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Sample records for connolly sj ezekowitz

  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. 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...

  8. 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.

  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....

  10. Mastocytosis and Sjögren's syndrome.

    OpenAIRE

    Bac, D J; van Marwijk Kooy, M

    1992-01-01

    A 61 year old man with dryness of the eyes and mouth, constitutional and musculoskeletal symptoms is described. A diagnosis of fibromyalgia with Sjögren's syndrome was made elsewhere. Further examination, however, disclosed diffuse mast cell infiltration in several organs, including the labial accessory salivary glands.

  11. 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

  12. 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....

  13. Quantitative assessment of clinical disease status in primary Sjögren's syndrome

    DEFF Research Database (Denmark)

    Asmussen, K.; Andersen, V.; Bendixen, Gunnar

    1999-01-01

    ophthalmology, Sjögren's syndrome, terminology, classification criteria, status indices, immunoinflammation, cytokines......ophthalmology, Sjögren's syndrome, terminology, classification criteria, status indices, immunoinflammation, cytokines...

  14. Genetics Home Reference: Sjögren-Larsson syndrome

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Sjögren-Larsson syndrome Sjögren-Larsson syndrome Printable PDF Open All Close All ... FALDH deficiency fatty aldehyde dehydrogenase deficiency ichthyosis oligophrenia syndrome Sjogren-Larsson syndrome SLS Related Information How are genetic ...

  15. 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 November, 2009. Myostatin is a negative regulator of animal skeletal muscle development and SJ01 is a microsatellite locus flanking porcine myostatin gene. In the present study, the polymorphism of microsatellite SJ01 in.

  16. Neurological Disorders in Primary Sjögren's Syndrome

    Directory of Open Access Journals (Sweden)

    Gabriel J. Tobón

    2012-01-01

    Full Text Available Sjögren's syndrome is an autoimmune disease characterized by an autoimmune exocrinopathy involving mainly salivary and lacrimal glands. The histopathological hallmark is periductal lymphocytic infiltration of the exocrine glands, resulting in loss of their secretory function. Several systemic manifestations may be found in patients with Sjögren's syndrome including neurological disorders. Neurological involvement ranges from 0 to 70% among various series and may present with central nervous system and/or peripheral nervous system involvement. This paper endeavors to review the main clinical neurological manifestations in Sjögren syndrome, the physiopathology, and their therapeutic response.

  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. 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....

  19. 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....

  20. 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.

  1. 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

  2. Tear fluid analysis in patients with primary Sjögren's syndrome using lectin probes

    DEFF Research Database (Denmark)

    Bjerrum, Kirsten Birgitte

    1999-01-01

    Ophthalmology, Sjögren's syndrome, dry eye, keratoconjunctivitis sicca, glycoprotein, mucus, lectins, Coomassie, electrophoresis, SDS-PAGE-blotting......Ophthalmology, Sjögren's syndrome, dry eye, keratoconjunctivitis sicca, glycoprotein, mucus, lectins, Coomassie, electrophoresis, SDS-PAGE-blotting...

  3. 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?

  4. 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...

  5. 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

  6. 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.

  7. 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.

  8. Prevalence and intraoral distribution of Candida albicans in Sjögren's syndrome.

    Science.gov (United States)

    Tapper-Jones, L; Aldred, M; Walker, D M

    1980-03-01

    An imprint culture technique has been employed to study the prevalence and intraoral distribution of Candida albicans in 16 patients with Sjögren's syndrome and in 16 healthy controls matched for age, sex, and dental status. The prevalence and intraoral density of C. albicans was found to be significantly higher at almost all sites in the Sjögren's patients than in the controls. The distribution of candida was also altered, being significantly higher in the floor of the mouth and anterior labial sulcus in the Sjögren's group. There was an approximate inverse relationship between candida populations and rate of salivary flow. Mean candida densities were found to be significantly higher in those Sjögren's patients with detectable serum rheumatoid factor in the serum. However, patients with primary Sjögren's syndrome had significantly higher mean candida densities compared with patients with secondary Sjögren's syndrome.

  9. Histopathological changes in exocrine glands of murine transplantation chimeras. II: Sjögren's syndrome-like exocrinopathy in mice without lupus nephritis. A model of primary Sjögren's syndrome

    DEFF Research Database (Denmark)

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

    1992-01-01

    Autoimmune disease, primary Sjögren's syndrome, transplantation chimeras, experimental model, exocrinopathy, inbred mouse strains......Autoimmune disease, primary Sjögren's syndrome, transplantation chimeras, experimental model, exocrinopathy, inbred mouse strains...

  10. 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...

  11. Osteomalacia as inaugural manifestation of Sjögren syndrome.

    Science.gov (United States)

    Cherif, Eya; Ben Hassine, Lamia; Kaoueche, Zouleikha; Khalfallah, Narjess

    2013-10-09

    Osteomalacia is a relatively common condition, which is frequently underdiagnosed due to lack of clinical suspicion and non-specific symptoms. Osteomalacia can complicate tubulo-interstital nephritis. However, it occurs exceptionally as the first manifestation of Sjögren syndrome with renal involvement. It is a consequence of chronic metabolic acidosis and is associated with distal renal tubular acidosis. We report a 31-year-old woman hospitalised for a 1 year history of muscle weakness and joint and chest wall pains. Skeletal imagery showed Looser's zones in the left femoral neck. Investigations concluded to the diagnosis of primary Sjogren's syndrome.

  12. Primary Sjögren's syndrome and keratoconjunctivitis sicca: Diagnostic methods, frequency and social disease aspects

    DEFF Research Database (Denmark)

    Bjerrum, Kirsten Birgitte

    ophthalmology, Sjögren's syndrome, keratoconjunctivitis sicca, conjunctiva, dry eye, Schirmer-1 test, Rose Bengal score, break-up time, tear film, Copenhagen criteria......ophthalmology, Sjögren's syndrome, keratoconjunctivitis sicca, conjunctiva, dry eye, Schirmer-1 test, Rose Bengal score, break-up time, tear film, Copenhagen criteria...

  13. Hydroxychloroquine treatment for primary Sjögren's syndrome: a two year double blind crossover trial

    NARCIS (Netherlands)

    Kruize, A. A.; Hené, R. J.; Kallenberg, C. G.; van Bijsterveld, O. P.; van der Heide, A.; Kater, L.; Bijlsma, J. W.

    1993-01-01

    In 1985 and 1988 a positive effect of treatment of primary Sjögren's syndrome with hydroxychloroquine was reported in two small open studies. To investigate further the clinical and laboratory effects of hydroxychloroquine in primary Sjögren's syndrome a two year study was performed. The design of

  14. Development of monoclonal antibodies against Sj29 and its possible application for schistosomiasis diagnosis

    Directory of Open Access Journals (Sweden)

    Cui-Ping Ren

    2017-08-01

    Conclusions: Sj29 double antibody sandwich ELISA was established. The specificity of this method for detecting healthy sera was 100%. Meanwhile, Sj29 sandwich ELISA may have a potential diagnostic capability to distinguish current from past infections and assess drug treatment responses.

  15. 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.

  16. 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.

  17. Managing Sjögren's Syndrome and non-Sjögren Syndrome dry eye with anti-inflammatory therapy.

    Science.gov (United States)

    Coursey, Terry G; de Paiva, Cintia S

    2014-01-01

    Dry eye from Sjögren's syndrome is a multifactorial disease that results in dysfunction of the lacrimal functional unit. Studies have shown changes in tear composition, including inflammatory cytokines, chemokines, and metalloproteinase. T-lymphocytes have been shown to increase in the conjunctiva and lacrimal glands in patient and animal models. This inflammation is in part responsible for the pathogenesis of the disease, which results in symptoms of eye irritation, ocular surface epithelial disease, and loss of corneal barrier function. There are a number of anti-inflammatory approaches for treating this disease. The current study reviews details of immune response and anti-inflammatory therapies used to control this disease.

  18. A chimeric human-mouse model of Sjögren's syndrome.

    Science.gov (United States)

    Young, Nicholas A; Wu, Lai-Chu; Bruss, Michael; Kaffenberger, Benjamin H; Hampton, Jeffrey; Bolon, Brad; Jarjour, Wael N

    2015-01-01

    Despite recent advances in the understanding of Sjögren's Syndrome (SjS), the pathogenic mechanisms remain elusive and an ideal model for early drug discovery is not yet available. To establish a humanized mouse model of SjS, peripheral blood mononuclear cells (PBMCs) from healthy volunteers or patients with SjS were transferred into immunodeficient NOD-scid IL-2rγ(null) mouse recipients to produce chimeric mice. While no difference was observed in the distribution of cells, chimeric mice transferred with PBMCs from SjS patients produced enhanced cytokine levels, most significantly IFN-γ and IL-10. Histological examination revealed enhanced inflammatory responses in the lacrimal and salivary glands of SjS chimeras, as measured by digital image analysis and blinded histopathological scoring. Infiltrates were primarily CD4+, with minimal detection of CD8+ T-cells and B-cells. These results demonstrate a novel chimeric mouse model of human SjS that provides a unique in vivo environment to test experimental therapeutics and investigate T-cell disease pathology. Copyright © 2014. Published by Elsevier Inc.

  19. 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.

  20. Higher frequency of brain abnormalities in neuromyelitis optica spectrum disorder patients without primary Sjögren's syndrome.

    Science.gov (United States)

    Gu, Li-Na; Zhang, Min; Zhu, Hui; Liu, Jing-Yao

    2016-10-01

    Neuromyelitis optica spectrum disorder often co-exists with primary Sjögren's syndrome. We compared the clinical features of 16 neuromyelitis optica spectrum disorder patients with ( n = 6) or without primary Sjögren's syndrome ( n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjögren's syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinuclear autoantibody, anti-Sjögren's-syndrome-related antigen A antibodies, anti-Sjögren's-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjögren's syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sjögren's syndrome and in 60% (6/10) of patients without primary Sjögren's syndrome. More brain abnormalities were observed in patients without primary Sjögren's syndrome than in those with primary Sjögren's syndrome. Segments lesions (> 3 centrum) were noted in 50% (5/10) of patients without primary Sjögren's syndrome and in 67% (4/6) of patients with primary Sjögren's syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjögren's syndrome are similar. However, neuromyelitis optica spectrum disorder patients without primary Sjögren's syndrome have a high frequency of brain abnormalities.

  1. 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.

  2. New Genetic Susceptibility Factors for Sjögren's Syndrome Revealed

    Science.gov (United States)

    ... Spotlight on Research Spotlight on Research New Genetic Susceptibility Factors for Sjögren’s Syndrome Revealed By Kirstie Saltsman, ... swallowing and speaking. “The identification of these genetic susceptibility factors opens up new avenues for understanding how ...

  3. 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.

  4. Autoantibodies against Muscarinic Type 3 Receptor in Sjögren's Syndrome Inhibit Aquaporin 5 Trafficking

    Science.gov (United States)

    Lee, Byung Ha; Gauna, Adrienne E.; Perez, Geidys; Park, Yun-jong; Pauley, Kaleb M.; Kawai, Toshihisa; Cha, Seunghee

    2013-01-01

    Sjögren's syndrome (SjS) is a chronic autoimmune disease that mainly targets the salivary and lacrimal glands. It has been controversial whether anti-muscarinic type 3 receptor (α-M3R) autoantibodies in patients with SjS inhibit intracellular trafficking of aquaporin-5 (AQP5), water transport protein, leading to secretory dysfunction. To address this issue, GFP-tagged human AQP5 was overexpressed in human salivary gland cells (HSG-hAQP5) and monitored AQP5 trafficking to the plasma membrane following carbachol (CCh, M3R agonist) stimulation. AQP5 trafficking was indeed mediated by M3R stimulation, shown in partial blockage of trafficking by M3R-antagonist 4-DAMP. HSG-hAQP5 pre-incubated with SjS plasma for 24 hours significantly reduced AQP5 trafficking with CCh, compared with HSG-hAQP5 pre-incubated with healthy control (HC) plasma. This inhibition was confirmed by monoclonal α-M3R antibody and pre-absorbed plasma. Interestingly, HSG-hAQP5 pre-incubated with SjS plasma showed no change in cell volume, compared to the cells incubated with HC plasma showing shrinkage by twenty percent after CCh-stimulation. Our findings clearly indicate that binding of anti-M3R autoantibodies to the receptor, which was verified by immunoprecipitation, suppresses AQP5 trafficking to the membrane and contribute to impaired fluid secretion in SjS. Our current study urges further investigations of clinical associations between SjS symptoms, such as degree of secretory dysfunction, cognitive impairment, and/or bladder irritation, and different profiles (titers, isotypes, and/or specificity) of anti-M3R autoantibodies in individuals with SjS. PMID:23382834

  5. American College of Rheumatology classification criteria for Sjögren's syndrome

    DEFF Research Database (Denmark)

    Shiboski, S C; Shiboski, C H; Criswell, L A

    2012-01-01

    We propose new classification criteria for Sjögren's syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS.......We propose new classification criteria for Sjögren's syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS....

  6. 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

  7. 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.

  8. Sjögren syndrome presenting with hypopotassemic periodic paralysis due to renal tubular acidosis

    Science.gov (United States)

    Ataoglu, Esra Hayriye; Demir, Betul; Tuna, Mazhar; Çavus, Bilger; Cetin, Faik; Temiz, Levent Umit; Ozturk, Savas; Yenigun, Mustafa

    2012-01-01

    Summary Background: Sjögren syndrome (SS) is an autoimmune-lymphoproliferative disorder characterized by mononuclear cell infiltration of exocrine glands. Clinically, Sjögren syndrome (SS) has a wide spectrum, varying from autoimmune exocrinopathy to systemic involvement. There have been few cases reporting that primary SS developed with distal renal tubular acidosis clinically. Case Report: Here, we present a case with primary Sjögren syndrome accompanied by hypopotassemic paralysis due to renal tubular acidosis. Severe hypopotassemia, hyperchloremic metabolic acidosis, alkaline urine and disorder in urinary acidification test were observed in the biochemical examination of the 16-year-old female patient, who had applied to our clinic for extreme loss of muscle force. After the examinations it was determined that the patient had developed Type 1 RTA (distal RTA) due to primary Sjögren syndrome. Potassium and alkaline replacement was made and an immediate total recovery was achieved. Conclusions: Hypopotassemic paralysis due to primary Sjögren syndrome is a rare but severe disorder that could lead to death if not detected early and cured appropriately. Thus, effective treatment should be immediately initiated in cases where severe hypopotassemia is accompanied by metabolic acidosis, and the cases should also be examined for extraglandular involvement of SS. PMID:23569525

  9. 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.

  10. Beyond the dryness of Sjögren’s syndrome : renal complications and bone metabolism : Meer dan droogte bij het syndroom van Sjögren : niercomplicaties en botmetabolisme

    NARCIS (Netherlands)

    T. Both (Tim)

    2017-01-01

    markdownabstractIn this thesis, we addressed three clinical important issues: 1) the prevalence and consequences of distal renal tubular acidosis in Primary Sjögren’s syndrome patients; 2) the effect of Primary Sjögren’s syndrome on bone metabolism; 3) the effect of hydroxychloroquine on

  11. 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.

  12. 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.

  13. Sjögren Syndrome Which Simulates Relapsing Remitting Multiple Sclerosis Clinical Features: Case Report

    Directory of Open Access Journals (Sweden)

    Haluk Gümüş

    2013-12-01

    Full Text Available Sjögren syndrome (SS is a chronic, inflammatory, autoimmune disease. It emerges as a dry mouth and eyes (sicca symptoms because, it fundamentally affects exocrine glands, frequently, salivary gland and lachrymal gland. Neurological involvement in Sjögren syndrome is observed in the approximately 20-25% of cases. 87% of the neurological involvements are peripheral nervous system involvement and around 13% of the neurological involvements are central nervous system involvement. Cerebral involvement represents heterogeneous features in terms of both localization (focal or diffuse and progress of the statement (acute, progressive or reversible. Affected central nervous system can show clinical and radiological signs similar to Multiple sclerosis (MS. In this paper, the case, which has a complaint of difficulty in walking and instability and MS like lesions in brain magnetic resonance imaging (MRI and is diagnosed as Sjögren syndrome by further research, is discussed

  14. 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.

  15. 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...

  16. 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

  17. Optic Neuropathy Associated with Primary Sjögren's Syndrome: A Case Series.

    Science.gov (United States)

    Bak, Eunoo; Yang, Hee Kyung; Hwang, Jeong-Min

    2017-04-01

    To determine the diverse clinical features of optic neuropathy associated with primary Sjögren's syndrome in Korean patients. Five women with acute and/or chronic optic neuropathy who were diagnosed as primary Sjögren's syndrome were retrospectively evaluated. Primary Sjögren's syndrome was diagnosed by signs and symptoms of keratoconjunctivitis sicca, positive serum anti-Ro/SSA and/or anti-La/SSB antibodies, and/or minor salivary gland biopsy. All patients underwent a complete ophthalmologic examination. Among the five patients diagnosed as optic neuropathy related to primary Sjögren's syndrome, four patients had bilateral optic neuropathy and one patient was unilateral. The clinical course was chronic in three patients and one of them showed acute exacerbation and was finally diagnosed with neuromyelitis optica spectrum disorder. The other two patients presented as acute optic neuritis and one was diagnosed with neuromyelitis optica spectrum disorder. Sicca symptoms were present in four patients, but only two patients reported these symptoms before the onset of optic neuropathy. Patients showed minimal response to systemic corticosteroids or steroid dependence, requiring plasmapheresis in the acute phase and immunosuppressive agents for maintenance therapy. Optic neuropathy associated with primary Sjögren's syndrome may show variable clinical courses, including acute optic neuritis, insidious progression of chronic optic atrophy, or in the context of neuromyelitis optica spectrum disorders. Optic neuropathy may be the initial manifestation of primary Sjögren's syndrome without apparent sicca symptoms, which makes the diagnosis often difficult. The presence of specific antibodies including anti-Ro/SSA, anti-La/SSB, and anti-aquaporin-4 antibodies are supportive for the diagnosis and treatment in atypical cases of optic neuropathy.

  18. Tocilizumab for refractory organising pneumonia associated with Sjögren's disease.

    Science.gov (United States)

    Justet, Aurelien; Ottaviani, Sebastien; Dieudé, Philippe; Taillé, Camille

    2015-05-14

    Lung involvement in primary Sjögren syndrome occurs in approximately 10-20% of patients. Tocilizumab, an anti-interleukin-6 receptor antibody, has demonstrated efficacy and safety in small series of systemic sclerosis, and systemic lupus erythematosus, but its effect on interstitial lung manifestations of connective tissue diseases is not well known. We report the use of tocilizumab in a refractory organising pneumonia associated with Sjögren's disease. Our observation suggests that tocilizumab could be an alternative therapeutic in refractory organising pneumonia. 2015 BMJ Publishing Group Ltd.

  19. 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.

  20. 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

  1. Prevalence and intraoral distribution of Candida albicans in Sjögren's syndrome.

    OpenAIRE

    Tapper-Jones, L; Aldred, M; Walker, D M

    1980-01-01

    An imprint culture technique has been employed to study the prevalence and intraoral distribution of Candida albicans in 16 patients with Sjögren's syndrome and in 16 healthy controls matched for age, sex, and dental status. The prevalence and intraoral density of C. albicans was found to be significantly higher at almost all sites in the Sjögren's patients than in the controls. The distribution of candida was also altered, being significantly higher in the floor of the mouth and anterior lab...

  2. 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.

  3. Manifestation of meibomian gland dysfunction in patients with Sjögren's syndrome, non-Sjögren's dry eye, and non-dry eye controls.

    Science.gov (United States)

    Kang, Yeon Soo; Lee, Hyo Seok; Li, Ying; Choi, Won; Yoon, Kyung Chul

    2018-06-01

    To evaluate the manifestation of meibomian gland dysfunction in patients with Sjögren's syndrome (SS), non-Sjögren's syndrome dry eye (non-SS) patients, and non-dry eye controls. We recruited 31 participants with SS dry eye, 30 participants with non-SS dry eye, and 35 healthy controls without dry eye symptoms. Noninvasive tear breakup time (NITBUT) and meibomian gland dropout score (meiboscore) were measured using the Oculus Keratograph 5 M. Meibomian gland expressibility and secretion quality were evaluated via slit lamp biomicroscopy. The correlation between measurements was analyzed. NITBUT was lower, and the meiboscore, meibomian gland expressibility, and secretion quality scores were significantly higher in the SS and non-SS groups than in the control group (p dry eye controls. SS patients had more severe meibomian gland dysfunction with poorer mean meiboscore and meibomian gland expressibility than non-SS patients.

  4. Managing Sjögren’s Syndrome and non-Sjögren Syndrome dry eye with anti-inflammatory therapy

    Directory of Open Access Journals (Sweden)

    Coursey TG

    2014-08-01

    Full Text Available Terry G Coursey, Cintia S de PaivaCullen Eye Institute, Baylor College of Medicine, Houston, TX, USAAbstract: Dry eye from Sjögren’s syndrome is a multifactorial disease that results in dysfunction of the lacrimal functional unit. Studies have shown changes in tear composition, including inflammatory cytokines, chemokines, and metalloproteinase. T-lymphocytes have been shown to increase in the conjunctiva and lacrimal glands in patient and animal models. This inflammation is in part responsible for the pathogenesis of the disease, which results in symptoms of eye irritation, ocular surface epithelial disease, and loss of corneal barrier function. There are a number of anti-inflammatory approaches for treating this disease. The current study reviews details of immune response and anti–inflammatory therapies used to control this disease.Keywords: keratoconjunctivitis sicca, SS, cyclosporin A, steroids, dry eye, Sjögren’s Syndrome

  5. 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...

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

    Science.gov (United States)

    ... Special Issues Subscribe March 2012 Print this issue Dry Eyes and Mouth? You May Have Sjögren’s Syndrome Send us your comments If your eyes and mouth feel as dry as a desert, there are many possible causes, ...

  7. Long-term followup of patients with Sjögren's syndrome

    NARCIS (Netherlands)

    Kruize, A. A.; Hené, R. J.; van der Heide, A.; Bodeutsch, C.; de Wilde, P. C.; van Bijsterveld, O. P.; de Jong, J.; Feltkamp, T. E.; Kater, L.; Bijlsma, J. W.

    1996-01-01

    To assess long-term outcome in patients with isolated keratoconjunctivitis sicca (KCS), primary Sjögren's syndrome (SS), and secondary SS. In 112 patients referred because of dry eyes, an ophthalmologic diagnosis of KCS was made based on results of the Schirmer I test, the tear fluid lysozyme

  8. 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%

  9. Unusual presentation Of Sjögren-associated neuropathy with plasma cell-rich infiltrate.

    Science.gov (United States)

    Naddaf, Elie; Berini, Sarah E; B Dyck, P James; Laughlin, Ruple S

    2017-04-01

    Sjögren syndrome is thought to be a lymphocyte-driven process. Peripheral nervous system involvement occurs in about 20%-25% of patients. A sensory-predominant, large-fiber peripheral neuropathy is most common, and it is usually associated with a subacute to chronic presentation. We report a rare case of an acute Sjögren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain-Barré syndrome. The patient presented with sensory ataxia preceded by fever and polyarthralgia. She gave a history of years of dry eyes and dry mouth. She had a positive Shirmer test, abnormal salivary gland scan, and positive SS-A and SS-B antibodies. A sural nerve biopsy showed an unusual, dense, non-IgG4, polyclonal, plasma-cell perivascular infiltrate. The patient responded to treatment with weekly pulse intravenous methylprednisolone. Sjögren syndrome can present with acute-onset, sensory predominant peripheral neuropathy. The role of plasma cells in Sjögren syndrome is unexplored and deserves further study. Muscle Nerve 55: 605-608, 2017. © 2016 Wiley Periodicals, Inc.

  10. NOD mouse model for Sjögren's syndrome: lack of longitudinal stability

    NARCIS (Netherlands)

    Lodde, B. M.; Mineshiba, F.; Kok, M. R.; Wang, J.; Zheng, C.; Schmidt, M.; Cotrim, A. P.; Kriete, M.; Tak, P. P.; Baum, B. J.

    2006-01-01

    OBJECTIVES: The non-obese diabetic (NOD) mouse is not only a widely used model for diabetes mellitus type I, but also for the chronic autoimmune disease Sjögren's syndrome (SS), mainly affecting salivary and lacrimal glands. We studied the efficacy of local recombinant serotype 2 adeno-associated

  11. 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......, osteogenesis imperfecta og hereditær rakitis...

  12. 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

  13. Biological therapy in Sjögren’s syndrome : outcomes and evaluation

    NARCIS (Netherlands)

    Meiners, Petra

    2014-01-01

    Het syndroom van Sjögren is een chronische ziekte waarbij klieren ontstoken raken, vooral de speeksel- en traanklieren, met een droge mond en droge ogen als gevolg. Daarnaast kunnen de ontstekingen zich ook uiten in andere organen zoals de longen, nieren of bloedvaten. Bijna alle patiënten zijn

  14. 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

  15. EULAR Sjögren's syndrome disease activity index (ESSDAI) : a user guide

    NARCIS (Netherlands)

    Seror, Raphaèle; Bowman, Simon J; Brito-Zeron, Pilar; Theander, Elke; Bootsma, Hendrika; Tzioufas, Athanasios; Gottenberg, Jacques-Eric; Ramos-Casals, Manel; Dörner, Thomas; Ravaud, Philippe; Vitali, Claudio; Mariette, Xavier; Asmussen, Karsten; Jacobsen, Soren; Bartoloni, Elena; Gerli, Roberto; Bijlsma, Johannes Wj; Kruize, Aike A; Bombardieri, Stefano; Bookman, Arthur; Kallenberg, Cees; Meiners, Petra; Brun, Johan G; Jonsson, Roland; Caporali, Roberto; Carsons, Steven; De Vita, Salvatore; Del Papa, Nicoletta; Devauchelle, Valerie; Saraux, Alain; Fauchais, Anne-Laure; Sibilia, Jean; Hachulla, Eric; Illei, Gabor; Isenberg, David; Jones, Adrian; Manoussakis, Menelaos; Mandl, Thomas; Jacobsson, Lennart; Demoulins, Frederic; Montecucco, Carlomaurizio; Ng, Wan-Fai; Nishiyama, Sumusu; Omdal, Roald; Parke, Ann; Praprotnik, Sonja; Tomsic, Matjia; Price, Elizabeth; Scofield, Hal; L Sivils, Kathy; Smolen, Josef; Laqué, Roser Solans; Steinfeld, Serge; Sutcliffe, Nurhan; Sumida, Takayuki; Valesini, Guido; Valim, Valeria; Vivino, Frederick B; Vollenweider, Cristina

    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 in

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

    DEFF Research Database (Denmark)

    Andersson, Åsa Inga Maria

    2009-01-01

    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. 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. Sjögren's Syndrome: Oral Manifestations and Treatment, a Dental Perspective.

    Science.gov (United States)

    Cartee, Deborah L; Maker, Shannon; Dalonges, Debra; Manski, Marion C

    2015-12-01

    Sjögren's syndrome is a systemic autoimmune disease affecting approximately 3 million Americans, primarily perimenopausal women. The syndrome is characterized by dysfunction and destruction of exocrine glands leading to oral and ocular manifestations, xerostomia and keratitis sicca. Sjögren's syndrome commonly remains either undiagnosed or is diagnosed years after the onset of symptoms. Diagnosis is based on the concurrent presence of various signs and symptoms of the disease as established by 6 diagnostic standards set by the American European Consensus Group standards: oral symptoms, ocular symptoms, evidence of oral signs, evidence of ocular dryness, evidence of salivary gland involvement with positive Anti-Ro/La autoantibodies and a positive gland biopsy. Currently no definitive test or cure exists; treatment is predominately palliative and supportive. With an aging population and heavier reliance on medications and treatments which cause xerostomia, oral health professionals are likely to encounter a higher incidence of xerostomia and Sjögren's syndrome more than ever before. The dental professional must recognize the signs and symptoms of xerostomia, include Sjögren's syndrome in their differential diagnosis, and communicate those findings and concerns to other health care providers, including the primary care physician, rheumatologist and ophthalmologist for evaluation in a timely fashion. This article discusses the dental professional's role in formulating a preventive oral health plan: meticulous oral hygiene instructions, dietary counseling, a complement of chemotherapeutic agents and more frequent recall care to avoid oral complications and improve quality of life. Dental hygienists can help patients understand the wide range of products available to substitute or stimulate salivary flow, prevent or remineralize early carious lesions and relieve candidal and bacterial infections. Ultimately this collaboration of care by the dental and medical

  20. Comparison of telomere length and association with progenitor cell markers in lacrimal gland between Sjögren syndrome and non-Sjögren syndrome dry eye patients

    Science.gov (United States)

    Kawashima, Motoko; Maida, Yoshiko; Kamoi, Mizuka; Ogawa, Yoko; Shimmura, Shigeto; Masutomi, Kenkichi; Tsubota, Kazuo

    2011-01-01

    Purpose Indicators of aging such as disruption of telomeric function due to shortening may be more frequent in dysfunctional lacrimal gland. The aims of this study were to 1) determine the viability of quantitative fluorescence in situ hybridization of telomeres (telo-FISH) for the assessment of telomere length in lacrimal gland in Sjögren and non- Sjögren syndrome patients; and 2) investigate the relationship between progenitor cell markers and telomere length in both groups. Methods Quantitative fluorescence in situ hybridization with a peptide nucleic acid probe complementary to the telomere repeat sequence was performed on frozen sections from human lacrimal gland tissues. The mean fluorescence intensity of telomere spots was automatically quantified by image analysis as relative telomere length in lacrimal gland epithelial cells. Immunostaining for p63, nucleostemin, ATP-binding cassette, sub-family G, member 2 (ABCG2), and nestin was also performed. Results Telomere intensity in the Sjögren syndrome group (6,785.0±455) was significantly lower than that in the non-Sjögren syndrome group (7,494.7±477; p=0.02). Among the samples from the non-Sjögren syndrome group, immunostaining revealed that p63 was expressed in 1–3 acinar cells in each acinar unit and continuously in the basal layer of duct cells. In contrast, in the Sjögren syndrome group, p63 and nucleostemin showed a lower level of expression. ABCG2 was expressed in acinar cells in both sjogren and non-Sjogren syndrome. Conclusions The results of this study indicate that 1) telo-FISH is a viable method of assessing telomere length in lacrimal gland, and 2) telomere length in Sjögren syndrome is shorter and associated with lower levels of expression of p63 and nucleostemin than in non-Sjögren syndrome. PMID:21655359

  1. Comparison of telomere length and association with progenitor cell markers in lacrimal gland between Sjögren syndrome and non-Sjögren syndrome dry eye patients.

    Science.gov (United States)

    Kawashima, Motoko; Kawakita, Tetsuya; Maida, Yoshiko; Kamoi, Mizuka; Ogawa, Yoko; Shimmura, Shigeto; Masutomi, Kenkichi; Tsubota, Kazuo

    2011-01-01

    Indicators of aging such as disruption of telomeric function due to shortening may be more frequent in dysfunctional lacrimal gland. The aims of this study were to 1) determine the viability of quantitative fluorescence in situ hybridization of telomeres (telo-FISH) for the assessment of telomere length in lacrimal gland in Sjögren and non- Sjögren syndrome patients; and 2) investigate the relationship between progenitor cell markers and telomere length in both groups. Quantitative fluorescence in situ hybridization with a peptide nucleic acid probe complementary to the telomere repeat sequence was performed on frozen sections from human lacrimal gland tissues. The mean fluorescence intensity of telomere spots was automatically quantified by image analysis as relative telomere length in lacrimal gland epithelial cells. Immunostaining for p63, nucleostemin, ATP-binding cassette, sub-family G, member 2 (ABCG2), and nestin was also performed. Telomere intensity in the Sjögren syndrome group (6,785.0±455) was significantly lower than that in the non-Sjögren syndrome group (7,494.7±477; p=0.02). Among the samples from the non-Sjögren syndrome group, immunostaining revealed that p63 was expressed in 1-3 acinar cells in each acinar unit and continuously in the basal layer of duct cells. In contrast, in the Sjögren syndrome group, p63 and nucleostemin showed a lower level of expression. ABCG2 was expressed in acinar cells in both sjogren and non-Sjogren syndrome. The results of this study indicate that 1) telo-FISH is a viable method of assessing telomere length in lacrimal gland, and 2) telomere length in Sjögren syndrome is shorter and associated with lower levels of expression of p63 and nucleostemin than in non-Sjögren syndrome.

  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. 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.

  4. 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.

  5. The Prevalence of Dry Eye and Sjögren Syndrome in Patients with Migraine.

    Science.gov (United States)

    Sarac, Ozge; Kosekahya, Pinar; Yildiz Tasci, Yelda; Keklikoglu, Hava D; Deniz, Orhan; Erten, Şükran; Çağıl, Nurullah

    2017-06-01

    To evaluate the presence of dry eye and primary Sjögren syndrome (SS) in patients with migraine. In total, 46 eyes of 46 patients with migraine (group 1) and 50 eyes of 50 healthy subjects (group 2) were included in this study. Detailed ophthalmologic, neurologic and rheumatologic examination were performed on all participants. Ocular surface disease index questionnaire, tear function tests, visual analog scale for pain, serologic analysis were also performed. Dry eye symptoms and findings were significantly higher and more severe in group 1 when compared with group 2. Primary SS was not found in any of the participants. The migraine lifetime duration was negatively correlated with the tear function tests while it was positively correlated with the ocular surface disease index scores. Dry eye symptoms and findings are higher in migraine patients when compared with the healthy subjects without the presence of Sjögren syndrome.

  6. 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.

  7. 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.

  8. The potential of the infrastructural system of Hammarby Sjöstad in Stockholm, Sweden

    International Nuclear Information System (INIS)

    Iveroth, Sofie Pandis; Johansson, Stefan; Brandt, Nils

    2013-01-01

    The purpose of this paper is to explore to what extent the integrated infrastructural system in Hammarby Sjöstad, Stockholm, also named the Hammarby Model, reduces the metabolic flows of the district, and to what extent the district is self-sufficient, in terms of generated energy. Furthermore, the paper aspires to help create a deeper understanding of the system in order to guide the implementation of similar models in other districts, creating more sustainable cities. The method has been to quantify the local mass and energy flows of the model, using the secondary energy generated within Hammarby Sjöstad as basis when creating the system boundaries of the calculations. The findings demonstrate that the Hammarby Model reduces the metabolic flows of Hammarby Sjöstad but that the district is far from self-sufficient in terms of secondary energy. The conclusions of the paper are that the development of integrated infrastructural systems is one way to help create more sustainable cities. However, in order to reduce metabolic flows even further, the efficiency of the system must be improved by integrating more renewable energy sources. At the same time less energy has to be used in the households. - Highlights: • The integrated infrastructural system reduces the metabolic flows of the district. • Integrated infrastructural systems helps creating sustainable cities. • However, Hammarby Sjöstad is far from self-sufficient. • To reduce metabolic flows further, integrate more renewables. • At the same time less energy has to be used in the households

  9. 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...

  10. What causes dryness in Sjögren's syndrome patients and how can it be targeted?

    Science.gov (United States)

    Pflugfelder, Stephen C

    2014-04-01

    Concepts regarding what causes dryness in Sjögren's syndrome have evolved over the past decade. Inflammation in the lacrimal functional unit contributes to development of dry eye by causing dysfunction and even death of tear secreting epithelium in the lacrimal gland and conjunctiva that alters tear composition and stability. Disease-relevant inflammatory mediators have been identified and therapies targeting these mediators are beginning to emerge.

  11. Cognition, depression, fatigue, and quality of life in primary Sj?gren's syndrome: correlations

    OpenAIRE

    Ko?er, Belgin; Tezcan, Mehmet Engin; Batur, Hale Zeynep; Haznedaro?lu, ?eminur; G?ker, Berna; ?rke?, Ceyla; ?etinkaya, R?meysa

    2016-01-01

    Abstract Objective The aim of the present study was to investigate the prevalence and pattern of cognitive dysfunction observed in primary Sj?gren's syndrome (PSS) and to examine the relationships between cognitive abilities, depression, fatigue, and quality of life. Materials and Methods Thirty?two subjects with PSS were compared with 19 healthy controls on comprehensive neuropsychological, depression, fatigue, health state, and daily?life activities tests. Results There was low performance ...

  12. GBA2 Mutations Cause a Marinesco-Sjögren-Like Syndrome: Genetic and Biochemical Studies.

    Directory of Open Access Journals (Sweden)

    Kristoffer Haugarvoll

    Full Text Available With the advent new sequencing technologies, we now have the tools to understand the phenotypic diversity and the common occurrence of phenocopies. We used these techniques to investigate two Norwegian families with an autosomal recessive cerebellar ataxia with cataracts and mental retardation.Single nucleotide polymorphism (SNP chip analysis followed by Exome sequencing identified a 2 bp homozygous deletion in GBA2 in both families, c.1528_1529del [p.Met510Valfs*17]. Furthermore, we report the biochemical characterization of GBA2 in these patients. Our studies show that a reduced activity of GBA2 is sufficient to elevate the levels of glucosylceramide to similar levels as seen in Gaucher disease. Furthermore, leucocytes seem to be the proper enzyme source for in vitro analysis of GBA2 activity.We report GBA2 mutations causing a Marinesco-Sjögren-like syndrome in two Norwegian families. One of the families was originally diagnosed with Marinesco-Sjögren syndrome based on an autosomal recessive cerebellar ataxia with cataracts and mental retardation. Our findings highlight the phenotypic variability associated with GBA2 mutations, and suggest that patients with Marinesco-Sjögren-like syndromes should be tested for mutations in this gene.

  13. 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.

  14. Botulinum Toxin for the Management of Sjögren Syndrome-Associated Recurrent Parotitis.

    Science.gov (United States)

    O'Neil, Luke M; Palme, Carsten E; Riffat, Faruque; Mahant, Neil

    2016-12-01

    Recurrent parotitis is a rare manifestation of Sjögren syndrome. The management of recurrent parotitis is challenging because conservative methods may be of limited efficacy and invasive approaches carry the risk of complications. Botulinum toxin has been shown to reduce salivary flow, and consequently, the results of its use in the management of recurrent parotitis have been encouraging. A 65-year-old female patient with recurrent parotitis due to Sjögren syndrome was referred to us, complaining of weekly bouts of inflammation. She required a course of antibiotics monthly to control bacterial superinfections. We treated her with onabotulinumtoxinA injections into both parotid glands at regular intervals. After her second injection cycle, she denied further inflammatory bouts, has not required antibiotics in more than 36 months, and denied any side effects. Botulinum toxin may be a safe and effective method of treating Sjögren syndrome-associated recurrent parotitis. Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  15. Effect of sialagogue on bleeding on probing in Sjögren's syndrome.

    Science.gov (United States)

    Singh, Medha; Papas, Athena

    2013-01-01

    Bleeding on probing (BOP) is a frequent observation in patients with Sjögren's syndrome and a sialagogue is routinely prescribed for these patients. The objective of this study was to evaluate the effect of sialagogue (muscarinic cholinergic agonists) on BOP in patients with Sjögren's syndrome. This observational study included 57 subjects. Study population was divided into two groups: Subjects on sialagogue (n = 32) and subjects not on sialagogue due to their side-effects (non-sialagogue, n = 25). The number of sites with BOP was recorded on all teeth. The subjects on sialagogue had a significantly lower mean (standard error) number of sites with BOP 22.97 (2.65) as compared with the non-sialagogue group 46.59 (6.20), P < 0.001. After adjusting for the use of remineralizing rinse the subjects on sialagogue had a significantly lower number of sites with BOP (P < 0.001). In this observational study treatment with sialagogue may prevent BOP in patients with Sjögren's syndrome.

  16. 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.

  17. Managing Sjögren’s Syndrome and non-Sjögren Syndrome dry eye with anti-inflammatory therapy

    Science.gov (United States)

    Coursey, Terry G; de Paiva, Cintia S

    2014-01-01

    Dry eye from Sjögren’s syndrome is a multifactorial disease that results in dysfunction of the lacrimal functional unit. Studies have shown changes in tear composition, including inflammatory cytokines, chemokines, and metalloproteinase. T-lymphocytes have been shown to increase in the conjunctiva and lacrimal glands in patient and animal models. This inflammation is in part responsible for the pathogenesis of the disease, which results in symptoms of eye irritation, ocular surface epithelial disease, and loss of corneal barrier function. There are a number of anti-inflammatory approaches for treating this disease. The current study reviews details of immune response and anti–inflammatory therapies used to control this disease. PMID:25120351

  18. Discovery of putative salivary biomarkers for Sjögren's syndrome using high resolution mass spectrometry and bioinformatics.

    Science.gov (United States)

    Zoukhri, Driss; Rawe, Ian; Singh, Mabi; Brown, Ashley; Kublin, Claire L; Dawson, Kevin; Haddon, William F; White, Earl L; Hanley, Kathleen M; Tusé, Daniel; Malyj, Wasyl; Papas, Athena

    2012-03-01

    The purpose of the current study was to determine if saliva contains biomarkers that can be used as diagnostic tools for Sjögren's syndrome (SjS). Twenty seven SjS patients and 27 age-matched healthy controls were recruited for these studies. Unstimulated glandular saliva was collected from the Wharton's duct using a suction device. Two µl of salvia were processed for mass spectrometry analyses on a prOTOF 2000 matrix-assisted laser desorption/ionization orthogonal time of flight (MALDI O-TOF) mass spectrometer. Raw data were analyzed using bioinformatic tools to identify biomarkers. MALDI O-TOF MS analyses of saliva samples were highly reproducible and the mass spectra generated were very rich in peptides and peptide fragments in the 750-7,500 Da range. Data analysis using bioinformatic tools resulted in several classification models being built and several biomarkers identified. One model based on 7 putative biomarkers yielded a sensitivity of 97.5%, specificity of 97.8% and an accuracy of 97.6%. One biomarker was present only in SjS samples and was identified as a proteolytic peptide originating from human basic salivary proline-rich protein 3 precursor. We conclude that salivary biomarkers detected by high-resolution mass spectrometry coupled with powerful bioinformatic tools offer the potential to serve as diagnostic/prognostic tools for SjS.

  19. Analysis of tear cytokines and clinical correlations in Sjögren syndrome dry eye patients and non-Sjögren syndrome dry eye patients.

    Science.gov (United States)

    Lee, Sang Yeop; Han, Soo Jung; Nam, Sang Min; Yoon, Sang Chul; Ahn, Ji Min; Kim, Tae-Im; Kim, Eung Kweon; Seo, Kyoung Yul

    2013-08-01

    To compare concentrations of tear cytokines in 3 groups composed of Sjögren syndrome (SS) dry eye, non-Sjögren syndrome (non-SS) dry eye, and normal subjects. Correlations between ocular surface parameters and tear cytokines were also investigated. Prospective cross-sectional study. SS dry eye patients (n = 24; 40 eyes) were diagnosed with primary SS according to the criteria set by the American-European Consensus Group. Non-SS dry eye patients (n = 25; 40 eyes) and normal subjects (n = 21; 35 eyes) were also enrolled. Tear concentrations of interleukin (IL)-17, IL-6, IL-10, IL-4, IL-2, interferon γ (IFN-γ), and tumor necrosis factor α (TNF-α) were measured by a multiplex immunobead assay. Ocular Surface Disease Index (OSDI), tear film breakup time (TBUT), Schirmer I test, and fluorescein staining scores were obtained from dry eye patients. All cytokine levels except for IL-2 were highest in the SS group, followed by non-SS dry eye group and control subjects. Concentrations of IL-17, TNF-α, and IL-6 were significantly different among the 3 groups (IL-17: SS > control P control P = .042, SS > non-SS P control P = .006, non-SS > control P = .034, SS > non-SS P = .029; IL-6: SS > control P = .002, non-SS > control P = .032, SS > non-SS P = .002). IL-17 was significantly correlated with TBUT (R = -0.22, P = .012) and Schirmer I test (R = -0.36, P = .027) scores in the SS group. IL-6 was significantly correlated only with TBUT (R = -0.38, P = .02) in the non-SS group. Differences in tear cytokine levels and correlation patterns between SS dry eye and non-SS dry eye patients suggest the involvement of different inflammatory processes as causes of dry eye syndrome. Copyright © 2013 Elsevier Inc. All rights reserved.

  20. Systematic review and meta-analysis of the epidemiology of polyautoimmunity in Sjögren's syndrome (secondary Sjögren's syndrome) focusing on autoimmune rheumatic diseases.

    Science.gov (United States)

    Alani, H; Henty, J R; Thompson, N L; Jury, E; Ciurtin, C

    2018-03-01

    The epidemiology of polyautoimmunity in Sjögren's syndrome (secondary Sjögren's syndrome - sSS) is not well defined and has not been investigated before using a systematic approach. We conducted a systematic review of the epidemiology of sSS associated with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma, and myositis, assessing the prevalence rates (PRs) and clinical and serological features of sSS. A systematic literature search of PubMed and Embase databases (updated to March 2016) was performed to identify all published data on PR, demographic profile, clinical manifestations, laboratory features, and causes of death associated with sSS. The PR's of sSS were summarized with PRs and 95% confidence intervals (CIs). The literature search identified 1639 citations, of which 42 fulfilled the inclusion criteria. Only 19 studies were of moderate to good quality and were selected for the meta-analysis. According to a random-effects model, the pooled PR for sSS associated with RA was 19.5% (95% CI 11.2 to 27.8) and the pooled PR for sSS associated with SLE was 13.96% (95% CI 8.88 to 19.04). The female/male ratio of sSS in the RA population was 14.7 (95% CI 7.09 to 256) and in the SLE population was 16.82 (95% CI 1.22 to 32.4). Prevalence rates of sSS vary widely in different populations. Both meta-analyses conducted in the RA and SLE populations were characterized by a high degree of study heterogeneity. The results of this meta-analysis highlight the need for better quality population studies.

  1. 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...

  2. Review of autoantigens in Sjögren's syndrome: an update.

    Science.gov (United States)

    Tong, Louis; Koh, Vanessa; Thong, Bernard Yu-Hor

    2017-01-01

    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 autoimmunity, or even lead to potential biomarkers. In conclusion, the understanding of autoantigens has led to exciting major advances in the biology of pSS and may influence diagnosis and management of pSS in future.

  3. 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

  4. 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

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

    LENUS (Irish Health Repository)

    Bergin, David A

    2010-12-01

    Hereditary deficiency of the protein α-1 antitrypsin (AAT) causes a chronic lung disease in humans that is characterized by excessive mobilization of neutrophils into the lung. However, the reason for the increased neutrophil burden has not been fully elucidated. In this study we have demonstrated using human neutrophils that serum AAT coordinates both CXCR1- and soluble immune complex (sIC) receptor-mediated chemotaxis by divergent pathways. We demonstrated that glycosylated AAT can bind to IL-8 (a ligand for CXCR1) and that AAT-IL-8 complex formation prevented IL-8 interaction with CXCR1. Second, AAT modulated neutrophil chemotaxis in response to sIC by controlling membrane expression of the glycosylphosphatidylinositol-anchored (GPI-anchored) Fc receptor FcγRIIIb. This process was mediated through inhibition of ADAM-17 enzymatic activity. Neutrophils isolated from clinically stable AAT-deficient patients were characterized by low membrane expression of FcγRIIIb and increased chemotaxis in response to IL-8 and sIC. Treatment of AAT-deficient individuals with AAT augmentation therapy resulted in increased AAT binding to IL-8, increased AAT binding to the neutrophil membrane, decreased FcγRIIIb release from the neutrophil membrane, and normalization of chemotaxis. These results provide new insight into the mechanism underlying the effect of AAT augmentation therapy in the pulmonary disease associated with AAT deficiency.

  6. 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 ...

  7. 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.

  8. 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.

  9. 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.

  10. 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.

  11. 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

  12. Inventering av Suldalslågen. Produksjonspotensial for sjøvandrende laksefisk

    OpenAIRE

    Foldvik, Anders; Pettersen, Oskar

    2017-01-01

    Foldvik, A. & Pettersen, O. 2017. Inventering av Suldalslågen. Produksjonspotensial for sjøvandrende laksefisk. - NINA Kortrapport 75, 18 s. Reguleringen av Suldalslågen til kraftproduksjon har hatt negative effekter for habitat for laksefisk, blant annet i form av sedimentering og begroing av substratet. Disse prosessene har blitt forsøkt motvirket ved å ha en serie med spyleflommer på over 200 m3/s om høsten. På oppdrag fra Statkraft inverterte NINA oppvekst- og gyteforhold for laks i Su...

  13. Effect of Oral Pilocarpine in Treating Severe Dry Eye in Patients With Sjögren Syndrome.

    Science.gov (United States)

    Kawakita, Tetsuya; Shimmura, Shigeto; Tsubota, Kazuo

    2015-01-01

    The aim of this study is to evaluate the efficacy and safety of oral pilocarpine in treating severe dry eye unresponsive to conventional conservative treatment in patients with Sjögren syndrome. A prospective study. Oral doses of pilocarpine were administered for at least 3 months to patients with Sjögren syndrome complicated by established dry eye of great severity unresponsive to conventional conservative treatment. Subjective eye symptoms (dry eye sensation and eye pain), fluorescein staining scores, rose Bengal staining scores, and tear film breakup time measurements improved significantly after 1 month and 3 months of oral treatment with pilocarpine, whereas no significant improvement was noted in Schirmer I testing. Oral administration of pilocarpine was useful in treating severe dry eye unresponsive to conventional conservative treatment in patients with Sjögren syndrome from the standpoint of efficacy and safety. Thus, we conclude that oral pilocarpine is effective as a new option in treating severe dry eye.

  14. 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.

  15. Jacobsens ukendte samling af stensager fra den græske oldtid. Obsidian, økser, matricer, ansigtssten og andre sjældne oldsager

    DEFF Research Database (Denmark)

    Sørensen, Lasse

    2012-01-01

    Jacobsens ukendte samling af stensager fra den græske oldtid. Obsidian, økser, matricer, ansigtssten og andre sjældne oldsager......Jacobsens ukendte samling af stensager fra den græske oldtid. Obsidian, økser, matricer, ansigtssten og andre sjældne oldsager...

  16. Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren's syndrome among 1,726 registry participants

    DEFF Research Database (Denmark)

    Daniels, Troy E; Cox, Darren; Shiboski, Caroline H

    2011-01-01

    To examine associations between labial salivary gland (LSG) histopathology and other phenotypic features of Sjögren's syndrome (SS).......To examine associations between labial salivary gland (LSG) histopathology and other phenotypic features of Sjögren's syndrome (SS)....

  17. 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.

  18. 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.

  19. 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

  20. 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.

  1. 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.

  2. Primary Sjögren's syndrome accompanied by pleural effusion: a case report and literature review.

    Science.gov (United States)

    Ma, Dedong; Lu, Hongxiu; Qu, Yiqing; Wang, Shanshan; Ying, Yangyang; Xiao, Wei

    2015-01-01

    Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes in exocrine glands, specifically the salivary and lacrimal glands, resulting in the typical symptoms of xerophthalmia and xerostomia. SS may be accompanied by pleural effusion when the lung is involved, but this occurrence has been reported in only 10 cases in the literature. We report the case of a 42 year-old woman with severe bilateral pleural effusion for eight years. Primary Sjögren's Syndrome was finally diagnosed based on the presence of xerophthalmia and xerostomia, biopsy of the minor salivary glands, and positive anti-SS-A antibody in the serum and pleural effusion. Biopsy of the parietal pleura through video-assisted thoracoscopy revealed infiltration of lymphocytes. The patient had a long history of pleural effusion without clear etiology. Malignant disease was first suspected because of abnormal density lesion on the left lung and malignant cells found on cytology, but PET-CT revealed no malignant lesion. Examinations did not support infection, malignant tumor, pulmonary sarcoidosis, or other connective tissue diseases. This data could be useful for the future study of pleural effusion in SS.

  3. B{sub c} → B{sub sJ} form factors and B{sub c} decays into B{sub sJ} in covariant light-front approach

    Energy Technology Data Exchange (ETDEWEB)

    Shi, Yu-Ji; Zhao, Zhen-Xing [Shanghai Jiao-Tong University, INPAC, Shanghai Key Laboratory for Particle Physics and Cosmology, Department of Physics and Astronomy, Shanghai (China); Wang, Wei [Shanghai Jiao-Tong University, INPAC, Shanghai Key Laboratory for Particle Physics and Cosmology, Department of Physics and Astronomy, Shanghai (China); Chinese Academy of Sciences, State Key Laboratory of Theoretical Physics, Institute of Theoretical Physics, Beijing (China)

    2016-10-15

    We suggest to study the B{sub s} and its excitations B{sub sJ} in the B{sub c} decays. We calculate the B{sub c} → B{sub sJ} and B{sub c} → B{sub J} form factors within the covariant light-front quark model, where the B{sub sJ} and B{sub J} denote an s-wave or p-wave anti bs and anti bd meson, respectively. The form factors at q{sup 2} = 0 are directly computed while their q{sup 2}-distributions are obtained by extrapolation. The derived form factors are then used to study semileptonic B{sub c} → (B{sub sJ}, B{sub J}) anti lν decays, and nonleptonic B{sub c} → B{sub sJ}π. Branching fractions and polarizations are predicted in the standard model. We find that the branching fractions are sizable and might be accessible at the LHC experiment and future high-energy e{sup +}e{sup -} colliders with a high luminosity at the Z-pole. The future experimental measurements are helpful to study the nonperturbative QCD dynamics in the presence of a heavy spectator and also of great value for the study of spectroscopy. (orig.)

  4. 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

  5. [Oral flaxseed oil (Linum usitatissimum) in the treatment for dry-eye Sjögren's syndrome patients].

    Science.gov (United States)

    Pinheiro, Manuel Neuzimar; dos Santos, Procópio Miguel; dos Santos, Regina Cândido Ribeiro; Barros, Jeison de Nadai; Passos, Luiz Fernando; Cardoso Neto, José

    2007-01-01

    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. In a randomized clinical trial, 38 female patients with rheumatoid arthritis or systemic lupus erithematosus associated with keratoconjunctivitis sicca and Sjögren's syndrome were consecutively selected from patients of the Department of Rheumatology of the Amazonas University Hospital. Keratoconjunctivitis sicca diagnosis was based on a dry-eye symptom survey score (Ocular Surface Disease Index - OSDI), Schirmer-I test, fluorescein break-up time, 1% Rose Bengal staining of ocular surface measured by the van Bijsterveld scale. All patients had ocular surface inflammation evaluated and quantified by conjunctival impression cytology, before and after the study. The subjects were divided into three groups with 13 (Group I), 12 (Group II) and 13 (Group III) patients. Group I received flaxseed oil capsules with a final 1 g/day dosis, Group II flaxseed oil capsules with a final 2 g/day dosis and Group III - controls - placebo, for 180 days. Comparing the results at the beginning and at the end of the treatment, statistically significant changes (poral flaxseed oil capsules 1 or 2 g/day reduces ocular surface inflammation and ameliorates the symptoms of keratoconjunctivitis sicca in Sjögren's syndrome patients. Long-term studies are needed to confirm the role of this therapy for keratoconjunctivitis sicca in Sjögren's syndrome.

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

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    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

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

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    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.

  8. SjCRT, a recombinant Schistosoma japonicum calreticulin, induces maturation of dendritic cells and a Th1-polarized immune response in mice

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    Lizhen Ma

    2017-11-01

    Full Text Available Abstract Background It is well known that immunization of radiation-attenuated (RA schistosoma cercariae or schistosomula can induce high levels of protective immunity against schistosoma cercariae reinfection in many animals. Many studies have shown that the Th1 cellular immune response is crucial for the protective effect elicited by RA schistosomula. However, the molecular mechanism of this strong protective immunity remains unclear. Methods The expression profiles of Schistosoma japonicum calreticulin (SjCRT in RA and normal schistosoma-derived cells were investigated by flow cytometry. The effect of recombinant SjCRT (rSjCRT on mouse dendritic cells (DCs was determined by FACS, ELISA and RT-PCR analysis. We also analyzed the effects of SjCRT on the activation of spleen cells from mice immunized with rSjCRT by detecting lymphocyte proliferation and the cytokine profiles of splenocytes. Results We found that the expression level of SjCRT in the cells from RA larvae was significantly higher than that in cells from normal schistosomula at early stages of development (day 4. The results of effect of rSjCRT on mouse DCs showed that rSjCRT could induce phenotypic and functional maturation of DCs, and SjCRT bound to the surface of DCs through the CD91 receptor and could be engulfed by DCs. The results of activation of splenocytes from mice immunized with rSjCRT also demonstrate that rSjCRT can effectively stimulate the proliferative response of splenic lymphocytes, elicit splenocytes from immunized mice to secrete high levels of IFN-γ, TNF-α and IL-4, and activate CD4+ T cells to produce high levels of IFN-γ. Conclusion SjCRT is one of the immunostimulatory molecules released from RA schistosomula cells, might play a crucial role in conferring a Th1-polarized immune response induced by RA cercariae/schistosomula in mice, and is a candidate molecule responsible for the high levels of protective immunity induced by RA schistosomula.

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

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    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.

  10. 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.

  11. 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.

  12. 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.

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

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    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.

  14. Factors associated with severe dry eye in primary Sjögren's syndrome diagnosed patients.

    Science.gov (United States)

    Fernandez Castro, Mónica; Sánchez-Piedra, Carlos; Andreu, Jose Luis; Martínez Taboada, Víctor; Olivé, Alejandro; Rosas, Jose

    2018-06-01

    Primary Sjögren's syndrome (pSS) is an autoimmune disease, characterized by lymphocytic infiltration of exocrine glands and other organs, resulting in dry eye, dry mouth and extraglandular systemic findings. To explore the association of severe or very severe dry eye with extraocular involvement in patients diagnosed with primary Sjögren's syndrome. SJOGRENSER registry is a multicenter cross-sectional study of pSS patients. For the construction of our main variable, severe/very severe dry eye, we used those variables that represented a degree 3-4 of severity according to the 2007 Dry Eye Workshop classification. First, bivariate logistic regression models were used to identify the effect of each independent variable on severe/very severe dry eye. Secondly, multivariate analysis using regression model was used to establish the independent effect of patient characteristics. Four hundred and thirty-seven patients were included in SJOGRENSER registry; 94% of the patients complained of dry eye and 16% developed corneal ulcer. Schirmer's test was pathological in 92% of the patients; 378 patients presented severe/very severe dry eye. Inflammatory articular involvement was significantly more frequent in patients with severe/very severe dry eye than in those without severe/very severe dry eye (82.5 vs 69.5%, p = 0,028). Inflammatory joint involvement was associated with severe/very severe dry eye in the multivariate analysis, OR 2.079 (95% CI 1.096-3.941). Severe or very severe dry eye is associated with the presence of inflammatory joint involvement in patients with pSS. These results suggest that a directed anamnesis including systemic comorbidities, such as the presence of inflammatory joint involvement or dry mouth in patients with dry eye, would be useful to suspect a pSS.

  15. 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

  16. 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

  17. Oral health-related quality of life in primary Sjögren's syndrome.

    Science.gov (United States)

    Fernández-Martínez, Gladyz; Zamora-Legoff, Víctor; Hernández Molina, Gabriela

    2018-05-10

    To assess health-related quality of life (HRQoL) and oral health-related quality of life, and correlate them with unstimulated whole salivary flow (UWSF) and oral sicca symptoms in patients with primary Sjögren's syndrome (PSS). We included 60 patients with PSS and 60 healthy controls matched according to gender and age (±3 years). We measured the UWSF and scored the European League Against Rheumatism (EULAR) Sjögren's Syndrome Patient Reported Index (ESSPRI). We assessed the short version of the SF-36 as a generic measurement of HRQoL and the Xerostomia Quality of Life Scale (XeQoLS) questionnaire to evaluate oral quality of life. We evaluated oral symptoms using an 8-item Visual Analogue Scale (VAS) questionnaire. We observed a poorer HRQoL (lower scores in SF-36) and oral quality of life (higher scores in XeQoLS), as well as a greater severity of symptoms in the VAS questionnaire upon comparing patients vs. controls. The XeQoL correlated with the UWSF (τ = -0.24, P = .008), the ESSPRI (τ =0.45, P = .0001), VAS 1-2 and VAS 5-8 and the SF-36 score (τ = -0.28, P = .002). Patients with PSS had a poorer HRQoL and oral quality of life than controls. UWSF contributes to the oral quality of life which, in turn, has an impact on HRQoL. Symptomatic treatment of xerostomia as well as the prevention of infections, decay and tooth loss would help to improve the oral quality of life in these patients. Copyright © 2018 Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. Publicado por Elsevier España, S.L.U. All rights reserved.

  18. Is salivary gland ultrasonography a useful tool in Sjögren's syndrome? A systematic review.

    Science.gov (United States)

    Jousse-Joulin, Sandrine; Milic, Vera; Jonsson, Malin V; Plagou, Athena; Theander, Elke; Luciano, Nicoletta; Rachele, Pascale; Baldini, Chiara; Bootsma, Hendrika; Vissink, Arjan; Hocevar, Alojzija; De Vita, Salvatore; Tzioufas, Athanasios G; Alavi, Zarin; Bowman, Simon J; Devauchelle-Pensec, Valerie

    2016-05-01

    Ultrasonography (US) is a sensitive tool in the diagnosis of major salivary gland abnormalities in primary Sjögren's syndrome (pSS). The aim of this systematic review was to assess the metric properties of this technique. PUBMED and EMBASE databases were searched. All publications between January 1988 and January 2013 were considered. Data were extracted from the articles meeting the inclusion criteria according to US definition of salivary gland scoring system and metric properties studied. The type and number of glands tested, study design and metric properties according to OMERACT filter (truth, discrimination, feasibility) were assessed. Of 167 publications identified initially with PUBMED and EMBASE, 31 met the inclusion criteria. The number of pSS patients varied among the studies from 16 to 140. The diagnosis of pSS was in line in most of the cases with the American-European Consensus Group (AECG) classification criteria for Sjögren's syndrome. The US examination was performed in suspected pSS only in studies in which the sensitivity ranged from 45.8 to 91.6% and specificity from 73 to 98.1%. There was heterogeneity in regard to the definition of US in B-mode and few studies used US in colour Doppler. Few studies reported reliability of US and sensitivity to change in pSS. US is a valuable tool for detecting salivary gland abnormalities in pSS. Its reliability has been poorly investigated and there is considerable variation in the definition of US abnormalities. Further studies are required to validate and standardize the US definition of salivary gland in pSS. © 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.

  19. Meibomian Gland Dysfunction in Primary and Secondary Sjögren Syndrome.

    Science.gov (United States)

    Sullivan, David A; Dana, Reza; Sullivan, Rose M; Krenzer, Kathleen L; Sahin, Afsun; Arica, Beril; Liu, Yang; Kam, Wendy R; Papas, Athena S; Cermak, Jennifer M

    2018-01-01

    We hypothesized that women with primary (pSS) and secondary Sjögren syndrome (sSS; with systemic lupus erythematosus [SLE] or rheumatoid arthritis [RA]) have meibomian gland dysfunction (MGD). We sought to test our hypothesis. Subjects with pSS, sSS + SLE, sSS + RA, and non-SS-related MGD were recruited from the Sjögren's Syndrome Foundation or outpatient clinics at Tufts University School of Dental Medicine or Brigham and Women's Hospital. The control population was recruited from the Greater Boston area. After providing written informed consent, the subjects underwent an eye examination and/or completed two questionnaires that assess symptoms of dry eye disease (DED). Our results demonstrate that pSS and sSS patients have MGD. These subjects had meibomian gland orifice metaplasia, an increased number of occluded meibomian gland orifices, and a reduced quality of meibomian gland secretions. Further, patients with pSS, sSS + SLE, sSS + RA, and MGD had significant alterations in their tear film, lid margin, cornea, and conjunctiva. Symptoms of DED were increased ∼10-fold in all pSS, sSS, and MGD groups relative to controls. Our findings support our hypothesis and show that individuals with pSS, sSS + SLE, and sSS + RA have MGD. In addition, our study indicates that patients with pSS and sSS have both aqueous-deficient and evaporative DED. © 2018 S. Karger AG, Basel.

  20. Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients: a cross-sectional study from the Big Data Sjögren Project Consortium.

    Science.gov (United States)

    Brito-Zerón, Pilar; Acar-Denizli, Nihan; Zeher, Margit; Rasmussen, Astrid; Seror, Raphaele; Theander, Elke; Li, Xiaomei; Baldini, Chiara; Gottenberg, Jacques-Eric; Danda, Debashish; Quartuccio, Luca; Priori, Roberta; Hernandez-Molina, Gabriela; Kruize, Aike A; Valim, Valeria; Kvarnstrom, Marika; Sene, Damien; Gerli, Roberto; Praprotnik, Sonja; Isenberg, David; Solans, Roser; Rischmueller, Maureen; Kwok, Seung-Ki; Nordmark, Gunnel; Suzuki, Yasunori; Giacomelli, Roberto; Devauchelle-Pensec, Valerie; Bombardieri, Michele; Hofauer, Benedikt; Bootsma, Hendrika; Brun, Johan G; Fraile, Guadalupe; Carsons, Steven E; Gheita, Tamer A; Morel, Jacques; Vollenveider, Cristina; Atzeni, Fabiola; Retamozo, Soledad; Horvath, Ildiko Fanny; Sivils, Kathy; Mandl, Thomas; Sandhya, Pulukool; De Vita, Salvatore; Sanchez-Guerrero, Jorge; van der Heijden, Eefje; Trevisani, Virginia Fernandes Moça; Wahren-Herlenius, Marie; Mariette, Xavier; Ramos-Casals, Manuel

    2017-06-01

    To analyse the influence of geolocation and ethnicity on the clinical presentation of primary Sjögren's syndrome (SjS) at diagnosis. The Big Data Sjögren Project Consortium is an international, multicentre registry designed in 2014. By January 2016, 20 centres from five continents were participating. Multivariable logistic regression analyses were performed. We included 7748 women (93%) and 562 men (7%), with a mean age at diagnosis of primary SjS of 53 years. Ethnicity data were available for 7884 patients (95%): 6174 patients (78%) were white, 1066 patients (14%) were Asian, 393 patients (5%) were Hispanic, 104 patients (1%) were black/African-American and 147 patients (2%) were of other ethnicities. SjS was diagnosed a mean of 7 years earlier in black/African-American compared with white patients; the female-to-male ratio was highest in Asian patients (27:1) and lowest in black/African-American patients (7:1); the prevalence of sicca symptoms was lowest in Asian patients; a higher frequency of positive salivary biopsy was found in Hispanic and white patients. A north-south gradient was found with respect to a lower frequency of ocular involvement in northern countries for dry eyes and abnormal ocular tests in Europe (OR 0.46 and 0.44, respectively) and Asia (OR 0.18 and 0.49, respectively) compared with southern countries. Higher frequencies of antinuclear antibodies (ANAs) were reported in northern countries in America (OR=1.48) and Asia (OR=3.80) while, in Europe, northern countries had lowest frequencies of ANAs (OR=0.67) and Ro/La (OR=0.69). This study provides the first evidence of a strong influence of geolocation and ethnicity on the phenotype of primary SjS at diagnosis. 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/.

  1. The Efficacy of Diquafosol Ophthalmic Solution in Non-Sjögren and Sjögren Syndrome Dry Eye Patients Unresponsive to Artificial Tear.

    Science.gov (United States)

    Jeon, Hyun Sun; Hyon, Joon Young

    2016-09-01

    This study evaluates the efficacy of 3% diquafosol tetrasodium ophthalmic solution in Sjögren's syndrome (SS) and non-SS dry eye patients unresponsive to conventional artificial tear. This retrospective study included 38 dry eye patients (11 with SS and 27 with non-SS) who were treated with 3% diquafosol due to lack of response to artificial tear treatment for more than 3 months. Signs such as tear film break-up time (TBUT), Schirmer-I test, and total ocular staining score (OSS), which consisted of corneal OSS and conjunctival OSS, were evaluated at baseline and the first visit after treatment. Symptoms were quantified using visual analog scale (VAS) and ocular surface disease index (OSDI) scores. Changes of parameters after treatment in all the patients and differences of changes in SS and non-SS were evaluated. In total population, total OSS and corneal OSS improved average 6 weeks after diquafosol treatment (all P < 0.05, paired t-test), although conjunctival OSS had no improvement. TBUT increased after treatment (P = 0.036), while Schirmer-I test, OSDI, and VAS showed no improvement. With respect to comparisons, total and corneal OSS improved in non-SS patients and TBUT improved in SS patients (all P < 0.05). Three percent diquafosol tetrasodium treatment could be considered as an additive or substitute treatment when artificial tear treatment is insufficient, as it improved OSS and TBUT. This study also demonstrated that diquafosol treatment is beneficial for TBUT improvement in SS, while it is beneficial for OSS improvement in non-SS dry eye patients.

  2. Comparison of corneal biomechanics in Sjögren's syndrome and non-Sjögren's syndrome dry eyes by Scheimpflug based device.

    Science.gov (United States)

    Long, Qin; Wang, Jing-Yi; Xu, Dong; Li, Ying

    2017-01-01

    To compare the corneal biomechanics of Sjögren's syndrome (SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology (CorVis ST). Corneal biomechanics and tear film parameters, namely the Schirmer I test value, tear film break-up time (TBUT) and corneal staining score (CSS) were detected in 34 eyes of 34 dry eye patients with SS (SSDE group) and 34 dry eye subjects without SS (NSSDE group) using CorVis ST. The differences of the above parameters between the two groups were examined, and the relationship between corneal biomechanics and tear film parameters were observed. The differences in age, sex, intraocular pressure (IOP) and central corneal thickness (CCT) were not significant between the two groups ( P >0.05). The tear film parameters had significant differences between the SSDE group and NSSDE group (all P <0.05). Patients in the SSDE group had significantly lower A1-time and HC-time, but higher DA ( P =0.01, 0.02, and 0.02, respectively) compared with the NSSDE group. In the SSDE group, DA was negatively correlated with TBUT ( rho =-0.38, P =0.03); HC-time was negatively correlated with CSS ( rho =-0.43, P =0.02). In the NSSDE group, HC-time was again negatively correlated with CSS ( rho =-0.39, P =0.02). There are differences in corneal biomechanical properties between SSDE and NSSDE. The cornea of SSDE tends to show less "stiffness", as seen by a significantly shorter A1-time and HC-time, but larger DA, compared with the cornea of NSSDE. Biomechanical parameters can be influenced by different tear film parameters in both groups.

  3. Sjögren SER: National registry of the Spanish Society of Rheumatology of patients with primary Sjögren syndrome: Objectives and methodology.

    Science.gov (United States)

    Fernández Castro, Mónica; Andreu, Jose Luis; Sánchez-Piedra, Carlos; Martínez Taboada, Víctor; Olivé, Alejandro; Rosas, José; Sánchez-Alonso, Fernando

    2016-01-01

    To describe the objectives and methods of the Spanish Society of Rheumatology primary Sjögren syndrome (pSS) registry (SJOGREN-SER) METHODS: This is a multicenter descriptive transversal study of a cohort of pSS patients fulfilling European/American consensus criteria collected from Rheumatology clinics all over Spain. Patients were included by randomisation from an anonymised list provided by every department. Data were collected by reviewing clinical records and an interviewing the patients. Two hundred and ninety eight variables were investigated: epidemiological, clinical, serological characteristics, treatments and complications. Informed consent was obtained and local ethics committees approved the study. Variables were analysed by descriptive statistical methods, using means, medians, and rates, with their deviations and interquartile ranges (p25-p75). A total of 3 rheumatology departments participated in the registry. A total of 437 patients were included. And 95% of them were women, with a median age of 58. Median age at pSS 's diagnosis was 50 years. Dryness symptoms (95%) were the most frequent complaint and anti-Ro/SS-A were present in 94% of the cases. Only 27% of the patients fulfilled the new 2012 SICCA-ACR classification criteria. SJOGREN-SER has been designed in order to characterize a representative pSS Spanish cohort, in clinical daily practice, to analyze the magnitude and distribution of its manifestations, activity, accumulated damage and therapeutic management of the disease. This will allow broadening the knowledge of this disease and plan strategies of action in pSS. Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

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

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    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

  5. The Interferon-signature of Sjögren’s Syndrome: How Unique Biomarkers Can Identify Underlying Inflammatory and Immunopathological Mechanisms of Specific Diseases

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    Cuong eNguyen

    2013-07-01

    Full Text Available Innate immune responses direct the nature and specificity of downstream adaptive responses in autoimmune diseases. One of the strongest markers of innate immunity is the up-regulated expression of interferon (IFN and IFN-responsive/stimulated genes (IRGs/ISGs. While multiple IRGs are induced during the innate phase of host responses, transcriptome data suggest unique IRG-signatures for different diseases. Sjögren’s syndrome (SjS is characterized by chronic immune attacks against exocrine glands leading to exocrine dysfunction, plus strong up-regulated expressions of IFN IRG transcripts. Genome-wide transcriptome analyses indicate that differentially-expressed IRGs are restricted during disease development and therefore define underlying etiopathological mechanisms. Here we review the innate immune-associated IFN-signature of SjS and show how differential gene expressions of IRG/ISG sets interact molecularly and biologically to identify critical details of SjS etiopathogenesis.

  6. Den inre och yttre självkänslans betydelse för tendensen att bruka self-handicapping

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    Ottosson, Olivia

    2008-01-01

    Självkänsla är något vi ständigt bär med oss och den inverkar på vårt agerande samt våra val i livet. Johnson (2003) delar in självkänslan i två delar den inre och yttre, vilka kan kombineras till fyra personligheter. Forskning har visat att självkänslan samvarierar med tendensen till att bruka self-handicapping. Self-handicapping innebär att människan skapar hinder för sig själv påhittade eller verkliga. Etthundrasju studenter fyllde i en enkät bestående av 58 påståenden, vilken mätte inre, ...

  7. Increased risk of concurrent gastroesophageal reflux disease among patients with Sjögren's syndrome: A nationwide population-based study.

    Science.gov (United States)

    Chang, Chen-Shu; Liao, Chun-Hui; Muo, Chih-Hsin; Kao, Chia-Huang

    2016-06-01

    Little data is available on the risk of gastroesophageal reflux disease in patients diagnosed with Sjögren's syndrome. We identified 4650 Sjögren's syndrome patients between 2000 and 2011 from the National Health Insurance Research Database. Each Sjögren's syndrome patient was matched to 4 controls based on age, sex, and index year, and all subjects were followed up from the index date to December 31, 2011. Cox proportional hazards regression model was used to estimate the risk of gastroesophageal reflux disease. The risk of gastroesophageal reflux disease for Sjögren's syndrome patients was 2.41-fold greater than that for the comparison cohort after adjusting for age, sex, and comorbidities. In age stratified analyses, the youngest Sjögren's syndrome cohort (age: 20-44years old) had the highest risk (HR=3.02; 95% CI=2.48-3.69) and the lowest risk at age ≥65years (HR=1.95; 95% CI=1.61-2.36). Regardless of in subjects with and without comorbidity, Sjögren's syndrome patients had a higher risk than the controls. Sjögren's syndrome subjects with ischemic heart disease, hyperlipidemia and renal disease had the highest risk for gastroesophageal reflux disease compared with the comparison cohort without those diseases (HR=7.67; 95% CI=5.32-11.1). Patients with Sjögren's syndrome have a significantly greater risk of developing subsequent gastroesophageal reflux disease than the general population. Copyright © 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

  8. 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

    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. METHODS: We performed genome-wide association study analyses using SICCA subjects and external controls obtained from dbGaP data sets, one using all......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...... subphenotype distributions differ by ethnicity, and whether this contributes to the heterogeneity of genetic associations. RESULTS: 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...

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

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    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. MUC19 expression in human ocular surface and lacrimal gland and its alteration in Sjögren syndrome patients.

    Science.gov (United States)

    Yu, D F; Chen, Y; Han, J M; Zhang, H; Chen, X P; Zou, W J; Liang, L Y; Xu, C C; Liu, Z G

    2008-02-01

    This study investigated the expression of MUC19, a newly discovered gel-forming mucin gene, in normal human lacrimal functional unit components and its alteration in Sjögren syndrome patients. Real-time PCR and immunohistochemistry were performed to determine the expression of MUC19 and MUC5AC in human cornea, conjunctiva, and lacrimal gland tissues. Conjunctival impression cytology specimens were collected from normal control subjects and Sjögren syndrome patients for Real-time PCR, PAS staining, and immunohistochemistry assays. In addition, conjunctiva biopsy specimens from both groups were examined for the expression differences of MUC19 and MUC5AC at both mRNA and protein level. The MUC19 mRNA was found to be present in cornea, conjunctiva and lacrimal gland tissues. The immunohistochemical staining of mucins showed that MUC19 was expressed in epithelial cells from corneal, conjunctival, and lacrimal gland tissues. In contrast, MUC5AC mRNA was only present in conjunctiva and lacrimal gland tissues, but not in cornea. Immunostaining demonstrates the co-staining of MUC19 and MUC5AC in conjunctival goblet cells. Consistent with the significant decrease of mucous secretion, both MUC19 and MUC5AC were decreased in conjunctiva of Sjögren syndrome patients compared to normal subjects. Considering the contribution of gel-forming mucins to the homeostasis of the ocular surface, the decreased expression of MUC19 and MUC5AC in Sjögren syndrome patients suggested that these mucins may be involved in the disruption of the ocular surface homeostasis in this disease.

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

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    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

  12. Distal renal tubular acidosis as a cause of osteomalacia in a patient with primary Sjögren's syndrome

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    Jovelić Aleksandra

    2005-01-01

    Full Text Available Background. One half of the patients with primary Sjögren’s syndrome has extraglandular manifestations, including renal involvement. The most frequent renal lesion is tubulo-interstitial nephritis, which manifests clinically as distal tubular acidosis and may result in the development of osteomalacia. Case report. In a 29 - year-old female patient, with bilateral nephrolithiasis, the diagnosis of primary Sjögren’s syndrome, tubulo-interstitial nephritis, distal renal tubular acidosis, and hypokalemia were established. She was treated for hypokalemia. Two years later she developed bone pains and muscle weakness, she wasn’t able to walk, her proximal muscles and pelvic bones were painful, with radiological signs of pelvic bones osteopenia and pubic bones fractures. The diagnosis of osteomalacia was established and the treatment started with Schol’s solution, vitamin D and calcium. In the following two months, acidosis was corrected, and the patient started walking. Conclusion. In our patient with primary Sjögren’s syndrome and interstitial nephritis, osteomalacia was a result of the long time decompensate acidosis, so the correction of acidosis, and the supplementation of vitamin D and calcium were the integral part of the therapy.

  13. Anti-cyclic citrullinated peptide antibodies and rheumatoid factor in Sjögren's syndrome.

    Science.gov (United States)

    Barcelos, Filipe; Abreu, Isabel; Patto, José Vaz; Trindade, Hélder; Teixeira, Ana

    2009-01-01

    The purpose of this study was to evaluate the prevalence and clinical significance of anti-cyclic citrullinated peptide antibodies (anti-CCP-Abs), IgM and IgA rheumatoid factors (RFs) in primary Sjögren's Syndrome (pSS). We compared clinical and serological characteristics of 31 pSS and 31 Rheumatoid Arthritis (RA) patients. Both, anti-CCP-Abs and RFs (IgM, IgA) directed against Fc determinants of IgG from humans and rabbit were detected by enzyme-linked immunosorbent assay (ELISA). We included 31 blood donors as control group for the evaluation of RFs and anti-CCP-Abs. Nine (29%) pSS patients presented arthritis, and 10 (32,3%) RA patients also had secondary Sjögren's syndrome (sSS) RESULTS: IgM and IgA RFs prevalence was similar in pSS and RA, whichever the antigene (Human or Rabbit IgG) used. However, RA patients with sSS showed a tendency to present more often RF positivity, longer disease duration and higher ESR and CRP when compared with pSS patients with arthritis. Anti-CCP-Abs were detected in 64,5% of RA patients and in only 6,9% of pSS patients (p<0,0005). Anti-CCP-Abs were more often positive in RA patients with sSS (RA/sSS) (8 patients, 80%) than in RA patients without sSS (18 patients, 58,1%), and were absent in pSS patients with arthritis. RF-positive pSS patients presented more often pulmonary involvement and higher inflammatory parameters, and less often neuropathy compared to RF-negative patients. In controls, anti-CCP-Abs were absent and RFs were negligible. Anti-CCP-Abs were detected in only a few pSS patients, none of whom presented arthritis, which contrasts with the high frequency of these antibodies in RA/sSS. These results suggest that anti-CCP-Abs could be useful in the distinction between pSS and RA with sSS. Although not useful for the differential diagnosis between RA and pSS, RFs may have a prognostic role in pSS.

  14. 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.

  15. Elevation of autophagy markers in Sjögren syndrome dry eye.

    Science.gov (United States)

    Byun, Yong-Soo; Lee, Hyun Jung; Shin, Soojung; Chung, So-Hyang

    2017-12-08

    Autophagy is known to be implicated in the pathogenesis of Sjögren syndrome (SS), but evidences are limited. We aimed to examine the levels of autophagy markers in tear film and conjunctival epithelial cells from SS dry eye patients, and analyze their correlations with clinical features. Patients with SS dry eye exhibited lower Schirmer values, lower tear breakup time, and higher ocular staining scores. In tears, ATG5 and LC3B-II/I levels were significantly higher in SS dry eye. ATG5 and LC3B-II mRNA in the conjunctiva were also elevated in SS dry eye compared with non-SS dry eye. The immunostaining of conjunctival epithelium showed a punctate pattern of ATG5 and LC3B-II in SS dry eye. These staining patterns were also observed in the lacrimal gland of SS animal models. ATG5 levels in tears and the conjunctival epithelium strongly correlated with ocular staining scores, and one month of topical corticosteroid treatment reduced both ATG5 and LC3B-II/I levels in tear film and the conjunctival epithelium of patients with SS dry eye. Our results suggest that autophagy is enhanced or dysregulated in SS and autophagy markers may be serve as both diagnostic and therapeutic biomarkers in SS dry eye.

  16. Association of IL-21 cytokine with severity of primary Sjögren syndrome dry eye.

    Science.gov (United States)

    Lim, Sung A; Nam, Doo Hyun; Lee, Jee Hye; Kwok, Seung-Ki; Park, Sung-Hwan; Chung, So-Hyang

    2015-03-01

    IL-21 plays an important role in primary Sjögren syndrome (SS) pathogenesis. The purpose of this study was to evaluate IL-21 expression in tears and the conjunctiva and to analyze the impact of IL-21 on primary SS dry eyes. Eighty subjects were enrolled in this study: 30 patients with primary SS dry eye (30 eyes); 30 patients with non-SS dry eye (30 eyes), and 20 normal controls. Tear IL-21 levels were measured by flow cytometry, and IL-21 gene expression in the conjunctiva from impression cytology was evaluated by quantitative polymerase chain reaction. Ocular Surface Disease Index, tear film breakup time, Schirmer I test, and ocular surface staining scores were obtained for all patients. Primary SS dry eyes had significantly higher tear IL-21 levels than non-SS dry eyes and normal controls (P dry eyes than in non-SS dry eyes and normal controls (P dry eyes and controls. The tear IL-21 level was significantly correlated with ocular surface stain scores (r = 0.54, P dry eyes. Our findings suggest that severity of primary SS dry eye is associated with IL-21.

  17. 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.

  18. Corneal confocal microscopy alterations in Sjögren's syndrome dry eye.

    Science.gov (United States)

    Lanza, Michele; Iaccarino, Stefania; Varricchi, Gilda; D'Errico, Tito; Gironi Carnevale, Ugo Antonello; Bifani, Mario

    2017-08-01

    To evaluate light backscattering (LB) in corneal layers in patients with primary Sjögren's syndrome dry eye (SSDE) utilizing in vivo corneal confocal microscopy (IVCM) and to determine the eventual association with the lacrimal functional test values. A complete ophthalmic evaluation, Schirmer test with and without stimulation, break-up time (BUT) and IVCM were performed on 55 patients affected by SSDE and in an age- and sex-matched cohort of healthy participants (HP). Light backscattering, measures as light reflectivity unit (LRU), detected by IVCM at Bowman's membrane (BM) at 50 μm, at 100 μm and at 200 μm deeper than BM was compared in the two groups. The correlations between LB values and lacrimal function results were evaluated. In patients affected by SSDE, LB was significantly higher (p lacrimal function. Light backscattering (LB) could be very useful for clinical diagnosis and management of SSDE. © 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

  19. Prevalence and Predictors of Sjögren's Syndrome in Patients with Burning Mouth Symptoms.

    Science.gov (United States)

    Lee, Young Chan; Song, Ran; Yang, You-Jung; Eun, Young-Gyu

    To investigate the prevalence and predictive factors of Sjögren's syndrome (SS) in a cohort of patients with burning mouth symptoms. A total of 125 patients with burning mouth symptoms were enrolled in a prospective study and assessed for the presence of SS. The severity of oral symptoms was evaluated by using questionnaires. Salivary flow rates and salivary scintigraphy were used to evaluate salivary function. Patient laboratory work-ups were reviewed, and SS was diagnosed by a rheumatologist based on the American-European Consensus Group criteria. The differences between the SS patient group and the non-SS patient group were analyzed with chi-square test or t test. A total of 12 of the 125 enrolled patients (9.5%) had a positive autoimmune antibody test, and 6 (4.8% of the entire cohort) had SS (4 [3.2%] primary and 2 [1.6%] secondary). Patients with SS exhibited significantly decreased hemoglobin levels, an increased erythrocyte sedimentation rate, and an increased prevalence of autoantibody positive results compared to non-SS patients. Salivary scintigraphy showed that the uptake ratio of the submandibular gland in SS patients was decreased significantly. The prevalence of SS in patients with burning mouth symptoms was 4.8%. Therefore, clinicians who treat patients with burning mouth symptoms should evaluate laboratory findings and salivary functions to identify patients with SS.

  20. Heat shock proteins and chronic fatigue in primary Sjögren's syndrome.

    Science.gov (United States)

    Bårdsen, Kjetil; Nilsen, Mari Mæland; Kvaløy, Jan Terje; Norheim, Katrine Brække; Jonsson, Grete; Omdal, Roald

    2016-04-01

    Fatigue occurs frequently in patients with cancer, neurological diseases and chronic inflammatory diseases, but the biological mechanisms that lead to and regulate fatigue are largely unknown. When the innate immune system is activated, heat shock proteins (HSPs) are produced to protect cells. Some extracellular HSPs appear to recognize cellular targets in the brain, and we hypothesize that fatigue may be generated by specific HSPs signalling through neuronal or glial cells in the central nervous system. From a cohort of patients with primary Sjögren's syndrome, 20 patients with high and 20 patients with low fatigue were selected. Fatigue was evaluated with a fatigue visual analogue scale. Plasma concentrations of HSP32, HSP60, HSP72 and HSP90α were measured and analysed to determine if there were associations with the level of fatigue. Plasma concentrations of HSP90α were significantly higher in patients with high fatigue compared with those with low fatigue, and there was a tendency to higher concentrations of HSP72 in patients with high fatigue compared with patients with low fatigue. There were no differences in concentrations of HSP32 and HSP60 between the high- and low-fatigue groups. Thus, extracellular HSPs, particularly HSP90α, may signal fatigue in chronic inflammation. This supports the hypothesis that fatigue is generated by cellular defence mechanisms. © The Author(s) 2016.

  1. Quantitative Proteomics of Parotid Saliva in Primary Sjögren’s Syndrome

    Science.gov (United States)

    Ambatipudi, Kiran S.; Swatkoski, Stephen; Moresco, James J.; Tu, Patricia G.; Coca, Andreea; Anolik, Jennifer H.; Gucek, Marjan; Sanz, Igñacio; Yates, John R.; Melvin, James E.

    2013-01-01

    The diagnosis of primary Sjögren’s syndrome (pSS) is difficult due to the lack of specific laboratory and clinical tests. As an initial step for the global discovery of changes in the abundance of parotid salivary proteins in pSS, a pooled sample was compared to that from healthy control (HC) subjects by multidimensional protein identification technology (MudPIT). A total of 1246 proteins were identified by MudPIT. The abundance of 477 of these proteins did not change, 529 were only detected in either the pSS or HC sample, while 206 of these proteins were significantly up-regulated ≥ 2-fold and 34 were down-regulated ≤ 0.5. Ingenuity Pathway Analyses of differentially expressed proteins identified by MudPIT resulted in the identification of 100 significant pathways. The same samples were quantified in parallel using reversed phase (RP) mass spectrometry. Fifty eight of 71 proteins identified by RP overlapped with MudPIT results. Five proteins were further analyzed by targeted label-free quantification to confirm the similar relative differential expression observed by RP and MudPIT approaches. The present study supports the use of mass spectrometry for global discovery and validation of marker proteins for improved and early diagnosis of pSS. PMID:22888089

  2. A Case of Erythema Annulare Centrifigum with Sjögren Syndrome

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    İlgen Ertam

    2010-03-01

    Full Text Available Erythema annulare centrifigum is a dermatose which is frequently seen in adults. It is characterized by erythematous lesions which spread asymmetrically to periphery and have a collarette desquamation. Although infection, tumor, food allergy, drug reaction can play a role in the aetiology, most of the cases are idiopathic. A forty-nine years old, female patient presented to our clinic with erythematous lesions on both of her lower extremities. Six weeks prior to her referral, she treated with quinine for Sjogren syndrome. She had a diagnosis of granuloma annulare in her personal history. There was no significance in her family history. In dermatologic examination; annular erythematous plaques and collarette desquamation were detected on lower extremities. Histopathologic examination of the lesional biopsy specimen revealed focal spongiosis in the epidermis, dermal oedema, vascular proliferation and perivascular infiltration of lymphocytes, eosinophils and histiocytes. In the laboratory examination; blood count, liver and kidney function tests, sedimentation, C-reactive protein was normal. Rheumatoid factor was 30. Antinuclear antibody was 1/640 granular pattern. A case of erythema annulare centrifigum with Sjögren Syndrome is discussed with the other skin findings of the disease.

  3. Heterogeneity of neutrophil antibodies in patients with primary Sjögren's syndrome.

    Science.gov (United States)

    Lamour, A; Le Corre, R; Pennec, Y L; Cartron, J; Youinou, P

    1995-11-01

    Our aims were to determine the prevalence of neutrophil antibodies in patients with primary Sjögren's syndrome (pSS), identify their target antigen(s), and evaluate their functional significance. Neutrophil antibodies were detected using an indirect immunofluorescence (IIIF) test and an enzyme-linked immunosorbent assay (ELISA), using recombinant human Fc-gamma receptor (Fc gamma RIIIb) as a capture agent. Luminol-dependent chemiluminescence was then measured by an established technique. Antibodies to neutrophils were detected in 30 of 66 patients (45%) and categorized on the basis of positivity for the two assays: IIF+/ELISA+ (group A: five patients), IIF+/ELISA- (group B: five patients), and IFF-/ELISA+ (group C: 20 patients). All positive sera contained antibodies directed to the neutrophil specific Fc gamma RIIIb, and none of them bound to NAnull neutrophils. The titer of neutrophil-reactive antibodies (groups A and B) showed no correlation with the neutrophil count, but these autoantibodies did reduce the cell ability to generate a respiratory burst. Thus, neutrophil antibodies are common in patients with pSS. Their main target appears to be Fc gamma RIII, and this may partly account for the dysfunction in Fc gamma R-mediated clearance by the reticuloendothelial system reported in these patients.

  4. IgG4-related sialadenitis and Sjögren's syndrome.

    Science.gov (United States)

    Fragoulis, G E; Zampeli, E; Moutsopoulos, H M

    2017-03-01

    IgG4-related disease (IgG4-RD) has emerged as a new entity in the last decade. It comprises numerous conditions previously thought to be unrelated. Macroscopically, these diseases cause diffuse organ swelling and formation of pseudotumorous masses. Histopathologically, they are characterized by a lymphoplasmacytic infiltrate with increased IgG4+ plasma cells and storiform fibrosis. Despite rapid progress within the last years, our knowledge on these conditions is still fragmented. To date, more than forty organs have been reported to be included in IgG4-RD, and salivary gland involvement is amongst the most common organs affected [IgG4-related sialadenitis (IgG4-RS)]. Interestingly, IgG4-RS shares commonalities with Sjögren's syndrome (SS), like glandular enlargement, sicca symptoms, arthralgias, hypergammaglobulinemia, hypocomplementemia, and circulating antinuclear antibodies. Nonetheless, they differ in that the incidence of anti-Ro and anti-La reactivity is not frequently found in patients with IgG4-RS, their salivary glands are infiltrated by a large number of IgG4+ plasma cells and IgG4-RS symptoms respond promptly to steroids. The aim of this review was to describe the clinical, serological, histopathological and pathophysiological aspects of IgG4-RS in the context of IgG4-RD and highlight the differences between IgG4-RS and SS. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  7. Influence of STAT4 polymorphism in primary Sjögren's syndrome.

    Science.gov (United States)

    Palomino-Morales, Rogelio J; Diaz-Gallo, Lina-Marcela; Witte, Torsten; Anaya, Juan-Manuel; Martín, Javier

    2010-05-01

    To examine the influence of STAT4 rs7574865 gene polymorphism on patients with primary Sjögren's syndrome (SS). Two different cohorts were studied: 69 patients with primary SS and 296 controls from Colombia and 108 patients with primary SS and 227 controls from Germany. Samples were genotyped for the STAT4 rs7574865 single-nucleotide polymorphism with a predesigned TaqMan single-nucleotide polymorphism genotyping assay. We carried out a metaanalysis of our results combined with data published to date. Although no significant differences were observed in the allele frequencies of STAT4 rs7574865 gene polymorphism between patients and controls in Colombians (p = 0.28, OR 1.24, 95% CI 0.82-1.87) and Germans (p = 0.08, OR 1.40, 95% CI 0.96-2.02), the metaanalysis disclosed a significant effect of the T allele on disease (p = 4.7 x 10(-6), OR 1.40, 95% CI 1.21-1.62). These data reinforce the influence of STAT4 gene on primary SS and as a general autoimmune gene.

  8. Secreted autoantibody repertoires in Sjögren's syndrome and systemic lupus erythematosus: A proteomic approach.

    Science.gov (United States)

    Al Kindi, Mahmood A; Colella, Alex D; Chataway, Tim K; Jackson, Michael W; Wang, Jing J; Gordon, Tom P

    2016-04-01

    The structures of epitopes bound by autoantibodies against RNA-protein complexes have been well-defined over several decades, but little is known of the clonality, immunoglobulin (Ig) variable (V) gene usage and mutational status of the autoantibodies themselves at the level of the secreted (serum) proteome. A novel proteomic workflow is presented based on affinity purification of specific Igs from serum, high-resolution two-dimensional gel electrophoresis, and de novo and database-driven sequencing of V-region proteins by mass spectrometry. Analysis of anti-Ro52/Ro60/La proteomes in primary Sjögren's syndrome (SS) and anti-Sm and anti-ribosomal P proteomes in systemic lupus erythematosus (SLE) has revealed that these antibody responses are dominated by restricted sets of public (shared) clonotypes, consistent with common pathways of production across unrelated individuals. The discovery of shared sets of specific V-region peptides can be exploited for diagnostic biomarkers in targeted mass spectrometry platforms and for tracking and removal of pathogenic clones. Copyright © 2016 Elsevier B.V. All rights reserved.

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

    Science.gov (United States)

    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.

  10. Effect of Lid Debridement-Scaling in Sjögren Syndrome Dry Eye.

    Science.gov (United States)

    Ngo, William; Caffery, Barbara; Srinivasan, Sruthi; Jones, Lyndon W

    2015-09-01

    To evaluate the effect of lid debridement-scaling (LDS) on dry eye signs and symptoms in subjects with Sjögren syndrome (SS). This prospective randomized controlled study enrolled 14 female subjects with SS. Seven subjects were randomized into the treatment group where they were selected to receive LDS; the remainder did not receive LDS and served as control subjects. Lid debridement-scaling was conducted using a stainless steel golf club spud (Hilco Wilson Ophthalmics, Plainville, MA) on both the upper and lower eyelids of both eyes. Outcome variables were assessed before LDS and again 1 month later. The outcome variables were the Ocular Surface Disease Index (OSDI), Symptom Assessment iN Dry Eye (SANDE) visual analog scores, ocular staining (SICCA OSS [Sjögren's International Collaborative Clinical Alliance Ocular Staining Score]), fluorescein tear breakup time (FLBUT), meibomian gland score (MGS), meibomian gland yielding liquid secretions (MGYLS) score, and line of Marx's (LOM) position. Thirteen subjects completed the study. Data from only the right eye were analyzed. For the control group (n = 6; mean [± SD] age, 62.3 [± 11.6] years), the pre-LDS, post-LDS, and significance level (pre-LDS mean [± SD] vs. post-LDS mean [± SD]; p value) were as follows: OSDI (58.3 [± 22.1] vs. 48.3 [± 29.0]; p = 0.051), SANDE (77.4 [± 22.1] vs. 89.6 [± 32.6]; p = 0.20), SICCA OSS (7.0 [± 4.5] vs. 8.2 [± 3.5]; p = 0.25), MGS (1.3 [± 1.5] vs. 1.0 [± 0.9]; p = 0.75), MGYLS (0.3 [± 0.5] vs. 0.0 [± 0.0]; p = 0.50), FLBUT (2.99 [± 1.54] vs. 2.85 [± 1.79]; p = 0.63), and LOM (2.0 [± 0.0] vs. 2.0 [± 0.0]; p = n/a). For the treatment group (n = 7; mean [± SD] age, 58.0 [± 8.1] years), the pre-LDS, post-LDS, and significance level were as follows: OSDI (63.2 [± 13.3] vs. 46.9 [± 19.4]; p = 0.04), SANDE (72.6 [± 17.1] vs. 77.0 [± 28.0]; p = 0.54), SICCA OSS (6.6 [± 2.9] vs. 5.0 [± 3.9]; p = 0.02), MGS (1.0 [± 1.2] vs. 3.1 [± 1.7]; p = 0.01), MGYLS (0.0 [± 0

  11. Tear osmolarity measurements in dry eye related to primary Sjögren's syndrome.

    Science.gov (United States)

    Utine, Canan Asli; Bıçakçıgil, Müge; Yavuz, Sule; Çiftçi, Ferda

    2011-08-01

    To evaluate the tear osmolarity in patients with dry eye syndrome related to primary Sjögren's Syndrome (SS). Twenty eyes of 10 patients with dry eye and primary SS (Group 1) and 20 eyes of 20 subjects who do not have dry eye syndrome (Group 2) were included in this cross-sectional study. In all eyes, ophthalmic examination was performed in the same order: International Ocular Surface Disease Index survey, visual acuity assessment, conjunctival hyperemia scoring, tear osmolarity measurement with TearLab(™) Osmolarity System, tear film break-up time assessment, corneal fluorescein staining scoring, ocular surface Lissamine Green staining scoring, anesthetized Schirmer test. Dry eye severity was graded according to Dry Eye Workshop (DEWS) classification system. Four eyes with grade 1, four eyes with grade 2, seven eyes with grade 3, and five eyes with grade 4 dryness, according to DEWS system, were included. The mean tear osmolarity value was 301.9 ± 11.40 mOsm/L (range: 290-328) in Group 1, and 294.85 ± 8.33 mOsm/L (range: 283-311) in Group 2 (p = 0.03). In Group 1, tear osmolarity values were positively correlated with OSDI scores (r(18) = 0.55, r(2) = 0.31, p = 0.01), DEWS classification grades (r(18) = 0.73, r(2) = 0.54, p dry eye syndrome related to primary SS compared to control subjects, and positively correlated with the severity of dry eye.

  12. Evolving concepts of diagnosis, pathogenesis, and therapy of Sjögren's syndrome.

    Science.gov (United States)

    Fox, R I; Törnwall, J; Maruyama, T; Stern, M

    1998-09-01

    Differences in diagnostic criteria for Sjögren's Syndrome (SS) have led to confusion in the research literature and in clinical practice. A particular challenge is the clinical diagnosis of the patients with sicca symptoms, fibromyalgia, chronic fatigue, vague cognitive defects, and a low titer antinuclear antibody. Until recently, many of these patients would have been classified as primary SS using the European criteria. A suggested revision of the European criteria will require inclusion of anti SS-A antibody or characteristic minor salivary gland biopsy, leading to greater agreement between European and San Diego criteria. Recent studies have emphasized that lacrimal and salivary gland flow involves an entire "functional" unit that includes the mucosal surface (the site of inflammation), efferent nerve signals sent to the midbrain (lacrimatory and salvatory nucleus), efferent neural signals from the brain, and acinal/ductal structures in the gland. Thus, symptoms of dryness or pain can result from interferences with any part of this functional unit. The initiating antigens in SS remain unknown, but immune reactivity against SS-A, SS-B, fodrin, alpha- amylase, and carbonic anhydrase have been demonstrated in patients with established disease. The inflammatory process in the gland releases metalloproteinases that alter the relationship of epithelial cells to their matrix, an interaction that is necessary for glandular function and survival. Therapies for SS remain inadequate. In SS patients with immune-mediated extraglandular manifestation (ie, lung, kidney, skin, nerve), the therapeutic approach is similar to systemic lupus erythematosus, although these therapies have relatively little effect on tear or saliva flow.

  13. Treatment of Sjögren's syndrome-associated dry eye an evidence-based review.

    Science.gov (United States)

    Akpek, Esen Karamursel; Lindsley, Kristina B; Adyanthaya, Rohit S; Swamy, Ramya; Baer, Alan N; McDonnell, Peter J

    2011-07-01

    Outcomes-based review of reported treatment options for patients with dry eye secondary to Sjögren's syndrome (SS). Dry eye affects many individuals worldwide. Significant proportion of patients with dry eye has underlying SS, a progressive autoimmune condition. The few suggested guidelines for the treatment of dry eye are mostly based on severity of symptoms and/or clinical findings rather than on outcomes analysis, and do not differentiate SS from other causes of dry eye. METHODS AND LITERATURE REVIEW: A search strategy was developed to identify prospective, interventional studies of treatments for SS-associated dry eye from electronic databases. Eligible references were restricted to English-language articles published after 1975. These sources were augmented by hand searches of reference lists from accessed articles. Study selection, data extraction, and grading of evidence were completed independently by ≥4 review authors. The searches identified 3559 references as of August 10, 2010. After duplicate review of the titles and abstracts, 245 full-text papers were assessed, 62 of which were relevant for inclusion in the review. In the current literature on SS-associated dry eye, there is a paucity of rigorous clinical trials to support therapy recommendations. Nonetheless, the recommended treatments include topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies. The efficacy of oral secretagogues seems greater in the treatment of oral dryness than ocular dryness. Although oral hydroxychloroquine is commonly prescribed to patients with SS to alleviate fatigue and arthralgias, the literature lacks strong evidence for the efficacy of this treatment for dry eye. Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  14. Myositis in primary Sjögren's syndrome: data from a multicentre cohort.

    Science.gov (United States)

    Colafrancesco, Serena; Priori, Roberta; Gattamelata, Angelica; Picarelli, Giovanna; Minniti, Antonina; Brancatisano, Filippo; D'Amati, Giulia; Giordano, Carla; Cerbelli, Bruna; Maset, Marta; Quartuccio, Luca; Bartoloni, Elena; Carubbi, Francesco; Cipriani, Paola; Baldini, Chiara; Luciano, Nicoletta; De Vita, Salvatore; Gerli, Roberto; Giacomelli, Roberto; Bombardieri, Stefano; Valesini, Guido

    2015-01-01

    In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies. Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected. According to Bohan and Peter's criteria, inflammatory myopathy (IM) was suspected in case of muscular weakness associated with increased creatine-phosphokinase (CPK) or abnormal electromyography (EMG). When performed, muscle biopsies were analysed. In a cohort of 1320 patients, 17 (1.28%) presented muscular weakness [in some cases myalgias (7/17, 41.1%)], accompanied by increased CPK [13/17, (76.4%)] and/or abnormal EMG [13/14, (92.8%)]. Ten out of 17 (58.8%) fulfilled at least three diagnostic criteria for IM. Muscular biopsy was performed in 13/17 (76.4%) cases with histologically confirmed myositis in 6/13 (46.1%) (1"IBM-like"-5"PM-like"). In two "PM-like" cases, several fibres showed a decreased histochemical cytochrome C oxidase (COX) stain. Two biopsies tested "negative", four showed "non-specific" findings. All patients were treated with corticosteroids followed by different DMARDs. Our retrospective analysis shows a prevalence of myositis in pSS lower than previously reported, mainly appearing as an overlapping syndrome. Histological findings confirm the possible presence of an IBM or of a myopathy more similar to PM with a decreased COX activity. Classical immunosuppressants are effective although in most difficult cases IVIg or RTX may be used with benefit.

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

    Science.gov (United States)

    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

  16. Prevalence of Primary Sjögren's Syndrome in a US Population-Based Cohort.

    Science.gov (United States)

    Maciel, Gabriel; Crowson, Cynthia S; Matteson, Eric L; Cornec, Divi

    2017-10-01

    To report the point prevalence of primary Sjögren's syndrome (SS) in the first US population-based study. Cases of all potential primary SS patients living in Olmsted County, Minnesota, on January 1, 2015, were retrieved using Rochester Epidemiology Project resources, and ascertained by manual medical records review. Primary SS cases were defined according to physician diagnosis. The use of diagnostic tests was assessed and the performance of classification criteria was evaluated. The number of prevalent cases in 2015 was also projected based on 1976-2005 incidence data from the same source population. A total of 106 patients with primary SS were included in the study: 86% were female, with a mean ± SD age of 64.6 ± 15.2 years, and a mean ± SD disease duration of 10.5 ± 8.4 years. A majority were anti-SSA positive (75%) and/or anti-SSB positive (58%), but only 22% met American-European Consensus Group or American College of Rheumatology criteria, because the other tests required for disease classification (ocular dryness objective assessment, salivary gland functional or morphologic tests, or salivary gland biopsy) were rarely performed in clinical practice. According to the physician diagnosis, the age- and sex-adjusted prevalence of primary SS was 10.3 per 10,000 inhabitants, but according to classification criteria, this prevalence was only 2.2 per 10,000. The analysis based on previous incidence data projected a similar 2015 prevalence rate of 11.0 per 10,000. The prevalence of primary SS in this geographically well-defined population was estimated to be between 2 and 10 per 10,000 inhabitants. Physicians rarely used tests included in the classification criteria to diagnose the disease in this community setting. © 2016, American College of Rheumatology.

  17. Cognition, depression, fatigue, and quality of life in primary Sjögren's syndrome: correlations.

    Science.gov (United States)

    Koçer, Belgin; Tezcan, Mehmet Engin; Batur, Hale Zeynep; Haznedaroğlu, Şeminur; Göker, Berna; İrkeç, Ceyla; Çetinkaya, Rümeysa

    2016-12-01

    The aim of the present study was to investigate the prevalence and pattern of cognitive dysfunction observed in primary Sjögren's syndrome (PSS) and to examine the relationships between cognitive abilities, depression, fatigue, and quality of life. Thirty-two subjects with PSS were compared with 19 healthy controls on comprehensive neuropsychological, depression, fatigue, health state, and daily-life activities tests. There was low performance in Clock Drawing, COWAT, Paced Auditory Serial Addition Test (PASAT), Colorless Word Reading (Stroop1) and Recognizing Colors (Stroop2) Patterns of STROOP test, SDLT, Auditory-Verbal Learning Test (AVLT), immediate and long-term verbal memory, Benton Judgment of Line Orientation Test (BJLOT), and in all the patterns of RCFT in PSS patients compared to the healthy control group ( p  < .05). It was observed an increased depression frequency and fatigue severity, impairment in health condition, and a decreased quality of life in PSS cases compared to the healthy controls ( p  < .05). All the depression, fatigue severity, and quality of life tests showed a significant positive correlation with each other ( p  < .05). A significant negative correlation between Clock Drawing and SF-36-BP ( p  = .031, r  = -.382) and SF-36-GH ( p  = .027, r  = -.392) was observed. Clock Drawing, PASAT, and AVLT are very useful tests to determine the subclinical and clinical cognitive dysfunction to evaluate attention, information processing speed, executive functions, and short-term and long-term verbal memory in PSS patients. Depression and fatigue may not affect the neuropsychological tests performance.

  18. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. The use of whole saliva in the differential diagnosis of Sjögren's syndrome.

    Science.gov (United States)

    Sreebny, L M; Zhu, W X

    1996-04-01

    Sjögren's Syndrome (SS) is a chronic, multisystem, autoimmune disorder. It is characterized by (1) generalized exocrine gland dysfunction, (2) serologic abnormalities, and (3) organ-system changes. Oral changes are a prominent feature of this disease. Among these are xerostomia and hypofunction of the salivary glands. Given the intimate relationship between SS and the salivary glands, it is reasonable to postulate that whole saliva (WS) contains the stigmata associated with the presence of this disease. But few studies have been conducted on this secretion. Indeed, WS has largely been neglected and ignored by physicians, dentists, and scientists. Objections to its use have included the fact that it is "impure", that it does not adequately represent what is present in the salivary glands, that no standards have been established for its rate of flow, and that findings based on it lack specificity. Yet, it is this secretion which coats and protects the hard and soft oral tissues, enables us to prepare our food for digestion, and assists our speech. This review will demonstrate that there is a uniqueness and constancy to whole saliva and that it may be used to diagnose the presence of SS. "Screening tests", which include several simple-to-perform sialometric, chemical, and microbiologic procedures, may be conducted in doctors' offices to establish the "profile" of an SS patient. Electrophoretic studies may be used to study the nature of the salivary proteins, and an immunologic test, which is performed on WS and utilizes Western Blot Autoantibody Strips (ImmunoVision, Springdale, AR), may be used to establish the definitive diagnosis of SS.

  1. Systemic Lupus Erythematosus and Sjögren's Syndrome Complicated by Conversion Disorder: a Case Report.

    Science.gov (United States)

    Nakamura, Misa; Tanaka, Seiji; Inoue, Tadashi; Maeda, Yasuto; Okumiya, Kiyohito; Esaki, Takuya; Shimomura, G O; Masunaga, Kenji; Nagamitsu, Shinichiro; Yamashita, Yushiro

    2018-05-21

    Conversion disorder (CD) is sometimes accompanied by motor and sensory impairments, such as muscle weakness, paralysis, sensory hypersensitivity, and sensory loss. Sjögren's syndrome (SS) complicates 5-10% of cases of systemic lupus erythematosus (SLE). Patients with SS or SLE present with various neurological symptoms and psychiatric manifestations. When neurological symptoms are present, it is important to distinguish whether the symptoms are caused by a neurological or a mental disorder because the former requires early intensive intervention, such as methylprednisolone pulse therapy (MPT), whereas psychotherapy or antidepressant drugs are recommended for mental disorders. We noticed SS- and SLE-like symptoms just after a diagnosis of idiopathic thrombocytopenic purpura in a 14-year-old girl. At approximately the same time, paralysis started in her lower limbs and subsequently spread to her upper limbs. An examination for neurological symptoms revealed no abnormalities. Because of the conversion reaction between her neurological symptoms and her disease state, CD was suspected as the etiology of her physical symptoms. Nevertheless, because of the progressive nature of the neurological symptoms, MPT was initiated with concurrent administration of intravenous immunoglobulin, but it failed to achieve a good outcome. The patient's symptoms eventually improved after she underwent psychotherapy treatment for a few months. Because early diagnosis of the cause of neurological symptoms accompanying SS and SLE is difficult, it may be necessary to combine the two lines of treatment in the early stages. However, when a mental disorder is suspected, psychotherapy should be started early to minimize the use of unnecessary neurological treatment.

  2. Altered cytokine balance in the tear fluid and conjunctiva of patients with Sjögren's syndrome keratoconjunctivitis sicca.

    Science.gov (United States)

    Pflugfelder, S C; Jones, D; Ji, Z; Afonso, A; Monroy, D

    1999-09-01

    To compare epidermal growth factor (EGF) concentration in tear fluid and levels of inflammatory cytokines in the conjunctival epithelium of patients with Sjögren's syndrome keratoconjunctivitis sicca with those of normal controls. Schirmer 1 tear testing, corneal fluorescein staining and conjunctival impression cytology for quantitation of goblet cell density were performed in ten patients with Sjögren's syndrome-associated keratoconjunctivitis sicca and ten asymptomatic normal controls. ELISA was used to detect the concentration of EGF in tear fluid and interleukin 6 in lysates of conjunctival cytology specimens obtained from all subjects. The levels of RNA transcripts encoding inflammatory cytokines [interleukin 1alpha_(IL-1alpha), interleukin 6 (IL-6), interleukin 8 (IL-8), tumor necrosis factor alpha_(TNF-alpha), and transforming growth factor beta1 (TGF-beta1)] as well as a housekeeping gene (G3PDH) were evaluated in conjunctival cytology specimens taken from all subjects by semiquantitative competitive reverse transcriptase polymerase chain reaction (RT-PCR). Decreased tear fluid EGF concentration was noted in Sjögren's syndrome patients (mean 0.68 +/- 0.59 ng/ml) compared to controls (mean 1.66 +/- 0.45 ng/ml, P = 0.004). Significantly increased levels of IL-1alpha, IL-6, IL-8, TNF-alpha and TGF-beta1 RNA transcripts were found in the conjunctival epithelium of Sjögren's syndrome patients compared to controls (P G3PDH was similar in both groups. The concentration of IL-6 protein was significantly higher in Sjögren's syndrome conjunctiva samples (P = 0.012). Tear EGF concentration correlated with Schirmer 1 scores (rho 0.767, P < 0.001), corneal fluorescein staining scores (rho -0.562, P = 0.01), conjunctival goblet cell density (rho 0.661, P = 0.001) and the levels of IL-1alpha_and IL-8 RNA in the conjunctival epithelium (rho -0.677 and -0.747, respectively, P = 0.001). Both IL-1alpha_and IL-8 RNA in the conjunctival epithelium increased as Schirmer 1

  3. Assessing health-related quality of life in primary Sjögren's syndrome-The PSS-QoL.

    Science.gov (United States)

    Lackner, Angelika; Stradner, Martin H; Hermann, Josef; Unger, Julia; Stamm, Tanja; Graninger, Winfried B; Dejaco, Christian

    2017-11-27

    To develop a questionnaire for the assessment of health-related quality of life (HRQL) in primary Sjögren's syndrome (PSS), and to test its psychometric properties. Based on the concepts of a previous qualitative study, a questionnaire for the assessment of HRQL in PSS (PSS-QoL) was developed. Psychometric testing of PSS-QoL was performed after revising the first draft with feedback of patients (n = 6) and clinicians (n = 4). Convergent construct validity was assessed by correlating the score with the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) and Euro-QoL 5D (EQ-5D). Reliability was examined by asking patients to complete the questionnaire twice 1-2 weeks apart. An English Version of the PSS-QoL was developed by using standard methodology with forward and back translation. Out of the 75 PSS patients, 91% were female, mean (±SD) age was 58.5 ± 12.5 years. PSS-QoL consists of 25 questions and can be divided into two main categories: physical (discomfort and dryness) and psychosocial. The internal consistency of the PSS-QoL revealed a Crohnbach's α of 0.892. Strong and moderate correlations were found between the PSS-QoL and ESSPRI (corr coeff = 0.755) and EQ. 5D-pain/discomfort (corr coeff = 0.531). Reproducibility of the PSS-QoL was high, yielding an ICC of 0.958 (95% CI: 0.926-0.981). The PSS-QoL is the first specific tool for the assessment of patients' HRQL in PSS and showed good psychometric properties. It may serve as a novel patient-reported outcome measure in future clinical studies. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. CLASP/SJ Observations of Rapid Time Variations in the Ly α Emission in a Solar Active Region

    Energy Technology Data Exchange (ETDEWEB)

    Ishikawa, Shin-nosuke [Institute of Space and Astronautical Science, Japan Aerospace Exploration Agency, 3-1-1 Yoshinodai, Chuo-ku, Sagamihara, Kanagawa 252–5210 (Japan); Kubo, Masahito; Katsukawa, Yukio; Kano, Ryouhei; Narukage, Noriyuki; Ishikawa, Ryohko; Bando, Takamasa [National Astronomical Observatory of Japan, 2-21-1 Osawa, Mitaka, Tokyo 181-8588 (Japan); Winebarger, Amy; Kobayashi, Ken [NASA Marshall Space Flight Center, Huntsville, AL 35812 (United States); Trujillo Bueno, Javier [Instituto de Astrofísica de Canarias, E-38205 La Laguna, Tenerife (Spain); Auchère, Frédéric, E-mail: s.ishikawa@solar.isas.jaxa.jp [Institut d’Astrophysique Spatiale, CNRS/Univ. Paris-Sud 11, Bätiment 121, F-91405 Orsay (France)

    2017-09-10

    The Chromospheric Ly α 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 Ly α 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 Ly α emission and the coronal structures observed by Solar Dynamics Observatory/Atmospheric Imaging Assembly (AIA). We compare the Ly α 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 Ly α features without a clear association with the coronal loop footpoints. We find more short (<30 s) temporal variations in the Ly α intensity in the footpoint regions. Those variations did not depend on the temperature of the coronal loops. Therefore, the temporal variations in the Ly α 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 Ly α intensity variations were related to the nanoflares. Waves or jets from the lower layers (lower chromosphere or photosphere) are possible causes for this phenomenon.

  5. 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.

  6. 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...

  7. 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.

  8. [Primary sclerosing cholangitis associated with Sjögren's syndrome, retroperitoneal fibrosis and chronic pancreatitis. Report of a case].

    Science.gov (United States)

    Barreda, F; Contardo, C; León, A; Navarrete, J; Figueroa, R; Attanasio, F

    1989-01-01

    Primary Sclerosing Cholangitis (PSC) is an unusual chronic, cholestatic disease of unknown etiology, more frequently seen in young adults in close relationship with Chronic Ulcerative Colitis. We report the case of a 30 year old woman, coming from the peruvian amazon with PSC associated with Sjögren Syndrome, Chronic Pancreatitis and Retroperitoneal Fibrosis, without colonic involvement. She was treated with external biliary drainage and controlled for 12 months. In this paper, clinical, biochemical, radiological, histological and therapeutic features are reviewed as well as its possible immunologie autoimmune origin.

  9. 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.

  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. Primary Sjögren's syndrome in Moroccan patients: characteristics, fatigue and quality of life.

    Science.gov (United States)

    Ibn Yacoub, Yousra; Rostom, Samira; Laatiris, Assia; Hajjaj-Hassouni, Najia

    2012-09-01

    Our aim was to evaluate fatigue and quality of life (QoL) in Moroccan patients with primary Sjögren's syndrome (PSS) and determine their correlates with disease-related parameters. Fifty-seven consecutive patients with PSS according to the American-European Consensus group (AEGG) criteria were included. Demographic, clinical, biological and immunological characteristics for all patients were collected. Xerostomia was demonstrated by histological grading of lower lip glandular biopsy. A Schirmer test was performed to measure lachrymal flow. Oral, ocular, skin, vaginal and tracheal dryness were evaluated by using a visual analogue scale (VAS). Fatigue was assessed by the Multidimensional assessment of fatigue (MAF) and the QoL by using the generic instrument: SF-36. 90% of our patients were women. The mean age of patients was 53.73 ± 7.69 years, and the mean disease duration was 5.38 ± 4.11 years. The mean oral dryness was 68.38 ± 20.29, and the mean ocular dryness was 51.91 ± 14.03. The mean total score of the MAF was 26.73 ± 8.33, and 87.5% of our patients experienced severe fatigue. Also, physical and mental domains of QoL were altered in a significant way, and the severity of fatigue had a negative impact on SF-36 scores. MAF and SF-36 scores were correlated with the delay of diagnosis, the intensity of xerostomia and the activity of joint involvement. A low socioeconomic and educational level had a negative impact on fatigue scores and QoL. Histological grading of lower lip glandular biopsy, immunological status and the severity of systemic involvement had no correlations with fatigue scores or the alteration of QoL. Patients receiving antidepressant have lesser fatigue and those receiving Methotrexate have better SF-36 scores. In our data, there was a high prevalence of fatigue in Moroccan patients with PSS associated with altered QoL. Severe fatigue and reduced QoL seem to be related to the severity of joint involvement, xerostomia and both educational

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

    Science.gov (United States)

    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.

  15. Role of the IL-12/IL-35 balance in patients with Sjögren syndrome.

    Science.gov (United States)

    Fogel, Olivier; Rivière, Elodie; Seror, Raphaèle; Nocturne, Gaetane; Boudaoud, Saida; Ly, Bineta; Gottenberg, Jacques-Eric; Le Guern, Véronique; Dubost, Jean-Jacques; Nititham, Joanne; Taylor, Kimberly E; Chanson, Philippe; Dieudé, Philippe; Criswell, Lindsey A; Jagla, Bernd; Thai, Alice; Mingueneau, Michael; Mariette, Xavier; Miceli-Richard, Corinne

    2017-09-12

    An interferon signature is involved in the pathogenesis of primary Sjögren syndrome (pSS), but whether the signature is type 1 or type 2 remains controversial. Mouse models and genetic studies suggest the involvement of T H 1 and type 2 interferon pathways. Likewise, polymorphisms of the IL-12A gene (IL12A), which encodes for IL-12p35, have been associated with pSS. The IL-12p35 subunit is shared by 2 heterodimers: IL-12 and IL-35. We sought to confirm genetic association of the IL12A polymorphism and pSS and elucidate involvement of the IL-12/IL-35 balance in patients with pSS by using functional studies. The genetic study involved 673 patients with pSS from 2 French pSS cohorts and 585 healthy French control subjects. Functional studies were performed on sorted monocytes, irrespective of whether they were stimulated. IL12A mRNA expression and IL-12 and IL-35 protein levels were assessed by using quantitative RT-PCR and ELISA and a multiplex kit for IL-35 and IL-12, respectively. We confirmed association of the IL12A rs485497 polymorphism and pSS and found an increased serum protein level of IL-12p70 in patients with pSS carrying the risk allele (P = .016). Serum levels of IL-12p70 were greater in patients than control subjects (P = .0001), especially in patients with more active disease (P = .05); conversely, IL-35 levels were decreased in patients (P = .0001), especially in patients with more active disease (P = .05). In blood cellular subsets both IL12p35 and EBV-induced gene protein 3 (EBI3) mRNAs were detected only in B cells, with a trend toward a lower level among patients with pSS. Our findings emphasize involvement of the IL-12/IL-35 balance in the pathogenesis of pSS. Serum IL-35 levels were associated with low disease activity, in contrast with serum IL-12p70 levels, which were associated with more active disease. Copyright © 2017 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  16. Novel and rare functional genomic variants in multiple autoimmune syndrome and Sjögren's syndrome.

    Science.gov (United States)

    Johar, Angad S; Mastronardi, Claudio; Rojas-Villarraga, Adriana; Patel, Hardip R; Chuah, Aaron; Peng, Kaiman; Higgins, Angela; Milburn, Peter; Palmer, Stephanie; Silva-Lara, Maria Fernanda; Velez, Jorge I; Andrews, Dan; Field, Matthew; Huttley, Gavin; Goodnow, Chris; Anaya, Juan-Manuel; Arcos-Burgos, Mauricio

    2015-06-02

    Multiple autoimmune syndrome (MAS), an extreme phenotype of autoimmune disorders, is a very well suited trait to tackle genomic variants of these conditions. Whole exome sequencing (WES) is a widely used strategy for detection of protein coding and splicing variants associated with inherited diseases. The DNA of eight patients affected by MAS [all of whom presenting with Sjögren's syndrome (SS)], four patients affected by SS alone and 38 unaffected individuals, were subject to WES. Filters to identify novel and rare functional (pathogenic-deleterious) homozygous and/or compound heterozygous variants in these patients and controls were applied. Bioinformatics tools such as the Human gene connectome as well as pathway and network analysis were applied to test overrepresentation of genes harbouring these variants in critical pathways and networks involved in autoimmunity. Eleven novel and rare functional variants were identified in cases but not in controls, harboured in: MACF1, KIAA0754, DUSP12, ICA1, CELA1, LRP1/STAT6, GRIN3B, ANKLE1, TMEM161A, and FKRP. These were subsequently subject to network analysis and their functional relatedness to genes already associated with autoimmunity was evaluated. Notably, the LRP1/STAT6 novel mutation was homozygous in one MAS affected patient and heterozygous in another. LRP1/STAT6 disclosed the strongest plausibility for autoimmunity. LRP1/STAT6 are involved in extracellular and intracellular anti-inflammatory pathways that play key roles in maintaining the homeostasis of the immune system. Further; networks, pathways, and interaction analyses showed that LRP1 is functionally related to the HLA-B and IL10 genes and it has a substantial impact within immunological pathways and/or reaction to bacterial and other foreign proteins (phagocytosis, regulation of phospholipase A2 activity, negative regulation of apoptosis and response to lipopolysaccharides). Further, ICA1 and STAT6 were also closely related to AIRE and IRF5, two very

  17. Subjective and Objective Measures of Dryness Symptoms in Primary Sjögren's Syndrome: Capturing the Discrepancy.

    Science.gov (United States)

    Bezzina, Oriana M; Gallagher, Peter; Mitchell, Sheryl; Bowman, Simon J; Griffiths, Bridget; Hindmarsh, Victoria; Hargreaves, Ben; Price, Elizabeth J; Pease, Colin T; Emery, Paul; Lanyon, Peter; Bombardieri, Michele; Sutcliffe, Nurhan; Pitzalis, Costantino; Hunter, John; Gupta, Monica; McLaren, John; Cooper, Anne M; Regan, Marian; Giles, Ian P; Isenberg, David A; Saravanan, Vadivelu; Coady, David; Dasgupta, Bhaskar; McHugh, Neil J; Young-Min, Steven A; Moots, Robert J; Gendi, Nagui; Akil, Mohammed; MacKay, Kirsten; Ng, W Fai; Robinson, Lucy J

    2017-11-01

    To develop a novel method for capturing the discrepancy between objective tests and subjective dryness symptoms (a sensitivity scale) and to explore predictors of dryness sensitivity. Archive data from the UK Primary Sjögren's Syndrome Registry (n = 688) were used. Patients were classified on a scale from -5 (stoical) to +5 (sensitive) depending on the degree of discrepancy between their objective and subjective symptoms classes. Sensitivity scores were correlated with demographic variables, disease-related factors, and symptoms of pain, fatigue, anxiety, and depression. Patients were on average relatively stoical for both types of dryness symptoms (mean ± SD ocular dryness -0.42 ± 2.2 and -1.24 ± 1.6 oral dryness). Twenty-seven percent of patients were classified as sensitive to ocular dryness and 9% to oral dryness. Hierarchical regression analyses identified the strongest predictor of ocular dryness sensitivity to be self-reported pain and that of oral dryness sensitivity to be self-reported fatigue. Ocular and oral dryness sensitivity can be classified on a continuous scale. The 2 symptom types are predicted by different variables. A large number of factors remain to be explored that may impact symptom sensitivity in primary Sjögren's syndrome, and the proposed method could be used to identify relatively sensitive and stoical patients for future studies. © 2016, The Authors. Arthritis Care & Research published by Wiley Periodicals, Inc. on behalf of American College of Rheumatology.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome

    Directory of Open Access Journals (Sweden)

    João Peres

    2017-01-01

    Full Text Available A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids. A simultaneous improvement of the cognitive abilities allowed for the detection of sensory ataxia of the lower limbs. Sensory ganglionopathy was then detected with electrophysiological studies. A diagnosis of Sjögren syndrome was suspected and confirmed by salivary gland scintigraphy, Schirmer’s test, and submaxillary gland biopsy. We report a case of Sjögren syndrome associated with central and peripheral nervous system involvement, without sicca symptoms preceding the neurological clinical picture. The coexistence of ganglionopathy and a favourable response to immunosuppression are key features that can lead to the correct diagnosis in cases with atypical CNS symptoms, mimicking a rapidly progressive dementia.

  20. High-resolution optical coherence tomography, autofluorescence, and infrared reflectance imaging in Sjögren reticular dystrophy.

    Science.gov (United States)

    Schauwvlieghe, Pieter-Paul; Torre, Kara Della; Coppieters, Frauke; Van Hoey, Anneleen; De Baere, Elfride; De Zaeytijd, Julie; Leroy, Bart P; Brodie, Scott E

    2013-01-01

    To describe the phenotype of three cases of Sjögren reticular dystrophy in detail, including high-resolution optical coherence tomography, autofluorescence imaging, and near-infrared reflectance imaging. Two unrelated teenagers were independently referred for ophthalmologic evaluation. Both underwent a full ophthalmologic workup, including electrophysiologic and extensive imaging with spectral-domain optical coherence tomography, autofluorescence imaging, and near-infrared reflectance imaging. In addition, mutation screening of ABCA4, PRPH2, and the mitochondrial tRNA gene was performed in Patient 1. Subsequently, the teenage sister of Patient 2 was examined. Strikingly similar phenotypes were present in these three patients. Fundoscopy showed bilateral foveal pigment alterations, and a lobular network of deep retinal, pigmented deposits throughout the posterior pole, tapering toward the midperiphery, with relative sparing of the immediate perifoveal macula and peripapillary area. This network is mildly to moderately hyperautofluorescent on autofluorescence and bright on near-infrared reflectance imaging. Optical coherence tomography showed abnormalities of the retinal pigment epithelium-Bruch membrane complex, photoreceptor outer segments, and photoreceptor inner/outer segment interface. The results of retinal function test were entirely normal. No molecular cause was detected in Patient 1. Imaging suggested that the lobular network of deep retinal deposits in Sjögren reticular dystrophy is the result of accumulation of both pigment and lipofuscin between photoreceptors and retinal pigment epithelium, as well as within the retinal pigment epithelium.

  1. Acute renal failure due to mesangial proliferative glomerulonephritis in a pregnant woman with primary Sjögren's syndrome.

    Science.gov (United States)

    Adam, Fatma Ulku; Torun, Dilek; Bolat, Filiz; Zumrutdal, Aysegul; Sezer, Siren; Ozdemir, Fatma Nurhan

    2006-02-01

    The most common form of renal involvement in Sjögren's syndrome (SS) is tubulointerstitial nephritis. Renal dysfunction is usually mild and subclinical. Glomerulonephritis (GMN) is rare in patients with SS. We report a 28-year-old multigravida patient with primary Sjögren's syndrome (pSS) and associated manifestations, who presented with acute renal failure in the 20th week of her fifth pregnancy. The complaints and clinical findings, positive Schirmer's test, findings of dry eye on ophthalmologic examination, and the salivary gland biopsy were compatible with SS. The patient exhibited no other clinical or laboratory findings indicative of other collagenous disease and/or rheumatoid arthritis. She refused renal biopsy, hesitating for fear of fetal loss; thus, based on the clinical and laboratory findings indicating rapidly progressive GMN and vasculitis, prednisolone, plasmapheresis, and one dose of cyclophosphamide were administered during the pregnancy. Hemodialysis five times weekly was performed. At the 28th week of gestation, she underwent a cesarean section due to early rupture of membranes and fetal distress. A healthy male boy was delivered. The renal biopsy performed 2 weeks after labor revealed mesangial proliferative glomerulonephritis. After the fourth cyclophosphamide treatment, her urinary output increased and she was discharged from the hemodialysis program. She remains in follow-up at our outpatient clinic free of hemodialysis for 4 months. This is the first report of mesangial proliferative GMN requiring dialysis in a pregnant pSS patient that has featured good maternal and fetal outcomes.

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

    Science.gov (United States)

    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.

  3. 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.

  4. 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 (Poral symptoms (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.

  5. Severe optic neuritis in a patient with combined neuromyelitis optica spectrum disease and primary Sjögren’s syndrome: a case report

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    Tan Petrina

    2012-11-01

    Full Text Available Abstract Introduction Optic neuritis, although uncommon, can be the initial presentation of Sjögren’s syndrome. Coexisting Sjögren’s syndrome has also been reported with neuromyelitis optica spectrum disorder. This case report highlights the association between the two diseases and the importance of rheumatological and neurological evaluations in patients with such diagnoses. Distinction of neuromyelitis optica with coexisting connective tissue disease has both prognostic and therapeutic significance for the patient. Case presentation We report a case of a 56-year-old Chinese woman who presented with bilateral asymmetric visual loss secondary to optic neuritis. She was subsequently found to be seropositive for neuromyelitis optica immunoglobulin G (NMO-IgG (anti-aquaporin-4 antibody and was diagnosed with neuromyelitis optica spectrum disorder. She also fulfilled the international criteria for Sjögren’s syndrome. Despite initial high dose immunosuppressive therapy, she failed to regain vision in one eye. Conclusion Patients presenting with optic neuritis and severe visual loss should be screened for neuromyelitis optica and treated appropriately. Neuromyelitis optica has been associated with systemic autoimmune diseases, in particular Sjögren’s syndrome, and current evidence indicates that they are two distinct entities. We recommend that both diagnoses be considered in cases of optic neuritis with severe visual loss.

  6. Autoantibodies to alfa-fodrin in patients with Hashimoto thyroiditis and Sjögren's syndrome: possible markers for a common secretory disorder.

    Science.gov (United States)

    Szanto, Antonia; Csipo, Istvan; Horvath, Ildiko; Biro, Edit; Szodoray, Peter; Zeher, Margit

    2008-09-01

    Presence of autoantibodies to alfa-fodrin was investigated in patients with Sjögren's syndrome (n = 61), Hashimoto thyroiditis (n = 27), Sjögren's syndrome associated with Hashimoto thyroiditis (n = 31) and in healthy persons (n = 77). In each group, level of alfa-fodrin antibodies was higher than in the controls. There was no significant difference in their presence either between patients with Hashimoto thyroiditis with or without Sjögren's syndrome, or-in IgA isotype-between Sjögren's and Hashimoto thyroiditis patients. Correlation was found between the level of IgG alfa-fodrin and anti-thyroglobulin antibodies. Based on these findings, fodrin can be associated with both endocrine and exocrine glandular secretion. Antibodies to alfa-fodrin might have a role in the pathogenesis of Hashimoto thyroiditis concerning the "final common effectory pathway", secretion. Alfa-fodrin antibodies can be good markers of secretory disorders. Assessment of these autoantibodies might help the diagnosis and follow-up of patients with impaired secretory capability of not only autoimmune origin.

  7. Immunization with 60 kD Ro peptide produces different stages of preclinical autoimmunity in a Sjögren's syndrome model among multiple strains of inbred mice.

    Science.gov (United States)

    Kurien, B T; Dsouza, A; Igoe, A; Lee, Y J; Maier-Moore, J S; Gordon, T; Jackson, M; Scofield, R H

    2013-07-01

    Sjögren's syndrome is a chronic illness manifested characteristically by immune injury to the salivary and lacrimal glands, resulting in dry mouth/eyes. Anti-Ro [Sjögren's syndrome antigen A (SSA)] and anti-La [Sjögren's syndrome antigen B (SSB)] autoantibodies are found frequently in Sjögren's subjects as well as in individuals who will go on to develop the disease. Immunization of BALB/c mice with Ro60 peptides results in epitope spreading with anti-Ro and anti-La along with lymphocyte infiltration of salivary glands similar to human Sjögren's. In addition, these animals have poor salivary function/low saliva volume. In this study, we examined whether Ro-peptide immunization produces a Sjögren's-like illness in other strains of mice. BALB/c, DBA-2, PL/J, SJL/J and C57BL/6 mice were immunized with Ro60 peptide-274. Sera from these mice were studied by immunoblot and enzyme-linked immunosorbent assay for autoantibodies. Timed salivary flow was determined after pharmacological stimulation, and salivary glands were examined pathologically. We found that SJL/J mice had no immune response to the peptide from Ro60, while C57BL/6 mice produced antibodies that bound the peptide but had no epitope spreading. PL/J mice had epitope spreading to other structures of Ro60 as well as to La, but like C57BL/6 and SJL/J had no salivary gland lymphocytic infiltration and no decrement of salivary function. DBA-2 and BALB/c mice had infiltration but only BALB/c had decreased salivary function. The immunological processes leading to a Sjögren's-like illness after Ro-peptide immunization were interrupted in a stepwise fashion in these differing mice strains. These data suggest that this is a model of preclinical disease with genetic control for epitope spreading, lymphocytic infiltration and glandular dysfunction. Published 2013. This article is a U.S. Government work and is in the public domain in the USA.

  8. Genes involved in degradation of para-nitrophenol are differentially arranged in form of non-contiguous gene clusters in Burkholderia sp. strain SJ98.

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    Surendra Vikram

    Full Text Available Biodegradation of para-Nitrophenol (PNP proceeds via two distinct pathways, having 1,2,3-benzenetriol (BT and hydroquinone (HQ as their respective terminal aromatic intermediates. Genes involved in these pathways have already been studied in different PNP degrading bacteria. Burkholderia sp. strain SJ98 degrades PNP via both the pathways. Earlier, we have sequenced and analyzed a ~41 kb fragment from the genomic library of strain SJ98. This DNA fragment was found to harbor all the lower pathway genes; however, genes responsible for the initial transformation of PNP could not be identified within this fragment. Now, we have sequenced and annotated the whole genome of strain SJ98 and found two ORFs (viz., pnpA and pnpB showing maximum identity at amino acid level with p-nitrophenol 4-monooxygenase (PnpM and p-benzoquinone reductase (BqR. Unlike the other PNP gene clusters reported earlier in different bacteria, these two ORFs in SJ98 genome are physically separated from the other genes of PNP degradation pathway. In order to ascertain the identity of ORFs pnpA and pnpB, we have performed in-vitro assays using recombinant proteins heterologously expressed and purified to homogeneity. Purified PnpA was found to be a functional PnpM and transformed PNP into benzoquinone (BQ, while PnpB was found to be a functional BqR which catalyzed the transformation of BQ into hydroquinone (HQ. Noticeably, PnpM from strain SJ98 could also transform a number of PNP analogues. Based on the above observations, we propose that the genes for PNP degradation in strain SJ98 are arranged differentially in form of non-contiguous gene clusters. This is the first report for such arrangement for gene clusters involved in PNP degradation. Therefore, we propose that PNP degradation in strain SJ98 could be an important model system for further studies on differential evolution of PNP degradation functions.

  9. [Applicability of syalometry and other instruments to evaluate xerostomia and xerophtalmia in a Sjögren's Syndrome outpatient clinic].

    Science.gov (United States)

    Barcelos, Filipe; Patto, José Vaz; Parente, Manuela; Medeiros, Dina; Sousa, Miguel; Figueiredo, Rui; Miguel, Cláudia; Teixeira, Ana

    2009-01-01

    To evaluate the applicability and utility of unstimulated syalometry and instruments of evaluation of sicca complaints in a Sjögren's syndrome outpatient clinic. We performed unstimulated syalometry to 45 consecutive Primary Sjögren's Syndrome patients (PSS) and 21 healthy asymptomatic individuals age and sex-matched. PSS patients were further evaluated with Schirmer's test. We applied 3 published questionnaires to PSS patients: Xerostomia Inventory (XI), Oral Health Impact Profile-short form (OHIP) and Ocular Surface Disease Index (OSDI), and correlated the results with syalometry and Schirmer's test. Statistical analysis was performed with SPSS (Mann-Whitney U-test and Spearman's correlation). Salivary flux was significantly lower in PSS patients, as compared to controls (0.08+/-0.01 ml/min versus 0.38+/-0.25 ml/min, p=0.000), and decreased with age. Syalometry didn't correlate with Schirmer's test. OHIP scores (mean 26.8 points, ranging from 2 to 43 for a maximum of 56 points) didn't correlate with syalometry neither with Schirmer's test, but showed an association with the XI (p<0.0005) and OSDI (p<0.0005) tests. The XI questionnaire (mean 28.4 points, ranging from 11 to 41 for a maximum of 44 points) correlated with syalometry (p=0.018), with the OHIP questionary (p<0.0005) and with the OSDI scale (p=0.004), although it didn't correlate with Schirmer's test. OSDI scores (mean 56.5 points, ranging from 7 to 90 for a maximum of 100 points) didn't correlate with Schirmer's test neither with syalometry, but associated with the XI (p=0.004) and OHIP (p<0.0005) scales. Unstimulated syalometry is useful in the evaluation of patients suspected of suffering from Sjögren's syndrome, since it can confirm salivary hypofunction in a quick and cheap manner, allowing to differentiate between healthy individuals and patients. In a specialized clinic, the immediate availability of a salivary functional test is important in the classification of PSS or sicca syndrome. The

  10. 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...... the amount of MUC5B (mRNA and protein) as well as sulfation levels in salivary glands of patients with normal or altered unstimulated salivary flow. Localisation of MUC5B and sulfated MUC5B, as well as total levels sulfated groups were determined and compared with acini basal lamina disorganisation. PATIENTS...... AND METHODS: In all, 18 patients with normal or altered unstimulated salivary flow and 16 controls were studied. MUC5B mRNA and protein were evaluated in salivary glands by semiquantitative RT-PCR and Western blot analysis. MUC5B sulfation was determined by Western blotting. MUC5B and sulfo-Lewis(a) antigen...

  11. Sjögren's syndrome associated with protein losing gastroenteropathy manifested by intestinal lymphangiectasia successfully treated with prednisolone and hydroxychloroquine.

    Science.gov (United States)

    Liao, C-Y; Chien, S-T; Wang, C-C; Chen, I-H; Chiu, H-W; Liu, M-Y; Lin, C-H; Ben, R-J; Tsai, M-K

    2015-12-01

    Protein-losing gastroenteropathy (PLGE), a rare manifestation of primary Sjögren's syndrome (SS), is characterized by profound edema and severe hypoalbuminemia secondary to excessive serum protein loss from the gastrointestinal tract and is clinically indistinguishable from nephrotic syndrome. We report a case of a 30-year-old Taiwanese woman with PLGE-associated SS. In addition to a positive Schirmer's test, she had eye-dryness, thirst, and high levels of anti-SSA antibodies, fulfilling SS criteria. PLGE diagnosis was highly appropriate given the clinical profile of hypoalbuminemia, hypercholesterolemia, pleural effusion, and ascites, with absent cardiac, hepatic, or renal disease. We were unable to perform technetium-99 m-labeled human serum albumin scintigraphy ((99m)Tc-HAS). However, the patient's edema and albumin level improved dramatically in response to a 3-month regime of oral prednisolone followed by oral hydroxychloroquine. © The Author(s) 2015.

  12. 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.

  13. Effects of hydroxychloroquine on symptomatic improvement in primary Sjögren syndrome: the JOQUER randomized clinical trial.

    Science.gov (United States)

    Gottenberg, Jacques-Eric; Ravaud, Philippe; Puéchal, Xavier; Le Guern, Véronique; Sibilia, Jean; Goeb, Vincent; Larroche, Claire; Dubost, Jean-Jacques; Rist, Stéphanie; Saraux, Alain; Devauchelle-Pensec, Valérie; Morel, Jacques; Hayem, Gilles; Hatron, Pierre; Perdriger, Aleth; Sene, Damien; Zarnitsky, Charles; Batouche, Djilali; Furlan, Valérie; Benessiano, Joelle; Perrodeau, Elodie; Seror, Raphaele; Mariette, Xavier

    2014-07-16

    Primary Sjögren syndrome is a systemic autoimmune disease characterized by mouth and eye dryness, pain, and fatigue. Hydroxychloroquine is the most frequently prescribed immunosuppressant for the syndrome. However, evidence regarding its efficacy is limited. To evaluate the efficacy of hydroxychloroquine for the main symptoms of primary Sjögren syndrome: dryness, pain, and fatigue. From April 2008 to May 2011, 120 patients with primary Sjögren syndrome according to American-European Consensus Group Criteria from 15 university hospitals in France were randomized in a double-blind, parallel-group, placebo-controlled trial. Participants were assessed at baseline, week 12, week 24 (primary outcome), and week 48. The last follow-up date for the last patient was May 15, 2012. Patients were randomized (1:1) to receive hydroxychloroquine (400 mg/d) or placebo until week 24. All patients were prescribed hydroxychloroquine between weeks 24 and 48. The primary end point was the proportion of patients with a 30% or greater reduction between weeks 0 and 24 in scores on 2 of 3 numeric analog scales (from 0 [best] to 10 [worst]) evaluating dryness, pain, and fatigue. At 24 weeks, the proportion of patients meeting the primary end point was 17.9% (10/56) in the hydroxychloroquine group and 17.2% (11/64) in the placebo group (odds ratio, 1.01; 95% CI, 0.37-2.78; P = .98). Between weeks 0 and 24, the mean (SD) numeric analog scale score for dryness changed from 6.38 (2.14) to 5.85 (2.57) in the placebo group and 6.53 (1.97) to 6.22 (1.87) in the hydroxychloroquine group. The mean (SD) numeric analog scale score for pain changed from 4.92 (2.94) to 5.08 (2.48) in the placebo group and 5.09 (3.06) to 4.59 (2.90) in the hydroxychloroquine group. The mean (SD) numeric analog scale for fatigue changed from 6.26 (2.27) to 5.72 (2.38) in the placebo group and 6.00 (2.52) to 5.94 (2.40) in the hydroxychloroquine group. All but 1 patient in the hydroxychloroquine group had detectable

  14. Is it Sjögren's syndrome or burning mouth syndrome? Distinct pathoses with similar oral symptoms.

    Science.gov (United States)

    Aljanobi, Hawra; Sabharwal, Amarpreet; Krishnakumar, Bralavan; Kramer, Jill M

    2017-04-01

    Sjögren's syndrome (SS) and burning mouth syndrome (BMS) typically occur in postmenopausal women. Although these conditions have significantly different etiopathogeneses, patients with SS or BMS often present with analogous oral complaints. The similarities between the two conditions have led to considerable confusion on the part of medical and dental practitioners, and those with BMS or SS often wait years to receive a diagnosis. Therefore, it is imperative for clinicians to understand the characteristic subjective and objective features of each disease and how these can be used to distinguish them. This review will discuss the proposed etiology, clinical manifestations, histopathology, diagnostic criteria, and patient management of SS and BMS. We also identify key differences between the two pathoses that aid in establishing the correct diagnosis. Recognition of the defining features of each condition will lead to reduced time to diagnosis and improved patient management for these poorly understood conditions. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. 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.

  16. 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

  17. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  19. 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 titre 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. © 2018 Nordic Association for the Publication of BCPT (former Nordic Pharmacological Society).

  20. 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

  1. Streptococcus pneumoniae bacteraemia due to parotitis in a patient with systemic sclerosis and secondary Sjögren's syndrome.

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    Yii, Irene Yuen Lin; Tan, Jamie Bee Xian; Fong, Warren Weng Seng

    2016-10-01

    Invasive pneumococcal disease is an uncommon and notifiable disease in Singapore. It is often associated with significant morbidity and mortality. We report a rare case of invasive pneumococcal bacteraemia due to parotitis in a patient with systemic sclerosis and secondary Sjögren's syndrome. We also present a retrospective review of Streptococcus pneumoniae bacteraemia cases in Singapore General Hospital from January 2011 to April 2016. A 59-year-old Malay lady with a history of systemic sclerosis with secondary Sjögren's syndrome presented with fever and left parotid gland swelling. Clinical examination revealed poor salivary pooling and left parotid swelling without fluctuance. Ultrasound of the left parotid gland confirmed acute parotitis without evidence of abscess or sialolithiasis. Blood cultures were positive for S. pneumoniae . She was diagnosed to have invasive pneumococcal bacteraemia secondary to acute parotitis, and treated with intravenous benzylpenicillin with clearance of bacteraemia after 3 days. Upon discharge, her antibiotics were changed to intravenous ceftriaxone to facilitate outpatient parenteral antibiotic therapy for another 2 weeks. She responded favourably to antibiotics at follow-up, with no complications from the bacteraemia. A review of the microbiological records of the Singapore General Hospital revealed 116 cases of pneumococcal bacteraemia, most (80.3 %) of which were due to pneumonia. None were due to parotitis. S. pneumoniae parotitis and subsequent bacteraemia is rare. Prompt recognition of the disease and appropriate use of antibiotics are important. This case highlights that close communication between healthcare workers (microbiologist, rheumatologist and infectious disease specialist) is essential in ensuring good clinical outcomes in patients with a potentially fatal disease.

  2. 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.

  3. 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

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

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    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.

  5. Neuropsychological, neuroimage and psychiatric aspects of primary Sjögren's syndrome Aspectos neuropsicológicos, psiquiátricos e de neuroimagem da síndrome de Sjögren primária

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    Gilberto N.O. Brito

    2002-03-01

    Full Text Available We report a case of a 49-year-old woman diagnosed with primary Sjögrens Syndrome (pSS who was submitted to extensive neuropsychobiological assessment. Examination revealed a Wechsler Adult Intelligence Scale-Revised (WAIS-R Full Scale IQ of 97 with no Verbal/Performance IQ discrepancy and performance below estimated premorbid levels on arithmetic skills, visual tracking, naming and delayed paired associate learning/memory. CT scans of the brain were normal. However, there were subcortical hyperintensities on MRI and left parieto-temporal hypoperfusion on SPECT. Neuropsychological impairment is consistent with the pattern of neuroimage findings. We hypothesize that the pathophysiological mechanisms of pSS involve direct immune attack on neurons in addition to indirect effects through small-vessel angiopathy and thereby induce natural fracture lines in behavior according to location in the central nervous system.No presente estudo, relatamos o caso de uma mulher de 49 anos de idade com o diagnóstico de síndrome de Sjögren primária (pSS submetida à extensa avaliação neuropsicobiológica. O exame revelou QI Global de 97 na Escala de Avaliação da Inteligência Adulta de Wechsler sem discrepância significativa entre os QIs Verbal e Performance, além de desempenho abaixo da estimativa do nível pré-mórbido na aritmética, atenção visual, nomeação e aprendizado/memória de pares de palavras. A tomografia computadorizada do cérebro revelou-se normal. Entretanto, a ressonância magnética mostrou hiperintensidades subcorticais e a tomografia por emissão única de fotons (SPECT revelou hipo-perfusão parieto-temporal esquerda. O perfil do desempenho neuropsicológico é compatível com o padrão de achados da neuroimagem. Pode-se hipotetizar que os mecanismos fisiopatológicos da pSS envolvem ataque imune direto aos neurônios e efeitos indiretos via angiopatia de pequenos vasos, assim induzindo linhas naturais de fratura no

  6. 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

  7. 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.

  8. 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...

  9. Severe Health-Related Quality of Life Impairment in Active Primary Sjögren's Syndrome and Patient-Reported Outcomes: Data From a Large Therapeutic Trial.

    Science.gov (United States)

    Cornec, Divi; Devauchelle-Pensec, Valérie; Mariette, Xavier; Jousse-Joulin, Sandrine; Berthelot, Jean-Marie; Perdriger, Aleth; Puéchal, Xavier; Le Guern, Véronique; Sibilia, Jean; Gottenberg, Jacques-Eric; Chiche, Laurent; Hachulla, Eric; Yves Hatron, Pierre; Goeb, Vincent; Hayem, Gilles; Morel, Jacques; Zarnitsky, Charles; Dubost, Jean Jacques; Saliou, Philippe; Pers, Jacques Olivier; Seror, Raphaèle; Saraux, Alain

    2017-04-01

    To identify the principal determinants of health-related quality of life (HRQOL) impairment in patients with active primary Sjögren's syndrome (SS) participating in a large therapeutic trial, Tolerance and Efficacy of Rituximab in Primary Sjögren's Syndrome (TEARS). At the inclusion visit for the TEARS trial, 120 patients with active primary SS completed the Short Form 36 health survey (SF-36), a validated HRQOL assessment tool. Univariate then multivariate linear regression analyses were used to assess associations linking SF-36 physical and mental components to demographic data, patient-reported outcomes (symptom intensity assessments for dryness, pain, and fatigue, including the European League Against Rheumatism [EULAR] Sjögren's Syndrome Patient Reported Index [ESSPRI]), objective measures of dryness and autoimmunity, and physician evaluation of systemic activity (using the EULAR Sjögren's Syndrome Disease Activity Index [ESSDAI]). SF-36 scores indicated marked HRQOL impairments in our population with active primary SS. Approximately one-third of the patients had low, moderate, and high systemic activity according to the ESSDAI. ESSPRI and ESSDAI scores were moderately but significantly correlated. The factors most strongly associated with HRQOL impairment were patient-reported symptoms, best assessed using the ESSPRI, with pain and ocular dryness intensity showing independent associations with HRQOL. Conversely, systemic activity level was not associated with HRQOL impairment in multivariate analyses, even in the patient subset with ESSDAI values indicating moderate-to-high systemic activity. The cardinal symptoms of primary SS (dryness, pain, and fatigue, best assessed using the ESSPRI) are stronger predictors of HRQOL impairment than systemic involvement (assessed by the ESSDAI) and should be used as end points in future therapeutic trials focusing on patients' well-being. New consensual and data-driven response criteria are needed for primary SS

  10. 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.

  11. 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.

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

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    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.

  13. pH salivary analysis of subjects suffering from Sjögren's syndrome and laryngopharyngeal reflux.

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    Corvo, Marco Antonio Dos Anjos; Eckley, Claudia Alessandra; Liquidato, Bianca Maria; Castilho, Gustavo Leão; Arruda, Cibelle Nunes de

    2012-02-01

    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. 19 patients with SS plus LPR, and 12 healthy controls had their saliva studied prospectively for volume and pH. Two salivary samples were obtained from each participant: whole unstimulated saliva(WUS) and whole stimulated saliva(WSS) while chewing parafilm M®. All the participants were females. Mean age was 60 years (study group) and 44 years (control). LPR was diagnosed on all 19 subjects. The mean pH of WUS was 7.53 (SS) and 7.57 (controls), raising to 7.87 and 7.83 respectively after stimulation. The mean salivary volume of patients with SS was 1.27 mL (WUS) and 3.78 mL (WSS), whereas controls had a significantly higher salivary volume both before and after stimuli. A very high prevalence of LPR was found in patients with SS, which is probably caused by a uniform drop in salivary volume and all its contents, rather than a specific deficiency in its components, as shown previously in patients without SS.

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

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    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.

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

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    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.

  16. Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu

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    Diogo C. Carvalho

    2014-06-01

    Full Text Available The relationship between Sjögren’s syndrome (SS and neuromyelitis optica spectrum disorder (NMOSD is not completely understood. We report two patients with both conditions and review 47 other previously reported cases meeting currently accepted diagnostic criteria, from 17 articles extracted from PubMed. Out of 44 patients whose gender was informed, 42 were females. Mean age at onset of neurological manifestation was 36.2 years (10-74. Serum anti-AQP4-IgG was positive in 32 patients, borderline in 1, and negative in 4. Our Case 1 was seronegative for AQP4-IgG and had no non-organ-specific autoantibodies other than anti-SSB antibodies. Our Case 2 had serum anti-AQP4, anti-SSA/SSB, anti-thyreoglobulin and anti-acethylcholine-receptor antibodies, as well as clinical hypothyreoidism, but no evidence of myasthenia gravis. Our Cases and others, as previously reported in literature, with similar heterogeneous autoimmune response to aquaporin-4, suggest that SS and NMO co-exist in a common autoimmune milieu which is not dependent on aquaporin-4 autoimmunity.

  17. Sjögren’s syndrome versus IgG4-related diseases – classification difficulties and treatment progress

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    Anna Nowakowska-Płaza

    2014-09-01

    Full Text Available Sjögren’s syndrome (SS is a chronic autoimmune disorder characterized by lymphocytic infiltration in exocrine glands mainly salivary and lacrimal which affects impairment of their functions. Some patients develop extraglandular symptoms such as chronic fatigue, arthralgia, or lung, renal, central or peripheral nervous system involvement. Recent decades have brought understanding of some pathogenetic mechanisms and offered new therapeutic options by depleting B cells. Furthermore, the American College of Rheumatology proposed a new set of classification criteria based on objective symptoms. IgG4-related diseases are new nosological entities. The clinical course similarities of SS to Mikulicz’s disease (a subtype of IgG4-related disease result in diagnostic difficulties. Typical conditions of them are: an increased IgG4 level and infiltrations of parenchymal organs by plasmatic cells. This review summarizes classification difficulties, pathogenesis and treatment strategies of SS and IgG4-related diseases.

  18. The Core Pattern Analysis on Chinese Herbal Medicine for Sjögren's syndrome: A Nationwide Population-Based Study

    Science.gov (United States)

    Chang, Ching-Mao; Chu, Hsueh-Ting; Wei, Yau-Huei; Chen, Fang-Pey; Wang, Shengwen; Wu, Po-Chang; Yen, Hung-Rong; Chen, Tzeng-Ji; Chang, Hen-Hong

    2015-01-01

    This large-scale survey aimed to evaluate frequencies and patterns of Chinese herbal medicine (CHM) used for Sjögren's syndrome (SS) in Taiwan by analyzing the National Health Insurance Research Database (NHIRD) for cases in which CHM was used as an alternative therapy to Western medicine for improving patients' discomforts. We analyzed cases of SS principal diagnosis (ICD-9:710.2) with a catastrophic illness certificate (CIC) in traditional Chinese medicine (TCM) outpatient clinics from three cohorts of the Longitudinal Health Insurance Database (LHID) in the NHIRD between 2002 and 2011. CHM prescription patterns for SS were evaluated from claimed visitation files and corresponding prescription files. There were 15,914 SS patients with CIC (SS/CIC), and we found only 130 SS/CIC cases visiting TCM clinics in LHID2000, 133 in LHID2005, and 126 in LHID2010. After removing duplicate data, 366 SS/CIC and 4,867 visits were analyzed. The 50–59 year age group showed the highest ratio (29.51%) in both women and men. “Qi-Ju-Di-Huang-Wan” and “Xuan-Shen” (Scrophularia ningpoensis Hemsl.) was the most commonly used formula and single herb, respectively. “Qi-Ju-Di-Huang-Wan, Gan-Lu-Yin, Xuan-Shen, Mai-Men-Dong (Ophiopogon japonicus (L. f.) Ker-Gawl.), and Sheng-Di-Huang (raw Rehmannia glutinosa Libosch)” were the core pattern prescriptions in treating SS/CIC. PMID:25923413

  19. Activation of IFN pathways and plasmacytoid dendritic cell recruitment in target organs of primary Sjögren's syndrome.

    Science.gov (United States)

    Gottenberg, Jacques-Eric; Cagnard, Nicolas; Lucchesi, Carlo; Letourneur, Franck; Mistou, Sylvie; Lazure, Thierry; Jacques, Sebastien; Ba, Nathalie; Ittah, Marc; Lepajolec, Christine; Labetoulle, Marc; Ardizzone, Marc; Sibilia, Jean; Fournier, Catherine; Chiocchia, Gilles; Mariette, Xavier

    2006-02-21

    Gene expression analysis of target organs might help provide new insights into the pathogenesis of autoimmune diseases. We used global gene expression profiling of minor salivary glands to identify patterns of gene expression in patients with primary Sjögren's syndrome (pSS), a common and prototypic systemic autoimmune disease. Gene expression analysis allowed for differentiating most patients with pSS from controls. The expression of 23 genes in the IFN pathways, including two Toll-like receptors (TLR8 and TLR9), was significantly different between patients and controls. Furthermore, the increased expression of IFN-inducible genes, BAFF and IFN-induced transmembrane protein 1, was also demonstrated in ocular epithelial cells by quantitative RT-PCR. In vitro activation showed that these genes were effectively modulated by IFNs in salivary gland epithelial cells, the target cells of autoimmunity in pSS. The activation of IFN pathways led us to investigate whether plasmacytoid dendritic cells were recruited in salivary glands. These IFN-producing cells were detected by immunohistochemistry in all patients with pSS, whereas none was observed in controls. In conclusion, our results support the pathogenic interaction between the innate and adaptive immune system in pSS. The persistence of the IFN signature might be related to a vicious circle, in which the environment interacts with genetic factors to drive the stimulation of salivary TLRs.

  20. Activation of IFN pathways and plasmacytoid dendritic cell recruitment in target organs of primary Sjögren’s syndrome

    Science.gov (United States)

    Gottenberg, Jacques-Eric; Cagnard, Nicolas; Lucchesi, Carlo; Letourneur, Franck; Mistou, Sylvie; Lazure, Thierry; Jacques, Sebastien; Ba, Nathalie; Ittah, Marc; Lepajolec, Christine; Labetoulle, Marc; Ardizzone, Marc; Sibilia, Jean; Fournier, Catherine; Chiocchia, Gilles; Mariette, Xavier

    2006-01-01

    Gene expression analysis of target organs might help provide new insights into the pathogenesis of autoimmune diseases. We used global gene expression profiling of minor salivary glands to identify patterns of gene expression in patients with primary Sjögren’s syndrome (pSS), a common and prototypic systemic autoimmune disease. Gene expression analysis allowed for differentiating most patients with pSS from controls. The expression of 23 genes in the IFN pathways, including two Toll-like receptors (TLR8 and TLR9), was significantly different between patients and controls. Furthermore, the increased expression of IFN-inducible genes, BAFF and IFN-induced transmembrane protein 1, was also demonstrated in ocular epithelial cells by quantitative RT-PCR. In vitro activation showed that these genes were effectively modulated by IFNs in salivary gland epithelial cells, the target cells of autoimmunity in pSS. The activation of IFN pathways led us to investigate whether plasmacytoid dendritic cells were recruited in salivary glands. These IFN-producing cells were detected by immunohistochemistry in all patients with pSS, whereas none was observed in controls. In conclusion, our results support the pathogenic interaction between the innate and adaptive immune system in pSS. The persistence of the IFN signature might be related to a vicious circle, in which the environment interacts with genetic factors to drive the stimulation of salivary TLRs. PMID:16477017

  1. The Effect of Immunologically Safe Plasma Rich in Growth Factor Eye Drops in Patients with Sjögren Syndrome.

    Science.gov (United States)

    Sanchez-Avila, Ronald Mauricio; Merayo-Lloves, Jesus; Riestra, Ana Cristina; Anitua, Eduardo; Muruzabal, Francisco; Orive, Gorka; Fernández-Vega, Luis

    2017-06-01

    The objective was to provide preliminary information about the efficacy and safety of immunologically safe plasma rich in growth factor (immunosafe PRGF) eye drops in the treatment of moderate to severe dry eye in patients with primary and secondary Sjögren's syndrome (SS) and to analyze the influence of several variables on treatment outcomes. This retrospective study included patients with SS. All patients were treated with previously immunosafe PRGF eye drops to reduce the immunologic component contents. Ocular Surface Disease Index (OSDI) scale, best-corrected visual acuity (BCVA), visual analog scale (VAS) frequency, and VAS severity outcome measures were evaluated before and after treatment with immunosafe PRGF. The potential influence of some patient clinical variables on results was also assessed. Safety assessment was also performed reporting all adverse events. Twenty-six patients (12 patients with primary SS, and 14 patients suffering secondary SS) with a total of 52 affected eyes were included and evaluated. Immunosafe PRGF treatment showed a significant reduction (P PRGF with corticosteroids. Only 2 adverse events were reported in 2 patients (7.7% of patients). Signs and symptoms of dry eye syndrome in patients with SS were reduced after treatment with PRGF-Endoret eye drops. Immunosafe PRGF-Endoret is safe and effective for treating patients with primary and secondary SS.

  2. 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.

  3. Oral involvement in patients with primary Sjögren's syndrome. Multidisciplinary care by dentists and rheumatologists.

    Science.gov (United States)

    López-Pintor, Rosa María; Fernández Castro, Mónica; Hernández, Gonzalo

    2015-01-01

    Primary Sjögren's syndrome is a chronic systemic autoimmune disease that causes destruction of lacrimal and salivary glands. The most common and earliest symptoms are oral and ocular dryness. Dry mouth makes talking difficult, tasting and chewing properly, impairing quality of life of these patients. The most common oral signs and symptoms are hyposialia with or without xerostomia, tooth decay, fungal infections, traumatic oral lesions, dysphagia, dysgeusia, and inflammation of salivary glands. There are different therapeutic strategies, depending on the severity of each case, and the increase in the amount of saliva, to reduce the number of cavities and oral infections. It is particularly important to establish a close relationship between the dentist and the rheumatologist in order to make an early and correct diagnosis, promoting appropriate dietary and hygiene measures, as well as to treat and prevent potential oral complications. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  4. First Isolation of carbon dioxide-dependent Proteus mirabilis from an uncomplicated cystitis patient with Sjögren's syndrome.

    Science.gov (United States)

    Oana, Kozue; Yamaguchi, Michiko; Nagata, Mika; Washino, Kei-Ichi; Akahane, Takayuki; Takamatsu, Yu-Uki; Tsutsui, Chie; Matsumoto, Takehisa; Kawakami, Yoshiyuki

    2013-01-01

    An uncomplicated cystitis caused by CO2-dependent Proteus mirabilis was observed in a 64-year-old Japanese female patient with Sjögren's syndrome in the Aomori Kyoritsu Hospital, Aomori, Japan. The initial P. mirabilis isolate came from a midstream urine specimen containing large numbers of Gram-negative, rod-shaped organisms that failed to grow on both Drigalski agar and sheep blood agar incubated in ambient air. The organism did grow when the urine was cultured overnight on blood agar under anaerobic conditions. Hence, we believed that the organism was an anaerobe. Further investigation revealed that the isolate grew on sheep blood agar along with swarming when the atmospheric CO2 concentrations were increased to 5%. Initially, we failed to characterize or identify the P. mirabilis isolate or determine its antimicrobial susceptibilities using the MicroScan WalkAway-40 System because the isolate did not grow in the system. However, the isolate was subsequently identified as P. mirabilis based on its morphological, cultural, and biochemical properties by using the commercially available kit systems, Quick ID-GN and ID-Test EB-20. This identification of the isolate was confirmed by sequencing the 16S rRNA gene of the organism. To our knowledge, this is the first clinical isolation of capnophilic P. mirabilis.

  5. Identification of a novel autoantibody against self-vimentin specific in secondary Sjögren's syndrome.

    Science.gov (United States)

    Li, Yu-Hui; Gao, Ya-Ping; Dong, Jie; Shi, Lian-Jie; Sun, Xiao-Lin; Li, Ru; Zhang, Xue-Wu; Liu, Yu; Long, Li; He, Jing; Zhong, Qun-Jie; Morand, Eric; Yang, Guang; Li, Zhan-Guo

    2018-02-12

    Sjögren's syndrome (SS) is a primary autoimmune disease (pSS) or secondarily associated with other autoimmune diseases (sSS). The mechanisms underlying immune dysregulation in this syndrome remain unknown, and clinically it is difficult to diagnose owing to a lack of specific biomarkers. We extracted immunoglobulins (Igs) from the sera of patients with sSS associated with rheumatoid arthritis (RA) and used them to screen a phage display library of peptides with random sequences. Our results show that an sSS-specific peptide, designated 3S-P, was recognized by sera of 68.2% (60 of 88) patients with sSS, 66.2% of patients with RA-sSS, and 76.5% of patients with systemic lupus erythematosus (SLE)-sSS. The anti-3S-P antibody was scarcely found in patients with pSS (1.8%), RA (1.3%), SLE (4.2%), ankylosing spondylitis (0%), and gout (3.3%), as well as in healthy donors (2%). The 3S-P-binding Igs (antibodies) were used to identify antigens from salivary glands and synovial tissues from patients with sSS. A putative target autoantigen expressed in the synovium and salivary gland recognized by anti-3S-P antibody was identified as self-vimentin. This novel autoantibody is highly specific in the diagnosis of sSS, and the underlying molecular mechanism of the disease might be epitope spreading involved with vimentin.

  6. Expression, purification, crystallization and preliminary X-ray analysis of maleylacetate reductase from Burkholderia sp. strain SJ98

    International Nuclear Information System (INIS)

    Chauhan, Archana; Islam, Zeyaul; Jain, Rakesh Kumar; Karthikeyan, Subramanian

    2009-01-01

    Purification and preliminary X-ray crystallographic analysis of maleylacetate reductase encoded by the pnpD gene is reported. Maleylacetate reductase (EC 1.3.1.32) is an important enzyme that is involved in the degradation pathway of aromatic compounds and catalyzes the reduction of maleylacetate to 3-oxoadipate. The gene pnpD encoding maleylacetate reductase in Burkholderia sp. strain SJ98 was cloned, expressed in Escherichia coli and purified by affinity chromatography. The enzyme was crystallized in both native and SeMet-derivative forms by the sitting-drop vapour-diffusion method using PEG 3350 as a precipitant at 293 K. The crystals belonged to space group P2 1 2 1 2, with unit-cell parameters a = 72.91, b = 85.94, c = 53.07 Å. X-ray diffraction data for the native and SeMet-derivative crystal were collected to 2.7 and 2.9 Å resolution, respectively

  7. 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.

  8. 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.

  9. Evaluation of possible factors affecting contrast sensitivity function in patients with primary Sjögren’s syndrome

    Directory of Open Access Journals (Sweden)

    Sedat Arikan

    2015-06-01

    Full Text Available ABSTRACT Purpose: The contrast sensitivity (CS function in patients with primary Sjögren’s syndrome (pSS may be impaired either frequently as a result of dry eye diseases or rarely as a result of optic neuropathy. In this study, we aimed to evaluate the CS function in pSS patients as well as to assess corneal aberrations and thickness of the peripapillary retinal nerve fiber layer (pRNFL. Methods: Fourteen eyes of 14 pSS patients (pSS group and 14 eyes of 14 healthy participants (control group were subjected to assessment of CS at the spatial frequencies of 1.5, 3.0, 6.0, 12, and 18 cycles/degree (cpd using a functional visual acuity contrast test (FACT; measurement of corneal high-order aberrations (HOAs in terms of coma-like, spherical-like, and total HOAs using Scheimpflug corneal topography; and measurement of the thickness of both the macular ganglion cell-inner plexiform layer (mGCIPL and pRNFL in all quadrants using optical coherence tomography. None of the participants were under treatment with artificial tears. Results: The results of the CS test did not differ between the 2 groups at all spatial frequencies (p>0.05. In addition, there were no statistically significant differences between the 2 groups in terms of corneal HOAs (p>0.05 and thickness of mGCIPL (p>0.05. However, among all quadrants, only the inferior quadrant of pRNFL in pSS patients was statistically significantly thinner than that in the healthy participants (p=0.04. Conclusions: The CS function in pSS patients can be maintained with normal thickness of both pRNFL and mGCIPL and with lack of increased corneal HOAs, which may be present even in the absence of artificial tear usage.

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

    Directory of Open Access Journals (Sweden)

    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.

  11. Utility of Novel Autoantibodies in the Diagnosis of Sjögren's Syndrome Among Patients With Dry Eye.

    Science.gov (United States)

    Karakus, Sezen; Baer, Alan N; Agrawal, Devika; Gurakar, Merve; Massof, Robert W; Akpek, Esen K

    2018-04-01

    To investigate the value of 3 novel autoantibodies [salivary protein 1 (SP1), carbonic anhydrase 6 (CA6), and parotid secretory protein (PSP)] in differentiating Sjögren's syndrome (SS)-related dry eye from non-SS dry eye. Forty-six dry eye patients with SS (SS dry eye), 14 dry eye patients without SS (non-SS dry eye), and 25 controls were included. The 2012 American College of Rheumatology classification criteria were used for the diagnosis of SS. After a detailed review of systems, the Ocular Surface Disease Index questionnaire, Schirmer test without anesthesia, tear film breakup time, and ocular surface staining were performed to assess dry eye. All participants underwent serological testing using a commercially available finger prick kit. Thirty-seven patients with SS (80.4%) had a positive traditional autoantibody and 28 (60.9%) had a positive novel autoantibody. Traditional autoantibodies were absent in all non-SS dry eye patients and controls. Novel autoantibodies were present in 7/14 (50%) non-SS dry eye patients and 4/25 (16%) controls. Among 3 novel autoantibodies, anti-CA6 was significantly more prevalent in the SS and non-SS dry eye groups than in controls (52.2% vs. 42.9% vs. 8.0%, P = 0.001). Dry eye patients with positive anti-CA6 alone were significantly younger than patients with only traditional autoantibodies. Anti-CA6 was associated with worse dry eye signs and symptoms. Anti-CA6 was the most prevalent novel autoantibody in patients with dry eye, and was associated with younger age and more severe disease. Longitudinal studies are needed to determine whether anti-CA6 is a marker for early SS or perhaps another form of an autoimmune dry eye disease.

  12. A possible genetic association with chronic fatigue in primary Sjögren's syndrome: a candidate gene study.

    Science.gov (United States)

    Norheim, Katrine Brække; Le Hellard, Stephanie; Nordmark, Gunnel; Harboe, Erna; Gøransson, Lasse; Brun, Johan G; Wahren-Herlenius, Marie; Jonsson, Roland; Omdal, Roald

    2014-02-01

    Fatigue is prevalent and disabling in primary Sjögren's syndrome (pSS). Results from studies in chronic fatigue syndrome (CFS) indicate that genetic variation may influence fatigue. The aim of this study was to investigate single nucleotide polymorphism (SNP) variations in pSS patients with high and low fatigue. A panel of 85 SNPs in 12 genes was selected based on previous studies in CFS. A total of 207 pSS patients and 376 healthy controls were genotyped. One-hundred and ninety-three patients and 70 SNPs in 11 genes were available for analysis after quality control. Patients were dichotomized based on fatigue visual analogue scale (VAS) scores, with VAS fatigue" (n = 53) and VAS ≥50 denominated "high fatigue" (n = 140). We detected signals of association with pSS for one SNP in SLC25A40 (unadjusted p = 0.007) and two SNPs in PKN1 (both p = 0.03) in our pSS case versus control analysis. The association with SLC25A40 was stronger when only pSS high fatigue patients were analysed versus controls (p = 0.002). One SNP in PKN1 displayed an association in the case-only analysis of pSS high fatigue versus pSS low fatigue (p = 0.005). This candidate gene study in pSS did reveal a trend for associations between genetic variation in candidate genes and fatigue. The results will need to be replicated. More research on genetic associations with fatigue is warranted, and future trials should include larger cohorts and multicentre collaborations with sharing of genetic material to increase the statistical power.

  13. Local and Systemic IKKε and NF-κB Signaling Associated with Sjögren’s Syndrome Immunopathogenesis

    Directory of Open Access Journals (Sweden)

    Weiqian Chen

    2015-01-01

    Full Text Available The activated NF-κB signaling pathway plays an important role in pathogenesis of primary Sjögren’s syndrome (pSS. The inhibitor of κB (IκB kinase (IKK family such as IKKα, IKKβ, IKKγ, and IKKε, is required for this signaling. Our aim was to investigate the role of IKKα/β/γ/ε in patients with untreated pSS. In minor salivary glands from pSS patients, phosphorylated IKKε (pIKKε, pIκBα, and pNF-κB p65 (p-p65 were highly expressed in ductal epithelium and infiltrating mononuclear cells by immunohistochemistry, compared to healthy individuals. pIKKα/β and pIKKγ were both negative. And pIKKε positively related to expression of p-p65. Furthermore, pIKKε and p-p65 expression significantly correlated with biopsy focus score and overall disease activity. Meanwhile, in peripheral blood mononuclear cells from pSS patients, pIKKε, total IKKε, pIKKα/β, and p-p65 were significantly increased by western blot, compared to healthy controls. However, there was no difference in IKKγ and IκBα between pSS patients and healthy individuals. These results demonstrated an abnormality of IKKε, IκBα, and NF-κB in pSS, suggesting a potential target of treatment for pSS based on the downregulation of IKKε expression and deregulation of NF-κB pathway.

  14. 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.

  15. Comparisons of presentations and outcomes of neuromyelitis optica patients with and without Sjögren's syndrome.

    Science.gov (United States)

    Zhong, Yu Hua; Zhong, Zhi Gang; Zhou, Zhou; Ma, Zhen Yu; Qiu, Meng Yao; Peng, Fu Hua; Zhang, Wei Xi

    2017-02-01

    Patients with neuromyelitis optica (NMO) often have an accompanying autoimmune disease, most commonly, but not limited to Sjögren's syndrome (SS). The aim of this study was to compare clinical and laboratory features between NMO patients with and without SS and to investigate the prognosis of NMO in patients with and without SS. Twenty-three NMO patients with SS and 42 NMO patients without SS were included. Clinical and laboratory profiles were compared, including annual relapse rate and time from onset of NMO to Expanded Disability Status Scale (EDSS) scores of 4.0 and 6.0. More NMO patients with SS than those without SS had anti-nuclear antibody, anti-SS-A/Ro and anti-SS-B/La antibodies (91.3 vs. 35.7%, p < 0.001, 87.0 vs. 2.3%, p < 0.001, and 34.8 vs. 0.0%, p < 0.001, respectively). Serum immunoglobulins (IgA, IgM and IgG) were markedly increased in NMO patients with SS in comparison with those without SS. Annual relapse rate and the time from disease onset to an EDSS score of 4.0 and 6.0 were not significantly different between the two groups. No differences between the two groups were found for the other parameters, including AQP-4 antibody status, length of spinal cord lesion and brain lesions. These results imply that NMO in SS more likely represents coexistence with SS rather than representing the result of direct central nervous system involvement in SS. Autoimmune response appears to be more intense in the NMO group with SS, but did not cause a more severe prognosis in comparison with the group without SS, indicating that we should pay attention to the potential benefit of the antinuclear antibodies in NMO.

  16. 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 esophageal reflux, current exposure to secondary tobacco smoke, frequent neck or throat tension, frequent throat clearing, chronic post-nasal drip, and stomach or duodenal ulcers. Swallowing disorders did not differ on the basis of primary or secondary SS. Swallowing disorders and specific swallowing symptoms were uniquely associated with reduced 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. 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.

  19. Sex influences eQTL effects of SLE and Sjögren's syndrome-associated genetic polymorphisms.

    Science.gov (United States)

    Lindén, Magdalena; Ramírez Sepúlveda, Jorge I; James, Tojo; Thorlacius, Gudny Ella; Brauner, Susanna; Gómez-Cabrero, David; Olsson, Tomas; Kockum, Ingrid; Wahren-Herlenius, Marie

    2017-10-25

    Systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS) are autoimmune disorders characterized by autoantibodies, dysregulated B cells, and notably high female-to-male incidence ratios. Genome-wide association studies have identified several susceptibility SNPs for both diseases. Many SNPs in the genome are expression quantitative trait loci (eQTLs), with context-dependent effects. Assuming that sex is a biological context, we investigated whether SLE/pSS SNPs act as eQTLs in B cells and used a disease-targeted approach to understand if they display sex-specific effects. We used genome-wide genotype and gene expression data from primary B cells from 125 males and 162 females. The MatrixEQTL R package was used to identify eQTLs within a genomic window of 2 Mb centered on each of 22 established SLE and/or pSS susceptibility SNPs. To find sex-specific eQTLs, we used a linear model with a SNP * sex interaction term. We found ten SNPs affecting the expression of 16 different genes (FDR rs7574865-INPP1, rs7574865-MYO1B, rs4938573-CD3D, rs11755393-SNRPC, and rs4963128-PHRF1 were novel observations for the immune compartment and B cells. By analyzing the SNP * sex interaction terms, we identified six genes with differentially regulated expression in females compared to males, depending on the genotype of SLE/pSS-associated SNPs: SLC39A8 (BANK1 locus), CD74 (TNIP1 locus), PXK, CTSB (BLK/FAM167A locus), ARCN1 (CXCR5 locus), and DHX9 (NCF2 locus). We identified several unknown sex-specific eQTL effects of SLE/pSS-associated genetic polymorphisms and provide novel insight into how gene-sex interactions may contribute to the sex bias in systemic autoimmune diseases.

  20. Three percent diquafosol ophthalmic solution as an additional therapy to existing artificial tears with steroids for dry-eye patients with Sjögren's syndrome.

    Science.gov (United States)

    Yokoi, N; Sonomura, Y; Kato, H; Komuro, A; Kinoshita, S

    2015-09-01

    To investigate the long-term results of 3% diquafosol ophthalmic solution as an alternative therapy to existing ophthalmic solutions, including topical immunosuppression, for the treatment of dry eye in patients with Sjögren's syndrome. This study involved 14 female dry-eye patients (mean age: 62.4 years) with Sjögren's syndrome who insufficiently responded to their current therapy. In all patients, 3% diquafosol ophthalmic solution was administered six times daily for 12 months in substitution for artificial tears and sodium hyaluronate ophthalmic solution. Their use of corticosteroid eye drops remained unchanged from that prior to the treatment with diquafosol sodium. The subjective symptoms assessed, and ocular signs including tear meniscus radius and the tear film breakup time, and ocular-surface epithelial damage score were examined at 1, 2, 3, 4, 5, 6, 9, and 12 months after initiating treatment. Among the subjective symptoms, significant improvement was obtained in dryness at 2 months post treatment, in eye fatigue at 1, 2, 3, 4, and 12 months post treatment, and in pain at 1, 2, 6, and 12 months post treatment. Difficulty in opening the eye, foreign body sensation, and redness were also significantly ameliorated at various time-points. The tear meniscus radius and the tear film breakup time were significantly improved throughout the observation period, and the corneal epithelial staining scores were significantly decreased at 3 months post treatment. In dry-eye patients with Sjögren's syndrome, treatment with 3% diquafosol ophthalmic solution improved both symptoms and signs, and that effectiveness was maintained for 12 months.

  1. 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.

  2. 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 (pdry eye. 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.

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  5. 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.

  6. How Are Ocular Signs and Symptoms of Dry Eye Associated With Depression in Women With and Without Sjögren Syndrome?

    Science.gov (United States)

    Gonzales, John A; Chou, Annie; Rose-Nussbaumer, Jennifer R; Bunya, Vatinee Y; Criswell, Lindsey A; Shiboski, Caroline H; Lietman, Thomas M

    2018-07-01

    To determine whether ocular phenotypic features of keratoconjunctivitis sicca (KCS) and/or participant-reported symptoms of dry eye disease are associated with depression in women participants enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA). Cross-sectional study. Women enrolled in the SICCA registry from 9 international research sites. Participants met at least 1 of 5 inclusion criteria for registry enrollment (including complaints of dry eyes or dry mouth, a previous diagnosis of Sjögren syndrome (SS), abnormal serology (positive anti-Sjögren syndrome antigen A and/or B [anti-SSA and/or anti-SSB]), or elevated antinuclear antibody and rheumatoid factor), bilateral parotid gland enlargement, or multiple dental caries). At baseline, participants had oral, ocular, and rheumatologic examination; blood and saliva collection; and a labial salivary gland biopsy (LSGB). They also completed an interview and questionnaires including assessment of depression with the Patient Health Questionnaire 9 (PHQ-9). Univariate logistic regression was used to assess the association between depression and demographic characteristics, participant-reported health, phenotypic features of Sjögren syndrome, and participant-reported symptoms. Mixed-effects modeling was performed to determine if phenotypic features of KCS and/or participant-reported symptoms of dry eye disease were associated with depression, controlling for health, age, country or residence, and sex and allowing for nonindependence within geographic site. Dry eye complaints produced a 1.82-fold (95% confidence interval [CI] 1.38-2.40) higher odds of having depression compared to being symptom-free (P dry eyes and/or dry mouth rather than SS itself resulted in higher odds of depression. One particular ocular phenotypic feature of SS, a positive ocular staining score, was inversely correlated with depression. Participant-reported eye symptoms, particularly specific ocular sensations such as

  7. Hepatitis inmune temprana en el curso del síndrome de Sjögren en pediatría

    OpenAIRE

    Baar Z., Alejandro; Harris D., Paul; Talesnik G., Eduardo; Duarte G. de C., Ignacio

    1999-01-01

    La hepatitis inmune corresponde a una enfermedad necroinflamatoria del hígado, de causa desconocida, asociada a la presencia de autoanticuerpos circulantes. Puede presentarse aisladamente, o en asociación a otras enfermedades autoinmunes. Entre estas, destaca el síndrome de Sjögren, que compromete preferentemente a las glándulas salivales, aunque puede afectar a cualquier segmento del tubo digestivo, especialmente el hígado. Reportamos el caso de una niña de 12 años de edad, portadora de Sínd...

  8. 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...

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

    OpenAIRE

    Daniela Oulego

    2016-01-01

    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 a...

  10. Från tonåring till toppbloggare : En kvalitativ fallstudie om självpresentation och det personliga varumärket kommunicerat genom en blogg

    OpenAIRE

    Laricchia, Daniela

    2016-01-01

    Sammanfattning Detta är en fallstudie över en Sveriges mest välkända och framgångsrika bloggar – www.kenzas.se, driven av Kenza Zouiten. Syftet med studien var att ta reda på hur man via självpresentation genom en blogg kan bygga upp och skapa sig ett starkt personligt varumärke. De frågeställningar studien utgick ifrån var hur Kenza Zouiten väljer att framställa sig själv som person i skriven text och i bilder, samt huruvida det finns några skillnader i hur hon väljer att framställa sig själ...

  11. 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.

  12. 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.

  13. Clinical and Molecular Inflammatory Response in Sjögren Syndrome-Associated Dry Eye Patients Under Desiccating Stress.

    Science.gov (United States)

    López-Miguel, Alberto; Tesón, Marisa; Martín-Montañez, Vicente; Enríquez-de-Salamanca, Amalia; Stern, Michael E; González-García, María J; Calonge, Margarita

    2016-01-01

    To evaluate the response of the lacrimal function unit in Sjögren syndrome (SS)-associated dry eye patients exposed to 2 simulated daily life environmental conditions. Prospective crossover pilot study. Fourteen female SS dry eye patients were exposed for 2 hours to a controlled normal condition (23 C, 45% relative humidity, and air flow 0.10 m/s) and a controlled adverse condition that simulates desiccating stress (23 C, 5% relative humidity, and air flow 0.10 m/s). The following dry eye tests were performed before and after the exposure: tear osmolarity, phenol red thread test, conjunctival hyperemia, fluorescein tear break-up time, corneal fluorescein staining, conjunctival lissamine green staining, and Schirmer test. Levels of 16 molecules were analyzed in tears by multiplex immunobead analysis. Clinical evaluation showed lacrimal functional unit impairment after the desiccating stress: significantly increased tear osmolarity (315.7 ± 3.0 vs 327.7 ± 5.1 mOsm/L, P = .03), conjunctival hyperemia (1.3 ± 0.1 vs 1.6 ± 0.1, P = .05), and corneal staining in temporal (3.5 ± 0.5 vs 4.7 ± 0.4, P = .01) and nasal (3.6 ± 0.5 vs 4.5 ± 0.5, P = .04) areas. Tear concentrations increased for interleukin-1 receptor antagonist (16 557.1 ± 4047.8 vs 31 895.3 ± 5916.5 pg/mL, P = .01), interleukin-6 (63.8 ± 20.2 vs 111.5 ± 29.6 pg/mL, P = .02), interleukin-8 (2196.1 ± 737.9 vs 3753.2 ± 1106.0 pg/mL, P = .03), and matrix metalloproteinase-9 (101 515.6 ± 37 088.4 vs 145 867.1 ± 41 651.5 pg/mL, P = .03). After the simulated normal condition, only a significant increase in nasal corneal staining (2.9 ± 0.5 vs 3.6 ± 0.5, P = .03) was observed. Even a short exposure to a desiccating environment can produce a significant deterioration of the lacrimal function unit in female SS dry eye patients. The often unnoticed exposure to these conditions during daily life may increase inflammatory activity rapidly, triggering an ocular surface deterioration. Copyright © 2016

  14. Studies on the structures of the Tm, Sj, M1, Can, Sext and Hu blood group antigens.

    Science.gov (United States)

    Dahr, W; Knuppertz, G; Beyreuther, K; Moulds, J J; Moulds, M; Wilkinson, S; Capon, C; Fournet, B; Issitt, P D

    1991-08-01

    The Glycophorins (GPs = sialoglycoproteins) in erythrocyte membranes from various Black individuals, some of which exhibit the M1, Can, Sj, Tm, Sext and/or Hu antigens, and several Caucasian donors, including pooled fetal red cells, were studied. Using agglutination inhibition assays with GP fractions, GP fragments and chemically modified GPs as well as trypsin treatment of intact red cells, the antigens defined by anti-M1, anti-M+M1, anti-Can and anti-Tm sera were found to be located on the N-terminal tryptic peptide (T2, residues 1-31) of the major GP (GP A = MN sialoglycoprotein). Evidence was obtained that the N-terminal amino-acid residue, NeuNAc and/or (a) different sugar residue(s) are involved in the antigens. Amino-acid sequence and composition analyses excluded an amino-acid exchange within the N-terminal region (residues 1-31) of GP A. Carbohydrate analyses revealed the attachment of GlcNAc residues (up to about five, dependent on the strength of the above-mentioned antigens) to O-glycosidically linked oligosaccharides within the N-terminal portion (residues 1-31) of GP A. As judged from the carbohydrate compositions of peptides, the alteration of the O-glycosidic oligosaccharides is associated with a slight increase of the Gal and Fuc contents and a slight decrease of the NeuNAc level. Analyses of small, secondary cyanogen bromide and V8 proteinase peptides from the N-terminal region of GP A from Blacks, Caucasians and Caucasian fetal cells suggest that the variable attachment of small quantities of GlcNAc (about 0.03 to about 0.2 residues per peptide molecule) accounts, at least in part, for the polymorphisms detected by anti-Can and the original anti-Tm (serum Sheerin). Remarkably, the GlcNAc-containing O-glycosidic oligosaccharides occur only in small quantities, or not all at, within the positions 32-61 of GP A and the glycosylated domains of GP B and GP C.(ABSTRACT TRUNCATED AT 400 WORDS)

  15. Chronic hepatitis B virus infection in Sjögren's syndrome. Prevalence and clinical significance in 603 patients.

    Science.gov (United States)

    Marcos, Miguel; Alvarez, Fausto; Brito-Zerón, Pilar; Bove, Albert; Perez-De-Lis, Marta; Diaz-Lagares, Candido; Sanchez-Tapias, Jose-Maria; Ramos-Casals, Manuel

    2009-06-01

    To analyze the prevalence and clinical characteristics of chronic hepatitis B virus (HBV) infection in a large series of patients with Sjögren syndrome (SS). We investigated the prevalence of chronic HBV infection in 603 consecutive patients with SS diagnosed in our department between 1994 and 2008. There were 517 patients with primary SS (482 women and 35 men, with a mean age at the time of fulfillment of the classification criteria of 57 years) and 86 patients with SS associated with chronic HCV infection (66 women and 20 men, with a mean age at the time of fulfillment of the classification criteria of 65 years). All patients fulfilled 4 or more of the 1993 European Community Study Group criteria for SS. The presence of HBsAg+ was detected in five (0.83%) of the 603 patients with SS. All HBsAg+ patients had primary SS. No patient with HCV-related SS had HBV coinfection. There were 4 women and 1 man, with a mean age at diagnosis of primary SS of 65 years (range 31 to 89 years). All patients showed sicca and systemic involvement. The main extraglandular feature was articular involvement in 5 (100%) patients (including arthritis in two). The main immunologic features were RF in 4 (80%) patients and ANA in 3 (60%). No patient had hypocomplementemia, cryoglobulinemia, antimitochondrial or anti-LKM1 antibodies. Liver involvement was detected in two patients and consisted of slightly raised levels of transaminases. No patient showed clinical manifestations of liver disease such as hepatomegaly, splenomegaly, jaundice or clinical features of hepatic decompensation (ascites, encephalopathy or gastrointestinal bleeding). We found a prevalence of chronic HBV infection of 0.83% in SS, very similar to the prevalence in general population in Spain (0.7%). In contrast to the close association between SS and HCV, chronic HBV infection is not associated with SS in our geographical area, with a ratio SS-HBV/SS-HCV cases of 1:10.

  16. Delivery of Bone Marrow-Derived Mesenchymal Stem Cells Improves Tear Production in a Mouse Model of Sjögren’s Syndrome

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    Hema S. Aluri

    2017-01-01

    Full Text Available The purpose of the present study was to test the potential of mouse bone marrow-derived mesenchymal stem cells (BD-MSCs in improving tear production in a mouse model of Sjögren’s syndrome dry eye and to investigate the underlying mechanisms involved. NOD mice (n=20 were randomized to receive i.p. injection of sterile phosphate buffered saline (PBS, control or murine BD-MSCs (1 × 106 cells. Tears production was measured at baseline and once a week after treatment using phenol red impregnated threads. Cathepsin S activity in the tears was measured at the end of treatment. After 4 weeks, animals were sacrificed and the lacrimal glands were excised and processed for histopathology, immunohistochemistry, and RNA analysis. Following BD-MSC injection, tears production increased over time when compared to both baseline and PBS injected mice. Although the number of lymphocytic foci in the lacrimal glands of treated animals did not change, the size of the foci decreased by 40.5% when compared to control animals. The mRNA level of the water channel aquaporin 5 was significantly increased following delivery of BD-MSCs. We conclude that treatment with BD-MSCs increases tear production in the NOD mouse model of Sjögren’s syndrome. This is likely due to decreased inflammation and increased expression of aquaporin 5.

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

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    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.

  18. 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.

  19. 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.

  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. 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

  2. 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

  3. Meibomian gland dysfunction patients with novel Sjögren’s syndrome biomarkers benefit significantly from a single vectored thermal pulsation procedure: a retrospective analysis

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    Epitropoulos AT

    2017-04-01

    Full Text Available Alice T Epitropoulos,1,2 Krysta Goslin,2 Raman Bedi,3 Caroline A Blackie4 1Ophthalmic Surgeons and Consultants of Ohio, The Eye Center of Columbus, 2The Ohio State University Wexner Medical Center, Department of Ophthalmology, Columbus, OH, USA; 3Iris Advanced Eye Centre, Chandigarh, India; 4TearScience Inc., Morrisville, NC, USA Purpose: To measure the effects from a single vectored thermal pulsation treatment of the meibomian glands on dry eye signs and symptoms in patients who tested positively versus negatively for novel Sjögren’s syndrome (SS biomarkers. Methods: The retrospective study included the deidentified data of 102 eyes of 59 patients with dry eye and meibomian gland dysfunction (MGD, who were also tested for novel biomarkers for SS and underwent a single 12-minute LipiFlow thermal pulsation procedure. All patients were already being treated with individualized dry eye therapy but remained symptomatic. Meibomian gland secretion (MGS scores, Standard Patient Evaluation of Eye Dryness (SPEED questionnaire scores and tear breakup times (TBUTs before and 8 weeks after thermal pulsation treatment were analyzed. Results: Twenty-three patients tested positive for novel biomarkers of SS and 36 patients tested negative. At baseline, MGS, SPEED and TBUT of both SS-positive and SS-negative patients were equivalent. At 8 weeks’ post-treatment, mean MGS score, SPEED and TBUT were 13.0±7.8, 12.5±6.8 and 9.6±4.6, respectively, in SS-positive patients and 15.9±7.9, 10.0±6.3 and 8.3±4.6, respectively, in SS-negative patients (P<0.001. While the post-treatment MGS was significantly better in SS-negative patients than SS-positive (P=0.021, no significant difference between post-treatment SPEED and TBUT was observed between the two groups (P>0.05. Conclusion: LipiFlow treatment in MGD patients who were SS-positive for novel biomarkers of SS demonstrated improvement in signs and symptoms of dry eye. While improvement in MGS scores in SS

  4. Somatostatin receptor scintigraphy in patients with rheumatoid arthritis and secondary Sjögren's syndrome treated with Infliximab: a pilot study.

    Science.gov (United States)

    Anzola-Fuentes, L K; Chianelli, M; Galli, F; Glaudemans, A W J M; Martin Martin, L; Todino, V; Migliore, A; Signore, A

    2016-12-01

    Human T lymphocytes infiltrating tissues in autoimmune diseases are known to express somatostatin receptors amongst other activation markers. In this study, we evaluated whether somatostatin receptor scintigraphy (SRS) using a radiolabelled somatostatin analogue ((99m)Tc-EDDA/tricine-HYNIC-tyr(3)-octreotide ((99m)Tc-EDDA/HYNIC-TOC)) is able to detect the presence of immune-mediated processes in patients with rheumatoid arthritis and secondary Sjögren's syndrome. We also aimed to evaluate whether positivity to SRS was predictive of therapeutic response and if SRS could be used for monitoring the efficacy of immunomodulatory treatment. Eighteen patients with rheumatoid arthritis and secondary Sjögren's syndrome not responding to conventional treatment were recruited for treatment with infliximab, a monoclonal antibody against TNF-α. All patients had complete blood cell count, renal and liver function tests, measurements of ESR, CRP, ANA, ENA, and anti-dsDNA antibodies, functional salivary gland scintigraphy, labial biopsy, and ophthalmologic assessment with Schirmer's test and tear film break-up time (BUT). Diagnosis was made according to the revised criteria of the American-European Consensus Group. All patients underwent SRS at baseline and after 3-6 months of therapy with infliximab. Eleven out of 18 had repeat SRS images. Images of the salivary glands and major joints were acquired 3 h after injection of 370 MBq of (99m)Tc-EDDA/HYNIC-TOC. Image analysis was performed semi-quantitatively. All patients showed uptake of (99m)Tc-EDDA/HYNIC-TOC in the joints. Salivary glands also showed variable radiopharmaceutical uptake in 12 out of 18 patients, but all patients showed presence of lymphocytic infiltration at labial salivary gland biopsy. All patients, who repeated the study after treatment, showed significant reduction of somatostatin uptake in the joints but not in the salivary glands. SRS using (99m)Tc-EDDA/HYNIC-TOC may be a useful imaging tool to assess

  5. Elevated levels of faecal calprotectin in primary Sjögren's syndrome is common and associated with concomitant organic gastrointestinal disease.

    Science.gov (United States)

    Andréasson, Kristofer; Ohlsson, Bodil; Mandl, Thomas

    2016-01-12

    Primary Sjögren's syndrome (pSS) is a systemic rheumatic disease in which gastrointestinal (GI) symptoms are common. Faecal calprotectin (FC) is a non-invasive biomarker that has been suggested to discriminate organic intestinal disease from functional disorders. The purpose of this study was to explore the usefulness of FC testing in patients with pSS. In total, 56 consecutive patients with pSS and 29 healthy control subjects were included in this cross-sectional study. FC was measured with a commercially available enzyme-linked immunosorbent assay kit. GI symptoms were evaluated with the Rome III questionnaire and the Visual Analogue Scale for Irritable Bowel Syndrome. In patients with pSS, disease activity was estimated using the European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI), and patient-reported outcomes were evaluated with the EULAR Sjögren's Syndrome Patient-Reported Index. Patients with pSS had higher levels of FC than healthy control subjects (median 54 μg/g, interquartile range [IQR 20-128]; vs. 20 μg/g [20-43]; p = 0.002). Concomitant organic GI disease was found in 14 patients with pSS and included inflammatory bowel disease (n = 3), colonic adenoma (n = 2) and GI lymphoma (n = 1). Patients with organic GI disease had higher FC levels than the other patients with pSS (median 274 μg/g [IQR 61-363] vs. median 34 μg/g [IQR 20-76]; p organic GI disease or elevated FC levels. FC correlated moderately with ESSDAI. Excluding patients with organic GI disease, we did not identify any significant association between ESSDAI and FC levels. GI symptoms are frequent in pSS. Contrary to patient-reported outcomes, elevated FC levels in pSS indicate possible organic GI disease that warrants further investigation.

  6. Anti-Ma2/Ta antibodies in a woman with primary lateral sclerosis-like phenotype and Sjögren syndrome.

    Science.gov (United States)

    Piccolo, Giovanni; Tavazzi, Eleonora; Jarius, Sven; Alfonsi, Enrico; Cavagna, Lorenzo; Piccolo, Laura; Zardini, Elisabetta; Voltz, Raymond; Franciotta, Diego

    2011-10-01

    Anti-Ma2/Ta antibodies are rare paraneoplastic antibodies, which are mostly associated with limbic encephalitis in male patients with testicular cancer. We report on a 50-year-old woman with a pure progressive spastic paraparesis. Next, she was diagnosed as having a Sjögren syndrome, with serological positivity for anti-SS-Ro antibodies. The patient's serum and cerebrospinal fluid samples were positive for anti-Ma2/Ta antibodies, which were also proved to be intrathecally produced. These findings, and the coexistence of systemic autoimmunity, led us to treat the patient with corticosteroids first, and then with plasma exchange. Neurological symptoms scarcely responded to both the therapies. The search for cancer was negative up to 4 years after the disease onset. Our case expands the spectrum of clinical syndromes associated with anti-Ma2/Ta antibodies.

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

    OpenAIRE

    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.

    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 ...

  8. Effects of gustatory stimulants of salivary secretion on salivary pH and flow in patients with Sjögren's syndrome: a randomized controlled trial.

    Science.gov (United States)

    da Silva Marques, Duarte Nuno; da Mata, António Duarte Sola Pereira; Patto, José Maria Vaz; Barcelos, Filipe Alexandre Duarte; de Almeida Rato Amaral, João Pedro; de Oliveira, Miguel Constantino Mendes; Ferreira, Cristina Gutierrez Castanheira

    2011-11-01

    To compare salivary pH changes and stimulation efficacy of two different gustatory stimulants of salivary secretion (GSSS) in patients with primary Sjögren syndrome. Portuguese Institute for Rheumatological Diseases. Double-blind randomized controlled trial. Eighty patients were randomized to two intervention groups. Sample size was calculated using an alpha error of 0.05 and a beta of 0.20. Participants were randomly assigned to receive a new GSSS containing a weaker malic acid, fluoride and xylitol or a traditionally citric acid-based one. Saliva collection was obtained by established methods at different times. The salivary pH of the samples was determined with a pH meter and a microelectrode. Salivary pH variations and counts of subjects with pH below 4.5 for over 1 min and stimulated salivary flow were the main outcome measures. Both GSSS significantly stimulated salivary output without significant differences between the two groups. The new gustatory stimulant of salivary secretion presented an absolute risk reduction of 52.78% [33.42-72.13 (95% CI)] when compared with the traditional one. In Xerostomic Primary Sjögren syndrome patients, gustatory stimulants of salivary secretion based on acid mail only with fluoride and xylitol present similar salivary stimulation capacity when compared to citric acid-based ones, besides significantly reducing the number of salivary pH drops below 4.5. This could be related to a diminished risk for dental erosion and should be confirmed with further studies. © 2011 John Wiley & Sons A/S.

  9. 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.

  10. Treatment of Sjögren's syndrome dry eye using 0.03% tacrolimus eye drop: Prospective double-blind randomized study.

    Science.gov (United States)

    Moscovici, Bernardo Kaplan; Holzchuh, Ricardo; Sakassegawa-Naves, Fernando Eiji; Hoshino-Ruiz, Diego Ricardo; Albers, Marcos Bottene Villa; Santo, Ruth Miyuki; Hida, Richard Yudi

    2015-10-01

    To describe the clinical efficacy of the treatment of Sjögren's syndrome dry eye using 0.03% tacrolimus eye drop. Prospective double-blind randomized study. Institutional outpatient clinic. Forty-eight eyes of twenty-four patients with dry eye related to Sjögren syndrome were enrolled in this study. The patients were randomized in 2 groups: tacrolimus (n=14) and vehicle (n=10) group. The tacrolimus group received a vial containing tacrolimus 0.03% (almond oil as vehicle) and the other group received the almond oil vehicle. All patients were instructed to use the eye drops every 12h in the lower conjunctival sac. Schirmer I test, break-up-time (BUT), corneal fluorescein and Rose Bengal staining scores were evaluated in all patients one day before the treatment (baseline), 7, 14, 28 and 90 days after treatment with the eye drops. The average fluorescein and Rose Bengal scores improved statistically after 7 days of treatment and even more after 90 days. The average Schirmer I and BUT values were unchanged after 7, 14 and 21 days but did show an improvement relative to baseline after 28 days of treatment. Schirmer I, BUT, fluorescein and Rose Bengal did not show any statistical significance in the vehicle group. Topical 0.03% tacrolimus eye drop improved tear stability and ocular surface status in cases of inflammatory or SS-related dry eye. ClinicalTrials.gov Identifier: NCT01850979. Copyright © 2015 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.

  11. 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.

  12. 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.

  13. 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.

  14. Condição oral dos pacientes com síndrome de Sjögren: uma revisão sistemática: uma revisão sistemática

    Directory of Open Access Journals (Sweden)

    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.

  15. Comparative analysis of novel autoantibody isotypes against citrullinated-inter-alpha-trypsin inhibitor heavy chain 3 (ITIH3)(542-556) peptide in serum from Taiwanese females with rheumatoid arthritis, primary Sjögren's syndrome and secondary Sjögren's syndrome in rheumatoid arthritis.

    Science.gov (United States)

    Liao, Chen-Chung; Chou, Pei-Lun; Cheng, Chao-Wen; Chang, Yu-Sheng; Chi, Wei-Ming; Tsai, Kai-Leun; Chen, Wei-Jung; Kung, Ting-Shuan; Tai, Chih-Chun; Lee, Kuan-Wei; Chen, You-Chia; Lin, Ching-Yu

    2016-06-01

    The purpose of this study was to discover and validate inter-alpha-trypsin inhibitor heavy chain H3 (ITIH3) as novel biomarkers, and evaluate autoantibody isotypes against an unmodified and citrullinated ITIH3(542-556) peptide among Taiwanese female patients with rheumatoid arthritis (RA), primary Sjögren's syndrome (pSS), secondary Sjögren's syndrome in rheumatoid arthritis (RA-sSS), and healthy controls (HCs). We used concanavalin A (Con A) affinity chromatography, 1-D SDS-PAGE, and label-free nano-LC-MS/MS to screen biomarker candidates (serum-derived Con A-captured proteins) and then identify PTMs of validated biomarkers (serum proteins) using pooled serum from 7 RA-sSS female patients and 7 age-matched HCs (the discovery set). Furthermore, the protein level and autoantibody isotype analyses were further validated against individual serum from 18 HCs, 18 RA, 18 pSS, and 18 RA-sSS patients (the validation set). Con A-bound ITIH3 was identified and validated as the only differentially expressed protein, which was elevated. Additionally, 2 novel PTMs in ITIH3 were identified and included citrullination at arginine-(546) and arginine-(556), and hexosamine at tryptophan-(558). Further, concentrations of anti-citrullinatd-ITIH3(542-556) peptide autoantibodies significantly increased in patients with RA, pSS, and RA-sSS compared to HCs. Especially, autoantibody IgM against the citrullinated-ITIH3(542-556) peptide showed better diagnostic performance in discriminating both RA versus pSS and pSS versus RA-sSS. By using comparative proteomic analysis of serum samples, the current study discovered and validates differentially expressed Con A-bound ITIH3 as a potential biomarker for secondary Sjögren's syndrome (SS) in rheumatoid arthritis (RA) patients and healthy controls (HCs). Besides, hexosamine and citrullination on ITIH3 were further identified. Through analyzing autoantibody isotypes against the citrullinated ITIH3 peptide, patients with RA, primary SS, and RA

  16. Micofenolato mofetil na síndrome de Sjögren primária: uma opção para o tratamento da agranulocitose Mycophenolate mofetil in primary Sjögren's syndrome: a treatment option for agranulocytosis

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    Sonia Cristina de Magalhães Souza Fialho

    2012-04-01

    Full Text Available A síndrome de Sjögren (SS é uma doença autoimune caracterizada pela presença de infiltrado linfocítico nas glândulas salivares e lacrimais. Manifestações hematológicas da síndrome de Sjögren primária (SSp geralmente consistem em anemia leve, trombocitopenia, neutropenia moderada e linfopenia. Agranulocitose é raramente descrita e, em geral, responde bem ao tratamento de imunossupressão. Neste trabalho, descrevemos o caso de uma paciente portadora de SSp que apresentou quadro de agranulocitose refratária ao tratamento. A biópsia de medula revelou medula óssea hipocelular com maturação normal da série granulocítica. A paciente foi sucessivamente tratada com prednisona em altas doses, fator estimulador de colônia de macrófagos e ciclosporina, todos sem resposta hematológica. Micofenolato mofetil (MMF foi iniciado, e após dois meses houve aumento na contagem das células brancas. Após um ano de seguimento a paciente não apresentou novos episódios de neutropenia, nem complicações infecciosas. Concluímos que, na agranulocitose refratária associada à SSp, o tratamento com MMF pode ser uma opção eficaz e bem tolerada.The Sjögren's syndrome (SS is an autoimmune disease characterized by a lymphocytic infiltration of salivary and lacrimal glands. Hematological manifestations of primary SS (pSS usually consist of mild anemia, thrombocytopenia, moderate neutropenia, and lymphopenia. Agranulocytosis is rarely reported and usually responds to immunosuppression. We report the case of a pSS patient who presented with refractory agranulocytosis. Bone marrow biopsy disclosed a hypocellular bone marrow with normal maturation of the granulocytic series. The patient was successively treated with high-dose prednisone, granulocyte-macrophage colony stimulation factor, and cyclosporine, with no hematological response. Mycophenolate mofetil (MMF was initiated and after two months there was a rise on the white blood cell count. After

  17. Is primary Sjögren’s syndrome a risk factor for malignancies different from lymphomas? What does the literature highlight about it?

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    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.

  18. Comparative analysis of the 2016 ACR-EULAR and the 2002 AECG classification criteria for Sjögren's syndrome: Findings from the NIH cohort.

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    Billings, M; Amin Hadavand, M; Alevizos, I

    2018-03-01

    The introduction of new classification criteria for Sjögren's syndrome, known as the 2016 American College of Rheumatology/European League against Rheumatism Classification Criteria (ACR-EULAR), created a need for the evaluation of its performance in an external cohort. The purpose of this study was to compare the performance of the 2016 ACR-EULAR classification set with the widely used American-European Consensus Group Classification criteria (AECG) in the cohort at the National Institutes of Health, USA, and to compare the performance of the sets in classifying both primary and secondary Sjögren's syndrome (pSS and sSS). The study cohort at the NIH (N = 1,303) was enrolled for clinical suspicion of SS. Participants were classified as SS, pSS, and sSS according to both classification sets. Performance of 2016 ACR-EULAR and AECG sets was compared holding each as gold standard to the other. Statistical analysis of test diagnostics and agreement between the two sets were undertaken. By the AECG set, 701 were classified as having SS (627 pSS, 74 sSS) and 714 were classified with SS (647 pSS, 67 sSS) by the 2016 ACR-EULAR set. Sensitivity and specificity of the two sets were comparable in classifying SS, pSS, and sSS. There was high agreement between the two sets for classifying SS (κ = 0.79), pSS (κ = 0.81), and sSS (κ = 0.87). The specificity of the 2016 ACR-EULAR set was significantly higher for classifying sSS than pSS, while the sensitivity was similar for the two disease groups. However, this pattern was also exhibited by the AECG set. There was high agreement between the two classification sets with comparable performance diagnostics. There was no evidence of superior performance value by the new 2016 ACR-EULAR set over the AECG set, and the two sets were found to be equivalent. Findings from our cohort indicate that 2016 ACR-EULAR classification could be extended to classification of sSS. Published 2018. This article is a U.S. Government work and

  19. 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.

  20. Correspondence between salivary proteomic pattern and clinical course in primary Sjögren syndrome and non-Hodgkin's lymphoma: a case report

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    Baldini Chiara

    2011-11-01

    Full Text Available Abstract Background In the last years human proteomic has represented a promising tool to promote the communication between basic and clinical science. Methods To explore the correspondence between salivary proteomic profile and clinical response, herein, we used a proteomic approach to analyse the whole saliva of a patient with primary Sjögren's Syndrome (pSS and non-Hodgkin's-MALT type parotid lymphoma before, during and after a standard treatment with cyclophosphamide (CTX and rituximab (RTX. To identify any discriminatory therapeutic salivary biomarker patient's whole saliva was collected at the baseline, after the fourth infusion of rituximab, and on remission and analysed combining two-dimensional electrophoresis (2DE and MALDI-TOF/TOF mass spectrometry. Results Proteomic results obtained from the comparison of salivary samples indicated several qualitative and quantitative modifications in the salivary expression of putative albumin, immunoglobulin J chain, Ig kappa chain C region, alpha-1-antitrypsin, haptoglobin and Ig alpha-1 chain C region. Conclusion This study suggests that clinical and functional changes of the salivary glands driven by autoimmune and lymphoproliferative processes might be reflected in patients' whole saliva proteins, shedding new light on the potential usefulness of salivary proteomic analysis in the identification of prognostic and therapeutic biomarkers for patients with pSS and non Hodgkin's lymphomas.

  1. 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.

  2. Temperatures of the Ocular Surface, Lid, and Periorbital Regions of Sjögren's, Evaporative, and Aqueous-Deficient Dry Eyes Relative to Normals.

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    Abreau, Kerstin; Callan, Christine; Kottaiyan, Ranjini; Zhang, Aizhong; Yoon, Geunyoung; Aquavella, James V; Zavislan, James; Hindman, Holly B

    2016-01-01

    To compare the temperatures of the ocular surface, eyelid, and periorbital skin in normal eyes with Sjögren's syndrome (SS) eyes, evaporative dry eyes (EDE), and aqueous deficient dry eyes (ADDE). 10 eyes were analyzed in each age-matched group (normal, SS, EDE, and ADDE). A noninvasive infrared thermal camera captured two-dimensional images in three regions of interest (ROI) in each of three areas: the ocular surface, the upper eyelid, and the periorbital skin within a controlled environmental chamber. Mean temperatures in each ROI were calculated from the videos. Ocular surface time-segmented cooling rates were calculated over a 5-s blink interval. Relative to normal eyes, dry eyes had lower initial central OSTs (SS -0.71°C, EDE -0.55°C, ADDE -0.95°C, KW Peyes had the lowest initial central OST (Peyes had the lowest central lid temperature and lower periorbital temperatures (Pdry eye. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Effectiveness of Autologous Serum Eye Drops Combined With Punctal Plugs for the Treatment of Sjögren Syndrome-Related Dry Eye.

    Science.gov (United States)

    Liu, Ying; Hirayama, Masatoshi; Cui, Xin; Connell, Samuel; Kawakita, Tetsuya; Tsubota, Kazuo

    2015-10-01

    To evaluate the efficacy and safety of autologous serum (AS) eye drops combined with punctal plugs (PPs) in patients with Sjögren syndrome (SS)-related dry eye. A retrospective clinical study was performed in patients with dry eye caused by SS. We evaluated the Schirmer test value, tear breakup time (tBUT), and fluorescein and Rose Bengal (RB) staining scores at baseline, 3 months, 6 months, 1 year, and >1 year after treatment. The dry eye indexes were also evaluated in 2 subgroups, which determined by the using of PPs, including the AS + PP group and AS only group. A total of 56 eyes of 28 patients were investigated with a mean follow-up of 42.3 ± 26.1 months. After the application of AS eye drops, the Schirmer test showed no significant changes. The tBUT (2.7 ± 1.9 seconds) was significantly improved at each time point (3.9 ± 3.1, 4.5 ± 3.1, 3.7 ± 2.5, and 5.1 ± 4.0; P eye drops was found to be an effective and apparently safe treatment for SS dry eye. Furthermore, PPs in combination with AS eye drops were considered to have an additive effect on SS dry eye.

  4. Metabolic bone disease as a presenting manifestation of primary Sjögren′s syndrome: Three cases and review of literature

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    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.

  5. IgV peptide mapping of native Ro60 autoantibody proteomes in primary Sjögren's syndrome reveals molecular markers of Ro/La diversification.

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    Wang, Jing J; Al Kindi, Mahmood A; Colella, Alex D; Dykes, Lukah; Jackson, Michael W; Chataway, Tim K; Reed, Joanne H; Gordon, Tom P

    2016-12-01

    We have used high-resolution mass spectrometry to sequence precipitating anti-Ro60 proteomes from sera of patients with primary Sjögren's syndrome and compare immunoglobulin variable-region (IgV) peptide signatures in Ro/La autoantibody subsets. Anti-Ro60 were purified by elution from native Ro60-coated ELISA plates and subjected to combined de novo amino acid sequencing and database matching. Monospecific anti-Ro60 Igs comprised dominant public and minor private sets of IgG1 kappa and lambda restricted heavy and light chains. Specific IgV amino acid substitutions stratified anti-Ro60 from anti-Ro60/La responses, providing a molecular fingerprint of Ro60/La determinant spreading and suggesting that different forms of Ro60 antigen drive these responses. Sequencing of linked anti-Ro52 proteomes from individual patients and comparison with their anti-Ro60 partners revealed sharing of a dominant IGHV3-23/IGKV3-20 paired clonotype but with divergent IgV mutational signatures. In summary, anti-Ro60 IgV peptide mapping provides insights into Ro/La autoantibody diversification and reveals serum-based molecular markers of humoral Ro60 autoimmunity. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Reduction of fatigue in Sjögren syndrome with rituximab: results of a randomised, double-blind, placebo-controlled pilot study.

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    Dass, S; Bowman, S J; Vital, E M; Ikeda, K; Pease, C T; Hamburger, J; Richards, A; Rauz, S; Emery, P

    2008-11-01

    Primary Sjögren syndrome (pSS) causes significant systemic symptoms including fatigue as well as glandular dysfunction. There are currently no effective systemic therapies; however, open label series have suggested that rituximab may be beneficial for systemic and glandular manifestations. Therefore, we performed a double blind, placebo-controlled, randomised pilot study of the efficacy of rituximab in reducing fatigue in pSS. A total of 17 patients with pSS and a score on fatigue visual analogue scale (VAS) >50 were randomised to receive either 2 infusions of rituximab 1 g or placebo; patients also received oral and intravenous steroids. Outcome measures included: the proportion of patients with >20% reduction in fatigue VAS, changes in pSS related symptoms, health related quality of life and immunological parameters of pSS. These were measured 6 months after therapy. There was significant improvement from baseline in fatigue VAS in the rituximab group (p<0.001) in contrast to the placebo group (p = 0.147). There was a significant difference between the groups at 6 months in the social functioning score of SF-36 (p = 0.01) and a trend to significant difference in the mental health domain score of SF-36 (p = 0.06). There was one episode of serum sickness in the rituximab treated group. This is the first double blind study of rituximab in pSS to show benefit; further studies are justified.

  7. Targeting the Ca2+ Sensor STIM1 by Exosomal Transfer of Ebv-miR-BART13-3p is Associated with Sjögren's Syndrome

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    Alessia Gallo

    2016-08-01

    Full Text Available Primary Sjögren's syndrome (pSS is a systemic autoimmune disease that is associated with inflammation and dysfunction of salivary and lacrimal glands. The molecular mechanism(s underlying this exocrinopathy is not known, although the syndrome has been associated with viruses, such as the Epstein Barr Virus (EBV. We report herein that an EBV-specific microRNA (ebv-miR-BART13-3p is significantly elevated in salivary glands (SGs of pSS patients and we show that it targets stromal interacting molecule 1 (STIM1, a primary regulator of the store-operated Ca2+ entry (SOCE pathway that is essential for SG function, leading to loss of SOCE and Ca2+-dependent activation of NFAT. Although EBV typically infects B cells and not salivary epithelial cells, ebv-miR-BART13-3p is present in both cell types in pSS SGs. Importantly, we further demonstrate that ebv-miR-BART13-3p can be transferred from B cells to salivary epithelial cells through exosomes and it recapitulates its functional effects on calcium signaling in a model system.

  8. Anti-inflammatory effects of rebamipide eyedrop administration on ocular lesions in a murine model of primary Sjögren's syndrome.

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    Rieko Arakaki

    Full Text Available Topical therapy is effective for dry eye, and its prolonged effects should help in maintaining the quality of life of patients with dry eye. We previously reported that the oral administration of rebamipide (Reb, a mucosal protective agent, had a potent therapeutic effect on autoimmune lesions in a murine model of Sjögren's syndrome (SS. However, the effects of topical treatment with Reb eyedrops on the ocular lesions in the murine model of SS are unknown.Reb eyedrops were administered to the murine model of SS aged 4-8 weeks four times daily. Inflammatory lesions of the extraorbital and intraorbital lacrimal glands and Harderian gland tissues were histologically evaluated. The direct effects of Reb on the lacrimal glands were analyzed using cultured lacrimal gland cells. Tear secretions of Reb-treated mice were significantly increased compared with those of untreated mice. In addition to the therapeutic effect of Reb treatment on keratoconjunctivitis, severe inflammatory lesions of intraorbital lacrimal gland tissues in this model of SS were resolved. The mRNA expression levels of IL-10 and mucin 5Ac in conjunctival tissues from Reb-treated mice was significantly increased compared with those of control mice. Moreover, lactoferrin production from lacrimal gland cells was restored by Reb treatment.Topical Reb administration had an anti-inflammatory effect on the ocular autoimmune lesions in the murine model of SS and a protective effect on the ocular surfaces.

  9. 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......SS) between the sexes and establish whether male sex is associated with a more severe form of long-term pSS. METHODS: Our study population included 967 patients with pSS (899 females and 68 males) from Scandinavian clinical centers. The mean follow-up time (years) was 8.8 ± 7.6 for women and 8.5 ± 6.2 for men...... (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...

  10. 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

    2014-02-01

    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.

  11. 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.

  12. 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.

  13. Vaginoperineal Fistula as a Complication of Perianal Surgery in a Patient with Sjögren’s Syndrome: A Case Report

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    Kemal Beksac

    2014-01-01

    Full Text Available Forty-seven-year-old woman with Sjögren’s syndrome had been operated on because of transsphincteric perianal fistula secondary to perianal abscess. Vaginal wall injury occurred during the course of the operation and injured tissue was repaired primarily. Three months later, patient suffered from the recurrence of perianal fistula symptoms and fistulectomy was performed once again under antibiotic suppression. Several months later, perineal discharge continued, and, therefore, patient was admitted to the hospital for the third time and a fistulotomy was performed. Two months after the third operation, patient was admitted with leukorrhea and a perineovaginal fistula was detected. This time, not only her surgical problem but also her immune system disorder was considered in the preoperative workup. Then, patient was hospitalized for the fourth time and “fistulectomy/perineoplasty” was performed successfully. We believe that patients with autoimmune disorders with or without medical treatment may have healing problems during the course of surgical processes and therefore such medical problems must be taken into consideration by the surgeons.

  14. Characterisation of a novel Bacillus sp. SJ-10 β-1,3-1,4-glucanase isolated from jeotgal, a traditional Korean fermented fish.

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    Kim, Yu-Ri; Kim, Eun-Young; Lee, Jong Min; Kim, Joong Kyun; Kong, In-Soo

    2013-06-01

    A novel β-1,3-1,4-glucanase gene was identified in Bacillus sp. SJ-10 (KCCM 90078) isolated from jeotgal, a traditional Korean fermented fish. We analysed the β-1,3-1,4-glucanase gene sequence and examined the recombinant enzyme. The open reading frame of the gene encoded 244 amino acids. The sequence was not identical to any β-glucanases deposited in GenBank. The gene was cloned into pET22b(+) and expressed in Escherichia coli BL21. Purification of recombinant β-1,3-1,4-glucanase was conducted by affinity chromatography using a Ni-NTA column. Enzyme specificity of β-1,3-1,4-glucanase was confirmed based on substrate specificity. The optimal temperature and pH of the purified enzyme towards barley β-glucan were 50 °C and pH 6, respectively. More than 80 % of activity was retained at temperatures of 30-70 °C and pH values of 4-9, which differed from all other bacterial β-1,3-1,4-glucanases. The degradation products of barley β-glucan by β-1,3-1,4-glucanase were analysed using thin-layer chromatography, and ultimately glucose was produced by treatment with cellobiase.

  15. 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.

  16. Kritisk självreflektion i komplexa frågor: Att hjälpa studenterna att ta makten över sitt tänkande

    Directory of Open Access Journals (Sweden)

    Maria Wolrath Söderberg

    2017-12-01

    Full Text Available Den här artikeln handlar om studenters kritiska självreflektion. Utgångspunkten är att de former av kritiskt tänkande som vi traditionellt odlar inom högskolan är otillräckliga när det gäller vår tids kritiska frågor, som exempelvis klimatfrågan. När det rör sig om komplexa och värdeladdade frågor där många perspektiv kan vara giltiga har vi att förhålla oss till olika mänskliga mekanismer som på olika vis hindrar oss från att tänka och agera klokt, exempelvis polarisering, bekräftelsebias, glapp mellan tanke och handling och olika former av legitimeringsstrategier för att låta blir att ta ansvar. Här diskuteras sådana hinder och hur vi kan förhålla oss till dem i högre utbildning.

  17. Functional effects of proinflammatory factors present in Sjögren's syndrome salivary microenvironment in an in vitro model of human salivary gland.

    Science.gov (United States)

    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.

  18. Significance and Implications of Patient-reported Xerostomia in Sjögren's Syndrome: Findings From the National Institutes of Health Cohort.

    Science.gov (United States)

    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

  19. Functional and morphological evaluation of hand microcirculation with nailfold capillaroscopy and laser Doppler imaging in Raynaud's and Sjögren's syndrome and poly/dermatomyositis.

    Science.gov (United States)

    Szabo, N; Csiki, Z; Szanto, A; Danko, K; Szodoray, P; Zeher, M

    2008-01-01

    Nailfold capillaroscopy is widely used in autoimmune patients to determine capillary morphology. Laser Doppler imaging (LDI) is a relatively new method for measuring the microcirculation of cutaneous perfusion. The aim of this study was to investigate the capillary morphology and microcirculation among patients with Sjögren's syndrome (SS) and poly/dermatomyositis (PM/DM) with these two non-invasive methods and to detect secondary Raynaud's syndrome (SRS) in these autoimmune diseases. Thirty patients with primary SS, 30 patients with PM/DM, 30 patients with primary Raynaud's syndrome (PRS), and 30 healthy volunteers were included in the study. Nailfold capillaroscopy and LDI were performed on each patient. A comprehensive analysis was performed among the patients and healthy individuals. Among SS patients avascularity and among PM/DM patients avascularity and capillary morphology changes were most often detected by capillaroscopy. With LDI the mean steady-state cutaneous perfusion was 1.25 perfusion units (PU) in region of interest 1 (ROI1), 1.22 in ROI2, and 1.49 at the fingertips in PRS patients; the corresponding values were 1.2, 1.03, and 1.48 PU in SS, 0.91, 0.76, and 1.19 PU in PM/DM, and 1.79, 1.62, and 2.2 PU in the controls. The differences were significant between each autoimmune group compared to the control group (pnailfold capillaroscopy, abnormalities in capillary morphology can be detected, and by using LDI, the reduced blood flow in the capillaries can be detected. These investigations can be useful in the detection of SRS, or in distinguishing whether the reduced blood flow is due to primary/systemic autoimmune diseases.

  20. 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.

  1. Isolated congenital heart block in undifferentiated connective tissue disease and in primary Sjögren’s syndrome: a clinical study of 81 pregnancies in 41 patients

    Directory of Open Access Journals (Sweden)

    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.

  2. Comparative Evaluation of Silicone Hydrogel Contact Lenses and Autologous Serum for Management of Sjögren Syndrome-Associated Dry Eye.

    Science.gov (United States)

    Li, Jinyang; Zhang, Xiahua; Zheng, Qinxiang; Zhu, Yirui; Wang, Hui; Ma, Huixiang; Jhanji, Vishal; Chen, Wei

    2015-09-01

    To comparatively evaluate the efficacy of a bandage contact lens (BCL) and autologous serum (AS) eye drops in the management of severe dry eye caused by Sjögren syndrome (SS). In this prospective randomized study, 40 patients with SS were enrolled. Patients were divided into 2 treatment groups: BCL and AS. A total of 37 patients were included, 18 patients (35 eyes) in the AS group and 19 patients (36 eyes) in the BCL group. At the end of 6 weeks, the best-corrected visual acuity improved significantly in the BCL group (0.5 ± 0.3 vs. 0.3 ± 0.2, P = 0.003) but not in the AS group (0.4 ± 0.3 vs. 0.3 ± 0.3, P = 0.11). The best-corrected visual acuity remained stable up to 6 weeks after discontinuation of the BCL (0.5 ± 0.3 vs. 0.4 ± 0.2, P = 0.03). Although the Ocular Surface Disease Index scores decreased significantly after treatment in both groups, patients in the BCL group had lower Ocular Surface Disease Index scores than those in the AS group (53.4 vs. 41.8 at week 3, 47.1 vs. 31.0 at week 6, 52.7 vs. 32.0 at week 12; P = 0.014, dry eye. Clinical Trial Registration—URL: http://www.clinicaltrials.gov. Unique identifier: NCT02147509.

  3. Effect of Hydroxychloroquine Treatment on Dry Eyes in Subjects with Primary Sjögren's Syndrome: a Double-Blind Randomized Control Study.

    Science.gov (United States)

    Yoon, Chang Ho; Lee, Hyun Ju; Lee, Eun Young; Lee, Eun Bong; Lee, Won-Woo; Kim, Mee Kum; Wee, Won Ryang

    2016-07-01

    The effect of hydroxychloroquine (HCQ) on dry eye has not been fully determined. This study aimed to compare the 12-week efficacy of HCQ medication with that of a placebo in the management of dry eye in primary Sjögren's syndrome (pSS). A double-blind, randomized control study was conducted in 39 pSS subjects from May 2011 through August 2013. pSS was diagnosed based on the classification criteria of the American-European Consensus Group. Subjects received 300 mg of HCQ or placebo once daily for 12 weeks and were evaluated at baseline, 6, and 12 weeks, with a re-visit at 16 weeks after drug discontinuance. The fluorescein staining score, Schirmer test score, tear film break-up time (TBUT), and ocular surface disease index (OSDI) were measured, and tears and blood were collected for ESR, IL-6, IL-17, B-cell activating factor (BAFF), and Th17 cell analysis. Color testing was performed and the fundus was examined to monitor HCQ complications. Twenty-six subjects completed the follow-up. The fluorescein staining score and Schirmer test score did not differ significantly. The OSDI improved with medication in the HCQ group but was not significantly different between the groups. TBUT, serum IL-6, ESR, serum and tear BAFF, and the proportion of Th17 cells did not change in either group. HCQ at 300 mg daily for 12 weeks has no apparent clinical benefit for dry eye and systemic inflammation in pSS (ClinicalTrials.gov. NCT01601028).

  4. Safety and efficacy of 0.1% clobetasone butyrate eyedrops in the treatment of dry eye in Sjögren syndrome.

    Science.gov (United States)

    Aragona, Pasquale; Spinella, Rosaria; Rania, Laura; Postorino, Elisa; Sommario, Margherita S; Roszkowska, Anna M; Puzzolo, Domenico

    2013-01-01

    To study the effects of a low administration rate and low concentration (0.1%) of clobetasone butyrate eyedrops in patients with Sjögren syndrome (SS).
 This prospective, double-masked, randomized, placebo-controlled study included 40 subjects divided into 2 treatment groups: group 1 (2% polyvinylpyrrolidone eyedrops and placebo) and group 2 (2% polyvinylpyrrolidone and 0.1% clobetasone butyrate, 1 drop BID). The treatment lasted for 30 days, with visits at enrollment, baseline, day 15, day 30, and after 15 days of treatment discontinuation. At each visit, symptoms questionnaire, tear film break-up time, corneal fluorescein stain, lissamine green stain, conjunctival impression cytology for human leukocyte antigen-DR (HLA-DR) expression, intraocular pressure (IOP) measurement, and fundus examination were performed. 
 No changes in IOP or fundus examination were observed in either group at each time point. Group 1 patients showed at day 30 a statistically significant amelioration of symptoms and reduction of HLA-DR expression. No changes in other parameters were detected. Group 2 patients showed at day 15 a statistically significant improvement of corneal and conjunctival stain versus baseline values and group 1 at the same time; after 30 days the symptoms score was statistically significantly better than baseline values and group 1 at the same time. The HLA-DR expression and the epithelial cells area were statistically significantly reduced versus baseline and group 1 at the same time. 
 Anti-inflammatory therapy is critical for the treatment of SS dry eye. Clobetasone butyrate, at low dosage, proved to be safe and effective in treating this condition.

  5. CXCL13 levels in serum but not in saliva are elevated in Asian Indian patients with primary Sjögren's syndrome.

    Science.gov (United States)

    Mandal, Santosh Kumar; Sandhya, Pulukool; Kabeerdoss, Jayakanthan; Ramya, Janardana; Mahasampath, Gowri; Danda, Debashish

    2018-05-01

    Human and animal model studies suggest CXCL13 is a potential biomarker in primary Sjögren's syndrome (pSS). CXCL13 has not been studied in Indian patients with pSS. pSS cases classified by American European Consensus Group (AECG) or American college of Rheumatology(ACR) 2012 criteria, attending rheumatology clinic between July 2014 and July 2015 were included. Hospital staff and healthy, non-blood related family members of patients constituted the control group. pSS cases underwent clinical evaluation, laboratory investigations, ESSDAI and ESSPRI scoring. Unstimulated saliva was collected by the spitting method. Salivary and serum CXCL13 were quantified by indirect ELISA. CXCL13 positivity was determined using Receiver Operator Characteristic (ROC) curve. STATA13.1 (StataCorpLP,Texas,USA) software was used for statistical analysis. In this study, 45 pSS cases and 42 healthy controls were recruited. In pSS, median levels of serum CXCL13, but not salivary CXCL13 was significantly higher as compared to the corresponding levels in healthy controls (p associated with oral symptoms (p = 0.02), ocular signs (p = 0.03) and hyperglobulinemia (p = 0.01). There was no association of salivary CXCL13 level with any of the clinical variables. While serum CXCL13 was elevated in pSS, salivary CXCL13 was not. In conclusion, serum CXCL13 positivity was found to be associated with oral symptoms, ocular signs and hyperglobulinemia in pSS.

  6. 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.

  7. 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.

  8. 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

  9. Effect of Hydroxychloroquine Treatment on Dry Eyes in Subjects with Primary Sjögren’s Syndrome: a Double-Blind Randomized Control Study

    Science.gov (United States)

    2016-01-01

    The effect of hydroxychloroquine (HCQ) on dry eye has not been fully determined. This study aimed to compare the 12-week efficacy of HCQ medication with that of a placebo in the management of dry eye in primary Sjögren's syndrome (pSS). A double-blind, randomized control study was conducted in 39 pSS subjects from May 2011 through August 2013. pSS was diagnosed based on the classification criteria of the American-European Consensus Group. Subjects received 300 mg of HCQ or placebo once daily for 12 weeks and were evaluated at baseline, 6, and 12 weeks, with a re-visit at 16 weeks after drug discontinuance. The fluorescein staining score, Schirmer test score, tear film break-up time (TBUT), and ocular surface disease index (OSDI) were measured, and tears and blood were collected for ESR, IL-6, IL-17, B-cell activating factor (BAFF), and Th17 cell analysis. Color testing was performed and the fundus was examined to monitor HCQ complications. Twenty-six subjects completed the follow-up. The fluorescein staining score and Schirmer test score did not differ significantly. The OSDI improved with medication in the HCQ group but was not significantly different between the groups. TBUT, serum IL-6, ESR, serum and tear BAFF, and the proportion of Th17 cells did not change in either group. HCQ at 300 mg daily for 12 weeks has no apparent clinical benefit for dry eye and systemic inflammation in pSS (ClinicalTrials.gov. NCT01601028). PMID:27366013

  10. Marcadores morfológicos y métricos del sexo en un conjunto osteológico del formativo de Tilcara (Sitio SJ Til. 20

    Directory of Open Access Journals (Sweden)

    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.

  11. The Prevalence of Dental Implants and Related Factors in Patients with Sjögren Syndrome: Results from a Cohort Study.

    Science.gov (United States)

    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.

  12. Characteristics of primary Sjögren's syndrome patients with IgG4 positive plasma cells infiltration in the labial salivary glands.

    Science.gov (United States)

    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.

  13. 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.

  14. Identification of a Sjögren's syndrome susceptibility locus at OAS1 that influences isoform switching, protein expression, and responsiveness to type I interferons.

    Directory of Open Access Journals (Sweden)

    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.

  15. 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; Wang, Shaofeng; He, Bo; Grundahl, Kiely M.; Glenn, Stuart B.; Miceli-Richard, Corinne; Bowman, Simon; Lester, Sue; Eriksson, Per; Brun, Johan G.; Gøransson, Lasse G.; Harboe, Erna; Guthridge, Joel M.; 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.; Hughes, Pamela J.; Lewis, David M.; Radfar, Lida; Vista, Evan S.; Rohrer, Michael D.; Stone, Donald U.; Vyse, Timothy J.; Harley, John B.; 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; Witte, Torsten; Rischmueller, Maureen; Omdal, Roald; Lessard, Christopher J.; Sivils, Kathy L.

    2017-01-01

    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. PMID

  16. 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.

  17. 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

  18. 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.

  19. Developing a service user informed intervention to improve participation and ability to perform daily activities in primary Sjögren's syndrome: a mixed-methods study protocol.

    Science.gov (United States)

    Hackett, Katie L; Newton, Julia L; Deane, Katherine H O; Rapley, Tim; Deary, Vincent; Kolehmainen, Niina; Lendrem, Dennis; Ng, Wan-Fai

    2014-08-21

    A significant proportion of patients with primary Sjögren's syndrome (PSS) is functionally impaired and experience difficulties participating in various aspects of everyday life. There is currently no evidence of efficacy for non-pharmacological interventions aimed specifically at supporting the patients with PSS to improve their participation and ability to perform daily activities. This paper describes a research protocol for a mixed-methods study to develop an intervention to improve these outcomes. The protocol follows the Medical Research Council framework for complex interventions. We will use group concept mapping with the patients, adults who live with them and healthcare professionals to identify factors which prevent people with PSS from participating in daily life and performing daily activities. The factors will be prioritised by participants for importance and feasibility and will inform an intervention to be delivered within a National Health Service (NHS) setting. Evidence-based intervention techniques will be identified for the prioritised factors and combined into a deliverable intervention package. Key stakeholders will comment on the intervention content and mode of delivery through focus groups, and the data will be used to refine the intervention. The acceptability and feasibility of the refined intervention will be evaluated in a future study. The study has been approved by an NHS Research Ethics Committee, REC Reference: 13/NI/0190. The findings of this study will be disseminated in peer-reviewed journals and through presentation at national and international conferences. UKCRN Study ID: 15939. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  20. An in silico approach reveals associations between genetic and epigenetic factors within regulatory elements in B cells from primary Sjögren’s syndrome patients

    Directory of Open Access Journals (Sweden)

    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.

  1. Reversible lacrimal gland-protective regulatory T-cell dysfunction underlies male-specific autoimmune dacryoadenitis in the non-obese diabetic mouse model of Sjögren syndrome

    Science.gov (United States)

    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

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

    OpenAIRE

    Oulego, Daniela

    2016-01-01

    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 a...

  3. Reprodutibilidade na classificação do teste de cristalização do filme lacrimal em pacientes com síndrome de Sjögren Reproducibility of the classification of ocular ferning patterns in Sjogren's syndrome patients

    Directory of Open Access Journals (Sweden)

    Sergio Felberg

    2008-04-01

    Full Text Available OBJETIVO: Verificar a reprodutibilidade da classificação dos padrões do teste de cristalização do filme lacrimal utilizando cinco examinadores diferentes e comparar os padrões de cristalização de pacientes portadores da síndrome de Sjögren com os de indivíduos não portadores de doenças da superfície ocular. MÉTODOS: Análise da cristalização da lágrima de 29 pacientes com Sjögren e 45 pacientes sem doenças da superfície ocular, através de microscópio com luz polarizada, utilizando a classificação de Rolando. Para fins estatísticos foi estudada a curva ROC (Receiver Operating Characteristic para determinar a melhor nota de corte do exame que separa indivíduos normais dos portadores da síndrome, índice de concordância Kappa (pPURPOSE: To verify the reproducibility of Rolando's classification of the tear ferning test using five different examiners and to compare the patterns of crystallization found in Sjögren's syndrome patients and normal subjects. METHODS: Tear ferning analysis of 29 patients with Sjögren's syndrome and of 45 patients without ocular disease were done using polarized light microscopy and the Rolando classification for tear ferning. Five examiners classified the ferning patterns of all the patients. ROC curve (Receiver Operating Characteristic was used to find out the best score for the correct syndrome diagnosis. Kappa index (p<0.0001 was used to compare the results of the examiners among them and check the test's reproducibility. Charts were drawn to compare the two groups' results. RESULTS: Throught the ROC curve the score of 2.50 for diagnosis of Sjögren's syndrome was stabilished. Considering the aggregated patterns I with II and III with IV, the examinors' level of pattern agreement was excellent (Kappa ranging from 0.82 to 0.97, p<0.0001. The group with Sjögren's syndrome was classified mostly as patterns III and IV and the patients without ocular disease mostly as I and II. CONCLUSION: The

  4. Automated Measurement of Tear Film Dynamics and Lipid Layer Thickness for Assessment of Non-Sjögren Dry Eye Syndrome With Meibomian Gland Dysfunction.

    Science.gov (United States)

    Ji, Yong Woo; Lee, Jeihoon; Lee, Hun; Seo, Kyoung Yul; Kim, Eung Kweon; Kim, Tae-Im

    2017-02-01

    To investigate automated values from an advanced corneal topographer with a built-in real keratometer, color camera, and ocular surface interferometer for the evaluation of non-Sjögren dry eye syndrome (NSDES) with meibomian gland dysfunction (MGD). Sixty-four patients (64 eyes) diagnosed with NSDES with MGD were enrolled. All eyes were evaluated using the Ocular Surface Disease Index (OSDI), fluorescence staining score, tear film breakup time (TBUT), Schirmer test, and MGD grade. Noninvasive Keratograph average tear film breakup time (NIKBUTav), tear meniscus height (TMHk), meibomian gland (MG) dropout grade, and lipid layer thickness (LLT) using interferometry were measured. Among automated indexes, NIKBUTav (mean 7.68 ± 4.07 s) and the MG dropout grade (mean 1.0 ± 0.5) significantly correlated with the OSDI (mean 40.6 ± 22.9) (r = -0.337, P = 0.006; and r = 0.201, P = 0.023, respectively), as did all conventional indicators, except the Schirmer score (mean 9.1 ± 5.9 mm). TMHk (mean 0.21 ± 0.18 mm) had significant correlation with the Schirmer score, the staining score (mean 1.2 ± 0.7), TBUT (mean 3.8 ± 1.8 s), and NIKBUTav (r = 0.298, P = 0.007; r = -0.268, P = 0.016; r = 0.459, P < 0.001; and r = 0.439, P < 0.001, respectively), but not any MGD indicator, even the MG dropout grade. NIKBUTav showed significant correlations with all clinical parameters and other automated values, except the Schirmer score and LLT (mean 83.94 ± 20.82 nm) (all (Equation is included in full-text article.)≥ 0.25 and P < 0.01). The MG dropout grade highly correlated with all indexes except TMHk (all (Equation is included in full-text article.)≥ 0.25 and P < 0.05). LLT was significantly associated with TBUT, MGD grade (mean 2.0 ± 0.7), and MG dropout grade (r = 0.219, P = 0.047; r = -0.221, P = 0.039; and r = 0.433, P < 0.001, respectively), although it was not related to patient symptoms. Automated noninvasive measurements using an advanced corneal topographer and LLT

  5. New epitopes and function of anti-M3 muscarinic acetylcholine receptor antibodies in patients with Sjögren's syndrome.

    Science.gov (United States)

    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.

  6. [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.

  7. 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

  8. 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.

  9. B cell subsets and dysfunction of regulatory B cells in IgG4-related diseases and primary Sjögren’s syndrome: the similarities and differences

    Science.gov (United States)

    2014-01-01

    Introduction IgG4-related disease (IgG4-RD) is a multisystem-involved autoimmune disease. Abnormally activated and differentiated B cells may play important roles. Regulatory B cells (Breg) are newly defined B cell subgroups with immunosuppressive functions. In this study, we investigated the differences of B cell subsets, the expressions of co-stimulatory molecules on B cells, and the function of Breg cells in patients with IgG4-RD, primary Sjögren’s syndrome (pSS) as well as in healthy controls (HC). Methods Newly diagnosed IgG4-RD patients (n = 48) were enrolled, 38 untreated pSS patients and 30 healthy volunteers were recruited as disease and healthy controls. To analyze B cell subsets and B cell activity, PBMCs were surface stained and detected by flow cytometry. The function of Breg cells was tested by coculturing isolated CD19 + CD24hiCD38hi Breg cells with purified CD4 + CD25- T cells. Serum cytokines were measured by ELISA and cytometric bead array. Relationship between clinical data and laboratory findings were analyzed as well. Results Compared with pSS patients and HC, IgG4-RD patients had a lower frequency of peripheral Breg cells. Interestingly, CD19 + CD24-CD38hi B cell subsets were significantly higher in peripheral B cells from IgG4-RD patients than in pSS patients and HC, which correlated with serum IgG4 levels. The expression of BAFF-R and CD40 on B cells was significantly lower in IgG4-RD patients compared with those in pSS patients and HC. Unlike HC, Breg cells from pSS patients lacked suppressive functions. Conclusions B cells in patients with IgG4-RD and pSS display a variety of abnormalities, including disturbed B cell subpopulations, abnormal expression of key signaling molecules, co-stimulatory molecules, and inflammatory cytokines. In addition, a significantly increased B cell subset, CD19 + CD24-CD38hi B cells, may play an important role in the pathogenesis of IgG4-RD. PMID:24887143

  10. 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

  11. Sjögren's Syndrome

    Science.gov (United States)

    ... OII) Timed Up & Go (TUG) Western Ontario & McMaster Universities Osteoarthritis Index (WOMAC) Young Investigators Resources for Doctoral Students/Post-Doctoral Fellows Evidence-Based Practice for Academic Researchers Responsible Data Management in Research Career Planning Treatments Patient ...

  12. 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

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    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

  13. 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.

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    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.

  14. 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

  15. Correlation between intravoxel incoherent motion MR parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome: A pilot study

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    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

  16. Effect of Autologous Serum Eye Drops in Patients with Sjögren Syndrome-related Dry Eye: Clinical and In Vivo Confocal Microscopy Evaluation of the Ocular Surface.

    Science.gov (United States)

    Semeraro, Francesco; Forbice, Eliana; Nascimbeni, Giuseppe; Taglietti, Marco; Romano, Vito; Guerra, Germano; Costagliola, Ciro

    To evaluate in vivo changes after therapy using autologous serum (AS) eye drops in Sjögren's syndrome (SS)-related dry eyes by confocal microscopy. In this study, 24 patients with SS-related dry eyes [12 in AS eye drop therapy and 12 in artificial tear (AT) therapy] and 24 healthy volunteers were recruited. Ocular Surface Disease Index (OSDI), central corneal thickness, tear film, break-up time, corneal and conjunctival staining, Schirmer's test and corneal confocal microscopy were investigated. Tear production, tear stability, corneal staining, inflammation, and central corneal thickness, Langherans cells, activated keratocytes, intermediate epithelial cell density, nerve tortuosity, number of sub-basal nerve branches, and number of bead-like formations differed between patients and controls (peye drops improve symptoms and confocal microscopy findings in SS-related dry eyes. Copyright © 2016 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  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. 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.

  19. Interferon gamma-inducible protein 16 (IFI16 and anti-IFI16 antibodies in primary Sjögren’s syndrome: findings in serum and minor salivary glands

    Directory of Open Access Journals (Sweden)

    A. Alunno

    2016-02-01

    Full Text Available The interferon (IFN signature, namely the overexpression of IFN-inducible genes is a crucial aspect in the pathogenesis of primary Sjögren’s syndrome (pSS. The IFN-inducible IFI16 protein, normally expressed in cell nuclei, may be overexpressed, mislocalized in the cytoplasm and secreted in the extracellular milieu in several autoimmune disorders including pSS. This leads to tolerance breaking to this self-protein and development of anti-IFI16 antibodies. The aim of this study was to identify pathogenic and clinical significance of IFI16 and anti-IFI16 autoantibodies in pSS. IFI16 and anti-IFI16 were assessed in the serum of 30 pSS patients and one-hundred healthy donors (HD by ELISA. IFI16 was also evaluated in 5 minor salivary glands (MSGs of pSS patients and 5 MSGs of non-pSS patients with sicca symptoms by immunohistochemistry. Normal MSGs do not constitutively express IFI16. Conversely, in pSS-MSGs a marked expression and cytoplasmic mislocalization of IFI16 by epithelial cells was observed with infiltrations in lymphocytes and peri/ intra-lesional endothelium. pSS patients display higher serum levels of both IFI16 and anti-IFI16 autoantibodies compared to HD. Our data suggest that IFI16 protein may be involved in the initiation and perpetuation of glandular inflammation occurring in pSS.

  20. 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.

  1. Anti-M(3) muscarinic cholinergic autoantibodies from patients with primary Sjögren's syndrome trigger production of matrix metalloproteinase-3 (MMP-3) and prostaglandin E(2) (PGE(2)) from the submandibular glands.

    Science.gov (United States)

    Reina, Silvia; Sterin-Borda, Leonor; Passafaro, Daniela; Borda, Enri

    2011-05-01

    We demonstrated that serum immunoglobulin G (IgG) from patients with primary Sjögren's syndrome (pSS), interacting with the second extracellular loop of human glandular M(3) muscarinic acetylcholine receptors (M(3) mAChR), trigger the production of matrix metalloproteinase-3 (MMP-3) and prostaglandin E(2) (PGE(2)). Enzyme-linked immunosorbent assays (ELISAs) were performed in the presence of M(3) mAChR synthetic peptide as antigen to detect in serum the autoantibodies. Further, MMP-3 and PGE(2) production were determined in the presence of anti-M(3) mAChR autoantibodies. An association was observed between serum and anti-M(3) mAChR autoantibodies and serum levels of MMP-3 and PGE(2) in pSS patients. Thus, we established that serum anti-M(3) mAChR autoantibodies, MMP-3 and PGE(2) may be considered to be early markers of pSS associated with inflammation. Affinity-purified anti-M(3) mAChR peptide IgG from pSS patients, whilst stimulating salivary-gland M(3) mAChR, causes an increase in the level of MMP-3 and PGE(2) as a result of the activation of phospholipase A(2) (PLA(2)) and cyclooxygenase-2 (COX-2) (but not COX-1). These results provide a novel insight into the role that cholinoceptor antibodies play in the development of glandular inflammation. This is the first report showing that an antibody interacting with glandular mAChR can induce the production of pro-inflammatory mediators (MMP-3/PGE(2)). Copyright © 2010 Elsevier Ltd. All rights reserved.

  2. 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.

  3. 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.

  4. African-American English i direkt anföring : Etta James självbiografi översatt till svenska – att anpassa översättningen efter en varietet som inte har någon motsvarighet på svenska

    OpenAIRE

    Anteryd, Linn

    2015-01-01

    Detta examensarbete är indelat i tre delar. Examensarbetet består av en översättning av tre kapitel ur Etta James självbiografi Rage to survive: the Etta James story skriven av James och spökförfattaren David Ritz, samt ett kapitel om vilka textspecifika översättningsproblem som uppstod vid översättningen. Den tredje delen består av en djupanalys av hur varieteten African-American English (AAE) översatts i två andra verk, nämligen I know why the caged bird sings av Maya Angelou och The bluest...

  5. 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.

  6. 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...

  7. What Is Sjögren's Syndrome?

    Science.gov (United States)

    ... as corticosteroids). Medicines that suppress the immune system. Treatment for dry eyes may include: Eye drops that come in different ... the tear ducts that drain tears from the eye. Treatment for dry mouth may include: Chewing gum or sucking on ...

  8. 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

  9. 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

  10. 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

  11. CalCOFI Larvae Counts, Scientific Names SJ to ST

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Fish larvae counts and standardized counts for larvae captured in CalCOFI icthyoplankton nets (primarily vertical [Calvet or Pairovet], oblique [bongo or ring nets],...

  12. 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 inddrag...

  13. Nye steder med liv og sjæl?

    DEFF Research Database (Denmark)

    Stender, Marie

    planlæggere, der har skabt dem, udforskes stedernes tilblivelsesproces og sociale liv. Det nye sted opfattes af både beboere og professionelle som en bar mark, og mange kræfter sættes ind på at genfortrylle det med et skær af liv, identitet og historie fra start. Hånd i hånd med branding, virtuelle fora...

  14. Biologic treatment in Sjögren's syndrome.

    Science.gov (United States)

    Sada, Pablo Ruiz; Isenberg, David; Ciurtin, Coziana

    2015-02-01

    SS is a chronic systemic autoimmune disease characterized by decreased exocrine gland function. A variety of other disease manifestations may also be present, including general constitutional symptoms and extraglandular features. A multidisciplinary approach focused on both local and systemic medical therapies is needed as the disease has a wide clinical spectrum. The current treatment for SS is mainly symptomatic. However, there is evidence that systemic drugs are effective in controlling extraglandular manifestations of the disease. Overall evidence for the role of conventional immunosuppressive therapy is limited. A number of attempts to use biologic therapies have led to variable results. Biologic agents targeting B cells, such as rituximab, epratuzumab and belimumab, have shown promising results, but further studies are needed to validate the findings. Early-phase studies with abatacept and alefacept proved that T cell stimulation inhibition is another potentially effective target for SS treatment. Modulation or inhibition of other targets such as IFN, IL-6 and Toll-like receptor are also currently being investigated. We have summarized the available evidence regarding the efficacy of biologic treatments and discuss other potential therapies targeting pathways or molecules recognized as being involved in the pathogenesis of SS. © 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.

  15. LOUVANDO (A DEUS EM ULPIANO VÁZQUEZ MORO SJ

    Directory of Open Access Journals (Sweden)

    Maria Clara Lucchetti Bingemer

    2016-01-01

    Full Text Available Vou fazer a louvação Louvação, louvação Do que deve ser louvado Ser louvado, ser louvado Meu povo, preste atenção Atenção, atenção Repare se estou errado Louvando o que bem merece Deixo o que é ruim de lado. (Gilberto Gil e Torquato Neto

  16. Genetics Home Reference: Sjögren syndrome

    Science.gov (United States)

    ... J. 2010 Jun;55 Suppl 1:39-47. doi: 10.1111/j.1834-7819.2010.01197.x. ... Am. 2008 Nov;34(4):847-68, vii. doi: 10.1016/j.rdc.2008.08.003. Review. ... Am. 2008 Nov;34(4):833-45, vii. doi: 10.1016/j.rdc.2008.08.004. Review. ...

  17. 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.

  18. Genetics Home Reference: Marinesco-Sjögren syndrome

    Science.gov (United States)

    ... abnormalities including short stature and a spine that curves to the side ( scoliosis ). Other features of Marinesco- ... Information What information about a genetic condition can statistics provide? Why are some genetic conditions more common ...

  19. Hammarby Sjöstad in Stockholm a model case?

    DEFF Research Database (Denmark)

    Grønlund, Bo

    2010-01-01

    central government agencies have contributed to put these guidelines in practice. Representatives have also taken part in similar work at CEN in Brussels. In Sweden, the Stockholm Police started to work out guidelines and give advice - with the mixed new urban district Ärvinge near Kista as their primary...

  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...

  1. 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.

  2. Comparison of Distinguishing Characteristics between Primary Sjögren’s Syndrome and Non-Primary Sjögren’s Syndrome Patients

    DEFF Research Database (Denmark)

    Pedersen, Anne Marie Lynge; Sembler-Møller, Maria Lynn; Belstrøm, Daniel

    -stimulated whole saliva (SWS) flow rates, a labial salivary gland biopsy and test for serum autoantibodies. Results: Fourteen females and one male (aged 57±12 years) fulfilled the American-European Consensus Classification Criteria, whereas 22 females and 3 males (aged 55±14 years) did not. No significant......SS but a lower specificity due to several false positive serum samples. Our on-going study includes a larger cohort to substantiate our preliminary findings and discover more specific biomarkers for pSS....

  3. 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.

  4. Kulturelle Fyrtårne i Region Sjælland

    DEFF Research Database (Denmark)

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

    2010-01-01

    niveauer. Det mere grundlæggende udbud af kulturelle tilbud kan siges at have en fyrtårnsværdi af social såvel som finansiel karakter indenfor enkelte kommuner, mens kulturelle attraktioner med større fyrtårnspotentiale der rækker udover det lokale og eventuelt det regionale, findes når større geografiske...

  5. Sjögren's and plasma cell variant Castleman disease: a case report ...

    African Journals Online (AJOL)

    It classically presents as a mediastinal mass that involves the lymphatic tissue primarily but can also affect extra lymphatic sites including the lungs, larynx, parotid glands, pancreas, meninges, and muscles. In HIV and HHV8-negative patients with idiopathic multicentric Castleman disease, pathogenesis may involve ...

  6. 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...

  7. Uretral cancer er sjælden og vanskelig at diagnosticere

    DEFF Research Database (Denmark)

    Klemann, Nina; Toft, Birgitte Grønkjær; Thind, Peter

    2014-01-01

    present a case of a 69-year-old woman with a one-year history of urinary obstruc-tion. MRI revealed a tumour surrounding the urethra, and biopsies showed adenocarcinoma in situ. Surgery with removal of the bladder, urethra, internal genitals and lymph nodes revealed a periurethral cyst with both...... adenocarcinoma in situ and focal adenocarcinoma....

  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. 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...

  10. The value of rituximab treatment in primary Sjögren's syndrome

    NARCIS (Netherlands)

    Verstappen, Gwenny M.; van Nimwegen, Jolien F.; Vissink, Arjan; Kroese, Frans G. M.; Bootsma, Hendrika

    The rationale for B cell depletion therapy with rituximab in primary Sjogren's syndrome relies upon the well-established role of B cell hyperactivity in immunopathogenesis. In line with this notion, several biomarkers of B cell activity are significantly affected by treatment, both in the target

  11. 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....

  12. Occurrence Of Pesticides In Surface And Groundwater In Two Catchments On Sjælland, Denmark

    DEFF Research Database (Denmark)

    McKnight, Ursula S.; Rasmussen, Jes J.; Kronvang, Brian

    2011-01-01

    (WFD), which requires member states to guarantee the good ecological status (or potential, in the case of “heavily modified water bodies”) of its receiving waters, is challenging since it is difficult to successfully evaluate all the pressures stressing an ecosystem. This study endeavours to identify...... – and ideally separate – potential stressors acting on surface water ecosystems at the catchment-scale. A secondary objective was to evaluate possible water quality differences between site types (distinguished as first-order stream locations potentially impacted by a single stressor) and seasons.......The release of chemicals such as chlorinated solvents, pesticides and other xenobiotic organic compounds to streams, either from contaminated sites, accidental or direct application/ release, is a significant threat to water resources. Fulfilling the requirements of the EU Water Framework Directive...

  13. 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.

  14. Sjögren's syndrome-associated myositis with germinal centre-like structures.

    Science.gov (United States)

    Espitia-Thibault, Alexandra; Masseau, Agathe; Néel, Antoine; Espitia, Olivier; Toquet, Claire; Mussini, Jean-Marie; Hamidou, Mohamed

    2017-02-01

    Muscular impairment is a rare systemic manifestation of SS that is rarely described in the literature and classically non-specific, both clinically and histologically. We reviewed the cases of 4 patients with primary SS presenting with myositis and a common histologic pattern on muscular biopsy with germinal centre-like structures resembling that which occurs in salivary glands. We analysed the data files of patients with SS who had muscular manifestations and underwent a muscular biopsy. Among 23 patients with SS who had muscle biopsies, 13 had non-specific myositis and 10 (4 primary and 6 secondary SS) had a common histologic pattern consisting of germinal centre-like structures. We analysed the data files of the 4 patients with primary SS presenting with myositis with muscular germinal-centre like structures. The 4 patients had an unspecific clinical presentation, with myalgias, muscular weakness and normal or elevated values of CPK. In the four patients, SS-associated myositis had common histologic characteristics, with endomysial and perimysial inflammatory infiltrate. The cellular infiltrate was composed predominantly of CD4+ T lymphocytes and B lymphocytes. The B and T CD4+ cells infiltrates may gather into masses, even forming lymphoid follicles. Three patients were treated with corticosteroids and/or hydroxychloroquine with improvement of myositis and 1 patient was lost to follow-up. We describe four patients with a common histologic appearance of myositis with lymphoid follicles associated with primary SS. The clinical presentation was non-specific and non-severe, with favorable outcome with corticosteroids and/or hydroxycholoroquine. The discovery of this particular histologic appearance in a muscle biopsy independent of the final diagnosis should indicate the possibility of SS. Copyright © 2016 Elsevier B.V. All rights reserved.

  15. Mesenterial pannikulitis er en sjælden inflammatorisk proces i mesenteriets fedtvæv

    DEFF Research Database (Denmark)

    Rolff, Hans Christian; Fallentin, Eva; Wettergren, André

    2011-01-01

    Mesenteric panniculitis (MP) is a rare condition affecting the mesentery of the small bowel and/or colon. Obtaining a diagnosis is dependent on radiological and histological findings. With the increasing use of abdominal CT, more cases of MP will be discovered. We report a case of MP accidentally...... found at CT in a 64 year-old woman who was evaluated for upper abdominal discomfort. Percutaneous ultrasonography guided biopsy secured the diagnosis. There is no consensus on the treatment and management of MP. An approach guided by the patient's symptoms seems suitable....

  16. Characterisation of recombinant human fatty aldehyde dehydrogenase: implications for Sjögren-Larsson syndrome

    NARCIS (Netherlands)

    Lloyd, Matthew D.; Boardman, Kieren D. E.; Smith, Andrew; van den Brink, Daan M.; Wanders, Ronald J. A.; Threadgill, Michael D.

    2007-01-01

    Fatty aldehyde dehydrogenase (FALDH) is an NAD+-dependent oxidoreductase involved in the metabolism of fatty alcohols. Enzyme activity has been implicated in the pathology of diabetes and cancer. Mutations in the human gene inactivate the enzyme and cause accumulation of fatty alcohols in

  17. Summary. “Materials Challenges in Nuclear Energy,” S.J. Zinkle, 2013

    Energy Technology Data Exchange (ETDEWEB)

    Pestovich, Kimberly Shay [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2015-11-05

    Nuclear energy continues to grow in abundance and importance. It offers a future electric grid based entirely off of green energy, and it has numerous applications. Nuclear power has capabilities to desalinate water, deliver process heat or steam, affordably crack hydrogen from water, and extract unconventional fossil fuel sources. Current light water reactors demonstrate high reliability under normal operating conditions. Researchers have shown significant interest and investigating how to extend reactor lifespans and into other possible reactor designs. Further understanding of mechanisms responsible for corrosion and stress corrosion cracking, radiation hardening and degradation, and nuclear fuels innovations can lead to safer, more reliable, and cost-effective water-cooled nuclear reactors for electricity production.

  18. 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 ...

  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. 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....

  2. 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 ...

  3. Work disability in newly diagnosed patients with primary Sjögren syndrome

    DEFF Research Database (Denmark)

    Mandl, Thomas; Jørgensen, Tanja Schjødt; Skougaard, Marie

    2017-01-01

    yrs, range 18-61 yrs, 50 women) diagnosed with pSS between January 2001 and December 2012 were included in the study. For each patient we randomly selected 4 reference subjects from the general population and matched for age, sex, and area of residence. We linked data to the Swedish Social Insurance...

  4. 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...

  5. Sjögren's and plasma cell variant Castleman disease: a case report

    African Journals Online (AJOL)

    of Ghana School of Biomedical and Allied Health Sciences, PO Box GP 4236, Accra, Ghana, 4Department of .... moval of the involved lymph nodes or use of anti-IL6 ... disease activity at later end points. 29 ... Yoshizaki K, Honda O, et al.

  6. 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

  7. 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.

  8. 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...

  9. 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...

  10. "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

  11. 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...... inflammation. Gastroscopy and colonoscopy were normal. Capsule endoscopy revealed small intestine ulcers and a stenosis. A CT scan of the abdomen confirmed stenosis and inflammation of terminal ileum. QuantiFERON-TB Gold Test was positive and Mycobacterium tuberculosis was detected in faeces cultures....

  12. Detection of antisalivary duct antibody from Sjögren's syndrome by an autoradiographic method.

    Science.gov (United States)

    Cummings, N A; Tarpley, T M

    1978-01-01

    A new technique to detect anti-salivary duct antibody (ASDA) has been developed by using autoradiographic, rather than immunofluorescent methods. The antibody activity detected by autoradiography is probably classic ASDA. Both techniques may be consecutively performed on the same tissue section without attenuation of either. Some of the potential advantages of the radiolabelling of ASDA are pointed out, and a few preliminary experiments using the labelled antibody as a marker are presented.

  13. 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....

  14. Primært effusionslymfom er en sjælden årsagtil pleuraeffusion

    DEFF Research Database (Denmark)

    Saxild, Sofie; Schjødt, Ida; Hutchings, Martin

    2016-01-01

    Primary effusion lymphoma (PEL) is a rare form of extranodal non-Hodgkin lymphoma, which presents as an accumulation of lymphatic cells within a body cavity. It is classified as a human herpesvirus 8 (HHV-8)-positive lymphoma and is associated with HIV. The prognosis is poor. Cases of HHV-8......-negative PEL have been described in immunocompetent patients. We report a case of an 86-year-old man diagnosed with HHV-8-negative PEL. Based on case reports he was treated with pleuracentesis and was still in complete remission one year later....

  15. 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

    Directory of Open Access Journals (Sweden)

    Maria Maślińska

    2017-07-01

    Full Text Available Objectives : 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. Materials and methods : 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. Results : 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. 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.

  16. 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....

  17. Sjöfarten som ett socialt system:om handelssjöfart, risk och säkerhet

    OpenAIRE

    Mårtensson, Magnus

    2006-01-01

    In spite of intensive international work on safety regulations the world has repeatedly witnessed major shipping accidents. The work that has been and is being carried out, both in practical and research terms, to increase safety at sea has mainly been focused on technical improvements and an effort to minimize different types of human errors. This, however, does not appear to lead to a decrease in the number of accidents and incidents. Very few studies of the safety problems for shipping exi...

  18. Torsion instability of Flexible Pipes at the TDP - Torsjonsinstabilitet av fleksible rør med sjøbunnskontakt

    OpenAIRE

    Hansson, Linn Storesund

    2017-01-01

    Dynamic flexible risers are a common term for cables, umbilicals and flexible pipes,that are freely or semi-freely suspended between two points (offshore). Flexible pipes are mostly used to transport fluids between platforms/ships etc. to a flow-line/sea floor installation or similar. The safe operating window for the installation is limited by whether induced motion of the installation vessel giving rise to dynamic tension and curvature at the touch-down-zone (TDZ). By investigation and ...

  19. 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

    . 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.......8, P less than 0.05, 'corrected' P greater than 0.05), and DQB1*0302 (DQw8) (RR = 4.5, P less than 10(-2). Negative associations were found between RA and DRB1*1501 (DR2/DRw15) (RR = 0.2, P less than 10(-2) and DQB1*0602 (DQw6) (RR = 0.2, P less than 10(-2), 'corrected' P greater than 0.......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...

  20. Primary Sjögren’s Syndrome with Sensory Ganglionopathy and Painful Legs and Moving Toes Syndrome

    Directory of Open Access Journals (Sweden)

    Mehmet Uğur Çevik

    2014-06-01

    Full Text Available Sjogren’s syndrome is characterized by the sicca syndrome, with dryness of the mouth (xerostomia and the eyes (xerophthalmia. Sjogren's syndrome is the only connective tissue disease that has been associated with sensory neuronopathy. The syndrome of painful legs and moving toes consisting of pain in the lower limbs with spontaneous movements of the toes or feet. The association between Sjogren’s syndrome and painful legs and moving toes syndrome is a rare condition

  1. Conditions for effective removal of pyrene from an artificially contaminated soil using Pseudomonas aeruginosa 57SJ rhamnolipids

    Energy Technology Data Exchange (ETDEWEB)

    Bordas, Francois [Institut national de la recherche scientifique, Centre INRS-Eau-Terre-Environnement, Universite du Quebec, 2800 rue Einstein, C.P. 7500, Sainte-Foy, Quebec, G1V 4C7 (Canada)]. E-mail: francois.bordas@unilim.fr; Lafrance, Pierre [Institut national de la recherche scientifique, Centre Eau, Terre et Environnement, Universite du Quebec, 490, rue de la Couronne, Quebec, G1K 9A9 (Canada)]. E-mail: pierre_lafrance@inrs-ete.uquebec.ca; Villemur, Richard [Institut national de la recherche scientifique, Centre INRS-Institut Armand Frappier, Universite du Quebec, 531 bd des Prairies, Laval, Quebec, H7V 1B7 (Canada)]. E-mail: richard.villemur@inrs-iaf.uquebec.ca

    2005-11-15

    The efficacy of a new rhamnolipid biosurfactants mixture to enhance the removal of pyrene from a soil artificially contaminated was investigated. The molar solubilization ratio (MSR) and the partition coefficient between the micelles and water (log K {sub m}) were found to be 7.5 x 10{sup -3} and 5.7, respectively. From soil column studies, the pyrene removal increased linearly with the concentration of the injected biosurfactants solution above the effective critical micellar concentration (0.4 g L{sup -1}). Flushing with a 5.0 g L{sup -1} biosurfactants solution increased the pyrene concentration in the effluent by 178 times. At high biosurfactants' concentrations (2.5 and 5.0 g L{sup -1}), the cumulative pyrene recovery reached 70%. This pyrene remobilization takes place independently of the soil organic carbon solubilization. This study provides a combination of batch and column experiments in order to find the conditions for effective soil remediation using a new rhamnolipids mixture. - The potential of newly isolated biosurfactants to mobilize PAHs from contaminated soils was evaluated from the determination in dynamic conditions of their effective critical micellar concentration.

  2. Conditions for effective removal of pyrene from an artificially contaminated soil using Pseudomonas aeruginosa 57SJ rhamnolipids

    International Nuclear Information System (INIS)

    Bordas, Francois; Lafrance, Pierre; Villemur, Richard

    2005-01-01

    The efficacy of a new rhamnolipid biosurfactants mixture to enhance the removal of pyrene from a soil artificially contaminated was investigated. The molar solubilization ratio (MSR) and the partition coefficient between the micelles and water (log K m ) were found to be 7.5 x 10 -3 and 5.7, respectively. From soil column studies, the pyrene removal increased linearly with the concentration of the injected biosurfactants solution above the effective critical micellar concentration (0.4 g L -1 ). Flushing with a 5.0 g L -1 biosurfactants solution increased the pyrene concentration in the effluent by 178 times. At high biosurfactants' concentrations (2.5 and 5.0 g L -1 ), the cumulative pyrene recovery reached 70%. This pyrene remobilization takes place independently of the soil organic carbon solubilization. This study provides a combination of batch and column experiments in order to find the conditions for effective soil remediation using a new rhamnolipids mixture. - The potential of newly isolated biosurfactants to mobilize PAHs from contaminated soils was evaluated from the determination in dynamic conditions of their effective critical micellar concentration

  3. Natural history and Predictors of Progression to Sjögren's Syndrome Among Participants of the SICCA registry

    DEFF Research Database (Denmark)

    Shiboski, Caroline H; Baer, Alan N; Shiboski, Stephen C

    2018-01-01

    found to have any objective measures of salivary hypofunction, dry eye, focal lymphocytic sialadenitis in minor salivary gland biopsy, or anti-SSA/B antibodies, were recalled over a window of 2 to 3 years after their baseline examinations to repeat all clinical examinations and specimen collections......BACKGROUND/PURPOSE: To explore changes in the phenotypic features of Sjoögren's syndrome (SS), and in SS status among participants in the Sjoögren's International Collaborative Clinical Alliance (SICCA) registry over a 2 to 3-year interval. METHODS: All participants in the SICCA registry who were...

  4. Bildskapande i relation till genus i förskolans verksamhet : Att skapa sig själv och genus

    OpenAIRE

    Brännlund, Angelika

    2018-01-01

    Studiens syfte är att bidra till ökad medvetenhet för verksamma inom förskolans område kring frågor som rör pedagogers förhållningssätt kring relationen mellan bildskapande och genus, men även hur dessa förhållningssätt kan påverka barnens möjligheter i deras bildskapande. En viktig fråga som burits med från starten av arbetet och som lyser igenom i resultatdelen har varit: Vilka möjligheter att förhålla sig till bildskapande och genus kan uppstå om ett kritiskt sätt att tänka kring könsstere...

  5. K Rybově kritice Karla Kolčavy SJ a jeho znalostí teorie dramatu

    Czech Academy of Sciences Publication Activity Database

    Förster, Josef

    2017-01-01

    Roč. 140, č. 3/4 (2017), s. 429-447 ISSN 0024-4457 Institutional support: RVO:67985955 Keywords : Carolus Kolczawa S. J. * Neo-Latin drama in Bohemia * theory of drama Subject RIV: AJ - Letters, Mass-media, Audiovision OBOR OECD: Literary theory

  6. 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...

  7. Mission-Enabling Photovoltaic Flexible Blanket Solar Array with SNC/SJ Surface Mount Technology, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — Deployable Space Systems, Inc. (DSS) in collaboration with Sierra Nevada Corporation (SNC) has developed a modular multi-junction photovoltaic flexible blanket...

  8. Baltic 21 - an exiting and concrete co-operation for sustainable development / Anna Carin Thomer, Sven Sjögren

    Index Scriptorium Estoniae

    Thomer, Anna Carin

    2002-01-01

    Läänemere Agenda 21 koostööga on seotud 11 riiki, 30 aasta perspektiivis pürgitakse säästva arengu poole, hõlmates kaheksat valdkonda: põllumajandust, energeetikat, kalandust, metsandust, tööstust, turismi, transporti ja haridust

  9. 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

  10. Era Sanctorum: la beatificación inconclusa del padre Diego Martínez, SJ (1627-1634

    Directory of Open Access Journals (Sweden)

    Coello de la Rosa, Alexandre

    2009-06-01

    Full Text Available This essay analyzes the politics of sanctity of the Society of Jesus in Viceregal Peru (17th Century through the process of beatification of the Spanish Father Diego Martínez (1627-34. An unfinished process that has been filed in the Jesuit archives for centuries and was never introduced in the Sacred Congregation of Rites, in the Vatican.Este ensayo analiza las políticas de santidad de la Compañía de Jesús en el Perú virreinal (siglo XVII a través del proceso de beatificación del padre extremeño Diego Martínez (1627-34. Un proceso inconcluso que ha permanecido en los archivos jesuitas durante siglos y que nunca se introdujo en la Sagrada Congregación de Ritos, en el Vaticano.

  11. Karel Slavíček, SJ and His Correspondence from China with European Astronomers and Scholars

    Czech Academy of Sciences Publication Activity Database

    Liščák, Vladimír

    3 /19/, č. 2 (2015), s. 57-72 ISSN 2232-5131 Institutional research plan: CEZ:AV0Z9021901 Institutional support: RVO:68378009 Keywords : Jesuit missionaries * China * Karel Slavíček Subject RIV: AA - Philosophy ; Religion

  12. 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...

  13. Faithful Is Successful: Notes to the Driven Pilgrim. Grills. N, Lewis. DE, and Swamidass. SJ, eds. USA: Outskirts 2014

    Directory of Open Access Journals (Sweden)

    Nicollette Maunganidze

    2015-01-01

    Full Text Available Faithful is Successful (Grills et al, 2014, a collection of memoirs penned by individuals from a designated demographic: “successful Christian professionals,” explores the topic of success from the Christian worldview. This work leverages, most specifically, lessons learned from struggle and search on the topic, to explicate how success in vocation can and ought to be achieved. Inevitably canvassed subthemes include the definition in essence, the measuring stick(s and expected exemplification of success. The latter, the authors suggest, a fruit one can only authentically interpret ex post. Overall, the undeniable page-turner is the refreshing honesty of each disclosure including contributions from a U.S Army serviceman turned hedge-fund co-founder in Africa and a respected artist working in Los Angeles, to name a few. Noteworthy is the fact that among contributors, authors Denholm and Grills, and editors Swamidass and Grills hail from the medical field. Not only them, but several of the authors have interfaced with the theme of this volume: “Caring in Conflict.” Whether at work or at home, interpersonal conflict has challenged some of the authors, and at times their families, as they negotiated their life journey’s in both old and new environments.

  14. Dural arteriovenøs fistel er en sjælden, men behandlelig årsag til transversal myelopati

    DEFF Research Database (Denmark)

    Hansen, Julie Richter; Poulsgaard, Lars; Holtmannspötter, Markus

    2017-01-01

    This is a case report of a 60-year-old male admitted on suspicion of relapse of idiopathic transverse myelitis (TM), who after further diagnostic workup underwent successful closure of a dural arteriovenous fistula (DAVF). Magnetic resonance imaging in DAVF usually shows longitudinal TM, which, u......, unlike DAVF, is also seen with the more common inflammatory or infectious causes usually showing inflammation in the cerebrospinal fluid. The natural history of DAVF is progressive. Since curable options exist, timely diagnosis is most important....

  15. S.J. Goulda koncepcja rozłącznych magisteriów nauki i wiary

    Directory of Open Access Journals (Sweden)

    Justyna Herda

    2007-12-01

    Full Text Available There are many different approaches to the problem of the relation between science and religion. It is often claimed that there exists a conflict between those two domains. However, S. J. Gould cannot see neither how the two enterprises can stay in any conflict nor how they can be reconciled or unified. He developed a concept of two Non-Overlapping Magisteria (NOMA. Gould claims that science and religion are characterized by completely different areas of investigation. The two magisteria ask different questions and provide different explanations. Science investigates the factual character of the natural world while religion explores the world of values, meanings and purposes of human life. One could say that this is a reasonable point of view, but at a closer glance it does not seem to be a satisfactory solution.

  16. Electron impact with argon excitation out of the e-Ar(3p54s,J = 2)

    International Nuclear Information System (INIS)

    Jin Shiqi; Xu Zhizhan

    1998-01-01

    It is shown that the electron impact with argon excitation out of the metastable to the 3p 5 4p levels is very important for spectrum, laser physics and plasma processing. Analysis of the cross sections of 3p 5 4p, J = 3 level in manifolds of 3p 5 4p is valuable. It also provides the atomic character of emission harmonics in intense laser field

  17. 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 comm...

  18. INAUGURAÇÃO DA SESSÃO SOLENE PELO REITOR P. ÁLVARO MENDONÇA PIMENTEL SJ

    Directory of Open Access Journals (Sweden)

    Álvaro Mendonça Pimentel

    2016-01-01

    Full Text Available Inauguro esta sessão solene, em homenagem ao Prof. Dr. Ulpiano Vázquez Moro, compartilhando a alegria serena que a preparação deste momento me tem oferecido. Nestes meses de amizade vivida, sobretudo, no silêncio e na oração, pude aproximar-me interiormente do meu irmão Ulpiano, além de conviver com ele, nos encontros que tivemos.

  19. Mirror syndrome er en sjælden graviditetskomplikation karakteriseret ved ødemer og føtal hydrops

    DEFF Research Database (Denmark)

    Eiland, Signe; Cvetanovska, Eleonora; Bjerre, Anne Høyrup

    2017-01-01

    was performed without improvement of the condition. Since maternal symptoms worsened, a caesarean section was performed at 34 weeks with delivery of a live hydropic girl who died after 17 hours. Maternal symptoms decreased four days after delivery with full recovery two weeks post-partum.......We describe a case of mirror syndrome. A 41-year-old woman, para 4, was referred to hospital at 32 weeks of gestational age with excessive oedema. She developed oliguria, mild hypertension and proteinuria. Fetal ultrasound confirmed severe hydrops fetalis. Intrauterine pleural drainage...

  20. J.e. Greeff* and A.S. Faure G.J. Minnaar S.J. Schoeman

    African Journals Online (AJOL)

    the fashion market. This market requires an attractive product of exceptional quality which, to a large extent, is determined by quality of hair and the excellence of pattern. Pattern and hair quality are both complex traits under poli- genic control (Nel, 1966). To increase accuracy of selection, it is important that any non-genetic ...

  1. Rätten till självbestämmande : Samernas rättigheter i samband med mineralutvinning

    OpenAIRE

    Lindström, Emelie

    2015-01-01

    Idag etablerar sig flertalet gruvbolag i Sverige på marker som även är av intresse för samernaoch den rennäring som de bedriver på markerna. Denna uppsats belyser det regelverk sombehandlar de rättigheter samerna, som urbefolkning, har gentemot Sverige. Sverige har fåttskarp kritik från FN: s rasdiskrimineringskommitté (CERD) för att samernas rättigheter somurbefolkning inte skyddas tillräckligt i samband med den mineralutvinning som pågår iSápmi. Främst riktas kritiken mot att samerna inte k...

  2. Marinesco-Sjögren syndrome protein SIL1 regulates motor neuron subtype-selective ER stress in ALS

    NARCIS (Netherlands)

    Filézac de L'Etang, Audrey; Maharjan, Niran; Cordeiro Braña, Marisa; Ruegsegger, Céline; Rehmann, Ruth; Goswami, Anand; Roos, Andreas; Troost, Dirk; Schneider, Bernard L.; Weis, Joachim; Saxena, Smita

    2015-01-01

    Mechanisms underlying motor neuron subtype-selective endoplasmic reticulum (ER) stress and associated axonal pathology in amyotrophic lateral sclerosis (ALS) remain unclear. Here we show that the molecular environment of the ER between motor neuron subtypes is distinct, with characteristic

  3. 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....

  4. Relationen mellan självkänsla, betyg, fysisk aktivitet och social deaktighet hos gymnasieelever: en korrelationsstudie

    OpenAIRE

    Sörbrand, Niclas

    2014-01-01

    Research has shown positive correlations between global self-esteem and physical activity, as well as between global self-esteem and school performance. However, research is ambiguous and other variables have shown importance for global self-esteem. This study aims to investigate the correlations between global self-esteem and physical activities, school performance and social participations within high-school students in the northern part of Sweden. The study also investigates gender differe...

  5. 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...

  6. D{sub sJ}(2860) from the semileptonic decays of B{sub s} mesons

    Energy Technology Data Exchange (ETDEWEB)

    Gan, Long-Fei, E-mail: lfgan@nudt.edu.cn; Zhang, Jian-Rong; Huang, Ming-Qiu; Zhuo, Hong-Bin; Ma, Yan-Yun; Zhu, Qing-Jun; Liu, Jian-Xun; Zhang, Guo-Bo [College of Science, National University of Defense Technology, 410073, Changsha, Hunan, People’s Republic of (China)

    2015-05-27

    In the framework of heavy quark effective theory, the leading-order Isgur–Wise form factors relevant to semileptonic decays of the ground state b{sup -bar}s meson B{sub s} into orbitally excited D-wave c{sup -bar}s mesons, including the newly observed narrow D{sub s1}{sup ∗}(2860) and D{sub s3}{sup ∗}(2860) states by the LHCb Collaboration, are calculated with the QCD sum rule method. With these universal form factors, the decay rates and branching ratios are estimated. We find that the decay widths are Γ(B{sub s}→D{sub s1}{sup ∗}ℓν{sup -bar})=1.25{sub -0.60}{sup +0.80}×10{sup -19} GeV , Γ(B{sub s}→D{sub s2}{sup ′}ℓν{sup -bar})=1.49{sub -0.73}{sup +0.97}×10{sup -19} GeV , Γ(B{sub s}→D{sub s2}ℓν{sup -bar})=4.48{sub -0.94}{sup +1.05}×10{sup -17} GeV , and Γ(B{sub s}→D{sub s3}{sup ∗}ℓν{sup -bar})=1.52{sub -0.31}{sup +0.35}×10{sup -16} GeV . The corresponding branching ratios are B(B{sub s}→D{sub s1}{sup ∗}ℓν{sup -bar})=2.85{sub -1.36}{sup +1.82}×10{sup -7}, B(B{sub s}→D{sub s2}{sup ′}ℓν{sup -bar})=3.40{sub -1.66}{sup +2.21}×10{sup -7}, B(B{sub s}→D{sub s2}ℓν{sup -bar})=1.02{sub -0.21}{sup +0.24}×10{sup -4}, and B(B{sub s}→D{sub s3}{sup ∗}ℓν{sup -bar})=3.46{sub -0.70}{sup +0.80}×10{sup -4}. The decay widths and branching ratios of corresponding B{sub s}{sup ∗} semileptonic processes are also predicted.

  7. Den potentiella människan : En undersökning av teorier om självförverkligande

    OpenAIRE

    Nilsson, Staffan

    2005-01-01

    “What characterizes an acceptable theory of self-realization?” The thrust of the present dissertation is towards seeking an answer to this central problem, which stems from the fundamental human experience that life involves change, and that in a modern society such change is often expected to be towards a realization of potentials and the good life for the individual. The dissertation has a three-fold purpose. The first is to clarify the content of five modern theories of self-realization f...

  8. Examensarbete ”Nuodå” Ett samarbete med AB O.H. Sjögrens

    OpenAIRE

    Leósdóttir Löfgren, Kolbrún

    2005-01-01

    The ambition of this project is to make an overview of the Swedish upholstery industry and to make a suitable complement to the Carl Malmsten upholstery collection. The Swedish furniture industry employs about 20 000 people in 815 companies. Totally the branch had a turnover of 17,5 billion Swedish krona in the year 2000. Of these 815 companies most of them are small family driven companies with low-tech industry. 10 % of the 815 companies stand for 60% of the production. These are large-scal...

  9. Boliglokalisering og andelen af regionale togrejser på Sjælland og i Østjylland

    DEFF Research Database (Denmark)

    Møller, Thomas Hjorth; Bro, Peter; Harder, Henrik

    2008-01-01

    Dette videnskabelige essay sammenligner tidligere studier af boliglokalisering og transport i en række danske byer, med et nyt studie omkring sammenhængen mellem boliglokalisering og andelen af regionale togrejser i to danske byregioner; den polycentriske region i Østjylland og den monocentriske...

  10. A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren's Syndrome International Registry

    DEFF Research Database (Denmark)

    Whitcher, John P; Shiboski, Caroline H; Shiboski, Stephen C

    2010-01-01

    To describe, apply, and test a new ocular grading system for assessing keratoconjunctivitis sicca (KCS) using lissamine green and fluorescein.......To describe, apply, and test a new ocular grading system for assessing keratoconjunctivitis sicca (KCS) using lissamine green and fluorescein....

  11. 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...

  12. Botany and zoology in the late seventeenth-century Philippines: the work of Georg Josef Camel SJ (1661-1706).

    Science.gov (United States)

    Reyes, Raquel A G

    2009-10-01

    Georg Josef Camel (1661-1706) went to the Spanish colony of the Philippine Islands as a Jesuit lay brother in 1687, and he remained there until his death. Throughout his time in the Philippines, Camel collected examples of the flora and fauna, which he drew and described in detail. This paper offers an overview of his life, his publications and the Camel manuscripts, drawings and specimens that are preserved among the Sloane Manuscripts in the British Library and in the Sloane Herbarium at the Natural History Museum, London. It also discusses Camel's links and exchanges with scientifically minded plant collectors and botanists in London, Madras and Batavia. Among those with whom Camel corresponded were John Ray, James Petiver, and the Dutch physician Willem Ten Rhijne.

  13. Dental implants in maxillofacial prosthodontics : An asset in head and neck cancer and Sjögren's syndrome patients

    NARCIS (Netherlands)

    Korfage, Anke

    2015-01-01

    Maxillofaciale prothetiek is het vakgebied dat zich bezig houdt met het maken van protheses bij patiënten met een afwijking of aandoening in het hoofd-halsgebied. Deze protheses kunnen zowel gebitsprotheses zijn als gezichtsprotheses, waarbij bijvoorbeeld een neus, een oog of een oor wordt

  14. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Voices Event Calendar Walk for Sjögren's Team Sjögren’s Running Program > Team Sjögren’s Goes Turkey! Sip for Sjögren’s National Patient Conference Team Sjögren’s Running Program National Patient Conference Shop Online to Support ...

  15. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Sjögren's Symptoms > Dry Eyes >> Resources > Dry Mouth Diagnosis Treatments Patient Information Faces of Sjögren's Living with Sjögren's ... Practice Guidelines Physician Resources Member Community Symptoms Diagnosis Treatments Patient Information Living with Sjögren's Survey Sjögren’s Clinical ...

  16. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Sjögren's Living with Sjögren's Survey Sjögren’s Clinical Trial Locations Clinical Trials – What’s Involved Video: A Place to ... Information Living with Sjögren's Survey Sjögren’s Clinical Trial Locations Clinical Trials – What’s Involved Video: A Place to ...

  17. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Dry Eyes >> Resources > Dry Mouth Diagnosis Treatments Patient Information Faces of Sjögren's Living with Sjögren's Survey Sjögren’s ... Involved Video: A Place to Begin Family & Friends Information Sjögren’s FAQs Glossary Sjögren’s Tip > Dry Eye Tips > ...

  18. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Day Little Voices Event Calendar Walk for Sjögren's Team Sjögren’s Running Program > Team Sjögren’s Goes Turkey! Sip for Sjögren’s National Patient Conference Team Sjögren’s Running Program National Patient Conference Shop Online ...

  19. 77 FR 5308 - Quarterly Publication of Individuals, Who Have Chosen To Expatriate, as Required by Section 6039G

    Science.gov (United States)

    2012-02-02

    ... ALBERT MAYER CONNOLLY WINSTON COOKSON GORDON POWER COONEY JOHN CUBAUD NICOLAS EDGARD CUDDY DAVID R... TAKEUCHI ERIKA TAN AH-SWAT TAN YEW KHUAN TANG MISI TANNER DAVID A TANNER JACQUELINE........ A TAUSCH HENRI...

  20. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Walk for Sjögren's > Sip for Sjogren's > Patient Seminars History World Sjögren's Day Advocacy > Social Security Disability & Sjögren’s Board of Directors Conflict of Interest Statement ...

  1. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... News & Events >Walk for Sjögren's > Sip for Sjogren's > Patient Seminars History World Sjögren's Day Advocacy > Social Security Disability & Sjögren’s Board of Directors Conflict of Interest ...

  2. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Friends Information Sjögren’s FAQs Glossary Sjögren’s Tip > Dry Eye Tips > Dry Mouth ... Sjögren’s Syndrome: A Place To Begin from Sjögren’s Syndrome Foundation ...

  3. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Trials – What’s Involved Video: A Place to Begin Family & Friends Information Sjögren’s FAQs Glossary Sjögren’s Tip > Dry ... Trials – What’s Involved Video: A Place to Begin Family & Friends Information Sjögren’s FAQs Glossary Sjögren’s Tip Survival ...

  4. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Patient Seminars History World Sjögren's Day Advocacy > Social Security Disability & Sjögren’s Board of Directors Conflict of Interest ... Special Support Groups > Canadian Support Groups > International Sjögren’s Network International Sjögren’s Network Volunteer as Patient Support Become ...

  5. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Sjögren's Survey Sjögren’s Clinical Trial Locations Clinical Trials – What’s Involved Video: A Place to Begin Family & Friends Information Sjögren’s FAQs Glossary Sjögren’s Tip > Dry Eye Tips > Dry Mouth Tips > Other Dryness Tips > Diet & Food Tips > Peripheral Neuropathy Tips > Surgery Tips Survival Tips ...

  6. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... What’s Involved Video: A Place to Begin Family & Friends Information Sjögren’s FAQs Glossary Sjögren’s Tip > Dry Eye ... What’s Involved Video: A Place to Begin Family & Friends Information Sjögren’s FAQs Glossary Sjögren’s Tip Survival Tips ...

  7. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Patient Seminars History World Sjögren's Day Advocacy > Social Security Disability & Sjögren’s ... Turkey! Sip for Sjögren’s National Patient Conference Team Sjögren’s Running Program National Patient ...

  8. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Symptoms > Dry Eyes >> Resources > Dry Mouth Diagnosis Treatments Patient Information Faces of Sjögren's Living with Sjögren's Survey ... News & Events >Walk for Sjögren's > Sip for Sjogren's > Patient Seminars History World Sjögren's Day Advocacy > Social Security ...

  9. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Patient Seminars History World Sjögren's Day Advocacy > Social Security Disability & Sjögren’s Board of Directors Conflict of Interest ... Team Sjögren’s Running Program National Patient Conference Shop Online to Support the SSF Blog: Conquering Sjögren’s SSF ...

  10. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... for Sjögren's > Sip for Sjogren's > Patient Seminars History World Sjögren's Day Advocacy > Social Security Disability & Sjögren’s Board ... More Faces of Sjögren's Wake Up, Koala! Provider/Researcher Research Grants > Grant Application > Current Recipients > Past Recipients ...

  11. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Contact Us About Sjögren's Symptoms > Dry Eyes >> Resources > Dry Mouth Diagnosis Treatments Patient Information Faces of Sjögren's Living ... Sjögren’s FAQs Glossary Sjögren’s Tip > Dry Eye Tips > Dry Mouth Tips > Other Dryness Tips > Diet & Food Tips > Peripheral ...

  12. Ectopic germinal centre-like structures in minor salivary gland biopsy predict lymphoma occurrence in patients with primary Sjögren syndrome.

    Science.gov (United States)

    Sène, Damien; Ismael, Sophie; Forien, Marine; Charlotte, Frédéric; Kaci, Rachid; Cacoub, Patrice; Abdourahmane, Diallo; Dieudé, Philippe; Lioté, Frédéric

    2018-04-18

    The objective was to determine risk factors of pSS-associated lymphoma in a multicenter cohort of patients, including in analyzed variables the presence of including ectopic germinal centre-like structures in minor salivary gland biopsy (MSGB). 115 pSS-patients were included and their MSGB were retrospectively examined focusing on the presence of ectopic germinal centre-like structures. Epidemiological, clinical, biological, immunological and histological data were collected at pSS diagnosis. Patients with a Non-Hodgkin Lymphoma (NHL) were compared to those without NHL during the follow-up period, using a Cox proportional hazards multiple regression model. NHL was diagnosed in 8 patients (6.96%), and ectopic germinal centre-like structures in 19 patients (16.5%). The presence of ectopic germinal centre-like structures was associated with a 7.8-fold risk of lymphoma occurrence (95%CI = 1.73-34.86; P = 0.0075). Other independent predictors included positive cryoglobulin (HR = 7.10; 95%CI = 1.74-28.92; P = 0.006), male gender (HR = 28.73; 95%CI = 4.46-144.87; P = 0.0004), sensorimotor neuropathy (HR = 35.48; 95%CI = 5.79-217.39; P = 0.0001) and splenomegaly (HR = 19.9; 95%CI = 4.4-90; P = 0.0001). In addition to traditional clinical and biological predictors, the presence of ectopic germinal centre-like structures in MSGB is associated to the risk of lymphoma occurrence in pSS patients. These data reinforce the major role of MSGB in pSS, for the diagnosis and identification a priori of a subgroup of patients with the highest risk of lymphoma. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  13. Combination of Salivary Gland Ultrasonography and Virtual Touch Quantification for Diagnosis of Sjögren’s Syndrome: A Preliminary Study

    Directory of Open Access Journals (Sweden)

    Shaoqi Chen

    2016-01-01

    Full Text Available A total of 136 subjects (51 SS patients, 35 sicca syndrome patients without SS, and 50 healthy volunteers were enrolled in this study. The mean SWV value for salivary glands of SS patients was statistically higher than that of controls (2.81 ± 0.66 m/s versus 1.85 ± 0.28 m/s for parotid glands and 2.29 ± 0.34 m/s versus 1.82 ± 0.25 m/s for submandibular glands, resp.. Combining SWV values of parotid and submandibular glands gives a sensitivity of 88.2% (95% CI: 76.1–95.6% and specificity of 96.0% (95% CI: 86.3–99.5% at the cutoff point of 2.19 m/s, with an AUROC of 0.954 (95% CI: 0.893–0.986. In addition, combining SGUS score and SWV value yields a sensitivity of 98.0% (95% CI: 89.6–100%, specificity of 90.0% (95% CI: 78.2–96.7%, and AUROC of 0.962 (95% CI: 0.904–0.990. Classification tree considering the sequential use of SGUS score and SWV value achieved 92.1% accuracy for diagnosis of SS. Similarly, the ROC curve of combined SGUS scores and SWV values yields an AUROC of 0.954 (95% CI: 0.885–0.987, sensitivity of 97.1% (95% CI: 85.1–99.9%, and specificity of 92.2% (95% CI: 81.1–97.8% for separating sicca syndrome patients (without SS from SS patients. Combining SGUS and VTQ provides a promising tool for diagnosis of SS.

  14. Severe Interstitial Lung Disease and Manic Symptoms Secondary to Corticosteroids in a Patient with Systemic Lupus Erythematosus and Secondary Sjögren’s Syndrome

    Directory of Open Access Journals (Sweden)

    Sofia Silvério Serra

    2017-03-01

    We introduced monthly perfusion of cyclophosphamide for six months and later started mycophenolate mofetil 2 g/day, reducing prednisolone to 10 mg/day and maintaining hydroxychloroquine 400 mg/day, with control of disease activity.

  15. Dynamic response of Sjögren Inlet glaciers, Antarctic Peninsula, to ice shelf breakup derived from multi-mission remote sensing time series.

    NARCIS (Netherlands)

    Seehaus, T.C.; Marinsek, S.; Skvarca, P.; van Wessem, J.M.; Reijmer, C.H.; Seco, J.L.; Braun, M.

    2016-01-01

    The substantial retreat or disintegration of numerous ice shelves has been observed on the Antarctic Peninsula. The ice shelf in the Prince Gustav Channel has retreated gradually since the late 1980s and broke up in 1995. Tributary glaciers reacted with speed-up, surface lowering and increased ice

  16. Snake-like chromatin in conjunctival cells of normal elderly persons and of patients with primary Sjögren's syndrome and other connective tissue diseases

    DEFF Research Database (Denmark)

    Bjerrum, Kirsten Birgitte

    1995-01-01

    Ophthalmology, snake-like chromatin, cytoplasm ratio, keratoconjunctivitis sicca, nucleus, goblet cell......Ophthalmology, snake-like chromatin, cytoplasm ratio, keratoconjunctivitis sicca, nucleus, goblet cell...

  17. Nitric oxide synthase induction and cytotoxic-related oxidant formation in conjunctival epithelium of dry eye (Sjögren´s syndrome).

    Czech Academy of Sciences Publication Activity Database

    Čejková, Jitka; Ardan, Taras; Šimonová, Zuzana; Čejka, Čestmír; Malec, J.; Jirsová, K.; Filipec, M.; Dostřelová, D.; Brůnová, B.

    2007-01-01

    Roč. 17, - (2007), s. 10-17 ISSN 1089-8603 R&D Projects: GA MZd NR8828; GA ČR GA304/06/1379 Institutional research plan: CEZ:AV0Z50390512 Keywords : Nitric oxide * Cytotoxic nitrogen-related oxidants * Ocular surface Subject RIV: FF - HEENT, Dentistry Impact factor: 2.900, year: 2007

  18. Greek versions of the Life of Saint Onnophrius the Great and an archive by Father Joseph Paramelle (s.j, 1925–2011

    Directory of Open Access Journals (Sweden)

    Voitenko Anton

    2015-08-01

    Full Text Available The article focuses on the studies of the Greek manuscript tradition of the Life of Saint Onnophrius the Great, which is still almost unexplored. In this paper, based on analysis of the published versions of the Life, as well as copies of unpublished Greek manuscripts made by Father Joseph Paramelle, the existence of fi ve diff erent Greek versions of the Life (types I–V is established. Among all the installed versions, the only one (type I contains a text the closest to the original version of the Life. It is attested by three Greek manuscripts. All other types are later redactions of the original text. Types II–IV present the abridged versions of the Life, the type V is a partially shortened and strongly metaphrastic text descended probably from the Mount Athos. On the basis of circumstantial evidence, it was assumed the existence of at least another three versions of the Life

  19. Increased Tregs associated with elevated Indoleamine-2,3-dioxygenase activity and an imbalanced Kynurenine pathway in IFNpositive primary Sjögren's syndrome

    NARCIS (Netherlands)

    N.I. Maria (Naomi); C.G. van Helden-Meeuwsen; Z. Brkić (Zana); S.M.J. Paulissen (Sandra); E.C. Steenwijk (Eline); V.A.S.H. Dalm (Virgil); P.L.A. van Daele (Paul); M.P. van Hagen (Martin); F.G.M. Kroese (Frans G.); J.A.G. van Roon (J. A G); A. Harkin (Andrew); A.W. Dik (Willem); H.A. Drexhage (Hemmo); E.W. Lubberts (Erik); M.A. Versnel (Marjan)

    2016-01-01

    textabstractIntroduction Indoleamine-2,3-dioxygenase (IDO), the rate-limiting enzyme converting tryptophan (TRP) to kynurenine (KYN), is driven in part by type I/II IFNs. Naïve T cells are polarized into FoxP3+ regulatory T cells (Tregs) upon exposure to either IDO+ cells or KYN. Recent studies

  20. Kroppens och känslornas betydelse för självförverkligandet. En rekonstruktion av G H Meads tänkande

    Directory of Open Access Journals (Sweden)

    Emma Engdahl

    2001-01-01

    Full Text Available Many social researchers mistakenly think that Mead had no interest in the body and the emotions of the human being. They seem to think that his social philosophy of the act is all about her mind. This is unfortunate since his idea of the significance of the body and emotions for the emergence and development of the self is of great relevance to the contemporary interest in the subject matter. First, this article presents Mead’s contribution to the area. Especially it considers his idea of emotions as a form of intersubjective corporeality. Secondly, to better fulfil Mead’s own wish to transcend the Cartesian mind-body dualism it reconstructs his idea of emotions by relating them not only to the social body of the human being, but also the structures of norms and values embodied in social life. In that way it becomes evident that not only mind but also the social body and the emotions of the human being is of great significance for her selfrealisation.

  1. Våga vara dig själv : Att förstå och skapa en illusion av ljus med digitala medier

    OpenAIRE

    Persson, Jasmin; Lundbladh, Mia

    2015-01-01

    Vi omges ständigt av ljus i vår vardag, det finns naturligt ljus samt artificiellt. Även under de grå dagarna finns det ljus och dess närvaro är alltid där även om den inte alltid är som klarast. I detta kandidatarbete beskrivs ljuset ur olika synvinklar, men framförallt hur Rembrandt och impressionisterna i sina konstverk använt ljuset i sitt skapande. För att få en bredare syn på vår process tar vi hjälp av de två begreppen “fluid” och “fire” som kommer från aktör-nätver...

  2. Simultan bilateral perifer facialisparese er en sjælden præsentationsform af Guillain-Barrés syndrom

    DEFF Research Database (Denmark)

    Frahm-Falkenberg, Siska; Dornonville de la Cour, Charlotte; Andersen, Preben Borring

    2014-01-01

    This case report describes a 63-year-old male presenting with five days progressing bilateral peripheral facial paralysis and mild sensory symptoms. Cerebrospinal fluid showed albumin-cytological dissociation. Nerve conduction studies showed slightly reduced conduction velocity, slightly prolonged...... F-wave latency and temporal dispersion. Following treatment with IV immunoglobulin (0.4 g/kg/day for five consecutive days) recovery was prominent, though incomplete. This is believed to be the first described case in Denmark of the very rare variant of Guillain-Barré syndrome termed "facial...

  3. [Visual functions' detailed evaluating in patients with Sjögren's syndrome before and after intracanalicular implants' (Smart Plug) insertion--(first results)].

    Science.gov (United States)

    Hejcmanová, D; Nemcová, I; Slezák, R

    2006-05-01

    The aim of the study was to determine exact visual functions (log MAR [minimal angle of resolution] and CS [contrast sensitivity]) and to evaluate corneal topographic maps in patients with established (by means of laboratory and biopsy examinations) Sjogren's Syndrome, and to determine the difference in subjective symptoms before and after insertion of the intracanalicular implants as well. Twelve eyes (1 man, 6 women) with established Sjogren's syndrome were examined before and during two months after the insertion of the plugs. The best-corrected visual acuity (BCVA) was assessed on Landolt C rings optotypes. CS was measured on computer-controlled device (Neuroscientific Corp., U.S.A.) in 6 space-frequencies (0.74-29.55 c/deg). The corneal topographic changes (Keraton Opticon) were established by means of comparing total aberrations values before and after the intracanalicular implants' (Smart Plugs type) insertion. The control group for visual functions assessment consisted of 10 woman (20 eyes) of similar middle age. The BCVA on log MAR optotypes was 0.84 (0.69-0.95) before and 0.88 (0.52-1.23) after the insertion, on both occasions, it was lower than in the control group. The CS was before the insertion in all of the spatial frequencies lower, the largest differences were in the frequencies range 1.97-7.29 c/deg (p test, in 100% positive before the treatment, was after the insertion in 75% negative; the height of the tear-meniscus was positive in 100% before the procedure, and after that, its measurement improved to 1 mm in 91%; in 9% it was 1.5 mm. We also noticed changes of the ocular surface by means of lissamine green staining; this test was before the procedure positive in 100%, the improvement after that was in 63%. The regularity of the corneal surface is the determining factor of visual functions in "dry eyes". The measurement of the corneal topography is useful in differential diagnosis and helps to distinguish mild and more serious conditions of dry eye. The improvement of BCVA and CS values after insertion corresponds with patients' subjective evaluating. The best value of the treatment is improvement of the patients' comfort; furthermore, after insertion of the permanent plugs they feel pronounced subjective relief and lowering of the frequency of drops application.

  4. The SSB-positive/SSA-negative antibody profile is not associated with key phenotypic features of Sjögren's syndrome

    DEFF Research Database (Denmark)

    Baer, Alan N; McAdams DeMarco, Mara; Shiboski, Stephen C

    2015-01-01

    phenotypic features. Among SICCA participants classified with SS on the basis of the American-European Consensus Group or American College of Rheumatology criteria, only 2% required the anti-SSB-alone test result to meet these criteria. CONCLUSIONS: The presence of anti-SSB, without anti-SSA antibodies, had...... participants, 2061 (63%) had negative anti-SSA/anti-SSB, 1162 (35%) had anti-SSA with or without anti-SSB, and 74 (2%) anti-SSB alone. Key SS phenotypic features were more prevalent and had measures indicative of greater disease activity in those participants with anti-SSA, either alone or with anti-SSB, than...... in those with anti-SSB alone or negative SSA/SSB serology. These between-group differences were highly significant and not explained by confounding by age, race/ethnicity or gender. Participants with anti-SSB alone were comparable to those with negative SSA/SSB serology in their association with these key...

  5. Structural characterisation of a water-soluble polysaccharide from tissue-cultured Dendrobium huoshanense C.Z. Tang et S.J. Cheng.

    Science.gov (United States)

    Si, Hua-Yang; Chen, Nai-Fu; Chen, Nai-Dong; Huang, Cheng; Li, Jun; Wang, Hui

    2018-02-01

    A water-soluble polysaccharide TC-DHPA4 with a molecular weight of 8.0 × 10 5  Da was isolated from tissue-cultured Dendrobium huoshanense by anion exchange and gel permeation chromatography. Monosaccharide analysis revealed that the homogeneous polysaccharide was made up of rhamnose, arabinose, mannose, glucose, galactose and glucuronic acid with a molar ratio of 1.28:1:1.67:4.71:10.43:1.42. The sugar residue sequence analysis based on the GC-MS files and NMR spectra indicated that the backbone of TC-DHPA4 consisted of the repeated units:→6)-β-Galp-(1→6)-β-Galp-(1→4)-β-GlcpA-(1→6)-β-Glcp-(1→6)-β-Glcp-(→. The sugar residue sequences β-Glcp-(1→)-α-Rhap-(1→3)-β-Galp-(1→, β-Glcp-(1→4)-α-Rhap-(1→3)-β-Galp-(1→, β-Galp-(1→6)-β-Manp-(1→3)-β-Galp-(1→, and α-l-Araf-(1→2)-β-Manp-(1→3)-β-Galp-(1→ were identified as the branches attached to the C-3 position of (1→6)-linked galactose in the backbone.

  6. Identification of a Sj?gren's syndrome susceptibility locus at OAS1 that influences isoform switching, protein expression, and responsiveness to type I interferons

    OpenAIRE

    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

    2017-01-01

    Sjogrens 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 performe...

  7. "Oftast du vet själv vad är det rätta" : Högstadieelevers sociala mediavanor och regelperception i onlinesammanhang

    OpenAIRE

    Bergman, Mikael

    2018-01-01

    Sammanfattning: Sociala medier spelar en allt större roll i hur vi kommunicerar och uttrycker oss. Denna studie är ämnad att utveckla och koppla ihop den redan existerande forskningen om ungdomars sociala mediavanor på Snapchat och Instagram, och forskning om vad sociala mediaanvändare upplever vad man kan och får publicera på dessa sociala medier. Denna studie utforskar detta genom tre fokusgruppsintervjuer med 15 åriga niondeklassare. Resultatet analyseras genom dramaturgi som till stor del...

  8. Självkänsla och bekräftelse på sociala medier : En kvantitativ enkätundersökning om självkänsla, bekräftelse på Instagram och skillnaden mellan män och kvinnor

    OpenAIRE

    Lööf, Malin

    2015-01-01

    This study aimed to investigate whether there was a correlation between an individual’s selfesteem and search for confirmation on social media. The study also sought to answer whether there were differences in self-esteem and search for confirmation from a gender perspective. In this study social media was defined as Instagram. The choice was founded on the basis that Instagram in the current situation is one of the world's largest social media that allows users to share their everydaylife in...

  9. Residual Salivary Secretion Ability May Be a Useful Marker for Differential Diagnosis in Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Etsuko Maeshima

    2014-01-01

    Full Text Available Background. We have elucidated decreased resting salivary flow in approximately 60% of patients with autoimmune diseases not complicated by Sjögren syndrome (SjS. In this study, salivary stimulation tests using capsaicin were performed to examine residual salivary secretion ability in patients with autoimmune diseases. Materials and Methods. Fifty-eight patients were divided into three groups: patients with primary or secondary SjS (SjS group, patients with systemic sclerosis not complicated by SjS (SSc group, and patients with other autoimmune diseases (non-SjS/non-SSc group. Simple filter paper and filter paper containing capsaicin were used to evaluate salivary flow rates. Results. Resting salivary flow rates were significantly lower in the SjS and SSc groups than in the non-SjS/non-SSc group but did not differ significantly between the SjS and SSc groups. Capsaicin-stimulated salivary flow rates were significantly lower in the SjS and SSc groups than in the non-SjS/non-SSc group, but not significantly different between the SjS and SSc groups. In the non-SjS/non-SSc group, salivary flow rates increased after capsaicin stimulation to the threshold level for determination of salivary gland dysfunction, whereas no improvement was observed in the SjS and SSc groups. Conclusion. Residual salivary secretion ability may be a useful marker for differential diagnosis in autoimmune diseases.

  10. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Virginia >> Washington >> Wisconsin > Special Support Groups > Canadian Support Groups > International Sjögren’s Network International Sjögren’s Network Volunteer as Patient Support Become ...

  11. Sleep Tips for Sjogren's Patients

    Science.gov (United States)

    ... Sjögren’s Patients.” Dr. Fisher reminds patients that adequate sleep is especially important for those with Sjögren’s syndrome, saying that sleep deprivation exacerbates daytime fatigue and can affect the ...

  12. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Patient Seminars History World Sjögren's Day Advocacy > Social Security Disability & Sjögren’s Board of Directors Conflict of Interest ... Fellowships > Student Fellowship Recipients Outstanding Abstract ... Initiatives Healthcare Provider Information > Diagnosis Criteria > Free ...

  13. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available ... Special Support Groups > Canadian Support Groups > International Sjögren’s Network International Sjögren’s Network Volunteer as Patient Support Become an Awareness Ambassador ...

  14. Anomalous left coronary artery from the pulmonary artery

    Science.gov (United States)

    ... anomalies of mitral valve. In: Sellke FW, del Nido PJ, Swanson SJ, eds. Sabiston and Spencer Surgery ... of the coronary arteries. In: Sellke FW, del Nido PJ, Swanson SJ, eds. Sabiston and Spencer Surgery ...

  15. Snake-like chromatin in conjunctival cells of a population aged 30-60 years from Copenhagen City

    DEFF Research Database (Denmark)

    Bjerrum, Kirsten Birgitte

    1998-01-01

    ophthalmology, keratoconjunctivitis sicca, Sjögrens Syndrome, epidemiology, imprint biopsy, snake-like chromatin......ophthalmology, keratoconjunctivitis sicca, Sjögrens Syndrome, epidemiology, imprint biopsy, snake-like chromatin...

  16. Sjogren's Syndrome: A Place to Begin

    Medline Plus

    Full Text Available YOUR CREDIBLE SJÖGREN'S RESOURCE Home | Login/Register | SSF Store | Contact Us About Sjögren's Symptoms > Dry Eyes >> Resources > Dry Mouth Diagnosis Treatments Patient Information Faces of Sjögren's Living ...

  17. Monte Carlo simulations of the stability of delta-Pu

    DEFF Research Database (Denmark)

    Landa, A.; Soderlind, P.; Ruban, Andrei

    2003-01-01

    The transition temperature (T-c) for delta-Pu has been calculated for the first time. A Monte Carlo method is employed for this purpose and the effective cluster interactions are obtained from first-principles calculations incorporated with the Connolly-Williams and generalized perturbation methods...

  18. Cognitive Adaptability: The Role of Metacognition and Feedback in Entrepreneural Decision Policies

    Science.gov (United States)

    2005-01-01

    24-34. 197 Earley, P.C., Connolly, T., & Lee, C. 1989. Task Strategy Interventions in Goal Setting: The Importance of Search in Strategy Development...diversification: a longitudinal study. Strategic Management Journal, 25: 1097-1119. Morris, B. 2002. Overcoming Dyslexia . Fortune Magazine, May 13. Mosakowski

  19. African Journal of Neurological Sciences 2013 - Vol. 32, No 2 http ...

    African Journals Online (AJOL)

    Gukiusu

    % vs. 10.5%, p = 0.007) were significantly higher among HIV + than HIV-. Similarly, stroke on admission ..... Neurology 2011 ; 76 : 444-50. 20. PATEL VB, SACOOR Z, FRANCIS P, BILL PL, BHIGJEE AI, Connolly C: Ischemic stroke in young.

  20. Study Design and Data Gathering Guide for Serious Games’ Evaluation

    NARCIS (Netherlands)

    Baalsrud Hauge, Jannicke; Boyle, Elizabeth; Mayer, Igor; Nadolski, Rob; Riedel, Johann C. K. H.; Moreno-Ger, Pablo; Bellotti, Francesco; Lim, Theodore; Ritchie, James

    2013-01-01

    Baalsrud Hauge, J., Boyle, E., Mayer, I., Nadolski, R. J., Riedel, J. C. K. H., Moreno-Ger, P., Bellotti, F., Lim, T., & Ritchie, J. (2013). Study Design and Data Gathering Guide for Serious Games’ Evaluation. In T. M. Connolly, T. Hainey, E. Boyle, G. Baxter, & P. Moreno-Ger (Eds.), Psychology,