. Score of the Short Form 36 (SF-36 questionnaire was used as the outcome measurement, and mean differences (MD with 95% confidence intervals (CIs were calculated.Results: Seven studies were included, comprising 521 patients with pSS and 9,916 healthy controls. The SF-36 questionnaire score of each domain (physical function, role physical [RP] function, emotional role function, vitality, mental health, social function, body pain, general health, physical component scale, mental component scale was lower in patients with pSS than in healthy controls, especially the score in the dimension of RP function.Conclusion: This meta-analysis showed that patients had lower pSS score in each dimension of the SF-36, mostly in the RP function. This demonstrated that targeted interventions should be carried out to improve the HRQoL of pSS patients. Keywords: primary Sjögren’s syndrome, quality of life, SF-36, meta-analysis

  15. The 52 000 MW Ro/SS-A autoantigen in Sjögren's syndrome/systemic lupus erythematosus (Ro52) is an interferon-γ inducible tripartite motif protein associated with membrane proximal structures

    Science.gov (United States)

    Rhodes, Davd A; Ihrke, Gudrun; Reinicke, Anna T; Malcherek, Georg; Towey, Michael; Isenberg, David A; Trowsdale, John

    2002-01-01

    The 52 000 MW Ro/SS-A (Ro52) protein is a major target of autoantibodies in autoimmune conditions such as systemic lupus erythematosus and Sjögren's syndrome. Recent genomic and bioinformatic studies have shown that Ro52 belongs to a large family of related RING/Bbox/coiled-coil (RBCC) tripartite motif proteins sharing overall domain structure and 40–50% identity at the amino acid level. Ro52 also has a B30.2 domain at the C-terminus. Using the human genome draft sequence, the genomic organization of the Ro52 gene on human chromosome 11p15.5 has been deduced and related to the protein domain structure. We show that the steady-state levels of Ro52 mRNA are normally very low but are induced by cell activation with interferon-γ. In transient transfection of HeLa cells, epitope-tagged Ro52 protein was localized to unidentified membrane proximal rod-like structures. Using in vitro coupled transcription/translation followed by immunoprecipitation, the autoimmune response to Ro52 protein was investigated and two distinct interactions were resolved. The Ro52 C-terminal B30.2 domain interacts with human immunoglobulin independently of antibody specificities. Sera derived from patients with Sjögren's syndrome and systemic lupus erythematosus, in addition, contained specific autoantibodies directed towards the rest of the Ro52 molecule. The majority of these autoimmune sera also immunoprecipitated the Ro52-related molecule RNF15. A possible role for Ro52 protein in alterations of plasma membranes during cellular activation or apoptosis is discussed. PMID:12047754

  16. Avaliação dos polimorfismos genéticos da interleucina-6, interleucina-10, fator de necrose tumoral-alfa e interferon-gama em pacientes com Síndrome de Sjögren secundária à artrite reumatóide

    OpenAIRE

    Souza, Thayse Rodrigues de

    2012-01-01

    Introdução: As citocinas desempenham um papel vital na regulação do sistema imunológico participando da patogênese de várias doenças auto-imunes, incluindo a síndrome de Sjögren. O objetivo deste estudo foi investigar a influência dos polimorfismos genéticos da interleucina-6, interleucina-10, fator de necrose tumoral alfa e interferon-gama na artrite reumatóide (AR), síndrome de Sjögren secundária (SSs) à artrite reumatóide e controles saudáveis (C). Materiais e métodos: Uma amostra de 138 p...

  17. The Ratio of Blood T Follicular Regulatory Cells to T Follicular Helper Cells Marks Ectopic Lymphoid Structure Formation While Activated Follicular Helper T Cells Indicate Disease Activity in Primary Sjögren's Syndrome.

    Science.gov (United States)

    Fonseca, Valter R; Romão, Vasco C; Agua-Doce, Ana; Santos, Mara; López-Presa, Dolores; Ferreira, Ana Cristina; Fonseca, João Eurico; Graca, Luis

    2018-05-01

    To investigate whether the balance of blood follicular helper T (Tfh) cells and T follicular regulatory (Tfr) cells can provide information about ectopic lymphoid neogenesis and disease activity in primary Sjögren's syndrome (SS). We prospectively recruited 56 patients clinically suspected of having SS. Sixteen of these patients subsequently fulfilled the American-European Consensus Group criteria for SS and were compared to 16 patients with non-SS sicca syndrome. Paired blood and minor salivary gland (MSG) biopsy samples were analyzed to study Tfr cells and subsets of Tfh cells in both compartments. Patients with primary SS had normal Tfh cell counts in peripheral blood; however, activated programmed death 1-positive (PD-1+) inducible costimulator-positive (ICOS+) Tfh cells in peripheral blood were strongly associated with disease activity assessed by the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (r = 0.8547, P = 0.0008). Conversely, the blood Tfr cell:Tfh cell ratio indicated ectopic lymphoid structure formation in MSGs, being strongly associated with B cell, CD4+ T cell, and PD-1+ICOS+ T cell infiltration in MSGs, and was especially increased in patients with focal sialadenitis. Further analysis showed that the blood Tfr cell:Tfh cell ratio allowed discrimination between SS patients and healthy donors with excellent accuracy and was a strong predictor of SS diagnosis (odds ratio [OR] 12.96, P = 0.028) and the presence of focal sialadenitis (OR 10, P = 0.022) in patients investigated for sicca symptoms, thus highlighting the potential clinical value of this marker. The blood Tfr cell:Tfh cell ratio and PD-1+ICOS+ Tfh cells constitute potential novel biomarkers for different features of primary SS. While the blood Tfr cell:Tfh cell ratio is associated with ectopic lymphoid neogenesis, activated Tfh cells indicate disease activity. © 2018, American College of Rheumatology.

  18. Primary Sjögren syndrome in a 2-year-old patient: role of the dentist in diagnosis and dental management with a 6-year follow-up.

    Science.gov (United States)

    De Oliveira, Marcio Augusto; De Rezende, Nathalie Pepe Medeiros; Maia, Célia Márcia Fernandes; Gallottini, Marina

    2011-11-01

    BACKGROUND. Primary Sjögren syndrome is a rare autoimmune disease, especially in children, mainly affecting girls (77%), and usually diagnosed around 10 years of age. Diagnosis during childhood is difficult, especially because of the diversity of the clinical presentation and difficulty obtaining reliable history data, accounting for a higher frequency of underdiagnosed cases. Differential conditions should be considered, especially the ones that promote xerostomia, such as diabetes, ectodermal dysplasia, rheumatoid arthritis, scleroderma, systemic lupus erythematosus, sarcoidosis, lymphoma, HIV and HTLV infection. Conditions associated with parotid enlargement should also be excluded, including juvenile recurrent parotitis (JRP), sialadenosis, sarcoidosis, lymphoma, infectious parotitis caused by streptococcal and staphylococcal infections, viral infections (paramyxovirus, Epstein-Barr virus, cytomegalovirus, and parvovirus), and diffuse infiltrative lymphocytosis syndrome (associated with HIV infection), and rare congenital conditions, such as polycystic parotid disease. CASE REPORT. A paediatric female patient was referred to our clinic for dental treatment complaining about dry mouth, oral discomfort, and dysphagia. The patient presented five of the required criteria to establish the diagnosis of pSS, including ocular symptoms, oral symptoms, evidence of keratoconjunctivitis sicca, focal sialadenitis confirmed by minor salivary gland biopsy, and evidence of major salivary gland involvement. Our patient did not have positive SS-A and SS-B autoantibodies. According to the literature, about 29% of individuals with pSS can present seronegativity for SS-A (anti-Ro) antibodies and about 33% can present seronegativity for SS-B (anti-La) antibodies. CONCLUSION. To the best of our knowledge, this is the youngest patient reported in the scientific English literature with pSS. Primary Sjögren syndrome has a wide clinical and immunologic spectrum and may progress with

  19. Perfil clínico y epidemiológico de pacientes con Síndrome de Sjögren concomitante a Artritis Reumatoide atendidos en una Clínica privada de la ciudad de Medellín (Colombia. 2005- 2010

    Directory of Open Access Journals (Sweden)

    Lina Maria Martinez Sanchez

    2013-08-01

    Full Text Available El Síndrome de Sjögren es una enfermedad crónica autoinmune en la cual se ven alteradas las glándulas mucosas del organismo, caracterizada principalmente por sequedad oral (xerostomía y ocular (xeroftalmia. Objetivo. Describir el perfil clínico y epidemiológico de pacientes con Sjögren concomitante a Artritis Reumatoide en una Clínica privada de la ciudad de Medellín-Colombia. 2005- 2010. Metodología. Estudio descriptivo del tipo de series clínicas en el que se estudiaron las historias clínicas de los pacientes atendidos por Sjögren concomitante a Artritis Reumatoide en una clínica privada de Medellín-Colombia. Para el análisis estadístico se utilizó el programa SPSS, a las variables cuantitativas se les calculó el promedio y la desviación estándar, además de los valores mínimo y máximo, y a las variables cualitativas se les estimó proporciones. Resultados. Se estudiaron 23 pacientes los cuales tenían una edad mediana de 55 años y 91.3% eran del sexo femenino. Los síntomas más frecuentemente reportados fueron la xerostomía y la xeroftalmia con un 69.6% y 56.5% respectivamente. Los métodos más empleados para el diagnóstico fueron el Factor Reumatoideo (78.3% y los anticuerpos antinucleares (69.6%, mientras que los principales medicamentos empleados fueron la cloroquina y la Prednisolona (cada uno con 52.2%. Conclusión. Sjögren concomitante a Artritis fue encontrado más comúnmente en el sexo femenino. Las manifestaciones clínicas más frecuentes fueron la xerostomía y xeroftalmia y la mediana del tiempo de diagnóstico fue de 61 meses. Es necesario seguir ahondando en el tema para caracterizar mejor aquellos pacientes con Sjögren y otra enfermedad inmunológica de base.

  20. El asombroso lenguaje de la sombra en La Carreta Fantasma (Victor Sjöström, 1920. Análisis cultural, contextual y fílmico

    Directory of Open Access Journals (Sweden)

    Pablo ALLEPUZ GARCÍA

    2015-12-01

    Full Text Available Desde finales del siglo XIX, y muy especialmente durante el primer cuarto del siglo XX, la cultura de la sombra se desarrolló por toda Europa con profusión y potencia inusitadas; no fue, ni mucho menos, un recurso exclusivo del expresionismo cinematográfico alemán. Para explicar el uso más sutil que Victor Sjöström hace de ella en La carreta fantasma (1920, el artículo traza primero una breve historia de la sombra y de su trasposición en el teatro y en el cine; contextualiza el filme dentro de la cinematografía escandinava de la época y de la trayectoria del propio director; y analiza, a partir de la base literaria de Selma Lagerlöf y mediante el discurso de las imágenes, varios fragmentos de la película que demuestran un determinado lenguaje lumínico. Desde dicha lectura, la sombra se convierte en el elemento central del film y permite reconsiderar su posición dentro de la historia del cine europeo.

  1. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren syndrome: a randomized, placebo-controlled, fixed-dose, multicenter trial. P92-01 Study Group.

    Science.gov (United States)

    Vivino, F B; Al-Hashimi, I; Khan, Z; LeVeque, F G; Salisbury, P L; Tran-Johnson, T K; Muscoplat, C C; Trivedi, M; Goldlust, B; Gallagher, S C

    1999-01-25

    Patients with Sjögren syndrome (SS) experience slowly progressive infiltration of lacrimal and salivary glands by mononuclear cells. This leads to diminished secretions, with resultant symptoms of xerostomia and xerophthalmia. Although pilocarpine hydrochloride tablets are currently indicated for the treatment of radiation-induced xerostomia, their effects on dry mouth or dry eyes in patients with SS are unclear. To assess the safety and efficacy of pilocarpine (Salagen) tablets as symptomatic treatment for dry mouth and dry eyes caused by SS in a multicenter, doubleblind, placebo-controlled trial. After providing written informed consent, 373 patients with primary or secondary SS and clinically significant dry mouth and dry eyes were randomized to receive 2.5-mg pilocarpine, 5-mg pilocarpine, or placebo tablets 4 times daily for 12 weeks. Symptoms were assessed by questionnaires with visual analog scales or categorical checkboxes. Whole-mouth salivary flow rates were measured. A significantly greater proportion of patients in the 5-mg pilocarpine group showed improvement compared with the placebo group (Pdry mouth, dry eyes, and other symptoms of dryness (Pdry mouth and dry eyes and other xeroses in patients with SS.

  2. Nailfold capillaroscopic findings in primary Sjögren's syndrome with and without Raynaud's phenomenon and/or positive anti-SSA/Ro and anti-SSB/La antibodies.

    Science.gov (United States)

    Corominas, Hèctor; Ortiz-Santamaría, Vera; Castellví, Iván; Moreno, Mireia; Morlà, Rosa; Clavaguera, Teresa; Erra, Alba; Martínez-Pardo, Silvia; Ordóñez, Sergi; Santo, Pilar; Reyner, Patricia; González, Maria José; Codina, Oriol; Gelman, Mario Saul; Juanola-Roura, Xavier; Olivé, Alex; Torrente-Segarra, Vicenç

    2016-03-01

    The aim of this study was to assess nailfold capillaroscopic (NC) findings in patients with primary Sjögren's syndrome (PSS) with and without Raynaud's phenomenon (RP) as well as in the presence of positive anti-SSA/Ro and anti-SSB/La antibodies. Videocapillaroscopy was performed in 150 patients with PSS. Data collected included demographics, presence of RP, PSS symptoms, antinuclear antibodies, rheumatoid factor, anti-Ro, anti-La, anti-CCP, salivary scintigraphy, labial biopsy, and NC findings. RP was present in 32% of PSS, keratoconjunctivitis sicca in 91%, oral xerosis in 93%, and skin or genital xerosis in 53%. In patients with positive anti-SSA/Ro (75%) and positive anti-SSB/La (40%), NC showed normal findings in 53% of cases and non-specific in 36%. In patients with PSS, NC was normal in 51% of cases and non-specific in 34%. Scleroderma pattern was found in 14 patients. RP associated with PSS had non-specific capillaroscopy in 40% of cases (p = 0.1). Pericapillary haemorrhages (p = 0.06) and capillary thrombosis (p = 0.2) were not increased, but more dilated capillaries were detected in 48% of cases. Patients with positive anti-Ro and/or anti-La have not a distinct NC profile. Patients with RP associated with PSS had more dilated capillaries, but neither pericapillary haemorrhages nor capillary thrombosis was observed.

  3. Paeoniflorin down-regulates ATP-induced inflammatory cytokine production and P2X7R expression on peripheral blood mononuclear cells from patients with primary Sjögren's syndrome.

    Science.gov (United States)

    Yu, Jingya; Chen, Yong; Li, Mingcai; Gao, Qiaoyan; Peng, Yong; Gong, Qiongyao; Zhang, Zhen; Wu, Xiudi

    2015-09-01

    This study determined the effects of paeoniflorin (PF) on the expression of purinergic receptor P2X ligand-gated ion channel 7 (P2X7R) expressed on peripheral blood mononuclear cells (PBMCs) and production of ATP-induced pro-inflammatory cytokines released by PBMCs in patients with primary Sjögren's syndrome (pSS). The pharmacological functions and cytotoxic effects of PF were dose dependent in PBMCs from 20 newly diagnosed pSS patients and 20 normal individuals. The optimum dose of PF was 100μM. PF significantly down-regulated the production of interleukin (IL)-1β and IL-6 from pSS PBMCs, and significantly inhibited ATP-induced expression of P2X7R, that might contribute to reduced IL-1β and IL-6. mRNA and protein levels of P2X7R on pSS PBMCs were significantly higher than in normal individuals (p=0.03, pP2X7R mRNA and protein levels were decreased significantly (pP2X7R on pSS PBMCs, indicating that PF might be useful for the management of pSS via down-regulating P2X7R expression. Thus, PF may provide a new therapeutic approach to regulate P2X7R-mediated pathologic responses of pSS. Copyright © 2015 Elsevier B.V. All rights reserved.

  4. Vasoactive Intestinal Peptide Protects Salivary Glands against Structural Injury and Secretory Dysfunction via IL-17A and AQP5 Regulation in a Model of Sjögren Syndrome.

    Science.gov (United States)

    Li, Chengyin; Zhu, Fenglin; Wu, Bin; Wang, Yue

    2018-04-04

    Sjögren syndrome (SS) is an autoimmune disease involving exocrine glands. Currently, drugs that can improve both abnormal immunity and exocrine gland function are needed. The study aimed to investigate the effect and mechanism of vasoactive intestinal peptide (VIP) on the immune response and exocrine gland function in SS. We investigated the effects of VIP on the immune response and secretory function of submandibular glands using NOD mice, and analyzed the expression of IL-17A and AQP5 (aquaporin 5). The submandibular gland cells from healthy 8-day-old Sprague-Dawley rats were used to observe the influence of VIP on AQP5 expression. Our study shows that treatment with VIP in an SS mouse model could not only reduce the immune injury to exocrine glands but also improve the secretory function of these glands. Furthermore, VIP was shown to improve the abnormal immune status by downregulating IL-17A expression in the exocrine glands. It also enhanced the secretory function of exocrine glands by upregulating AQP5 expression. Using a model of SS, we found that VIP could not only modulate the immune response but also affect exocrine gland function, and that these therapeutic effects were associated with IL-17A and AQP5 regulation. © 2018 S. Karger AG, Basel.

  5. High prevalence of Chlamydophila psittaci subclinical infection in Italian patients with Sjögren's syndrome and parotid gland marginal zone B-cell lymphoma of MALT-type.

    Science.gov (United States)

    Fabris, Martina; Dolcetti, Riccardo; Pasini, Elisa; Quartuccio, Luca; Pontarini, Elena; Salvin, Sara; Ponzoni, Maurilio; Scott, Cathryn Anne; De Vita, Salvatore

    2014-01-01

    To assess Chlamydophila psittaci (Cp) subclinical infection in patients with Sjögren's syndrome (SS). Seventy-four SS patients (55.4 ±13.4 yrs; 94.6% females) were studied. Among them, 18 had salivary gland mucosa-associated lymphoid tissue (MALT) B-cell lymphoma, 20 myoepithelial sialoadenitis (MESA), and 36 no lymphoproliferative disorders (LPD). The presence of Cp DNA was assessed in peripheral blood of all patients by specific PCR protocols. Paired salivary gland samples were also investigated whenever available (34 cases), including lymphomatous and non-lymphomatous samples, as well as major and minor salivary gland tissues. As controls, 225 blood donors were analysed in the peripheral blood. Overall, Cp DNA was detected in 11/74 (14.9%) SS patients vs. 1/225 (0.4%) controls (pMALT lymphoma patients (6/18, 33.3%), as compared with MESA (3/20, 15%) or patients without LPD (2/36, 5.6%), (MALT lymphomas vs. others: p=0.02). A similar Cp prevalence was observed in blood vs. salivary gland tissues, however with a higher frequency in the major than in the minor salivary glands (5/18, 27.8%, vs. 1/17, 5.9%, p=0.18). Cp-positive patients were all rheumatoid factor positive (11/11, 100% vs. 40/63, 63.5% Cp-negative; p=0.014), while no difference was noticed for anti-SSA/SSB positivity. In the light of accepted models of MALT B-cell lymphomagenesis and considering previous data implicating Cp infection in ocular adnexa MALT lymphoma, our results suggest that Cp infection could be involved also in a fraction of patients with SS developing lymphoma. The potential therapeutic implications of these findings appear worthwhile.

  6. Is extra-glandular organ damage in primary Sjögren's syndrome related to the presence of systemic auto-antibodies and/or hypergammaglobulinemia? A long-term cohort study with 110 patients from the Netherlands.

    Science.gov (United States)

    Ter Borg, Evert-Jan; Kelder, Johannes Cornelis

    2017-07-01

    To test the hypothesis that systemic auto-antibodies or hypergammaglobulinemia are related to the prevalence of extra-glandular tissue organ damage (EGOD) in primary Sjögren's syndrome (SS). A real practice-based investigation of a relatively large (n = 110) Dutch cohort of primary SS patients systematically followed up in a large non-academic hospital. After a follow up of mean 8.2 years a significant correlation was found between disease duration and the prevalence of EGOD. We did not observe a relationship between the total number or type of systemic auto-antibodies or hypergammaglobulinemia and the total number of EGOD. However, there was a correlation between the prevalence of polyneuropathy (PNP) and antinuclear antibodies (ANA) as well as anti-Ro/SS-A positivity and there was an inverse relationship between the presence of anti-Ro/SS-A antibodies and primary biliary cirrhosis (PBC). All PBC cases were anti-Ro/SS-A and anti-La/SS-B negative but ANA positive. There was a trend for a higher occurrence of pleuro-pulmonary disease in the ANA negative cases. Although we did not find a relationship between the total number or type of systemic auto-antibodies and the total number of EGOD, there were correlations between specific systemic auto-antibodies and specific types of EGOD. The presence of ANA and anti-Ro/SS-A was associated with the occurrence of PNP, as well as was the absence of anti-Ro/SS-A with PBC. © 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

  7. Illness perceptions and psychological distress associated with physical health-related quality of life in primary Sjögren's syndrome compared to systemic lupus erythematosus and rheumatoid arthritis.

    Science.gov (United States)

    Kotsis, Konstantinos; Voulgari, Paraskevi V; Tsifetaki, Niki; Drosos, Alexandros A; Carvalho, André F; Hyphantis, Thomas

    2014-12-01

    Notwithstanding that psychological distress and illness perceptions are important in determining outcomes in rheumatic diseases, few studies investigated these variables in primary Sjögren's syndrome (pSS). We aimed to compare illness perceptions and psychological distress in patients with pSS, systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) and to test whether their associations with health-related quality of life (HRQoL) are similar in these groups of patients. In 57 patients with pSS, 75 with SLE and 199 with RA, we administered the Patient Health Questionnaire (PHQ-9), the Symptom Check-List and the Brief-Illness Perception Questionnaire to assess psychological variables and the World Health Organization Quality of Life Instrument, Short-Form to assess HRQoL. Hierarchical regression models determined the associations of psychological variables with HRQoL after adjusting for demographic variables and clinical parameters. The prevalence of clinically significant depressive symptoms (PHQ-9 ≥ 10) was 24.6 % in pSS, 29.3 % in SLE and 25.1 % in RA. Patients with pSS showed little understanding of their disease (comprehensibility) and attributed more symptoms to their illness (identity) compared with the other groups of patients. Illness perceptions and depressive symptoms were independently associated with physical HRQoL in a similar pattern in all three groups. In pSS, however, the patients' worries about the consequences of their illness was a stronger correlate of physical HRQoL than pain. These findings indicate that psychological factors are important correlates of HRQoL in these disease groups and encourage the design of psycho-educational therapies targeting disease-related cognitions in pSS in an attempt to improve patient's physical HRQoL.

  8. NF-κB2 Controls the Migratory Activity of Memory T Cells by Regulating Expression of CXCR4 in a Mouse Model of Sjögren's Syndrome.

    Science.gov (United States)

    Kurosawa, Mie; Arakaki, Rieko; Yamada, Akiko; Tsunematsu, Takaaki; Kudo, Yasusei; Sprent, Jonathan; Ishimaru, Naozumi

    2017-11-01

    Dysregulated chemokine signaling contributes to autoimmune diseases by facilitating aberrant T cell infiltration into target tissues, but the specific chemokines, receptors, and T cell populations remain largely unidentified. The aim of this study was to examine the role of the potent chemokine CXCL12 and its receptor CXCR4 in the T cell autoimmune response, using alymphoplasia (aly)/aly mice, a model of Sjögren's syndrome (SS). T cell phenotypes in the salivary gland of aly/aly mice were evaluated using immunologic analysis. An in vitro migration assay was used to assess T cell migratory activity toward several chemokines. Gene expression of chemokine receptors and transforming growth factor β receptors (TGFβRs) was measured by quantitative reverse transcription-polymerase chain reaction. The CXCR4 antagonist AMD3100 was administered to the aly/aly mice in order to evaluate its suppressive effect on autoimmune lesions. Effector memory T (TEM) cells derived from aly/aly mice demonstrated higher in vitro migratory activity toward CXCL12 than did TEM cells from aly/+ mice. CXCL12 expression was specifically up-regulated in the SS target cells of aly/aly mice. TEM cells from RelB -/- mice, but not Nfkb1 -/- mice, also showed high migratory activity toward CXCL12, implicating a role of the nonclassical RelB/NF-κB2 pathway in the regulation of TEM cell migration. TEM cells from aly/aly mice also overexpressed TGFβR type I (TGFβRI) and TGFβRII. The CXCR4 antagonist AMD3100 suppressed autoimmune lesions in aly/aly mice by reducing TEM cell infiltration. Our results suggest that the RelB/NF-κB2 pathway regulates T cell migration to autoimmune targets through TGFβ/TGFβR-dependent regulation of CXCL12/CXCR4 signaling. This suggests that these signaling pathways are potential therapeutic targets for the treatment of autoimmune diseases. © 2017, American College of Rheumatology.

  9. Efficacy of Carbopol 974P (Siccafluid in the treatment of severe to moderate keratoconjunctivitis sicca (KCS in patients with primary Sjögren’s syndrome (SS not responding to standard treatment with artificial tears

    Directory of Open Access Journals (Sweden)

    A. Furlan

    2011-09-01

    Full Text Available Objective. To determine efficacy and safety of Carbopol 974P in the treatment of severe to moderate keratoconjunctivitis sicca (KCS in patients with primary Sjögren’s syndrome (SS not responding to standard treatment with artificial tears. Methods. 60 patients (57 F, 3 M, mean age 52.5±12.0, mean disease duration 12.2±7.1 yrs affected with primary SS diagnosed according to the European Community Study Group criteria were studied. Foregoing medications for SS and artificial tears for KCS have not been changed within 3 and 2 months respectively prior to the study onset. In all cases Carbopol 974P was added because symptoms of KCS were not adequately controlled with traditional lubricants. Schirmer I test, B.U.T. (break up time, rose Bengal-stain, clinical ophthalmological examination (i.e. fluorescein staining, keratis, corneal infiltrates and ulcers and a questionnaire for dry eye symptoms (range 0-30 were performed at entry (T0 and after 2 (T1 and 12 (T2 weeks. Assessment of global efficacy was obtained by VAS 0-100 at T2 either by patients and by the ophthalmologist. Results. Lachrymal tests significantly improved after 2 and, even more, after 12 weeks. Clinical ophthalmologic picture also ameliorated: a remarkable reduction of fluorescein positive lesions was demonstrated from 71.6% of the cases at T0 to 38.3% at T2. Total score of symptoms (T0: 16.1±7.3 dropped to 11.9±6.6 (T1 (p=0.000 and then to 6.7±5.3 (T2 (p=0.000. Global efficacy expressed by patients and physician was 74.8±15.9 and 76.6±13.0, respectively. No adverse events (blurred vision, allergy were reported throughout the study. Conclusions. Our study seems to demonstrate that addition of Carbopol 974P to the traditional therapeutic armamentarium for moderate to severe KCS is useful and well accepted in patients with primary SS in which management of ocular symptoms is unsatisfactory.

  10. What Is Sjögren's Syndrome?

    Science.gov (United States)

    ... mouth may include: Chewing gum or sucking on hard candy helps your glands make more saliva. Use sugar- ... your throat before speaking. Clearing your throat is hard on the vocal cords. To avoid ... gum. Suck on sugar-free candy. Make an “h” sound, hum, or laugh to ...

  11. Utvikling af sjøfronten

    DEFF Research Database (Denmark)

    Bates, Gary; Bonesmo, Gro; Kurdahl, Adam

    2000-01-01

    Udstillingskatalog til Arkitektur Triennalen i Oslo sept 2000. Projekt Liquid Edge v TRANSFORM & Space Group......Udstillingskatalog til Arkitektur Triennalen i Oslo sept 2000. Projekt Liquid Edge v TRANSFORM & Space Group...

  12. Study of microRNAs (miRNAs) that are predicted to target the autoantigens Ro/SSA and La/SSB in primary Sjögren’s Syndrome

    Science.gov (United States)

    Gourzi, V C; Kapsogeorgou, E K; Kyriakidis, N C; Tzioufas, A G

    2015-01-01

    The elevated tissue expression of Ro/SSA and La/SSB autoantigens appears to be crucial for the generation and perpetuation of autoimmune humoral responses against these autoantigens in Sjögren’s syndrome (SS). The mechanisms that govern their expression are not known. miRNAs, the post-transcriptional regulators of gene expression, might be implicated. We have identified previously the miRNAs let7b, miR16, miR181a, miR200b-3p, miR200b-5p, miR223 and miR483-5p that are predicted to target Ro/SSA [Ro52/tripartite motif-containing protein 21 (TRIM21), Ro60/TROVE domain family, member 2 (TROVE2)] and La/SSB mRNAs. To study possible associations with autoantigen mRNA expression and disease features, their expression was investigated in minor salivary gland (MSG) tissues, peripheral blood mononuclear cells (PBMC) and long-term cultured non-neoplastic salivary gland epithelial cells (SGEC) from 29 SS patients (20 of 29 positive for autoantibodies to Ro/SSA and La/SSB) and 24 sicca-complaining controls. The levels of miR16 were up-regulated in MSGs, miR200b-3p in SGECs and miR223 and miR483-5p in PBMCs of SS patients compared to sicca-complaining controls. The MSG levels of let7b, miR16, miR181a, miR223 and miR483-5p were correlated positively with Ro52/TRIM21-mRNA. miR181a and miR200b-3p were correlated negatively with Ro52/TRIM21 and Ro60/TROVE2 mRNAs in SGECs, respectively, whereas let7b, miR200b-5p and miR223 associated with La/SSB-mRNA. In PBMCs, let7b, miR16, miR181a and miR483-5p were correlated with Ro52/TRIM21, whereas let7b, miR16 and miR181a were also associated with La/SSB-mRNA expression. Significantly lower miR200b-5p levels were expressed in SS patients with mucosa-associated lymphoid tissue (MALT) lymphoma compared to those without. Our findings indicate that miR16, miR200b-3p, miR223 and miR483-5p are deregulated in SS, but the exact role of this deregulation in disease pathogenesis and autoantigen expression needs to be elucidated. PMID:26201309

  13. HTLV-I Associated uveitis, myelopathy, rheumatoid arthritis and Sjögren's syndrome Uveite, mielopatia, artrite reumatóide e sindrome de Sjogren associadas ao HTLV-I

    Directory of Open Access Journals (Sweden)

    Sônia Regina A. A. Pinheiro

    1995-12-01

    Full Text Available A 62 year-old white female presented with a 10-year-history of slowly progressive spastic paraparesis, pain and dysesthesia in the lower limbs and sphincter disturbance. A few years after the onset of the neurologic symptoms she developped migratory arthritis with swelling of the knees and pain on palpation of knees and fingers, dry eyes, mouth and skin. Two months before admission she presented bilateral nongranulomatous anterior uveitis. Examination revealed spastic paraparesis with bilateral Babinski sign, a decreased sensation level below L3, decreased vibration sense in the lower extremities, and a postural tremor of the upper limbs. Laboratory work-up disclosed HTLV-I positive tests in the blood and cerebrospinal fluid (CSF, and a mild pleocytosis in the CSF with a normal protein content. Nerve conduction velocity studies were normal. The present case shows the association of uveitis, arthritis and Sjögren's syndrome in a patient with tropical spastic paraparesis / human T-cell lymphotropic virus type I (HTLV-I associated myelopathy (TSP/HAM, and illustrates the wide spectrum of clinical manifestations which may accompany this infection with this virus.Uma mulher branca de 62 anos foi internada apresentando história de paraparesia lentamente progressiva durante 10 anos. Dois meses antes da internação ela apresentou uveíte anterior não granulomatosa bilateral. Poucos anos após o início dos sintomas neurológicos, ela desenvolveu artrite migratória com edema dos joelhos e dor a palpação dos joelhos e dedos dos pés, boca, pele e olhos secos. Ao exame físico foi observado paraparesia espástica com sinal de Babinski positivo, sensibilidade diminuída abaixo de L3, diminuição da sensação de vibração nas extremidades inferiores, e tremor postural dos membros superiores. Apresentou testes positivos para o HTLV-I no sangue. O estudo do líquido cefalorraquidiano mostrou discreta pleocitose, proteínas normais e ELISA e Western

  14. Clinical utility of circulating anti-N-methyl-d-aspartate receptor subunits NR2A/B antibody for the diagnosis of neuropsychiatric syndromes in systemic lupus erythematosus and Sjögren's syndrome: An updated meta-analysis.

    Science.gov (United States)

    Tay, Sen Hee; Fairhurst, Anna-Marie; Mak, Anselm

    2017-02-01

    Neuropsychiatric (NP) events are found in patients with rheumatic diseases, commonly in systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). The standard nomenclature and case definitions for 19 NPSLE syndromes by the American College of Rheumatology (ACR) Committee on Research cover a wide range of NP events seen in both SLE and SS. Despite advances in the understanding of SLE and SS, NP syndromes continue to pose diagnostic challenges. Correct attribution of NP events is critical in determining the correct treatment and prognosis. Anti-N-methyl- d -aspartate receptor subunits NR2A/B (anti-NR2A/B) antibodies have been demonstrated in the sera of SLE and SS patients and have been associated with collective or specific NP syndromes, though not consistently. Interpretation of anti-NR2A/B antibody data in the medical literature is rendered difficult by small sample size of patient groups. By combining different studies to generate a pooled effect size, a meta-analysis can increase the power to detect differences in the presence or absence of NP syndromes. Hence, we set out to perform a meta-analysis to assess the association between anti-NR2A/B antibodies and NP syndromes in SLE and SS. A literature search was conducted using PubMed and other databases from inception to June 2016. We abstracted data relating to anti-NR2A/B antibodies from the identified studies. The random effects model was used to calculate overall combined odds ratio (OD) with its corresponding 95% confidence interval (CI) to evaluate the relationship between anti-NR2A/B antibodies and NP syndromes in SLE and SS patients with and without NP events. We also included our own cohort of 57 SLE patients fulfilling the ACR 1997 revised classification criteria and 58 healthy controls (HCs). In total, 17 studies with data on anti-NR2A/B antibodies in 2212 SLE patients, 66 SS patients, 99 disease controls (DCs) (e.g. antiphospholipid syndrome, myasthenia gravis and autoimmune polyendocrine

  15. Hvorfor samtalen ikke er demokratiets sjæl ikke er demokratiets sjæl

    Directory of Open Access Journals (Sweden)

    Michael Schudson

    2001-09-01

    Full Text Available Medie- og kommunikationsforskningen har ofte opfattet samtalen, der føres ansigt-til-ansigt, som en hjørnesten i den demokratiske proces. Denne opfattelse stammer bl.a. fra medieforskeren James Carey, der selv har været inspireret af den amerikanske filosof John Dewey. I den- ne artikel præsenterer Michael Schudson en kritik af, hvad han kalder den romantiske forestilling om samtalen. Samtalen ansigt-til-ansigt har været lige så meget i højsædet i aristokratier som i demokratier, og der er faktisk to distinkte og modsatte idealer for samtale: den selskabelige samtale og den problemløsende samtale. Samtalen, der tjener demo- kratiet, er ikke kendetegnet ved egalitarisme, men ved normstyrethed og offentlighed, ikke ved spontanitet men ved høflighed, og ikke ved dens forrang eller overlegenhed i forhold til trykte medier, radio eller tv, men ved dens nødvendige afhængighed af dem. Der argumenteres for, at institutioner og demokratiske normer giver anledning til demokratiske samtaler, snarere end at samtalens iboende demokrati fører til politisk demokratiske normer og institutioner. Artiklen er oversat af Stig Hjarvard.

  16. Hvorfor samtalen ikke er demokratiets sjæl ikke er demokratiets sjæl

    Directory of Open Access Journals (Sweden)

    Michael Schudson

    2001-09-01

    Full Text Available Medie- og kommunikationsforskningen har ofte opfattet samtalen, der føres ansigt-til-ansigt, som en hjørnesten i den demokratiske proces. Denne opfattelse stammer bl.a. fra medieforskeren James Carey, der selv har været inspireret af den amerikanske filosof John Dewey. I den- ne artikel præsenterer Michael Schudson en kritik af, hvad han kalder den romantiske forestilling om samtalen. Samtalen ansigt-til-ansigt har været lige så meget i højsædet i aristokratier som i demokratier, og der er faktisk to distinkte og modsatte idealer for samtale: den selskabelige samtale og den problemløsende samtale. Samtalen, der tjener demo- kratiet, er ikke kendetegnet ved egalitarisme, men ved normstyrethed og offentlighed, ikke ved spontanitet men ved høflighed, og ikke ved dens forrang eller overlegenhed i forhold til trykte medier, radio eller tv, men ved dens nødvendige afhængighed af dem. Der argumenteres for, at institutioner og demokratiske normer giver anledning til demokratiske samtaler, snarere end at samtalens iboende demokrati fører til politisk demokratiske normer og institutioner. Artiklen er oversat af Stig Hjarvard.

  17. Genetics Home Reference: Sjögren-Larsson syndrome

    Science.gov (United States)

    ... in a type of brain tissue called white matter. White matter consists of nerve fibers covered by a substance ( ... for Links Data Files & API Site Map Subscribe Customer Support USA.gov Copyright Privacy Accessibility FOIA Viewers & ...

  18. ME O uwehand , Het groeiende kruis, GF Callenbach, Nijkerk, SJ ...

    African Journals Online (AJOL)

    Test

    de taal en gedagtes. Sagaria, die vader van die Doper, is eerste aan die woord. Hy ken die moderne digter Aafjes, en dis nie ver gesog nie want hy is self 'n digter of liewer het 'n ... Hy eindig, „Stel u voor, dat u in uw tijd weer de lof van God leerde ... het om met 'n man uit die geslag van Dawid te trou, om die moeder te.

  19. Forbrugerens sjæl og branding med succes

    DEFF Research Database (Denmark)

    Hermansen, Judy

    2008-01-01

    Branding er en slags besjæling af produkter - det er at tage en genstand, som bare er et fysisk, funktionel ting og så få den til at betyde noget for nogen. Det, der karakteriserer brands er netop, at vi føler noget overfor dem. Det kan godt være vi føler afsky - men vi føler noget. Og vi inddrager...

  20. Malignt peritonealt mesoteliom er en sjælden sygdom

    DEFF Research Database (Denmark)

    Poulsen, Anja; Pedersen, Gitte

    2014-01-01

    Malignant peritoneal mesothelioma is characterized by diffuse symptoms such as bloating, abdominal pain, weight loss, anorexia, fever, diarrhoea, vomiting and ascites. The findings by radiographic imaging are unspecific and the diagnosis is therefore often first achieved by biopsy from the affected...... area. Although treatment with chemotherapy and cytoreductive surgery has improved the survival prognosis for the patients is poor with a median survival of 38 month....

  1. Genetics Home Reference: Sjögren syndrome

    Science.gov (United States)

    ... syndrome in particular. The inheritance pattern of this predisposition is unknown. Related Information What does it mean if a disorder seems to run in my family? What are the different ways in which a genetic condition can be inherited? More about Inheriting Genetic ...

  2. Sjæl eller sex - en havfrues metamorfoser

    OpenAIRE

    Poulsen, Agnethe

    2012-01-01

    Specialet tager udgangspunkt i den kritiske holdning til Disneys tegnefilm, som bl.a. folklorist Jack Zipes har stået for, og den modvilje mod Disneys filmatisering af H.C.Andersens Den lille havfrue (1837), der synes gennemgående i den vestlige akademiske litteratur. For at undersøge kritikken nærmere har jeg valgt at sætte Disneys The Little Mermaid (1989) i forhold til Hayao Miyazakis animefilm Gake No Ue no Ponyo (2008), en anden H.C.Andersen-adaptation, og sammenligne de to film med der...

  3. Malignt peritonealt mesoteliom er en sjælden sygdom

    DEFF Research Database (Denmark)

    Poulsen, Anja; Pedersen, Gitte

    2014-01-01

    Malignant peritoneal mesothelioma is characterized by diffuse symptoms such as bloating, abdominal pain, weight loss, anorexia, fever, diarrhoea, vomiting and ascites. The findings by radiographic imaging are unspecific and the diagnosis is therefore often first achieved by biopsy from the affected...

  4. The position of the political parties before the general election in 2007 from a marketing perspective Markaðsfræðilegt sjónarhorn á stöðu stjórnmálaflokka fyrir alþingiskosningarnar 2007

    Directory of Open Access Journals (Sweden)

    Þórhallur Guðlaugsson

    2008-06-01

    Full Text Available Abstract in English is unavailable.Viðfangsefni greinarinnar er mat á staðfærslu stjórnmálaflokka og hvernig ímynd og staðfærsla tengjast faglegu markaðsstarfi, samkeppnishæfni og árangri. Fjallað er um niðurstöður rannsóknar þar sem lagt er mat á staðfærslu stjórnmálaflokka hjá ungu fólki og skoðað með hvaða hætti hún endurspeglar árangur í kosningum. Í þessu sambandi eiga stjórnmálaflokkar í nokkrum vanda. Stjórnmálaflokkar eru ekki fyrirtæki þar sem hægt er að gefa út tiltekna línu hvað samskipti og skilaboð varðar. Vissulega reyna menn þó að stilla saman strengi sína, sérstaklega í kosningabaráttunni. Þar virðast flestir flokkar leggja áherslu á loforð, þ.e. kjósi menn flokkinn þá muni þetta eða hitt gerast eða breytast eftir kosningar. Fólk virðist þó ekki gera ráð fyrir því að stjórnmálamenn standi við gefin loforð og virðast margir bera meira traust til einstaklinga almennt en stjórnmálamanna. Kjósendur hafa iðulega óljósa mynd af því fyrir hvað tiltekinn stjórnmálaflokkur stendur en ýmislegt bendir til þess að tengsl séu á milli staðfærslu og árangurs í kosningum. Þrír stjórnmálaflokkar virðast hafa skýra og afgerandi stöðu hjá ungu fólki, Sjálfstæðisflokkurinn, Vinstrihreyfingin grænt framboð og Framsóknarflokkurinn. Tveir þeirra tengjast jákvæðum eiginleikum en einn neikvæðum gildum. Tveir stjórnmálaflokkar virðast hafa óljósa stöðu í huga ungra kjósenda, Samfylkingin og Frjálslyndi flokkurinn. Hafa þarf í huga að staðfærsla verður ekki mæld í eitt skipti fyrir öll og mikilvægt að mæla hana reglulega ef niðurstöðurnar eiga að nýtast í faglegu markaðsstarfi flokkanna.

  5. Viral mimicry of the complement system

    Indian Academy of Sciences (India)

    Unknown

    et al 2002), hepatitis C virus (Ishii et al 2001) and HIV. (Ezekowitz et al 1989; Saifuddin et al 2000). Activation of the complement system in the absence of proper regulation can lead to virus neutralization. The various mechanisms which are known to inactivate viruses are: (i) neutralization by complement dependant ...

  6. Cooling rate of chondrules in ordinary chondrites revisited by a new geospeedometer based on the compensation rule

    Science.gov (United States)

    Béjina, Frédéric; Sautter, Violaine; Jaoul, Olivier

    2009-01-01

    For several decades efforts to constrain chondrite cooling rates from diffusion zoning in olivine gave rise to a range of values from 5 to 8400 K/h (Desch, S.J., Connolly Jr., H.C., 2002. A model for the thermal processing of particles in solar nebula shocks: application to cooling rates of chondrules. Meteorit. Planet. Sci. 37, 183-208; Greeney, S., Ruzicka, A., 2004. Relict forsterite in chondrules: implications for cooling rates. Lunar Planet. Sci. XXXV, abstract # 1246.). Such large uncertainties directly reflect the variability of diffusion data. Alternatively, from this variability results a compensation rule, log D0 = a + bE (diffusion coefficients are written D = D0 exp(- E/ RT)). We test a new geospeemetry approach, based on this rule, on cooling of chondrules in chondrites, Sahara-97210 LL 3.2 and Wells LL 3.3. Greeney and Ruzicka (2004) matched Fe-Mg diffusion profiles in olivine from these chondrites with cooling rates between 200 and 6000 K/h. In our geospeedometry model, the use of the compensation rule greatly reduces the uncertainties by avoiding the choice of one diffusion coefficient among many. The cooling rates we found are between 700 and 3600 K/h for Sahara and 700-1600 K/h for Wells. Finally, we discuss the influence of our analytical model parameters on our cooling rate estimates.

  7. Psychological profiles in patients with Sjögren's syndrome related to fatigue: a cluster analysis.

    Science.gov (United States)

    van Leeuwen, Ninke; Bossema, Ercolie R; Knoop, Hans; Kruize, Aike A; Bootsma, Hendrika; Bijlsma, Johannes W J; Geenen, Rinie

    2015-05-01

    Fatigue is a highly prevalent and debilitating symptom in the autoimmune disease SS. Although the disease process plays a role in fatigue, psychological factors may influence fatigue and the ability to deal with its consequences. Profiles of co-occurring psychological factors may suggest potential targets for the treatment of fatigue. The aim of this study was to identify psychological profiles in patients with SS and the accompanying levels of fatigue. Three hundred patients with primary SS (mean age 57 years, 93% female) completed questionnaires on fatigue (multidimensional fatigue inventory), physical activity cognitions (TAMPA-SK), illness cognitions, cognitive regulation, emotion processing and regulation [Toronto Alexithymia Scale 20, Emotion Regulation Questionnaire (ERQ), Berkeley Expressivity Questionnaire], coping strategies (Brief COPE) and social support. Principal axis factor analysis (oblimin rotation) yielded six psychological factors: social support, negative thinking, positive thinking, emotional expressivity, avoidance and alexithymia (i.e. the inability to differentiate emotions). Using cluster analyses, these factors were grouped in four psychological profiles: functional (39%), alexithymic (27%), self-reliant (23%) and dysfunctional (11%). Irrespective of the psychological profile, the level of fatigue was substantially higher in patients than in the general population. Patients with a dysfunctional or an alexithymic profile reported more fatigue than those with a self-reliant profile. Our study in SS yielded four psychological profiles that were differentially associated with fatigue. These profiles can be used to examine determinants and prognosis of fatigue as well as the possibility of customizing cognitive behavioural interventions for chronic fatigue. © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  8. Medveten lärare? : Om självreflektion i undervisning

    OpenAIRE

    Palm, Katarina

    2008-01-01

    In this paper I have examined how aware teachers are of their influence on pupils development. The method I´ve used are interviews with three teachers and one pupil each about Musicality - how do the teachers and pupils look at the concept musicality generally and personally? Influence I- do the teachers believe that their thinking about musicality influence the pupils self-confidence and development? Influence II- do the teachers think that other pedagogues have influenced them in their teac...

  9. Stiff baby syndrome er en sjælden årsag til neonatal hypertonicitet

    DEFF Research Database (Denmark)

    Rønne, Maria Sode; Nielsen, Preben Berg; Mogensen, Christian Backer

    2014-01-01

    Stiff baby syndrome (hyperekplexia) is a rare genetic disorder. The condition can easily be misdiagnosed as epilepsy or severe sepsis because of hypertonicity and seizure-like episodes and has an increased risk of severe apnoea and sudden infant death. Tapping of the nasal bridge inducing a start...... response is the clinical hallmark. We report cases of two sisters born with stiff baby syndrome with hypertonicity, exaggerated startle reaction and cyanosis. The syndrome has a good prognosis if treated with clonazepam and both cases were developmental normal after one year....

  10. Aplasia cutis congenita er en sjælden og mulig overset kongenit anomali

    DEFF Research Database (Denmark)

    E Rogvi, Rasmus; Sommerlund, Mette; Vestergaard, Esben Thyssen

    2014-01-01

    Aplasia cutis congenita (ACC) is a rare congenital defect of the skin. In this study we present the diagnosis and management of the condition. In 1997-2009 a total of 65 ACC cases were registered in the Danish National Patient Registry (1:13.000 births). The mortality among these cases was 1...

  11. Exploring BAFF: its expression, receptors and contribution to the immunopathogenesis of Sjögren's syndrome.

    Science.gov (United States)

    Thompson, Nicolyn; Isenberg, David A; Jury, Elizabeth C; Ciurtin, Coziana

    2016-09-01

    SS is an autoimmune condition characterized by exocrine gland destruction, autoantibody production, immune complex deposition and systemic complications associated with lymphocytic infiltration of many organs. Genetic, environmental and viral factors play a role in disease aetiology, however, the exact mechanisms driving the immunopathogenesis of SS remain uncertain. Here we discuss a role for B cell activating factor (BAFF), whereby B cell hyperactivity and increased BAFF secretion observed in patients and animal models of the disease can be explained by the altered expression of cell-specific BAFF/BAFF receptor (BAFF-R) variants in several immune cell types. Understanding the role of BAFF/BAFF-R heterogeneity in SS pathogenesis could help to facilitate new treatment strategies for patients. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  12. Fatigue in patients with Sjögren's syndrome and intervention of ...

    African Journals Online (AJOL)

    With a long history, Traditional Chinese Medicine (TCM) as alternative therapy has become increasingly popular among patients with various kinds of disease, especially in pSS. Based on the unique principle of therapy, practitioners have achieved a satisfactory effect on relieving disease related symptoms with Chinese ...

  13. Sjögren's and plasma cell variant Castleman disease: a case report ...

    African Journals Online (AJOL)

    Castleman disease is a rare cause of lymphoid hyperplasia and may result in localized symptoms or an aggressive, multisystem disorder. It can mimic other diseases like lymphoma or tuberculosis. It classically presents as a mediastinal mass that involves the lymphatic tissue primarily but can also affect extra lymphatic sites ...

  14. Stiff baby syndrome er en sjælden årsag til neonatal hypertonicitet

    DEFF Research Database (Denmark)

    Rønne, Maria Sode; Nielsen, Preben Berg; Mogensen, Christian Backer

    2014-01-01

    Stiff baby syndrome (hyperekplexia) is a rare genetic disorder. The condition can easily be misdiagnosed as epilepsy or severe sepsis because of hypertonicity and seizure-like episodes and has an increased risk of severe apnoea and sudden infant death. Tapping of the nasal bridge inducing a start...

  15. Ung same i Sverige : livsvillkor, självvärdering och hälsa

    OpenAIRE

    Omma, Lotta

    2013-01-01

    Background The Sami are the indigenous people in Scandinavia. They have a long history of discrimination, racism and conflict which has had a significant impact on Sami self-esteem and possibly also on their health, especially mental health. There are some recent studies on the mental health of reindeer herding Sami in Sweden showing a high prevalence of self reported depression and anxiety compared to other Swedes in the area. Also a moderately elevated risk of suicide amongst reindeer herdi...

  16. Mycobacterium chelonae keratitis in a patient with Sjögren's syndrome

    NARCIS (Netherlands)

    van der Beek, M. T.; Bernards, A. T.; Lapid-Gortzak, R.

    2008-01-01

    In this report a case of Mycobacterium chelonae keratitis in a patient without any previously described risk factors is described. The only risk factor found was a rheumatoid arthritis related Sjogren''s syndrome. Case report. A 60-year-old woman was referred to the hospital with an infectious

  17. O LEGADO HUMANO, TEOLÓGICO E ESPIRITUAL DE J.B. LIBANIO SJ

    Directory of Open Access Journals (Sweden)

    Leonardo Boff

    2014-01-01

    Full Text Available O escritor e teólogo Leonardo Boff presta sua homenagem a João Batista Libanio, discorrendo acerca do legado humano, teológico e espiritual desse grande teólogo jesuíta. Fazendo uma espécie de “leitura de cego”, segundo as palavras do próprio autor, ele capta relevâncias da pessoa, atividade teológica e obra literária de Libanio. Durante anos, compartilharam sonhos e realizaram projetos. Participaram intensamente na forja dos anos dourados da Igreja e da Teologia no Brasil. Encontram-se, ambos, na origem das Comunidades Eclesiais de Base e da Teologia da Libertação. Juntos assessoraram por muitos anos a CRB (Conferência dos Religiosos do Brasil e a CNBB (Conferência Nacional dos Bispos do Brasil. Leonardo Boff inicia seu texto dizendo que Libanio é “o amigo que nunca perdi” e conclui confessando ser graça do Altíssimo ter tido como companheiro de caminhada “um amigo e um irmão dessa grandeza”. ABSTRACT: The writer and theologian Leonardo Boff pays his homage to João Batista Libanio, talking about the human, theological and spiritual legacy of this great Jesuit theologian. Doing a sort of “blind reading”, according to the words of the author himself, he captures relevancies of person, theological activity and literary work of Libanio. For years, they shared dreams and accomplished projects together. They also participated intensely in the forging of the golden years of the Church and theology in Brazil. Both are at the origin of the “Comunidades Eclesiais de Base” and of Liberation Theology. Together they accompanied for many years the CRB (Conference of Religious of Brazil and the CNBB (National Conference of Bishops of Brazil. Leonardo Boff begins his text saying that Libanio is “the friend who I never lost” and concludes saying that it was by the grace of the Almighty to have had as a companion on the journey “a friend and a brother of such grandeur”.

  18. Primary Sjögren's syndrome associated agranulocytosis: a benign disorder?

    Science.gov (United States)

    Coppo, P; Sibilia, J; Maloisel, F; Schlageter, M; Voyer, A; Gouilleux-Gruart, V; Goetz, J; Desablens, B; Tribout, B; Lassoued, K

    2003-01-01

    Methods: The clinical, haematological, and immunological features of seven patients with primary SS associated with a chronic (>6 months) agranulocytosis, and the outcome of the patients, were analysed. Results: Patients were white women with an unexplained agranulocytosis. They all had non-erosive arthritis and three had a thrombocytopenia or Evan's syndrome. In three patients, transient or durable expansion of T lymphocytes was present in the peripheral blood or in the bone marrow, but evolved independently from neutrophil counts. There was no paroxysmal nocturnal haemoglobinuria clone or antibodies to neutrophil surface antigens. In vitro bone marrow culture was normal (four patients) or showed a decrease in colony forming unit-granulocyte monocyte (CFU-GM) and colony forming unit-erythroblast (CFU-E) (one patient). Serum levels of soluble Fas ligand (sFasL) were normal, and granulocyte-colony stimulating factor (G-CSF) concentrations were either normal or raised. One patient was treated with steroids associated with intravenous immunoglobulins and achieved a lasting response. Two other patients were treated with steroids and methotrexate, with poor efficacy. Short courses of subcutaneous G-CSF produced a transient and mild response in all three patients. Complete recovery of the neutrophils occurred temporarily during pregnancy in two patients. After a mean follow up of 34.8 months (range 6–139) all patients were alive and none developed serious infections. Conclusion: A subset of patients with primary SS and non-destructive arthritis may develop a chronic but well tolerated agranulocytosis that is usually poorly responsive to steroids and oral methotrexate. PMID:12695164

  19. Primary Sjögren's syndrome associated agranulocytosis: a benign disorder?

    OpenAIRE

    Coppo, P; Sibilia, J; Maloisel, F; Schlageter, M; Voyer, A; Gouilleux-Gruart, V; Goetz, J; Desablens, B; Tribout, B; Lassoued, K

    2003-01-01

    Methods: The clinical, haematological, and immunological features of seven patients with primary SS associated with a chronic (>6 months) agranulocytosis, and the outcome of the patients, were analysed.

  20. R/V Seward Johnson Cruise Report (SJ-9807) ACCE S-PALACE Float Deployments

    National Research Council Canada - National Science Library

    Montgomery, Ellyn

    1999-01-01

    ...). These floats are neutrally buoyant and drift with the water in which they are deployed. They are programmed to obtain temperature and salinity profiles of the top 1000 meters of the ocean every ten days...

  1. Självkänsla och självpresentation online : En studie kring några individers självpresentationer i form av träningsbilder på Instagram och hur den upplevda självkänslan påverkar/påverkas av detta.

    OpenAIRE

    Bostedt, Malin

    2013-01-01

    Through the birth and arise of the Internet people has been given great opportunities to make selective selfpresentations online, which in the long run has resulted in an easier retaining of the self- esteem than in the offline world. Simultaneously, the self- esteem has been viewed as a cause for both success and failure since it plays a vital role regarding our attitudes, emotional reactions and behavior. On this basis I wanted to study the relation between self- esteem and self-presentatio...

  2. Philosophy In-sistencial at present: the in-sistencial communitarian personalism of Ismael Quiles SJ

    Directory of Open Access Journals (Sweden)

    Raúl Francisco Sebastián Solanes

    2016-05-01

    Full Text Available This text presents the proposal of In-sistencial philosophy developed by Ismael Quiles, analyzing various aspects. The first is to place Quiles in the philosophical and historical context, analyzing his intellectual formation in Spain and in his exile in Argentina, seeing the stages of his thought. Secondly, I see Quiles relations with existentialism of Heidegger, Sartre and Gabriel Marcel. Third, I try to justify that his thinking belongs to the current communitarian personalism, but not shed Buber’s Jewish orientation, but Christian. Lastly argue that despite his adherence to Christianity, we can not consider him just as a Christian philosopher, but as a philosopher who, influenced by Eastern thought, who opens in-sistencial philosophy to mysticism.

  3. Självkörande fordon : En analys av energianvändning och kapacitet

    OpenAIRE

    Örarbäck, Jonas

    2015-01-01

    Self-driving cars are soon to become a reality to the commercial market. A self-driving car does not have a human operator, which enables some interesting characteristics. The main benefits are considered to be: The number of accidents and traffic jams should be reduced. Enhanced comfort. Energy usage could be decreased while capacity could be increased. This study concerns the causes for improved energy efficiency. The reason for improved energy efficiency is mainly because of the variation...

  4. A new medication for treatment of dry mouth in Sjögren's syndrome.

    Science.gov (United States)

    al-Hashimi, I; Taylor, S E

    2001-04-01

    Traditionally, treatment of dry mouth in SS is focused on palliative measures (using salivary substitutes). However, due to the dynamic nature of the oral cavity, the salivary substitute is removed from the mouth during swallowing. Therefore, the duration of effect of salivary substitutes is short. Another drawback is that salivary substitutes do not provide the protective roles of saliva. Effective treatment of dry mouth requires increasing salivary output. Gustatory stimulation of the salivary glands with sugar free gum and sugar free candies may be effective in inducing salivary output, however, they impose significant inconvenience on the patient which can compromise compliance. Pharmacological stimulants provide an alternative effective measure and improve compliance. Both Salagen and Evoxac are FDA approved salivary stimulants. They are effective, and safe given awareness of their indications, contraindications, potential adverse effects, and patient's tolerance.

  5. "Silja Line is back in business" / Pär-Henrik Sjöström

    Index Scriptorium Estoniae

    Sjöström, Pär-Henrik, 1954-

    2008-01-01

    Pärast Silja liitumist Tallink Groupiga on täieliku muutumise läbi teinud organisatsioon taas konkurentsis, kaubamärgid Tallink ja Silja Line. 2009. a. jaanuaris selgub uue kruiisilaeva Cruise 5 marsruut. Kaubaveo areng. Vt. samas: Kaks kaubamärki

  6. Classes and problems of natural philosophy of P. Francisco Mendoça SJ

    Directory of Open Access Journals (Sweden)

    Carlota Miranda Urbano

    2016-08-01

    Full Text Available The author presents De floribus Philosophiae written by the famous Jesuit Francisco Mendoça (†1626, professor of Philosophy and rector of the College of Arts in Coimbra in the first years of the XVIIth century. The work was published in a thick volume edited by the said College after the author’s death with the title Viridarium Sacrae et profanae eruditionis a real monument of encyclopaedic knowledge. 47 problemas are presented in De floribus Philosophiae, pertaining to Geography, Anatomy, Physics, Psychology, Astronomy and Anthropology. The text can thus be read as a contribution to the history of science.

  7. SAMEW, SJ", MQHAMTVEEIT, E09 OJQQ SBAG and ESlE\\/0'1 ...

    African Journals Online (AJOL)

    Notomelia is a congenital malformation that is characterized by the presence of additional limb(s) attached to the back. According to the manifestations, notomelia can be classified into two groups. One group is characterized by symmetric or asymmetric double malformations (twin malformations) ~ a partial or total doubling ...

  8. 13- :K 3 =SJR.:K ~ ;Sj13 ~~K

    African Journals Online (AJOL)

    Brink vir fondse gepleit om die werksaamhede te kan voortsit. Finansiele hulp uit die han- dels- en nywerheidswereld, van oud-stryders- organisasies, die SA Weermag, die landsower- hede, die RGN en die publiek e.a. het gevolg. In Johannesburg het die Adviserende Komitee in verband met die Suid-Afrikaanse oorlogs-.

  9. Sult er en sjælden årsag til metabolisk acidose

    DEFF Research Database (Denmark)

    Vestergaard, Thea; Aaen, Jeppe Frøkjær; Bruun, Jens Meldgaard

    2014-01-01

    of infection, dehydration and normoglycaemic metabolic acidosis with elevated P-3-hydroxybutyrate. The case presents a starvation- (weight loss ~ 13 kg) and infection-induced non-diabetic metabolic ketoacidosis treated with intravenous supplementation of isotonic saline, potassium, bicarbonate and insulin....

  10. Spinale epidurale abscesser er sjældne og kræver hurtig intervention

    DEFF Research Database (Denmark)

    Skaaby, Tea; Topsøe, Jakob Fink; Dragsted, Ulrik Bak

    2014-01-01

    , resulting in grave neurological sequelae and death. Common risk factors are diabetes mellitus and intravenous drug abuse. Successful treatment relies on a multidisciplinary approach. We describe a case of spinal epidural abscesses in a previously healthy young man without known risk factors.......A spinal epidural abscess is a rare infectious disease that warrants urgent treatment. Symptoms involve a classic triad of fever, backache and neurological deficits but all the symptoms are rarely seen at the first contact. The low incidence and the non-specific symptoms can delay the diagnosis...

  11. Isovalerianeacidæmi--en sjælden og alvorlig defekt i nedbrydningen af leucin

    DEFF Research Database (Denmark)

    Lund, Ann-Britt Kiholm; Lund, Allan

    2011-01-01

    Isovaleric acidaemia (IVA) is an organic acidemia caused by deficient metabolism of the essential amino acid leucine. We describe the biochemistry, diagnostics, and treatment of IVA, and present the known Danish patients....

  12. Healing ministry among the Zulu speaking people troubled by evil spirits / S.J. Veenstra

    OpenAIRE

    Veenstra, Sietse Jan

    2006-01-01

    The main aim of this study is to let the light of the Scriptures fall on the occult schemes of the devil that has such a strong hold on many Zulu-speaking people by the .ancestral veneration and the seduction of spirits among them, and to propose a reformed/biblical healing ministry. The method of research followed in this study was to study literature and to get the necessary information through interviews, but the main sources of input were the Old and New Testament. The c...

  13. Polyorkisme er en sjælden årsag til udfyldning i scrotum

    DEFF Research Database (Denmark)

    Padkær, Grete; Mahdi, Bassam Hamid; Andersen, Poul Erik

    2017-01-01

    of which the most were malignant. If the supernumerary testicle is not contributing to the spermatogenesis, it is therefore recommended to remove the testicle. Otherwise, recommendations are a yearly follow-up by Doppler ultrasound, including a physical examination to discover development of cancer.......In this case report we present a 14-year-old boy with two testicles in the right hemiscrotum - a case of polyorchidism. He did not have surgery for his condition, and his condition was followed according to the guidelines. In a study, 6.4% of cases with polyorchidism were found to contain neoplasms...

  14. Syndrome de Gougerot-Sjögren primitif du sujet âgé ...

    African Journals Online (AJOL)

    Les objectifs de notre etude est d'etudier les caracteristiques cliniques et immunologiques du syndrome de Gougerot Sjogren primitif (SGSp) du sujet age et les comparer a une population temoin plus jeune. Nous avons analyse retrospectivement les dossiers de 60 patients atteints de SGSp consecutifs, suivis au service de ...

  15. Work disability in newly diagnosed patients with primary Sjögren syndrome

    DEFF Research Database (Denmark)

    Mandl, Thomas; Jørgensen, Tanja Schjødt; Skougaard, Marie

    2017-01-01

    -disabled, while 37% and 41% were disabled at 12 and 24 months after diagnosis, respectively (p diagnosis (OR 15.4, 95% CI 2.9-81.9; p = 0.001), concomitant fibromyalgia (OR 10.5, 95% CI 2.0-56.0; p = 0.006), and each additional year of age (OR 1...... at diagnosis, concomitant fibromyalgia, and increasing age, but not anti-SSA/anti-SSB antibodies or disease activity, were associated with longterm work disability....... Agency and calculated the proportion as well as net days of work disability in 30-day intervals from 12 months before pSS diagnosis until 24 months after . Results. Work disability was increased in patients with pSS in comparison to general population comparators. At diagnosis, 26% of patients were work...

  16. Spinale epidurale abscesser er sjældne og kræver hurtig intervention

    DEFF Research Database (Denmark)

    Skaaby, Tea; Topsøe, Jakob Fink; Dragsted, Ulrik Bak

    2014-01-01

    , resulting in grave neurological sequelae and death. Common risk factors are diabetes mellitus and intravenous drug abuse. Successful treatment relies on a multidisciplinary approach. We describe a case of spinal epidural abscesses in a previously healthy young man without known risk factors....

  17. Uretral cancer er sjælden og vanskelig at diagnosticere

    DEFF Research Database (Denmark)

    Klemann, Nina; Toft, Birgitte Grønkjær; Thind, Peter

    2014-01-01

    Primary cancer of the urethra is a rare condition with symptoms ranging from haematuria, urethral obstruction and pelvic pain to urethrocutaneous fistula and abscess. Magnetic resonance imaging (MRI) is considered first choice of diagnostic imaging, but experience with treatment is scarce. We pre...

  18. Innovative Columnar Type of Grid Array SJ BIST HALT Method, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — Ridgetop will develop a superior method for testing and qualifying columnar type of grid arrays such as field programmable gate arrays (FPGAs) packaged in column...

  19. Sjælden immundefekt bag svær atopisk dermatitis i barnealderen

    DEFF Research Database (Denmark)

    Wolsk, Helene Mygind; Marquart, Hanne V; Laub, Bodil

    2015-01-01

    Two children are presented with autosomal recessive hyper IgE syndrome caused by a mutation in the dedicator of cytokinesis 8 gene (DOCK8). The manifestations are typically severe atopic dermatitis, food allergies, elevated serum IgE concentration, viral skin infections and risk of malignancies....... DOCK8 deficiency was first reported in 2009, following the death of the oldest sibling. The youngest sibling was cured after allogenic stem cell transplantation. This case report illustrates the need of awareness of primary immunodeficiency in children with atypical manifestation of atopic dermatitis...... in combination with recurrent infections....

  20. Sjöfartens långsiktiga drivmedelsförsörjning

    OpenAIRE

    Kågeson, Per

    2012-01-01

    This study compares the effectiveness of potential future shipping fuels in terms of cost and climate change impact and in particular the pros and cons of LNG compared to liquefied fuels. Today there are only a small number of LNG-fuelled ships and none that use FTdiesel, methanol or DME. This makes the comparison complicated. However, both LNG and methanol are traded in large quantities. In his choice of fuel the owner has to consider the individual vessels energy intensity and remaining yea...

  1. New Genetic Susceptibility Factors for Sjögren's Syndrome Revealed

    Science.gov (United States)

    ... the newly identified genetic risk factors have a role in the immune system, which fits well with the immune dysfunction that ... as well, suggesting that they play a broader role in disturbing the immune system. For example, IRF5, STAT4, and TNIP1 have all ...

  2. Isovalerianeacidæmi--en sjælden og alvorlig defekt i nedbrydningen af leucin

    DEFF Research Database (Denmark)

    Lund, Ann-Britt Kiholm; Lund, Allan

    2011-01-01

    Isovaleric acidaemia (IVA) is an organic acidemia caused by deficient metabolism of the essential amino acid leucine. We describe the biochemistry, diagnostics, and treatment of IVA, and present the known Danish patients.......Isovaleric acidaemia (IVA) is an organic acidemia caused by deficient metabolism of the essential amino acid leucine. We describe the biochemistry, diagnostics, and treatment of IVA, and present the known Danish patients....

  3. Intestinal tuberkulose, en sjælden differentialdiagnose til Crohns sygdom hos en etnisk dansker

    DEFF Research Database (Denmark)

    Alexandraki, Maria Joanna; Wejse, Christian; Esbjørn, Mette

    2015-01-01

    We report a case of intestinal tuberculosis in a 42-year-old Danish woman with stomach pain, weight loss and diarrhoea for months suspective of Crohn‘s disease. She underwent hysterectomy where white, small nodules were found on the small intestine. Biopsies showed non-necrotizing granulomatous...

  4. The Academic Reward System is the Primary Influence Toward Faculty Non-Participation in Institutional Repositories. A review of: Davis, Phillip M., and Matthew J.L. Connolly. “Institutional Repositories: Evaluating the Reasons for Non‐Use of Cornell University’s Installation of DSpace.” D‐Lib Magazine 13.3/4 (2007. 16 Oct. 2007 .

    Directory of Open Access Journals (Sweden)

    Kurt Blythe

    2007-12-01

    Full Text Available Objective – To better understand the lack of faculty participation in Cornell University’s DSpace institutional repository (IR, and to learn if this lack of participation is peculiar to Cornell or reflective of a larger trend in faculty non-participation in IRs.Design – Comparative analysis and interviews.Setting – Cornell University’s DSpace IR and sciences, social sciences, and humanities faculties; and DSpace installations at 7 other universities.Subjects – The DSpace IR at Cornell University and at 7 other locations. Eleven sciences, social sciences, and humanities faculty members at Cornell University.Methods – The authors analyzed data over a fifteen‐month period from Cornell’s DSpace IR to determine the total deposits, the types of objects deposited, the communities and collections that received deposits, the frequency of deposits, the IP addresses which made deposits, and how often objects in the IR were viewed. These data were compared to equivalent data taken from seven other IRs on all aspects except deposits from IP addresses and how oftenobjects were viewed. Finally, 11 Cornell faculty members from various departments in the sciences, social sciences, and humanities were interviewed over a two month period to provide context to the comparative analysis.Main results – At the time of the study, the IR at Cornell was organized into 193 communities of collections. These collections numbered 196, with 139 of them holding a combined total of 2646 objects: The other 57 collections were empty. While the IR as a whole showed steady growth, 77% of Cornell’s collections reflected a plateau growth pattern of primarily “one time deposits,” approximately 18% exhibited a stair‐step growth pattern of“periodic batch additions of material,” approximately 3% showed steady growth, and 1.4% were “uncatagorizable.” Five hundred nineteen unique IP addresses made deposits to Cornell’s IR over the course of the fifteen-month study, but 50% of these deposited only one object, and only 32 IP addresses deposited 10 or more objects. Of the other IRs studied, the lowest number of communities is zero and the highest is 390, the number of collections ranged from 10 to 282, and the number of objects ranged from 500 to 32,676. In most statistical categories, Cornell fell in the midrange. The two repositories with the fewest communities and collections – zero communities and 18 collections in one instance, and 6 and 10 in the other – are the only two with no empty collections. The repository with the most communities and collections also had the most empty collections (58%. The repository with the most objects was the one with zero communities and only 18 collections; and the repository with the fewest objects was the one with only 6 communities and 10collections. The third largest IR, with 3111 objects, had far and away the highest rate of steady growth (16.7%; while the IR with the most objects had the highest rate of stairstep growth (56.3%, and was the only IR to have a higher percentage of growth in any category other than plateau. Interviews with faculty indicated that they do not make deposits to IRs for a number of reasons. Faculty considered their primary audience to be their peers, so access to their scholarship was largely considered a “nonissue” as it was adequately provided through personal Web pages, subject repositories, or journal literature. Likewise, long-term preservation was not an overarching area of concern. The chief factors for not using an IR, however, all revolved around restrictions brought on by the academic reward system. Questions of copyright and whether depositing objects qualifies as publishing, thereby hindering efforts to publish in journals, were paramount, as were fears that depositing scholarship alongside less rigorous works in a catch-all IR would diminish the work and the reputation of the scholar by association. Hesitancy to make work available before ithad been certified and peer-reviewed was also a foremost concern. Conclusion – Although objects in Cornell’s DSpace are accessed both locally for items that are tied into the curriculum, and outside of the university for items that are of national (and international interest, the repository was not supported well by the faculty. The majority of the collections defined in Cornell’s IR were under populated, and what growth was evident arose primarily from deposits made by nonfaculty. The reasons for this were manifold, but centered primarily on the established culture of the academic reward system, which encourages publishing in recognized journals and does little to foster thoughts for long-term preservation or dissemination outside of a given scholar’s peer group. These issues were evident in faculty concerns that depositing materials in an IR might prevent later publication in a journal; the idea that depositing scholarship in a non‐vetted repository would diminish thatwork by association with less scholarly materials; the feeling in some fields that it would be irresponsible to provide access to any unfinished, non-vetted work; the thought that IRs are not sufficient to the task of certifying scholarship; and the concern that deposit in an IR might lead to plagiarism or the loss of initiative on unpublished ideas.

  5. The prevalence of ANA antibodies, anticentromere antibodies, and anti-cyclic citrullinated peptide antibodies in patients with primary Sjögren's syndrome compared to patients with dryness symptoms without primary Sjögren's syndrome confirmation.

    Science.gov (United States)

    Maślińska, Maria; Mańczak, Małgorzata; Wojciechowska, Bożena; Kwiatkowska, Brygida

    2017-01-01

    Our study analyses the prevalence of ANA, anti-SS-A, anti-SS-B, and ACA and ACPA antibodies in patients with pSS and with dryness symptoms without pSS confirmation, and the association of ACPA and ACA antibodies with specific clinical symptoms. 113 patients were divided into two groups: I - with diagnosed pSS ( N = 75); and II - with dryness without pSS evidence ( N = 38). Diagnostics: indirect immunofluorescence (IF; Hep-2 cell line) of antinuclear antibodies (ANA), anti-SS-A anti-SS-B antibodies determined with semi-quantitative method, autoantibody profile (14 antigens, ANA Profil 3 EUROLINE); basic laboratory, ophthalmic examination tests, minor salivary gland biopsy with focus score (FS), joint and lung evaluation, and ESSDAI questionnaire (pSS activity). 88% of group I had ANA antibodies (1 : 320 titre), 5.3% at 1 : 160. Anti-SS-A antibodies were present in 88% of group I, including all ANA 1 : 160. Anti-SS-A antibodies positively correlated with greater and moderate activity of ESSDAI 5 ( p = 0.046) and FS. The presence of SS-B antibodies significantly affected disease activity. ACPA present: group I - 13% (associated with higher arthritis incidence; p = 0.003); group II - 8%. ACA antibodies present in 4% of group I, but not in group II. No ACA association with interstitial lung changes (small ACA + group excludes full conclusions). ANA antibodies should also be considered in a titre of less than 1 : 320, but the presence of anti-SS-A antibodies is still the most important immunological marker for pSS. Anti-SS-A antibodies correlate with higher disease activity (ESSDAI ≥ 5) and higher FS. The presence of the anti-SS-B antibody was significantly affected by higher activity of the disease. The incidence of arthritis was higher in patients with ACPA+ pSS compared to ACPA- ( p = 0.003). There was no relationship between ACPA and arthritis in patients with dry-type syndrome without diagnosis of pSS.

  6. Secondary Sjögren's syndrome and disease activity of rheumatoid arthritis Síndrome de Sjögren secundária e atividade da artrite reumatoide

    Directory of Open Access Journals (Sweden)

    Daniel C. Antero

    2011-06-01

    Full Text Available OBJECTIVE: To study the relationship of the presence of secondary SS with disease activity, duration in RA. METHODS: Eighty two patients with RA were submitted to Schirmer test, minor salivary gland biopsy, questionnaire on sicca symptoms, DAS-28 4v determination. RESULTS: In this population, 20 (24.3% patients fulfilled the American-European classification criteria for secondary SS. No relation could be found between the presence of secondary SS and disease activity (p = 0.31 and RA duration (p = 0.95. CONCLUSION: Appearance of Secondary SS in RA patients is independent of RA duration or activity.OBJETIVO: Estudar a associação entre presença de SS secundária e atividade e duração da artrite reumatoide. MÉTODOS: Oitenta e dois pacientes com artrite reumatoide foram submetidos ao teste de Schirmer, biópsia de glândula salivar menor, questionários acerca de sintomas de secura e determinação do DAS28 4v. RESULTADOS: Nesta população, 20 (24,3% dos pacientes preenchiam os Critérios Americanos Europeus para classificação de SS Secundário. Nenhuma associação foi encontrada entre presençade SS secundário e atividade da doença (p = 0.31 e duração da doença (p = 0.95. CONCLUSÃO: O aparecimento de SS secundário em AR é independente da duração e atividade da AR.

  7. Psychological profiles in patients with Sjögren’s syndrome related to fatigue : a cluster analysis

    NARCIS (Netherlands)

    van Leeuwen, N.; Bossema, E.R.; Knoop, H.; Kruize, A.A.; Bootsma, H.; Bijlsma, J.W.J.; Geenen, R.

    OBJECTIVE: Fatigue is a highly prevalent and debilitating symptom in the autoimmune disease SS. Although the disease process plays a role in fatigue, psychological factors may influence fatigue and the ability to deal with its consequences. Profiles of co-occurring psychological factors may suggest

  8. Reversed halo sign as initial manifestation of interstitial pneumonitis associated with Sjögren’s syndrome

    Directory of Open Access Journals (Sweden)

    M.A. Duarte-Millán

    2017-07-01

    Conclusion: To the best of our knowledge this is the second reported OP case associated with SS manifesting as RHS. RSH as an expression of IP related to SS is exceptional, either in the pattern of OP or non-specific IP.

  9. Salmonella er en sjælden årsag til meningitis hos nyfødte

    DEFF Research Database (Denmark)

    Jensen, Lotte; Hoffmann, Thomas; Knudsen, Inge Jenny Dahl

    2013-01-01

    In developed countries, invasive disease caused by non typhoidal Salmonella spp. is rare. Here we present a Danish case of Salmonella enterica serovar Enteritidis (S. Enteritidis) meningitis in an infant who had no underlying diseases. The child had had no known expositions, and the source...... of the infection was never identified. The chance of finding uncommon microorganisms as cause of invasive infections such as meningitis and the choice of initial empiric antimicrobial treatments is discussed....

  10. Præpylorisk antral web--en sjælden årsag til pylorusobstruktion

    DEFF Research Database (Denmark)

    Liu, Xiuping; Fonnest, Gert

    2011-01-01

    A case of gastric outlet obstruction secondary to prepyloric antral web in a four-year-old boy with cerebral pareses is reported. Routine roentgenographic examination was initially misinterpreted as duodenal obstruction. Prepyloric antral web was suspected by subsequent endoscopy and was confirmed...

  11. Den samhälleliga självreflexionens möjligheter : Big data på 1980-talet

    OpenAIRE

    Gullberg, Anders

    1991-01-01

    Möjligheterna att nå kunskap om dagens samhälle synes, särskilt om blott ”data-frågan” beaktas, mycket goda. Detta sken är dock på många sätt bedrägligt. Genom att kontrastera vårt i högsta grad levande och dessutom tecken- och textöversväm-mande samhälle mot möjligheterna att nå kunskap om utdöda och/eller illitterata kulturer belyses vad som kan vetas om vår egen värld. Qc 20140825

  12. "Godis för kropp och själ" : Välbefinnande och vardagsandlighet i tre svenska kvinnotidningar

    OpenAIRE

    Winell, Anneli

    2016-01-01

    This thesis analyses discourses on health and wellbeing in three Swedish lifestyle magazines for women, Amelia, Tara and M-magasin, and how readers of these magazines reflect on and negotiate the values and identities presented in them. The aim of the thesis is to contribute to increased knowledge about mediatized religion, directed to women by commercial women's magazines on a secular market, and how this religion is presented, perceived and used as a resource for women's wellbeing, lifestyl...

  13. Cursus Conimbricensis – Sebastian Couto S.J. Nature considered in itself in the context of the problem of universals

    Directory of Open Access Journals (Sweden)

    David Svoboda

    2014-04-01

    Full Text Available Přirozenost o sobě v kontextu problematiky univerzálií Článek pojednává o pojmu přirozenosti o sobě v díle významného portugalského filosofa Sebastiana Couto (1567–1639, autora jedné části slavného díla „Cursus Conimbricenses“. Článek je rozdělen do čtyř základních částí. Nejprve je v historických souvislostech vyložen význam uvedeného díla. Dále je z historicko-sytematického hlediska vysvětlena problematika přirozenosti o sobě a do ní je následně zasazeno Coutovo řešení. Na závěr je Coutovo pojetí porovnáno s jinými důležitými scholastickými koncepcemi přirozenosti o sobě a je zhodnocen jeho přínos.

  14. Cursus Conimbricensis - Sebastian Couto S.J. Nature Considered in itself in the Context of the Problem of Universals

    Czech Academy of Sciences Publication Activity Database

    Svoboda, David

    2013-01-01

    Roč. 3, č. 2 (2013), s. 135-150 ISSN 1804-5588 Institutional support: RVO:67985955 Keywords : Cursus Conimbricensis * Sebastian Couto * universals * common nature Subject RIV: AA - Philosophy ; Religion

  15. Diagnostik og behandling af sjældne årsager til iskæmisk apopleksi

    DEFF Research Database (Denmark)

    Kondziella, Daniel

    2016-01-01

    require sophisticated diagnostics (e.g. fluorescence angiography in Susac's syndrome) and they are increasingly treatable (e.g. enzyme replacement for Fabry's disease). This review discusses practical considerations and recent diagnostic and therapeutic advances in uncommon causes of ischaemic stroke.......Rare causes of ischaemic stroke comprise a plethora of diagnoses of cardioembolic, inflammatory and genetic origin. The differential diagnosis is challenging but important because these disorders (e.g. monogenetic disorders such as COL4A1 mutations) often affect young individuals, they typically...

  16. Immunglobulin G4-relateret sygdom er en sjælden differentialdiagnose til maligne og autoimmune sygdomme

    DEFF Research Database (Denmark)

    Storgaard, Anders; Detlefsen, Sönke

    2015-01-01

    Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory and fibrotic disease with the potential to produce diffuse enlargement, massforming lesions or stenoses in a wide range of organs. Elevation of serum IgG4 concentration and high levels of IgG4-positive cells in the inflamed tissue...

  17. B-cell depletion and primary Sjögren’s syndrome: is there a role for rituximab?

    Directory of Open Access Journals (Sweden)

    P. Sarzi-Puttini

    2011-06-01

    Full Text Available L’idea che la deplezione dei B linfociti potesse essere utilizzata per il trattamento delle malattie reumatiche sistemiche deriva dall’ipotesi che, eliminando le cellule alterate dal circolo, si possa curare la malattia. In realtà, nelle malattie reumatiche sistemiche, contrariamente a quanto accade nei linfomi, la deplezione dei B linfociti persiste per alcuni mesi e successivamente si assiste alla riacutizzazione della malattia. Molteplici sono le ipotesi che possono spiegare tale evento: a insufficiente deplezione dei cloni patogeni; b persistenza di anticorpi patogeni; c alterazione primaria o secondaria della tolleranza dei B linfociti; d ricomparsa delle B cellule; e reminiscenza della malattie da parte di cellule diverse dai B linfociti, probabilmente T linfociti (1, 2...

  18. Pontocerebellar hypoplasi er en sjælden årsag til slapt spædbarn

    DEFF Research Database (Denmark)

    Christiansen, Susanne; Roos, Laura Kirstine Sønderberg; Miranda, Maria J

    2015-01-01

    The hypotone neonate, floppy infant, often proves to be a diagnostic challenge, as the causes of floppy infant syndrome are many and often rare. In this case story a floppy girl was diagnosed with the rare, autosomal recessive disease pontocerebellar hypoplasia type I. The tests for the most common...... causes of floppy infant syndrome showed nothing abnormal, but an array comparative genomic hybridization test gave information of loss of heterozygosity. This helped to narrow the list of plausible diagnoses and eventually led to the diagnosis of pontocerebellar hypoplasia type I....

  19. Benign galdeblærepolyp er en sjælden årsag til hæmobilli

    DEFF Research Database (Denmark)

    Juul Nielsen, Liv Bjerre; Schultz, Nicolai Aagaard; Hasselby, Jane Preuss

    2013-01-01

    Haemobilia can present with gastrointestinal bleeding, biliary colic and jaundice. Causes include trauma, iatrogenic causes, calculi, inflammation, vascular malformations and neoplasms. Benign gallbladder polyp is a very rare cause. A 63-year-old male with suspected gallbladder cancer due...

  20. Biliær papillomatose er en sjælden årsag til intermitterende obstruktiv ikterus

    DEFF Research Database (Denmark)

    Veedfald, Simon; Vainer, Ben; Wettergren, André

    2011-01-01

    Over a five-year period a 67 year-old male had been experiencing recurring bouts of biliary obstruction with occasional superimposed cholangitis. Renewed endoscopic retrograde cholangiopancreatography revealed amorphous filling defects and excessive mucinous discharge from the papilla of Vater. S...