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Sample records for systemic lupus erythematodes

  1. Excacerbation of systemic lupus erythematodes, aseptic meningitis and acute mental symptoms, following metrizamide lumbar myelography

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    Gelmers, H.J.

    1984-01-01

    A clinical constellation of excacerbation of systemic lupus erythematodes (SLE), together with aseptic meningitis, and acutre mental symptoms occurred following lumbar myelography with metrizamide. Excacerbation of SLE has not been previously described following myelography with any contrast agent. Meningeal reactions and acute mental symptoms have been reported earlier, but this clinical constellation is new.

  2. Tumour necrosis factor α (TNF-α, interleukin-6 (IL-6 and their soluble receptors (sTNF-α-Rp55 and slL-6R serum levels in systemic lupus erythematodes

    Directory of Open Access Journals (Sweden)

    E. Robak

    1996-01-01

    Full Text Available We investigated a possible association between serum concentrations of tumour necrosis factor α (TNF-α, interleukin-6 (IL-6 and their soluble receptors (sTNF-α-Rp55 and sIL-6R using an enzyme-linked immunosorbent assay (ELISA in 55 patients with systemic lupus erythematodes (SLE and 16 healthy controls. We also examined a possible association between the serum levels of these peptides and SLE activity, as well as TNF-α and IL-6 concentrations and the levels of their soluble receptors. The median concentrations of TNF-α, sTNF-α-Rp55 and IL-6 were significantly higher in SLE patients than in normal individuals. In contrast, there was no difference between the serum level of sIL-6R in both groups. We found positive correlations between the serum concentrations of TNF-α and IL-6 as well as their soluble receptors and disease activity. There were also correlations between TNF-α and sTNF-α-Rp55 as well as IL-6 and sIL-6R serum levels in SLE patients but there were no such correlations in the normal control group. In conclusion, an increase in the serum levels of TNF-α, sTNF-α-Rp55 and IL-6 may become useful markers for SLE activity. Patients with SLE have sIL-6R serum concentration similar to that as in normal individuals. However, it correlates with disease activity and the level of IL-6.

  3. Systemic Lupus Erythematosus (Lupus)

    Science.gov (United States)

    ... Topics English Español 한국어 繁體中文 tiếng Việt Systemic Lupus Erythematosus (Lupus) Basics In-Depth Download Download EPUB Download PDF What is it? Points To Remember About Systemic Lupus Erythematosus (Lupus) Lupus can affect many body parts, ...

  4. Kazuistika fyzioterapeutické péče o pacienta s diagnózou systémový lupus erythematodes

    OpenAIRE

    Mrázková, Tereza

    2015-01-01

    Title: Case study of physiotherapy treatment of patient with diagnosis systemic lupus erythematosus Objective: A summary of theoretical background, study of comprehensive rehabilitation methodology, proposal of therapy and evaluation of its effect in a patient with diagnosis systemic lupus erythematosus. Methods: The Review of literature and the case study were made during practice in Institute of Rheumatology in Prague from 5. to 30. 1. 2015. It is devided into two parts. Theoretical part su...

  5. Systemic lupus erythematosus

    Science.gov (United States)

    Disseminated lupus erythematosus; SLE; Lupus; Lupus erythematosus; Butterfly rash - SLE; Discoid lupus ... Arntfield RT, Jicks CM. Systemic lupus erythematosus and the ... Medicine . Philadelphia, PA: Elsevier Saunders; 2014:chap 118. ...

  6. Lupus

    Science.gov (United States)

    What is lupus? Lupus is an autoimmune disease. This means that your immune system attacks healthy cells and tissues by mistake. This can ... vessels, and brain. There are several kinds of lupus Systemic lupus erythematosus (SLE) is the most common ...

  7. Neuropsychiatric Systemic Lupus Erythematosus

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    Popescu, Alexandra; Kao, Amy H

    2011-01-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) is the least understood, yet perhaps the most prevalent manifestation of lupus. The pathogenesis of NPSLE is multifactorial and involves various inflammatory cytokines, autoantibodies, and immune complexes resulting in vasculopathic, cytotoxic and autoantibody-mediated neuronal injury. The management of NPSLE is multimodal and has not been subjected to rigorous study. Different treatment regimens include nonsteroidal anti-inflammatory drugs, anticoagulation, and immunosuppressives such as cyclophosphamide, azathioprine, mycophenolate mofetil, and methotrexate. For refractory NPSLE, intravenous immunoglobulin (IVIG), plasmapheresis, and rituximab have been used. Adjunctive symptomatic treatment complements these therapies by targeting mood disorders, psychosis, cognitive impairment, seizures or headaches. Several new biological agents are being tested including Belimumab, a human monoclonal antibody that targets B lymphocyte stimulator. This review focuses on the pathophysiology, treatment, and new potential therapies for neuropsychiatric manifestations of systemic lupus erythematosus. PMID:22379459

  8. Systemic lupus erythematosus

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    Jayakumar N

    2006-01-01

    Full Text Available Desquamative gingival lesions are non-plaque induced inflammatory gingival lesions. It is a clinical description and not a diagnosis. These desquamative lesions represent oral manifestations of various dermatoses. Systemic lupus erythematous (SLE, one of the rare dermatoses shows desquamative lesions as the oral manifestation. We here with report a case of SLE with oral lesions involving gingiva of a 36 year old female patient. The clinical presentation, histological features, and investigatory findings are discussed.

  9. Ectopic Axillary Breast during Systemic Lupus

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    Besma Ben Dhaou

    2012-01-01

    Full Text Available Many breast changes may occur in systemic lupus erythematosus. We report a 41-year-old woman with lupus who presented three years after the onset of lupus an ectopic mammary gland confirmed by histological study.

  10. Headache in Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Hanly, John G; Urowitz, Murray B; O'Keeffe, Aidan G

    2013-01-01

    To examine the frequency and characteristics of headaches and their association with global disease activity and health-related quality of life (HRQOL) in patients with systemic lupus erythematosus (SLE).......To examine the frequency and characteristics of headaches and their association with global disease activity and health-related quality of life (HRQOL) in patients with systemic lupus erythematosus (SLE)....

  11. Systemic lupus erythematosus conundrums

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    Wardle E

    2009-01-01

    Full Text Available Although the pathogenesis of systemic lupus erythematosus (SLE might now seem very complicated, various aspects are coming together to make a coherent story. Firstly, there is a loss of tolerance by the T and B lymphocytes, so accounting for the formation of autoantibodies that characterize this autoimmune disease. Failure of methylation of vital genes could underlie this aspect. Secondly, as much emphasized in recent years, poor clearance of apoptotic cells on account of de-fective phagocyte receptors and complement deficiencies involves stimulation of Toll like Receptors (TLRs 7 and 9 on plasmacytoid dendritic cells (pDCs and results in release of type I interferon alpha (IFNa. Thirdly, the familiar Th-1 lymphocytes produce interleukins 12 and 18 and inter-feron gamma (IFNy, along with chemokines, but now Th-17 lymphocytes are recognized to play an important role.

  12. Lupus cystitis: An unusual presentation of systemic lupus erythematosus

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    S Mukhopadhyay

    2014-01-01

    Full Text Available Lupus cystitis is a rare complication of systemic lupus erythematosus (SLE and occurs in association with gastrointestinal symptoms. This rare disorder has been reported mainly from Japan. We report a 20 year old female who diagnosed as having SLE associated with paralytic ileus and chronic interstitial cystitis. Treatment with intravenous methylprednisolone, cyclophosphamide pulse therapy followed by oral prednisolone and azathioprine led to amelioration of manifestations. Later she developed lupus nephritis which was treated with mycophenolate mofetil.

  13. Lupus cystitis: An unusual presentation of systemic lupus erythematosus.

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    Mukhopadhyay, S; Jana, S; Roy, M K; Chatterjee, A; Sarkar, A; Mazumdar, S; Mukherjee, P; Mukhopadhyay, J

    2014-09-01

    Lupus cystitis is a rare complication of systemic lupus erythematosus (SLE) and occurs in association with gastrointestinal symptoms. This rare disorder has been reported mainly from Japan. We report a 20 year old female who diagnosed as having SLE associated with paralytic ileus and chronic interstitial cystitis. Treatment with intravenous methylprednisolone, cyclophosphamide pulse therapy followed by oral prednisolone and azathioprine led to amelioration of manifestations. Later she developed lupus nephritis which was treated with mycophenolate mofetil.

  14. Incidence of systemic lupus erythematosus and lupus nephritis in Denmark

    DEFF Research Database (Denmark)

    Hermansen, Marie-Louise From; Lindhardsen, Jesper; Torp-Pedersen, Christian

    2016-01-01

    Objective. To determine the incidence of systemic lupus erythematosus (SLE) and SLE with concomitant or subsequent lupus nephritis (LN) in Denmark during 1995.2011, using data from the Danish National Patient Registry (NPR).  Methods. To assess the incidence of SLE, we identified all persons aged...

  15. Systemic lupus erythematosus in Denmark

    DEFF Research Database (Denmark)

    Voss, A; Green, A; Junker, P

    1998-01-01

    A population based cohort of patients with systemic lupus erythematosus (SLE) was recruited from a for epidemiological purposes representative Danish region. Patients were ascertained from 4 different sources with a high degree of completeness as estimated by using capture-recapture analysis...

  16. Lymphoma risk in systemic lupus

    DEFF Research Database (Denmark)

    Bernatsky, Sasha; Ramsey-Goldman, Rosalind; Joseph, Lawrence

    2014-01-01

    OBJECTIVE: To examine disease activity versus treatment as lymphoma risk factors in systemic lupus erythematosus (SLE). METHODS: We performed case-cohort analyses within a multisite SLE cohort. Cancers were ascertained by regional registry linkages. Adjusted HRs for lymphoma were generated...

  17. Lupus anticoagulants in systemic lupus erythematosus: prevalence and clinical associations.

    OpenAIRE

    Padmakumar, K; R R Singh; Rai, R; Malaviya, A N; Saraya, A K

    1990-01-01

    The prevalence of lupus anticoagulant (LAC) and its relation with reported clinical associations has been determined in 55 patients with systemic lupus erythematosus (SLE) from northern India who were studied prospectively. Kaolin clotting time was used to screen for LAC, which was detected in seven (13%) of the patients. Significant associations were found between LAC and thrombotic events, onset of disease at an early age, and disease of shorter duration. No statistically significant associ...

  18. Lupus cystitis: An unusual presentation of systemic lupus erythematosus

    OpenAIRE

    Mukhopadhyay, S.; Jana, S.; Roy, M. K.; Chatterjee, A.; Sarkar, A.; Mazumdar, S.; Mukherjee, P.; Mukhopadhyay, J.

    2014-01-01

    Lupus cystitis is a rare complication of systemic lupus erythematosus (SLE) and occurs in association with gastrointestinal symptoms. This rare disorder has been reported mainly from Japan. We report a 20 year old female who diagnosed as having SLE associated with paralytic ileus and chronic interstitial cystitis. Treatment with intravenous methylprednisolone, cyclophosphamide pulse therapy followed by oral prednisolone and azathioprine led to amelioration of manifestations. Later she develop...

  19. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Petri, Michelle; Orbai, Ana-Maria; Alarcón, Graciela S

    2012-01-01

    The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new...

  20. 75 FR 35492 - Guidance for Industry on Lupus Nephritis Caused By Systemic Lupus Erythematosus-Developing...

    Science.gov (United States)

    2010-06-22

    ... HUMAN SERVICES Food and Drug Administration Guidance for Industry on Lupus Nephritis Caused By Systemic... availability of a guidance for industry entitled ``Lupus Nephritis Caused By Systemic Lupus Erythematosus... nephritis (LN) caused by systemic lupus erythematosus (SLE). This guidance finalizes the parts of the draft...

  1. Infections and systemic lupus erythematosus.

    Science.gov (United States)

    Skare, Thelma Larocca; Dagostini, Jéssica Scherer; Zanardi, Patricia Imai; Nisihara, Renato Mitsunori

    2016-01-01

    To determine the incidence of infections in a population of systemic lupus erythematosus individuals and the characteristics of infections regarding original site, as well as to study the possible associations between infections and treatment. An analytical retrospective study using data from medical charts of systemic lupus erythematosus patients from a single university hospital. A total of 144 patients followed up for five years were included. Data collected comprised age of patients and age at onset of lupus, sex and ethnicity, disease duration before the study period, medications, cumulative dose of prednisone, occurrence of infections and their original site. The most frequent infections were urinary tract infections (correlated to use of prednisone - p<0.0001 and cyclophosphamide - p=0.045), upper airways infections (correlated to use of prednisone - p=0.0004, mycophenolate mofetil - p=0.0005, and cyclosporine - p=0.025), and pneumonia (associated to prednisone - p=0.017). Prednisone was the drug more often associated with presence of infections, pointing to the need for a more judicious management of this drug.

  2. 77 FR 38305 - Guidance for Industry on Lupus Nephritis Caused by Systemic Lupus Erythematosus-Developing...

    Science.gov (United States)

    2012-06-27

    ... HUMAN SERVICES Food and Drug Administration Guidance for Industry on Lupus Nephritis Caused by Systemic Lupus Erythematosus--Developing Medical Products for Treatment; Withdrawal of Guidance AGENCY: Food and... availability of a guidance entitled ``Lupus Nephritis Caused By Systemic Lupus Erythematosus--Developing...

  3. Genetics and pathogenesis of systemic lupus erythematosus and lupus nephritis.

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    Mohan, Chandra; Putterman, Chaim

    2015-06-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder that has a broad spectrum of effects on the majority of organs, including the kidneys. Approximately 40-70% of patients with SLE will develop lupus nephritis. Renal assault during SLE is initiated by genes that breach immune tolerance and promote autoantibody production. These genes might act in concert with other genetic factors that augment innate immune signalling and IFN-I production, which in turn can generate an influx of effector leucocytes, inflammatory mediators and autoantibodies into end organs, such as the kidneys. The presence of cognate antigens in the glomerular matrix, together with intrinsic molecular abnormalities in resident renal cells, might further accentuate disease progression. This Review discusses the genetic insights and molecular mechanisms for key pathogenic contributors in SLE and lupus nephritis. We have categorized the genes identified in human studies of SLE into one of four pathogenic events that lead to lupus nephritis. We selected these categories on the basis of the cell types in which these genes are expressed, and the emerging paradigms of SLE pathogenesis arising from murine models. Deciphering the molecular basis of SLE and/or lupus nephritis in each patient will help physicians to tailor specific therapies.

  4. "Bound" globulin in the skin of patients with chronic discoid lupus erythematosus and systemic lupus erythematosus

    NARCIS (Netherlands)

    Cormane, R.H.

    1964-01-01

    In what respect chronic discoid lupus erythematosus is related to systemic lupus erythematosus is still uncertain. In discoid lupus the lupus-erythematosus (L.E.) phenomenon is negative, and the history does not suggest vascular lesions or involvement of serous membranes. In both diseases the

  5. Lupus anticoagulants in systemic lupus erythematosus: prevalence and clinical associations.

    Science.gov (United States)

    Padmakumar, K; Singh, R R; Rai, R; Malaviya, A N; Saraya, A K

    1990-01-01

    The prevalence of lupus anticoagulant (LAC) and its relation with reported clinical associations has been determined in 55 patients with systemic lupus erythematosus (SLE) from northern India who were studied prospectively. Kaolin clotting time was used to screen for LAC, which was detected in seven (13%) of the patients. Significant associations were found between LAC and thrombotic events, onset of disease at an early age, and disease of shorter duration. No statistically significant association could be found between LAC and recurrent abortions, pulmonary hypertension, thrombocytopenia, and neurological manifestations. It is concluded that LAC is a useful marker for a subset of patients with SLE at risk of thromboembolic events. PMID:2125409

  6. Breast Cancer in Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Tessier Cloutier, B; Clarke, A E; Ramsey-Goldman, R

    2013-01-01

    Evidence points to a decreased breast cancer risk in systemic lupus erythematosus (SLE). We analyzed data from a large multisite SLE cohort, linked to cancer registries.......Evidence points to a decreased breast cancer risk in systemic lupus erythematosus (SLE). We analyzed data from a large multisite SLE cohort, linked to cancer registries....

  7. Systemic lupus erythematosus and exercise.

    Science.gov (United States)

    Ayán, C; Martín, V

    2007-01-01

    Systemic lupus erythematosus (SLE) is a rheumatic disease characterized by a variety of symptoms, especially fatigue, pain and reduced quality of life. Physical exercise is a useful tool for improving cardiovascular fitness, reducing metabolic abnormalities and fatigue and improving quality of life. However, very few studies have focused on the relationship between SLE and physical exercise. This paper reviews the main SLE symptoms that can be alleviated by exercising, as well as the results of studies seeking to analyse the exercise capacity and physical training possibilities of SLE patients. Considerations for future research are also discussed.

  8. Childhood-onset bullous systemic lupus erythematosus.

    Science.gov (United States)

    Lourenço, D M R; Gomes, R Cunha; Aikawa, N E; Campos, L M A; Romiti, R; Silva, C A

    2014-11-01

    Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childreńs Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  9. OSTEOPOROSIS IN SYSTEMIC LUPUS ERYTHEMATOSUS

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    N V Seredavkina

    2009-01-01

    Full Text Available Patients with systemic lupus erythematosus (SLE form a high risk group osteoporosis (OP. Its main causes are autoimmune inflammation, concomitant pathology, and their treatment. When OP occurs in SLE, bone mass loss is shown to occur early and is associated with the use of glucocorticosteroids (GC. To prevent OP, all patients with SLE should modify their lifestyle. To verify bone changes, densitometry is performed in patients who have risk factors of OP and/or a menopause. Calcium preparations and vitamin D are used to prevent OP; bisphosphonates that significantly reduce the risk of fractures of the vertebral column and femoral neck are employed for therapy of OP. A SLE patient with gluco-corticoid-induced OP and a good effect of bisphophonate treatment is described.

  10. Breast cancer in systemic lupus

    DEFF Research Database (Denmark)

    Bernatsky, S.; Ramsey-Goldman, R.; Petri, M.

    2017-01-01

    Objective There is a decreased breast cancer risk in systemic lupus erythematosus (SLE) versus the general population. We assessed a large sample of SLE patients, evaluating demographic and clinical characteristics and breast cancer risk. Methods We performed case-cohort analyses within a multi......-center international SLE sample. We calculated the breast cancer hazard ratio (HR) in female SLE patients, relative to demographics, reproductive history, family history of breast cancer, and time-dependent measures of anti-dsDNA positivity, cumulative disease activity, and drugs, adjusted for SLE duration. Results...... There were 86 SLE breast cancers and 4498 female SLE cancer-free controls. Patients were followed on average for 7.6 years. Versus controls, SLE breast cancer cases tended to be white and older. Breast cancer cases were similar to controls regarding anti-dsDNA positivity, disease activity, and most drug...

  11. Humor in systemic lupus erythematosus.

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    Moura, Cristiano S; Li, Rui; Lawrie, Sarah; Bar-Or, Amit; Clarke, Ann E; Da Costa, Deborah; Banerjee, Devi; Bernatsky, Sasha; Lee, Jennifer L; Pineau, Christian A

    2015-03-01

    Humor has neurophysiological effects influencing the release of cortisol, which may have a direct impact on the immune system. Laughter is associated with a decreased production of inflammatory cytokines both in the general population and in rheumatoid arthritis (RA). Our objective was to explore the effects of humor on serum cytokines [particularly interleukin-6 (IL-6)] and cortisol levels in systemic lupus erythematosus (SLE), after a standard intervention (120 min of visual comedy). We enrolled 58 females with SLE from consecutive patients assessed in the Montreal General Hospital lupus clinic. The subjects who consented to participate were randomized in a 1:1 ratio to the intervention (watching 120 min of comedy) or control group (watching a 120 min documentary). Measurements of cytokine and serum cortisol levels as well as 24-h urine cortisol were taken before, during, and after the interventions. We compared serum cytokine levels and serum and 24-h urine cortisol levels in the humor and control groups and performed regression analyses of these outcomes, adjusting for demographics and the current use of prednisone. There were no significant differences between the control and humor groups in demographics or clinical variables. Baseline serum levels of IL-6, IL-10, tumor necrosis factor-alpha, and B-cell activating factor were also similar in both groups. There was no evidence of a humor effect in terms of decreasing cytokine levels, although there was some suggestion of lowered cortisol secretion in the humor group based the 24-h urinary cortisol levels in a subgroup. In contrast to what has been published for RA, we saw no clear effects of humor in altering cytokine levels in SLE, although interesting trends were seen for lower cortisol levels after humor intervention compared with the control group.

  12. Systemic lupus erythematosus in India.

    Science.gov (United States)

    Malaviya, A N; Chandrasekaran, A N; Kumar, A; Shamar, P N

    1997-01-01

    The first case of systemic lupus erythematosus (SLE) was reported from India in 1995 followed by two more case reports and further, a series of eight cases, till 1969. Since the establishment of a clinical immunology laboratory at a major teaching institution in New Delhi in 1968, SLE was extensively studied and reported from that centre. From mid-1980 onwards several other centres in different regions in India including Chennai (old name Madras), Mumbai (old name Bombay), Calcutta and Hydrabad, also published their regional experience on SLE. Based on these data, the present report describes the clinical and laboratory characteristics of 1366 SLE patients seen in different regions of India. Arthritis, rash, photosensitivity, seizures and psychosis were seen in comparable proportions to other racial groups. Similarly, ANA and anti-DNA antibody positivity was also within the range seen in other racial groups. When compared with other series, however, alopecia, renal lupus, oral ulcers and neurological involvement was seen in higher proportions, reaching statistically significant figures in comparison to some racial groups. In contrast, haematological manifestations were seen in significantly less proportions in comparison to some of the racial groups. Serositis and discoid lesions were also seen in lower proportions than in most of other races. The proportion of those with anti-Sm antibodies was in between two extremes of highest among Africans and Israelis and lowest among Chinese and Europeans. Other manifestations were comparable to most other racial groups. Compared to North American and European reports, significantly low 5 and 10 year survival was observed among patients from India. This could be related to the general public health situation in the country including less than optimal management facilities in hospitals, delay in diagnosis due to lack of awareness of the disease, referral bias where only serious patients reach major city hospitals, or a truly

  13. Visceral leishmaniasis complicating systemic lupus erythematosus

    OpenAIRE

    Wallis, P. J. W.; Clark, C. J. M.

    1983-01-01

    Active systemic lupus erythematosus in a 32-year-old Chinese woman was successfully controlled by plasmapheresis and steroids. However, occult visceral leishmaniasis was uncovered during therapy and responded to appropriate treatment.

  14. [Systemic lupus erythematous and CD24v].

    Science.gov (United States)

    Jiménez-Uscanga, Rubén Darío; Carsolio-Trujano, Margarita; Herrera-Sánchez, Diana Andrea; Castrejón-Vázquez, María Isabel; Irazoque-Palacios, Fedra; Vargas-Camaño, María Eugenia; Martínez-Aguilar, Nora; Chima-Galán, María Carmen

    2015-01-01

    Systemic lupus erythematous is an autoimmune disease of multifactorial etiology with genetic predisposition. Its pathogenesis involved more than 100 genes. CD24 gene can mediate various functions such as their costimulatory activity in the clonal expansion of T cells. The single nucleotide polymorphism, resulting in a non-conservative replacement of alanine to valine (CD24v) precedes immediately GPI anchorage site (position ω-1), determines CD24 loss activity. CD24v has been associated with multiple sclerosis and systemic lupus erythematous in other populations. To find the presence of CD24v in Mexican patients with systemic lupus erythematous. A study of fenotyping of CD24v included 65 subjects, 32 cases (systemic lupus erythematous): 28 women and 4 men; and 32 controls: 9 women and 23 men; cases and controls from patients with systemic lupus erythematous in National Medical Center 20 de Noviembre ISSSTE, Mexico City, services of Clinical Immunology and Rheumatology. In cases, 19 patients had a wild homozygous genotype, 12 were heterozygous and only one patient showed homozygous polymorphism. In controls, 17 showed wild heterozygous genotypes; 14 were heterozygous and 1 was found to be polymorphic homozygote. With odds ratio: 0.84 and chi-squared of 0.17; therefore there was no statistically significant difference. Study population showed that there is no statistically significant difference between systemic lupus erythematous cases and controls with respect to the presence of CD24v.

  15. Periodontitis and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Manuela Rubim Camara Sete

    2016-04-01

    Full Text Available ABSTRACT A large number of studies have shown a potential association between periodontal and autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus (SLE. Similar mechanisms of tissue destruction concerning periodontitis and other autoimmune diseases have stimulated the study of a possible relationship between these conditions. This study aims to review the literature about this potential association and their different pathogenic mechanisms. Considering that periodontal disease is a disease characterized by inflammation influenced by infectious factors, such as SLE, it is plausible to suggest that SLE would influence periodontal disease and vice-versa. However, this issue is not yet fully elucidated and several mechanisms have been proposed to explain this association, as deregulation mainly in innate immune system, with action of phagocytic cells and proinflammatory cytokines such as IL-1β and IL-18 in both conditions’ pathogenesis, leading to tissue destruction. However, studies assessing the relationship between these diseases are scarce, and more studies focused on common immunological mechanisms should be conducted to further understanding.

  16. Osteonecrosis in systemic lupus erythematosus.

    Science.gov (United States)

    Gontero, Romina Patricia; Bedoya, María Eugenia; Benavente, Emilio; Roverano, Susana Graciela; Paira, Sergio Oscar

    2015-01-01

    To define the proportion of osteonecrosis (ON) in our patient population with lupus and to identify factors associated with the development of ON in systemic lupus erythematosus, as well as to carry out a descriptive analysis of ON cases. Observational retrospective study of 158 patients with SLE (ACR 1982 criteria). Demographic and laboratory data, clinical manifestations, SLICC, SLEDAI, cytotoxic and steroid treatments were compared. In patients with ON, we analyzed time of disease progression and age at ON diagnosis, form of presentation, joints involved, diagnostic methods, Ficat-Arlet classification, and treatment. To compare the means, t-test or Mann-Whitney's test were employed and the cHi-2 test or Fisher's exact test, as appropriate, were used to measure the equality of proportions. ON was present in 15 out 158 patients (9.5%), 13 women and 2 men, with a mean age of 30 (r: 16-66) at diagnosis and 35 months of evolution until diagnosis (r: 1-195). Among the 15 patients, 34 joints presented ON, 23 were symptomatic and 22 were diagnosed by magnetic resonance images. Twenty-six occurred in hips (24 bilateral), 4 in knees and 4 in shoulders. In 13 patients, ON involved 2 or more joints. At onset, 28 joints were in stage i-ii, one in stage iii and 5 had no data and; in the end, 14 were in stage iii-iv, 5 in stage i-ii and 15 had no data. Twenty-nine underwent conservative treatment with rest and 8 hips required joint replacement. ON progression was associated with Cushing's syndrome (P=0.014) OR 4.16 (95% CI 1.4-12.6) and 2nd year SLICC (P=0.042). No relation with clinical manifestations, lab results, cytotoxic treatment, steroid treatment (total accumulated dose, mean daily dose and duration) metilprednisolone pulses, nor activity was found. All patients with ON received antimalarials, in contrast to 77% of those without ON. The proportion of ON was 9.5%, mainly in women, 76% in hips (26) and 92% bilaterally. They were associated significantly with Cushing

  17. Thrombocytopenia in Systemic Lupus Erythematosus

    Science.gov (United States)

    Jung, Jin-Hee; Soh, Moon-Seung; Ahn, Young-Hwan; Um, Yoo-Jin; Jung, Ju-Yang; Suh, Chang-Hee; Kim, Hyoun-Ah

    2016-01-01

    Abstract The aim of the study was to examine the clinical characteristics and prognosis according to severity of thrombocytopenia and response to treatment for thrombocytopenia in patients with systemic lupus erythematosus (SLE). We retrospectively evaluated 230 SLE patients with thrombocytopenia, and reviewed their clinical data and laboratory findings. Thrombocytopenia was defined as platelet counts under 100,000/mm3, and patients were divided into 3 thrombocytopenia groups according to severity: mild (platelet counts >50,000/mm3), moderate (>20,000/mm3, ≤50,000/mm3), and severe (≤20,000/mm3). Clinical characteristics, treatments, and prognoses were compared among the groups. Furthermore, complete remission of thrombocytopenia was defined as platelet counts >100,000/mm3 after treatment. There was no significant difference in clinical or laboratory findings among the groups according to severity of thrombocytopenia. However, hemorrhagic complications were more frequent in severe thrombocytopenia (P thrombocytopenia (odds ratio = 0.049, 95% confidence interval: 0.013–0.191, P thrombocytopenia in SLE patients can be a useful independent prognostic factor to predict survival. Moreover, complete remission of thrombocytopenia after treatment is an important prognostic factor. The severity of thrombocytopenia and response to treatment should be closely monitored to predict prognosis in SLE patients. PMID:26871854

  18. A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients.

    Science.gov (United States)

    Chanprapaph, K; Sawatwarakul, S; Vachiramon, V

    2017-10-01

    Objective The aim of this study was to investigate the clinical features, laboratory findings, systemic manifestations, treatment and outcome of patients with bullous systemic lupus erythematosus in a tertiary care center in Thailand. Methods We performed a retrospective review from 2002 to 2014 of all patients who fulfilled the diagnostic criteria for bullous systemic lupus erythematosus to evaluate for the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence pattern, serological abnormalities, internal organ involvement, treatments and outcome. Results Among 5149 patients with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 15 developed vesiculobullous lesions. Ten patients had validation of the diagnosis of bullous systemic lupus erythematosus, accounting for 0.19%. Bullous systemic lupus erythematosus occurred after the diagnosis of systemic lupus erythematosus in six patients with a median onset of 2.5 months (0-89). Four out of 10 patients developed bullous systemic lupus erythematosus simultaneously with systemic lupus erythematosus. Hematologic abnormalities and renal involvement were found in 100% and 90%, respectively. Polyarthritis (40%) and serositis (40%) were less frequently seen. Systemic corticosteroids, immunosuppressants, antimalarials and dapsone offered resolution of cutaneous lesions. Conclusion Bullous systemic lupus erythematosus is an uncommon presentation of systemic lupus erythematosus. Blistering can occur following or simultaneously with established systemic lupus erythematosus. We propose that clinicians should carefully search for systemic involvement, especially hematologic and renal impairment, in patients presenting with bullous systemic lupus erythematosus.

  19. ACUTE RESPIRATORY DISEASE AS THE DEBUT OF SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    A. Yu. Ischenko

    2015-01-01

    Full Text Available Systemic lupus erythematosus — a chronic autoimmune disease that is often associated with infectious processes. The paper presents two clinical cases of systemic lupus erythematosus , debuted with acute respiratory infection.

  20. The Complement System in Lupus Nephritis.

    Science.gov (United States)

    Birmingham, Daniel J; Hebert, Lee A

    2015-09-01

    The complement system is composed of a family of soluble and membrane-bound proteins that historically has been viewed as a key component of the innate immune system, with a primary role of providing a first-line defense against microorganisms. Although this role indeed is important, complement has many other physiological roles, including the following: (1) influencing appropriate immune responses, (2) disposing of waste in the circulation (immune complexes, cellular debris), and (3) contributing to damage of self-tissue through inflammatory pathways. These three roles are believed to be significant factors in the pathogenesis of systemic lupus erythematosus, particularly its renal manifestation (lupus nephritis), contributing both protective and damaging effects. In this review, we provide an overview of the human complement system and its functions, and discuss its intricate and seemingly contradictory roles in the pathogenesis of lupus nephritis. Copyright © 2015. Published by Elsevier Inc.

  1. Lupus nephritis susceptibility loci in women with systemic lupus erythematosus.

    Science.gov (United States)

    Chung, Sharon A; Brown, Elizabeth E; Williams, Adrienne H; Ramos, Paula S; Berthier, Celine C; Bhangale, Tushar; Alarcon-Riquelme, Marta E; Behrens, Timothy W; Criswell, Lindsey A; Graham, Deborah Cunninghame; Demirci, F Yesim; Edberg, Jeffrey C; Gaffney, Patrick M; Harley, John B; Jacob, Chaim O; Kamboh, M Ilyas; Kelly, Jennifer A; Manzi, Susan; Moser-Sivils, Kathy L; Russell, Laurie P; Petri, Michelle; Tsao, Betty P; Vyse, Tim J; Zidovetzki, Raphael; Kretzler, Matthias; Kimberly, Robert P; Freedman, Barry I; Graham, Robert R; Langefeld, Carl D

    2014-12-01

    Lupus nephritis is a manifestation of SLE resulting from glomerular immune complex deposition and inflammation. Lupus nephritis demonstrates familial aggregation and accounts for significant morbidity and mortality. We completed a meta-analysis of three genome-wide association studies of SLE to identify lupus nephritis-predisposing loci. Through genotyping and imputation, >1.6 million markers were assessed in 2000 unrelated women of European descent with SLE (588 patients with lupus nephritis and 1412 patients with lupus without nephritis). Tests of association were computed using logistic regression adjusting for population substructure. The strongest evidence for association was observed outside the MHC and included markers localized to 4q11-q13 (PDGFRA, GSX2; P=4.5×10(-7)), 16p12 (SLC5A11; P=5.1×10(-7)), 6p22 (ID4; P=7.4×10(-7)), and 8q24.12 (HAS2, SNTB1; P=1.1×10(-6)). Both HLA-DR2 and HLA-DR3, two well established lupus susceptibility loci, showed evidence of association with lupus nephritis (P=0.06 and P=3.7×10(-5), respectively). Within the class I region, rs9263871 (C6orf15-HCG22) had the strongest evidence of association with lupus nephritis independent of HLA-DR2 and HLA-DR3 (P=8.5×10(-6)). Consistent with a functional role in lupus nephritis, intra-renal mRNA levels of PDGFRA and associated pathway members showed significant enrichment in patients with lupus nephritis (n=32) compared with controls (n=15). Results from this large-scale genome-wide investigation of lupus nephritis provide evidence of multiple biologically relevant lupus nephritis susceptibility loci. Copyright © 2014 by the American Society of Nephrology.

  2. Validation of the Lupus Nephritis Clinical Indices in Childhood-Onset Systemic Lupus Erythematosus.

    Science.gov (United States)

    Mina, Rina; Abulaban, Khalid; Klein-Gitelman, Marisa S; Eberhard, Barbara A; Ardoin, Stacy P; Singer, Nora; Onel, Karen; Tucker, Lori; O'neil, Kathleen; Wright, Tracey; Brooks, Elizabeth; Rouster-Stevens, Kelly; Jung, Lawrence; Imundo, Lisa; Rovin, Brad; Witte, David; Ying, Jun; Brunner, Hermine I

    2016-02-01

    To validate clinical indices of lupus nephritis activity and damage when used in children against the criterion standard of kidney biopsy findings. In 83 children requiring kidney biopsy, the Systemic Lupus Erythematosus Disease Activity Index renal domain (SLEDAI-R), British Isles Lupus Assessment Group index renal domain (BILAG-R), Systemic Lupus International Collaborating Clinics (SLICC) renal activity score (SLICC-RAS), and SLICC Damage Index renal domain (SDI-R) were measured. Fixed effects and logistic models were calculated to predict International Society of Nephrology/Renal Pathology Society (ISN/RPS) class; low-to-moderate versus high lupus nephritis activity (National Institutes of Health [NIH] activity index [AI]) score: ≤10 versus >10; tubulointerstitial activity index (TIAI) score: ≤5 versus >5; or the absence versus presence of lupus nephritis chronicity (NIH chronicity index) score: 0 versus ≥1. There were 10, 50, and 23 patients with ISN/RPS class I/II, III/IV, and V, respectively. Scores of the clinical indices did not differentiate among patients by ISN/RPS class. The SLEDAI-R and SLICC-RAS but not the BILAG-R differed with lupus nephritis activity status defined by NIH-AI scores, while only the SLEDAI-R scores differed between lupus nephritis activity status based on TIAI scores. The sensitivity and specificity of the SDI-R to capture lupus nephritis chronicity was 23.5% and 91.7%, respectively. Despite being designed to measure lupus nephritis activity, SLICC-RAS and SLEDAI-R scores significantly differed with lupus nephritis chronicity status. Current clinical indices of lupus nephritis fail to discriminate ISN/RPS class in children. Despite its shortcomings, the SLEDAI-R appears best for measuring lupus nephritis activity in a clinical setting. The SDI-R is a poor correlate of lupus nephritis chronicity. © 2016, American College of Rheumatology.

  3. Intravenous immunoglobulin therapy and systemic lupus erythematosus.

    Science.gov (United States)

    Zandman-Goddard, Gisele; Levy, Yair; Shoenfeld, Yehuda

    2005-12-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with diverse manifestations. We suggest that intravenous immunoglobulin (IVIg) therapy may be beneficial and safe for various manifestations in SLE. A structured literature search of articles published on the efficacy of IVIg in the treatment of SLE between 1983 and 2005 was conducted. We searched the terms "IVIg," "intravenous immunoglobulin," "lupus," "SLE," and "systemic lupus erythematosus." The various clinical manifestations of SLE that were reported to be successfully treated by IVIg in case reports include autoimmune hemolytic anemia, acquired factor VIII inhibitors, acquired von Willebrand disease, pure red cell aplasia, thrombocytopenia, pancytopenia, myelofibrosis, pneumonitis, pleural effusion, pericarditis, myocarditis, cardiogenic shock, nephritis, end-stage renal disease, encephalitis, neuropsychiatric lupus, psychosis, peripheral neuropathy, polyradiculoneuropathy, and vasculitis. The most extensive experience is with lupus nephritis. There are only a few case series of IVIg use in patients with SLE with various manifestations, in which the response rate to IVIg therapy ranged from 33 to 100%. We suggest that IVIg devoid of sucrose, at a dose of 2 g/kg over a 5-d period given uniformly and at a slow infusion rate in patients without an increased risk for thromboembolic events or renal failure, is a safe and beneficial adjunct therapy for cases of SLE that are resistant to or refuse conventional treatment. The duration of therapy is yet to be established. Controlled trials are warranted.

  4. Mood Disorders in Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Hanly, John G; Su, Li; Urowitz, Murray B

    2015-01-01

    OBJECTIVE: To examine the frequency, characteristics, and outcome of mood disorders, as well as clinical and autoantibody associations, in a multiethnic/racial, prospective inception cohort of patients with systemic lupus erythematosus (SLE). METHODS: Patients were assessed annually for mood...... disorders (4 types, according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition) and 18 other neuropsychiatric events. Global disease activity scores (SLE Disease Activity Index 2000 [SLEDAI-2K]), damage scores (Systemic Lupus International Collaborating Clinics/American College...... was associated with Asian race/ethnicity (P = 0.01) and treatment with immunosuppressive drugs (P = 0.003). Mood disorders were associated with lower mental health and mental component summary scores but not with the SLEDAI-2K, SDI, or lupus autoantibodies. Among the 232 patients with depression, 168 (72...

  5. Systemic Lupus Erythematosus Presenting as Acute Adrenal ...

    African Journals Online (AJOL)

    hanumantp

    Kashmir, India. E‑mail: bhatdrriaz@hotmail.com. Introduction. Systemic lupus erythematosus (SLE) is the prototypic autoimmune disease characterized by the production of autoantibodies to components of the cell nucleus and is an association with diverse clinical manifestations encompassing almost all organ systems.

  6. Systemic lupus erythematous revealed by cytomegalovirus infection ...

    African Journals Online (AJOL)

    Cytomegalovirus (CMV) infection have been described as exacerbing systemic lupus erythematous (SLE). The role of CMV in starting off SLE remains object of debate. We report a severe presentation of SLE revealed by CMV infection with hemophogocytic syndrome. A 22 old women without a history of systemic disease ...

  7. Echocardiographic abnormalities in systemic lupus erythematosus ...

    African Journals Online (AJOL)

    Background: The cardiovascular system is frequently affected in patients with Systemic Lupus Erythematosus (SLE). Involvement of the pericardium, endocardium, myocardium, coronary and pulmonary vessels has been found in several clinical and autopsy studies in patients with SLE; most of which can be detected by ...

  8. Systemic lupus erythematosus exacerbated by piroxicam.

    Science.gov (United States)

    Roura, M; Lopez-Gil, F; Umbert, P

    1991-01-01

    A patient with Sjögren's syndrome and seronegative polyarthritis is reported. After piroxicam intake and sun exposure she developed subacute cutaneous lupus erythematosus lesions with Ro antibodies. Despite drug withdrawal, typical cutaneous lesions and serological markers of systemic lupus erythematosus (SLE) progressively appeared. The use of piroxicam and other nonsteroidal anti-inflammatory drugs with photosensitizing potential in patients with Sjögren's syndrome, sicca syndrome or a high suspicion of a collagen disorder should be avoided because these drugs may trigger a latent SLE.

  9. ENDOCARDITIS IN SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    AMEL Harzallah

    2017-04-01

    Full Text Available Endocarditis is one of the most prevalent forms of cardiac involvement in patients with lupus, as it is considered as one a life-threatening complication. Libman-Sacks endocarditis is common. Infective endocarditis can also cause complications within immunocompromised patients. The aim of this study is to determine particularities of endocarditis in patients with lupus and to look for distinguishing features between infectious or immunological origin. A retrospective study was conducted on patients with lupus presenting endocarditis. Lupus was diagnosed according to the American college of rheumatology criteria. The diagnosis of endocarditis was made based on the modified Duke criteria. The present case report studies seven cases of endocarditis. Six of these patients are women and the other one is a man. They are aged meanly of 29.4 years (extremes: 20-36. Fever was present in all the cases with a new cardiac murmur in six cases and a modification of its intensity in one case. Biologic inflammatory syndrome was present in six cases. Cardiac ultrasound performed in six cases made the diagnosis of endocarditis which involved the left heart valves in five cases and the right heart valves in one case. Valvular insufficiency was identified in six patients. The valve involvement was mitral in two cases, mitro-aortic in two others, aortic in the fifth one and tricuspid in the sixth one. Endocarditis was infectious in 4 cases, thanks to positive blood culture. The germs identified were gram negative bacilli in two cases, anaerobic organism in one case and gram positive cocci in one case. Candida albicans was isolated in one case. Libman-Sacks endocarditis was objectified in three cases. A combination of Libman-Sacks endocarditis with infectious endocarditis was diagnosed in one case. The treatment consisted of antibiotics in four cases with surgery in two cases. The outcome was favorable in five cases and fatal in the two others. Endocarditis in lupus

  10. [Seronegative systemic lupus erythematosus and autoimmune thyroiditis].

    Science.gov (United States)

    González-Gay, M A; Cereijo, M J; Agüero, J J; Alonso, M D; Fernández Sueiro, J L; Vidal, J I

    1993-08-01

    The association of systemic lupus erythematosus (SLE) and autoimmune thyroiditis has been previously described. We report a woman with negative antinuclear antibodies (ANA) and criteria for the diagnosis of SLE. The patient was also diagnosed with autoimmune thyroiditis. We review the clinical characteristics and the association of both entities. We also remark in this case the association of autoimmune thyroiditis with seronegative SLE.

  11. Autoimmune hepatitis and juvenile systemic lupus erythematosus

    NARCIS (Netherlands)

    Deen, M. E. J.; Porta, G.; Fiorot, F. J.; Campos, L. M. A.; Sallum, A. M. E.; Silva, C. A. A.

    Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that

  12. Clinical Characteristics of Patients with Systemic Lupus ...

    African Journals Online (AJOL)

    Background: Systemic lupus erythematosus (SLE), a chronic multisystem autoimmune disease with a wide spectrum of manifestations, shows considerable variation across the globe, although there is data from Africa is limited. Quantifying the burden of SLE across Africa can help raise awareness and knowledge about the ...

  13. Hypocomplementemic urticarial vasculitis in systemic lupus erythematosus.

    Science.gov (United States)

    Her, Min Young; Song, Joo Yeon; Kim, Dong Yook

    2009-02-01

    Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.

  14. Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

    OpenAIRE

    Her, Min Young; Song, Joo Yeon; Kim, Dong Yook

    2009-01-01

    Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.

  15. Systemic lupus erythematosus. Unusual cutaneous manifestations

    NARCIS (Netherlands)

    Stockinger, T.; Richter, L.; Kanzler, M.; Melichart-Kotik, M.; Pas, H.; Derfler, K.; Schmidt, E.; Rappersberger, K.

    2016-01-01

    Various different mucocutaneous symptoms may affect up to 80 % of systemic lupus erythematosus (SLE) patients. To investigate, various unspecific, but otherwise typical clinical symptoms of skin and mucous membranes that arise in SLE patients other than those defined as SLE criteria such as

  16. Systemic lupus erythematosus : a behavioural medicine perspective

    NARCIS (Netherlands)

    Daleboudt, Gabriëlle Mathilde Nicoline

    2014-01-01

    Systemic lupus erythematosus (SLE) and its immunosuppressive treatment have a great impact on the patient’s life. Previous studies on SLE have focused on the optimisation of diagnosis and treatment. Less attention has been given to the impact of diagnosis and treatment on patients’ well-being.

  17. [Determinant Factors of Morbidity in Patients with Systemic Lupus Erythematosus].

    Science.gov (United States)

    Jacinto, Margarida; Silva, Eliana; Riso, Nuno; Moraes-Fontes, Maria Francisca

    2017-05-31

    Severity in systemic lupus erythematosus may vary from mild to even fatal consequences. There are no biomarkers to predict the disease's prognosis. The Systemic Lupus International Collaborating Clinics/ Systemic Damage Index defines systemic lupus erythematosus disease severity and is found to predict prognosis. To test damage determinants in a single-centre systemic lupus erythematosus cohort. Retrospectively followed systemic lupus erythematosus female patients (defined by the identification of at least four systemic lupus erythematosus American College of Rheumatology criteria - fulfillment 100%, n = 76) over the past five years. Age of onset, ethnicity, disease duration, number of American College of Rheumatology criteria at the end of follow-up, cumulative: renal, neuropsychiatric and articular phenotypes, hypertension, dyslipidaemia, smoking and Systemic Lupus Erythematosus Disease Activity Index 2K were correlated to the presence and degree of irreversible damage (Systemic Lupus International Collaborating Clinics Damage Index). Accumulation of American College of Rheumatology criteria was measured in a sub-group of patients followed from disease onset (within a year of the first symptom ascribed to systemic lupus erythematosus) (n = 39 - 51%); Systemic Lupus Erythematosus Disease Activity Index and Systemic Lupus International Collaborating Clinics Damage Index were performed. Statistical analysis was performed using Chi-square, Wilcoxon Mann-Whitney tests and Spearman correlation rho (Sig. 2-tailed p Systemic Lupus International Collaborating Clinics/Systemic Damage Index > 0 was present in 56.6% and significantly associated to a longer duration, a higher number of American College of Rheumatology criteria and a neuropsychiatric phenotype when compared with those with no damage. The final number of American College of Rheumatology criteria accrued was positively correlated to a higher disease activity over the past five years of follow-up (Spearman´s rho

  18. Epigenetics and Systemic Lupus Erythematosus: Unmet Needs.

    Science.gov (United States)

    Meroni, Pier Luigi; Penatti, Alessandra Emiliana

    2016-06-01

    Systemic lupus erythematosus (SLE) is a chronic relapsing-remitting autoimmune disease affecting several organs. Although the management of lupus patients has improved in the last years, several aspects still remain challenging. More sensitive and specific biomarkers for an early diagnosis as well as for monitoring disease activity and tissue damage are needed. Genome-wide association and gene mapping studies have supported the genetic background for SLE susceptibility. However, the relatively modest risk association and the studies in twins have suggested a role for environmental and epigenetic factors, as well as genetic-epigenetic interaction. Accordingly, there is evidence that differences in DNA methylation, histone modifications, and miRNA profiling can be found in lupus patients versus normal subjects. Moreover, impaired DNA methylation on the inactive X-chromosome was suggested to explain, at least in part, the female prevalence of the disease. Epigenetic markers may be help in fulfilling the unmet needs for SLE by offering new diagnostic tools, new biomarkers for monitoring disease activity, or to better characterize patients with a silent clinical disease but with an active serology. Anti-DNA, anti-phospholipid, and anti-Ro/SSA autoantibodies are thought to be pathogenic for glomerulonephritis, recurrent thrombosis and miscarriages, and neonatal lupus, respectively. However, tissue damage occurs occasionally or, in some patients, only in spite of the persistent presence of the antibodies. Preliminary studies suggest that epigenetic mechanisms may explain why the damage takes place in some patients only or at a given time.

  19. Cutaneous lupus erythematosus and systemic lupus erythematosus are associated with clinically significant cardiovascular risk

    DEFF Research Database (Denmark)

    Hesselvig, J Halskou; Ahlehoff, O; Dreyer, L

    2017-01-01

    Systemic lupus erythematosus (SLE) is a well-known cardiovascular risk factor. Less is known about cutaneous lupus erythematosus (CLE) and the risk of developing cardiovascular disease (CVD). Therefore, we investigated the risk of mortality and adverse cardiovascular events in patients diagnosed...

  20. Pregnancy complications in a patient with systemic lupus erythematosus and lupus nephritis

    DEFF Research Database (Denmark)

    Bisgaard, Helene; Jacobsen, Søren; Tvede, Niels

    2014-01-01

    A woman with systemic lupus erythematosus (SLE) and lupus nephritis had two pregnancies which both resulted in complications known to be associated with SLE, i.e. late abortion, preterm delivery and pre-eclampsia. We conclude that disease quiescence is important for a successful outcome...

  1. Central nervous system involvement in systemic lupus erythematosus.

    Institute of Scientific and Technical Information of China (English)

    1994-01-01

    This paper deals with the clinical, immunological and pathological data of 5 eases of systemic lupus erythematosus (SLE). Each of the five cases has typical SLE damages on the skin and multiple organs. Among

  2. Concomitant systemic lupus erythematosus and ankylosing spondylitis.

    OpenAIRE

    Olivieri, I; Gemignani, G; Balagi, M; Pasquariello, A; Gremignai, G; G. Pasero

    1990-01-01

    The case is reported of a 42 year old white woman meeting currently used diagnostic criteria for both ankylosing spondylitis and systemic lupus erythematosus (SLE). As found in a previously described similar case of a black man, HLA typing showed antigens associated with both SLE and seronegative spondyloarthropathy. This case thus supports the hypothesis that the two diseases occur together only when this rare combination of HLA antigens is present.

  3. Concomitant systemic lupus erythematosus and ankylosing spondylitis.

    Science.gov (United States)

    Olivieri, I; Gemignani, G; Balagi, M; Pasquariello, A; Gremignai, G; Pasero, G

    1990-05-01

    The case is reported of a 42 year old white woman meeting currently used diagnostic criteria for both ankylosing spondylitis and systemic lupus erythematosus (SLE). As found in a previously described similar case of a black man, HLA typing showed antigens associated with both SLE and seronegative spondyloarthropathy. This case thus supports the hypothesis that the two diseases occur together only when this rare combination of HLA antigens is present.

  4. Intraveous gammaglobulin in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Badillo Tenorio Rocio Elizabeth

    2014-07-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic autoinmune multisystemic disease. Intravenous immunoglobulins (IVIg have been previously used for the treatment of primary immunodefi- ciency diseases. In addition IVIg is used for a few autoimmune diseases, such as immune thrombocytopenic purpura. IVIg is an immunomodulatory agent capable of modulating autoinmu- nity and also provide defense against infection, IVIg has been used successfully in SLE. We review the role of IVIg in SLE as a therapeutic option.

  5. Sweet syndrome revealing systemic lupus erythematosus.

    LENUS (Irish Health Repository)

    Quinn, N

    2015-02-01

    Sweet Syndrome is an acute inflammatory skin eruption which is rare in children. We report a case of childhood Systemic Lupus Erythematosus (SLE) that presented with Sweet syndrome. This case is a unique presentation of a common disorder which provides a new facet for the differential diagnosis of SLE in children. It is also the first paediatric case to be reported in a Caucasian child.

  6. Cutaneous Manifestations of Systemic Lupus Erythematosus

    OpenAIRE

    Uva, Luís; Miguel, Diana; Pinheiro, Catarina; Freitas, João Pedro; Marques Gomes, Manuel; Filipe, Paulo

    2012-01-01

    Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these...

  7. Systemic lupus erythematosus : abdominal radiologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jae Cheon; Cho, On Koo; Lee, Yong Joo; Bae, Jae Ik; Kim, Yong Soo; Rhim, Hyun Chul; Ko, Byung Hee [Hanyang Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-06-01

    Systemic lupus erythematosus(SLE) is a systemic disease of unknown etiology. Its main pathology is vasculitis and serositis, due to deposition of the immune complex or antibodies. Most findings are nonspecific ; abdominal manifestations include enteritis, hepatomegaly, pancreatic enlargement, serositis, lymphadenopathy, splenomegaly, nephritis, interstitial cystitis, and thrombophlebitis. We described radiologic findings of various organ involvement of SLE; digestive system, serosa, reticuloendothelial system, urinary system, and venous system. Diagnosis of SLE was done according to the criteria of American Rheumatism Association. Understanding of the variable imaging findings in SLE may be helpful for the early detection of abdominal involvement and complications.

  8. Gestational outcomes in patients with neuropsychiatric systemic lupus erythematosus.

    Science.gov (United States)

    de Jesus, G R; Rodrigues, B C; Lacerda, M I; Dos Santos, F C; de Jesus, N R; Klumb, E M; Levy, R A

    2017-04-01

    This study analyzed maternal and fetal outcomes of pregnancies of neuropsychiatric systemic lupus erythematosus patients followed in a reference unit. This retrospective cohort study included 26 pregnancies of patients seen between 2011 and 2015 included with history and/or active neuropsychiatric systemic lupus erythematosus among 135 pregnancies. Three patients had active neuropsychiatric systemic lupus erythematosus at conception, but only one remained with neurological activity during gestation, characteristically related to the inadvertent suspension of medications. Twenty six percent of the newborns were small for gestational age and 40% of live births were premature, with no neonatal death or early complications of prematurity. Preeclampsia was diagnosed in nine pregnancies, with two cases of early severe form that resulted in intrauterine fetal death. Patients with neuropsychiatric systemic lupus erythematosus had more prematurity and preeclampsia compared to patients without neuropsychiatric disease. However, when concomitant lupus nephritis was excluded, the gestational results of neuropsychiatric systemic lupus erythematosus patients were more favorable.

  9. Hydroxychloroquine and pregnancy on lupus flares in Korean patients with systemic lupus erythematosus.

    Science.gov (United States)

    Koh, J H; Ko, H S; Kwok, S-K; Ju, J H; Park, S-H

    2015-02-01

    We investigated the clinical and laboratory characteristics of pregnancies with systemic lupus erythematosus (SLE) and identified lupus flare predictors during pregnancy. Additionally, we examined lupus activity and pregnancy outcomes in SLE patients who continued, discontinued or underwent no hydroxychloroquine (HCQ) treatment during pregnancy. We retrospectively analyzed 179 pregnancies in 128 SLE patients at Seoul St. Mary's Hospital, Korea, between 1998 and 2012 and then assessed the clinical profiles and maternal and fetal outcomes. Overall, 90.5% of pregnancies resulted in a successful delivery and were divided into two groups: those who experienced lupus flares (80 pregnancies, 44.7%) and those who did not (99 pregnancies, 55.3%). Increased preeclampsia, preterm births, low birth weight, intrauterine growth restriction (IUGR), and low 1-minute Apgar scores occurred in pregnancies with lupus flares compared to pregnancies in quiescent disease. Lupus flares were predicted by HCQ discontinuation, a history of lupus nephritis, high pre-pregnancy serum uric acid and low C4 levels. Our study indicates that achieving pre-pregnancy remission and continuing HCQ treatment during pregnancy are important for preventing lupus flares. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  10. Dengue fever triggering systemic lupus erythematosus and lupus nephritis: a case report

    Directory of Open Access Journals (Sweden)

    Talib SH

    2013-10-01

    Full Text Available SH Talib, SR Bhattu, R Bhattu, SG Deshpande, DB Dahiphale Department of Medicine and Nephrology, MGM Medical College and Hospital, Aurangabad, Maharashtra, India Abstract: We report a rare case of dengue fever triggering systemic lupus erythematosus and lupus nephritis. The patient presented herself during a large outbreak of dengue fever in December 2012 in Maharashtra, India. The diagnosis of dengue fever was confirmed by the presence of NS-1 antigen during the first few days of febrile illness. Eight weeks later, kidney tissue biopsy studies revealed evidence of lupus nephritis on microscopic examination and immunofluorescence. The report interpreted it as focal proliferative glomerulonephritis and segmental sclerosis (Stage IIIC. The case was also found positive for perinuclear antineutrophil cytoplasmic antibodies by indirect immunofluorescence assay. An active and effective management of a case essentially calls for clear perception of differentiating dengue-induced lupus flare, antineutrophil cytoplasmic antibody-related nephropathy, and/or dengue-induced de-novo lupus disease. Dengue viremia may be the trigger for immune complex formation in patients who are predisposed to developing autoimmune diseases. The present case explains the importance of considering the diagnosis of dengue-related lupus nephritis as an atypical occurrence in appropriate situations, as in this case. It would not be improper to regard this escalating disease as an expanded feature of dengue. Keywords: kidney biopsy, glomerulonephritis, segmental sclerosis, lupus flare, dengue viremia, autoimmune, de-novo lupus nephritis

  11. Lupus pneumonitis as the initial presentation of systemic lupus erythematosus: case series from a single institution.

    Science.gov (United States)

    Wan, S A; Teh, C L; Jobli, A T

    2016-11-01

    Objective The aim of this study was to examine the clinical features, treatment and outcome of systemic lupus erythematosus (SLE) patients in our centre who presented with lupus pneumonitis as the initial manifestation. Methods We performed a retrospective review of all patients who presented with lupus pneumonitis during the initial SLE manifestation from March 2006 to March 2015. Results There were a total of five patients in our study who presented with fever and cough as the main clinical features. All patients had pulmonary infiltrates on chest radiographs. High-resolution computed tomography, which was performed in two patients, showed ground glass opacities with patchy consolidations bilaterally. All patients received high-dose steroids, 80% received intravenous cyclophosphamide and 60% received intravenous immunoglobulin. Two patients died from severe lupus pneumonitis within 2 weeks of admission despite treatment with ventilation, steroids, cyclophosphamide and intravenous immunoglobulin. Conclusions Acute lupus pneumonitis is an uncommon presentation of SLE. Mortality in this case series is 40%.

  12. Different Types of Lupus

    Science.gov (United States)

    ... Twitter Facebook Pinterest Email Print Different types of lupus Lupus Foundation of America September 18, 2017 Resource ... lupus. Learn more about each type below. Systemic lupus erythematosus Systemic lupus is the most common form ...

  13. Cutaneous Manifestations of Systemic Lupus Erythematosus

    Science.gov (United States)

    Uva, Luís; Miguel, Diana; Pinheiro, Catarina; Freitas, João Pedro; Marques Gomes, Manuel; Filipe, Paulo

    2012-01-01

    Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient. PMID:22888407

  14. Acquired enophthalmos with systemic lupus erythematosus.

    Science.gov (United States)

    Park, K R; Seo, M R; Ryu, H J; Chi, M J; Baek, H J; Choi, H J

    2016-01-01

    Ocular involvement sometimes occurs with systemic lupus erythematosus (SLE) but enophthalmos with SLE is rare. We report a case of enophthalmos with SLE. A 25-year-old male was admitted for two weeks of fever, sore throat, arthralgia, chest pain and right arm weakness with pain. We diagnosed him with SLE with malar rash, arthritis, pleural effusion, proteinuria, leukopenia, positive antinuclear antibody, anti-dsDNA, and lupus anticoagulant. The patient was prescribed high-dose prednisolone and hydroxychloroquine 400 mg. One week after discharge, he complained about a sensation of a sunken right eye. CT showed right enophthalmos, a post-inflammatory change and chronic inflammation. Proteinuria increased to 3.8 g/day after the patient stopped taking prednisolone. Cyclophosphamide therapy was administered for three months without improvement. We decided to restart prednisolone and change cyclophosphamide to mycophenolate mofetil. Proteinuria decreased but enophthalmos remains as of this reporting. © The Author(s) 2015.

  15. Cutaneous Manifestations of Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Luís Uva

    2012-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient.

  16. Cutaneous manifestations of systemic lupus erythematosus.

    Science.gov (United States)

    Uva, Luís; Miguel, Diana; Pinheiro, Catarina; Freitas, João Pedro; Marques Gomes, Manuel; Filipe, Paulo

    2012-01-01

    Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient.

  17. Lupus

    Science.gov (United States)

    ... age. race/ethnicity: Lupus occurs more often in African-American, Asian-American, Latin-American, and Native-American women than in non-Hispanic Caucasian women. family history/genetics: About 10% of people with lupus have ...

  18. Monoclonal Antibodies for Systemic Lupus Erythematosus (SLE

    Directory of Open Access Journals (Sweden)

    Gabriella Moroni

    2010-01-01

    Full Text Available A number of monoclonal antibodies (mAb are now under investigation in clinical trials to assess their potential role in Systemic Lupus Erythematosus (SLE. The most frequently used mAb is rituximab, which is directed against CD20, a membrane protein expressed on B lymphocytes. Uncontrolled trials reported an improvement of SLE activity in non-renal patients and other studies even reported an improvement of severe lupus nephritis unresponsive to conventional treatments. However two randomized trials failed to show the superiority of rituximab over conventional treatment in non renal SLE and in lupus nephritis. Preliminary trials reported promising results with epratuzumab, a humanized mAb directed against CD22, and with belimumab, a human mAb that specifically recognizes and inhibits the biological activity of BLyS a cytokine of the tumornecrosis-factor (TNF ligand superfamily. Other clinical trials with mAb directed against TNF-alpha, interleukin-10 (Il-10, Il-6, CD154, CD40 ligand, IL-18 or complement component C5 are under way. At present, however, in spite of good results reported by some studies, no firm conclusion on the risk-benefit profile of these mAbs in patients with SLE can be drawn from the available studies.

  19. Catatonia due to systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Francisco de Assis Pinto Cabral Júnior Rabello

    2014-07-01

    Full Text Available Objectives Discuss neuropsychiatric aspects and differential diagnosis of catatonic syndrome secondary to systemic lupus erythematosus (SLE in a pediatric patient. Methods Single case report. Result A 13-year-old male, after two months diagnosed with SLE, started to present psychotic symptoms (behavioral changes, hallucinations and delusions that evolved into intense catatonia. During hospitalization, neuroimaging, biochemical and serological tests for differential diagnosis with metabolic encephalopathy, neurological tumors and neuroinfections, among other tests, were performed. The possibility of neuroleptic malignant syndrome, steroid-induced psychosis and catatonia was also evaluated. A complete reversal of catatonia was achieved after using benzodiazepines in high doses, associated with immunosuppressive therapy for lupus, which speaks in favor of catatonia secondary to autoimmune encephalitis due to lupus. Conclusion Although catatonia rarely is the initial clinical presentation of SLE, the delay in recognizing the syndrome can be risky, having a negative impact on prognosis. Benzodiazepines have an important role in the catatonia resolution, especially when associated with parallel specific organic base cause treatment. The use of neuroleptics should be avoided for the duration of the catatonic syndrome as it may cause clinical deterioration.

  20. Systemic lupus erythematosus and granulomatous lymphadenopathy.

    Science.gov (United States)

    Shrestha, Devendra; Dhakal, Ajaya Kumar; Shiva, Raj K C; Shakya, Arati; Shah, Subhash Chandra; Shakya, Henish

    2013-11-05

    Systemic lupus erythematosus (SLE) is known to present with a wide variety of clinical manifestations. Lymphadenopathy is frequently observed in children with SLE and may occasionally be the presenting feature. SLE presenting with granulomatous changes in lymph node biopsy is rare. These features may also cause diagnostic confusion with other causes of granulomatous lymphadenopathy. We report 12 year-old female who presented with generalized lymphadenopathy associated with intermittent fever as well as weight loss for three years. She also had developed anasarca two years prior to presentation. On presentation, she had growth failure and delayed puberty. Lymph node biopsy revealed granulomatous features. She developed a malar rash, arthritis and positive ANA antibodies over the course of next two months and showed WHO class II lupus nephritis on renal biopsy, which confirmed the final diagnosis of SLE. She was started on oral prednisolone and hydroxychloroquine with which her clinical condition improved, and she is currently much better under regular follow up. Generalized lymphadenopathy may be the presenting feature of SLE and it may preceed the other symptoms of SLE by many years as illustrated by this patient. Granulomatous changes may rarely be seen in lupus lymphadenitis. Although uncommon, in children who present with generalized lymphadenopathy along with prolonged fever and constitutional symptoms, non-infectious causes like SLE should also be considered as a diagnostic possibility.

  1. [Systemic lupus erythematosus : Unusual cutaneous manifestations].

    Science.gov (United States)

    Stockinger, T; Richter, L; Kanzler, M; Melichart-Kotik, M; Pas, H; Derfler, K; Schmidt, E; Rappersberger, K

    2016-12-01

    Various different mucocutaneous symptoms may affect up to 80 % of systemic lupus erythematosus (SLE) patients. To investigate, various unspecific, but otherwise typical clinical symptoms of skin and mucous membranes that arise in SLE patients other than those defined as SLE criteria such as butterfly rash, chronic cutaneous lupus erythematosus, oral ulcers, and increased photosensitivity. Extensive search of peer-reviewed scientific articles was performed, medical histories of several SLE patients seen in our department were analyzed, and the rare disease courses in three SLE patients are presented. Here we present a variety of unspecific but typical mucocutaneous manifestations in SLE patients: periungual erythema, periungual telangiectasia and periungual splinter hemorrhage, papules on the dorsum of the hands, scaling erythema, sometimes associated with necrosis, especially of the ears, along with complement deficiency, and the bizarre necroses of antiphospholipid syndrome. Furthermore, we show the typical clinico-histological features of neutrophilic urticarial dermatosis, as well as those of bullous SLE and finally a severe course of bacterial sepsis with Neisseria flavescens/macacae. Here we show several unspecific but rather typical mucocutaneous symptoms in lupus patients that are indicative of SLE and thus may lead to an early diagnosis. Also, life-threatening bacterial sepsis may occur with microorganisms that are commonly considered "apathogenic", such as Neisseria flavescens/macacae, which exclusively affect immunosuppressed patients.

  2. Anti-C1q antibodies in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Orbai, A-M; Truedsson, L; Sturfelt, G

    2015-01-01

    OBJECTIVE: Anti-C1q has been associated with systemic lupus erythematosus (SLE) and lupus nephritis in previous studies. We studied anti-C1q specificity for SLE (vs rheumatic disease controls) and the association with SLE manifestations in an international multicenter study. METHODS: Information...... in combination with anti-dsDNA and low complement was the strongest serological association with renal involvement. These data support the usefulness of anti-C1q in SLE, especially in lupus nephritis....

  3. Pulmonary manifestations of systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Yang, Kee Hyuk; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Joo, Kyung Bin; Hahm, Chang Kok; Lee, Seung Ro [College of Medicine, Hanyang Univ., Seoul (Korea, Republic of)

    2004-02-01

    Pulmonary involvement is more common in systemic lupus erythematosus (SLE) than in any other connective tissue disease, and more than half of patients with SLE suffer from respiratory dysfunction during the course of their illness. Although sepsis and renal disease are the most common causes of death in SLE, lung disease is the predominant manifestation and is an indicator of overall prognosis. Respiratory disease may be due to direct involvement of the lung or as a secondary consequence of the effect of the disease on other organ systems.

  4. [Seronegative systemic lupus erythematosus (author's transl)].

    Science.gov (United States)

    Goff, P L; Youinou, P; Le Menn, G

    Seronegative systemic lupus erythematosus is defined as a SLE devoided of antinuclear factors as well as of LE cells when at least 4 out of the 14 ARA criteria are present. We describe herein two case reports and review previous literature concerning this topic. This kind of SLE is characterized by a high incidence of Raynaud's disease, photosensitivity, oral ulcerations, alopecia and perhaps less frequent kidney and central nervous system involvement. The hypotheses about the absence of ANF are discussed. The awareness of such a SLE ("devoided of ANF rather than" seronegative") is to be kept in mind from a chemical, therapeutic and pathophysiologic point of view.

  5. Occurrence of systemic lupus erythematosus in a Danish community

    DEFF Research Database (Denmark)

    Laustrup, H; Voss, A; Green, A

    2009-01-01

    Objectives: To determine the prevalence and annual incidence of definite systemic lupus erythematosus (D-SLE) and incomplete SLE (I-SLE) in a community-based lupus cohort of predominantly Nordic ancestry in an 8-year prospective study from 1995 to 2003, and also to calculate the annual transition......-years at risk [95% confidence interval (CI) 1.44-7.55]. Conclusions: Denmark is a low-incidence lupus area but lupus prevalence is increasing slowly. I-SLE is a disease variant that may eventually convert into D-SLE....

  6. Parvovirus B19 viremia in children with systemic lupus erythematosus

    African Journals Online (AJOL)

    Background: Parvovirus B19 infection may present with fever, rash, nonerosive arthritis, hepatitis, anemia, thrombocytopenia, leucopenia and positive ANA, B19 infection may be misdiagnosed as new onset systemic lupus erythematosus. At the same time, B19 infection and systemic lupus erythematosus may occur ...

  7. Unique Protein Signature of Circulating Microparticles in Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Østergaard, Ole; Nielsen, Christoffer; Iversen, Line V

    2013-01-01

    To characterize the unique qualities of proteins associated with circulating subcellular material in systemic lupus erythematosus (SLE) patients compared with healthy controls and patients with other chronic autoimmune diseases.......To characterize the unique qualities of proteins associated with circulating subcellular material in systemic lupus erythematosus (SLE) patients compared with healthy controls and patients with other chronic autoimmune diseases....

  8. Case Report: Systemic Lupus Erythematosus Presenting as Acute ...

    African Journals Online (AJOL)

    Systemic lupus erythematosus is a complex autoimmune disease with multisystem involvement with varied presentation. Autoimmune adrenal disease, on the other hand, can be associated with other autoimmune diseases. Adrenal insufficiency as a presenting feature of Systemic lupus erythematosus is a rare occurrence.

  9. [The ocular manifestations in systemic lupus erythematosus].

    Science.gov (United States)

    Gheorghiu, M

    1996-01-01

    Retinal involvement in systemic lupus erythematosus is dominated by a microangiopathy of unknown aetiology, with occlusive consequences. Although cases of severe retinal ischaemia have been recently described, from a statistic point of view, the occlusive phenomena seem to have declined over the last decades, under the influence of a better therapy and follow-up of the disease. The relatively high incidence of papilloedema and the fact that this oedema might be unilateral or asymptomatic are pointed out. The importance of ophthalmic examination in patients with SLE-like syndromes or in seronegative cases of SLE is revealed. Other ocular manifestation of the disease are reviewed.

  10. Treatment Algorithms in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Muangchan, Chayawee; van Vollenhoven, Ronald F; Bernatsky, Sasha R; Smith, C Douglas; Hudson, Marie; Inanç, Murat; Rothfield, Naomi F; Nash, Peter T; Furie, Richard A; Senécal, Jean-Luc; Chandran, Vinod; Burgos-Vargas, Ruben; Ramsey-Goldman, Rosalind; Pope, Janet E

    2015-09-01

    To establish agreement on systemic lupus erythematosus (SLE) treatment. SLE experts (n = 69) were e-mailed scenarios and indicated preferred treatments. Algorithms were constructed and agreement determined (≥50% respondents indicating ≥70% agreement). Initially, 54% (n = 37) responded suggesting treatment for scenarios; 13 experts rated agreement with scenarios. Fourteen of 16 scenarios had agreement as follows: discoid lupus: first-line therapy was topical agents and hydroxychloroquine and/or glucocorticoids then azathioprine and subsequently mycophenolate (mofetil); uncomplicated cutaneous vasculitis: initial treatment was glucocorticoids ± hydroxychloroquine ± methotrexate, followed by azathioprine or mycophenolate and then cyclophosphamide; arthritis: initial therapy was hydroxychloroquine and/or glucocorticoids, then methotrexate and subsequently rituximab; pericarditis: first-line therapy was nonsteroidal antiinflammatory drugs, then glucocorticoids with/without hydroxychloroquine, then azathioprine, mycophenolate, or methotrexate and finally belimumab or rituximab, and/or a pericardial window; interstitial lung disease/alveolitis: induction was glucocorticoids and mycophenolate or cyclophosphamide, then rituximab or intravenous gamma globulin (IVIG), and maintenance followed with azathioprine or mycophenolate; pulmonary hypertension: glucocorticoids and mycophenolate or cyclophosphamide and an endothelin receptor antagonist were initial therapies, subsequent treatments were phosphodiesterase-5 inhibitors and then prostanoids and rituximab; antiphospholipid antibody syndrome: standard anticoagulation with/without hydroxychloroquine, then a thrombin inhibitor for venous thrombosis, versus adding aspirin or platelet inhibition drugs for arterial events; mononeuritis multiplex and central nervous system vasculitis: first-line therapy was glucocorticoids and cyclophosphamide followed by maintenance with azathioprine or mycophenolate, and

  11. Hypogammaglobulinemia in pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Lim, E; Tao, Y; White, A J; French, A R; Cooper, M A

    2013-11-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease typically associated with elevated serum immunoglobulin G (IgG). Hypogammaglobulinemia in SLE patients has been attributed to immunosuppressive treatment or a transient effect associated with nephrotic syndrome. We retrospectively reviewed pediatric SLE patients from a single institution to identify patients with hypogammaglobulinemia and risk factors for hypogammaglobulinemia. A total of 116 pediatric SLE cases from 1997 to 2011 were reviewed and patients with hypogammaglobulinemia (IgG lupus nephritis at SLE diagnosis, disease activity at diagnosis, initial IgG level, and drug treatment. Eighty-six patients were included in our study, with a median age of 15 years and a median follow-up of 39.5 months. Seven percent (six of 86) of patients had hypogammaglobulinemia with a median onset of 27 months (0-72 months) after SLE diagnosis. Significant associations were noted for white race (p value 0.029), male sex (p value 0.009), and the presence of lupus nephritis at SLE diagnosis (p value 0.004). Use of immunosuppressive treatment did not show a statistical association with hypogammaglobulinemia, although two of the patients with hypogammaglobulinemia did receive rituximab. Most patients with hypogammaglobulinemia received intravenous immunoglobulin (IVIG) replacement therapy because of infections and/or concern for infection. Measurement of immunoglobulin levels during treatment in SLE could help identify patients with hypogammaglobulinemia who might require more aggressive follow-up to monitor for increased risk of infection and need for IVIG treatment. A prospective study is needed to validate associated risk factors identified in this study.

  12. Cardiovascular events prior to or early after diagnosis of systemic lupus erythematosus in the systemic lupus international collaborating clinics cohort

    DEFF Research Database (Denmark)

    Urowitz, M B; Gladman, D D; Anderson, N M

    2016-01-01

    OBJECTIVE: To describe the frequency of myocardial infarction (MI) prior to the diagnosis of systemic lupus erythematosus (SLE) and within the first 2 years of follow-up. METHODS: The systemic lupus international collaborating clinics (SLICC) atherosclerosis inception cohort enters patients withi...

  13. Interferon Alpha in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Timothy B. Niewold

    2010-01-01

    Full Text Available The pleiotropic cytokine interferon alpha is involved in multiple aspects of lupus etiology and pathogenesis. Interferon alpha is important under normal circumstances for antiviral responses and immune activation. However, heightened levels of serum interferon alpha and expression of interferon response genes are common in lupus patients. Lupus-associated autoantibodies can drive the production of interferon alpha and heightened levels of interferon interfere with immune regulation. Several genes in the pathways leading to interferon production or signaling are associated with risk for lupus. Clinical and cellular manifestations of excess interferon alpha in lupus combined with the genetic risk factors associated with interferon make this cytokine a rare bridge between genetic risk and phenotypic effects. Interferon alpha influences the clinical picture of lupus and may represent a therapeutic target. This paper provides an overview of the cellular, genetic, and clinical aspects of interferon alpha in lupus.

  14. Systemic lupus erythematosus: a review for dentists.

    Science.gov (United States)

    Albilia, Jonathan B; Lam, David K; Clokie, Cameron M L; Sándor, George K B

    2007-11-01

    Systemic lupus erythematosus (SLE) is a chronic disease with far-reaching systemic implications. The hallmark feature in SLE is chronic inflammation. It can affect the skin, joints, kidneys, lungs, nervous system, serous membranes such as the pleura and pericardium, mucous membranes and other organs of the body. It is imperative that the dental practitioner be familiar with the broad range of systemic and oral implications, including the clinical and biochemical features of SLE. This review article offers an overview of the multiple organ systems affected by this complex heterogeneous disease process that are most relevant to both the general practitioner and the dental specialist. In particular, ways to recognize and manage the oral and dental manifestations of this systemic illness are presented.

  15. Association between leptin and systemic lupus erythematosus.

    Science.gov (United States)

    Xu, Wang-Dong; Zhang, Min; Zhang, Yu-Jing; Liu, Shan-Shan; Pan, Hai-Feng; Ye, Dong-Qing

    2014-04-01

    Leptin, the product of ob gene, is a 16-kDa nonglycosylated peptide hormone produced by adipocytes that regulates appetite and energy expenditure at the hypothalamic level. As is known to be a satiety factor that can regulate body weight by inhibiting food intake and stimulating energy expenditure, leptin is a pleiotropic hormone whose multiple effects include regulation of endocrine function, reproduction and immunity. Since leptin has been considered to be a pro-inflammatory cytokine, investigations of leptin in the pathogenesis of autoimmune diseases have been detected, such as systemic lupus erythematosus, rheumatoid arthritis and osteoarthritis. Recently, the role of leptin in the modulation of immune response and inflammation has been discussed in the autoimmune diseases increasingly but less in systemic lupus erythematosus (SLE). Therefore, this article will focus on the current understanding of the role of leptin with such similar pathogenic mechanism in SLE in order to provide insights which may assist in the development of leptin-based approaches for the treatment of SLE.

  16. Plasma C4d as marker for lupus nephritis in systemic lupus erythematosus.

    Science.gov (United States)

    Martin, Myriam; Smoląg, Karolina I; Björk, Albin; Gullstrand, Birgitta; Okrój, Marcin; Leffler, Jonatan; Jönsen, Andreas; Bengtsson, Anders A; Blom, Anna M

    2017-12-06

    In the present study, we sought to evaluate the complement activation product C4d as a marker for lupus nephritis in systemic lupus erythematosus (SLE). C4d levels were determined by enzyme-linked immunosorbent assay in plasma samples of patients with established SLE using a novel approach based on detection of a short linear cleavage neoepitope. Cross-sectional associations were studied in 98 patients with SLE with samples taken at lower or higher respective disease activity. Temporal associations were investigated in 69 patients with SLE who were followed longitudinally for up to 5 years. Plasma samples from 77 healthy donors were included as controls. C4d levels were negligible in healthy control subjects and significantly increased in patients with SLE in the cross-sectional study (p Lupus Erythematosus Disease Activity Index (p = 0.011) and predominantly with lupus nephritis (p = 0.003), exhibiting a sensitivity of 79% to identify patients with nephritis. High C4d levels together with the presence of anti-dsDNA autoantibodies preceded and thus predicted future lupus nephritis in the longitudinal study (OR 5.4, 95% CI 1.4-21.3). When we considered only patients with renal involvement (19 of 69) during the longitudinal study, we found that high C4d levels alone could forecast recurrence of future lupus nephritis (OR 3.3, 95% CI 1.2-9.6). C4d appears to be a valuable marker for use in monitoring of patients with SLE, particularly for lupus nephritis. Importantly, C4d levels can predict impending flares of lupus nephritis and may thus be useful for informing treatment.

  17. Illness perceptions in patients with systemic lupus erythematosus and proliferative lupus nephritis.

    Science.gov (United States)

    Daleboudt, G M N; Broadbent, E; Berger, S P; Kaptein, A A

    2011-03-01

    This study investigated the illness perceptions of patients with systemic lupus erythematosus (SLE) and whether perceptions are influenced by type of treatment for proliferative lupus nephritis. In addition, the illness perceptions of SLE patients were compared with those of patients with other chronic illnesses. Thirty-two patients who had experienced at least one episode of proliferative lupus nephritis were included. Patients were treated with either a high or low-dose cyclophosphamide (CYC) regimen (National Institutes of Health [NIH] vs. Euro-Lupus protocol). Illness perceptions were measured with the Brief Illness Perception Questionnaire (B-IPQ) and a drawing assignment. The low-dose CYC group perceived their treatment as more helpful than the high-dose CYC group. In comparison with patients with asthma, SLE patients showed more negative illness perceptions on five of the eight illness perception domains. Drawings of the kidney provided additional information about perceptions of treatment effectiveness, kidney function and patients' understanding of their illness. Drawing characteristics showed associations with perceptions of consequences, identity, concern and personal control. These findings suggest that the type of treatment SLE patients with proliferative lupus nephritis receive may influence perceptions of treatment effectiveness. In addition, patients' drawings reveal perceptions of damage caused by lupus nephritis to the kidneys and the extent of relief provided by treatment. The finding that SLE is experienced as a more severe illness than other chronic illnesses supports the need to more frequently assess and aim to improve psychological functioning in SLE patients.

  18. Dyslipidemia in systemic lupus erythematosus: just another comorbidity?

    Science.gov (United States)

    Tselios, Konstantinos; Koumaras, Charalambos; Gladman, Dafna D; Urowitz, Murray B

    2016-04-01

    Among traditional atherosclerotic risk factors, dyslipidemia is believed to decisively affect the long-term prognosis of lupus patients, not only with regard to cardiovascular events but also by influencing other manifestations, such as lupus nephritis. The aim of this study was to review the epidemiology, pathogenesis, evidence for its impact on atherosclerosis manifestations and management of dyslipidemia in lupus patients. English-restricted MEDLINE database search (Medical Subject Headings: lupus or systemic lupus erythematosus and dyslipidemia or hyperlipidemia). The prevalence of dyslipidemia in systemic lupus erythematosus (SLE) ranges from 36% at diagnosis to 60% or even higher after 3 years, depending on definition. Multiple pathogenetic mechanisms are implicated, including antibodies against lipoprotein lipase and cytokines affecting the balance between pro- and anti-atherogenic lipoproteins. Dyslipidemia has a clear impact on clinical cardiovascular disease and surrogate markers for subclinical atherosclerosis. Moreover, it negatively affects end-organ damage (kidneys and brain). Treatment with statins yielded contradictory results as per minimizing cardiovascular risk. Dyslipidemia is a significant comorbidity of lupus patients with multiple negative effects in the long term. Its treatment represents a modifiable risk factor; prompt and adequate treatment can minimize unnecessary burden in lupus patients, thus reducing hospitalizations and their overall morbidity and mortality. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. [NEUROPSYCHIATRIC MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS].

    Science.gov (United States)

    Stryjer, Rafael; Shriki Tal, Liron; Gizunterman, Alex; Amital, Daniela; Amital, Howard; Kotler, Moshe

    2017-12-01

    This review deals with the neuropsychiatric disorders resulting from systemic lupus erythematosus (SLE). SLE is a chronic autoimmune disease that impacts all systems in the human body, including the central nervous system. Neuropsychiatric symptoms in SLE are a common complication of the disease. This complication has significant implications for the severity of the illness. In most cases no thorough psychiatric assessment is performed during initial evaluation of the disease and no protocol or clear guidelines for treating the psychiatric symptoms in SLE are available. Early diagnosis of the psychiatric symptoms in SLE is critical since absence of treatment may result in severe psychiatric complications. Clinical pharmacological studies are needed in order to develop guidelines for treating psychiatric symptoms in SLE.

  20. The Existential Experience of everyday life with Systemic Lupus Erythematosus.

    Science.gov (United States)

    Larsen, Janni Lisander; Hall, Elisabeth O C; Jacobsen, Søren; Birkelund, Regner

    2018-01-19

    To explore, from the perspective of women the nature of basic existential conditions while living with systemic lupus erythematosus. Systemic lupus erythematosus has an unpredictable disease course and is documented to cause an existential rearrangement of life. The significance of changes in existential conditions and related experiences are unclear in the context of nursing and women with systemic lupus erythematosus. A qualitative design guided by Van Manen's hermeneutic phenomenological methodology. Individual in-depth interviews with 15 women diagnosed with systemic lupus erythematosus and of various ages, disease durations and severities were undertaken from September 2013 - October 2015. Data were analysed following van Manen's phenomenological approach and using drawing as an interpretive tool. The main existential experience was interpreted as a person "moving with the waves of systemic lupus erythematosus" constituted by the themes "oscillating between presence and absence of systemic lupus erythematosus", "recognizing space and bodily possibilities and limitations" and "being enriched through relationships and activities". When systemic lupus erythematosus was flaring, well-being was threatened and a laborious time to escape the feeling of a setback-in-life persisted long after the disease was medically under control. Daily life with systemic lupus erythematosus is conditioned by a prominent need to be in existential motion, related to the absence and presence of systemic lupus erythematosus. The experience of a setback-in-life by illness might challenge well-being and indicates that periods of disease flares or disturbing symptoms are critical time points to provide support. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  1. Erythematodes chronicus profundus as dermatology, surgery and cosmetology problem

    Directory of Open Access Journals (Sweden)

    Alendar Faruk

    2011-07-01

    Full Text Available Lupus profundus is a rare skin condition originally described by Kaposi in 1883. It is a clinical variant of LE in which the deep dermis and subcutaneous fat are predominantly affected. A 38-year-old female presented with history of development of well-circumscribed patches of hair loss since 1994 year. In 2004 on the skin of left cheek she noticed a single erythematous plaque on which place later became dint of subcutaneous tissue. Today she has massive defects across whole scalp, right cheek and the upper part of left arm with multiple ulcers(fig.3and4. case reposrt, laboratory examinations, skin biopsy and influence of corticosteroids on further progression /regression of this disease.

  2. Protein losing enteropathy in systemic lupus erythematosus.

    Science.gov (United States)

    Murali, A; Narasimhan, Denesh; Krishnaveni, J; Rajendiran, G

    2013-10-01

    Systemic lupus erythematosus (SLE) is a chronic immunologic disorder that may affect multiple organ systems and present with myriad of clinical features. Gastro-intestinal (GI) manifestations are oral ulcers, dysphagia and abdominal pain caused by autoimmune peritonitis/intestinal vasculitis. Hypoalbuminaemia due to GI loss is uncommon. Protein losing enteropathy (PLE) is a group of clinical entities where there is loss of protein through GI tract. PLE due to SLE is rare but it can be the initial manifestation. Patients usually present with pedal oedema mimicking nephrotic syndrome clinically. It is diagnosed by excluding other causes of hypoalbuminaemia. Radio nucleotide labelled albumin scan is useful in confirming albumin loss through GI tract. Often there is a good response to corticosteroids and immunosuppressive drugs. Here we present two SLE patients whose presenting manifestation was protein losing enteropathy and both improved with corticosteroids.

  3. Transverse myelitis in antiphospholipid antibody negative systemic lupus erythematosus.

    Science.gov (United States)

    Gude, Dilip Chandra; Chennamsetty, Sashidhar; Bansal, Dharam Pal; Jha, Ratan

    2013-06-01

    Acute transverse myelitis is a well known neurological complication occurring in systemic lupus erythematosus. Many prior studies have shown a link between transverse myelitis and the presence of antiphospholipid antibodies. Earlier theories have linked thrombotic tendency to be the culprit in such manifestations but currently there is evidence to support other causative mechanisms. A case of a young female diagnosed as systemic lupus erythematosus has been reported who presented with acute transverse myelitis and was found to be seronegative for antiphospholipid antibody. It is important to pay heed to and accordingly treat complications like acute transverse myelitis that occur regardless of antiphospholipid antibody positivity in a systemic lupus erythematosus setting.

  4. [Systemic lupus erythematosus presenting as Stevens-Johnson syndrome].

    Science.gov (United States)

    Bellakhal, S; Ben Kaab, B; Teyeb, Z; Souissi, A; Derbel, F; Douggui, M-H

    2015-09-01

    Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening dermatological conditions. Their most common cause is medication. However, in a small proportion of patients these dermatological conditions could be the first presentation of systemic lupus erythematosus. We now describe a 34-year-old patient who presented with manifestations of Stevens-Johnson as a first feature of systemic lupus erythematosus. Systemic lupus erythematosus reveled by Stevens-Johnson syndrome has been infrequently reviewed in the previous literature. This diagnosis should be considered when cutaneous adverse drug reactions occur without clear drug causality. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  5. 75 FR 35493 - Guidance for Industry on Systemic Lupus Erythematosus-Developing Medical Products for Treatment...

    Science.gov (United States)

    2010-06-22

    ... announcing the availability of the guidance entitled ``Lupus Nephritis Caused by Systemic Lupus Erythematosus... finalizing the guidance. The recommendations regarding medical product development for lupus nephritis were... Systemic Lupus Erythematosus--Developing Medical Products for Treatment; Availability AGENCY: Food and Drug...

  6. Dengue fever evolving into systemic lupus erythematosus and lupus nephritis: a case report.

    Science.gov (United States)

    Rajadhyaksha, A; Mehra, S

    2012-08-01

    Dengue viremia may be the trigger for immune complex formation in patients who are predisposed to developing autoimmune disease. We report a rare case of dengue virus infection evolving into systemic lupus erythematosus (SLE) and lupus nephritis. To the best of our knowledge this is the first case of dengue fever evolving into lupus nephritis. A 22 year old female presented with having had high grade fever, skin rash, breathlessness, retro-orbital pain, abdominal pain, arthralgias and myalgias for 10 days. She tested positive for dengue immunoglobulin M (IgM). She was given supportive treatment and was subsequently discharged. Four weeks later she developed recurrent fever, arthralgia, rash and anasarca. She was suspected as having SLE with active lupus nephritis. Antinuclear antibody (ANA), and anti double stranded deoxyribonucleic acid (anti dsDNA) titers were positive and complements were low. Renal biopsy showed diffuse proliferative glomerulonephritis grade IV. She was treated with steroids and immunosuppressants to which she responded. Dengue viremia incites antibody production, which if excessive causes deposition of viral antigen-antibody immune complexes. This could possibly lead to renal tubular damage and glomerulonephritis in susceptible individuals. Dengue fever leading to development of glomerulonephritis is rarely seen. Our patient developed dengue fever and after a month presented with manifestations of SLE and lupus nephritis. Both dengue fever and SLE have common manifestations of fever, arthralgia, rash, leucopenia with thrombocytopenia and serositis. Bacterial and viral infections may act as a 'trigger' for starting or relapsing lupus activity in genetically predetermined individuals. In our case it may be possible that dengue virus could have triggered a dysfunctional immune response, resulting in the developing of autoimmunity and SLE with lupus nephritis.

  7. Biological Therapy in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Mariana Postal

    2012-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a prototypic inflammatory autoimmune disorder characterized by multisystem involvement and fluctuating disease activity. Symptoms range from rather mild manifestations such as rash or arthritis to life-threatening end-organ manifestations. Despite new and improved therapy having positively impacted the prognosis of SLE, a subgroup of patients do not respond to conventional therapy. Moreover, the risk of fatal outcomes and the damaging side effects of immunosuppressive therapies in SLE call for an improvement in the current therapeutic management. New therapeutic approaches are focused on B-cell targets, T-cell downregulation and costimulatory blockade, cytokine inhibition, and the modulation of complement. Several biological agents have been developed, but this encouraging news is associated with several disappointments in trials and provide a timely moment to reflect on biologic therapy in SLE.

  8. Circular RNAs and systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Li, Lian-Ju; Huang, Qing; Pan, Hai-Feng; Ye, Dong-Qing, E-mail: ydqahmu@gmail.com

    2016-08-15

    Circular RNAs (circRNAs) are a large class of noncoding RNAs that form covalently closed RNA circles. The discovery of circRNAs discloses a new layer of gene regulation occurred post-transcriptionally. Identification of endogenous circRNAs benefits from the advance in high-throughput RNA sequencing and remains challenging. Many studies probing into the mechanisms of circRNAs formation occurred cotranscriptionally or posttranscriptionally emerge and conclude that canonical splicing mechanism, sequence properties, and certain regulatory factors are at play in the process. Although our knowledge on functions of circRNAs is rather limited, a few circRNAs are shown to sponge miRNA and regulate gene transcription. The clearest case is one circRNA CDR1as that serves as sponge of miR-7. Researches on circRNAs in human diseases such as cancers highlight the function and physical relevance of circRNAs. Given the implication of miRNAs in the initiation and progression of systemic lupus erythematosus (SLE) and the roles of circRNAs in sponging miRNA and gene regulation, it is appealing to speculate that circRNAs may associate with SLE and may be potential therapeutic targets for treatment of SLE. Future studies should attach more importance to the relationship between circRNAs and SLE. This review will concern identification, biogenesis, and function of circRNAs, introduce reports exploring the association of circRNAs with human diseases, and conjecture the potential roles of circRNAs in SLE. - Highlights: • Studies have discovered thousands of circRNAs and interpreted their biogenesis. • Cytoplasmic circRNAs sponge miRNA and nuclear circRNAs modulate gene transcription. • Aberrant expression of circRNAs has been observed in various cancers. • CircRNAs may partake in the pathogenesis of systemic lupus erythematosus.

  9. [Antiphospholipid antibodies in systemic lupus erythematosus: incidence, significance and relation to lupus nephritis].

    Science.gov (United States)

    Zea Mendoza, A; Rodríguez García, A; Irigoyen Oyarzábal, M V; Vázquez Díaz, M; Pardo Vigo, A; Mampaso, F M; Ortuño Mirete, J

    1989-05-20

    The prevalence of three different types of antiphospholipid antibody in 88 consecutive patients with systemic lupus were 27.2% for lupus anticoagulant (LAC), 31.8% for anticardiolipin antibody (aCL), and 13.6% for falsely positive serologic tests for syphilis (FPSTS). The three tests were correlated, thus confirming the overlapping specificities of this family of antibodies. Although FPSTS was not associated with any particular manifestation of systemic lupus, aCL correlated with thrombosis (p = 0.0001), thrombopenia (p = 0.009), neuropsychiatric features (p = 0.02) and membranous nephropathy (p = 0.001), while LAC correlated with thrombosis (p = 0.001) and hemolytic anemia (p = 0.04). The previously unreported association between membranous nephropathy and aCL might explain some features of the former, particularly the higher incidence of thromboembolic complications and the poorly known relation with renal vein thrombosis.

  10. Oral manifestation of systemic lupus erythematosus: lupus nephritis--report of a case.

    Science.gov (United States)

    Sverzut, Alexander Tadeu; Allais, Marvis; de Maurette, Marvis Allais; Mazzonetto, Renato; de Moraes, Marcio; Passeri, Luis Augusto; Moreira, Roger William Fernandes

    2008-01-01

    Systemic lupus erythematosus (SLE) is a disease of unknown origin that can affect organs and cause severe damage. Lupus is diagnosed through biopsies and laboratory examinations; however, certain clinical characteristics and the presence of lesions can help with early diagnosis and improve the disease prognosis. SLE patients generally receive immunosuppressants that may cause systemic implications---such as suture dehiscence, increased risk of infection, and delayed healing--that deserve specific attention during dental treatment. This article presents a case of a SLE patient with oral manifestations: ulcerative lesions in the mouth and development of lupus nephritis. This article seeks to emphasize the importance of recognizing the lesions related to SLE, which may help the dentist to establish an early diagnosis.

  11. Lupus Anticoagulant-hypoprothrombinemia Syndrome (LAC-HPS) in Children With Systemic Lupus Erythematosus: Report of 3 Cases.

    Science.gov (United States)

    Komvilaisak, Patcharee; Wisanuyotin, Suwannee; Jettrisuparb, Arunee; Wiangnon, Surapon

    2017-11-01

    Lupus anticoagulant, also known as lupus antibody, is generally associated with thrombosis rather than bleeding events. Lupus anticoagulant-hypoprothrombinemia syndrome in children is rather rare but can lead to mild to life-threatening bleeding. Here, we report 3 cases of lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus. They initially presented with mucocutaneous bleedings, and subsequently developed other symptoms fulfilling the laboratory criteria for systemic lupus erythematosus. Case 2 and 3 had significant epistaxis and intracerebral hemorrhage responded to systemic corticosteroid along with fresh frozen plasma. Three cases demonstrated acquired hypoprothrombinemia with no correction of mixing studies. Case 1 had low factor X level, which has never been reported previously. In all 3 cases, their coagulogram returned to normal level after corticosteroid treatment.

  12. Distinct proteome pathology of circulating microparticles in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Østergaard, Ole; Nielsen, Christoffer Tandrup; Tanassi, Julia T

    2017-01-01

    BACKGROUND: The pathogenesis of systemic lupus erythematosus (SLE) is poorly understood but has been linked to defective clearance of subcellular particulate material from the circulation. This study investigates the origin, formation, and specificity of circulating microparticles (MPs) in patients...

  13. Seizure disorders in Systemic Lupus Erythematosus

    Science.gov (United States)

    Hanly, John G.; Urowitz, Murray B.; Su, Li; Gordon, Caroline; Bae, Sang-Cheol; Sanchez-Guerrero, Jorge; Romero-Diaz, Juanita; Wallace, Daniel J; Clarke, Ann E.; Ginzler, E.M.; Merrill, Joan T.; Isenberg, David A.; Rahman, Anisur; Petri, M.; Fortin, Paul R.; Gladman, D. D.; Bruce, Ian N.; Steinsson, Kristjan; Dooley, M.A.; Khamashta, Munther A.; Alarcón, Graciela S.; Fessler, Barri J.; Ramsey-Goldman, Rosalind; Manzi, Susan; Zoma, Asad A.; Sturfelt, Gunnar K.; Nived, Ola; Aranow, Cynthia; Mackay, Meggan; Ramos-Casals, Manuel; van Vollenhoven, R.F.; Kalunian, Kenneth C.; Ruiz-Irastorza, Guillermo; Lim, Sam; Kamen, Diane L.; Peschken, Christine A.; Inanc, Murat; Theriault, Chris; Thompson, Kara; Farewell, Vernon

    2015-01-01

    Objective To describe the frequency, attribution, outcome and predictors of seizures in SLE Methods The Systemic Lupus International Collaborating Clinics (SLICC) performed a prospective inception cohort study. Demographic variables, global SLE disease activity (SLEDAI-2K), cumulative organ damage (SLICC/ACR Damage Index (SDI)) and neuropsychiatric events were recorded at enrollment and annually. Lupus anticoagulant, anticardiolipin, anti-β2 glycoprotein-I, anti-ribosomal P and anti-NR2 glutamate receptor antibodies were measured at enrollment. Physician outcomes of seizures were recorded. Patient outcomes were derived from the SF-36 mental (MCS) and physical (PCS) component summary scores. Statistical analyses included Cox and linear regressions. Results The cohort was 89.4% female with a mean follow up of 3.5±2.9 years. 75/1631 (4.6%) had ≥1 seizure, the majority around the time of SLE diagnosis. Multivariate analysis indicated a higher risk of seizures with African race/ethnicity (HR(CI):1.97 (1.07–3.63); p=0.03) and lower education status (1.97 (1.21–3.19); pattributed to SLE frequently resolved (59/78(76%)) in the absence of anti-seizure drugs. There was no significant impact on the MCS or PCS scores. Anti-malarial drugs in absence of immunosuppressive agents were associated with reduced seizure risk (0.07(0.01–0.66); p=0.03). Conclusion Seizures occurred close to SLE diagnosis, in patients with African race/ethnicity, lower educational status and cumulative organ damage. Most seizures resolved without a negative impact on health-related quality of life. Anti-malarial drugs were associated with a protective effect. PMID:22492779

  14. The Physiotherapy in Patients with Systemic Lupus Erythematosus

    OpenAIRE

    Hladíková, Alena

    2016-01-01

    Name: Alena Hladíková Supervisor: Mgr. Renáta Muchová Opponent: Title of bachelor thesis: The Physiotherapy in Patients with Systemic Lupus Erythematosus Abstract: This bachelor thesis concerns physiotherapy in patients with systemic lupus erythematosus diagnose. It mainly focuses on physiotherapeutic techniques indicated in this kind of patients. Another task is evaluating the effect of physiotherapy in acute issues caused by this disease. The thesis is divided into theoretical and practical...

  15. Juvenile systemic lupus erythematosus onset patterns in Vietnamese children

    DEFF Research Database (Denmark)

    Dung, Nguyen Thi Ngoc; Loan, Huynh Thoai; Nielsen, Susan

    2013-01-01

    to have systemic lupus erythematosus (f/m = 4/1) were referred to the Ho Chi Minh City Children's Hospital No.1 during a 12-month period in 2009. RESULTS: The mean age at diagnosis was 12.8 years (SD = 2.5). Thirty-seven (82%) fulfilled criteria for lupus nephritis (LN). At diagnosis, impressively high...... No. 1 during a16 month period from 2008-2009. These patients had a strikingly high prevalence of Coombs positive anaemia, a high prevalence of lupus nephritis, and very high SLEDAI and ECLAM scores at the time of diagnosis. While there may be referral biases, our Vietnamese SLE patients appear...

  16. Lupus and Kidney Disease (Lupus Nephritis)

    Science.gov (United States)

    ... to Z Health Guide Lupus and Kidney Disease (Lupus Nephritis) Print Email What is lupus nephritis? There are two types of lupus. Systemic lupus ... like they would attack a disease. What causes lupus nephritis? No one knows what causes the disease. Your ...

  17. Sjögren's syndrome associated with systemic lupus erythematosus.

    Science.gov (United States)

    Taşdemir, Mehmet; Hasan, Chiar; Ağbaş, Ayşe; Kasapçopur, Özgür; Canpolat, Nur; Sever, Lale; Çalışkan, Salim

    2016-09-01

    Systemic lupus erythematosus and Sjögren's syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren's syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed up with a diagnosis of Sjögren's syndrome for six years and then was observed to have overlap of systemic lupus erythematosus. In the baseline, she did not have any clinical or serological evidence for systemic lupus erythematosus. After six year, massive proteinuria and serological findings developed and systemic lupus erythematosus nephritis was diagnosed by kidney biopsy. Currently, systemic lupus erythematosus and Sjögren's syndrome cannot be differentiated definetely. We need more valuable diagnostic and classification criteria to differentiate these two important conditions.

  18. Paroxysmal nocturnal hemoglobinuria in systemic lupus erythematosus: a case report

    Directory of Open Access Journals (Sweden)

    Nakamura Norio

    2011-11-01

    Full Text Available Abstract Introduction Paroxysmal nocturnal hemoglobinuria is an acquired disorder of hemopoiesis and is characterized by recurrent episodes of intravascular hemolysis due to an increased sensitivity to complement-mediated hemolysis. Systemic lupus erythematosus with paroxysmal nocturnal hemoglobinuria is very rare. We report a case of paroxysmal nocturnal hemoglobinuria that developed in a patient with systemic lupus erythematosus and lupus nephritis. Case presentation A 29-year-old Mongolian woman had systemic lupus erythematosus, which manifested only as skin lesions when she was 12 years old. She had leg edema and proteinuria when she was 23 years old, and a renal biopsy revealed lupus nephritis (World Health Organization type IV. She had been treated with steroids and immunosuppressant therapy. At 29, she had headaches, nausea, general fatigue, and severe pancytopenia and was admitted to our hospital. A laboratory evaluation showed hemolytic anemia. Further examination showed a neutrophil alkaline phosphatase score of 46 points, a CD55 value of 18%, and a CD59 value of 78.6%. The results of Ham test and sugar water tests were positive. The constellation of symptoms throughout the clinical course and the laboratory findings suggested paroxysmal nocturnal hemoglobinuria. Conclusions To the best of our knowledge, systemic lupus erythematosus with paroxysmal nocturnal hemoglobinuria is very rare. Clinicians should be aware of the association between autoimmune and hematological diseases.

  19. Intestinal dysbiosis associated with systemic lupus erythematosus.

    Science.gov (United States)

    Hevia, Arancha; Milani, Christian; López, Patricia; Cuervo, Adriana; Arboleya, Silvia; Duranti, Sabrina; Turroni, Francesca; González, Sonia; Suárez, Ana; Gueimonde, Miguel; Ventura, Marco; Sánchez, Borja; Margolles, Abelardo

    2014-09-30

    Systemic lupus erythematosus (SLE) is the prototypical systemic autoimmune disease in humans and is characterized by the presence of hyperactive immune cells and aberrant antibody responses to nuclear and cytoplasmic antigens, including characteristic anti-double-stranded DNA antibodies. We performed a cross-sectional study in order to determine if an SLE-associated gut dysbiosis exists in patients without active disease. A group of 20 SLE patients in remission, for which there was strict inclusion and exclusion criteria, was recruited, and we used an optimized Ion Torrent 16S rRNA gene-based analysis protocol to decipher the fecal microbial profiles of these patients and compare them with those of 20 age- and sex-matched healthy control subjects. We found diversity to be comparable based on Shannon's index. However, we saw a significantly lower Firmicutes/Bacteroidetes ratio in SLE individuals (median ratio, 1.97) than in healthy subjects (median ratio, 4.86; P dysbiosis is reflected, based on in silico functional inference, in an overrepresentation of oxidative phosphorylation and glycan utilization pathways in SLE patient microbiota. Growing evidence suggests that the gut microbiota might impact symptoms and progression of some autoimmune diseases. However, how and why this microbial community influences SLE remains to be elucidated. This is the first report describing an SLE-associated intestinal dysbiosis, and it contributes to the understanding of the interplay between the intestinal microbiota and the host in autoimmune disorders. Copyright © 2014 Hevia et al.

  20. Antiphospholipid antibodies in pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Seaman, D E; Londino, A V; Kwoh, C K; Medsger, T A; Manzi, S

    1995-12-01

    Antiphospholipid antibodies (aPLs) have been extensively studied in adults with systemic lupus erythematosus (SLE) and have been associated with arterial and venous thrombosis, thrombocytopenia, neurologic disorders, and recurrent fetal loss. In contrast, very little is known about the frequency and clinical significance of aPLs in pediatric SLE. This study was designed to determine the frequency of aPLs in pediatric SLE and the temporally associated clinical manifestations. We studied 29 consecutive patients with onset of SLE in childhood seen in the Pediatric Rheumatology Clinic at the University of Pittsburgh, Children's Hospital, between 1985 and 1992. We defined aPL as the presence of a lupus anticoagulant (LAC), immunoglobulin G or immunoglobulin M anticardiolipin antibodies (aCLs), or a biologic false-positive serologic test for syphilis determined by a VDRL test. Clinical manifestations were temporally correlated to the presence of aPLs if they occurred within 6 months. Overall, 19 (65%) of 29 children with SLE had one of the three laboratory abnormalities defining aPL. LAC was detected in 16 (62%) of 26, aCL in 18 (66%) of 27, and false-positive VDRL test results in 11 (39%) of 28. Twenty-five of the 29 patients had all three tests performed. In 10 patients, all three tests were abnormal. The presence of thrombosis in 7 patients (4 venous, 2 arterial, and 1 both) was associated with a positive aPL, specifically aCL. The presence of an aPL was significantly associated with anti-double-stranded DNA antibodies, but not with neuropsychiatric manifestations or with thrombocytopenia. The presence of an aCL was significantly associated with hemolytic anemia. A prolonged prothrombin time, in the setting of an LAC (all with a prolonged activated partial thromboplastin time), was associated with life-threatening disease in 6 of 15 patients. Sixty-five percent of 29 consecutive pediatric patients with SLE had evidence of aPL. The presence of aPL, specifically a

  1. Anti-chromatin antibodies in systemic lupus erythematosus: a useful marker for lupus nephropathy

    Science.gov (United States)

    Cervera, R; Vinas, O; Ramos-Casals, M; Font, J; Garcia-Carrasco, M; Siso, A; Ramirez, F; Machuca, Y; Vives, J; Ingelmo, M; Burlingame, R

    2003-01-01

    Background: Anti-chromatin antibodies have recently been described in patients with systemic lupus erythematosus (SLE) and it has been suggested that their presence is associated with lupus nephritis. Objective: To assess the prevalence and clinical associations of these antibodies in SLE. Methods: The presence of anti-chromatin antibodies in 100 patients with SLE was investigated by an enzyme linked immunosorbent assay (ELISA). To determine the specificity of these antibodies, 100 patients with primary Sjögren's syndrome, 30 with primary antiphospholipid syndrome (APS), 10 with systemic sclerosis, and 100 normal controls were also tested. Results: Positive levels were detected in 69/100 (69%) patients with SLE. In contrast, they were found in only 8/100 (8%) of those with primary Sjögren's syndrome, in 1/10 (10%) with systemic sclerosis, in 2/30 (7%) with primary APS, and in none of the 100 healthy controls. Patients with anti-chromatin antibodies had a twofold higher prevalence of lupus nephropathy than those without these antibodies (58% v 29%, p<0.01). A significant correlation was found between the levels of anti-chromatin antibodies and disease activity score as measured by the European Consensus Lupus Activity Measurement (ECLAM; p=0.011). Conclusions: The measurement of anti-chromatin antibodies appears to be a useful addition to the laboratory tests that can help in the diagnosis and treatment of SLE. These antibodies are both sensitive and specific for SLE, and are a useful marker for an increased risk of lupus nephritis. PMID:12695155

  2. Systemic lupus erythematosus complicating simple silicosis.

    Science.gov (United States)

    Lucas, C D; Amft, N; Reid, P T

    2014-07-01

    Inhalation of crystalline silica is known to result in silicosis: an irreversible, disabling and potentially fatal occupational lung disease, which is associated with a variety of pulmonary and non-pulmonary complications including autoimmunity. A potential link between silicosis and systemic lupus erythematosus (SLE) is currently recognized only in cases of acute or accelerated silicosis. We report a case of SLE, a disease which usually affects young females, arising in a male former stonemason with simple silicosis. Epidemiological and clinical literature on the association of silica exposure and development of SLE are briefly reviewed. This case report and literature review highlight the link between occupational silica exposure and autoimmune disease including SLE, establishes that even simple silicosis appears linked to development of autoimmunity and emphasizes the importance of an occupational history, especially in male patients who develop SLE. © The Author 2014. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  3. Unmet medical needs in systemic lupus erythematosus

    Science.gov (United States)

    2012-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease of diverse manifestations, with onset usually in young women in the third to fourth decade of life. The chronic nature of this relapsing remitting disease leads to organ damage accrual over time. Mortality and morbidity are increased in patients with SLE compared with the general population. Therapeutic advances over the last few decades have led to significant improvements in patient outcomes. Five-year survival has improved to over 90% from a low of 50% in the 1950s. However, multiple aspects of the management of SLE patients are still far from optimal. Early diagnosis remains a challenge; diagnostic delays leading to delay in definitive treatment are common. Monitoring treatment remains problematic due to the paucity of sensitive biomarkers. Current treatment regimens rely heavily on corticosteroids, even though corticosteroids are well known to cause organ damage. Treatment of refractory disease manifestations such as nephritis, recalcitrant cutaneous lesions and neurological involvement require new approaches with greater efficacy. Cognitive dysfunction is common in SLE patients, but early recognition and adequate treatment are yet to be established. Premature accelerated atherosclerosis remains a leading cause of morbidity and mortality. Fatigue is one of the most disabling symptoms, and contributes to the poor quality of life in patients with SLE. Ongoing research in SLE faces many challenges, including enrollment of homogeneous patient populations, use of reliable outcome measures and a standard control arm. The current review will highlight some of the outstanding unmet challenges in the management of this complex disease. PMID:23281889

  4. Juvenile systemic lupus erythematosus in Nigeria.

    Science.gov (United States)

    Adelowo, O O; Olaosebikan, B H; Animashaun, B A; Akintayo, R O

    2017-03-01

    Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014. Out of the 12 patients studied, eight were girls and four were boys. All patients had positive antinuclear antibody and extractable nuclear antibody tests. Anti-dsDNA antibody was positive in 10 patients. Eight patients had renal disease while four patients had neuropsychiatric manifestations. Haematological abnormalities and constitutional symptoms were present in all patients. Patients were treated with pulse methylprednisolone, oral prednisolone, hydroxychloroquine and azathioprine. Three patients also received rituximab. In conclusion, JSLE exists in Nigeria and exhibits clinical and immunological characteristics similar to its pattern in other parts of the world. It is, however, diagnosed late and is possibly being underdiagnosed as there is no paediatric rheumatologist in the country.

  5. The Pathology of T Cells in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Anselm Mak

    2014-01-01

    Full Text Available Systemic lupus erythematosus (SLE is characterized by the production of a wide array of autoantibodies. Thus, the condition was traditionally classified as a “B-cell disease”. Compelling evidence has however shown that without the assistance of the helper T lymphocytes, it is indeed difficult for the “helpless” B cells to become functional enough to trigger SLE-related inflammation. T cells have been recognized to be crucial in the pathogenicity of SLE through their capabilities to communicate with and offer enormous help to B cells for driving autoantibody production. Recently, a number of phenotypic and functional alterations which increase the propensity to trigger lupus-related inflammation have been identified in lupus T cells. Here, potential mechanisms involving alterations in T-cell receptor expressions, postreceptor downstream signalling, epigenetics, and oxidative stress which favour activation of lupus T cells will be discussed. Additionally, how regulatory CD4+, CD8+, and γδ T cells tune down lupus-related inflammation will be highlighted. Lastly, while currently available outcomes of clinical trials evaluating therapeutic agents which manipulate the T cells such as calcineurin inhibitors indicate that they are at least as efficacious and safe as conventional immunosuppressants in treating lupus glomerulonephritis, larger clinical trials are undoubtedly required to validate these as-yet favourable findings.

  6. Economic evaluation of lupus nephritis in the Systemic Lupus International Collaborating Clinics inception cohort using a multistate model approach

    DEFF Research Database (Denmark)

    Barber, Megan R W; Hanly, John G; Su, Li

    2018-01-01

    OBJECTIVE: Little is known about the long-term costs of lupus nephritis (LN). These were compared between patients with and without LN based on multistate modelling. METHODS: Patients from 32 centres in 11 countries were enrolled in the Systemic Lupus International Collaborating Clinics (SLICC...

  7. Antiphospholipid antibodies and non-thrombotic manifestations of systemic lupus erythematosus.

    Science.gov (United States)

    İlgen, U; Yayla, M E; Ateş, A; Okatan, İ E; Yurteri, E U; Torgutalp, M; Keleşoğlu, A B D; Turgay, T M; Kınıklı, G

    2017-01-01

    Objectives The aim of this study was to investigate the association between antiphospholipid antibodies and non-thrombotic and non-gestational manifestations of systemic lupus erythematosus. Methods Systemic lupus erythematosus patients with persistently positive antiphospholipid antibodies or lupus anticoagulant were identified and grouped as systemic lupus erythematosus with antiphospholipid syndrome (SLE-APS), systemic lupus erythematosus with positive antiphospholipid antibodies/lupus anticoagulant without antiphospholipid syndrome (SLE-aPL), and systemic lupus erythematosus with negative aPLs (SLE-No aPL). Groups were compared in terms of non-thrombotic systemic lupus erythematosus manifestations and laboratory features retrospectively. Results A total of 150 systemic lupus erythematosus patients, 26 with SLE-APS, 25 with SLE-aPL, and 99 with SLE-No aPL, were identified. Livedo reticularis, neurologic involvement, and thrombocytopenia were more common in antiphospholipid antibody positive systemic lupus erythematosus cases. Malar rash, arthritis, and pleuritis were more common in the SLE-No aPL, SLE-APS, and SLE-aPL groups, respectively. Positivity rates and titers of specific antiphospholipid antibodies did not differ between the SLE-APS and SLE-aPL groups. Conclusions Presence of antiphospholipid syndrome or persistent antiphospholipid antibodies may be related to non-thrombotic and non-gestational systemic lupus erythematosus manifestations. Patients with systemic lupus erythematosus plus antiphospholipid syndrome and persistent antiphospholipid antibodies without antiphospholipid syndrome also differ in terms of systemic lupus erythematosus manifestations.

  8. Gut Microbiota in Human Systemic Lupus Erythematosus and a Mouse Model of Lupus.

    Science.gov (United States)

    Luo, Xin M; Edwards, Michael R; Mu, Qinghui; Yu, Yang; Vieson, Miranda D; Reilly, Christopher M; Ahmed, S Ansar; Bankole, Adegbenga A

    2018-02-15

    Gut microbiota dysbiosis has been observed in a number of autoimmune diseases. However, the role of the gut microbiota in systemic lupus erythematosus (SLE), a prototypical autoimmune disease characterized by persistent inflammation in multiple organs of the body, remains elusive. Here we report the dynamics of the gut microbiota in a murine lupus model, NZB/W F1, as well as intestinal dysbiosis in a small group of SLE patients with active disease. The composition of the gut microbiota changed markedly before and after the onset of lupus disease in NZB/W F1 mice, with greater diversity and increased representation of several bacterial species as lupus progressed from the predisease stage to the diseased stage. However, we did not control for age and the cage effect. Using dexamethasone as an intervention to treat SLE-like signs, we also found that a greater abundance of a group of lactobacilli (for which a species assignment could not be made) in the gut microbiota might be correlated with more severe disease in NZB/W F1 mice. Results of the human study suggest that, compared to control subjects without immune-mediated diseases, SLE patients with active lupus disease possessed an altered gut microbiota that differed in several particular bacterial species (within the genera Odoribacter and Blautia and an unnamed genus in the family Rikenellaceae ) and was less diverse, with increased representation of Gram-negative bacteria. The Firmicutes / Bacteroidetes ratios did not differ between the SLE microbiota and the non-SLE microbiota in our human cohort. IMPORTANCE SLE is a complex autoimmune disease with no known cure. Dysbiosis of the gut microbiota has been reported for both mice and humans with SLE. In this emerging field, however, more studies are required to delineate the roles of the gut microbiota in different lupus-prone mouse models and people with diverse manifestations of SLE. Here, we report changes in the gut microbiota in NZB/W F1 lupus-prone mice and a

  9. Voice disorder in systemic lupus erythematosus.

    Directory of Open Access Journals (Sweden)

    Milena S F C de Macedo

    Full Text Available Systemic lupus erythematosus (SLE is a chronic disease characterized by progressive tissue damage. In recent decades, novel treatments have greatly extended the life span of SLE patients. This creates a high demand for identifying the overarching symptoms associated with SLE and developing therapies that improve their life quality under chronic care. We hypothesized that SLE patients would present dysphonic symptoms. Given that voice disorders can reduce life quality, identifying a potential SLE-related dysphonia could be relevant for the appraisal and management of this disease. We measured objective vocal parameters and perceived vocal quality with the GRBAS (Grade, Roughness, Breathiness, Asthenia, Strain scale in SLE patients and compared them to matched healthy controls. SLE patients also filled a questionnaire reporting perceived vocal deficits. SLE patients had significantly lower vocal intensity and harmonics to noise ratio, as well as increased jitter and shimmer. All subjective parameters of the GRBAS scale were significantly abnormal in SLE patients. Additionally, the vast majority of SLE patients (29/36 reported at least one perceived vocal deficit, with the most prevalent deficits being vocal fatigue (19/36 and hoarseness (17/36. Self-reported voice deficits were highly correlated with altered GRBAS scores. Additionally, tissue damage scores in different organ systems correlated with dysphonic symptoms, suggesting that some features of SLE-related dysphonia are due to tissue damage. Our results show that a large fraction of SLE patients suffers from perceivable dysphonia and may benefit from voice therapy in order to improve quality of life.

  10. Myocardial perfusion abnormalities in asymptomatic patients with systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Hosenpud, J.D.; Montanaro, A.; Hart, M.V.; Haines, J.E.; Specht, H.D.; Bennett, R.M.; Kloster, F.E.

    1984-08-01

    Accelerated coronary artery disease and myocardial infarction in young patients with systemic lupus erythematosus is well documented; however, the prevalence of coronary involvement is unknown. Accordingly, 26 patients with systemic lupus were selected irrespective of previous cardiac history to undergo exercise thallium-201 cardiac scintigraphy. Segmental perfusion abnormalities were present in 10 of the 26 studies (38.5 percent). Five patients had reversible defects suggesting ischemia, four patients had persistent defects consistent with scar, and one patient had both reversible and persistent defects in two areas. There was no correlation between positive thallium results and duration of disease, amount of corticosteroid treatment, major organ system involvement or age. Only a history of pericarditis appeared to be associated with positive thallium-201 results (p less than 0.05). It is concluded that segmental myocardial perfusion abnormalities are common in patients with systemic lupus erythematosus. Whether this reflects large-vessel coronary disease or small-vessel abnormalities remains to be determined.

  11. Systemic lupus erythematosus diagnostics in the ‘omics’ era

    Science.gov (United States)

    Arriens, Cristina; Mohan, Chandra

    2014-01-01

    Systemic lupus erythematosus is a complex autoimmune disease affecting multiple organ systems. Currently, diagnosis relies upon meeting at least four out of eleven criteria outlined by the ACR. The scientific community actively pursues discovery of novel diagnostics in the hope of better identifying susceptible individuals in early stages of disease. Comprehensive studies have been conducted at multiple biological levels including: DNA (or genomics), mRNA (or transcriptomics), protein (or proteomics) and metabolites (or metabolomics). The ‘omics’ platforms allow us to re-examine systemic lupus erythematosus at a greater degree of molecular resolution. More importantly, one is hopeful that these ‘omics’ platforms may yield newer biomarkers for systemic lupus erythematosus that can help clinicians track the disease course with greater sensitivity and specificity. PMID:24860621

  12. Psoriasis in systemic lupus erythematosus: a single-center experience.

    Science.gov (United States)

    Tselios, Konstantinos; Yap, Kristy Su-Ying; Pakchotanon, Rattapol; Polachek, Ari; Su, Jiandong; Urowitz, Murray B; Gladman, Dafna D

    2017-04-01

    The coexistence of psoriasis with systemic lupus erythematosus (SLE) has been reported in limited case series, raising hypotheses about shared pathogenetic mechanisms. Nevertheless, important differences regarding treatment do exist. The aim of the present study was to determine the prevalence and characteristics of psoriasis in a defined cohort of lupus patients. Patients with psoriasis were retrieved from the University of Toronto Lupus Clinic from its inception in 1970 up to 2015. Charts were hand-searched to collect information concerning demographic, clinical, and therapeutic variables. Patients were matched with non-psoriasis lupus patients to identify the impact of supervening psoriasis on lupus activity, damage accrual, and venous thromboembolic (VTEs) and cardiovascular events (CVEs). Psoriasis was diagnosed in 63 patients (49 females, 14 males) for a prevalence of 3.46% (63/1823). The male-to-female ratio was significantly higher in non-psoriasis patients (0.286 vs. 0.138, p = 0.017). Plaque psoriasis was the most prominent type (55/63, 87.3%) whereas three patients had pustular disease; one had psoriatic arthritis. Nine patients (14.3%) were administered systemic treatment with methotrexate (n = 5), azathioprine (n = 1), ustekinumab (n = 3), and etanercept (n = 1). Psoriasis was definitely deteriorated by hydroxychloroquine in one patient. There was no significant impact of psoriasis on disease activity, damage accrual, VTEs, and CVEs. The prevalence of psoriasis was twice as high as that of the general Canadian population in this lupus cohort. Plaque psoriasis was the most prominent subtype, and topical treatment was adequate in the majority of patients. Supervening psoriasis had no significant impact on lupus activity and damage accrual.

  13. Massive intracranial calcifications in a patient with systemic lupus erythematosus; Calcificacoes intracranianas macicas em um paciente com lupus eritematoso sistemico

    Energy Technology Data Exchange (ETDEWEB)

    Gasparetto, Emerson L.; Carvalho Neto, Arnolfo de [Parana Univ., Curitiba, PR (Brazil). Dept. de Clinica Medica. Servico de Radiologia Medica]. E-mail: gasparetto@hotmail.com; Ono, Sergio E. [Parana Univ., Curitiba, PR (Brazil). Faculdade de Medicina

    2004-12-01

    Central nervous system involvement is frequently reported in patients with systemic lupus erythematosus. Computed tomography and magnetic resonance imaging studies usually show brain atrophy, cerebral infarction and/or intracranial bleeding. Extensive intracranial calcification in patients with systemic lupus erythematosus is rare. We report a case of a patient with systemic lupus erythematosus who presented with seizures and massive basal ganglia calcification and mild calcifications in the frontal lobes, seen on the brain computed tomography scan. Magnetic resonance imaging showed hyperintensity on FLAIR images and hypointense signals on T2{sup *} gradient echo images in the basal ganglia. (author)

  14. Thrombotic Thrombocytopenic Purpura and Systemic Lupus ...

    African Journals Online (AJOL)

    The diagnosis of SLE was made based on these clinical findings along with positive antinuclear and anti dsDNA antibodies. Renal biopsy revealed class IV/ V lupus nephritis (LN) with active lesions of thrombotic microangiopathy. The evolution of neurological deficit, persistent thrombocytopenia and active microangiopathic ...

  15. Immunoglobulin E and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Atta A.M.

    2004-01-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease characterized by intense polyclonal production of autoantibodies and circulating immune complexes. Some reports have associated SLE with a Th2 immune response and allergy. In the present study 21 female patients with SLE were investigated for total IgE and IgE antibodies to dust house aeroallergens by an automated enzyme-linked fluorescent assay, and were also evaluated for antinuclear IgE autoantibodies by a modified indirect immunofluorescence test using HEp-2 cells as antigen substrate. Additionally, immunocapture ELISA was used to investigate serum anti-IgE IgG autoantibodies. Serum IgE above 150 IU/ml, ranging from 152 to 609 IU/ml (median = 394 IU IgE/ml, was observed in 7 of 21 SLE patients (33%, 5 of them presenting proteinuria, urinary cellular casts and augmented production of anti-dsDNA antibodies. While only 2 of 21 SLE patients (9.5% were positive for IgE antibodies to aeroallergens, all 10 patients with respiratory allergy (100% from the atopic control group (3 males and 7 females, had these immunoglobulins. SLE patients and healthy controls presented similar anti-IgE IgG autoantibody titers (X = 0.37 ± 0.20 and 0.34 ± 0.18, respectively, differing from atopic controls (0.94 ± 0.26. Antinuclear IgE autoantibodies were detected in 17 of 21 (81% sera from SLE patients, predominating the fine speckled pattern of fluorescence, that was also observed in IgG-ANA. Concluding, SLE patients can present increased IgE levels and antinuclear IgE autoantibodies without specific clinical signs of allergy or production of antiallergen IgE antibodies, excluding a possible association between SLE and allergy.

  16. Phenotypic associations of genetic susceptibility loci in systemic lupus erythematosus.

    Science.gov (United States)

    Sanchez, Elena; Nadig, Ajay; Richardson, Bruce C; Freedman, Barry I; Kaufman, Kenneth M; Kelly, Jennifer A; Niewold, Timothy B; Kamen, Diane L; Gilkeson, Gary S; Ziegler, Julie T; Langefeld, Carl D; Alarcón, Graciela S; Edberg, Jeffrey C; Ramsey-Goldman, Rosalind; Petri, Michelle; Brown, Elizabeth E; Kimberly, Robert P; Reveille, John D; Vilá, Luis M; Merrill, Joan T; Anaya, Juan-Manuel; James, Judith A; Pons-Estel, Bernardo A; Martin, Javier; Park, So-Yeon; Bang, So-Young; Bae, Sang-Cheol; Moser, Kathy L; Vyse, Timothy J; Criswell, Lindsey A; Gaffney, Patrick M; Tsao, Betty P; Jacob, Chaim O; Harley, John B; Alarcón-Riquelme, Marta E; Sawalha, Amr H

    2011-10-01

    Systemic lupus erythematosus is a clinically heterogeneous autoimmune disease. A number of genetic loci that increase lupus susceptibility have been established. This study examines if these genetic loci also contribute to the clinical heterogeneity in lupus. 4001 European-derived, 1547 Hispanic, 1590 African-American and 1191 Asian lupus patients were genotyped for 16 confirmed lupus susceptibility loci. Ancestry informative markers were genotyped to calculate and adjust for admixture. The association between the risk allele in each locus was determined and compared in patients with and without the various clinical manifestations included in the ACR criteria. Renal disorder was significantly correlated with the lupus risk allele in ITGAM (p=5.0 × 10(-6), OR 1.25, 95% CI 1.12 to 1.35) and in TNFSF4 (p=0.0013, OR 1.14, 95% CI 1.07 to 1.25). Other significant findings include the association between risk alleles in FCGR2A and malar rash (p=0.0031, OR 1.11, 95% CI 1.17 to 1.33), ITGAM and discoid rash (p=0.0020, OR 1.20, 95% CI 1.06 to 1.33), STAT4 and protection from oral ulcers (p=0.0027, OR 0.89, 95% CI 0.83 to 0.96) and IL21 and haematological disorder (p=0.0027, OR 1.13, 95% CI 1.04 to 1.22). All these associations are significant with a false discovery rate of manifestations and the FCGR2A, ITGAM, STAT4, TNSF4 and IL21 genes. The findings suggest that genetic profiling might be a useful tool to predict disease manifestations in lupus patients in the future.

  17. [Systemic lupus erythematosus and antiphospholipid syndrome: How to manage pregnancy?].

    Science.gov (United States)

    Guettrot-Imbert, G; Le Guern, V; Morel, N; Vauthier, D; Tsatsaris, V; Pannier, E; Piette, J-C; Costedoat-Chalumeau, N

    2015-03-01

    Pregnancy in systemic lupus erythematosus patients is a common situation that remains associated with higher maternal and fetal mortality/morbidity than in the general population. Complications include lupus flares, obstetrical complications (fetal loss, in utero growth retardation, prematurity) and neonatal lupus syndrome. The association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetrical complications. Improving the care of these pregnancies depends upon a systematic pregnancy planning, ideally during a preconception counseling visit and a multidisciplinary approach (internist/rheumatologist, obstetrician and anesthetist). The absence of lupus activity, the use of appropriate medications during pregnancy adjusted to the patient's medical history and risk factors, and a regular monitoring are the best tools for a favorable outcome for these high-risk pregnancies. The aim of this review article is to perform an update on the medical care of pregnancy in systemic lupus erythematosus or antiphospholipid syndrome to reduce the risk of complications and to ensure the best maternal and fetal prognosis. Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  18. Pregnancies in women with systemic lupus erythematosus and antiphospholipid antibodies

    DEFF Research Database (Denmark)

    Schreiber, K

    2016-01-01

    Systemic lupus erythematosus (SLE) has preponderance in women in their childbearing years; consequently pregnancy has always been an important issue of concern for the patient and the treating physician. Based upon numerous reports on successful pregnancy outcomes in the past decades, the initial...... of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus (PROMISSE) study, so far the largest multicentre cohort study of pregnant women with underlying stable SLE, has given some important answers to long-discussed questions. Future studies on data collected from...

  19. Coincident systemic lupus erythematosus and psoriasis vulgaris: a case report.

    Science.gov (United States)

    Wang, Y; Da, G; Yu, Y; Han, J; Li, H

    2015-12-01

    Psoriasis vulgaris is an autoimmune chronic inflammatory skin disease, but its association with other typical autoimmune disease such as systemic lupus erythematosus has only occasionally been reported. We presented a 25-year-old female who developed systemic lupus erythematosus associated with psoriasis vulgaris. Her conditions were in good control after she got administration of prednisolone (5 mg/day) and Tripterygium Wilfordii Hook (20 mg/day). It is necessary to integrate past history and physical examination to diagnose coincident SLE and psoriasis, and combined treatment with prednisolone and Tripterygium Wilfordii Hook proves effective.

  20. Total lymphoid irradiation in refractory systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Ben-Chetrit, E.; Gross, D.J.; Braverman, A.; Weshler, Z.; Fuks, Z.; Slavin, S.; Eliakim, M.

    1986-07-01

    In two patients with systemic lupus erythematosus, conventional therapy was considered to have failed because of persistent disease activity and unacceptable side effects. Both were treated with total lymphoid irradiation without clinical benefit, despite adequate immunosuppression as documented by markedly reduced numbers of circulating T lymphocytes and T-lymphocyte-dependent proliferative responses in vitro. The first patient developed herpes zoster, gram-negative septicemia, neurologic symptoms, and deterioration of lupus nephritis. The second patient developed massive bronchopneumonia, necrotic cutaneous lesions, and progressive nephritis and died 2 weeks after completion of radiotherapy. These observations, although limited to two patients, indicate that total lymphoid irradiation in patients with severe systemic lupus erythematosus should be regarded as strictly experimental.

  1. Cell death in the pathogenesis of systemic lupus erythematosus and lupus nephritis.

    Science.gov (United States)

    Mistry, Pragnesh; Kaplan, Mariana J

    2017-12-01

    Nephritis is one of the most severe complications of systemic lupus erythematosus (SLE). One key characteristic of lupus nephritis (LN) is the deposition of immune complexes containing nucleic acids and/or proteins binding to nucleic acids and autoantibodies recognizing these molecules. A variety of cell death processes are implicated in the generation and externalization of modified nuclear autoantigens and in the development of LN. Among these processes, apoptosis, primary and secondary necrosis, NETosis, necroptosis, pyroptosis, and autophagy have been proposed to play roles in tissue damage and immune dysregulation. Cell death occurs in healthy individuals during conditions of homeostasis yet autoimmunity does not develop, at least in part, because of rapid clearance of dying cells. In SLE, accelerated cell death combined with a clearance deficiency may lead to the accumulation and externalization of nuclear autoantigens and to autoantibody production. In addition, specific types of cell death may modify autoantigens and alter their immunogenicity. These modified molecules may then become novel targets of the immune system and promote autoimmune responses in predisposed hosts. In this review, we examine various cell death pathways and discuss how enhanced cell death, impaired clearance, and post-translational modifications of proteins could contribute to the development of lupus nephritis. Published by Elsevier Inc.

  2. Ultraviolet-A1 irradiation therapy for systemic lupus erythematosus

    Science.gov (United States)

    2017-01-01

    Systemic lupus erythematosus (lupus, SLE) is a chronic autoimmune disease characterized by the production of autoantibodies, which bind to antigens and are deposited within tissues to fix complement, resulting in widespread systemic inflammation. The studies presented herein are consistent with hyperpolarized, adenosine triphosphate (ATP)-deficient mitochondria being central to the disease process. These hyperpolarized mitochondria resist the depolarization required for activation-induced apoptosis. The mitochondrial ATP deficits add to this resistance to apoptosis and also reduce the macrophage energy that is needed to clear apoptotic bodies. In both cases, necrosis, the alternative pathway of cell death, results. Intracellular constituents spill into the blood and tissues, eliciting inflammatory responses directed at their removal. What results is “autoimmunity.” Ultraviolet (UV)-A1 photons have the capacity to remediate this aberrancy. Exogenous exposure to low-dose, full-body, UV-A1 radiation generates singlet oxygen. Singlet oxygen has two major palliative actions in patients with lupus and the UV-A1 photons themselves have several more. Singlet oxygen depolarizes the hyperpolarized mitochondrion, triggering non-ATP-dependent apoptosis that deters necrosis. Next, singlet oxygen activates the gene encoding heme oxygenase (HO-1), a major governor of systemic homeostasis. HO-1 catalyzes the degradation of the oxidant heme into biliverdin (converted to bilirubin), Fe, and carbon monoxide (CO), the first three of these exerting powerful antioxidant effects, and in conjunction with a fourth, CO, protecting against injury to the coronary arteries, the central nervous system, and the lungs. The UV-A1 photons themselves directly attenuate disease in lupus by reducing B cell activity, preventing the suppression of cell-mediated immunity, slowing an epigenetic progression toward SLE, and ameliorating discoid and subacute cutaneous lupus. Finally, a combination of

  3. Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Wu, E Y; Schanberg, L E; Wershba, E C; Rabinovich, C E

    2017-05-01

    Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Methods We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus. Information was gathered at drug initiation and 6-month follow-up. The Wilcoxon matched-pairs signed-rank test was used to assess change in quantitative parameters of disease activity. Results Nine subjects were girls and six were African-American. Indications for lenalidomide treatment included alopecia, nasal and oral ulcers, extensive malar rash, discoid lesions, bullous lesions, panniculitis, cutaneous vasculitis, and Raynaud's phenomenon with digital ulcerations. Within 6 months, all patients demonstrated complete or near resolution based on physician report. Prednisone dose decreased from a mean 23.5 mg (SD± 13.3) to 12.25 mg (SD± 9.2) ( P= 0.008). Sedimentation rate decreased from a mean 29 mm/hour (SD± 31.5) to 17 mm/hour (SD± 18.1) ( P= 0.004). Lenalidomide was well tolerated. Conclusion Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide's role in treating cutaneous manifestations of systemic lupus erythematosus.

  4. Apoptotic cell clearance in Systemic Lupus Erythematosus (SLE)

    NARCIS (Netherlands)

    Reefman, Esther

    2006-01-01

    Systemic lupus erythematosus (SLE) is an incurable multi-organ autoimmune disease characterized by a wide array of clinical manifestations, ranging from skin and mucosal lesions to severe injuries in the central nervous system, kidneys and other organs. The disease primarily affects women in their

  5. Atypical presentation of systemic lupus erythematosus in a west ...

    African Journals Online (AJOL)

    Systemic lupus erythematosus (SLE) is a chronic, autoimmune multi-system disorder. About seventy to ninety percent of all cases of SLE occur in women. Although the disease is common in black young women residing in Europe and North America, it is reputed to be a very rare diagnosis in West Africa. A case of atypical ...

  6. Acute Stroke as the first manifestation of Systemic Lupus ...

    African Journals Online (AJOL)

    Systemic lupus erythematosus (SLE) is an autoimmune, multisystem disease that is characterized by a bewildering array of antibodies. Central nervous system manifestations of SLE are highly diverse and often have major prognostic consequences, accounting for 15% of cases. It is a known cause of 'stroke in the young' ...

  7. Systemic Lupus Erythematosus with Severe Nephritis That Mimicked ...

    African Journals Online (AJOL)

    Introduction: Systemic lupus erythematosus (SLE) belongs to a family of related autoimmune rheumatic disorders that are capable of affecting multiple organs, and they are all associated with a variety of autoantibodies. Henoch Schoenlein purpura (HSP) is a sort of systemic vasculitis that is not associated with ...

  8. Guillain barre syndrome as initial presentation of systemic lupus ...

    African Journals Online (AJOL)

    Background: Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by multiple organ involvement including the peripheral nervous system. Guillan- Barrè syndrome (GBS) has an established association with SLE as one of its neurologic manifestations. However, GBS as an initial manifestation of ...

  9. Clinical features of Systemic Lupus Erythematosus (SLE) patients ...

    African Journals Online (AJOL)

    Background: Systemic lupus erythematosus (SLE) is an autoimmune disease, a type of self-allergy, whereby the patient's immune system creates antibodies that attack the person's own body tissues instead of protecting the body from bacteria and viruses. In most cases the cause of SLE is unknown, although it is believed ...

  10. Dietary micronutrient intake and atherosclerosis in systemic lupus erythematosus.

    Science.gov (United States)

    Lourdudoss, C; Elkan, A-C; Hafström, I; Jogestrand, T; Gustafsson, T; van Vollenhoven, R; Frostegård, J

    2016-12-01

    The aim of this study was to investigate the role of dietary micronutrient intake in systemic lupus erythematosus (SLE). This study included 111 SLE patients and 118 age and gender-matched controls. Data on diet (food frequency questionnaires) were linked with data on Systemic Lupus Activity Measure, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and carotid atherosclerotic/echolucent plaque (B-mode ultrasound). Dietary micronutrient intake were compared between SLE patients and controls and in relation to lupus activity and atherosclerosis in SLE. Associations between micronutrient intake and plaque were analyzed through logistic regression, adjusted for potential confounders. Micronutrient intake did not differ between patients and controls, and between lower and higher lupus activity, apart from the fact that phosphorus was associated with SLEDAI > 6. In SLE patients, some micronutrients were associated with atherosclerotic plaque, left side. Lower intake of riboflavin and phosphorus was associated with atherosclerotic plaque, left side (odds ratio (OR) 3.06, 95% confidence interval (CI) 1.12-8.40 and OR 4.36, 95% CI 1.53-12.39, respectively). Higher intake of selenium and thiamin was inversely associated with atherosclerotic plaque, left side (OR 0.28, 95% CI 0.09-0.89 and OR 0.26, 95% CI 0.08-0.80, respectively). In addition, higher intake of thiamin was inversely associated with echolucent plaque, left side (OR 0.22, 95% CI 0.06-0.84). Lower intake of folate was inversely associated with bilateral echolucent plaque (OR 0.36, 95% CI 0.13-0.99). SLE patients did not have different dietary micronutrient intake compared to controls. Phosphorus was associated with lupus activity. Riboflavin, phosphorus, selenium and thiamin were inversely associated with atherosclerotic plaque, left side in SLE patients, but not in controls. Dietary micronutrients may play a role in atherosclerosis in SLE. © The Author(s) 2016.

  11. Association of Sweet's Syndrome and Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    J. L. Barton

    2011-01-01

    Full Text Available Sweet's syndrome is an acute febrile neutrophilic dermatosis which usually presents as an idiopathic disorder but can also be drug induced, associated with hematopoetic malignancies and myelodysplastic disorders, and more, infrequently, observed in autoimmune disorders. Sweet's syndrome has been reported in three cases of neonatal lupus, three cases of hydralazine-induced lupus in adults, and in nine pediatric and adult systemic lupus erythematosus (SLE patients. We describe three additional adult cases of Sweet's associated with SLE and provide a focused review on nondrug-induced, nonneonatal SLE and Sweet's. In two of three new cases, as in the majority of prior cases, the skin rash of Sweet's paralleled underlying SLE disease activity. The pathogenesis of Sweet's remains elusive, but evidence suggests that cytokine dysregulation may be central to the clinical and pathological changes in this condition, as well as in SLE. Further research is needed to define the exact relationship between the two conditions.

  12. Refractory Angioedema in a Patient with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Zahra Habibagahi

    2015-07-01

    Full Text Available Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule. Herein we report a new case of lupus nephritis that developed angioedema and a rapid course of disease progression with acute renal failure and alveolar hemorrhage without response to high dose steroid and plasmapheresis.

  13. Cerebral toxoplasmosis in systemic lupus erythematosus following intravenous methylprednisolone.

    Science.gov (United States)

    Pagalavan, L; Kan, F K

    2011-03-01

    Cerebral toxoplasmosis is a rare complication of systemic lupus erythematosus (SLE). An 18 year old male student, newly diagnosed to have SLE, developed neurological symptoms two days after completing intravenous methylprednisolone. Computed tomography (CT) scan showed features consistent with a diagnosis of probable cerebral toxoplasmosis. He responded dramatically to antitoxoplasma therapy. To our knowledge, this is the first case report in the literature that presents a newly diagnosed SLE patient who rapidly developed cerebral toxoplasmosis following administration of intravenous methylprednisolone. Our case illustrates that this drug is potentially fatal and the importance of differentiating cerebral infection from neuropsychiatric lupus.

  14. The serum levels of connective tissue growth factor in patients with systemic lupus erythematosus and lupus nephritis.

    Science.gov (United States)

    Wang, F-M; Yu, F; Tan, Y; Liu, G; Zhao, M-H

    2014-06-01

    The expression of connective tissue growth factor mRNA in human kidneys may serve as an early marker for lupus nephritis progression. Therefore, we speculated that connective tissue growth factor may be involved in the pathogenesis of systemic lupus erythematosus and lupus nephritis. In this study, we set out to investigate the associations between serum connective tissue growth factor levels and clinicopathological features of patients with systemic lupus erythematosus and lupus nephritis. Serum samples from patients with non-renal systemic lupus erythematosus, renal biopsy-proven lupus nephritis and healthy control subjects were detected by enzyme-linked immunosorbent assay for serum connective tissue growth factor levels. The associations between connective tissue growth factor levels and clinicopathological features of the patients were further analysed. The levels of serum connective tissue growth factor in patients with non-renal systemic lupus erythematosus and lupus nephritis were both significantly higher than those in the normal control group (34.14 ± 12.17 ng/ml vs. 22.8 ± 3.0 ng/ml, plupus erythematosus and lupus nephritis group (34.14 ± 12.17 ng/ml vs. 44.1 ± 46.8 ng/ml, p = 0.183). Serum connective tissue growth factor levels were significantly higher in lupus nephritis patients with the following clinical manifestations, including anaemia (51.3 ± 51.4 ng/ml vs. 23.4 ± 9.7 ng/ml, plupus nephritis (63.3 ± 63.4 ng/ml vs. 38.3 ± 37.9 ng/ml, p = 0.035, respectively). Serum connective tissue growth factor levels were negatively associated with estimated glomerular filtration rate (r = -0.46, plupus nephritis (plupus and correlated with chronic renal interstitial injury and doubling of serum creatinine in patients with lupus nephritis. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  15. Pericarditis as initial clinical manifestation of systemic lupus ...

    African Journals Online (AJOL)

    The most common diagnostic features of systemic lupus erythematosus (SLE) include mucocutaneous lesions, nephritis, arthritis and haematological disorder. .... Anti-DNA antibodies such as anti-nucleosomal, anti-double- stranded DNA, and ... tissues and organs, leading to structural damage and disturbance of function.

  16. Apartheid and healthcare access for paediatric systemic lupus ...

    African Journals Online (AJOL)

    South Africa (SA) still faces the legacy of apartheid: the history, politics and economics have a lasting, indelible effect on the health of its people. Here, we discuss the challenges of caring for patients with chronic disease, focusing on paediatric systemic lupus erythematosus (SLE), as a framework for evaluating the structural ...

  17. The skin immune system: lupus erythematosus as a paradigm

    NARCIS (Netherlands)

    Bos, J. D.

    1994-01-01

    Lupus erythematosus (LE) was first described as a clinical dermatological entity in 1851. The possibility of serious systemic manifestations became recognized by 1872 as the result of the work of M. Kaposi. Since then, it took a long time before LE was recognized to be an immunological disease.

  18. Systemic lupus Erythematosus: 2 case reports from Eritrea | Zerai ...

    African Journals Online (AJOL)

    Systemic Lupus Erythematosus is an autoimmune disorder, occurring predominantly in women during reproductive age and characterized by the presence of antibodies in the serum against the nuclear components (ANA), leading to inflammation in kidney, brain, and skin manifestations. The diversity of the disease the ...

  19. Psychiatric symptoms in systemic lupus erythematosus: an update

    NARCIS (Netherlands)

    Wekking, E. M.

    1993-01-01

    Twenty-one studies on the prevalence and type of psychiatric symptoms in systemic lupus erythematosus (SLE) are reviewed and evaluated. Substantial differences in prevalence of psychiatric symptoms in SLE-patients (from 17%-71%) have been reported. Of the investigated methodological aspects,

  20. Perihepatitis in systemic lupus erythematosus | Laabidi | Pan African ...

    African Journals Online (AJOL)

    Perihepatitis in systemic lupus erythematosus. J Laabidi, F Ajili. Abstract. No Abstract. http://dx.doi.org/10.11604/pamj.2015.20.280.5534 · AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners · Terms and Conditions of Use · Contact ...

  1. Transancestral mapping and genetic load in systemic lupus erythematosus

    NARCIS (Netherlands)

    Langefeld, Carl D.; Ainsworth, Hannah C.; Graham, Deborah S. Cunninghame; Kelly, Jennifer A.; Comeau, Mary E.; Marion, Miranda C.; Howard, Timothy D.; Ramos, Paula S.; Croker, Jennifer A.; Morris, David L.; Sandling, Johanna K.; Almlof, Jonas Carlsson; Acevedo-Vasquez, Eduardo M.; Alarcon, Graciela S.; Babini, Alejandra M.; Baca, Vicente; Bengtsson, Anders A.; Berbotto, Guillermo A.; Bijl, Marc; Brown, Elizabeth E.; Brunner, Hermine I.; Cardiel, Mario H.; Catoggio, Luis; Cervera, Ricard; Cucho-Venegas, Jorge M.; Dahlqvist, Solbritt Rantapaa; D'Alfonso, Sandra; Da Silva, Berta Martins; de la Rua Figueroa, Inigo; Doria, Andrea; Edberg, Jeffrey C.; Endreffy, Emoke; Esquivel-Valerio, Jorge A.; Fortin, Paul R.; Freedman, Barry I.; Frostegard, Johan; Garcia, Mercedes A.; Garcia de la Torre, Ignacio; Gilkeson, Gary S.; Gladman, Dafna D.; Gunnarsson, Iva; Guthridge, Joel M.; Huggins, Jennifer L.; James, Judith A.; Kallenberg, Cees G. M.; Kamen, Diane L.; Karp, David R.; Kaufman, Kenneth M.; Kottyan, Leah C.; Kovacs, Laszlo; Laustrup, Helle; Lauwerys, Bernard R.; Li, Quan-Zhen; Maradiaga-Cecena, Marco A.; Martin, Javier; McCune, Joseph M.; McWilliams, David R.; Merrill, Joan T.; Miranda, Pedro; Moctezuma, Jose F.; Nath, Swapan K.; Niewold, Timothy B.; Orozco, Lorena; Ortego-Centeno, Norberto; Petri, Michelle; Pineau, Christian A.; Pons-Estel, Bernardo A.; Pope, Janet; Raj, Prithvi; Ramsey-Goldman, Rosalind; Reveille, John D.; Russell, Laurie P.; Sabio, Jose M.; Aguilar-Salinas, Carlos A.; Scherbarth, Hugo R.; Scorza, Raffaella; Seldin, Michael F.; Sjowall, Christopher; Svenungsson, Elisabet; Thompson, Susan D.; Toloza, Sergio M. A.; Truedsson, Lennart; Tusie-Luna, Teresa; Vasconcelos, Carlos; Vila, Luis M.; Wallace, Daniel J.; Weisman, Michael H.; Wither, Joan E.; Bhangale, Tushar; Oksenberg, Jorge R.; Rioux, John D.; Gregersen, Peter K.; Syvanen, Ann-Christine; Ronnblom, Lars; Criswell, Lindsey A.; Jacob, Chaim O.; Sivils, Kathy L.; Tsao, Betty P.; Schanberg, Laura E.; Behrens, Timothy W.; Silverman, Earl D.; Alarcon-Riquelme, Marta E.; Kimberly, Robert P.; Harley, John B.; Wakeland, Edward K.; Graham, Robert R.; Gaffney, Patrick M.; Vyse, Timothy J.

    2017-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with marked gender and ethnic disparities. We report a large transancestral association study of SLE using Immunochip genotype data from 27,574 individuals of European (EA), African (AA) and Hispanic Amerindian (HA) ancestry. We identify 58

  2. Transancestral mapping and genetic load in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Langefeld, Carl D; Ainsworth, Hannah C; Graham, Deborah S Cunninghame

    2017-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with marked gender and ethnic disparities. We report a large transancestral association study of SLE using Immunochip genotype data from 27,574 individuals of European (EA), African (AA) and Hispanic Amerindian (HA) ancestry. We identify...

  3. Systemic lupus erythematosus South African child a black

    African Journals Online (AJOL)

    1991-01-19

    Jan 19, 1991 ... Vb), alopecia, lymphadenopathy, hepatomegaly, positive. CoOmbs test, hypeocomplementaemia, anti-DNA antibodies and positive anti-nuclear factor. S Atr Med J 1991; 79: 101-103. Systemic lupus erythematosus (SLE) is exceedingly rare among black children in Mrica despite the fact that it occurs more.

  4. Systemic lupus erythematosus: A possible cause of non-alcoholic ...

    African Journals Online (AJOL)

    Systemic lupus erythematosus (SLE) is a multisystemic auto immune dis- order, with neurological manifestations being common. Attribution of neurological features to SLE is often a challenging process that requires a thorough clinical evaluation, relevant laboratory investiga- tions and imaging studies. Acute cerebellar ...

  5. Influenza vaccination in systemic lupus erythematosus : Safe and protective?

    NARCIS (Netherlands)

    Holvast, Bert; Huckriede, Anke; Kallenberg, Cees G. M.; Bijl, Marc

    Patients with systemic lupus erythematosus (SLE) show decreased immune responsiveness and are vulnerable for infectious diseases, due to the underlying disease and the frequent use of immunosuppressive drugs. Influenza has a high incidence in the population and is associated with increased morbidity

  6. Treat-to-target in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Mosca, Marta; Boumpas, Dimitrios T; Bruce, Ian N

    2013-01-01

    on May 8, 2012 to discuss whether a treat-to-target approach could be applied in the treatment of systemic lupus erythematosus (SLE) (T2T/SLE), define a research agenda, and establish a plan for moving forward. In the present paper, observations raised at the meeting and literature data on potential...

  7. Case Report: An atypical case of systemic lupus erythematosus ...

    African Journals Online (AJOL)

    Background: Systemic lupus erythematosus (SLE) is a multisystem disease that can be a diagnostic conundrum. Case report: We describe a patient who presented with recurrent fleeting exudative and hemorrhagic pleural effusion. It took multiple visits over 3 months and renal biopsy to con rm the diagnosis of SLE.

  8. Calcinosis cutis universalis – a rare manifestation of systemic lupus ...

    African Journals Online (AJOL)

    Calcinosis cutis (or skin and subcutaneous calcification) is a feature of a variety of rheumatic conditions (most commonly dermatomyositis and scleroderma), but is rarely reported in systemic lupus erythematosus (SLE ). When calcinosis cutis does occur in patients with SLE, it tends to be localised (circumscripta) rather than ...

  9. Pattern of systemic Lupus erythematosus in Egyptian patients: The ...

    African Journals Online (AJOL)

    Introduction: Systemic lupus erythematosus (SLE) afflicts young people disproportionately, often at a crucial time in their lives when they are trying to establish relationships, start families and launch careers. As a result, persons with SLE may experience a wide range of physical and psychosocial problems that are not ...

  10. Treat-to-target in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    van Vollenhoven, Ronald F; Mosca, Marta; Bertsias, George

    2014-01-01

    guidance for healthcare providers and administrators. Thus, an initiative to evaluate possible therapeutic targets and develop treat-to-target guidance was believed to be highly appropriate in the management of systemic lupus erythematosus (SLE) patients as well. Specialists in rheumatology, nephrology...

  11. Circulating surfactant protein D is decreased in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Hoegh, Silje Vermedal; Voss, Anne; Sorensen, Grith Lykke

    2009-01-01

    Objective. Deficiencies of innate immune molecules like mannan binding lectin (MBL) have been implicated in the pathogenesis of systemic lupus erythematosus (SLE). Surfactant protein D (SP-D) and MBL belong to the same family of innate immune molecules - the collectins, which share important...

  12. Systemic lupus erythematosus in a black South African child ...

    African Journals Online (AJOL)

    Systemic lupus erythematosus (SLE) is poorly described among black children in Africa despite being more frequent among some black adult populations than their white counterparts. The first black South African child with SLE is documented. The patient was a 10-year-old girl who had fever, facial rash (with complement ...

  13. Outcome of pregnancy in patients with systemic lupus erythematosis ...

    African Journals Online (AJOL)

    Objective: To study maternal and fetal outcomes in Ghanaian women with systemic lupus erythematosus (SLE). Methods: Retrospective study of pregnancies in women with SLE in a single centre in Ghana. Results: The mean age was 30.1 years and all were nulliparous. Two out of the seven pregnancies were in disease ...

  14. Circulating cell free DNA as a predictor of systemic lupus ...

    African Journals Online (AJOL)

    Background: Systemic lupus erythematosus (SLE) is the most heterogeneous chronic autoimmune disease; it is characterized by the presence of auto reactive B and T cells, responsible for the aberrant production of a broad and heterogeneous group of autoantibodies. Recent studies using various detection methods have ...

  15. Renal Tubular Function in Systemic Lupus Erythematosus | Jessop ...

    African Journals Online (AJOL)

    Renal function is commonly assessed by measurement of glomerular filtration rate (GFR). However, defects in tubular function may still exist in the presence of a normal GFR. In 12 patients with systemic lupus erythematosus (SLE), 6 of whom had previously been shown to have renal impairment, glomerular and tubular ...

  16. Paraoxonase 1 activity and genotyping in systemic lupus ...

    African Journals Online (AJOL)

    Introduction: Systemic lupus erythematosus (SLE) is characterized by an enhanced risk of atherosclerosis and cardiovascular diseases (CVD). Human serum paraoxonase 1 (PON1), an antioxidant enzyme closely associated with high density lipoprotein (HDL), has been implicated in the prevention of low density ...

  17. Amyloïdosis, sarcoidosis and systemic lupus erythematosus ...

    African Journals Online (AJOL)

    The occurrence of renal and multiple organ Amyloïdosis is currently considered exceptional in the course of systemic lupus erythematosus. We report a case of a concomitant SLE and Amyloïdosis in a 57 year old female patient with hypothyroidism history, who presented with erythema nodosum, fever, arthralgia and sicca ...

  18. Incidence of systemic lupus erythematosus (SLE) in HIV seropositive ...

    African Journals Online (AJOL)

    Background: Viral agents especially retroviruses play either an aetiologic or contributory role in autoimmune diseases. Aim: To determine the rate of occurrence of systemic lupus erythematosus (SLE) in already confirmed HIV positive individuals. Methods: Subjects comprised of already diagnosed patients with HIV and ...

  19. Reversible blindness in a patient with systemic lupus erythematosus ...

    African Journals Online (AJOL)

    Neuropsychiatric manifestations can occur in majority of patients with Systemic Lupus Erythematosus (SLE). We describe a patient who presented with acute onset binocular visual loss and was found to have inflammation of optic chiasma on imaging. The patient was treated with immunosuppression. She responded ...

  20. Diffuse alveolar hemorrhage in a young woman with systemic lupus ...

    African Journals Online (AJOL)

    Diffuse Alveolar Hemorrhage (DAH) is rarely reported complication of Systemic Lupus Erythematosus (SLE). A young woman diagnosed SLE, with a previously normal plain chest radiograph, developed acute onset cough, dyspnoea and hemoptysis. The repeat urgent chest radiograph revealed alveolar opacities. The triad ...

  1. Crusted scabies in a chid with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Nurimar C.F. Wanke

    1992-03-01

    Full Text Available A child with systemic lupus erythematosus who has been treated with prednisone for three years, developed crusted scabies. Scrapings from lesions revealed Sarcoptes scabiei adult mites mad eggs. The patient died with septicemia and renal failure soon after starting topical 20% sulfur. A marked improvement was observed in the cutaneous lesions.

  2. Circulating cell free DNA as a predictor of systemic lupus ...

    African Journals Online (AJOL)

    Olfat M. Hendy

    2015-07-29

    Jul 29, 2015 ... C4;. CRP;. Procalcitonin;. RT-PCR;. Anti-nucleosome. Abstract Background: Systemic lupus erythematosus (SLE) is the most heterogeneous chronic autoimmune .... SLE can cause various tissue inflammation and damage in a ... chronic or acute inflammatory diseases were excluded from the study. 3.

  3. Systemic lupus erythematosus South African child a black

    African Journals Online (AJOL)

    1991-01-19

    Jan 19, 1991 ... Systemic lupus erythematosus (SLE) is poorly described among black children in Africa despite being more frequent among some black adult populations than their white counter- parts. The first black South African child with SLE is docu- mented. The patient was a 10-year-old girl who had fever, facial rash ...

  4. Organ involvement other than lupus nephritis in childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Huggins, J L; Holland, M J; Brunner, H I

    2016-07-01

    In this review we critically analyze pulmonary, gastrointestinal and cardiac manifestations of childhood-onset systemic lupus erythematosus (cSLE). Clinical manifestations of these organ systems may be the initial manifestation of cSLE; frequently occur with very active cSLE; and are potential life-threatening manifestations often presenting to the emergency department and requiring admission to the intensive care unit. Early recognition and treatment of the pulmonary, gastrointestinal and cardiac manifestations of cSLE will result in improved prognosis and better outcomes. © The Author(s) 2016.

  5. The lupus impact tracker is responsive to changes in clinical activity measured by the systemic lupus erythematosus responder index.

    Science.gov (United States)

    Devilliers, H; Bonithon-Kopp, C; Jolly, M

    2017-04-01

    Objective The lupus impact tracker (LIT) is a 10-item patient reported outcome tool to measure the impact of systemic lupus erythematosus or its treatment on patients' daily lives. Herein, we describe the responsiveness of the LIT and LupusQoL to changes in disease activity, using the systemic lupus erythematosus responder index (SRI). Methods A total of 325 adult systemic lupus erythematosus patients were enrolled in an observational, longitudinal, multicentre study, conducted across the USA and Canada. Data (demographics, LIT, LupusQoL, BILAG, SELENA-SLEDAI) were obtained three months apart. Modified SRI was defined as: a decrease in SELENA-SLEDAI (4 points); no new BILAG A, and no greater than one new BILAG B; and no increase in the physician global assessment. Standardised response mean and effect size for LIT and LupusQoL domains were calculated among SRI responders and non-responders. Wilcoxon's test was used to compare the LIT and LupusQoL variation by SRI responder status. Results Of the participants 90% were women, 53% were white, 33% were of African descendant and 17% were Hispanic. Mean (SD) age and SELENA-SLEDAI at baseline were 42.3 (16.2) years and 4.3 (3.8), respectively. Mean (SD) LIT score at baseline was 39.4 (22.9). LIT standardised response mean (effect size) among SRI responders and non-responders were -0.69 (-0.36) and -0.20 (-0.12), respectively ( P = 0.02). For LupusQoL, two domains were responsive to SRI: standardised response mean (effect size) for physical health and pain domains were 0.42 (0.23) and 0.65 (0.44), respectively. Conclusions LIT is moderately responsive to SRI in patients with systemic lupus erythematosus. Inclusion of this tool in clinical care and clinical trials may provide further insights into its responsiveness. This is the first systemic lupus erythematosus patient reported outcome tool to be evaluated against composite responder index (SRI) used in clinical trials.

  6. DEPRESSION--A FELLOW TRAVELER WITH SYSTEMIC LUPUS ERYTHEMATOSUS.

    Science.gov (United States)

    Cojocaru, Doina-Clementina; Costin, Melania; Bădeanu, Lucia Elena; Negru, R D; Aursulesei, Viviana

    2015-01-01

    Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory disorder that occurs primarily in women of childbearing age, immunologic abnormalities being a prominent feature of the disease. Psychiatric disorders frequently coexist, depression being the most common mood disorder in neuropsychiatric lupus. This literature review was performed through searching MEDLINE database for full-text English-language articles--original research, systematic review and updates published in the last five years (2010-2015), using the keywords "depression and systemic lupus erythematosus". The main outcomes identified were prevalence and predictors of depression in various cultural and ethnic groups, depression-related clinical issues (suicidal ideation, cognitive impairment, altered body image, sleep and sexual disturbances, influence of SLE treatment), and influence on quality of life. A multidisciplinary approach that takes into account the polymorphism and individual variability of the SLE clinical manifestations helps to improve early detection of depression, which is responsible for the increased risk of comorbidities, suicidal attempts, decreased treatment adherence, and impaired quality of life. Physicians across all specialties involved in the care for lupus patients should be aware of the major prevalence of this condition, while helping patients to cope with their disabling disease.

  7. Lupus eritematoso sistêmico associado a miastenia gravis: relato de caso Systemic lupus erythematosus and myasthenia gravis: case report

    Directory of Open Access Journals (Sweden)

    MARCIO F. DE CARVALHO

    1998-03-01

    Full Text Available Os autores descrevem o caso de uma mulher branca de 24 anos de idade admitida com lupus eritematoso sistêmico (com 4 anos de evolução de doença e início recente de miastenia gravis. São discutidos os principais diagnósticos diferenciais para a fraqueza muscular e a fadiga apresentadas por esta paciente. Uma revisão de literatura abordando a associação de miastenia gravis e lupus eritematoso é feita, com ênfase às características clínicas desses pacientes e ao papel do timoma e timectomia no desenvolvimento de lupus eritematoso em pacientes previamente miastênicos.We report the case of a 24-year-old white woman admitted with a four year diagnosis of systemic lupus erythematosus and the recent onset of myasthenia gravis discussing the main differential diagnosis of weakness and fatigue in this patient. A review of literature approaching the association of myasthenia gravis and systemic lupus erythematosus is also done with emphasis on the clinical characteristics of these patients and the role of thymoma and thymectomy in the development of systemic lupus erythematosus in myasthenic patients.

  8. Systemic lupus erythematosus presenting with eosinophilic enteritis: a case report

    Directory of Open Access Journals (Sweden)

    Kalany Mohammad

    2011-06-01

    Full Text Available Abstract Introduction Systemic lupus erythematosus (SLE is a multisystem disorder that may present with various symptoms. It may involve the gastrointestinal tract in a variety of ways; some of the most well-known ones are transaminitis, lupus mesenteric vasculitis, lupus enteritis and mesenteric vascular leakage. We describe a case of a patient with SLE who presented with a five-month history of diarrhea caused by eosinophilic enteritis. To the best of our knowledge, there are few cases reported in the literature of patients with SLE who initially present with chronic diarrhea due to eosinophilic enteritis. Case presentation A 38-year-old Persian Iranian woman was admitted with a five-month history of diarrhea and abdominal pain. A physical examination showed nothing abnormal. Initially, she had only lymphopenia and mild eosinophilia. No autoimmune or infectious etiology was detected to justify these abnormalities. A thorough evaluation was not helpful in finding the etiology, until she developed a scalp lesion similar to discoid lupus erythematosus. Computed tomography showed small bowel wall thickening. Briefly, she manifested full-blown SLE, and it was revealed that the diarrhea was caused by eosinophilic enteritis. Conclusion Considering SLE in a patient who presents with chronic diarrhea and lymphopenia may be helpful in earlier diagnosis and therapy. This is an original case report of interest to physicians who practice internal medicine, family medicine and gastroenterology.

  9. Quality of Life in Patient with Systemic Lupus Erythematosus (SLE

    Directory of Open Access Journals (Sweden)

    Irma Yanih

    2016-11-01

    Full Text Available The number of patient with Systemic Lupus Erythematosus (SLE was keep growing. SLE attacked many women and developed between the age of 15-45. The treatment which used by patient with SLE just for reduced or faded the symptoms that occurred. It couldn’t heals the patient properly yet, so that sometimes that symptoms would occur again. The object of this study were to see the quality of life from 13 patient SLE in 8 domain from Quality of Life (QoL such as physical health, emotional health, body image, pain, planning, fatigue intimate relationship and burden to others. This study was a study case in 13 patient with SLE which lived in Surabaya and a member of Lupus Indonesia Foundation branch Surabaya. Primary data were collected by interview using LupusQoL questionnaire and the weight was measured by digital weight scales. In this study, there were 13 woman patient with SLE in the age between 15–40 years old with highly educated and having normal nutrition status. They usually work and having monthly income > Rp 1,740,000, were suffer SLE for more than 5 years and having good knowledge in Lupus and SLE. This study showed a good value quality of life in patient with SLE at 3 domain. That were body image, intimate relationship and physical health domain. Some patients had a bad even worse quality in pain, fatigue and burden to others domain. Some of patients with SLE had a good quality of life except pain, fatigue, and burden to others domain. Keywords: Quality of Life (QoL, Systemic Lupus Erythematosus (SLE, desease duration

  10. Mood Disorders in Systemic Lupus Erythematosus

    Science.gov (United States)

    Hanly, John G.; Su, Li; Urowitz, Murray B.; Romero-Diaz, Juanita; Gordon, Caroline; Bae, Sang-Cheol; Bernatsky, Sasha; Clarke, Ann E.; Wallace, Daniel J.; Merrill, Joan T.; Isenberg, David A.; Rahman, Anisur; Ginzler, Ellen M.; Petri, Michelle; Bruce, Ian N.; Dooley, M. A.; Fortin, Paul; Gladman, Dafna D.; Sanchez-Guerrero, Jorge; Steinsson, Kristjan; Ramsey-Goldman, Rosalind; Khamashta, Munther A.; Aranow, Cynthia; Alarcón, Graciela S.; Fessler, Barri J.; Manzi, Susan; Nived, Ola; Sturfelt, Gunnar K.; Zoma, Asad A.; van Vollenhoven, Ronald F.; Ramos-Casals, Manuel; Ruiz-Irastorza, Guillermo; Lim, S. Sam; Kalunian, Kenneth C.; Inanc, Murat; Kamen, Diane L.; Peschken, Christine A.; Jacobsen, Soren; Askanase, Anca; Theriault, Chris; Thompson, Kara; Farewell, Vernon

    2015-01-01

    Objective To determine the frequency, clinical and autoantibody associations and outcome of mood disorders in a multi-ethnic/racial, prospective, inception cohort of SLE patients. Methods Patients were assessed annually for mood disorders (4 types as per DSM-IV) and 18 other neuropsychiatric (NP) events. Global disease activity (SLEDAI-2K), SLICC/ACR damage index (SDI) and SF-36 subscale, mental (MCS) and physical (PCS) component summary scores were collected. Time to event, linear and ordinal regressions and multi-state models were used as appropriate. Results Of 1,827 SLE patients, 88.9% were female, 48.9% Caucasian, mean ± SD age 35.1±13.3 years, disease duration 5.6±4.8 months and follow-up 4.73±3.45 years. Over the study 863 (47.2%) patients had 1,627 NP events. Mood disorders occurred in 232/1827 (12.7%) patients and 98/256 (38.3%) events were attributed to SLE. The estimated cumulative incidence of any mood disorder after 10 years was 17.7% (95%CI=[15.1%,20.2%]). There was a greater risk of mood disorder in patients with concurrent NP events (p ≤ 0.01) and lower risk with Asian race/ethnicity (p=0.01) and immunosuppressive drugs (p=0.003). Mood disorders were associated with lower mental health subscale and MCS scores but not with SLEDAI-2K, SDI scores or lupus autoantibodies. Antidepressants were used in 168/232 (72.4%) patients with depression. 126/256 (49.2%) mood disorders resolved in 117/232 (50.4%) patients. Conclusion Mood disorders, the second most frequent NP event in SLE patients, have a negative impact on HRQoL and improve over time. The lack of association with global SLE disease activity, cumulative organ damage and lupus autoantibodies emphasize their multifactorial etiology and a role for non-lupus specific therapies. PMID:25778456

  11. Infection in systemic lupus erythematosus, similarities, and differences with lupus flare

    Science.gov (United States)

    Jung, Ju-Yang; Suh, Chang-Hee

    2017-01-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse manifestations, and its pathogenesis is unclear and complicated. Infection and SLE are similar in that they both cause inf lammatory reactions in the immune system; however, one functions to protect the body, whereas the other is activated to damage the body. Infection is known as one of the common trigger factors for SLE; there are a number of reports on infectious agents that provoke autoimmune response. Several viruses, bacteria, and protozoa were revealed to cause immune dysfunction by molecular mimicry, epitope spreading, and bystander activation. In contrast, certain pathogens were revealed to protect from immune dysregulation. Infection can be threatening to patients with SLE who have a compromised immune system, and it is regarded as one of the common causes of mortality in SLE. A clinical distinction between infection and lupus f lare up is required when patients with SLE present fevers. With a close-up assessment of symptoms and physical examination, C-reactive protein and disease activity markers play a major role in differentiating the different disease conditions. Vaccination is necessary because protection against infection is important in patients with SLE. PMID:28490724

  12. Lupus anticoagulants and antiphospholipid antibodies

    Science.gov (United States)

    Blood clots - lupus anticoagulants; DVT - anticoagulants ... Most often, lupus anticoagulants and aPL are found in people with diseases such as systemic lupus erythematosus (SLE). Lupus anticoagulants and ...

  13. Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients.

    Science.gov (United States)

    Touzot, Maxime; Terrier, Cécile Saint-Pastou; Faguer, Stanislas; Masson, Ingrid; François, Hélène; Couzi, Lionel; Hummel, Aurélie; Quellard, Nathalie; Touchard, Guy; Jourde-Chiche, Noémie; Goujon, Jean-Michel; Daugas, Eric

    2017-12-01

    Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome.We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chronic infections with HIV or hepatitis B or C; patients with a bacterial infectious disease; and patients with pure membranous nephropathy. Electron microscopy was retrospectively performed when the material was available. End points were the proportion of patients with a complete response (urine protein to creatinine ratio lupus nephritis in all cases. Electron microscopy was performed on samples from 5 patients. The results showed mesangial and subendothelial dense deposits consistent with LN in 4 cases, and a retrospective diagnosis of pseudo-amyloid fibrillary glomerulonephritis was made in 1 patient.Patients received immunosuppressive therapy consisting of induction therapy followed by maintenance therapy, similar to treatment for severe lupus nephritis. Remission was recorded in 10 patients at 12 months after the initiation of treatment. One patient reached end-stage renal disease. After a median follow-up of 24 months, 2 patients relapsed.Lupus nephritis in the absence of overt SLE is a nosological entity requiring careful etiological investigation, including systematic electron microscopy examination of renal biopsies to rule out fibrillary glomerulonephritis. In this series, most patients presented with severe glomerulonephritis, which was highly similar to lupus nephritis at presentation and in terms of response to immunosuppressive therapy.

  14. Toxocara canis infection: Unusual trigger of systemic lupus erythematosus.

    Science.gov (United States)

    Levy, Michaël; Bourrat, Emmanuelle; Baudouin, Véronique; Guillem, Colette; Peuchmaur, Michel; Deschênes, Georges; Fila, Marc

    2015-08-01

    Infection by Toxocara canis can cause systemic vasculitis. We report here a unique case of systemic lupus erythematosus (SLE) triggered by T. canis infection. An 8-year-old girl was treated with albendazole therapy for common toxocariasis, but she developed two weeks later, asthenia, fever, infiltrated maculopapular eruption of the face, peripheral vascular disease with necrosis of the fingers and inflammatory anemia with proteinuria. Anti-nuclear, anti-DNA and anti-Sm antibodies positivity, together with minimal change nephritis with mesangial exclusive IgM deposit on renal biopsy and clinical relapse after initially successful steroid therapy, led to the diagnosis of SLE. T. canis infection can trigger systemic lupus but must also be ruled out of the differential diagnosis given its association with autoimmunity. © 2015 Japan Pediatric Society.

  15. Hypocomplementaemic urticarial vasculitis syndrome: a mimicker of systemic lupus erythematosus.

    Science.gov (United States)

    Roy, Krishnendu; Talukdar, Arunansu; Kumar, Bappaditya; Sarkar, Sumanta

    2013-05-22

    A middle aged female patient presented with generalised palpable purpura associated with intense pruritus along with subconjunctival haemorrhage and orbital inflammation. There was extensive dermographism. Other systemic examinations were within normal limits. Haematological profile was normal except raised D-dimer. Skin biopsy revealed the presence of leucocytoclastic vasculitis. Antinuclear antibody was positive in a titre of 1 : 160, but antidouble-stranded DNA was negative. Urine examination revealed haematuria and proteinuria. Complement C3, C4 and C1q levels were decreased with the presence of anti-C1q antibody. There was a diagnostic dilemma between systemic lupus erythematosus and hypocomplementaemic urticarial vasculitis syndrome. However, as the patient did not fulfil the American College of Rheumatology criteria for systemic lupus erythematosus, but fulfilled all the criteria for hypocomplementaemic urticarial vasculitis syndrome, the case was finally diagnosed as hypocomplementaemic urticarial vasculitis syndrome and treated accordingly with favourable outcome.

  16. Differential activation mechanisms of serum C5a in lupus nephritis and neuropsychiatric systemic lupus erythematosus.

    Science.gov (United States)

    Sakuma, Yuko; Nagai, Tatsuo; Yoshio, Taku; Hirohata, Shunsei

    2017-03-01

    To explore the role of C5a in the pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE) and lupus nephritis (LN). Sera were obtained from 29 patients with NPSLE, 25 with LN, 26 without NPSLE or LN [SLE alone], and 21 healthy donors. Cerebrospinal fluid (CSF) was obtained from 29 NPSLE patients. C5a and C5 were measured by ELISA. Blood-brain barrier (BBB) function was evaluated by Q albumin ([CSF albumin/serum albumin] × 10 3 ). Serum C5a, but not C5, was significantly increased in SLE compared with healthy control. Serum C5a, but not C5, was significantly higher in NPSLE and in LN than in SLE alone. Serum C4, but not C3, was lower in LN than in NPSLE. Q albumin was significantly higher in diffuse NPSLE than in focal NPSLE, whereas there were no significant differences in CSF or serum C5a between both groups. Notably, CSF C5 and C5a were significantly correlated with Q albumin, whereas serum C5a, but not C5, appeared to be inversely correlated with Q albumin. These results disclosed that serum C5a was elevated not only in NPSLE but also in LN through different mechanisms. Moreover, it is suggested that C5a might be consumed during BBB damages.

  17. Dickkopf-1 Is a Biomarker for Systemic Lupus Erythematosus and Active Lupus Nephritis.

    Science.gov (United States)

    Xue, Jing; Yang, Jiali; Yang, Lijuan; Zhou, Shaolan; Ji, Chen; Wang, Xuemei; Yu, Nan; Liu, Xiaoming; Chi, Shuhong

    2017-01-01

    An early diagnosis of lupus nephritis (LN) has an important clinical implication in guiding treatments of systemic lupus erythematosus (SLE) in clinical settings. In this study, the concentrations of Wnt-3A, Frizzled-8 (FZD-8), and Dickkopf-1 (DKK-1) of Wnt signaling, as well as their diagnostic values for accessing LN, were evaluated by ELISA in sera and urine of 111 SLE patients (31 with LN and 80 without LN) and 70 healthy cohorts. Significantly more abundances of DKK-1 protein were determined in both of sera and urine of SLE patients compared to healthy cohorts ( p < 0.0001); in particular the serum DKK-1 concentration was even higher in LN-SLE patients relative to non-LN SLE subjects ( p < 0.0001). Intriguingly, concentrations of above examined proteins in SLE patients showed no correlation between serum and urine. Moreover, a combination of DKK-1 with anti-dsDNA and/or levels of complement C3 and C4 could not increase the specificity and/or sensitivity for identification of patients with LN diseases, but both ROC curve and multiple-factor nonconditional logistic regression analysis showed that serum DKK-1 was considered better positive biomarker for identification of LN in SLE patients. These results imply that serum and/or urine DKK-1 may be a valuable and independent biomarker for identification of SLE patients with LN.

  18. Effect of Autoantibodies to Erythropoietin Receptor in Systemic Lupus Erythematosus with Biopsy-proven Lupus Nephritis.

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    Hara, Akinori; Furuichi, Kengo; Yamahana, Junya; Yasuda, Haruka; Iwata, Yasunori; Sakai, Norihiko; Shimizu, Miho; Kaneko, Shuichi; Wada, Takashi

    2016-07-01

    We examined the clinical significance of autoantibodies to the erythropoietin receptor (EPOR) in patients with systemic lupus erythematosus (SLE) who had biopsy-proven lupus nephritis (LN). Forty-six Japanese patients with SLE with LN who had undergone renal biopsy during 1993-2014 were enrolled in this study and followed for a mean of 83 months. Sera from those patients were screened for anti-EPOR antibodies using ELISA. Anti-EPOR antibodies were detected in 18 (39%) of the 46 patients with SLE with anemia. Anti-EPOR antibodies were associated with low hemoglobin concentrations and reticulocytopenia. In addition, anti-EPOR antibodies were positively correlated with SLE disease activity, even though serum levels of the complement factors 3 and 4 did not differ between the 2 groups. In patients with International Society of Nephrology/Renal Pathology Society 2003 class IV LN, anti-EPOR antibodies were associated with active lesions including cellular crescents in glomeruli. Decrease in renal function was more frequently observed in patients without complete or partial renal response than in patients with it, and serum levels of the antibodies as well as renal response to treatment were significant risk factors for progression of renal dysfunction. The present study suggests that anti-EPOR antibodies might be involved in overall disease activity and active renal lesions, as well as in the impaired erythropoiesis in patients with SLE with LN. Further, the levels of anti-EPOR antibodies may be an additional predictor for renal injury.

  19. Interleukin-27 and interleukin-23 in patients with systemic lupus erythematosus: possible role in lupus nephritis.

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    Xia, L P; Li, B F; Shen, H; Lu, J

    2015-05-01

    To analyse the concentration of interleukin (IL)-27 and IL-23 in serum and urine of patients with systemic lupus erythematosus (SLE) compared with healthy controls (HC). An enzyme-linked immunosorbent assay (ELISA) was used to analyse the serum and urine concentration of IL-27 and IL-23 from 50 patients with lupus nephritis (LN), 55 patients without LN, and 30 HC. The correlations between the levels of IL-27, IL-23, and disease activity, clinical parameters in SLE patients were analysed. The levels of IL-27 and IL-23 increased significantly in the serum and urine of SLE patients with and without LN compared with HC. Moreover, urine levels of IL-27 and IL-23 were correlated with the renal SLE Disease Activity Index (rSLEDAI) score and 24-h urinary protein levels. After 6 months of immunosuppressive treatment, urine IL-27 expression rose significantly in SLE patients with LN. IL-27 and IL-23 may be involved in the pathogenesis of LN.

  20. Hydrocephalus in an Elderly Man with Systemic Lupus Erythematosus

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    Wei-Sheng Chen

    2009-06-01

    Full Text Available A 71-year-old man presented with quadriplegia, seizures, dysarthria, motor aphasia and urinary incontinence lasting for several years. The development of proteinuria and increased susceptibility to infections brought the physician's attention to possible underlying autoimmune diseases. Laboratory investigations revealed evidence for systemic lupus erythematosus (SLE and antiphospholipid syndrome. Imaging studies showed obstructive hydrocephalus. Several courses of methylprednisolone therapies followed by maintenance therapy with low-dose steroid, ventriculoperitoneal shunt, and antihypertensives improved the proteinuria and dysarthria but not the urinary incontinence or dementia. A thromboembolic event in the central nervous system secondary to phospholipid antibodies or lupus activity may represent a pathogenetic basis for hydrocephalus. When encountering a patient with hydrocephalus but without apparent predisposing factors, it is always important to include SLE as a differential diagnosis.

  1. Aberrant Epstein–Barr viral infection in systemic lupus erythematosus☆

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    Poole, Brian D.; Templeton, Amanda K.; Guthridge, Joel M.; Brown, Eric J.; Harley, John B.; James, Judith A.

    2009-01-01

    Serologic association, cross-reactivity of select EBV-specific antibodies with SLE autoantigens, SLE-like autoimmunity after immunization with EBV peptides, increased EB viral load in SLE patients, and SLE-specific alterations in EBV humoral and cellular immunity implicate Epstein–Barr virus (EBV) in the development of systemic lupus erythematosus (SLE). To investigate SLE-specific differences in EBV gene expression, levels of eight EBV genes were compared between SLE patients and controls by...

  2. Pyoderma gangrenosum Preceding the diagnosis of systemic lupus erythematosus.

    Science.gov (United States)

    Waldman, Mark A; Callen, Jeffrey P

    2005-01-01

    Patients with systemic lupus erythematosus (SLE) often develop leg ulceration, particularly those with antiphospholipid antibodies or with vasculitis. Pyoderma gangrenosum (PG) is an idiopathic ulcerative neutrophilic dermatosis that is commonly associated with inflammatory bowel disease or seronegative polyarthritis. Although PG-like lesions have been commonly described in patients with the antiphospholipid antibody syndrome, the occurrence of PG as a preceding manifestation of SLE has only rarely been reported. We present a patient who developed PG roughly 8 years prior to developing SLE.

  3. Pharmacokinetic modeling of therapies for systemic lupus erythematosus

    OpenAIRE

    Yang, Xiaoyan; Sherwin, Catherine MT; Yu, Tian; Yellepeddi, Venkata K.; Brunner, Hermine I; Vinks, Alexander A.

    2015-01-01

    With the increasing use of different types of therapies in treating autoimmune diseases such as systemic lupus erythematosus (SLE), there is a need to utilize pharmacokinetic (PK) strategies to optimize the clinical outcome of these treatments. Various PK analysis approaches, including population PK modeling and physiologically based PK modeling, have been used to evaluate drug PK characteristics and population variability or to predict drug PK profiles in a mechanistic manner. This review ou...

  4. Growth Pattern in Children with Systemic Lupus Erythematosus

    OpenAIRE

    Eiman Abdalla; Lakshamanan Jeyaseelan; Irfan Ullah; Reem Abdwani

    2017-01-01

    Objectives: Children with childhood-onset systemic lupus erythematosus (cSLE) enter adulthood with considerable morbidity. Of the recognized morbidities, growth failure is unique to cSLE. The aim of this study was to evaluate the growth pattern in children with cSLE longitudinally and identify possible risk factors. Methods: Serial anthropometric measurements of cSLE patients were obtained over two years and expressed as z-scores. Parental heights were obtained to calculate target height. Par...

  5. Gastrointestinal Manifestations of Systemic Lupus Erythematosus and Scleroderma

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    Cherag Daruwala; Giancarlo Mercogliano; Thomas P. Harder

    2009-01-01

    In this review, we analyze the effects of systemic lupus erythematosus and scleroderma on the gastrointestinal tract. There is a wide variation of gastrointestinal manifestations from these autoimmune disorders including but not limited to: oral ulcers, dysphagia, gastroesophageal reflux disease, abdominal pain, constipation, diarrhea, fecal incontinence, pseudo-obstruction, perforation and gastrointestinal bleeding. The purpose of this review is to discuss these manifestations, the appropria...

  6. Gastrointestinal Manifestations of Systemic Lupus Erythematosus and Scleroderma

    Directory of Open Access Journals (Sweden)

    Cherag Daruwala

    2009-01-01

    Full Text Available In this review, we analyze the effects of systemic lupus erythematosus and scleroderma on the gastrointestinal tract. There is a wide variation of gastrointestinal manifestations from these autoimmune disorders including but not limited to: oral ulcers, dysphagia, gastroesophageal reflux disease, abdominal pain, constipation, diarrhea, fecal incontinence, pseudo-obstruction, perforation and gastrointestinal bleeding. The purpose of this review is to discuss these manifestations, the appropriate diagnostic tests, and treatment.

  7. Management of systemic lupus erythematosus during pregnancy: challenges and solutions

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    Knight CL

    2017-03-01

    Full Text Available Caroline L Knight, Catherine Nelson-Piercy Division of Women’s Health, Women’s Health Academic Centre, King’s College London and King’s Health Partners, St Thomas’ Hospital, London, UK Abstract: Systemic lupus erythematosus (SLE is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal–neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Effective prepregnancy risk assessment and counseling includes exploration of factors for poor pregnancy outcome, discussion of risks, and appropriate planning for pregnancy, with consideration of discussion of relative contraindications to pregnancy. In pregnancy, early referral for hospital-coordinated care, involvement of obstetricians and rheumatologists (and other specialists as required, an individual management plan, regular reviews, and early recognition of flares and complications are all important. Women are at risk of lupus flares, worsening renal impairment, onset of or worsening hypertension, preeclampsia, and/or venous thromboembolism, and miscarriage, intrauterine growth restriction, preterm delivery, and/or neonatal lupus syndrome (congenital heart block or neonatal lupus erythematosus. A cesarean section may be required in certain obstetric contexts (such as urgent preterm delivery for maternal and/or fetal well-being, but vaginal birth should be the aim for the majority of women. Postnatally, an ongoing individual management plan remains important, with neonatal management where necessary and rheumatology follow-up. This article explores the challenges at each stage of pregnancy, discusses the effect of SLE on pregnancy and

  8. Intravenous immunoglobulin therapy for hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus in a child.

    Science.gov (United States)

    Yamazaki-Nakashimada, Marco A; Duran-McKinster, Carola; Ramírez-Vargas, Nadia; Hernandez-Bautista, Victor

    2009-01-01

    Hypocomplementemic urticarial vasculitis is a type of urticarial vasculitis with multisystemic involvement and poor prognosis, sometimes associated with systemic lupus erythematosus. Several therapies have been attempted with no consensus on an effective therapeutic regimen. Intravenous immunoglobulin has been used in severe manifestations of systemic lupus erythematosus and recently in hypocomplementemic urticarial vasculitis. We present a 7-year-old girl with hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus and pneumonia who responded favorably to intravenous immunoglobulin.

  9. B-cell-depleting Therapy in Systemic Lupus Erythematosus

    Science.gov (United States)

    Ramos-Casals, Manuel; Sanz, Iñaki; Bosch, Xavier; Stone, John H.; Khamashta, Munther A.

    2014-01-01

    The emergence of a new class of agents (B-cell-depleting therapies) has opened a new era in the therapeutic approach to systemic lupus erythematosus, with belimumab being the first drug licensed for use in systemic lupus erythematosus in more than 50 years. Four agents deserve specific mention: rituximab, ocrelizumab, epratuzumab, and belimumab. Controlled trials have shown negative results for rituximab, promising results for epratuzumab, and positive results for belimumab. Despite these negative results, rituximab is the most-used agent in patients who do not respond or are intolerant to standard therapy and those with life-threatening presentations. B-cell-depleting agents should not be used in patients with mild disease and should be tailored according to individual patient characteristics, including ethnicity, organ involvement, and the immunological profile. Forthcoming studies of B-cell-directed strategies, particularly data from investigations of off-label rituximab use and postmarketing studies of belimumab, will provide new insights into the utility of these treatments in the routine management of patients with systemic lupus erythematosus. PMID:22444096

  10. Correlation between the Modified Systemic Lupus Erythematosus Disease Activity Index 2000 and the European Consensus Lupus Activity Measurement in juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Sato, J O; Corrente, J E; Saad-Magalhães, C

    2016-11-01

    Objective The objective of this study was to assess Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and European Consensus Lupus Activity Measurement (ECLAM) disease activity correlation in addition to their respective correlation to Pediatric Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (Ped-SDI), in juvenile systemic lupus erythematosus (JSLE). Methods The activity indices were scored retrospectively and summarized by adjusted means during follow-up. The Ped-SDI was scored during the last visit for those with more than six months follow-up. Pearson correlation between the Modified SLEDAI-2K and ECLAM, as well as Spearman correlations between the Modified SLEDAI-2K, ECLAM, and Ped-SDI were calculated. The receiver operating characteristic (ROC) curve was calculated for both activity indices discriminating damage measured by Ped-SDI. Results Thirty-seven patients with mean age at diagnosis 11 ± 2.9 years and mean follow-up time 3.2 ± 2.4 years were studied. The Modified SLEDAI-2K and ECLAM adjusted means were highly correlated ( r = 0.78, p  0.7, p < 0.001), but Modified SLEDAI-2K and ECLAM correlation with Ped-SDI was only moderate. ROC analysis discriminant performance for both activity indices resulted in area under curve (AUC) of 0.74 and 0.73 for Modified SLEDAI-2K and ECLAM, respectively. Conclusion The high correlation found between the Modified SLEDAI-2K and ECLAM adjusted means indicated that both tools can be equally useful for longitudinal estimates of JSLE activity.

  11. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

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    Burns, Natalie S. [University of Washington Medical Center, Department of Radiology, Seattle, WA (United States); Stevens, Anne M. [Seattle Children' s Hospital, Division of Rheumatology, Department of Pediatrics, Seattle, WA (United States); Iyer, Ramesh S. [University of Washington School of Medicine, Seattle Children' s Hospital, Department of Radiology, Seattle, WA (United States)

    2014-10-15

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  12. Genetically engineered biological agents in therapy for systemic lupus erythematosus

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    Elena Aleksandrovna Aseeva

    2013-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a prototype for chronic autoimmune disease. Its prevalence is 20 to 70 cases per 100,000 women and varies by race and ethnicity. Despite considerable progress in traditional therapy, many problems associated with the management of these patients need to be immediately solved: thus, 50-80% are found to have activity signs and/or frequent exacerbations and about 30% of the patients have to stop work; Class IV lupus nephritis increases the risk of terminalrenal failure. In the past 20 years great progress has been made in studying the pathogenesis of SLE: biological targets to affect drugs have been sought and fundamentally new therapeutic goals defined. Belimumab is the first genetically biological agent specially designed to treat SLE, which is rightly regarded as one of the most important achievements of rheumatology in the past 50 years.

  13. Crusted scabies in a chid with systemic lupus erythematosus

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    Nurimar C.F. Wanke

    1992-03-01

    Full Text Available A child with systemic lupus erythematosus who has been treated with prednisone for three years, developed crusted scabies. Scrapings from lesions revealed Sarcoptes scabiei adult mites mad eggs. The patient died with septicemia and renal failure soon after starting topical 20% sulfur. A marked improvement was observed in the cutaneous lesions.E descrito um caso de sarna crostosa em criança portadora de lupus eritematoso sistêmico em tratamento com prednisona há três anos. O raspado das lesões cutâneas revelou ovos e ácaros adultos de Sarcoptes scabiei. A paciente faleceu por sepsis e insuficiência renal pouco tempo após início da terapêutica tópica com enxofre a 20%. Melhora importante foi observada no quadro dermatológico.

  14. Polyarthritis due to systemic lupus erythematosus in a dog

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    R.M. Krüger

    2013-04-01

    Full Text Available A five year old male mongrel dog was presented for medical consultation with a history of arthralgia. Complete blood count revealed linfopenia and neutropenia, antinuclear antibody was positive at 1:1,256, and synovial fluid analysis showed inflammatory arthritis with lupus erythematosus cells. No significant proteinuria was detected on urinalysis, and microalbuminuria measurement was performed to determine glomerulonephritis in early stage. Based on clinical signs, synovial fluid analysis, antinuclear antibody test and complete blood count, the diagnosis was systemic lupus erythematosus. The measurement of microalbuminuria was useful to demonstrate the absence of glomerulonephritis, and the performance of complementary tests proved to be indispensable for diagnosis and prognosis. Glucocorticoid treatment led to complete remission.

  15. [Lupus hepatitis].

    Science.gov (United States)

    Ben Hadj, Yahia Chiraz; Chaabouni, Lilia; Montacer, Kchir Mohamed; Abid, Feriel; Zouari, Rafik

    2002-07-01

    We report the case of 42 year-old man who presents an acute polyarthritis associated with systemic manifestation and immunologic disorders related to systemic lupus erythematosus. Hepatic tests show cholostase and cytolysis. Hepatic involvement is linked with systemic lupus erythematosus after exclusion of hepatotoxic drugs, viral hepatitis and absence of anti mitochondrial and anti muscle antibodies. Lupus hepatitis seems to be correlated with autoantibodies to ribosomal P protein. Its treatment remains to be defined.

  16. Gestational weight gain in women with systemic lupus erythematosus.

    Science.gov (United States)

    Eudy, A M; Siega-Riz, A M; Engel, S M; Franceschini, N; Howard, A G; Clowse, M E B; Petri, M

    2017-05-01

    Objective The objective of this study was to estimate the proportion of pregnant women with systemic lupus erythematosus meeting Institute of Medicine guidelines for gestational weight gain and determine correlates of adherence to guidelines. Methods Singleton, live births in the Hopkins Lupus Pregnancy Cohort 1987-2015 were included. Pre-pregnancy weight was the weight recorded 12 months prior to pregnancy/first trimester. Final weight was the last weight recorded in the third trimester. Adherence to Institute of Medicine guidelines (inadequate, adequate, or excessive) was based on pre-pregnancy body mass index. Fisher's exact test and analysis of variance determined factors associated with not meeting guidelines. Stepwise selection estimated predictors of gestational weight gain. Results Of the 211 pregnancies, 34%, 24% and 42% had inadequate, adequate and excessive gestational weight gain, respectively. In exploratory analyses, differences in Institute of Medicine adherence were observed by pre-pregnancy body mass index, race, elevated creatinine during pregnancy and pre-pregnancy blood pressure. Odds of inadequate and excessive gestational weight gain increased 12% with each 1 kg/m 2 increase in pre-pregnancy body mass index. Lower maternal education was associated with increased odds of inadequate and excessive gestational weight gain. Conclusions As in the general population, most women with systemic lupus erythematosus did not meet Institute of Medicine guidelines. Our results identified predictors of gestational weight gain to aid in targeted interventions to improve guideline adherence in this population.

  17. [Gastrointestinal manifestations of systemic lupus erythematosus. Case report].

    Science.gov (United States)

    Zietkiewicz, Marcin; Smoleńska, Zaneta; Zdrojewski, Zbigniew

    2010-01-01

    Systemic lupus erythematosus (SLE) is an inflammatory connective tissue disease with an autoimmune background, involving various organs and systems during its course. The most important and characteristic clinical manifestations have been included in the revised diagnostic criteria for the classification of SLE published by the American College of Rheumatology (ACR) in 1987. One of them is oral ulceration which occurs in 50% of SLE patients. Oral ulcers and other gastrointestinal complaints such as dyspepsia, abdominal pain and diarrhea, usually attributed to the side-effects of medications, are among the most frequent symptoms in patients with lupus. We report the case of a 42-year-old female suffering from long-standing lupus with kidney and joint involvement, who developed abdominal pain, diarrhea, edema, and cachexia. Our case illustrates the difficulties encountered when searching for the cause of gastrointestinal symptoms. Attention during diagnosis should be given to rare gastrointestinal manifestations of SLE, such as intestinal pseudo-obstruction (IPO) and protein-losing enteropathy (PLE).

  18. [Dyslipidaemia and atherogenic risk in patients with systemic lupus erythematosus].

    Science.gov (United States)

    Batún Garrido, José Antonio de Jesús; Radillo Alba, Hugo Alberto; Hernández Núñez, Éufrates; Olán, Francisco

    2016-07-15

    Dyslipidaemia is a common comorbidity in patients with systemic lupus erythematosus. Fifty-one patients were included. Variables associated with the disease and the drugs used were recorded. Atherogenic risk was calculated. Chi square was used for categorical variables. ANOVA was performed and a logistic regression model to determine the association of the variables with the presence of dyslipidaemia. A percentage of 68.6 had dyslipidaemia. A significant difference between the presence of dyslipidaemia and activity index measured by SLEDAI was found, the presence of lupus nephritis, use of prednisone≥20mg/day, evolution of the disease<3 years. Significance between the absence of dyslipidaemia and use of hydroxychloroquine was found. SLEDAI≥4 and the use of prednisone≥20mg/day were independently associated with the presence of dyslipidaemia. The average of Castelli rate was 5.02, the Kannel index was 2.97 and triglyceride/HDL-C ratio was 5.24. Patients with systemic lupus erythematosus have a high prevalence of dyslipidaemia and a high atherogenic rate, which increases cardiovascular risk significantly. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  19. Infodemiology of systemic lupus erythematous using Google Trends.

    Science.gov (United States)

    Radin, M; Sciascia, S

    2017-07-01

    Objective People affected by chronic rheumatic conditions, such as systemic lupus erythematosus (SLE), frequently rely on the Internet and search engines to look for terms related to their disease and its possible causes, symptoms and treatments. 'Infodemiology' and 'infoveillance' are two recent terms created to describe a new developing approach for public health, based on Big Data monitoring and data mining. In this study, we aim to investigate trends of Internet research linked to SLE and symptoms associated with the disease, applying a Big Data monitoring approach. Methods We analysed the large amount of data generated by Google Trends, considering 'lupus', 'relapse' and 'fatigue' in a 10-year web-based research. Google Trends automatically normalized data for the overall number of searches, and presented them as relative search volumes, in order to compare variations of different search terms across regions and periods. The Menn-Kendall test was used to evaluate the overall seasonal trend of each search term and possible correlation between search terms. Results We observed a seasonality for Google search volumes for lupus-related terms. In the Northern hemisphere, relative search volumes for 'lupus' were correlated with 'relapse' (τ = 0.85; p = 0.019) and with fatigue (τ = 0.82; p = 0.003), whereas in the Southern hemisphere we observed a significant correlation between 'fatigue' and 'relapse' (τ = 0.85; p = 0.018). Similarly, a significant correlation between 'fatigue' and 'relapse' (τ = 0.70; p < 0.001) was seen also in the Northern hemisphere. Conclusion Despite the intrinsic limitations of this approach, Internet-acquired data might represent a real-time surveillance tool and an alert for healthcare systems in order to plan the most appropriate resources in specific moments with higher disease burden.

  20. Current role of rituximab in systemic lupus erythematosus.

    Science.gov (United States)

    Mok, Chi Chiu

    2015-02-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by periods of flares and remission, resulting in organ damage over time caused by persistent disease activity and treatment-related complications. Conventional therapies are not ideal in terms of efficacy and safety. Novel biological therapies are being developed to enhance therapeutic efficacy, minimize disease exacerbation and reduce toxicities. As dysregulation of B cells is the hallmark of SLE, B-cell targeted therapies are the focus of recent clinical research. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been used with success in recalcitrant lupus manifestations. However, randomized controlled trials have failed to reveal its benefit in renal and non-renal SLE when combined with conventional immunosuppressive protocols. Although heterogeneity of SLE manifestations, pitfalls in study design and the limitations of the assessment tools for various clinical end points may have contributed to the discouraging results, rituximab remains an option in patients who are refractory or intolerant to conventional therapies. Recently, a regimen consisting of rituximab and mycophenolate mofetil without oral corticosteroids was reported to be effective in lupus nephritis. While the efficacy of this regimen has to be confirmed, future controlled trials should focus on the efficacy of rituximab in refractory lupus manifestations and its synergistic effect with other immunosuppressive agents such as cyclophosphamide. In short-term randomized controlled trials, a non-significant increase in serious adverse events was observed in SLE patients treated with rituximab. Long-term safety data of rituximab in SLE, in particular the incidence of hypogammaglobulinemia and serious/opportunistic infections, have to be continuously surveyed. © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  1. The innate immune system in human systemic lupus erythematosus.

    Science.gov (United States)

    Weidenbusch, Marc; Kulkarni, Onkar P; Anders, Hans-Joachim

    2017-04-25

    Although the role of adaptive immune mechanisms, e.g. autoantibody formation and abnormal T-cell activation, has been long noted in the pathogenesis of human systemic lupus erythematosus (SLE), the role of innate immunity has been less well characterized. An intricate interplay between both innate and adaptive immune elements exists in protective anti-infective immunity as well as in detrimental autoimmunity. More recently, it has become clear that the innate immune system in this regard not only starts inflammation cascades in SLE leading to disease flares, but also continues to fuel adaptive immune responses throughout the course of the disease. This is why targeting the innate immune system offers an additional means of treating SLE. First trials assessing the efficacy of anti-type I interferon (IFN) therapy or modulators of pattern recognition receptor (PRR) signalling have been attempted. In this review, we summarize the available evidence on the role of several distinct innate immune elements, especially neutrophils and dendritic cells as well as the IFN system, as well as specific innate PRRs along with their signalling pathways. Finally, we highlight recent clinical trials in SLE addressing one or more of the aforementioned components of the innate immune system. © 2017 The Author(s). published by Portland Press Limited on behalf of the Biochemical Society.

  2. Environmental factors predicting nephritis in systemic lupus erythematosus.

    Science.gov (United States)

    McAlindon, T; Giannotta, L; Taub, N; D'Cruz, D; Hughes, G

    1993-01-01

    OBJECTIVES--To evaluate social class, ethnic origin, and various endocrine variables as potential risk factors in the development of nephritis in patients with systemic lupus erythematosus (SLE). METHODS--A cross-sectional survey was carried out of all outpatients with SLE attending the lupus Clinic of St Thomas's Hospital from March to October 1992 using retrospective survival data. The main outcome measure was the duration of SLE before the onset of nephritis. RESULTS--Two hundred and ninety six women and 11 men were studied; the male patients were excluded from the analysis. Univariate analysis showed an increased risk of nephritis in patients with SLE of West Indian origin with 54 v 19% with nephritis at five years, in patients of lower social class, in patients who did not drink alcohol, and in those with a history of fetal loss after the onset of lupus. No significant effect of the age of onset of SLE, use of oral contraceptives, normal pregnancy, or smoking was seen. Multivariate analysis showed that ethnic origin did not influence the risk of nephritis independently of social class. CONCLUSIONS--Factors associated with socioeconomic deprivation may increase disease severity in patients with SLE. PMID:8257208

  3. Pathogenesis of pregnancy complications in systemic lupus erythematosus.

    Science.gov (United States)

    Ostensen, Monika; Clowse, Megan

    2013-09-01

    In spite of the advances made in the management of pregnancies in women with systemic lupus erythematosus (SLE), maternal complications and adverse fetal outcomes still exceed the rate of pregnancy complications in the general population. An intriguing question relates to the observation that pregnancy loss, intrauterine growth restriction (IUGR), preterm birth, and preeclampsia remain major complications in SLE pregnancies, not substantially altered by improved therapy and monitoring. From the review of the recent literature on the pathogenesis of pregnancy loss, IUGR, preeclampsia, and prematurity, it appears that clinical or subclinical inflammation, presence of autoantibodies, hormonal dysfunction, and immune alterations of lupus contribute to pregnancy complications. Impairment of early placenta development leads to poor vascularization, resulting in placental ischemia and subsequent endothelial damage. Depending on the extent of the pathological process, pregnancy loss, IUGR, and preeclampsia can develop. Early recognition of pregnancy complications is desirable in order to prevent their development or to prompt intervention that improves the outcomes. Several biomarkers have been investigated for their ability to predict complications at an early stage of pregnancy. However, up to date only lupus anticoagulant has emerged as a consistent predictor for adverse pregnancy outcomes.

  4. Manifestaciones pulmonares del Lupus Eritematoso Sistémico Pulmonary manifestations of systemic lupus erithematosus

    Directory of Open Access Journals (Sweden)

    José Fernando Molina

    1991-03-01

    Full Text Available En esta revisión se describen las diversas manifestacionespulmonares del Lupus Eritematoso Sistémico; se Incluyen tanto los cuadrosrelacionados con la enfermedad (pleuritis con o sin derrame, neumonitis lúpicaaguda, enfermedad intersticlaidifusa, hipertensión pulmonar, disfunción diafragmática,atelectasia y hemorragia pulmonar como los asociados a ella (infección, edemapulmonar urémico, embolismo pulmonar, neumotórax, pseudolinfoma y sarcoidosis.Se consideran someramente aspectos clínicos, patológicos, patogénicos,diagnósticos y terapéuticos. En cuanto a los últimos se enfatizan algunasconsideraciones generales de importancia en el manejo de estos pacientes; sonellas: la necesidad de descartar ante todo la posibilidad de un proceso Infecciosoy de emplear antibióticos de amplio espectro hasta excluir1o; la de agotarrecursos hasta establecer un diagnóstico definitivo y la de recurrir a laterapia inmunosupresora una vez excluida la infección O cuando no ha habidorespuesta a los antibióticos adecuados

    The various pulmonary manifestations of Systemic Lupus Erythematosus are described in this review; it includes related (pleurisy with/without effusion, acute lupus pneumonitis, diffuse interstitial disease, pulmonary hypertension, diaphragmatic dysfunction, atelectasis, pulmonary hemorrhage as well as associated (infection, uremic pulmonary edema, pulmonary embolism, pneumothorax, pseudolymphoma, sarcoldosis, miscellaneous conditions. Clinical, pathological, pathogenic, diagnostic and therapeutic aspects are con. sidered. Emphasis is done on certain general therapeutic considerations, namely: to rule out the possibillty of an infectious process and use wide-spectrum antibiotics until certainty is acquired that it is not present; to use every available diagnostic resource until a definite diagnosis Is established

  5. The complement system in systemic lupus erythematosus: an update.

    Science.gov (United States)

    Leffler, Jonatan; Bengtsson, Anders A; Blom, Anna M

    2014-09-01

    The complement system plays a major role in the autoimmune disease, systemic lupus erythematosus (SLE). However, the role of complement in SLE is complex since it may both prevent and exacerbate the disease. In this review, we explore the latest findings in complement-focused research in SLE. C1q deficiency is the strongest genetic risk factor for SLE, although such deficiency is very rare. Various recently discovered genetic associations include mutations in the complement receptors 2 and 3 as well as complement inhibitors, the latter related to earlier onset of nephritis. Further, autoantibodies are a distinct feature of SLE that are produced as the result of an adaptive immune response and how complement can affect that response is also being reviewed. SLE generates numerous disease manifestations involving contributions from complement such as glomerulonephritis and the increased risk of thrombosis. Furthermore, since most of the complement system is present in plasma, complement is very accessible and may be suitable as biomarker for diagnosis or monitoring of disease activity. This review highlights the many roles of complement for SLE pathogenesis and how research has progressed during recent years. 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.

  6. Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system

    OpenAIRE

    Mônica Jaques Spinosa; Márcia Bandeira; Paulo Breno Noronha Liberalesso; Simone Carreiro Vieira; Loris Lady Janz Jr; Eliane Gomes de Sá; Alfredo Löhr Jr.

    2007-01-01

    OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7%): seizures (17 / 36.2%), i...

  7. Lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus: report of 2 cases and review of literature.

    Science.gov (United States)

    Erkan, D; Bateman, H; Lockshin, M D

    1999-01-01

    We describe two patients whose initial presentation of systemic lupus erythematosus (SLE) was accompanied by haemorrhagic episodes and significant coagulopathy. Further investigation demonstrated positive lupus anticoagulant and decreased Factor II (prothrombin) activity. Both patients were diagnosed with lupus anticoagulant-hypoprothrombinemia syndrome (LAC-HPS) as a result of non-neutralizing antibodies directed against Factor II. LAC-HPS is a rare clinical entity that can occur in association with SLE, transient viral infections, drug reactions or even in healthy individuals. Mixing studies, which can be affected by other coagulation factor inhibitors, play an important role in the diagnosis of LAC-HPS. Factor VII level was decreased in the second patient, a finding that has not previously been reported in association with SLE. In both patients, bleeding stopped promptly and coagulation studies improved significantly with high dose corticosteroids. We discuss the pathogenesis, diagnosis and management of LAC-HPS in patients with SLE.

  8. Incidence of Systemic Lupus Erythematosus and Lupus Nephritis in Denmark: A Nationwide Cohort Study.

    Science.gov (United States)

    Hermansen, Marie-Louise F; Lindhardsen, Jesper; Torp-Pedersen, Christian; Faurschou, Mikkel; Jacobsen, Søren

    2016-07-01

    To determine the incidence of systemic lupus erythematosus (SLE) and SLE with concomitant or subsequent lupus nephritis (LN) in Denmark during 1995-2011, using data from the Danish National Patient Registry (NPR). To assess the incidence of SLE, we identified all persons aged ≥ 18 years in the NPR with at least 1 International Classification of Diseases, 10th ed (ICD-10) code of SLE and at least 365 days of followup under this diagnosis. Identification of LN cases was based on fulfillment of these criteria and ≥ 1 registration under an ICD-10 code of nephritis concomitantly with or after first SLE registration. The overall annual incidence rate per 100,000 for SLE was 2.35 (95% CI 2.24-2.49); 0.69 (95% CI 0.60-0.78) for men and 3.96 (95% CI 3.75-4.17) for women. For LN, the mean annual incidence rate per 100,000 was estimated to be 0.45 (95% CI 0.38-0.53); 0.20 (95% CI 0.13-0.28) for men and 0.69 (95% CI 0.57-0.83) for women. The differences in SLE incidence rates between sexes decreased by age, and the incidence did not differ between men and women after the age of 60 years for LN. The estimated incidences showed no trends by calendar time. Estimated overall point prevalence (December 31, 2011) per 100,000 was 45.2 (95% CI 43.3-47.4) and 6.4 (95% CI 5.7-7.2) for SLE and LN, respectively. Our Danish population-based data showed a stable incidence of SLE and LN. As expected, we found higher incidence rates among women than among men, particularly in younger persons.

  9. Experience of long-term belimumab use in patients with systemic lupus erythematosus (a case report

    Directory of Open Access Journals (Sweden)

    Natalia Gennadyevna Klyukvina

    2013-01-01

    Full Text Available In the past years considerable progress has been made in the treatment of systemic lupus erythe-matosus; however, not all questions have been answered. The range of medications has substan-tially increased. The paper describes a case of the long-term use of the new genetically engineered agent belimumab in a patient with systemic lupus erythematosus.

  10. Mannose-binding lectin polymorphisms and susceptibility to infection in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Garred, P; Madsen, H O; Halberg, P

    1999-01-01

    To determine whether variant alleles in the coding portion of the mannose-binding lectin (MBL) gene are associated with increased susceptibility to systemic lupus erythematosus (SLE) and concomitant infections.......To determine whether variant alleles in the coding portion of the mannose-binding lectin (MBL) gene are associated with increased susceptibility to systemic lupus erythematosus (SLE) and concomitant infections....

  11. Exercise training in childhood-onset systemic lupus erythematosus: a controlled randomized trial

    OpenAIRE

    Prado, Danilo M; Benatti, Fabiana B; de Sá-Pinto, Ana L; Hayashi, Ana P; Gualano, Bruno; Pereira, Rosa M; Sallum, Adriana M; Bonfá, Eloisa; Silva, Clovis A; Roschel, Hamilton

    2013-01-01

    Abstract Introduction Exercise training has emerged as a promising therapeutic strategy to counteract physical dysfunction in adult systemic lupus erythematosus. However, no longitudinal studies have evaluated the effects of an exercise training program in childhood-onset systemic lupus erythematosus (C-SLE) patients. The objective was to evaluate the safety and the efficacy of a super...

  12. Retinal nerve fiber layer thickness and neuropsychiatric manifestations in systemic lupus erythematosus.

    Science.gov (United States)

    Shulman, S; Shorer, R; Wollman, J; Dotan, G; Paran, D

    2017-11-01

    Background Cognitive impairment is frequent in systemic lupus erythematosus. Atrophy of the corpus callosum and hippocampus have been reported in patients with systemic lupus erythematosus, and diffusion tensor imaging studies have shown impaired white matter integrity, suggesting that white matter damage in systemic lupus erythematosus may underlie the cognitive impairment as well as other neuropsychiatric systemic lupus erythematosus manifestations. Retinal nerve fiber layer thickness, as assessed by optical coherence tomography, has been suggested as a biomarker for white matter damage in neurologic disorders such as multiple sclerosis, Alzheimer's disease and Parkinson's disease. Retinal nerve fiber layer thinning may occur early, even in patients with mild clinical symptoms. Aim The objective of this study was to assess the association of retinal nerve fiber layer thickness, as a biomarker of white matter damage in systemic lupus erythematosus patients, with neuropsychiatric systemic lupus erythematosus manifestations, including cognitive impairment. Methods Twenty-one consecutive patients with systemic lupus erythematosus underwent neuropsychological testing using a validated computerized battery of tests as well as the Rey-Auditory verbal learning test. All 21 patients, as well as 11 healthy, age matched controls, underwent optical coherence tomography testing to assess retinal nerve fiber layer thickness. Correlations between retinal nerve fiber layer thickness and results in eight cognitive domains assessed by the computerized battery of tests as well as the Rey-Auditory verbal learning test were assessed in patients with systemic lupus erythematosus, with and without neuropsychiatric systemic lupus erythematosus, and compared to retinal nerve fiber layer thickness in healthy controls. Results No statistically significant correlation was found between retinal nerve fiber layer thickness in patients with systemic lupus erythematosus as compared to healthy

  13. Multiple myeloma complicating the course of seronegative systemic lupus erythematosus.

    Science.gov (United States)

    Jordan, E; Burnstein, S L; Calabro, J J; Henderson, E S

    1978-03-01

    A patient with a 20-year history of clinical systemic lupus erythematosus (SLE) who later developed multiple myeloma is described. SLE was diagnosed on the basis of a butterfly rash, photosensitivity, nondeforming arthritis, pleuropericarditis, and alopecia. However, the patient has never had LE cells, antinuclear antibody, or depressed complement. The patient was treated with intermittent courses of corticosteroids over a 20-year period with good results. Multiple myeloma, diagnosed by bone marrow biopsy, has responded favorably to therapy with L-phenylalanine mustard and prednisone.

  14. Posterior reversible encephalopathy syndrome due to seronegative systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Sawan Verma

    2014-09-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a neurotoxic state coupled with a unique computed tomography or magnetic resonance imaging (MRI appearance. Recognized in the setting of a number of complex conditions (preeclampsia/eclampsia, allogeneic bone marrow transplantation, organ transplantation, autoimmune disease and high-dose chemotherapy in the imaging, clinical and laboratory features of this toxic state are becoming better elucidated. We are presenting a case of PRES due to seronegative systemic lupus erythematosus, with MRI findings of diffuse vasogenic edema.

  15. Systemic lupus erythematosus presenting as congenital nephrotic syndrome.

    Science.gov (United States)

    Dudley, J; Fenton, T; Unsworth, J; Chambers, T; MacIver, A; Tizard, J

    1996-12-01

    A male Caucasian infant developed nephrotic syndrome at 10 weeks of age. He had high titres of antinuclear antibody (ANA) and anti-double-stranded DNA antibody, with hypocomplementaemia, antiplatelet antibodies and anticardiolipin antibodies. There were no detectable antibodies to extractable nuclear antigens (ENA). His mother was consistently seronegative for anti-ENA (anti-Ro) antibodies and ANA. He developed severe, progressive multisystem involvement, however renal function has remained stable. Immunosuppression has been the mainstay of therapy in this rare presentation of systemic lupus erythematosus.

  16. Neurological manifestations of children with systemic lupus erythematosus.

    Science.gov (United States)

    Loh, W F; Hussain, I M; Soffiah, A; Lim, Y N

    2000-12-01

    In a cross-sectional study of 21 children with Systemic Lupus Erythematosus, 15 (71%) were found to have neuropsychiatric manifestations. The most common finding was generalised seizures (42.8%) followed by encephalopathy (19%) and hallucinations (19%). One child (4.76%) had hemichorea. In 3 children neurological manifestations were the first symptom of SLE. Computerised Axial Tomograms (CAT scans) showed cerebral atrophy in 7 of 12 scans available for review. Ten children had abnormal EEGs. Although none of the children had clinical evidence of a peripheral neuropathy, 8 had neurophysiological evidence of a neuropathy. One child died of intracranial haemorrhage. Six children had residual neuropsychiatric sequalae.

  17. Mortality patterns in Malaysian systemic lupus erythematosus patients.

    Science.gov (United States)

    Yeap, S S; Chow, S K; Manivasagar, M; Veerapen, K; Wang, F

    2001-09-01

    A retrospective analysis of the case records of 494 systemic lupus erythematosus (SLE) patients under follow-up at University Hospital, Kuala Lumpur during 1976-1990 was performed. Overall mortality was 20.2% (100 patients). The causes of death were infection (30%), renal (15%), respiratory (14%), neurological (5%), cardiovascular (7%), other causes (2%) and unknown (27%). Active SLE was a contributing factor in 19% of the deaths. The patients who died had significantly more renal disease, neurological disease, serositis or thrombocytopenia by the end of the first year of disease compared to the survivors. As in other series, infection and active SLE remain important causes of death.

  18. A complicated multisystem flare of systemic lupus erythematosus during pregnancy.

    Science.gov (United States)

    Webster, Philip; Nelson-Piercy, Catherine; Lightstone, Liz

    2017-02-08

    We report a case of systemic lupus erythematosus (SLE) in a young woman who became pregnant amid a severe flare. She continued to have active disease in the face of aggressive treatments complicated by several side effects of immunosuppressive drugs including recurrent sepsis and gestational diabetes. Her fetus was at risk for congenital heart block during the second and third trimesters. Despite an extremely guarded prognosis, she delivered a healthy baby girl. This case highlights the complexities of SLE management during pregnancy. We discuss the therapeutic options available in pregnancy, and highlight the importance of cross-specialty multidisciplinary care in these women. 2017 BMJ Publishing Group Ltd.

  19. Alveolar hemorrhage as the initial presentation of systemic lupus erythematosus.

    Science.gov (United States)

    de Holanda, Bruna A; Barreto, Isabela G Menna; de Araujo, Isadora S Gomes; de Araujo, Daniel B

    2016-01-01

    Alveolar hemorrhage (AH) is a rare syndrome that can often occur in autoimmune diseases, blood clotting disorders, infection or by acute inhalation injury, presenting rapid evolution and high mortality, especially with late diagnosis and treatment. Among the autoimmune diseases, there are reported cases in patients with primary antiphospholipid syndrome (PAPS), vasculitis and systemic lupus erythematosus (SLE). An early diagnosis is an essential tool in the successful management of this complication, requiring aggressive treatment based on vigorous immunosuppression and broad-spectrum antibiotic. We describe here a case of alveolar hemorrhage associated with glomerulonephritis as the open presentation in a patient with SLE.

  20. Chronic meningitis in systemic lupus erythematosus: An unusual etiology

    Directory of Open Access Journals (Sweden)

    Anu Gupta

    2014-01-01

    Full Text Available Chronic aseptic meningitis is a rare manifestation of systemic lupus erythematosus (SLE. Apart from immunological causes and drugs, the aseptic meningitis group can include some unidentified viral infections that cannot be detected by routine microbiological testing. It is imperative to do complete cerebrospinal fluid (CSF workup before implicating the symptoms to disease activity or drugs, as untreated infections cause significant mortality in SLE. We present a case of young female with SLE who presented with chronic meningitis of an uncommon etiology.

  1. Disseminated tuberculosis masquerading as a presentation of systemic lupus erythematosus.

    Science.gov (United States)

    Li, Justin C-H; Fong, Warren; Wijaya, Limin; Leung, Ying Y

    2018-01-01

    Tuberculosis (TB) infection is the endemic in Asia-Pacific region. Miliary TB is a disseminated form which may present similarly as autoimmune conditions. Here we describe a 17-year-old girl who had miliary TB with manifestations mimicking new-onset systemic lupus erythematosus (SLE) including oral ulcers, serositis, cytopenia, proteinuria and raised autoantibody titers. Complex associations between SLE and TB are highlighted. High index of clinical suspicion for TB infection is needed upon presentations resembling immune diseases like SLE. © 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

  2. Association of smoking with cutaneous manifestations in systemic lupus erythematosus.

    Science.gov (United States)

    Bourré-Tessier, Josiane; Peschken, Christine A; Bernatsky, Sasha; Joseph, Lawrence; Clarke, Ann E; Fortin, Paul R; Hitchon, Carol; Mittoo, Shikha; Smith, C Douglas; Zummer, Michel; Pope, Janet; Tucker, Lori; Hudson, Marie; Arbillaga, Hector; Esdaile, John; Silverman, Earl; Chédeville, Gaelle; Huber, Adam M; Belisle, Patrick; Pineau, Christian A

    2013-08-01

    To examine the association between smoking and cutaneous involvement in systemic lupus erythematosus (SLE). We analyzed data from a multicenter Canadian SLE cohort. Mucocutaneous involvement was recorded at the most recent visit using the Systemic Lupus Erythematosus Disease Activity Index 2000 Update (rash, alopecia, and oral ulcers), Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index (alopecia, extensive scarring, and skin ulceration), and the ACR revised criteria for SLE (malar rash, discoid rash, photosensitivity, and mucosal involvement). Multivariate logistic regression models were used to estimate the independent association between mucocutaneous involvement and cigarette smoking, age, sex, ethnicity, lupus duration, medications, and laboratory data. In our cohort of 1,346 patients (91.0% women), the mean ± SD age was 47.1 ± 14.3 years and the mean ± SD disease duration was 13.2 ± 10.0 years. In total, 41.2% of patients were ever smokers, 14.0% current smokers, and 27.1% past smokers. Active mucocutaneous manifestations occurred in 28.4% of patients; cutaneous damage occurred in 15.4%. Regarding the ACR criteria, malar rash was noted in 59.5%, discoid rash in 16.9%, and photosensitivity in 55.7% of patients. In the multivariate analysis, current smoking was associated with active SLE rash (odds ratio [OR] 1.63 [95% confidence interval (95% CI) 1.07, 2.48]). Having ever smoked was associated with ACR discoid rash (OR 2.36 [95% CI 1.69, 3.29]) and photosensitivity (OR 1.47 [95% CI 1.11, 1.95]), and with the ACR total cutaneous score (OR 1.50 [95% CI 1.22, 1.85]). We did not detect any associations between previous smoking and active cutaneous manifestations. No association was found between smoking and cutaneous damage or mucosal ulcers. No interaction was seen between smoking and antimalarials. Current smoking is associated with active SLE rash, and ever smoking with the ACR total cutaneous score. This

  3. [Biological causes of depression in Systemic Lupus Erythematosus].

    Science.gov (United States)

    Braga, J; Campar, A

    2014-01-01

    The high prevalence of depression in patients with systemic lupus erythematosus (SLE) may result from the psychosocial impact of this chronic disease as well as from a lesion of the central nervous system (CNS). Recently, the biological basis of depression in SLE has been confirmed, under the influence of several factors, which will be reviewed here. Published evidence points to the participation of biochemical and neurophysiological changes, induced by cytokines, in the development of neuropsychiatric symptoms. Through activation of the enzyme indoleamine 2,3-dioxygenase (IDO), the alteration of neurotransmitters' bioavailability, the modification of neuroplasticity and neurogenesis and the overstimulation of certain neural circuits, cytokines are capable of causing mood swings and depression. On the other hand, associated with the immune deregulation, the Hypothalamic-Pituitary-Adrenal (HPA) axis dysfunction correlates with neurophysiological changes involved in depression. Moreover, cerebro-reactive autoantibodies present in the cerebrospinal fluid (CSF), such as anti-N-methyl-D-aspartate(NMDA) and anti-ribosomal P, can cause significant damage to neurons in brain areas which are relevant to humor and behavior, potentially leading to depressive symptoms. In neuroanatomical terms, brain lesions in areas of the limbic system present in lupus patients, despite their unclear etiology, suggest impairment of cerebral achievement in emotional and behavioral functions. In summary, several biological changes are able to cause depression in SLE and their identification is essential to the management of the disease.

  4. Study of Flare Assessment in Systemic Lupus Erythematosus Based on Paper Patients

    DEFF Research Database (Denmark)

    Isenberg, D; Sturgess, J; Allen, E

    2018-01-01

    OBJECTIVE: To determine the level of agreement of disease flare severity (distinguishing severe, moderate, and mild flare and persistent disease activity) in a large paper-patient exercise involving 988 individual cases of systemic lupus erythematosus. METHODS: A total of 988 individual lupus cas...

  5. Study of Flare Assessment in Systemic Lupus Erythematosus Based on Paper Patients

    NARCIS (Netherlands)

    Isenberg, D.; Sturgess, J.; Allen, E.; Aranow, C.; Askanase, A.; Sang-Cheol, B.; Bernatsky, S.; Bruce, I.; Buyon, J.; Cervera, R.; Clarke, A.; Dooley, Mary Anne; Fortin, P.; Ginzler, E.; Gladman, D.; Hanly, J.; Inanc, M.; Jacobsen, S.; Kamen, D.; Khamashta, M.; Lim, S.; Manzi, S.; Nived, O.; Peschken, C.; Petri, M.; Kalunian, K.; Rahman, A.; Ramsey-Goldman, R.; Romero-Diaz, J.; Ruiz-Irastorza, G.; Sanchez-Guerrero, J.; Steinsson, K.; Sturfelt, G.; Urowitz, M.; van Vollenhoven, R.; Wallace, D. J.; Zoma, A.; Merrill, J.; Gordon, C.

    2018-01-01

    To determine the level of agreement of disease flare severity (distinguishing severe, moderate, and mild flare and persistent disease activity) in a large paper-patient exercise involving 988 individual cases of systemic lupus erythematosus. A total of 988 individual lupus case histories were

  6. Plasma ficolin levels and risk of nephritis in Danish patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Tanha, Nima; Pilely, Katrine; Faurschou, Mikkel

    2017-01-01

    Given the scavenging properties of ficolins, we hypothesized that variation in the plasma concentrations of the three ficolins may be associated with development of lupus nephritis (LN), type of LN, end-stage renal disease (ESRD), and/or mortality among patients with systemic lupus erythematosus...

  7. [Treatment of systemic lupus erythematosus: myths, certainties and doubts].

    Science.gov (United States)

    Ruiz-Irastorza, Guillermo; Danza, Alvaro; Khamashta, Munther

    2013-12-21

    Systemic lupus erythematosus (SLE) is a complex disease with different clinical forms of presentation, including a wide range of severity and organic involvement. Such circumstance, along with the fact of the uncommon nature of the disease and the absence of clinically representative response criteria, make it difficult to design controlled clinical trials in SLE patients. As a result, observational studies have a special relevance, being a source of valuable information of SLE prognosis and outcome as well as of the efficacy and adverse effects of the different therapies. Herein we update some of the main treatments used in SLE. Steroids may have more risks than benefits if used at high doses. New mechanisms of action have been described, supporting the use of lower doses, possibly with the same efficacy and less adverse effects. Intravenous pulses of cyclophosphamide still have a role in the treatment of proliferative lupus nephritis and other serious SLE manifestations. Mycophenolate mofetil has shown its efficacy both as induction and maintenance therapy of selected cases of lupus nephritis. Biological therapies have emerged as new promising options. Although clinical trials have not confirmed a clear superiority of rituximab in SLE, observational studies have shown good response rates in severe SLE manifestations or refractory forms. Belimumab has recently been added to the therapeutic armamentarium of SLE; although its place in clinical practice is not well-defined, it may be recommended in active patients with no response or good tolerance to standard therapies. Hydroxichloroquine improves survival, decreases the risk of thrombosis and flares and is safe in pregnancy, and should be considered the baseline therapy in most SLE patients. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  8. Establishing the Jamaica lupus registry: report of patients with systemic lupus erythematosus attending a major referral hospital in Jamaica.

    Science.gov (United States)

    Soyibo, A K; DeCuelaer, K; Miller, R K; Smith, R; Maloney, K; Barton, E N

    2012-06-01

    Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by multisystem microvascular inflammation with the generation of autoantibodies. There are reports on demographic data and clinical manifestation of lupus in the United States of America and some other developed countries. There is a single study that has reported on the clinical and immunological features of SLE patients in Jamaica and another that reported that the prevalence of SLE in Jamaica was 5-17/100,000 in 1979. A Jamaican lupus registry was established in 2008 at the Department of Medicine, The University of the West Indies. Data were collected using patient records and interview of patients fulfilling the American College of Rheumatology revised diagnostic criteria for SLE. Information on demographics, presence of diagnostic criteria for SLE, presence of complications and other clinical parameters were collected. There were a total of 107 patients that met the criteria for diagnosis of SLE at the referral centre, 96.3% of them female. Positive ANA (90.7%), arthritis (70.0%), malar rash (53.5%) and a positive dsDNA (40.1%) were the more frequent manifestations and diagnostic indices of the disease. Up to 41.7% of the SLE population suffered some form of complication. The initiation of a lupus registry has allowed for reporting ofpreliminary demographic, clinical and serological data and identifying of disease burden.

  9. Immunological aspects of biopsy-proven lupus nephritis in Bahraini patients with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Eman M Farid

    2013-01-01

    Full Text Available Lupus nephritis (LN is a frequent and potentially serious complication of systemic lupus erythematosus (SLE that may influence morbidity and mortality. Immunological investigations are aiding tools to the kidney biopsy findings in early diagnosis, in addition to monitoring the effect of therapy. The aim of the present study is to highlight the role of these investigations in a group of Bahraini patients and to determine whether there is any positive association between these findings and the outcome of LN. The current study is a retrospective case-control study of randomly selected 88 SLE patients, 44 with biopsyproven LN and 44 without, acting as controls. All renal biopsies performed during the period from 1996 to 2012 were classified according to the World Health Organization classification. Immunological investigations analyzed are: Antinuclear antibodies (ANA, anti-ds DNA, anti-ENA, anti-cardiolipin antibodies (abs and complement components C3, C4. Human leukocyte antigen (HLA typing class II was performed on selected cases. All patients had positive ANA (100%. A significantly high frequency of anti-Smith abs among the non-LN group (43.18% compared with the LN group (18.18% was found (P <0.001. On the other hand, the anti-Ro/SSA abs in the non-LN group was also found at a statistically higher frequency (20.45% compared with that in the LN group (4.54% (P <0.01. Anti-ds-DNA abs were found to be higher in the LN group (84.09% compared with the non-LN group (70.45%, but the difference was not statistically significant (P = 0.082. There was a positive association of ANA positivity and low C3 and or C4 in the studied group. In our study, 88.2% of the HLA typed patients had HLADR2, DR3 or both. In conclusion, in our Arabic Bahraini SLE patients, the presence of anti-Smith, anti-Ro/SSA and anti-RNP antibodies and the absence of anti-dsDNA antibodies are independent predictive markers for renal involvement. However, more prospective studies with a

  10. Early Lupus Project - A multicentre Italian study on systemic lupus erythematosus of recent onset.

    Science.gov (United States)

    Sebastiani, G D; Prevete, I; Piga, M; Iuliano, A; Bettio, S; Bortoluzzi, A; Coladonato, L; Tani, C; Spinelli, F R; Fineschi, I; Mathieu, A

    2015-10-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a high degree of variability at onset that is problematic for a correct and prompt diagnosis. We undertook this project with the purpose of collecting an inception cohort of Italian patients with recent-onset SLE, in order to obtain information on the main clinical and serological characteristics at the beginning of the disease. In this first report we describe the characteristics of this cohort at study entry. All patients with a diagnosis of SLE (1997 ACR criteria) and a disease duration less than 12 months were consecutively enrolled between 1 January 2012 and 31 December 2013 in a multicentre prospective study. Information on clinical and serological characteristics at study entry and then every six months was collected into a specific electronic database. Statistical analysis was performed by means of the Openstat program. Among 122 patients enrolled (103 F) 94.3% were Caucasians. Mean age (SD) of patients at study entry was 37.3 (14.3) years, mean age at disease onset was 34.8 (14.3) years, mean age at diagnosis was 36.9 (14.3) years, and mean disease duration was 2.9 (3.9) months. The frequency of the manifestations included in the 1997 ACR criteria was as follows: ANA 97.5%, immunologic disorders (anti-dsDNA, anti-Sm, antiphospholipid antibodies) 85.2%, arthritis 61.8%, haematologic disorders 55.7%, malar rash 31.1%, photosensitivity 29.5%, serositis 27%, renal disorders 27%, oral/nasal ulcers 11.5%, neurologic disorders 8.2%, and discoid rash 5.7%. The cumulative frequency of mucocutaneous symptoms was 77.8%. At enrolment, autoantibody frequency was: ANA 100%, anti-dsDNA 83.6%, anti-SSA 28%, anticardiolipin 24.5%, anti-nRNP 20.4%, anti-beta2GPI 17.2%, lupus anticoagulant 16.3%, anti-Sm 16%, and anti-SSB 13.1%. In this paper we describe the main clinical and serological characteristics of an Italian inception cohort of patients with recent-onset SLE. At disease onset, mucocutaneous

  11. Activation of Type I Interferon Pathway in Systemic Lupus Erythematosus: Association with Distinct Clinical Phenotypes

    Science.gov (United States)

    Karageorgas, Theophanis P.; Tseronis, Dimitrios D.; Mavragani, Clio P.

    2011-01-01

    Growing evidence over the last few years suggests a central role of type I IFN pathway in the pathogenesis of systemic autoimmune disorders. Data from clinical and genetic studies in patients with systemic lupus erythematosus (SLE) and lupus-prone mouse models, indicates that the type I interferon system may play a pivotal role in the pathogenesis of several lupus and associated clinical features, such as nephritis, neuropsychiatric and cutaneous lupus, premature atherosclerosis as well as lupus-specific autoantibodies particularly against ribonucleoproteins. In the current paper, our aim is to summarize the latest findings supporting the association of type I IFN pathway with specific clinical manifestations in the setting of SLE providing insights on the potential use of type I IFN as a therapeutic target. PMID:22162633

  12. Role of the Fc gamma receptor IIa polymorphism in susceptibility to systemic lupus erythematosus and lupus nephritis - A meta-analysis

    NARCIS (Netherlands)

    Karassa, FB; Trikalinos, TA; Ioannidis, JPA

    Objective. To assess the impact of the Fcgamma receptor type IIa (FcgammaRIIa)-R/H131 polymorphism on the risk for systemic lupus erythematosus (SLE) and development of lupus nephritis. Methods. A meta-analysis was performed based on the Medline and Embase databases (last retrieval August 2001),

  13. Fc gamma receptors in the initiation and progression of systemic lupus erythematosus

    NARCIS (Netherlands)

    Reefman, E; Dijstelbloem, HM; Limburg, PC; Kallenberg, CGM; Bijl, M

    2003-01-01

    Systemic lupus erythematosus, a systemic autoimmune disorder, is characterized by the production of autoantibodies to nuclear constituents and inflammatory lesions in multiple organ systems. Although the pathogenesis of the disease is largely unknown, recent studies have suggested that disturbances

  14. Estrogen in Cardiovascular Disease during Systemic Lupus Erythematosus

    Science.gov (United States)

    Gilbert, Emily L.; Ryan, Michael J.

    2015-01-01

    Purpose Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that disproportionately affects women during their childbearing years. Cardiovascular disease is the leading cause of mortality in this patient population at an age when women often have low cardiovascular risk. Hypertension is a major cardiovascular disease risk factor, and its prevalence is markedly increased in women with SLE. Estrogen has traditionally been implicated in SLE disease progression because of the prevalence of the disease in women; however, its role in cardiovascular risk factors such as hypertension is unclear. The objective of this review is to discuss evidence for the role of estrogen in both human and murine SLE with emphasis on the effect of estrogen on cardiovascular risk factors, including hypertension. Methods PubMed was used to search for articles with terms related to estradiol and SLE. The references of retrieved publications were also reviewed. Findings The potential permissive role of estrogen in SLE development is supported by studies from experimental animal models of lupus in which early removal of estrogen or its effects leads to attenuation of SLE disease parameters, including autoantibody production and renal injury. However, data about the role of estrogens in human SLE are much less clear, with most studies not reaching firm conclusions about positive or negative outcomes after hormonal manipulations involving estrogen during SLE (ie, oral contraceptives, hormone therapy). Significant gaps in knowledge remain about the effect of estrogen on cardiovascular risk factors during SLE. Studies in women with SLE were not designed to determine the effect of estrogen or hormone therapy on blood pressure even though hypertension is highly prevalent, and risk of premature ovarian failure could necessitate use of hormone therapy in women with SLE. Recent evidence from an experimental animal model of lupus found that estrogen may protect against

  15. Bone mineral density in juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Castro T.C.M.

    2002-01-01

    Full Text Available We evaluated spine bone mineral density (BMD in Brazilian children with juvenile systemic lupus erythematosus (JSLE in order to detect potential predictors of reduction in bone mass. A cross-sectional study of BMD at the lumbar spine level (L2-L4 was conducted on 16 female JSLE patients aged 6-17 years. Thirty-two age-matched healthy girls were used as control. BMD at the lumbar spine was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in patients and controls. Disease duration, mean daily steroid doses, mean cumulative steroid doses and JSLE activity measured by the systemic lupus erythematosus disease activity index (SLEDAI were determined for all JSLE patients based on their medical charts. All parameters were used as potential determinant factors for bone loss. Lumbar BMD tended to be lower in the JSLE patients, however, this difference was not statistically significant (P = 0.10. No significant correlation was observed in JSLE girls between BMD and age, height, Tanner stage, disease duration, corticosteroid use or disease activity. We found a weak correlation between BMD and weight (r = 0.672. In the JSLE group we found no significant parameters to correlate with reduced bone mass. Disease activity and mean cumulative steroid doses were not related to BMD values. We did not observe reduced bone mass in female JSLE.

  16. The blood-brain barrier in systemic lupus erythematosus.

    Science.gov (United States)

    Abbott, N J; Mendonça, L L F; Dolman, D E M

    2003-01-01

    Central nervous system (CNS) involvement may occur in 20-70% of systemic lupus erythematosus (SLE) patients where neurological symptoms are overt; this is termed neuropsychiatric lupus or NPSLE. This review summarizes evidence that damage to the brain endothelium forming the blood-brain barrier (BBB) is a contributory factor in NPSLE. The normal CNS is protected by blood-tissue barriers at three sites, the brain endothelium (BBB), the choroid plexus epithelium (blood-CSF barrier) and the arachnoid epithelium. The tight junctions of the barrier layers severely restrict entry of plasma constituents including proteins, so that the CSF and brain interstitial fluid contain low levels of protein. Methods for diagnosing BBB damage include imaging (CT, MRI) using contrast agents, and analysing protein content and profiles of CSF Changes in the albumin quotient Qalbumin show evidence for barrier damage, while changes in the immunoglobulin (Ig) index can indicate intrathecal antibody production. However, BBB damage may be transient, and hence undetected or underestimated. Few mechanistic studies exist, but the two main candidate mechanisms for BBB damage are microthrombi in cerebral vessels leading to ischaemia, and immune-mediated attack and activation of the endothelium leading to local cytokine production. Both can result in barrier breakdown. Neurological syndromes could then be secondary to damage to the BBB. The implications for treatment of NPSLE are discussed.

  17. The complex immunogenetic basis of systemic lupus erythematosus.

    Science.gov (United States)

    Castro, Jesús; Balada, Eva; Ordi-Ros, Josep; Vilardell-Tarrés, Miquel

    2008-05-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology with a complex genetic basis that includes many susceptibility genes on multiple chromosomes. As complex human diseases like SLE involve multiple, interacting genetic and environmental determinants, identifying genes for complex traits is challenging and has had limited success so far. Several key approaches that are necessary to identify disease susceptibility genes in common diseases such as SLE are now available. Collectively, these approaches will allow the prioritization of candidate genes based on available knowledge of map position and biologic relevance. They will also allow us to obtain the genomic structure of these genes as well as to study sequence variants that will facilitate the identification of genes that are important in the development and expression (severity) of lupus and associated phenotypes. Although it is still a labor-intensive and expensive project to identify susceptibility genes in common diseases such as SLE, the new techniques that are now being used will undoubtedly improve gene mapping in such a kind of diseases. In this review we highlight the current findings in the genetics of human SLE after using these approaches.

  18. Aconitine: A potential novel treatment for systemic lupus erythematosus.

    Science.gov (United States)

    Li, Xiaodong; Gu, Liwei; Yang, Lan; Zhang, Dong; Shen, Jianying

    2017-03-01

    Aconitum plants have been widely used in China for thousands of years. Recent evidences indicate that aconitine, the main active ingredient of Aconitum, has immunomodulatory properties that might be useful for treating autoimmune diseases, such as rheumatoid arthritis. In this study, we conducted a pilot study to explore the effect and mechanisms of aconitine on the treatment of systemic lupus erythematosus. A pristane-induced murine model was used. The pristane-induced mice were treated with aconitine (25, 75 μg kg-1 d-1, po) for 9 weeks. Every three weeks, proteinuria was detected to monitor the kidney damage and blood was collected to measure serum levels of autoantibodies, besides the kidney pathological examination. The major B cell activating factor and major pro-inflammatory mediators, PGE2, IL-17a and IL-6, were also detected. We found that aconitine significantly improved the mouse health, decreased the elevated blood leukocyte counts, reduced the serum level of anti-double-stranded DNA (anti-dsDNA) antibody, greatly ameliorated renal histopathologic damage and reduced IgG deposit in glomerular. Furtherly, the levels of PGE2, IL-17a and IL-6, were found to have decreased in aconitine treated mice. We have demonstrated that aconitine can inhibit the progression of disease and ameliorate the pathologic lesion of systemic lupus erythematosus. Copyright © 2017 The Authors. Production and hosting by Elsevier B.V. All rights reserved.

  19. Pleural and pulmonary involvement in systemic lupus erythematosus.

    Science.gov (United States)

    Torre, Olga; Harari, Sergio

    2011-01-01

    Systemic lupus erythematosus (SLE) is a rare complex autoimmune disease with a multisystem involvement. The clinical manifestations of this disease include an erythematous rash, oral ulcers, polyarthralgia, nonerosive arthritis, polyserositis, hematologic, renal, neurologic, pulmonary and cardiac abnormalties. The involvement of the respiratory system is frequent. Pleuro-pulmonary manifestations are present in almost half of the patients during the disease course and may be the presenting symptoms in 4-5% of patients with SLE. Complications directly associated to the disease include pleuritis with or without pleural effusion, alveolitis, interstitial lung disease, lupus pneumonitis, pulmonary hemorrhage, pulmonary arterial hypertension, and pulmonary thromboembolic disease. Complications due to secondary causes include pleuro-pulmonary manifestations of cardiac and renal failure, atelectasis due to diaphragmatic dysfunction, opportunistic pneumonia, and drug toxicity. The prevalence, clinical presentation, prognosis and response to treatment vary, depending on the pattern of involvement. As with other connective tissue diseases, early and specific therapeutic intervention may be indicated for many of these pleuro-pulmonary manifestations. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  20. Sex differences in monocyte activation in systemic lupus erythematosus (SLE.

    Directory of Open Access Journals (Sweden)

    Wei Jiang

    Full Text Available TLR7/8 and TLR9 signaling pathways have been extensively studied in systemic lupus erythematosus (SLE as possible mediators of disease. Monocytes are a major source of pro-inflammatory cytokines and are understudied in SLE. In the current project, we investigated sex differences in monocyte activation and its implications in SLE disease pathogenesis.Human blood samples from 27 healthy male controls, 32 healthy female controls, and 25 female patients with SLE matched for age and race were studied. Monocyte activation was tested by flow cytometry and ELISA, including subset proportions, CD14, CD80 and CD86 expression, the percentage of IL-6-producing monocytes, plasma levels of sCD14 and IL-6, and urine levels of creatinine.Monocytes were significantly more activated in women compared to men and in patients with SLE compared to controls in vivo. We observed increased proportions of non-classic monocytes, decreased proportions of classic monocytes, elevated levels of plasma sCD14 as well as reduced surface expression of CD14 on monocytes comparing women to men and lupus patients to controls. Plasma levels of IL-6 were positively related to sCD14 and serum creatinine.Monocyte activation and TLR4 responsiveness are altered in women compared to men and in patients with SLE compared to controls. These sex differences may allow persistent systemic inflammation and resultant enhanced SLE susceptibility.

  1. Fatal evolution of systemic lupus erythematosus associated with Crohn's disease

    Directory of Open Access Journals (Sweden)

    CHEBLI Júlio M. Fonseca

    2000-01-01

    Full Text Available The authors describe the case of a young Brazilian woman who was treated of ileocolonic Crohn's disease sparing rectum, as confirmed by colonoscopy and histopathological examination. After a 4-year course of sulfasalazine treatment, she presented with skin facial lesions in vespertilio, fever, arthralgias and high titers of anti-ANA and LE cells. A sulfasalazine-induced lupus syndrome was diagnosed, because after sulfasalazine withdrawal and a short course of prednisone, the clinical symptoms disappeared and the laboratory tests returned to normal. Mesalazine 3 g/day was started and the patient remained well for the next 3 years, when she was again admitted with fever, weakness, arthralgias, diplopy, strabismus and hypoaesthesia in both hands and feet, microhematuria, haematic casts, hypocomplementemia and high titers of autoimmune antibodies. A diagnosis of associated systemic lupus erythematosus was made. Although a pulsotherapy with methylprednisolone was started, no improvement was noticed. A cyclophosphamide trial was tried and again no positive results occurred. The patient evolved to severe clinical manifestations of general vasculitis affecting the central and peripheral nervous system and lungs, having a fatal evolution after 2 weeks. Although uncommon, the association of both disease may occur, and the authors call attention to this possibility, making a brief review of literature.

  2. [Comparison of severity and native anti-DNA antibody levels in systemic lupus erythematosus. Serodiscordant lupus].

    Science.gov (United States)

    Manent-Ducreuzet, C; Margulis, J; Kahn, M F

    1981-11-01

    The authors studied the clinicobiological correlations between the severity of systemic lupus erythematosus (SLE) and native anti-DNA antibody levels in 176 patients suffering from this disease. Certain patients were studied at different stages of their disease. This correlation was very close in one out of two cases and relative in one case out of four. In one-fourth of these patients, a significant and persistent discordance existed, and these patients were therefore studied apart. Certain SLE which have been suppressed over long periods of time continue to have elevated anti-DNA levels which are occasionally associated with lowered levels of complement. This has been previously reported in the literature. All decisions to modify corticosteroid treatment based only on biological data is therefore questionable. In contrast, in certain cases of SLE where the evolution of the disease is relatively severe, no anti-DNA or antinuclear antibodies are present. These "seronegative" SLE cases are proof that the "specific" biology of this disease may be erroneous for diagnosis as well as for prognosis. The authors review the possible or known causes for the absence of these antibodies in SLE. They believe that native anti-DNA levels are only of relative value in SLE and feel that these values must be examined with respect to clinical and other biological data in order to treat and survey patients affected with this disease.

  3. Renal Tubular Function in Systemic Lupus Erythematosus*

    African Journals Online (AJOL)

    evidence of hyperparathyroidism. Three patients were unable to concentrate urine normally, as judged by the maximal urine osmolality attained 6-8 hours after the administration of pitressin tannate in oil. The pattern of acid excretion in the presence of systemic acidosis, induced with ammonium chloride, is shown in Fig. 2.

  4. Outcomes of neuropsychiatric events in systemic lupus erythematosus based on clinical phenotypes; prospective data from the Leiden NP SLE cohort.

    Science.gov (United States)

    Magro-Checa, C; Beaart-van de Voorde, L J J; Middelkoop, H A M; Dane, M L; van der Wee, N J; van Buchem, M A; Huizinga, T W J; Steup-Beekman, G M

    2017-04-01

    Objective The objective of this study was to assess whether clinical and patient's reported outcomes are associated with a different pathophysiological origin of neuropsychiatric events presenting in systemic lupus erythematosus. Methods A total of 232 neuropsychiatric events presenting in 131 systemic lupus erythematosus patients were included. Neuropsychiatric systemic lupus erythematosus diagnosis was established per event by multidisciplinary evaluation. All neuropsychiatric events were divided according to a suspected underlying pathophysiological process into one of the following: non-neuropsychiatric systemic lupus erythematosus related, inflammatory and ischaemic neuropsychiatric systemic lupus erythematosus. The clinical outcome of all neuropsychiatric events was determined by a physician-completed four-point Likert scale. Health-related quality of life was measured with the subscales of the patient-generated Short Form 36 (SF-36) health survey questionnaire. The change between scores at paired visits of all domain scores, mental component summary (SF-36 MCS) and physical component summary (SF-36 PCS) scores were retrospectively calculated and used as patient-reported outcome. The association among these outcomes and the different origin of neuropsychiatric events was obtained using multiple logistic regression analysis. Results The clinical status of 26.8% non-neuropsychiatric systemic lupus erythematosus events, 15.8% ischaemic neuropsychiatric systemic lupus erythematosus and 51.6% inflammatory neuropsychiatric systemic lupus erythematosus improved after re-assessment. Almost all SF-36 domains had a positive change at re-assessment in all groups independently of the origin of neuropsychiatric events. Neuropsychiatric systemic lupus erythematosus ( B = 0.502; p lupus erythematosus ( B = 0.827; p lupus erythematosus ( B = 5.783; p lupus erythematosus ( B = 11.133; p lupus erythematosus events have better clinical outcome and

  5. Value of HLA-DR genotype in systemic lupus erythematosus and lupus nephritis: a meta-analysis.

    Science.gov (United States)

    Niu, Zhili; Zhang, Pingan; Tong, Yongqing

    2015-01-01

    Human leukocyte antigen (HLA)-DRB1 allele polymorphisms have been reported to be associated with systemic lupus erythematosus (SLE) susceptibility, but the results of these previous studies have been inconsistent. The purpose of the present study was to systematically summarize and explore whether specific HLA-DRB1 alleles confer susceptibility or resistance to SLE and lupus nephritis. This review was guided by the preferred reporting items for systematic reviews and meta-analyses (PRISMA) approach. A comprehensive search was made for articles from PubMed, Medline, Elsevier Science, Springer Link and Cochrane Library database. A total of 25 case-control studies on the relationship between gene polymorphism of HLA-DRB l and SLE were performed and data were analyzed and processed using Review Manager 5.2 and Stata 11.0. At the allelic level, HLA-DR4, DR11 and DR14 were identified as protective factors for SLE (0.79 [0.69,0.91], P  0.05). DR4 and 11 (OR, 0.55 [0.39, 0.79], P lupus nephritis. DR3 and DR15 (OR, 2.00 [1.49, 2.70], P lupus nephritis. HLA-DR8, DR9 and DR14 (OR, 1.47 [0.9, 2.33], P > 0.05; 0.90 [0.64, 1.27], P > 0.05; 0.61 [0.36, 1.03], P > 0.05, respectively) were not statistically significant between the lupus nephritis and control groups. The HLA-DR4, DR11, DR14 alleles might be protective factors for SLE and HLA-DR3, DR9, DR15 were potent risk factors. In addition, HLA-DR4 and DR11 alleles might be protective factors for lupus nephritis and DR3 and DR15 suggest a risk role. These results proved that HLA-DR3, DR15, DR4 and DR11 might be identified as predictors for lupus nephritis and SLE. © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  6. Lupus Nephritis

    Science.gov (United States)

    ... Kidneys & How They Work Lupus and Kidney Disease (Lupus Nephritis) What is lupus nephritis? Lupus nephritis is a type of kidney disease ... women will develop lupus. 2 How common is lupus nephritis? Kidney damage is one of the more common ...

  7. Venous and Arterial Thrombotic Events in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Hinojosa-Azaola, Andrea; Romero-Diaz, Juanita; Vargas-Ruiz, Angel Gabriel; Nuñez-Alvarez, Carlos A; Cicero-Casarrubias, Alba; Ocampo-Torres, Mario C; Sanchez-Guerrero, Jorge

    2016-03-01

    The incidence of thrombosis in patients with systemic lupus erythematosus (SLE) is 25 to 50-fold higher than in the general population; we aimed to define the characteristics of venous thrombotic events (VTE) and arterial thrombotic events (ATE) to identify the patients at highest risk. The study included 219 patients with recent-onset SLE. At baseline, standardized medical history and laboratory tests were done. Followup visits occurred quarterly, and information about damage accrual, comorbidities, and cardiovascular risk factors was updated annually. Main outcome was development of TE after SLE diagnosis. Thirty-five patients (16%) developed TE (27 VTE, 8 ATE) during 5.21 years of followup; incidence rate 31/1000 patient-years. Most events (57%) developed within the first year of diagnosis, and 69% were not associated with lupus anticoagulant (LAC), determined with 1 method. VTE developed earlier than ATE (2.0 vs 57.5 mos, p = 0.02). In the multivariate analysis, variables preceding VTE included cutaneous vasculitis, nephrotic syndrome, dose of prednisone, and LAC in combination with anti-RNP/Sm antibodies (p LAC in combination with anti-RNP/Sm antibodies was associated with VTE and improved the accuracy for predicting it. Our study suggests that in SLE, VTE and ATE have different risk factors. Understanding these differences is helpful for identifying patients at highest risk. The use of LAC plus anti-RNP/Sm for predicting VTE deserves further study.

  8. Imaging Assessment of Cardiovascular Disease in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Sara C. Croca

    2012-01-01

    Full Text Available Systemic lupus erythematosus is a multisystem, autoimmune disease known to be one of the strongest risk factors for atherosclerosis. Patients with SLE have an excess cardiovascular risk compared with the general population, leading to increased cardiovascular morbidity and mortality. Although the precise explanation for this is yet to be established, it seems to be associated with the presence of an accelerated atherosclerotic process, arising from the combination of traditional and lupus-specific risk factors. Moreover, cardiovascular-disease associated mortality in patients with SLE has not improved over time. One of the main reasons for this is the poor performance of standard risk stratification tools on assessing the cardiovascular risk of patients with SLE. Therefore, establishing alternative ways to identify patients at increased risk efficiently is essential. With recent developments in several imaging techniques, the ultimate goal of cardiovascular assessment will shift from assessing symptomatic patients to diagnosing early cardiovascular disease in asymptomatic patients which will hopefully help us to prevent its progression. This review will focus on the current status of the imaging tools available to assess cardiac and vascular function in patients with SLE.

  9. Genetic risk factors for thrombosis in systemic lupus erythematosus.

    Science.gov (United States)

    Kaiser, Rachel; Li, Yonghong; Chang, Monica; Catanese, Joseph; Begovich, Ann B; Brown, Elizabeth E; Edberg, Jeffrey C; McGwin, Gerald; Alarcón, Graciela S; Ramsey-Goldman, Rosalind; Reveille, John D; Vilá, Luis M; Petri, Michelle A; Kimberly, Robert P; Taylor, Kimberly E; Criswell, Lindsey A

    2012-08-01

    Thrombosis is a serious complication of systemic lupus erythematosus (SLE). We investigated whether genetic variants implicated in thrombosis pathways are associated with thrombosis among 2 ethnically diverse SLE cohorts. Our discovery cohort consisted of 1698 patients with SLE enrolled in the University of California, San Francisco, Lupus Genetics Project and our replication cohort included 1361 patients with SLE enrolled in the PROFILE cohort. Patients fulfilled American College of Rheumatology SLE criteria, and data relevant to thrombosis were available. Thirty-three single nucleotide polymorphisms (SNP) previously shown to be associated with risk of deep venous thrombosis in the general population or implicated in thrombosis pathways were genotyped and tested for association with thrombosis in bivariate allelic analyses. SNP with p FGG) rs2066865 (OR 1.91, p = 0.01) in Hispanic Americans. SNP in these genes showed association with venous thrombosis risk in whites: MTHFR rs1801131 (OR 1.51, p = 0.01), MTHFR rs1801133 (OR 0.70, p = 0.04), FVL rs6025 (OR 2.69, p = 0.002), and FGG rs2066865 (OR 1.49, p = 0.02) in whites. A SNP in FGG rs2066865 (OR 2.19, p = 0.003) demonstrated association with arterial thrombosis risk in Hispanics. Our results implicate specific genetic risk factors for thrombosis in patients with SLE and suggest that genetic risk for thrombosis differs across ethnic groups.

  10. Distinct proteome pathology of circulating microparticles in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Østergaard, Ole; Nielsen, Christoffer Tandrup; Tanassi, Julia Tanas

    2017-01-01

    BACKGROUND: The pathogenesis of systemic lupus erythematosus (SLE) is poorly understood but has been linked to defective clearance of subcellular particulate material from the circulation. This study investigates the origin, formation, and specificity of circulating microparticles (MPs) in patients...... with SLE based on comprehensive MP proteome profiling using patients with systemic sclerosis (SSc) and healthy donors (HC) as controls. METHODS: We purified MPs from platelet-poor plasma using differential centrifugation of samples from SLE (n = 45), SSc (n = 38), and two sets of HC (n = 35, n = 25). MP......-MPs (which we propose to call luposomes) are highly specific for SLE, i.e. not found in MP preparations from HC or patients with another autoimmune, systemic disease, SSc. In SLE-MPs platelet proteins and mitochondrial proteins are significantly diminished, cytoskeletal proteins deranged, and glycolytic...

  11. Risk of infective endocarditis in patients with systemic lupus erythematosus in Taiwan: a nationwide population-based study.

    Science.gov (United States)

    Chang, Y S; Chang, C C; Chen, Y H; Chen, W S; Chen, J H

    2017-10-01

    Objectives Patients with systemic lupus erythematosus are considered vulnerable to infective endocarditis and prophylactic antibiotics are recommended before an invasive dental procedure. However, the evidence is insufficient. This nationwide population-based study evaluated the risk and related factors of infective endocarditis in systemic lupus erythematosus. Methods We identified 12,102 systemic lupus erythematosus patients from the National Health Insurance research-oriented database, and compared the incidence rate of infective endocarditis with that among 48,408 non-systemic lupus erythematosus controls. A Cox multivariable proportional hazards model was employed to evaluate the risk of infective endocarditis in the systemic lupus erythematosus cohort. Results After a mean follow-up of more than six years, the systemic lupus erythematosus cohort had a significantly higher incidence rate of infective endocarditis (42.58 vs 4.32 per 100,000 person-years, incidence rate ratio = 9.86, p lupus erythematosus cohort had lower risk (adjusted hazard ratio 11.64) than that of the younger-than-60-years systemic lupus erythematosus cohort (adjusted hazard ratio 15.82). Cox multivariate proportional hazards analysis revealed heart disease (hazard ratio = 5.71, p lupus erythematosus patients. Conclusions A higher risk of infective endocarditis was observed in systemic lupus erythematosus patients. Risk factors for infective endocarditis in the systemic lupus erythematosus cohort included heart disease, chronic kidney disease, steroid pulse therapy within 30 days, and a recent invasive dental procedure within 30 days.

  12. Serum interleukin-17 levels are associated with nephritis in childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Peliçari, Karina de Oliveira; Postal, Mariana; Sinicato, Nailú Angelica; Peres, Fernando Augusto; Fernandes, Paula Teixeira; Marini, Roberto; Costallat, Lilian Tereza Lavras; Appenzeller, Simone

    2015-05-01

    To determine the serum interleukin-17 (IL-17) levels in childhood-onset systemic lupus erythematosus patients and to evaluate the association between IL-17 and clinical manifestations, disease activity, laboratory findings and treatment. We included 67 consecutive childhood-onset systemic lupus erythematosus patients [61 women; median age 18 years (range 11-31)], 55 first-degree relatives [50 women; median age 40 years (range 29-52)] and 47 age- and sex-matched healthy controls [42 women; median age 19 years (range 6-30)]. The childhood-onset systemic lupus erythematosus patients were assessed for clinical and laboratory systemic lupus erythematosus manifestations, disease activity [Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)], cumulative damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index] and current drug use. Serum IL-17 levels were measured by an enzyme-linked immunosorbent assay using commercial kits. The median serum IL-17 level was 36.3 (range 17.36-105.92) pg/mL in childhood-onset systemic lupus erythematosus patients and 29.47 (15.16-62.17) pg/mL in healthy controls (p=0.009). We observed an association between serum IL-17 levels and active nephritis (p=0.01) and migraines (p=0.03). Serum IL-17 levels were not associated with disease activity (p=0.32), cumulative damage (p=0.34), or medication use (p=0.63). IL-17 is increased in childhood-onset systemic lupus erythematosus and may play a role in the pathogenesis of neuropsychiatric and renal manifestations. Longitudinal studies are necessary to determine the role of IL-17 in childhood-onset systemic lupus erythematosus.

  13. Breakdown of Immune Tolerance in Systemic Lupus Erythematosus by Dendritic Cells

    Science.gov (United States)

    Reihl, Alec M.

    2016-01-01

    Dendritic cells (DC) play an important role in the pathogenesis of systemic lupus erythematosus (SLE), an autoimmune disease with multiple tissue manifestations. In this review, we summarize recent studies on the roles of conventional DC and plasmacytoid DC in the development of both murine lupus and human SLE. In the past decade, studies using selective DC depletions have demonstrated critical roles of DC in lupus progression. Comprehensive in vitro and in vivo studies suggest activation of DC by self-antigens in lupus pathogenesis, followed by breakdown of immune tolerance to self. Potential treatment strategies targeting DC have been developed. However, many questions remain regarding the mechanisms by which DC modulate lupus pathogenesis that require further investigations. PMID:27034965

  14. Health related quality of life in Mexican women with systemic lupus erythematosus: a descriptive study using SF-36 and LupusQoL(C).

    Science.gov (United States)

    García-Carrasco, M; Mendoza-Pinto, C; Cardiel, M H; Méndez-Martínez, S; García-Villaseñor, A; Jiménez-Hernández, C; Alonso-García, N E; Briones-Rojas, R; Ramos-Álvarez, G; López-Colombo, A

    2012-10-01

    The LupusQoL© questionnaire is a disease-specific health related quality of life (HRQOL) instrument for adults with systemic lupus erythematosus (SLE). The Short Form-36 (SF-36) is a generic instrument that captures the physical, psychological, and social impact. We conducted a descriptive study of women aged ≥ 18 years attending our Lupus Clinic. HRQOL was assessed by applying the LupusQoL© and SF-36. Lupus activity was measured using the Mexican Systemic Lupus Erythematosus Disease Activity Index (Mex-SLEDAI) and chronic damage using the Systemic Lupus Collaborative Clinics Damage Index (SDI). Data were analyzed using descriptive statistics, the chi-square test and Pearson's product moment correlation coefficient. A total of 127 patients were included with a mean age of 40.5 ± 12.6 years. The mean disease duration was 8.2 ± 5.6 years, the mean disease activity score was 2.4 ± 3.0, and the mean SDI score 0.77 ± 1.06. The mean SF-36 score was 58.1 ± 21.1 and the mean LupusQoL© score was 69 ± 22.7. The correlation between global scores of the SF-36 and LupusQoL© was rho = 0.73 (p SF-36 and the LupusQoL© was -0.26 (p = 0.003) and -0.25 (p = 0.004), respectively. The correlation between the SDI and the SF-36 and the LupusQoL© was -0.28 (p = 0.001) and -0.38 (p SF-36 were useful instruments in assessing HRQOL in Mexican lupus female patients. The usefulness of the LupusQoL© should be evaluated in lupus patients with moderate to severe disease activity.

  15. Apoptosis-induced histone H3 methylation is targeted by autoantibodies in systemic lupus erythematosus

    NARCIS (Netherlands)

    Bavel, C.C.A.W. van; Dieker, J.W.C.; Kroeze, Y.L.; Tamboer, W.P.M.; Voll, R.; Muller, S.; Berden, J.H.M.; Vlag, J. van der

    2011-01-01

    OBJECTIVES: In systemic lupus erythematosus (SLE) apoptotic chromatin is present extracellularly, which is most likely the result of disturbed apoptosis and/or insufficient removal. Released chromatin, modified during apoptosis, activates the immune system resulting in the formation of

  16. Potential benefits of vitamin D for patients with systemic lupus erythematosus

    National Research Council Canada - National Science Library

    Singh, Abha; Kamen, Diane L

    2012-01-01

    Systemic lupus erythematosus (SLE) is a complex multi-system autoimmune disease. Vitamin D deficiency has been proposed as an environmental trigger of disease onset and as a contributor to increased SLE activity...

  17. A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis

    Directory of Open Access Journals (Sweden)

    Pamela Traisak

    2016-01-01

    Full Text Available The clinical presentation of Systemic Lupus Erythematosus (SLE is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA, polyarteritis nodosa (PAN, and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA, among others, but only a few cases attributed to SLE. Awareness of this condition and a prompt diagnosis are warranted as this is a severe and potentially life-threatening condition.

  18. [The clinico-immunological subtypes of systemic lupus erythematosus].

    Science.gov (United States)

    Speranskiĭ, A I; Nasonova, V A; Ivanova, S M; Riazantseva, T A; Alekberova, Z S

    1992-01-01

    Based on clinico-immunologic studies subtypes of systemic lupus erythematosus (SLE) were distinguished. The ANF-R++H-DNA-CH50 variant determines acute onset of SLE with renal injury in the form of diffuse glomerulonephritis. The ANF-Sp-RNP-RF mirrors the development of Raynaud's syndrome, Sjögren's syndrome, polymyositis, pneumosclerosis and myocarditis. The ANF-Sp-Ro-RF variant is associated with skin derangement in the form of discoid foci, anular, papulosquamous eruption, vitiligo, hyperpigmentation, and cerebrovasculitis. The Ro-anti-Po, La-anti-La system is related to the idea of SLE, seronegative in accordance with ANF, when rat liver sections are used as a substrate in immunofluorescence.

  19. Late-onset systemic lupus erythematosus: clinical and immunological characteristics.

    Science.gov (United States)

    Shaikh, S K; Wang, F

    1995-03-01

    Between January 1976 and December 1992, 17 patients on follow-up at Systemic Erythematosus (SLE) Clinic in the University Hospital, Kuala Lumpur had onset of the disease after the age of 50 years. This constituted about 4% of our total SLE patients. They formed a distinct subgroup of the lupus population with an insidious onset and have a benign course compared to the younger SLE patients. Arthritis and skin rashes were the commonest initial manifestations. Renal and central nervous system manifestations were uncommon but pulmonary involvement was frequent compared to young SLE patients. The prevalence of positive autoantibodies and hypocomplementaemia were lower. Disease activity showed no correlation with erythrocyte sendimentation rate, autoantibodies or complement levels. Overall prognosis in these late-onset patients was favourable with a good response to steroids and less frequent relapses.

  20. Regulatory T cells in systemic lupus erythematosus and pregnancy.

    Science.gov (United States)

    Tower, Clare; Mathen, Stephy; Crocker, Ian; Bruce, Ian N

    2013-06-01

    Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disorder that predominantly affects women of reproductive age. As clinical outcomes improve, pregnancy in these women is becoming more common. Although epidemiological data have documented an improvement in the prognosis of pregnancy in these women over recent years, they are still at significantly increased risk of pregnancy complications, such as miscarriage, stillbirth, pre-eclampsia and impaired foetal growth. The pathogenesis of SLE involves marked immune dysfunction, and in particular, the function of immunosuppressive elements of the immune system is impaired, including regulatory T-cell function. Because regulatory T cells are likely to be the key cell-modulating feto-maternal tolerance, this review overviews the possibility that regulatory T-cell impairments contribute to pregnancy pathology in women with SLE and contribute to the clinical challenge of managing these women during pregnancy. © 2013 John Wiley & Sons Ltd.

  1. Basal ganglia calcification on computed tomography in systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Nagaoka, Shohei; Tani, Kenji; Ishigatsubo, Yoshiaki and others

    1988-09-01

    The development of basal ganglia calcification was studied in 85 patients with systemic lupus erythematosus (SLE) by computed tomography (CT). Bilateral calcification of the basal ganglia was found to occur in 5 patients (5.9 %) with SLE, but was not seen in patients with rheumatoid arthritis and progressive systemic sclerosis. All were female with a mean age of 42 years (range 29 - 49). The patients with calcification of the basal ganglia had neurological symptoms, such as psychiatric problems (3 cases), grand mal seizures (1 case), CSF abnormalities (2 cases), and EEG changes (4 cases). There were significantly higher incidences of alopecia, cutaneous vasculitis, leukopenia, and thrombocytopenia in the group with calcifications than those in the group with normal CT findings. Circulating immune complexes were detected and LE tests were positive in 2 patients. Endocrinological examination showed no abnormality in any. We suggest that basal ganglia calcification in SLE might be related to cerebral vasculitis.

  2. Role of Vitamin D in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Rada Miskovic

    2014-12-01

    Full Text Available Vitamin D is a steroid hormone that in addition to its well known role in the metabolism of calcium and phosphorus exerts immunoregulatory properties. Data from animal studies and from prospective clinical trials on patients with rheumatoid arthritis, multiple sclerosis and type 1 diabetes point to the potential role of vitamin D as important environmental factor in the development of autoimmune diseases. Such role of vitamin D in systemic lupus erythematosus (SLE has not yet been sufficiently studied. This review shows the sources, metabolism and mechanism of action of vitamin D, its effect on the cells of the immune system, prevalence and causes of vitamin D deficiency in patients with SLE, the link between vitamin D status and disease activity as well as recommendations for vitamin D supplementation.

  3. Active necrotizing cerebral vasculitis in systemic lupus erythematosus.

    Science.gov (United States)

    Goel, Deepa; Reddy, S Rajashekhar; Sundaram, Challa; Prayaga, Aruna K; Rajasekhar, Liza; Narsimulu, Gumdal

    2007-12-01

    Systemic lupus erythematosus (SLE) is a multisystemic disease with varied clinical manifestations. Focal cortical brain infarcts and CNS infections are the most common neuropathological features reported in most studies. This report describes a 32-year-old woman who had repeated episodes of strokes over 5 years. In view of polyarthritis, oral ulcers, presence of high titres of serum antinuclear antibodies, high titres of double-stranded DNA and strokes, she was treated as SLE. Despite prolonged immunosuppressive therapy with azathioprine and pulse cyclophosphamide, she succumbed to a brainstem stroke. Complete body autopsy showed multiple cerebral cortical and brainstem infarcts with fibrinoid necrosis of the vessel wall. Renal infarction with healed vasculitis and systemic vasculitis involving small vessels was seen. Extensive thrombosis was remarkable by its absence. Active necrotizing vasculitis of cerebral and renal vessels is a rare complication of SLE, which contributed to a fatal outcome in this patient.

  4. Pigmented villonodular synovitis of the hip in systemic lupus erythematosus: a case report

    Directory of Open Access Journals (Sweden)

    Anders Hans-Joachim

    2011-09-01

    Full Text Available Abstract Introduction Pigmented villonodular synovitis is a rare disease of unknown etiology mostly affecting the knee and foot. Until now an association with autoimmune diseases has not been reported. Case presentation The diagnosis of systemic lupus erythematosus was made in a 15-year-old Caucasian girl based on otherwise unexplained fatigue, arthralgia, tenosynovitis, leukopenia, low platelets and the presence of antinuclear and deoxyribonucleic antibodies. At the age of 20 a renal biopsy revealed lupus nephritis class IV and she went into complete remission with mycophenolate mofetil and steroids. She was kept on mycophenolate mofetil for maintenance therapy. At the age of 24 she experienced a flare-up of lupus nephritis with nephrotic syndrome and new onset of pain in her right hip. Magnetic resonance imaging, arthroscopy and subtotal synovectomy identified pigmented villonodular synovitis as the underlying diagnosis. Although her systemic lupus erythematosus went into remission with another course of steroids and higher doses of mycophenolate mofetil, the pigmented villonodular synovitis persisted and she had to undergo open synovectomy to control her symptoms. Conclusion Systemic lupus erythematosus is associated with many different musculoskeletal manifestations including synovitis and arthritis. Pigmented villonodular synovitis has not previously been reported in association with systemic lupus erythematosus, but as its etiology is still unknown, the present case raises the question about a causal relationship between systemic lupus erythematosus and pigmented villonodular synovitis.

  5. Successful treatment of recurrent pancreatitis secondary to systemic lupus erythematosus with B-cell depletion therapy.

    Science.gov (United States)

    Al-Musawi, Zakiya S; Nabar, Umesh J

    2011-01-01

    Recurrent pancreatitis secondary to systemic lupus erythematosus is a rare entity of unknown etiology. We report an adolescent with systemic lupus erythematosus and recurrent attacks of acute pancreatitis, which were poorly controlled with conventional therapy for approximately four years. Rituximab, a chimeric anti-CD20 monoclonal antibody therapy resulted in remission of symptoms for more than two years without major toxicity of treatment. Based on tolerability and high efficiency of rituximab therapy, we would suggest using B-cell depletion therapy as an alternative therapy for refractory pancreatitis secondary to systemic lupus erythematosus.

  6. Peripheral Gangrene Complicating Systemic Lupus Erythematosus in a Patient with Spina Bifida: A Case Report

    Directory of Open Access Journals (Sweden)

    Vijay S

    2017-03-01

    Full Text Available An adolescent girl, a known case of spina bifida with systemic lupus, presented with bluish discolouration of three toes of the right foot. She had thrombosis of bilateral popliteal arteries. She underwent percutaneous transluminal angioplasty (PTA of both legs and Chopart amputation of the right foot. Systemic lupus erythematosus (SLE occurring in a patient with spina bifida has not been previously reported. Weakness, sensory loss, lack of normal ambulation, endarteritis, antiphospholipid antibody syndrome are common contributory factors for peripheral gangrene in patients with spina bifida with systemic lupus erythematosus.

  7. Urinary interleukin 22 binding protein as a marker of lupus nephritis in Egyptian children with juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Badr, Ahmed Mohamed Mahmoud; Farag, Yomna; Abdelshafy, Maie; Riad, Nermine Magdi

    2018-02-01

    Juvenile systemic lupus erythematosus (JSLE) is a multi-system autoimmune inflammatory disease. Generally, 60% of patients will develop lupus nephritis (LN); thus, early recognition and treatment is associated with better outcome. Interleukin 22 (IL-22) is involved in tissue inflammation and is regulated by interleukin 22 binding protein (IL-22BP). This study aimed to use IL-22BP as a non-invasive marker for disease activity in JSLE and LN. This is a cross-sectional study conducted on 82 subjects: 51 JSLE patients and 31 healthy controls of matched age and gender. Urinary IL-22BP was measured using enzyme-linked immunosorbent assay, and its level was correlated with different clinical and laboratory data in JSLE as well as Systemic Lupus Erythematous Disease Activity Index 2000 (SLEDAI-2k), renal SLEDAI-2k, and Systemic Lupus International Collaborating Clinics (SLICC) renal activity score which were used to assess overall disease and renal activity. Our results showed that urinary IL-22BP level was significantly higher in JSLE patients with mean level of 4.13 ± 1.10, as compared to controls 1.63 ± 0.61 (P value < 0.001); also, patients with active LN had urinary levels of IL-22BP (5.47 ± 1.03) higher than patients with active JSLE without LN (4.23 ± 0.72) and patients with non-active JSLE/LN (3.5 ± 0.65) with a highly significant P value < 0.001. There was a positive correlation with SLEDAI-2k, renal SLEDAI, and renal activity scores (P < 0.001). Urinary IL-22BP may be used as a non-invasive marker for assessment of disease activity in children with JSLE and LN.

  8. Juvenile systemic lupus erythematosus: neurological involvement Lupus erítematoso sistêmico juvenil: comprometimento neurológico

    OpenAIRE

    Katia M.R.S. Schmutzler; Vilanova, Luiz Celso P.; Lima, José Geraldo C. [UNIFESP; Maria Odete Hilário; Naspitz,Charles K.

    1997-01-01

    With the purpose of analyzing the neurological involvement due to systemic lupus erythematosus (SLE), we evaluated 17 female patients who were seen regularly at the hospital and had been diagnosed as having SLE according to classification criteria proposed by the American College of Rheumatology revised in 1982, before the age of 16. Neurological involvement was detected in 12 patients (71%): headache (35%), extrapyramidal syndrome (35%), epileptic syndrome (24%) pyramidal syndrome (24%), per...

  9. Granulocytic invasion of the central nervous system after hematopoietic stem cell transplantation for systemic lupus erythematosus.

    Science.gov (United States)

    Muraro, Paolo A; Nikolov, Nikolay P; Butman, John A; Abati, Andrea; Gea-Banacloche, Juan; Gress, Ronald; Lipsky, Peter; Illei, Gabor; Pavletic, Steven

    2006-06-01

    We report on the likely mechanism of an exacerbation of neurological symptoms developed during immune reconstitution after autologous non-myeloablative hematopoietic stem cell transplantation in a 33-year-old man with systemic lupus erythematosus- associated recurrent transverse myelitis. Cerebrospinal fluid examination revealed prominent neutrophilic pleocytosis and no evidence of infection or of reactivation of lupus. Following a course of corticosteroid treatment the exacerbation resolved completely and the patient's neurological function continued to improve, resulting in net gain above pre-treatment for over 1 year follow-up without maintenance immunosuppression. Granulocytic invasion of the central nervous system represents a novel and possibly preventable cause of neurological complications during haematologic reconstitution.

  10. Successful delivery in a pregnant woman with lupus anticoagulant positive systemic lupus erythematosus treated with double filtration plasmapheresis.

    Science.gov (United States)

    Takeshita, Y; Turumi, Y; Touma, S; Takagi, N

    2001-02-01

    The case was a 29 year old female who has suffered from systemic lupus erythematosus (SLE) since 15 years of age. The activity of SLE was low, and she took prednisolone orally. Her first pregnancy failed after 14 weeks. In the second pregnancy, she had thrombocytopenia, prolonged activated partial thromboplastin time (APTT), positive lupus anticoagulant (LAC) and thus was diagnosed with antiphospholipid antibody syndrome (APS). Combination therapy with steroids and aspirin was started, and she underwent treatment of double filtration plasmapheresis (DFPP) in the early stage of pregnancy. Her platelet count increased, and the value of APTT has normalized with DFPP treatment. She delivered successfully on the 32nd week of pregnancy. We think that DFPP is an effective and safe treatment in patients with an LAC positive pregnancy.

  11. Heterogeneity and diversity of IgM and IgG lupus anticoagulants in an individual with systemic lupus erythematosus.

    Science.gov (United States)

    Nakamura, N; Azuma, C; Akamizu, T; Sugawa, H; Matsuda, F; Mitsuda, N; Honjo, T; Mori, T; Yamaji, K

    1994-09-30

    From one patient with systemic lupus erythematosus retaining lupus anticoagulant (LAC), we established 6 Epstein-Barr virus-transformed human B cell clones secreting antibodies that affect the coagulation assay. Two and 4 of the clones secreted IgM and IgG antibodies, respectively. Although all 6 antibodies displayed anticardiolipin activity in ELISA, the increased binding activity in the presence of beta 2-glycoprotein I was limited only to the IgG antibodies. Five antibodies (two IgM and three IgG) had LAC activity which prolonged the activated partial thromboplastin time (APTT), whereas one IgG antibody shortened the APTT. Two of the IgG producing clones had an identical Ig heavy chain gene rearrangement despite their opposite effects on the coagulation assay. These results demonstrated the heterogeneity of LACs and diversity among their physiological functions.

  12. Prognostically distinct clinical patterns of systemic lupus erythematosus identified by cluster analysis.

    Science.gov (United States)

    To, C H; Mok, C C; Tang, S S K; Ying, S K Y; Wong, R W S; Lau, C S

    2009-12-01

    The objective of this study was to evaluate the patterns of clinical manifestations and their mortality in a large cohort of Chinese patients with systemic lupus erythematosus. The cumulative clinical manifestations of a large group of Chinese systemic lupus erythematosus patients who fulfilled at least four American College of Rheumatology criteria for systemic lupus erythematosus were studied. Patients were divided into distinct groups by using the K-mean cluster analysis. Clinical features, prevalence of proliferative lupus nephritis (World Health Organization class III, IV), autoantibody profile, and treatment data were compared and the standardized mortality ratios were calculated for each cluster of patients. There were 1082 patients included in the study (mean age at systemic lupus erythematosus diagnosis 30.5 years; mean systemic lupus erythematosus duration 10.3 years). Three distinct groups of patients were identified. Cluster 1 (n = 347) was characterized predominantly by mucocutaneous manifestations (malar rash, discoid rash, photosensitivity, oral ulcer) and arthritis but having the lowest prevalence of serositis, hematologic manifestations (hemolytic anemia, leukopenia, and thrombocytopenia), and proliferative lupus nephritis. Patients in cluster 2 (n = 409) had mainly renal and hematological manifestations but having the lowest prevalence of mucocutaneous manifestations. Pulmonary and gastrointestinal manifestations were significantly more frequent in cluster 2 than the other clusters. Cluster 3 patients (n = 326) had the most heterogeneous features. Besides having a high prevalence of mucocutaneous manifestations, serositis and hematologic manifestations, renal involvement, and proliferative lupus nephritis was also most prevalent among the three clusters. Patients in cluster 2 had a much higher standardized mortality ratio [standardized mortality ratio 7.23 (6.7-7.7), p lupus erythematosus could be clustered into prognostically distinct patterns of

  13. Living with Lupus (For Parents)

    Science.gov (United States)

    ... Kidney Transplant Vision Facts and Myths Living With Lupus KidsHealth > For Parents > Living With Lupus Print A ... disease for both doctors and their patients. About Lupus A healthy immune system produces proteins called antibodies ...

  14. a possible biomarker of pediatric systemic lupus erythematosus

    African Journals Online (AJOL)

    Patients in lupus flare and those in remission had comparable levels (p> 0.05). Serum IL-27 did not vary significantly between patients with lupus nephritis (LN) and those without evident LN, moreover, it was comparable among different histological classes of LN (p> 0.05). The disease status in terms of SLE disease activity ...

  15. Clinical features of patients with systemic lupus erythematosus (SLE ...

    African Journals Online (AJOL)

    DR2 increases a person's risk of developing lupus nephritis, although the vast majority of individuals with the gene are healthy.5 There recently have been discoveries of a gene on chromosome 1 that is associated with lupus in certain families.5. Many researchers suspect that a special type of immune reaction causes the ...

  16. Systemic lupus erythematosus: epidemiology, pathophysiology, manifestations, and management.

    Science.gov (United States)

    Fortuna, Giulio; Brennan, Michael T

    2013-10-01

    Systemic lupus erythematosus is a chronic autoimmune disorder characterized by production of autoantibodies directed against nuclear and cytoplasmic antigens, affecting several organs. Although cause is largely unknown, pathophysiology is attributed to several factors. Clinically, this disorder is characterized by periods of remission and relapse and may present with various constitutional and organ-specific symptoms. Diagnosis is achieved via clinical findings and laboratory examinations. Therapies are based on disease activity and severity. General treatment considerations include sun protection, diet and nutrition, smoking cessation, exercise, and appropriate immunization, whereas organ-specific treatments include use of steroidal and nonsteroidal anti-inflammatory drugs, immunosuppressive agents, and biologic agents. Copyright © 2013 Elsevier Inc. All rights reserved.

  17. Dexamethasone-cyclophosphamide pulse therapy in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Dhabhai Ravindra

    2005-01-01

    Full Text Available BACKGROUND AND AIMS: Therapy systemic lupus erythematosus (SLE has been generally discouraging. Methyl-prednisolone pulse therapy has been used for various connective tissue disorders. We used intravenous dexamethasone cyclophosphamide pulse therapy to treat SLE. METHODS: Fourteen patients (10 females and 4 males between the age of 15-48 years with definite or classical clinical criteria laid by American Rheumatism Association criteria were treated by Dexamethasone-Cyclophosphamide pulse (DCP therapy at our center. RESULTS: It was possible to induce a complete clinical remission with DCP therapy in most of the patients thereby offering them life free from disease and drugs. The side effects commonly observed with conventional daily dose regimen of corticosteroids were not present or were mild. CONCLUSIONS: Almost all patients had good response after 3-4 pulses to allow them a normal life style. Fever, malar rash and oral ulceration responded early but photosensitivity, discoid rash, alopecia and joint pains took some more time.

  18. [A case of skin cryptococcosis in systemic lupus erythematosus].

    Science.gov (United States)

    Halweg, H; Korzeniewska-Koseła, M; Podsiadło, B; Krakówka, P

    1990-01-01

    Here is presented a case of woman treated by immunosuppressive preparations because of systemic lupus erythematosus with ski manifestations as tubercles and ulcerations on skin of trunk and extremities. On the basis of histological examination of tubercle skin specimens and mycological examinations of material obtained from skin ulcerations cryptococcosis was diagnosed. Disease was limited to skin that was an entry of infection. Patient was treated by Amphotericin B administered intravenously and Flucitosine per os. Amphotericin B was also applied topically. The results of cultures became gradually negative, up to total disappearance of fungus cells in direct specimens, prepared from examined material. After treatment continuing for 5 months only discoloured scars were observed on sick skin.

  19. [A case of seronegative systemic lupus erythematosus (an immunopathological study)].

    Science.gov (United States)

    Beletskaia, L V; Anokhina, G I; Samsonova, E S; Bektimirov, R A; Mogireva, I A; Kovalenko, N S

    1990-01-01

    A case of systemic lupus erythematosus (SLE) in a 37-year-old female is described which was primarily diagnosed as epilepsy. Immunopathologically, an immunoglobulin in the nuclei of the majority of cells in the skin biopsy and blood cells was found against the background of the lack of SLE manifestations (antinuclear factor in the circulating blood, skin lesions, etc.). Antibodies against the antigen of vascular intima are revealed in the blood by means of the indirect immunofluorescence. The epilepsy diagnosis was established due to the prevalence of the brain vessels affection (frequent loss of consciousness). The progression of the disease with the involvement of joints, heart, lungs and other organs, high indexes of the rheumatoid factor made it necessary to differentiate between rheumatoid arthritis and SLE. The immunopathological examination confirmed the SLE diagnosis. Hemosorption combined with a standard therapy was followed by the decrease of the immunopathological manifestations.

  20. Extracellular Vesicles as Therapeutic Agents in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Perez-Hernandez, Javier; Redon, Josep; Cortes, Raquel

    2017-03-28

    Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease that affects multiple organs. Currently, therapeutic molecules present adverse side effects and are only effective in some SLE patient subgroups. Extracellular vesicles (EV), including exosomes, microvesicles and apoptotic bodies, are released by most cell types, carry nucleic acids, proteins and lipids and play a crucial role in cell-to-cell communication. EVs can stimulate or suppress the immune responses depending on the context. In SLE, EVs can work as autoadjuvants, enhance immune complex formation and maintaining inflammation state. Over the last years, EVs derived from mesenchymal stem cells and antigen presenting cells have emerged as cell-free therapeutic agents to treat autoimmune and inflammatory diseases. In this review, we summarize the current therapeutic applications of extracellular vesicles to regulate immune responses and to ameliorate disease activity in SLE and other autoimmune disorders.

  1. Fluorescent light photosensitivity in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Rihner, M; McGrath, H

    1992-08-01

    To determine the prevalence of fluorescent light toxicity in patients with systemic lupus erythematosus (SLE). SLE patients were polled about their symptomatic responses to sunlight and cool white fluorescent light. Photometry was used to determine the levels of ultraviolet (UV) emissions from fluorescent lamps. Thirteen of 30 photosensitive SLE patients described increases in disease activity following exposure to unshielded fluorescent lamps. Photometry indicated that these lamps emit substantial levels of UV-B (280-320 nm) radiation, which is toxic to patients with SLE. Standard acrylic diffusers absorbed this radiation, and their use was associated with almost no patient-reported problems. Fluorescent lamps, emitting UV-B radiation, induce disease activity in photosensitive SLE patients. Standard acrylic diffusers absorb UV-B radiation and appear to be protective against induction of disease activity with the use of fluorescent lamps.

  2. Treat-to-target in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Mosca, Marta; Boumpas, Dimitrios T; Bruce, Ian N

    2013-01-01

    Multiple clinical trials performed over twenty years in the treatment of rheumatoid arthritis (RA) have clearly demonstrated that patients have better outcomes if their disease activity at each time-point for follow-up includes a pre-specified target. A European SLE expert panel met in Zurich...... on May 8, 2012 to discuss whether a treat-to-target approach could be applied in the treatment of systemic lupus erythematosus (SLE) (T2T/SLE), define a research agenda, and establish a plan for moving forward. In the present paper, observations raised at the meeting and literature data on potential...... therapeutic targets are reported. The working group on T2T/SLE will continue work over the coming year....

  3. Relapsed hydroxychloroquine induced thrombocytopenia in a systemic lupus erythematosus patient.

    Science.gov (United States)

    Antón Vázquez, Vanesa; Pascual, Luis; Corominas, Héctor; Giménez Torrecilla, Isabel

    Hydroxychloroquine is used in the long-term therapy of systemic lupus erythematosus (SLE). Although considered to be a safe treatment, side effects have been documented. An uncommon side effect is thrombocytopenia. In order to establish the diagnosis of thrombocytopenia secondary to Hydroxychloroquine, non-pharmacological causes must be ruled out and it is necessary to determine a recurrence after re-exposure to the drug. We present one case of severe thrombocytopenia occurring in a patient with SLE undergoing treatment with Hydroxychloroquine. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  4. Acute sideroblastic anemia in active systemic lupus erythematosus.

    Science.gov (United States)

    Jiménez-Balderas, F J; Morales-Polanco, M R; Gutierrez, L

    1994-06-01

    This work describes the development of sideroblastic anemia (SA) during the clinical activity of systemic lupus erythematosus (SLE) in two patients. The cases refer to two women with SLE who developed SA during a relapse of the illness. Both patients fulfilled at least four criteria of the American Association of Rheumatology for SLE. In one patient, the treatment with prednisone was followed by the resolution of the SLE activity and the disappearance of the SA. Several years later there was no evidence of ringed sideroblasts or malignancy in the bone marrow. In the other patient, the clinical activity of the SLE and SA developed during the beginning of a septicemic event which finally led to her death. Our clinical cases allow us to show that the transitory development of SA can occur during a period of clinical activity of SLE and that its association, although infrequent, is due to a common physiopathogenic mechanism.

  5. The Real Culprit in Systemic Lupus Erythematosus: Abnormal Epigenetic Regulation

    Science.gov (United States)

    Wu, Haijing; Zhao, Ming; Chang, Christopher; Lu, Qianjin

    2015-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organs and the presence of anti-nuclear antibodies. The pathogenesis of SLE has been intensively studied but remains far from clear. B and T lymphocyte abnormalities, dysregulation of apoptosis, defects in the clearance of apoptotic materials, and various genetic and epigenetic factors are attributed to the development of SLE. The latest research findings point to the association between abnormal epigenetic regulation and SLE, which has attracted considerable interest worldwide. It is the purpose of this review to present and discuss the relationship between aberrant epigenetic regulation and SLE, including DNA methylation, histone modifications and microRNAs in patients with SLE, the possible mechanisms of immune dysfunction caused by epigenetic changes, and to better understand the roles of aberrant epigenetic regulation in the initiation and development of SLE and to provide an insight into the related therapeutic options in SLE. PMID:25988383

  6. Pharmacokinetic modeling of therapies for systemic lupus erythematosus

    Science.gov (United States)

    Yang, Xiaoyan; Sherwin, Catherine MT; Yu, Tian; Yellepeddi, Venkata K; Brunner, Hermine I; Vinks, Alexander A

    2017-01-01

    With the increasing use of different types of therapies in treating autoimmune diseases such as systemic lupus erythematosus (SLE), there is a need to utilize pharmacokinetic (PK) strategies to optimize the clinical outcome of these treatments. Various PK analysis approaches, including population PK modeling and physiologically based PK modeling, have been used to evaluate drug PK characteristics and population variability or to predict drug PK profiles in a mechanistic manner. This review outlines the PK modeling of major SLE therapies including immunosuppressants (methotrexate, azathioprine, mycophenolate and cyclophosphamide, among others) and immunomodulators (intravenous immunoglobulin). It summarizes the population PK modeling, physiologically based PK modeling and model-based individualized dosing strategies to improve the therapeutic outcomes in SLE patients. PMID:26143647

  7. Why Targeted Therapies are Necessary for Systemic Lupus Erythematosus

    Science.gov (United States)

    Durcan, Laura; Petri, Michelle

    2016-01-01

    Systemic lupus erythematosus (SLE) continues to have important morbidity and accelerated mortality despite therapeutic advances. Targeted therapies offer the possibility of improved efficacy with fewer side-effects. Current management strategies rely heavily on non-specific immunosuppressive agents. Prednisone, in particular, is responsible for a considerable burden of later organ damage. There are a multitude of diverse mechanisms of disease activity, immunogenic abnormalities and clinical manifestations to take into consideration in SLE. Many targeted agents with robust mechanistic pre-clinical data and promising early phase studies have ultimately been disappointing in phase III randomized controlled studies. Recent efforts have focused on B cell therapies, in particular given the success of belimumab in clinical trials, with limited success. We remain optimistic regarding other specific therapies being evaluated including interferon alpha blockade. It is likely that in SLE, given the heterogeneity of the population involved, precision medicine is needed, rather than expecting that any single biologic will be universally effective. PMID:27497251

  8. A critical review of clinical trials in systemic lupus erythematosus

    Science.gov (United States)

    Mahieu, Mary A.; Strand, Vibeke; Simon, Lee S.; Lipsky, Peter E.; Ramsey-Goldman, Rosalind

    2016-01-01

    One challenge in caring for patients with systemic lupus erythematosus (SLE) is a paucity of approved therapeutics for treatment of the diverse disease manifestations. In the last 60 years, only one drug, belimumab, has been approved for SLE treatment. Critical evaluation of investigator initiated and pharma-sponsored randomized controlled trials (RCTs) highlights barriers to successful drug development in SLE, including disease heterogeneity, inadequate trial size or duration, insufficient dose finding before initiation of large trials, handling of background medications, and choice of primary endpoint. Herein we examine lessons learned from landmark SLE RCTs and subsequent advances in trial design, as well as discuss efforts to address limitations in current SLE outcome measures that will improve detection of true therapeutic responses in future RCTs. PMID:27497257

  9. Treatment of osteonecrosis in systemic lupus erythematosus: a review.

    Science.gov (United States)

    Ehmke, T Andrew; Cherian, Jeffrey J; Wu, Eddie S; Jauregui, Julio J; Banerjee, Samik; Mont, Michael A

    2014-01-01

    Osteonecrosis (ON) is a devastating illness that can lead to severe joint disease in young patients. The pathogenesis of ON is largely unknown; however, there have been numerous reports associating risk factors including systemic lupus erythematosus (SLE) with the disease. The risk of ON for SLE patients is believed to be a result of both the SLE disease state itself and the concomitant use of corticosteroids. The objective of osteonecrosis treatment is typically to halt progression or delay the onset of end-stage arthritis that may require a total joint arthroplasty (TJA). Joint-preserving procedures are attempted for pre-collapse and some post-collapse lesions. After severe subchondral collapse has occurred, TJA is often necessary to relieve pain. The purpose of this article is to draw attention to recent evidence regarding several treatment options for the management of SLE-associated ON, including lesion observation, medication, joint-preserving techniques, and TJA.

  10. Resilience and Treatment Adhesion in Patients with Systemic Lupus Erythematosus

    Science.gov (United States)

    Faria, Daniella Antunes Pousa; Revoredo, Luciana Silva; Vilar, Maria José; Eulália Maria Chaves, Maia

    2014-01-01

    Background: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune, rheumatic inflammatory disease that can cause significant morbidity with evident psychological impacts and obvious harm to quality-of-life that require the patient to adapt treatment. Objective: Assessment of resilience and the self-reported treatment adhesion behaviors of patients with SLE, investigating which of these factors are associated to resilience. Method: Cross-sectional study of 40 women with SLE. A questionnaire with social demographic data, health history and the Wagnild Young Resilience Scale were used. Results: 62.5% followed the medical treatment properly but 55% found it difficult. 27.5% of the patients presented low resilience, 57.5% medium and 15% high resilience. Resilience was associated in the chi-square test (p-value individual capacities to learn how to tackle with the disease for which psychological support of family and doctors can play a significant role. PMID:24665352

  11. Systemic lupus erythematosus with hepatosplenic granuloma: a rare case.

    Science.gov (United States)

    Bharti, Anju; Meena, Lalit Prashant

    2014-01-01

    Background. Systemic lupus erythematosus (SLE) is an autoimmune disease which is known to present with a wide variety of clinical manifestations. Case Report. A 15-year-old male presented with complaints of moderate grade fever and generalized body swelling. There was no history of cough, weight loss, joint pain, oral ulcerations, skin rash, photosensitivity, loss of hair, pain abdomen, jaundice, or any significant illness in the past. Contrast enhanced computerized tomography of the abdomen revealed hypodense lesions in both liver and spleen (without contrast enhancement), suggestive of granulomas along with few retroperitoneal and mesenteric lymph nodes. On the basis of immunological tests and renal biopsy report, SLE with hepatosplenic granulomatosis diagnosis was made. He was given pulse methylprednisolone 500 mg, for 3 days and he showed dramatic improvement clinically. Conclusion. Hepatic and splenic granulomas are not common in SLE, but this should be kept in differential diagnosis.

  12. USE OF MYCOPHENOLATE MOPHETYL IN PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    S.I. Valiyeva,

    2006-01-01

    Full Text Available The article reports a case of highly active SLE and lupusbnephritis in a 15 years old boy, who was treated with mycophenolate mophetyl the case was notable for high activity and aggressive course of the disease with rapid development of renal unsufficiency, polyorganic unsufficiency and antiphospholipid syndrome. Although the patient received an appropriate active therapy, including synchronized therapy (consisting of timebrelated plasmopherresis and infusions of cyclophosphamide and metyl prednisolone, glucocorticoides, preparations improving blood circulation (pentoxyphillin, dipiridamol, heparine, intravenous immunoglobulins, the disease activity control was unsufficient. The administration of mycophenolate mophetyl has led to diminuition of the disease activity, which was registered at the end of the second week of treatment, and finally has reached a level of clinical and laboratoty remission of the disease.Key words: systemic lupus erythematosus, mycophenolate mophetyl, children, treatment.

  13. Multichannel perimetric alterations in systemic lupus erythematosus treated with hydroxychloroquine.

    Science.gov (United States)

    Piñero, David P; Monllor, Begoña; Camps, Vicente J; de Fez, Dolores

    Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. We report the first case of SLE in which visual alterations were evaluated with multichannel perimetry. Some achromatic and color vision alterations may be present in SLE, especially when treated with hydroxychloroquine. The sensitivity losses detected in the chromatic channels in the central zone of the visual field were consistent with the results of the FM 100 Hue color test. Likewise, the multichannel perimetry detected sensitivity losses in the parafoveal area for both chromatic channels, especially for the blue-yellow. Copyright © 2016 Spanish General Council of Optometry. Published by Elsevier España, S.L.U. All rights reserved.

  14. Systemic Lupus Erythematosus with Hepatosplenic Granuloma: A Rare Case

    Directory of Open Access Journals (Sweden)

    Anju Bharti

    2014-01-01

    Full Text Available Background. Systemic lupus erythematosus (SLE is an autoimmune disease which is known to present with a wide variety of clinical manifestations. Case Report. A 15-year-old male presented with complaints of moderate grade fever and generalized body swelling. There was no history of cough, weight loss, joint pain, oral ulcerations, skin rash, photosensitivity, loss of hair, pain abdomen, jaundice, or any significant illness in the past. Contrast enhanced computerized tomography of the abdomen revealed hypodense lesions in both liver and spleen (without contrast enhancement, suggestive of granulomas along with few retroperitoneal and mesenteric lymph nodes. On the basis of immunological tests and renal biopsy report, SLE with hepatosplenic granulomatosis diagnosis was made. He was given pulse methylprednisolone 500 mg, for 3 days and he showed dramatic improvement clinically. Conclusion. Hepatic and splenic granulomas are not common in SLE, but this should be kept in differential diagnosis.

  15. Toxic Epidermal Necrolysis-Like Lesions and Systemic Lupus Erythematosus Possibly Triggered by Sulfasalazine

    DEFF Research Database (Denmark)

    Krabbe, Simon; Gül, Cigdem; Andersen, Bjarne

    2016-01-01

    elevated ferritin, and muscle wasting. A diagnosis of systemic lupus erythematosus was made, and mycophenolate mofetil and systemic glucocorticoids brought this severe disease under control. Toxic epidermal necrolysis-like lesions and hemophagocytic syndrome have been reported as manifestations of systemic...... lupus erythematosus. This patient possibly had spondyloarthritis or an undifferentiated connective tissue disease at presentation, and we suggest, based on the timing of events, that sulfasalazine may have acted as a trigger of the severe disease manifestations....

  16. Magnetic resonance imaging in neuropsychiatric systemic lupus erythematosus: current state of the art and novel approaches.

    Science.gov (United States)

    Postal, M; Lapa, A Tamires; Reis, F; Rittner, L; Appenzeller, S

    2017-04-01

    Systemic lupus erythematosus is a chronic, inflammatory, immune-mediated disease affecting 0.1% of the general population. Neuropsychiatric manifestations in systemic lupus erythematosus have been more frequently recognized and reported in recent years, occurring in up to 75% of patients during the disease course. Magnetic resonance imaging is known to be a useful tool for the detection of structural brain abnormalities in neuropsychiatric systemic lupus erythematosus patients because of the excellent soft-tissue contrast observed with MRI and the ability to acquire multiplanar images. In addition to conventional magnetic resonance imaging techniques to evaluate the presence of atrophy and white matter lesions, several different magnetic resonance imaging techniques have been used to identify microstructural or functional abnormalities. This review will highlight different magnetic resonance imaging techniques, including the advanced magnetic resonance imaging methods used to determine central nervous system involvement in systemic lupus erythematosus.

  17. Influence of Education on Disease Activity and Damage in Systemic Lupus Erythematosus: Data From the 1000 Canadian Faces of Lupus.

    Science.gov (United States)

    George, Angela; Wong-Pak, Andrew; Peschken, Christine A; Silverman, Earl; Pineau, Christian; Smith, C Douglas; Arbillaga, Hector; Zummer, Michel; Bernatsky, Sasha; Hudson, Marie; Hitchon, Carol; Fortin, Paul R; Nevskaya, Tatiana; Pope, Janet E

    2017-01-01

    To determine whether socioeconomic status assessed by education is associated with disease activity and the risk of organ damage in systemic lupus erythematosus (SLE). Data from the 1000 Canadian Faces of Lupus, a multicenter database of adult SLE patients, was used to compare education as either low (did not complete high school) or high (completed high school or further) for disease activity and damage. Education was also studied as a continuous variable. The relationships between education and SLE outcomes (any organ damage defined as a Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI] score ≥1, serious organ damage [SDI score ≥3], and end-stage renal disease) were evaluated using logistic regression analyses adjusted for age, sex, race/ethnicity, and disease duration. A total of 562 SLE patients met inclusion criteria (mean age 47 years, 91% female, and mean disease duration of 10 years); 81% had high education. The low education group was twice as likely to be work disabled (30%; P education was significantly associated with higher disease activity at enrollment into the 1000 Canadian Faces of Lupus database, after adjustment for age (at entry and at diagnosis), race/ethnicity, and sex (B 1.255 + 0.507 [SE], β = 0.115, P = 0.014). In our adjusted logistic regression models we were unable to demonstrate significant associations between education and SLE damage. Results did not change when varying the education variable. In this cohort, low education was associated cross-sectionally with higher disease activity and work disability, but not damage. © 2016, American College of Rheumatology.

  18. [Progress and perspectives in the treatment of systemic lupus erythematosus].

    Science.gov (United States)

    Robak, Ewa; Sysa-Jedrzejowska, Anna; Wozniacka, Anna

    2005-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that predominantly affects women in childbearing age. SLE tissue damage is mediated by autoantibodies, complement activation and immune complexes deposition. The disease is diagnosed on the basis of its clinical manifestations and the demonstration of characteristic immunological phenomena, especially antinuclear antibodies. Management of the disease includes regular monitoring of disease activity, avoidance of predisposing factors and therapy guided by the activity and severity of the leading organ manifestation. Treatment ranges from nonsteroidal antirheumatic drugs to intensive treatment with cytotoxic agents. Corticosteroids form the basis of all regimens. Antimalarials and azathioprine are important for treating mild and moderate SLE cases, especially for the long time. Cyclophosphamide given intravenously is the current gold standard for severe lupus nephritis. More recently new strategies for immunosuppression in SLE, that interfere with the syntesis of DNA and nucleotides have been developed (such as mycophenolate mofetil, fludarabine and cladribine). Other agents like cyclosporine and tacrolimus inhibit effect of the activation signals for T cells by inhibition of calcineurin. Some monoclonal antibodies against cytokines or components of the complement system interfere with the effector phase of the immune response. Abetimus (LJP-394) inhibits the production of anti-dsDNA antibodies and may prevent glomerulonephritis caused by anti-DNA containing immune complexes. Somatic gene therapy is also a novel approach in autoimmune disorders and my be a valuable method of SLE therapy in the future. The adrenal steroid prasterone (DHEA) has also shown benefitial effects in mild to moderate SLE. Finally, autologous stem cell transplantation can induce tolerance to self-antigens and cause significant improvement in SLE patients. However, new therapeutic strategies must be tested according to the established

  19. Metabolic control of the epigenome in systemic Lupus erythematosus.

    Science.gov (United States)

    Oaks, Zachary; Perl, Andras

    2014-06-01

    Epigenetic mechanisms are proposed to underlie aberrant gene expression in systemic lupus erythematosus (SLE) that results in dysregulation of the immune system and loss of tolerance. Modifications of DNA and histones require substrates derived from diet and intermediary metabolism. DNA and histone methyltransferases depend on S-adenosylmethionine (SAM) as a methyl donor. SAM is generated from adenosine triphosphate (ATP) and methionine by methionine adenosyltransferase (MAT), a redox-sensitive enzyme in the SAM cycle. The availability of B vitamins and methionine regulate SAM generation. The DNA of SLE patients is hypomethylated, indicating dysfunction in the SAM cycle and methyltransferase activity. Acetyl-CoA, which is necessary for histone acetylation, is generated from citrate produced in mitochondria. Mitochondria are also responsible for de novo synthesis of flavin adenine dinucleotide (FAD) for histone demethylation. Mitochondrial oxidative phosphorylation is the dominant source of ATP. The depletion of ATP in lupus T cells may affect MAT activity as well as adenosine monophosphate (AMP) activated protein kinase (AMPK), which phosphorylates histones and inhibits mechanistic target of rapamycin (mTOR). In turn, mTOR can modify epigenetic pathways including methylation, demethylation, and histone phosphorylation and mediates enhanced T-cell activation in SLE. Beyond their role in metabolism, mitochondria are the main source of reactive oxygen intermediates (ROI), which activate mTOR and regulate the activity of histone and DNA modifying enzymes. In this review we will focus on the sources of metabolites required for epigenetic regulation and how the flux of the underlying metabolic pathways affects gene expression.

  20. Posterior reversible encephalopathy syndrome and association with systemic lupus erythematosus.

    Science.gov (United States)

    Ferreira, T S; Reis, F; Appenzeller, S

    2016-10-01

    Posterior reversible encephalopathy syndrome (PRES) is a neurological complex disorder with many clinical associations and causative factors. It is important to recognize this condition because early diagnosis and treatment usually result in its complete resolution, radiological imaging becoming the key for the correct diagnosis. We retrospectively reviewed charts and magnetic resonance imaging findings in the University of Campinas from January 2005 to July 2015, selecting three cases of patients with systemic lupus erythematosus syndrome who developed PRES, for whom risk factors, characteristics, magnetic resonance imaging findings and neurological resolution were analyzed. We also conducted a review of the English-language literature. The three cases had neurological symptoms like acute onset of headache, altered mental status, cortical blindness and seizures. Brain magnetic resonance imaging demonstrated posterior cortical and white matter alterations involving posterior brain territories, which were more conspicuous on T2-weighted and fluid-attenuated inversion recovery. Spectroscopy, diffusion-weighted imaging and susceptibility-weighted imaging were also important for neuroradiological evaluation. Immunosuppressive drugs were taken in all cases. Partial clinical and radiological recovery was observed in two cases, and complete resolution was observed in the third patient. We found 52 cases of PRES in systemic lupus erythematosus patients. Almost all patients were women 94%, ranging from 8 to 62 years old. Posterior brain territory involvements were found in 98% of patients. Hemorrhagic complications involved 26% of patients, becoming a risk factor for clinical sequels. The total percentage of patients with no complete resolution of radiological findings on follow-up images was 27.5%. In patients with autoimmune disorders, endothelial dysfunction may occur secondary to autoimmunity and the use of cytotoxic drugs, supposedly facilitating the occurrence of more

  1. Pathogenic Inflammation and Its Therapeutic Targeting in Systemic Lupus Erythematosus

    Science.gov (United States)

    Gottschalk, Timothy A.; Tsantikos, Evelyn; Hibbs, Margaret L.

    2015-01-01

    Systemic lupus erythematosus (SLE, lupus) is a highly complex and heterogeneous autoimmune disease that most often afflicts women in their child-bearing years. It is characterized by circulating self-reactive antibodies that deposit in tissues, including skin, kidneys, and brain, and the ensuing inflammatory response can lead to irreparable tissue damage. Over many years, clinical trials in SLE have focused on agents that control B- and T-lymphocyte activation, and, with the single exception of an agent known as belimumab which targets the B-cell survival factor BAFF, they have been disappointing. At present, standard therapy for SLE with mild disease is the agent hydroxychloroquine. During disease flares, steroids are often used, while the more severe manifestations with major organ involvement warrant potent, broad-spectrum immunosuppression with cyclophosphamide or mycophenolate. Current treatments have severe and dose-limiting toxicities and thus a more specific therapy targeting a causative factor or signaling pathway would be greatly beneficial in SLE treatment. Moreover, the ability to control inflammation alongside B-cell activation may be a superior approach for disease control. There has been a recent focus on the innate immune system and associated inflammation, which has uncovered key players in driving the pathogenesis of SLE. Delineating some of these intricate inflammatory mechanisms has been possible with studies using spontaneous mouse mutants and genetically engineered mice. These strains, to varying degrees, exhibit hallmarks of the human disease and therefore have been utilized to model human SLE and to test new drugs. Developing a better understanding of the initiation and perpetuation of disease in SLE may uncover suitable novel targets for therapeutic intervention. Here, we discuss the involvement of inflammation in SLE disease pathogenesis, with a focus on several key proinflammatory cytokines and myeloid growth factors, and review the known

  2. Systemic lupus erythematosus in the Fars Province of Iran.

    Science.gov (United States)

    Nazarinia, M A; Ghaffarpasand, F; Shamsdin, A; Karimi, A A; Abbasi, N; Amiri, A

    2008-03-01

    Clinical features of systemic lupus erythematosus (SLE) have been described from different geographical regions in the world. However, data from many Middle East countries, including Iran, are scarce. This study aims to demonstrate the demographic, clinical, and laboratory characteristics in Iranian patients with SLE. In this prospective study, all the patients referring to Shiraz educational hospitals (Nemazi-Hafez) with SLE (American College of Rheumatology criteria) during a 5-year period (2001 to 2006) were included. A complete history was taken; physical examination and routine hematological, serological, and immunological tests were done for each patient. There were 356 women and 54 men with an average age of 30.27 years at the onset of disease. Of the patients, 78% had hematological abnormalities, 65.5% had articular involvement, 54.5% had photosensitivity, and 60.5% had malar rash. Serositis occurred in 38% of patients of whom 12% had pericarditis and 26% had pleuritis. Nephritis was diagnosed in 48% of the cases and consisted always of glomerular nephritis. Biopsy-proven lupus nephritis was in most cases class IV(49.7% of all the biopsies). Oral ulcers were observed in 28% of patients. Neuropsychiatric manifestations, gastrointestinal involvement, and lymphadenopathy were observed in 31.5%, 8.3%, and 14.2% of patients, respectively. In all, 93% of patients were positive for antinuclear antibodies, whereas antidouble-stranded DNA was positive in 83% of patients. Coomb's positive hemolytic anemia appeared in 12.4% of the cases. Rheumatoid factor was detected in 9.7% of patients, and lupus erythematosus cell was seen in 32.5% of them. In all, 196 (47.8%) patients represented hypocomplementemia. Regarding hematological manifestations, 74.5% had microcytic hypochromic anemia, 64.6% had leukopenia, and 44.6% had thrombocytopenia; 18 (4.4%) patients died during the study period of which eight (2%) died because of cardiopulmonary involvement. Generally, there was

  3. Low prevalence of Pneumocystis pneumonia in hospitalized patients with systemic lupus erythematosus: review of a clinical data warehouse.

    Science.gov (United States)

    Kapoor, T M; Mahadeshwar, P; Nguyen, S; Li, J; Kapoor, S; Bathon, J; Giles, J; Askanase, A

    2017-12-01

    Objective In the era of powerful immunosuppression, opportunistic infections are an increasing concern in systemic lupus erythematosus. One of the best-studied opportunistic infections is Pneumocystis pneumonia; however, the prevalence of Pneumocystis pneumonia in systemic lupus erythematosus is not clearly defined. This study evaluates the prevalence of Pneumocystis pneumonia in hospitalized systemic lupus erythematosus patients, with a focus on validating the Pneumocystis pneumonia and systemic lupus erythematosus diagnoses with clinical information. Methods This retrospective cohort study evaluates the prevalence of Pneumocystis pneumonia in all systemic lupus erythematosus patients treated at Columbia University Medical Center-New York Presbyterian Hospital between January 2000 and September 2014, using electronic medical record data. Patients with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) and patients with renal transplants (including both early and late post-transplant patients) represented immunocompromised control groups. Patients with systemic lupus erythematosus, Pneumocystis pneumonia, HIV/AIDS, or renal transplant were identified using diagnostic codes from the International Classification of Diseases, Ninth Revision (ICD-9). Results Out of 2013 hospitalized systemic lupus erythematosus patients, nine had presumed Pneumocystis pneumonia, yielding a low prevalence of Pneumocystis pneumonia in systemic lupus erythematosus of 0.45%. Three of the nine Pneumocystis pneumonia cases were patients with concomitant systemic lupus erythematosus and HIV/AIDS. Only one of these nine cases was histologically confirmed as Pneumocystis pneumonia, in a patient with concomitant systemic lupus erythematosus and HIV/AIDS and a CD4 count of 13 cells/mm 3 . The prevalence of Pneumocystis pneumonia in renal transplant patients and HIV/AIDS patients was 0.61% and 5.98%, respectively. Conclusion Given the reported high rate of adverse effects

  4. Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature.

    Science.gov (United States)

    Chiewchengchol, Direkrit; Murphy, Ruth; Edwards, Steven W; Beresford, Michael W

    2015-01-01

    Patients diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) often have skin and oral lesions as part of their presentation. These mucocutaneous lesions, as defined by the American College of Rheumatology (ACR) in 1997, include malar rash, discoid rash, photosensitivity and oral ulcers. It is therefore essential to recognize mucocutaneous lesions to accurately diagnose JSLE. The mucocutaneous lesions can be divided into those with classical histological features (LE specific) and those strongly associated with and forming part of the diagnostic spectrum, but without the classical histological changes of lupus (LE nonspecific). A malar rash is the most commonly associated LE specific dermatological presentation. This skin manifestation is an acute form and also correlates with disease activity. Subacute (polycyclic or papulosquamous lesions) and chronic (discoid lesions) forms, whilst showing classical histological changes supportive of lupus, are less commonly associated with systemic lupus and do not correlate with disease activity. The most commonly associated skin lesions without classical lupus changes are cutaneous vasculitis, oral ulcers and diffuse non-scarring alopecia. These signs frequently relate to disease activity. An understanding of cutaneous signs and symptoms of lupus in children is important to avoid delay in diagnosis. They will often improve as lupus is adequately controlled and their reappearance is often the first indicator of a disease flare.

  5. Pauci-immune and Immune Glomerular Lesions in Kidney Transplants for Systemic Lupus Erythematosus

    OpenAIRE

    Meehan, Shane M.; Chang, Anthony; Khurana, Amandeep; Baliga, Rajendra; Kadambi, Pradeep V.; Javaid, Basit

    2008-01-01

    Background and objectives: Glomerular lesions in allografts in recipients with end-stage nephritis resulting from systemic lupus erythematosus (SLE) were examined to determine the spectrum of glomerular pathology in recurrent glomerulonephritis (GN).

  6. Cardiovascular disease in systemic lupus erythematosus: has the time for action come?

    NARCIS (Netherlands)

    van Leuven, Sander I.; Kastelein, John J. P.; Hayden, Michael R.; d'Cruz, David; Hughes, Graham R.; Stroes, Erik S.

    2005-01-01

    PURPOSE OF REVIEW: The recognition that inflammation is a hallmark of atherosclerotic disease has led to a series of studies reporting accelerated atherogenesis in chronic inflammatory diseases. Indeed, systemic lupus erythematosus is associated with an increased incidence of cardiovascular disease

  7. Antibodies to early EBV, CMV, and HHV6 antigens in systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Rasmussen, N S; Draborg, A H; Nielsen, C T

    2015-01-01

    OBJECTIVES: We investigated the antibody levels against early antigens of Epstein-Barr virus (EBV), cytomegalovirus (CMV), and human herpesvirus 6 (HHV6) in systemic lupus erythematosus (SLE) patients and healthy controls, and further correlated these antibodies to haematology...

  8. [Magnetic resonance angiography in the diagnosis of cerebrovascular diseases in systemic lupus erythematosus].

    Science.gov (United States)

    Pizova, N V; Spirin, N N; Maksimov, G A; Bakhtin, A L

    2004-01-01

    Fifty one patients with systemic lupus erythematous were examined using magnetic resonance angiography (MRA) to determine cerebral hemodynamic features. A comprehensive study revealed different cerebral circulatory changes in this abnormality.

  9. Noma management in a child with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Irna Sufiawati

    2010-03-01

    Full Text Available Background: Noma, also known as cancrum oris, is an orofacial gangrene, which during its fulminating stage causes progressive and mutilating destruction of the infected tissues. The disease occurs mainly in children with malnutrition, poor oral hygiene and debilitating concurrent illness. Purpose: The aim of this paper was to report a unique case of noma associated with systemic lupus erythematosus in an 8-year-old boy. Case: An 8-year-old boy referred to Oral Medicine Department complaining about an ulcer at the left corner of his mouth for 1 month, painful and difficulty in opening the mouth. The patient was diagnosed systemic lupus erythematosus since 14 months before and had been given immunosuppressive therapy. The patient was also diagnosed severe malnutrition. Haematologic investigations revealed anemia. Case management: Panoramic radiography was performed to check for dental or periodontal foci of infection, but no abnormalities were present. The microbiology examination revelaed Fusobacterium necrophorum, Staphylococcus aureus, and Klabsiella. The patient has been treated with oral irrigation using hydrogen peroxide, saline and 0.2% chlorhexidine, thus helped to slough the necrotic tissue. Oral antibiotics and analgesics were prescribed. The patient was admitted to hospital under the care of a pediatrician, allergy and immunology specialist, and a nutritionist. The result of the comprehensive disease management showed that the lesion healed completely, but leaving a scar on his corner of the mouth. Its physical effects are permanent and may require reconstructive surgery to be repaired by oral surgeon. Conclusion: Noma is not a primary disease, there are various predisposing factors usually precede its occurrence. The management of noma requires a multidisciplinary approach.Latar Belakang: Noma, dikenal sebagai cancrum oris, adalah gangren pada daerah orofasial, yang menyebabkan kerusakan progresif dari jaringan yang terinfeksi

  10. Validation of the systemic lupus erythematosus responder index for use in juvenile-onset systemic lupus erythematosus.

    Science.gov (United States)

    Mina, Rina; Klein-Gitelman, Marisa S; Nelson, Shannen; Eberhard, B Anne; Higgins, Gloria; Singer, Nora G; Onel, Karen; Tucker, Lori; O'Neil, Kathleen M; Punaro, Marilynn; Levy, Deborah M; Haines, Kathleen; Martini, Alberto; Ruperto, Nicolino; Lovell, Daniel; Brunner, Hermine I

    2014-02-01

    This study tested the concurrent validity of the systemic lupus erythematosus responder index (SRI) in assessing improvement in juvenile-onset systemic lupus erythematosus (jSLE). The SRI considers changes in the SELENA-SLEDAI, BILAG and a 3-cm visual analogue scale of physician-rated disease activity (PGA) to determine patient improvement. Using prospectively collected data from 760 unique follow-up visit intervals of 274 jSLE patients, we assessed the sensitivity and specificity of the SRI using these external standards: physician-rated improvement (MD-change), patient/parent-rated major improvement of wellbeing (patient-change) and decrease in prescribed systemic corticosteroids (steroid-change). Modifications of the SRI that considered different thresholds for the SELENA-SLEDAI, BILAG and 10-cm PGA were explored and agreement with the American College of Rheumatology/PRINTO provisional criteria for improvement of jSLE (PCI) was examined. The sensitivity/specificity in capturing major improvement by the MD-change were 78%/76% for the SRI and 83%/78% for the PCI, respectively. There was fair agreement between the SRI and PCI (kappa=0.35, 95% CI 0.02 to 0.73) in capturing major improvement by the MD-change. Select modified versions of the SRI had improved accuracy overall. All improvement criteria tested had lower sensitivity when considering patient-change and steroid-change as external standards compared to MD-change. The SRI and its modified versions based on meaningful changes in jSLE have high specificity but at most modest sensitivity for capturing jSLE improvement. When used as an endpoint of clinical trials in jSLE, the SRI will provide a conservative estimate regarding the efficacy of the therapeutic agent under investigation.

  11. Clinical features and antinuclear antibodies profile among adults with systemic lupus erythematosus and lupus nephritis: a cross-sectional study.

    Science.gov (United States)

    Ahmed, Nashwa; Shigidi, Mazin; Al Agib, Al Nour; Abdelrahman, Hassan; Taha, Elshafie

    2017-01-01

    Limited data is available regarding the clinical manifestations and pattern of Systemic Lupus Erythematosus (SLE) in Sudan. This study aimed to determine the clinical manifestations and Antinuclear Antibodies (ANA) profile among Sudanese adults with SLE and lupus nephritis (LN). A descriptive study was conducted in Omdurman Military Hospital, Sudan. It included all adults with SLE and on regular follow-up during the study period (December 2012 to May 2013). These were investigated regarding their demographic details, clinical features, and immunological profile (ANA, anti-double stranded DNA, and ANA profile 3 levels). Patients with LN had their pattern of renal involvement described; furthermore, associations between the various SLE reactive antibodies and the histological diagnosis of lupus were studied. Sixty-two Sudanese adults with SLE were included, their mean age was 31 ± 10.9 year. Females made 93.5% of patients. A clear predominance of those of Arab ancestry was seen, with most patients being from the Ja'alin and Shaigiya ethnic groups accounting for 29% and 12.9%, respectively. Arthritis was the dominant clinical manifestation seen in 85.5%, whereas renal involvement was seen in 66.1% of patients. Lupus nephritis class III was the dominant histological lesion, seen in 39% of patients. On correlating the ANA profile to the histopathological diagnosis of LN, anti-Nucleosomes and anti-AMA-M2 autoantibodies were found to be significantly associated with LN class IV and class VI, respectively (P values < 0.05). Further epidemiological studies regarding SLE and its ANA profile remain essential as they might help predicting the clinical patterns of the disease and its prognosis.

  12. Immunological and molecular analysis of three monoclonal lupus anticoagulant antibodies from a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Lai, C J; Rauch, J; Cho, C S; Zhao, Y; Chukwuocha, R U; Chen, P P

    1998-02-01

    Antiphospholipid antibodies (APA), including lupus anticoagulants (LAC; as detected by in vitro blood clotting tests) and anti-cardiolipin antibodies (ACA; as assayed by solid-phase immunoassay), are strongly associated with recurrent thrombosis, thrombocytopenia, and recurrent fetal loss in some patients with systemic lupus erythematosus (SLE). The combined presence of APA and these clinical manifestations is termed antiphospholipid syndrome (APS). LAC and ACA comprise heterogeneous and somewhat overlapping autoantibody subsets. To date, it is unclear what degree of heterogeneity is present in an individual patient and between patients. To begin to address these issues, we generated three monoclonal LAC antibodies from a patient with SLE and APS. These antibodies were studied for their binding specificities and variable (V) region nucleotide sequences. All three LAC were unreactive with DNA, cardiolipin or other phospholipids. Sequence analysis of these antibodies revealed extensive overlap in their Ig V genes with anti-DNA antibodies and other autoantibodies characteristic of lupus. These data provide the first V gene sequence information on a group of SLE-derived LAC without ACA activity, representative of a similar subset of LAC found in patients with APS. Copyright 1998 Academic Press Limited.

  13. Mycophenolate mofetil as maintenance therapy for childhood-onset systemic lupus erythematosus patients with severe lupus nephritis.

    Science.gov (United States)

    Kizawa, Toshitaka; Nozawa, Tomo; Kikuchi, Masako; Nagahama, Kiyotaka; Okudela, Koji; Miyamae, Takako; Imagawa, Tomoyuki; Nakamura, Tomoko; Mori, Masaaki; Yokota, Shumpei; Tsutsumi, Hiroyuki

    2015-03-01

    We evaluated histological changes occurring in renal biopsy specimens, between the time before initial induction therapy and after 12 months' maintenance therapy, as well as changes in laboratory parameters, SLE disease activity (SLEDAI), and dosage of corticosteroid (CS) in childhood-onset systemic lupus erythematosus (SLE) patients treated with mycophenolate mofetil (MMF). A retrospective analysis was performed on nine patients diagnosed with childhood-onset SLE and lupus nephritis. They were treated with pulsed mPSL and intravenous cyclophosphamide as induction therapy and MMF (500-1500 mg/day) plus CS as maintenance therapy. Renal biopsy was performed before the initial induction therapy and after 12 months' maintenance therapy. Pathological findings at second biopsy were improved in eight of nine patients (89%). The findings of SLEDAI, urinalysis, and blood tests also showed improvement. CS doses could be tapered satisfactorily. Adverse events were observed in two patients. No patients treated with MMF experienced any disease flares during maintenance therapy. MMF as maintenance therapy might be useful in that not only the histological findings of lupus nephritis were improved, but also CS doses could be beneficially tapered. Nonetheless, this is a retrospective report of only nine cases and further prospective multicenter studies are necessary.

  14. Thrombosis in systemic lupus erythematosus: congenital and acquired risk factors.

    Science.gov (United States)

    Afeltra, Antonella; Vadacca, Marta; Conti, Laura; Galluzzo, Sara; Mitterhofer, Anna P; Ferri, Giovanni M; Del Porto, Flavia; Caccavo, Domenico; Gandolfo, Giuseppe M; Amoroso, Antonio

    2005-06-15

    To investigate the thrombotic tendency in patients with systemic lupus erythematosus (SLE) by evaluating congenital or acquired abnormalities associated with an increased risk of venous and/or arterial thrombosis. A total of 57 patients with SLE were included in the study. Twenty-one patients (37%) had a history of arterial and/or venous thrombosis and 36 patients (63%) did not have such a history. Sera from 50 healthy controls were examined. Protein C, protein S, antithrombin, D-dimer, fibrinogen, homocysteine, anticardiolipin antibodies (aCL), lupus anticoagulant (LAC), prothrombin G20210A, and methylenetetrahydrofolate reductase (MTHFR) C677T gene mutation were evaluated. Protein C, antithrombin, fibrinogen, D-dimer, and homocysteine levels were significantly higher in patients with SLE than in controls. A prothrombin mutation was observed in 2 (4%) of 50 controls and in 6 (11%) of 57 patients. A significantly higher prevalence (P = 0.036) of MTHFR homozygous mutation was observed in patients with SLE (14 [25%] of 57) in comparison with controls (4 [8%] of 50). IgG-aCL and IgM-aCL levels were significantly higher in patients with SLE than in controls (P or = 20 IgG phospholipid units/ml) IgG-aCL antibody titers was significantly higher (P = 0.005) in patients with thrombosis (11 [52%] of 21) than in patients without (5 [14%] of 36) thrombosis. LAC was present in 22 (38.5%) of 57 patients and in none of 50 controls. In this study, we confirm the association between thrombosis and IgG-aCL at medium-high titers and suggest that the coexistence of other risk factors can affect the expression of thrombosis in patients with SLE.

  15. Placental pathology in systemic lupus erythematosus with antiphospholipid antibodies.

    Science.gov (United States)

    Ogishima, D; Matsumoto, T; Nakamura, Y; Yoshida, K; Kuwabara, Y

    2000-03-01

    Systemic lupus erythematosus (SLE) is associated with a poor pregnancy outcome. Antiphospholipid antibodies (APL), which include lupus anticoagulant (LAC) and anticardiolipin antibodies (aCL), are frequently found in patients with SLE, and their presence has been associated with fetal loss. To examine placental pathologic features of SLE patients with APL, we performed a pathologic study on 47 placental tissue samples from 47 pregnant SLE patients with APL (15 patients; four LAC single-positive patients, seven aCL single-positive patients, four LAC and aCL double-positive patients) and without APL (32 LAC and aCL double-negative patients). The incidence of extensive infarction, decidual vasculopathy, decidual thrombosis and perivillous fibrinoid change, which have been thought to be characteristic lesions of APL placenta, was significantly higher in the LAC and aCL double-positive patients than in the patients without APL. Conversely, the above-mentioned lesions between the LAC or aCL single-positive patients and the APL negative patients did not differ significantly. Among the 15 patients with APL, two of the three patients with both decidual vasculopathy and thrombosis had extensive infarction associated with fetal death. Moreover, the patients having fetal death showed LAC and aCL double-positivity. In conclusion, this study indicated that the LAC and aCL double-positivity is an important factor for extensive infarction resulting from decidual vasculopathy and decidual thrombosis in the SLE placenta. Moreover, it was indicated that LAC and aCL double-positivity is an important risk factor for fetal death in the SLE patient.

  16. Cardiovascular disease and cognitive dysfunction in systemic lupus erythematosus.

    Science.gov (United States)

    Murray, Sara G; Yazdany, Jinoos; Kaiser, Rachel; Criswell, Lindsey A; Trupin, Laura; Yelin, Edward H; Katz, Patricia P; Julian, Laura J

    2012-09-01

    Cognitive dysfunction and cardiovascular disease are common and debilitating manifestations of systemic lupus erythematosus (SLE). In this study, we evaluated the relationship between cardiovascular events, traditional cardiovascular risk factors, and SLE-specific risk factors as predictors of cognitive dysfunction in a large cohort of participants with SLE. Subjects included 694 participants from the Lupus Outcomes Study (LOS), a longitudinal study of SLE outcomes based on an annual telephone survey querying demographic and clinical variables. The Hopkins Verbal Learning Test-Revised and the Controlled Oral Word Association Test were administered to assess cognitive function. Multiple logistic regression was used to identify cardiovascular events (myocardial infarction, stroke), traditional cardiovascular risk factors (hypertension, hyperlipidemia, diabetes mellitus, obesity, smoking), and SLE-specific risk factors (antiphospholipid antibodies [aPL], disease activity, disease duration) associated with cognitive impairment in year 7 of the LOS. The prevalence of cognitive impairment as measured by verbal memory and verbal fluency metrics was 15%. In adjusted multiple logistic regression analyses, aPL (odds ratio [OR] 2.10, 95% confidence interval [95% CI] 1.3-3.41), hypertension (OR 2.06, 95% CI 1.19-3.56), and a history of stroke (OR 2.27, 95% CI 1.16-4.43) were significantly associated with cognitive dysfunction. In additional analyses evaluating the association between these predictors and severity of cognitive impairment, stroke was significantly more prevalent in participants with severe impairment when compared to those with mild or moderate impairment (P = 0.036). These results suggest that the presence of aPL, hypertension, and stroke are key variables associated with cognitive impairment, which may aid in identification of patients at greatest risk. Copyright © 2012 by the American College of Rheumatology.

  17. Obesity and Cytokines in Childhood-Onset Systemic Lupus Erythematosus

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    Nailú Angélica Sinicato

    2014-01-01

    Full Text Available Background. In systemic lupus erythematosus (SLE, atherosclerosis is attributed to traditional and lupus related risk factors, including metabolic syndrome (MetS, obesity, and inflammation. Objective. To evaluate the association between obesity, measures of body fat content, serum tumor necrosis factor alpha (TNF-α, and interleukin (IL-6 and -10 levels in childhood-onset SLE (cSLE. Methods. We screened consecutive cSLE patients followed up in the Pediatric Rheumatology Outpatient Clinic of the State University of Campinas. cSLE patients were assessed for disease and damage. Obesity was definite as body mass index (BMI ≥30 kg/m2. Serum TNF-α, IL-6, and IL-10 levels were measured by ELISA. Dual-energy X-ray absorptiometry was used to determine total fat mass, lean mass, and percent of body fat. Results. We included 52 cSLE patients and 52 controls. cSLE patients had higher serum TNF-α  (P=0.004, IL-6 (P=0.002, and IL-10 (P<0.001 levels compared to controls. We observed higher serum TNF-α  (P=0.036 levels in cSLE patients with obesity. An association between serum TNF-α levels and body fat percent (P=0.046 and total fat mass on trunk region (P=0.035 was observed. Conclusion. Serum TNF-α levels were associated with obesity and body fat content in cSLE. Our finding suggests that obesity may contribute to the increase of serum TNF-α levels in cSLE.

  18. Factors associated with metabolic syndrome in patients with systemic lupus erythematosus from Puerto Rico

    OpenAIRE

    Negrón, AM; Molina, MJ; Mayor, AM; Rodriguez, VE; Vilá, LM

    2008-01-01

    The aim of this study was to determine the factors associated with metabolic syndrome in patients with systemic lupus erythematosus from Puerto Rico. A total of 204 patients with systemic lupus erythematosus (per the American College of Rheumatology classification criteria) were evaluated. Metabolic syndrome was assessed using the American Heart Association and the National Heart, Lung, and Blood Institute classification. Socioeconomic–demographic parameters, health-related behaviours, clinic...

  19. SYSTEMIC LUPUS ERITHEMATOSUS (SLE: KELAINAN AUTOIMUN BAWAAN YANG LANGKA DAN MEKANISME BIOKIMIAWINYA

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    Evi Roviati

    2012-04-01

    Full Text Available Belum banyak orang yang mengenal penyakit lupus atau Systemic Lupus Erithematosus (SLE, karena memang penyakit langka. Namun akhir-akhir ini ada kecenderungan peningkatan prevalensi terjadinya kasus lupus ini.  Gejala yang paling mudah dikenali adalah adanya bercak merah di sekitar wajah yang menyerupai kupu-kupu yang disebut “Butterfly Rush”.  Mekanisme biokimiawinya belum banyak diketahui, namun penyebab pastinya adalah disfungsional sistem imun.  Kelainan ini disebabkan adanya mutasi pada gen-gen yang menentukan permukaan sel limpfosit, sehingga antibodi menyerang bagian-bagian tubuh sendiri, atau yang disebut dengan autoimun.

  20. Aggressive Cardiac Involvement in Systemic Lupus Erythematosus: A Case Report and a Comprehensive Literature Review

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    Reza Ashrafi

    2011-01-01

    Full Text Available Background. We present the case of a 35-year-old gentleman who presented with an aggressive cardiomyopathy with normal coronary arteries. He was later diagnosed with systemic lupus-related cardiomyopathy. Methods. We undertook an extensive review of the literature regarding cardiac manifestations of lupus and used over 100 journals to identify the key points in pathology, diagnosis, and treatment. Results. We have shown that cardiac lupus can be rapidly progressive and, unless treated early, can have severe consequences. The predominant pathologies are immune complex and accelerated atherosclerosis drive. Treatment comprised of high-level immunosuppression.

  1. Health-related quality of life assessed by LupusQoL questionnaire and SF-36 in Turkish patients with systemic lupus erythematosus.

    Science.gov (United States)

    Yilmaz-Oner, Sibel; Oner, Can; Dogukan, Fatih Mert; Moses, Toklong Filam; Demir, Kubra; Tekayev, Nazar; Atagunduz, Pamir; Tuglular, Serhan; Direskeneli, Haner

    2016-03-01

    The LupusQoL is a disease-specific health-related quality of life (HRQoL) measure for patients with lupus. We conducted this study to compare the efficiency of LupusQoL-TR (validated Turkish version of the LupusQoL questionnaire) with the 36-item Short-Form Health Survey (SF-36), a generic quality of life (QoL) scale, in Turkish patients with lupus. Both questionnaires were conducted at a single visit to the clinic. Disease activity was measured with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Associations between the LupusQoL-TR and SF-36 domains were examined while also examining age, disease duration, and disease activity for each questionnaire. Descriptive statistics, Spearman's correlation coefficients, and Students t test were performed to analyze the data. A total of 113 consecutive patients with lupus (F/M 108:5, mean age 40.6 ± 11.9 years, mean disease duration 8.5 ± 7.0 years) were included, and 69 % of these were active. The median SLEDAI score was 2 (0-24), the mean global LupusQoL-TR score was 60.9 ± 23.3, and the mean SF-36 score was 41.2 ± 9.0. There was a significant correlation between LupusQoL-TR and SF-36 mean scores (r = 0.83; p SF-36 did not correlate with disease activity (r = -0.11; p = 0.244 and r = -0.03; p = 0.721, respectively). LupusQoL-TR and SF-36 questionnaires were beneficial instruments in evaluating HRQoL in Turkish lupus patients. However, LupusQoL-TR and SF-36 were not associated with SLEDAI scores, which suggested that QoL might be affected by other factors besides disease activity, especially in clinically inactive or mildly active patients.

  2. Sjögren’s syndrome associated with systemic lupus erythematosus

    Science.gov (United States)

    Taşdemir, Mehmet; Hasan, Chiar; Ağbaş, Ayşe; Kasapçopur, Özgür; Canpolat, Nur; Sever, Lale; Çalışkan, Salim

    2016-01-01

    Systemic lupus erythematosus and Sjögren’s syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren’s syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed up with a diagnosis of Sjögren’s syndrome for six years and then was observed to have overlap of systemic lupus erythematosus. In the baseline, she did not have any clinical or serological evidence for systemic lupus erythematosus. After six year, massive proteinuria and serological findings developed and systemic lupus erythematosus nephritis was diagnosed by kidney biopsy. Currently, systemic lupus erythematosus and Sjögren’s syndrome cannot be differentiated definetely. We need more valuable diagnostic and classification criteria to differentiate these two important conditions. PMID:27738403

  3. A Case of Systemic Lupus Erythematosus Confused with Infective Endocarditis

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    Sibel Serin

    2014-09-01

    Full Text Available Systemic lupus erythematosus (SLE is a multisystemic autoimmune disease resulting from immune system-mediated tissue damage. Clinical findings of SLE can involve skin, kidney, central nervous system, cardiovascular system, serosal membranes, and the hematologic and immune systems. In the differential diagnosis, other connective tissue diseases, infective endocarditis, infections such as viral hepatitis, endocrine disorders such as hypothyroidism, sarcoidosis, and some malignant tumors should be considered. Infective endocarditis can imitate all the symptoms of SLE depending on immune complex accumulation glomerulonephritis. Hemolytic anemia, skin lesions, arthralgia, arthritis, decreased complement levels, and autoantibody positivity, including antinuclear autoantibody (ANA, positivity can be seen. Therefore, high fever, blood cultures, eye examination, and echocardiographic findings are of particular value. Here, we present a case of SLE that was confused with infective endocarditis (IE due to the presence of high fever associated with autoimmune hemolytic anemia (AHA and proteinuria as well as increased erythrocyte sedimentation rate (ESR, cardiac murmur, and Roth’s spots. (The Me­di­cal Bul­le­tin of Ha­se­ki 2014; 52: 212-15

  4. A study of flare assessment in systemic lupus erythematosus (SLE) based on paper patients

    OpenAIRE

    Isenberg, D.; Sturgess, J; Allen, Jeannette E.; Aranow, C.; Askanase, Anca; Sang-Cheol, B; Bernatsky, S; Bruce, I; Buyon, Jp; Cervera, R.; Clarke, A; Dooley, Mary Anne; Fortin, P.; Ginzler, E.; Gladman, D D

    2017-01-01

    OBJECTIVE: To determine the level of agreement of disease flare severity (distinguishing severe, moderate and mild flare and persistent disease activity) in a large paper patient exercise involving 988 individual cases of systemic lupus erythematosus.METHODS: 988 individual lupus case histories were assessed by three individual physicians. Complete agreement about the degree of flare (or persistent disease activity) was obtained in 451 cases (46%) and these provided the reference standard for...

  5. Treatment of Systemic Lupus Erythematosus by Nutrition and Dendritic Cell Targeting

    OpenAIRE

    Liao, Xiaofeng

    2017-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease involving the inflammatory damages of multiple organs. Lupus nephritis (LN) as the manifestation in the kidney occurs in more than 50% of SLE patients and is a major cause of morbidity and mortality. Current treatments consist of immunosuppressants that always lead to compromised immune responses with increased risks of infections as the major side effect. To minimize this side effect, it is crucial to develop new treatments that are...

  6. Miliary tuberculosis: a severe opportunistic infection in juvenile systemic lupus erythematosus patients

    OpenAIRE

    Freire, Priscilla S.; Montoni, João D.; Ribeiro, Aline S.M.; Marques, Heloísa H.; Mauad, Thais; Silva, Clovis A.

    2016-01-01

    Abstract Introduction One of the main issues in juvenile systemic lupus erythematosus (JSLE) patients is infection, such as tuberculosis (TB). Of note, SLE patients are susceptible to pulmonary and extrapulmonary TB. However, to our knowledge, this contagious disease was rarely reported in pediatric lupus population, particularly diffuse or miliary TB. Therefore, from January 1983 to December 2011, 5,635 patients were followed-up at our Pediatric Rheumatology Unit and 285 (5%) of them met th...

  7. Oral Ulcers in Juvenile-Onset Systemic Lupus Erythematosus: A Review of the Literature

    OpenAIRE

    Rodsaward, Pongsawat; Prueksrisakul, Titipong; Deekajorndech, Tawatchai; Edwards, Steven W.; Beresford, Michael W.; Chiewchengchol, Direkrit

    2017-01-01

    Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Oral ulcers are classified as lupus erythematosus (LE...

  8. Neuropsychiatric events in systemic lupus erythematosus: attribution and clinical significance.

    Science.gov (United States)

    Hanly, John G; McCurdy, Grace; Fougere, Lisa; Douglas, Jo-Anne; Thompson, Kara

    2004-11-01

    To describe the range and attribution of neuropsychiatric (NP) disease in an unselected cohort of patients with systemic lupus erythematosus (SLE) and to examine the association with cumulative organ damage, medication use, and quality of life. One hundred eleven patients with SLE in a single referral center were studied. NP syndromes were defined using the American College of Rheumatology (ACR) nomenclature and case definitions. Overall disease activity was measured by the SLE Disease Activity Index (SLEDAI); cumulative organ damage was determined by the ACR/SLICC damage index; and quality of life by the SF-36. Patients' mean age was 44.7 years, 87% were female, and 92% were Caucasian. The mean (+/- SE) disease duration was 10.1 +/- 0.7 years. A total of 74 NP events were identified in 41 of 111 (37%) patients. Thirteen of the 19 ACR NP syndromes were identified and 2 or more NP manifestations occurred in 56% of patients. Central nervous system manifestations accounted for 92% of the events compared to involvement of the peripheral nervous system in 8%. Thirty-five (47%) of these events were attributed entirely to SLE, 30 (41%) were attributed exclusively to non-SLE factors, and in the remaining 9 events (12%) both SLE and non-SLE factors were felt to be contributory. Cumulative organ damage was higher in patients with NP disease, although this was not statistically significant and they were more likely to have received prednisone or immunosuppressive drugs (p attribution of NP disease. In contrast, the occurrence of renal disease in the same cohort of patients was not associated with lower SF-36 scores or fatigue. In patients with SLE, NP disease has diverse manifestations and can be attributed to lupus in roughly half of the cases. The occurrence of NP disease is associated with more frequent use of corticosteroids and immunosuppressive drugs. In contrast to other serious manifestations of SLE, such as renal disease, NP disease is associated with a significant

  9. Cutaneous manifestations of systemic lupus erythematosus in a tertiary referral center.

    Science.gov (United States)

    Kole, Alakes Kumar; Ghosh, Alakendu

    2009-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with multiorgan involvement. The skin is the second most commonly affected organ. SLE with skin lesions can produce considerable morbidity resulting from painful skin lesions, alopecia, disfigurement, etc. Skin lesions in patients with lupus may be specific (LE specific) or may be non specific (LE non specific). Acute cutaneous LE (Lupus specific) has a strong association with systemic disease and non-specific skin lesions always indicate disease activity for which patients present to rheumatologists and internists. Therefore, a thorough understanding of the cutaneous manifestations of SLE is essential for most efficient management. The aims of this study were to evaluate the patterns and prevalence of skin lesions in patients with SLE and to assess the relationship between skin lesions and other systemic involvement. At the Department of Rheumatology and Clinical Immunology, IPGME&R in Kolkata, 150 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association (updated 1982) were examined and followed-up for cutaneous manifestations between January 2002 and January 2007. Skin lesions were important clinical features. About 45 patients (30%) presented with skin lesions although all patients had skin lesions during the follow-up period. Skin changes noted were as follows: Lupus specific lesions: malar rash in 120 patients (80%), photosensitive dermatitis in 75 patients (50%), generalized maculopapular rash in 40 patients (26.67%), discoid rash in 30 patients (20%), subacute cutaneous lupus erythematosus (SCLE) in 5 patients (3.34%), lupus profundus in 5 patients (3.34%). The lupus non-specific lesions were non-scarring alopecia in 130 patients (86.67%), oral ulcers in 85 patients (56.67%), vasculitic lesions in 50 patients (33.34%), bullous lesions in 15 patients (10%), Raynaud's phenomenon in 10 patients (6.67%), pyoderma gangrenosum in 2 patients (1

  10. Cutaneous manifestations of systemic lupus erythematosus in a tertiary referral center

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    Kole Alakes

    2009-01-01

    Full Text Available Background : Systemic lupus erythematosus (SLE is an autoimmune disease with multiorgan involvement. The skin is the second most commonly affected organ. SLE with skin lesions can produce considerable morbidity resulting from painful skin lesions, alopecia, disfigurement, etc. Skin lesions in patients with lupus may be specific (LE specific or may be non specific (LE non specific. Acute cutaneous LE (Lupus specific has a strong association with systemic disease and non-specific skin lesions always indicate disease activity for which patients present to rheumatologists and internists. Therefore, a thorough understanding of the cutaneous manifestations of SLE is essential for most efficient management. Aims: The aims of this study were to evaluate the patterns and prevalence of skin lesions in patients with SLE and to assess the relationship between skin lesions and other systemic involvement. Materials and Methods: At the Department of Rheumatology and Clinical Immunology, IPGME&R in Kolkata, 150 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association (updated 1982 were examined and followed-up for cutaneous manifestations between January 2002 and January 2007. Results: Skin lesions were important clinical features. About 45 patients (30% presented with skin lesions although all patients had skin lesions during the follow-up period. Skin changes noted were as follows: Lupus specific lesions: malar rash in 120 patients (80%, photosensitive dermatitis in 75 patients (50%, generalized maculopapular rash in 40 patients (26.67%, discoid rash in 30 patients (20%, subacute cutaneous lupus erythematosus (SCLE in 5 patients (3.34%, lupus profundus in 5 patients (3.34%. The lupus non-specific lesions were non-scarring alopecia in 130 patients (86.67%, oral ulcers in 85 patients (56.67%, vasculitic lesions in 50 patients (33.34%, bullous lesions in 15 patients (10%, Raynaud′s phenomenon in 10 patients (6

  11. Expression of CD64 on Surface of Circulating Monocytes in Systemic Lupus Erythematosus Patients: Relation to Disease Activity and Lupus Nephritis.

    Science.gov (United States)

    Abd-Elhamid, Yasser A; Eltanawy, Refaat M; Fawzy, Rasha M; Fouad, Nehad A; Atlm, Ann M

    2017-01-01

    CD64 is a type of integral membrane glycoprotein known as FC receptor that binds monomeric IgG-type antibodies with high affinity. It is more commonly known as FC gamma receptor 1 (FC?R1) and it is expressed on monocytes surface. The goal of this study was to investigate the association of CD64 expression on the surface of peripheral blood monocytes of systemic lupus erythematosus patients with disease activity, and lupus nephritis. 30 SLE patients were enrolled into this study. They were subdivided into: 15 SLE patients with lupus nephritis and 15 SLE patients without lupus nephritis. In addition,Together with 25 age and sex matched healthy volunteers were enrolled as controls group. Disease activity was defined by SLE Disease Activity Index (SLEDAI) score and the renal Systemic Lupus Erythematosus Disease Activity Index (rSLEDAI) score. Surface expression of CD64 on peripheral blood monocytes was evaluated by Flowcytometry. Renal biopsies of Lupus nephritis patients was obtained and evaluated using the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification scheme. There was a statistically significant difference in surface expression of CD64 on circulating monocytes (P > 0.001) in SLE patients with nephritis especially those with class II/III as compared to SLE without nephritis and healthy controls. The mean fluorescent intensity of CD64 staining correlated positively with markers of systemic inflammation, lupus nephritis, SLEDAI and rSLEDAI scores. In conclusions, surface expression of CD64 on circulating monocytes reflects systemic inflammation, renal injury and could be used as a rapid approach and good biomarker for disease activity and lupus nephritis in SLE patients. Copyright© by the Egyptian Association of Immunologists.

  12. Immunoglobulin light chain allelic inclusion in systemic lupus erythematosus.

    Science.gov (United States)

    Fraser, Louise D; Zhao, Yuan; Lutalo, Pamela M K; D'Cruz, David P; Cason, John; Silva, Joselli S; Dunn-Walters, Deborah K; Nayar, Saba; Cope, Andrew P; Spencer, Jo

    2015-08-01

    The principles of allelic exclusion state that each B cell expresses a single light and heavy chain pair. Here, we show that B cells with both kappa and lambda light chains (Igκ and Igλ) are enriched in some patients with the systemic autoimmune disease systemic lupus erythematosus (SLE), but not in the systemic autoimmune disease control granulomatosis with polyangiitis. Detection of dual Igκ and Igλ expression by flow cytometry could not be abolished by acid washing or by DNAse treatment to remove any bound polyclonal antibody or complexes, and was retained after two days in culture. Both surface and intracytoplasmic dual light chain expression was evident by flow cytometry and confocal microscopy. We observed reduced frequency of rearrangements of the kappa-deleting element (KDE) in SLE and an inverse correlation between the frequency of KDE rearrangement and the frequency of dual light chain expressing B cells. We propose that dual expression of Igκ and Igλ by a single B cell may occur in some patients with SLE when this may be a consequence of reduced activity of the KDE. © 2015 The Authors. European Journal of Immunology published by WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  13. Treatment of lupus nephritis

    NARCIS (Netherlands)

    Dolff, Sebastian; Berden, Jo H. M.; Bijl, Marc

    2010-01-01

    Renal involvement in systemic lupus erythematosus patients is a severe disease manifestation characterized by various clinical and histopathological alterations The revised International Society of Nephrology/Renal Pathology Society 2003 classification defines the subclasses of lupus nephritis (LN)

  14. Evidence for aerobic insufficiency in women with systemic Lupus erythematosus.

    Science.gov (United States)

    Keyser, Randall E; Rus, Violeta; Cade, William Todd; Kalappa, Neeta; Flores, Raymond H; Handwerger, Barry S

    2003-02-15

    To determine if fatigue is associated with diminished aerobic capacity in women with systemic lupus erythematosus (SLE). Eighteen women (age 35 +/- 9 years) with mild SLE (Systemic Lupus Activity Measure = 3.1 +/- 2.1) and 16 healthy but sedentary controls (age 38 +/- 8 years) completed peak treadmill exercise tests to determine aerobic capacity and Fatigue Severity Scales to quantify the severity of fatigue. Measures of oxygen consumption (VO(2)) were recorded during the treadmill tests. Peak VO(2) was lower in patients with SLE (19.2 +/- 4.4 ml/kg/minute) as compared with controls (27.4 +/- 4.7 ml/kg/minute) and expected values (30.7 +/- 3.1 ml/kg/minute; P < 0.0006 versus controls and P < 0.0001 versus expected). Functional aerobic impairment was observed in 14 of the 18 patients with SLE. In patients with SLE, ventilatory threshold, a marker for the onset of lactic acidemia, was observed at the lowest energy requirement for instrumental activities of daily living. Peak VO(2) in the patients with SLE was similar to the highest energy requirements for instrumental activities of daily living, leaving little or no reserve for more intense occupational and recreational activities. Peak VO(2) was significantly higher (P < 0.0001) than the activity of daily living requirements in controls, providing a substantial energy reserve. Fatigue severity score (FSS) was 5.0 +/- 1.4 in patients with SLE, with 14 of the 18 patients having scores above 4.0, a score indicating that fatigue severity limited physical activity. Of the 14, 12 had functional aerobic impairment. An FSS of greater than 4.0 was not observed in controls (mean = 2.5 +/- 0.7). In women with SLE, aerobic capacity was diminished to levels that were insufficient for engaging in activities of daily living and below those expected to result from physiologic deconditioning. This functional aerobic impairment was strongly correlated with the perception of severe, activity-limiting fatigue.

  15. Neonatal and Obstetrical Outcomes of Pregnancies in Systemic Lupus Erythematosus

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    Reem Abdwani

    2018-01-01

    Full Text Available Objectives: Systemic lupus erythematous (SLE is a chronic autoimmune disease that affects women primarily of childbearing age. The objective of this study was to determine the neonatal and maternal outcomes of pregnancies in SLE patients compared to pregnancies in healthy controls. Methods: We conducted a retrospective cohort study in a tertiary care hospital in Oman between January 2007 and December 2013. We analyzed 147 pregnancies and compared 56 (38.0% pregnancies in women with SLE with 91 (61.9% pregnancies in healthy control women. Disease activity was determined using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI. Results: The mean age of the cohort was 30.0±5.0 years ranging from 19 to 44 years old. Patients with SLE were treated with hydroxychloroquine (n = 41; 73.2%, prednisolone (n = 38; 67.8%, and azathioprine (n = 17; 30.3%. There was no disease activity in 39.2% (n = 22 of patients while 41.0% (n = 23, 12.5% (n = 7, and 7.1% (n = 4 had mild (SLEDAI 1–5, moderate (SLEDAI 6–10, and severe (SLEDAI ≥ 11 disease activity, respectively, at onset of pregnancy. Pregnancies in patients with SLE were associated with higher abortions (42.8% vs. 15.3%; p < 0.001, gestational diabetes (28.3% vs. 10.2%; p = 0.004, polyhydramnios (7.1% vs. 0.0%; p = 0.020, previous preterm pregnancies (8.9% vs. 1.0%; p = 0.030, and intrauterine growth retardation (21.4% vs. 0.0%; p < 0.001 when compared to pregnancies in healthy control women. Furthermore, the neonates born to mothers with SLE were more likely to be preterm (28.5% vs. 1.0%; p < 0.001, have a low birth weight (< 2500 g (32.1% vs. 1.0%; p < 0.001, and were associated with stillbirth (7.1% vs. 0.0%; p = 0.010 when compared to neonates born to healthy control mothers. Conclusions: Pregnancies in women with SLE were associated with higher neonatal and maternal complications. Therefore, pregnant women with SLE should have their pregnancy accurately planned, monitored, and

  16. Contraception in women with systemic erythematosus lupus = Anticoncepción en mujeres con lupus eritematoso sistémico

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    Luis Alonso González Naranjo

    2013-07-01

    Full Text Available The use of contraceptives, particularly of those containing estrogens by women with systemic lupus erythematosus (SLE, has been thought to carry risks such as disease exacerbation, thrombosis and other adverse effects. However, the available evidence suggests that many women with SLE, particularly those with stable disease, are not at increased risk of disease flare while taking oral contraceptives and therefore can be good candidates for most contraceptive methods, including the hormonal ones. Contrariwise, women with positive antiphospholipid antibodies are at increased risk of arterial and venous thrombosis and therefore the use of combined hormonal contraceptive methods should be avoided in them.

  17. The sense of smell in systemic lupus erythematosus.

    Science.gov (United States)

    Shoenfeld, Netta; Agmon-Levin, Nancy; Flitman-Katzevman, Iveta; Paran, Daphna; Katz, Bat-sheva Porat; Kivity, Shaye; Langevitz, Pnina; Zandman-Goddard, Gisele; Shoenfeld, Yehuda

    2009-05-01

    To assess the olfactory functions in systemic lupus erythematosus (SLE) patients compared with age- and sex-matched healthy controls, and to examine the association between the sense of smell and disease activity and central nervous system (CNS) involvement. Olfactory functions in 50 SLE patients and 50 age- and sex-matched controls were evaluated using the Sniffin' Sticks test, the 3 stages of which are threshold, discrimination, and identification (TDI) of different odors. TDI scores were analyzed according to SLE disease activity and CNS involvement. In both the SLE and control groups, smell deficit correlated with male sex and older age. A decrease in the sense of smell was observed in SLE patients (46%) and controls (25%) (Psmell (anosmia) was documented only in SLE patients (10%). Total TDI scores and individual stages of smell correlated with SLE Disease Activity Index (Psmell in SLE patients compared with healthy subjects and that the decrease in the sense of smell among SLE patients correlates with disease activity and CNS involvement.

  18. Nonenzymatic antioxidants in saliva of patients with systemic lupus erythematosus.

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    Moori, M; Ghafoori, H; Sariri, R

    2016-03-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody-directed self-antigens, immune complex formation and immune deregulation, resulting in damage to essentially all the organs. SLE is associated with the increased production of free radicals. Increase in free radicals or impaired antioxidant defense system in SLE causes oxidative stress. Considering that saliva could be a reflection of the state of health, the purpose of this study was to evaluate some antioxidants in the saliva and serum of patients with SLE and compare these with healthy individuals. This could help us in obtaining a possible marker in saliva in the future. During the course of the practical part of the project, 30 patients with SLE and 30 healthy controls were investigated. After centrifugation of un-stimulated saliva and blood samples, they were examined using spectrophotometric methods and the results were analyzed by statistical software. According to the results, concentrations of malondialdehyde, uric acid and total antioxidants were significantly increased but the level of reduced glutathion was reduced significantly in the saliva and serum of SLE patients as compared to controls. It is therefore suggested that antioxidant power is impaired in saliva and serum of SLE patients. As there was a positive correlation between the antioxidant level of saliva and blood serum, the antioxidant status of saliva could be an indicator of serum antioxidants. © The Author(s) 2015.

  19. Alveolar hemorrhage in systemic lupus erythematosus: a cohort review.

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    Andrade, C; Mendonça, T; Farinha, F; Correia, J; Marinho, A; Almeida, I; Vasconcelos, C

    2016-01-01

    Diffuse alveolar hemorrhage (DAH) is a rare but potentially catastrophic manifestation with a high mortality. Among rheumatologic diseases, it occurs most frequently in patients with systemic lupus erythematosus (SLE) and systemic vasculitis. Despite new diagnostic tools and therapies, it remains a diagnostic and therapeutic challenge. The aim of this work was to characterize the SLE patients with an episode of alveolar hemorrhage followed in our Clinical Immunology Unit (CIU). A retrospective chart review was carried out for all patients with SLE followed in CIU between 1984 and the end of 2013. We reviewed the following data: demographic characteristics, clinical and laboratory data, radiologic investigations, histologic studies, treatment, and outcome. We identified 10 episodes of DAH, corresponding to seven patients, all female. These represent 1.6% of SLE patients followed in our Unit. The age at DAH attack was 42.75 ± 18.9 years. The average time between diagnosis of SLE and the onset of DAH was 7.1 years. Three patients had the diagnosis of SLE and the DAH attack at the same time. Disease activity according to SLEDAI was high, ranging from 15 to 41. All patients were treated with methylprednisolone, 37.5% cyclophosphamide and 28.6% plasmapheresis. The overall mortality rate was 28.6%. © The Author(s) 2015.

  20. Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus.

    Science.gov (United States)

    Aydogan, Kenan; Karadogan, Serap Koran; Adim, Saduman Balaban; Tunali, Sukran

    2006-09-01

    Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement, whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7-8% of SLE, while 50% of HUVS patients are diagnosed with SLE. We report a case of HUVS associated with SLE with fatal outcome unresponsive to the combination of systemic corticosteroids and azathioprine. SLE and HUVS share both clinical and laboratory features and are probably not separate entities. It is mostly likely that HUVS and SLE fall into the same spectrum of autoimmune diseases. HUVS is probably a subset of SLE. As both diseases can fatally, it should be kept in mind that the overlap of SLE and HUVS may exhibit a relatively rapid progression and poor prognosis.

  1. Cardiac tamponade as an initial presentation for systemic lupus erythematosus.

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    Li, William; Frohwein, Thomas; Ong, Kenneth

    2017-08-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease which follows a relapsing and remitting course that can manifest in any organ system. While classic manifestations consist of arthralgia, myalgia, frank arthritis, a malar rash and renal failure to name a few, cardiac tamponade, however, is a far less common and far more dangerous presentation. We highlight the case of a 61year-old male with complaints of acute onset shortness of breath and generalized body aches associated with a fever and chills in the ER. A bedside echocardiogram revealed a significant pericardial effusion concerning for pericardial tamponade. An emergent pericardiocentesis performed drained 800mL of serosanguinous fluid. While denying a history of any rash, photosensitivity, oral ulcers, or seizures, his physical examination did reveal metacarpal phalangeal joint swelling along with noted pulsus paradoxus of 15-200mmHg. Subsequent lab work revealed ANA titer of 1:630 and anti-DS DNA antibody level of 256IU/mL consistent with SLE. This case highlights cardiac tamponade as a rare but life-threatening presentation for SLE and raises the need to keep it in the differential when assessing patients presenting with pertinent exam findings. Published by Elsevier Inc.

  2. Alcohol, smoking and illicit drug use in pediatric systemic lupus erythematosus patients

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    Marlon van Weelden

    2016-06-01

    Full Text Available Abstract Objective To evaluate alcohol, smoking and/or illicit drug use, and history of bullying in adolescent childhood-onset systemic lupus erythematosus and healthy controls. Methods 174 adolescents with pediatric rheumatic diseases were selected. All of the 34 childhood-onset systemic lupus erythematosus patients and 35 healthy controls participated in this study. A cross-sectional study included demographic/anthropometric data and puberty markers assessments; structured questionnaire and CRAFFT screening interview. Results McNemar tests indicated an excellent test–retest reliability of the structured questionnaire (p = 1.0. The median current age was similar between childhood-onset systemic lupus erythematosus patients and controls [15 (12–18 vs. 15 (12–18 years, p = 0.563]. The median of menarche age was significantly higher in childhood-onset systemic lupus erythematosus patients compared to controls [12 (10–15 vs. 11.5 (9–15 years, p = 0.041], particularly in those that lupus had occurred before first menstruation [13 (12–15 vs. 11.5(9–15 years, p = 0.007]. The other puberty marker and sexual function parameters were similar in both groups (p > 0.05. Alcohol use was similar in both childhood-onset systemic lupus erythematosus patients and controls (38% vs. 46%, p = 0.628. A trend of lower frequency of CRAFFT score ≥2 (high risk for substance abuse/dependence was evidenced in childhood-onset systemic lupus erythematosus patients compared to controls (0% vs. 15%, p = 0.053. Bullying was reported similarly for the two groups (43% vs. 44%, p = 0.950. Further analysis in lupus patients regarding alcohol/smoking/illicit drug use showed no differences in demographic data, puberty markers, history of bullying, sexual function, contraceptive use, disease activity/damage scores, clinical/laboratorial features and treatments (p > 0.05. Conclusion This study showed high frequencies of early alcohol use in lupus adolescents and

  3. Clinical and immunological characteristics of 150 systemic lupus erythematosus patients in Jamaica: a comparative analysis.

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    Maloney, K C; Ferguson, T S; Stewart, H D; Myers, A A; De Ceulaer, K

    2017-11-01

    Background Epidemiological studies in systemic lupus erythematosus have been reported in the literature in many countries and ethnic groups. Although systemic lupus erythematosus in Jamaica has been described in the past, there has not been a detailed evaluation of systemic lupus erythematosus patients in urban Jamaica, a largely Afro-Caribbean population. The goal of this study was to describe the clinical features, particularly disease activity, damage index and immunological features, of 150 systemic lupus erythematosus subjects. Methods 150 adult patients (≥18 years) followed in rheumatology clinic at a tertiary rheumatology hospital centre (one of two of the major public referral centres in Jamaica) and the private rheumatology offices in urban Jamaica who fulfilled Systemic Lupus International Collaborating Clinics (SLICC) criteria were included. Data were collected by detailed clinical interview and examination and laboratory investigations. Hence demographics, SLICC criteria, immunological profile, systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) and SLICC/American College of Rheumatology (ACR) damage index (SDI) were documented. Results Of the 150 patients, 145 (96.7%) were female and five (3.3%) were male. The mean age at systemic lupus erythematosus onset was 33.2 ± 10.9. Mean disease duration was 11.3 ± 8.6 years. The most prevalent clinical SLICC criteria were musculoskeletal, with 141 (94%) of subjects experiencing arthralgia/arthritis, followed by mucocutaneous manifestations of alopecia 103 (68.7%) and malar rash 46 (30.7%), discoid rash 45 (30%) and photosensitivity 40 (26.7%). Lupus nephritis (biopsy proven) occurred in 42 (28%) subjects and 25 (16.7%) met SLICC diagnostic criteria with only positive antinuclear antibodies/dsDNA antibodies and lupus nephritis on renal biopsy. The most common laboratory SLICC criteria were positive antinuclear antibodies 136 (90.7%) followed by anti-dsDNA antibodies 95 (63.3%) and

  4. Beta-adducin and sodium-calcium exchanger 1 gene variants are associated with systemic lupus erythematosus and lupus nephritis.

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    Ramirez, Giuseppe A; Lanzani, Chiara; Bozzolo, Enrica P; Zagato, Laura; Citterio, Lorena; Casamassima, Nunzia; Canti, Valentina; Sabbadini, Maria Grazia; Rovere-Querini, Patrizia; Manunta, Paolo; Manfredi, Angelo A

    2015-12-01

    Genetic research in systemic lupus erythematosus (SLE) is rapidly developing, and numerous sets of genes are being associated with specific clinical subphenotypes in the setting of SLE. On the other hand, basic science studies are revealing strong connections between salt-water balance and inflammation. The aim of this study was to evaluate whether variants of genes known to influence the individual susceptibility to hypertension also influence the renal function in a cohort of SLE patients with and without lupus nephritis (LN). This study is a case-control study with candidate gene approach. A total of 111 patients with SLE (50 with SLE without nephritis, 55 with LN and 6 with simple urinary sediment abnormalities) and 62 healthy controls (HC) were genotyped for NCX1 rs11893826 (NCX1a) and rs434082 (NCX1b) and ADD2 rs4984 SNPs. Patients with ADD2 CT genotype were protected from LN and skin involvement; ADD2 CC | NCX1a AA/AG genotypes were associated with the presence of anti-cardiolipin antibodies; NCX1a AA genotype was slightly more frequent in lupus patients than in HC and associated with relapse risk and higher creatinine in patients with LN. NCX1b GG patients with LN had increased chances to reach complete remission. NCX1b GG | NCX1a GG genotype is associated with joint involvement. ADD2 and NCX1 variants influence the risk and the clinical features of SLE and LN, highlighting their potential role in regulating systemic inflammation and/or the local response to immune-mediated injury.

  5. Preterm deliveries in women with systemic lupus erythematosus.

    Science.gov (United States)

    Clark, Christine A; Spitzer, Karen A; Nadler, Jamie N; Laskin, Carl A

    2003-10-01

    To compare the clinical, laboratory, and demographic variables of women in our clinic with systemic lupus erythematosus (SLE) who have had a pregnancy resulting in a live birth and identify any correlations with either term or preterm delivery. Pregnancies in women with SLE from 1999 to 2001 were retrospectively reviewed. We recorded demographic data, disease activity (SLE Disease Activity Index, SLEDAI), obstetric history, prednisone dosage, other medications taken during pregnancy, history of renal disease, and autoantibody status [including antinuclear antibody, anti-DNA, anticardiolipin IgG (aCL), and lupus anticoagulant (LAC)]. Preterm delivery was defined as gestational age at delivery or = 10 mg/day prednisone during their pregnancy (50.0% vs 22.2%; p = 0.028), and the mean dose was significantly higher than the term group taking > or = 10 mg/day (24.8 vs 16.7 mg/day; p = 0.047). There was a higher prevalence of women with aCL IgG in the preterm group (p = 0.023). The mean weeks gestation was shorter for women positive for aCL IgG compared to the group negative for aCL (34.9 +/- 4.4 vs 37.5 +/- 3.2 weeks, respectively; p = 0.032). There was no difference in second trimester disease activity between the term and preterm groups (33.3% and 36.4% of each group had a SLEDAI of 0). However, significantly more women in the term group received no medication during their pregnancies compared to women in the preterm group (20.0% vs 0.0%; p = 0.031). The rates of preterm deliveries, premature rupture of membranes, intrauterine growth restriction, and aPL in SLE pregnancies vary considerably in published reports, most of which are retrospective analyses. Our rates closely approximate the median values for all measures. We found preterm deliveries to be associated with disease activity (as determined by the use of any medication throughout pregnancy vs no medication, and prednisone dose > or = 10 mg/day) and the presence of aCL IgG but not LAC. Our results suggest that

  6. CYTOKINE PROFILE IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

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    Maria Eduardovna Tsanyan

    2013-01-01

    Full Text Available Objective: to investigate the levels of cytokines in patients with systemic lupus erythematous (SLE and to determine their correlation with SLE activity and organ damage, as well as their changes during rituximab (RTM therapy.Subjects and methods. The trial enrolled 26 patients (5 men and 21 women with a median (Me age of 27 [range 23-40] years and a SLE duration of 9 to 300 months (Me 72 [range 36-108] months and 30 healthy donors marched withthe examinees for gender and age. Disease activity was assessed using the SLEDAI-2K index. The serum levels of 27 cytokines were measured utilizing X-MAP technologies on a BioPlex 200 device (Bio-Rad, USA. The patients were divided into 2 groups of 13 persons in each: lupus nephritis (LN and no renal injury. All the patients included in the trial received RTM therapy.Results. A statistically significant elevation in the concentrations of interleukin 13 (IL-13 and G-CSF was found in the patients with SLE versus the healthy control group (p = 0.03 and p = 0.03, respectively. The LN group displayed a statistically significant increase in the concentrations of IL-4, IL-6, IL-7, IL-13, and G-CSF as compared with the non-LN group (р = 0.039, р = 0.03, р = 0.037, р = 0.03, and р = 0.028, respectively. ROC analysis established the sensitivity and specificity of all indices in the LN group. The area under the ROC curve was equal for all indicators. The highest specificity of IL-13 (85% was observed in the LN group. These cytokines other than that of IL13 (62% showed the same sensitivity (70%.Conclusion. The patients with LN had statistically significantly elevated concentrations of IL4, IL6, IL7, IL13, and G-CSF. The findings suggest that these cytokines are associated with VN.

  7. [Systemic lupus erythematosus and contraception: A systematic literature review].

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    Gensous, N; Doassans-Comby, L; Lazaro, E; Duffau, P

    2017-06-01

    The aim of our study was to evaluate the safety of contraceptive methods use among women with systemic lupus erythematosus (SLE). A systematic review of the literature was performed using the two databases MEDLINE and SCOPUS, in order to identify articles concerning the safety of contraceptive methods use among women with SLE, through May 2016. Information on study characteristics, objectives, population, contraception and outcomes were extracted. A total of 907 articles were identified and 21 were selected for the systematic review. Two randomised controlled trials found no worsening of disease activity with the use of combined oral contraceptive in women with stable or inactive SLE. Disease activity was not exacerbated with the use of progestogens-only contraceptive. There was an increased risk of thrombosis with the use of combined contraceptive, particularly in women with positive antiphospholipid antibodies and this must lead to a restriction of use in these patients. Use of oral combined hormonal contraceptives should be limited to patients with non-severe and stable disease, without thrombosis risk factors. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  8. Cardiopulmonary correlates of cognition in systemic lupus erythematosus.

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    Kozora, E; Zell, J; Swigris, J; Strand, M; Duggan, E C; Burleson, A; Make, B

    2015-02-01

    We aimed to evaluate the relationship between cognitive dysfunction and lung function, exercise endurance, and self-reported activity levels in patients with systemic lupus erythematosus (SLE). Cognitive dysfunction is present in 20%-60% of SLE patients. No studies to date have investigated the inter-relationships between cardiopulmonary factors and cognition in this population. Thirty-seven SLE patients without overt neuropsychiatric histories and 16 healthy controls completed neuropsychological testing, measures of lung function, exercise capacity (distance walked during a timed walk test,(1) maximal oxygen uptake(2)), and exercise questionnaires. Thirty-two percent of SLE patients demonstrated cognitive impairment. Cognitive impairment was correlated with Six-Minute Walk Distance (6MWD) (r = 0.37, p = 0.02) and certain measures of lung function. Also, in SLE patients, self-reported physical activity was correlated with 6MWD (p = 0.012), but none of the more complex measures of physical activity (VO2max). Patients with mild SLE disease activity have cognitive dysfunction associated with certain objective markers of exercise capacity and activity levels. The lack of associations between self-report activity and VO2max suggests the possibility that multiple factors mediate the relationships between perceived and actual physical ability. Additional studies are needed to better understand the relationship between cognition and physical activity in patients with SLE. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  9. Determinants of sleep quality in women with systemic lupus erythematosus.

    Science.gov (United States)

    Da Costa, Deborah; Bernatsky, Sasha; Dritsa, Maria; Clarke, Ann E; Dasgupta, Kaberi; Keshani, Anahita; Pineau, Christian

    2005-04-15

    To characterize sleep complaints in women with systemic lupus erythematosus (SLE) and to identify correlates of sleep quality. Sleep quality in 100 women with SLE was assessed using the Pittsburgh Sleep Quality Index (PSQI). Participants completed standardized questionnaires assessing depressed mood, leisure time physical activity, functional disability, and pain severity. A clinical examination determined disease activity, cumulative damage, and whether patients fulfilled the American College of Rheumatology criteria for fibromyalgia. A series of hierarchical multiple regressions were computed. The mean +/- SD global PSQI score was 6.98 +/- 4.03, with moderate to severe sleep impairment reported by 56% of the sample. The first model testing the importance of demographic factors was not statistically significant. In the disease-related model, the use of prednisone and functional disability both contributed to poor sleep quality (P exercise participation to the demographic set significantly added to the model (P = 0.001). Depression significantly added to the demographic set, explaining 29% of the variance (P exercise and prednisone use. A significant proportion of women with SLE suffer from poor sleep quality. The findings suggest that depressed mood, prednisone use, and lack of exercise contribute to decreased overall sleep quality.

  10. Aerobic fitness, fatigue, and physical disability in systemic lupus erythematosus.

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    Tench, Colin; Bentley, David; Vleck, Veronica; McCurdie, Ian; White, Peter; D'Cruz, David

    2002-03-01

    To measure aerobic fitness, muscle strength, fatigue, and physical disability in patients with systemic lupus erythematosus (SLE). Ninety-three patients with SLE and 41 sedentary controls were recruited into the study. Aerobic fitness was assessed by monitoring peak and submaximal oxygen uptake, heart rate, duration of exercise, and perceived exertion during a treadmill-walking test. Strength was measured using voluntary isometric quadriceps contraction. Symptomatic measures included physical and mental fatigue, mood, sleep, and functional incapacity. Compared to sedentary controls patients with SLE had significantly reduced levels of aerobic fitness (mean VO2peak SLE patients, 23.2 ml/kg/min vs controls, 29.6 ml/kg/min; p exercise capacity (mean exercise duration SLE patients, 10.4 min vs controls, 13.1 min; p exercise capacity, reduced muscle strength, more fatigue, and greater disability compared to sedentary controls. Treatments developed to manage depression and improve aerobic fitness should be considered in the overall treatment of fatigue and disability in SLE.

  11. Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Prevalence and Predictors.

    Science.gov (United States)

    Pérez-Peñate, Gregorio Miguel; Rúa-Figueroa, Iñigo; Juliá-Serdá, Gabriel; León-Marrero, Fernándo; García-Quintana, Antonio; Ortega-Trujillo, José Ramón; Erausquin-Arruabarrena, Celia; Rodríguez-Lozano, Carlos; Cabrera-Navarro, Pedro; Ojeda-Betancor, Nazario; Gómez-Sánchez, Miguel Ángel

    2016-02-01

    Pulmonary arterial hypertension (PAH) prevalence has been reported to be between 0.5% and 17% in systemic lupus erythematosus (SLE). This study assessed PAH prevalence and predictors in an SLE cohort. The Borg dyspnea scale, DLCO, N-terminal pro-brain natriuretic peptide (NT-proBNP), and Doppler echocardiographic (DE) were performed. An echocardiographic Doppler exercise test was conducted in selected patients. When DE systolic pulmonary arterial pressure was ≥ 45 mmHg or increased during exercise > 20 mmHg, a right heart catheterization was performed. Hemodynamic during exercise was measured if rest mean pulmonary arterial pressure was exercise-induced pulmonary artery pressure increase (4 with occult left diastolic dysfunction). These patients had significantly more dyspnea, higher NT-proBNP, and lower DLCO. These data confirm the low prevalence of PAH in SLE. In our cohort, occult left ventricular diastolic dysfunction was a frequent diagnosis of unexplained dyspnea. Dyspnea, DLCO, and NT-proBNP could be predictors of pulmonary hypertension in patients with SLE.

  12. Variants of psychiatric disorders in patients with systemic lupus erythematosus

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    T A Lisitsyna

    2008-01-01

    Full Text Available Objective. To analyze prevalence and structure of psychiatric disorders in pts with systemic lupus erythematosus (SLE examining in the Institute of rheumatology of RAMS. Material and methods. 115 pts with SLE with median age 34 [24; 45] years and median disease duration 8 [4; 17] years were included. SLE activity was assessed with SLEDAI. Psychiatric disorders were diagnosed by a psychiatrist according to ICD-10 using some psychiatric and psychological scales. Results. Psychiatric disorders were revealed in 76 from 115 (66% pts. Anxiety-depressive spectrum disorders prevailed (83%: depressive episode (40%, adjustment disorders (24%, generalized anxiety disorder (10%, dysthymia (9%. Severe cognitive dysfunction was revealed in 7% of pts. Pts with and without psychiatric disorders did not significantly differ in age, sex, duration and activity of the disease, duration of treatment and cumulative dose of prednisolone and cytotoxic drugs. Conclusion. Psychiatric disorders are frequent in pts with SLE (66%. Anxiety-depressive disorders prevail among them (83%. Relationship between SLE and psychiatric disorders requires further examination.

  13. Healthcare Utilization and Costs of Systemic Lupus Erythematosus in Medicaid

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    Hong J. Kan

    2013-01-01

    Full Text Available Objective. Healthcare utilization and costs associated with systemic lupus erythematosus (SLE in a US Medicaid population were examined. Methods. Patients ≥ 18 years old with SLE diagnosis (ICD-9-CM 710.0x were extracted from a large Medicaid database 2002–2009. Index date was date of the first SLE diagnosis. Patients with and without SLE were matched. All patients had a variable length of followup with a minimum of 12 months. Annualized healthcare utilization and costs associated with SLE and costs of SLE flares were assessed during the followup period. Multivariate regressions were conducted to estimate incremental healthcare utilization and costs associated with SLE. Results. A total of 14,777 SLE patients met the study criteria, and 14,262 were matched to non-SLE patients. SLE patients had significantly higher healthcare utilization per year than their matched controls. The estimated incremental annual cost associated with SLE was $10,984, with the highest increase in inpatient costs (P<0.001. Cost per flare was $11,716 for severe flares, $562 for moderate flares, and $129 for mild flares. Annual total costs for patients with severe flares were $49,754. Conclusions. SLE patients had significantly higher healthcare resource utilization and costs than non-SLE patients. Patients with severe flares had the highest costs.

  14. HLA class II haplotypes in Mexican systemic lupus erythematosus patients.

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    Cortes, Lizette M; Baltazar, Luz M; Lopez-Cardona, Maria G; Olivares, Norma; Ramos, Cesar; Salazar, Mario; Sandoval, Lucila; Lorenz, Matthias G O; Chakraborty, Ranajit; Paterson, Andrew D; Rivas, Fernando

    2004-12-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease in which polymorphisms within the human leukocyte antigen (HLA) region have been associated to its etiology. For this study, HLA-DQB1, DQA1, and DRB1 genes were typed by polymerase chain reaction-sequence-specific primer in 237 individuals, taken from 74 families, who had a member with SLE, and who had their residence in the western region of Mexico; as well as in 159 ethnically matched healthy volunteers taken from 32 families. Genotype and allele frequency analysis was performed in 74 SLE patients and 54 unrelated controls. Precise three-loci identification of independent haplotypes was performed in 48 patients and 54 controls by familial segregation. Genotype distribution at each loci was concordant with Hardy-Weinberg's equilibrium in the control group. In general, no genotype effect was observed in SLE patients. Allele distribution comparison showed in the SLE group a significant increase of HLA-DQA1*0102, DQB1*0402, and DRB1*15; whereas alleles HLA-DQB1*0303 and *0501 were significantly decreased. SLE patients showed haplotype DQB1*0602-DQA1-*0102-DRB1*15 increased. As expected, patients with SLE have a reduced haplotype genetic diversity. The associations found in this study are related to an ancestral haplotype that has been observed in SLE populations of different origins.

  15. Living with systemic lupus erythematosus: A patient engagement perspective.

    Science.gov (United States)

    Mazzoni, Davide; Cornet, Alain; van Leeuw, Bernadette; Myllys, Kirsi; Cicognani, Elvira

    2017-07-07

    Patient engagement is recognized as a crucial component of high-quality healthcare services. Among rheumatic diseases, systemic lupus erythematosus (SLE) appears particularly challenging for the engagement of patients in their own care. According to the patient health engagement (PHE) model, patient engagement is a dynamic phenomenon that proceeds through four experiential positions (blackout, arousal, adhesion and eudaimonic project). The aim of the present study was to describe the engagement process from the point of view and the experiences of SLE patients. Ten in-depth interviews and four focus groups were conducted with an international sample of SLE patients from different European countries. Transcripts were analysed through thematic content analysis. Findings showed that a fully engaged patient results from reframing emotional, cognitive and behavioural dimensions. The advances along the process depends on how the patient succeeds in each position. In conclusion, PHE represents an appropriate model by which to understand the engagement process of SLE patients. In order to meet patients' needs, healthcare providers and patient support groups should consider the specific position of SLE patients, providing adequate and tailored support. Copyright © 2017 John Wiley & Sons, Ltd.

  16. T Cell Transcriptomes Describe Patient Subtypes in Systemic Lupus Erythematosus.

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    Sean J Bradley

    Full Text Available T cells regulate the adaptive immune response and have altered function in autoimmunity. Systemic Lupus Erythematosus (SLE has great diversity of presentation and treatment response. Peripheral blood component gene expression affords an efficient platform to investigate SLE immune dysfunction and help guide diagnostic biomarker development for patient stratification.Gene expression in peripheral blood T cell samples for 14 SLE patients and 4 controls was analyzed by high depth sequencing. Unbiased clustering of genes and samples revealed novel patterns related to disease etiology. Functional annotation of these genes highlights pathways and protein domains involved in SLE manifestation.We found transcripts for hundreds of genes consistently altered in SLE T cell samples, for which DAVID analysis highlights induction of pathways related to mitochondria, nucleotide metabolism and DNA replication. Fewer genes had reduced mRNA expression, and these were linked to signaling, splicing and transcriptional activity. Gene signatures associated with the presence of dsDNA antibodies, low complement levels and nephritis were detected. T cell gene expression also indicates the presence of several patient subtypes, such as having only a minimal expression phenotype, male type, or severe with or without induction of genes related to membrane protein production.Unbiased transcriptome analysis of a peripheral blood component provides insight on autoimmune pathophysiology and patient variability. We present an open source workflow and richly annotated dataset to support investigation of T cell biology, develop biomarkers for patient stratification and perhaps help indicate a source of SLE immune dysfunction.

  17. Anticardiolipin antibodies in patients from Malaysia with systemic lupus erythematosus.

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    Jones, H W; Ireland, R; Senaldi, G; Wang, F; Khamashta, M; Bellingham, A J; Veerapan, K; Hughes, G R; Vergani, D

    1991-03-01

    Systemic lupus erythematosus (SLE) is highly prevalent in Malaysia, which has a mixed population of Malays, Chinese, and Indians. A quantitative enzyme linked immunosorbent assay (ELISA) was used to determine anticardiolipin antibody (aCL) levels (total immunoglobulin, IgG, and IgM) in 200 patients with SLE (164 Chinese, 26 Malay, and 10 Indian) attending the University Hospital of Kuala Lumpur, Malaysia, and 103 matched controls. Only 33 (16.5%) of the patients had raised aCL levels; 26 had raised IgG aCL, five IgM aCL, and two both IgG and IgM aCL. There was a low prevalence of raised levels of aCL in the population studied, which was seen in conjunction with a rare occurrence of thrombosis. The classical association of high aCL levels with thrombocytopenia and recurrent abortions was noted, though not with cerebral disease. The low prevalence of aCL in this study population of mixed racial origin contrasts with findings in European patients with SLE and lends support to the influence of local factors, be they genetic or environmental, on the clinical manifestations of this disease.

  18. Thrombosis in systemic lupus erythematosus: role of impaired fibrinolysis.

    Science.gov (United States)

    Dhillon, Parvinderjit K; Adams, Murray J

    2013-06-01

    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect any part of the body, including the skin, liver, kidneys, and blood. Thrombosis is a frequent manifestation in SLE, contributing significantly to patient morbidity and mortality, although the precise mechanism(s) of how this occurs remains unclear. Fibrinolysis is the physiologic process of thrombus digestion and provides an important balance to hemostasis. This process is triggered upon vessel injury with the release of tissue-type plasminogen activator (t-PA) from endothelial cells. The central component of the fibrinolytic pathway is plasminogen, a zymogen that is converted to plasmin by t-PA. Plasminogen/plasmin is absorbed into the developing thrombus and digests fibrinogen and fibrin within the hemostatic plug to prevent excessive clot formation. Abnormalities of the fibrinolytic pathway are associated either with the development of thrombosis (impaired fibrinolysis) or, to a lesser extent, bleeding (excessive fibrinolysis). Indeed, impaired fibrinolysis has been reported in patients with SLE and may contribute to both the development of hypercoagulability and an increased risk of thrombosis. Here we discuss the role of impaired fibrinolysis and its contribution to hypercoagulability and thrombosis in SLE. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  19. Reproductive Health Concerns in Women with Systemic Lupus Erythematosus.

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    Kartoz, Connie Rutan

    2015-01-01

    Women are far more likely than men to have a diagnosis of systemic lupus erythematosus (SLE), with a peak incidence during the childbearing years. Contraceptive methods and pregnancy can both adversely affect the health of women with SLE, thus careful planning and interventions are necessary to help women manage their reproductive health choices. Women with SLE may experience infertility, difficulties conceiving and maintaining pregnancy, and ultimately have less children than they had planned. Although poor health status may account for some of this disparity, inadequate counseling and management by members of the healthcare team may also be responsible. The purpose of this article is to review the pathophysiology of SLE and its effects on reproductive health, as well as to highlight recent literature supporting evidence-based practices in reproductive health counseling, nursing care during pregnancy, and monitoring for disease complications in women with SLE. Nurses play a central role in care coordination and patient education for women with SLE making decisions about family planning.

  20. Periodontal disease in Chinese patients with systemic lupus erythematosus.

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    Zhang, Qiuxiang; Zhang, Xiaoli; Feng, Guijaun; Fu, Ting; Yin, Rulan; Zhang, Lijuan; Feng, Xingmei; Li, Liren; Gu, Zhifeng

    2017-08-01

    Disease of systemic lupus erythematosus (SLE) and periodontal disease (PD) shares the common multiple characteristics. The aims of the present study were to evaluate the prevalence and severity of periodontal disease in Chinese SLE patients and to determine the association between SLE features and periodontal parameters. A cross-sectional study of 108 SLE patients together with 108 age- and sex-matched healthy controls was made. Periodontal status was conducted by two dentists independently. Sociodemographic characteristics, lifestyle factors, medication use, and clinical parameters were also assessed. The periodontal status was significantly worse in SLE patients compared to controls. In univariate logistic regression, SLE had a significant 2.78-fold [95% confidence interval (CI) 1.60-4.82] increase in odds of periodontitis compared to healthy controls. Adjusted for potential risk factors, patients with SLE had 13.98-fold (95% CI 5.10-38.33) increased odds against controls. In multiple linear regression model, the independent variable negatively and significantly associated with gingival index was education (P = 0.005); conversely, disease activity (P periodontitis of SLE in multivariate logistic regression (OR 1.348; 95% CI: 1.183-1.536, P < 0.001). Chinese SLE patients were likely to suffer from higher odds of PD. These findings confirmed the importance of early interventions in combination with medical therapy. It is necessary for a close collaboration between dentists and clinicians when treating those patients.

  1. TIPE2 Alleviates Systemic Lupus Erythematosus Through Regulating Macrophage Polarization

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    Feng Li

    2016-01-01

    Full Text Available Background/Aims: We have recently shown that macrophage polarization may alter the pathogenesis and severity of systemic lupus erythematosus (SLE. However, a practical approach to modulate macrophage polarization in vivo is so far not available. In the current study, we aimed to use tumor necrosis factor (TNF-alpha-induced protein 8-like 2 (TIPE2 to regulate macrophage polarization in vitro and in vivo, and to study the effects on experimental SLE. Methods: We prepared adeno-associated virus carrying TIPE2 (AAV-TIPE2. We induced experimental SLE in mice with an activated lymphocyte-derived DNA (ALD-DNA method. We examined the effects of TIPE2 overexpression on macrophage polarization in vitro, and in vivo in the SLE model. We also examined the effects of TIPE2 overexpression on the severity of SLE, by serum anti-dsDNA autoantibody, renal pathological changes, and urine protein levels. Results: ALD-DNA induced SLE-like features in mice, manifested by induction of serum anti-dsDNA autoantibody, renal pathological changes, and increases in urine protein levels. TIPE2 overexpression by AAV-TIPE2 induced macrophage polarization to a M2 phenotype, in vitro and in vivo in the SLE mouse model. TIPE2 overexpression significantly decreased SLE severity. Conclusion: TIPE2 alleviates experimental SLE through induction of macrophage polarization to a M2 phenotype, which may be used as a promising therapeutic strategy for treating SLE.

  2. What constitutes uncertainty in systemic lupus erythematosus and rheumatoid arthritis?

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    Cleanthous, S; Newman, S P; Shipley, M; Isenberg, D A; Cano, S J

    2013-01-01

    Patient uncertainty is associated with conditions with no known cause or cure, such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), and despite its potential role in chronic illness management, it is still a poorly understood concept. This study constitutes an in-depth investigation of patient uncertainty in SLE and RA. We conducted (i) structured interviews with a sample of rheumatology health care professionals (HCPs) (n = 8) and (ii) in-depth, semi-structured interviews with a sample of SLE (n = 17) and RA (n = 15) patients. Interviews were audio-taped, transcribed verbatim and analysed thematically using detailed line-by-line coding. Patient uncertainty was conceptualised in a framework of five domains: symptoms and prognosis, medical management, self-management, impact and social functioning. Even though these five domains were present in both the SLE and RA data, there were some differences at the sub-domain level. Several sources of uncertainty were put forward by the HCPs and subsequently confirmed in the patient interviews including the illness trajectory, age, gender and timing. Patients reported uncertainty relative to various aspects of their illness, its management and impact. Finally, HCPs discussed the behavioural and psychosocial impact of uncertainty, which further suggests its important role in patient management.

  3. Acquired Von Willebrand’s Syndrome in Systemic Lupus Erythematosus

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    Sara Taveras Alam

    2014-01-01

    Full Text Available Acquired von Willebrand syndrome (AVWS is an uncommon, underdiagnosed, and heterogeneous disease which is increasingly recognized as a cause of bleeding diatheses. Systemic lupus erythematosus (SLE is an infrequent cause of AVWS. Herein, we report a case of AVWS diagnosed during the initial presentation of SLE in a previously healthy young man with no family history of bleeding diathesis who presented with worsening epistaxis, gastrointestinal bleeding, and anasarca. He was found to have severe anemia and prolonged activated partial thromboplastin time (aPTT with severely decreased levels of von Willebrand factor (VWF measurements in addition to markedly decreased factor VIII levels. Further evaluation revealed nephrotic syndrome and interstitial lung disease due to SLE. He initially received combination therapy with intravenous immunoglobulin (IVIG and von Willebrand factor/factor VIII concentrates without significant improvement. Treatment with steroids, cyclophosphamide, and rituximab was followed by clinical improvement evidenced by cessation of bleeding. The short follow-up did not allow us to definitely prove the therapeutic effect of immunosuppressive treatment on AVWS in SLE patients. This case adds to the literature supporting the relationship between AVWS and SLE and highlights the importance of combination therapy in the treatment of severe AVWS as well as the role of IVIG, cyclophosphamide, and rituximab in AVWS associated with SLE.

  4. Epileptic syndrome in systemic lupus erythematosus and neuronal autoantibody associations.

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    Kampylafka, E I; Alexopoulos, H; Fouka, P; Moutsopoulos, H M; Dalakas, M C; Tzioufas, A G

    2016-10-01

    We investigated systemic lupus erythematosus (SLE) patients with epilepsy, a major and organic neurological symptom. Our aim was to test patients for the autoimmune epilepsy-associated antibodies anti-GAD, anti-NMDAR, anti-AMPAR1/2, anti-GABABR and anti-VGKC. We tested sera from ten SLE patients with current or previous episodes of epileptic seizures. In addition, sera were tested for staining on primary hippocampal neurons. The patients' clinical and neuroimaging profile, disease activity and accumulated damage scores and therapeutic regimens administered were recorded, and correlations were evaluated. Patients were negative for all anti-neuronal autoantibodies tested, and showed no staining on primary hippocampal cells, which suggests the absence of autoantibodies against neuronal cell surface antigens. Epileptic seizures were all tonic-clonic, and all patients had high disease activity (mean SLE Damage Acticity Index score 19.3 ± 7.3). Six patients had minor or no brain magnetic resonance imaging findings, and three had major findings. 9/10 patients received immunosuppression for 5 ± 4 months, while anti-convulsive treatment was administered to all patients (4.2 ± 3 years). Our results suggest that the majority of SLE-related epileptic seizures cannot be attributed to the action of a single antibody against neuronal antigens. Studies with larger neuropsychiatric SLE populations and stricter inclusion criteria are necessary to verify these findings. © The Author(s) 2016.

  5. Splenectomy increases the subsequent risk of systemic lupus erythematosus.

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    Hsu, Chao-Yu; Chen, Hsuan-Ju; Hsu, Chung Y; Kao, Chia-Hung

    2016-02-01

    Splenectomy may be necessary to treat systemic lupus erythematosus (SLE) patients with thrombocytopenia; however, whether performing a splenectomy on patients without SLE increases the subsequent risk of SLE remains unknown. Therefore, this study was conducted to determine the association between splenectomy and SLE. We conducted a cohort study by using data from the Taiwan National Health Institute Research Database to identify 10,298 patients with received a splenectomy between 2000 and 2006 and 41,192 participants without received a splenectomy who were selected by frequency matched based on sex, age, and the index year. Cox proportional hazards models were used to estimate the hazard ratios (HRs) and 95 % confidence intervals (CIs) of developing SLE associated with splenectomy compared with patients who did not receive a splenectomy. During the study period, the overall incidence density rate of SLE was higher in the splenectomy cohort than in the non-splenectomy cohort (adjusted HR 10.55; 95 % CI 50.55-20.05). The incidence density rates of SLE in women and men who received a splenectomy were higher than those of patients who did not receive a splenectomy. Non-traumatic splenectomy increases the subsequent risk of SLE. The risk of SLE should be considered before performing a splenectomy, particularly in women and younger patients.

  6. Systemic lupus erythematosus activity and beta two microglobulin levels

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    Thelma Larocca Skare

    Full Text Available CONTEXT AND OBJECTIVE: Systemic lupus erythematosus (SLE is an autoimmune disease with a cyclical clinical course. Evaluation of the clinical activity of this disease is important for choosing the correct treatment. The objective of this study was to analyze the value of beta-2 microglobulin (β2M serum levels in determining SLE clinical activity.DESIGN AND SETTING: Cross-sectional analytical study conducted at the rheumatology outpatient clinic of a private university hospital.METHODS: 129 SLE patients were studied regarding disease activity using SLEDAI (SLE Disease Activity Index and cumulative damage using SLICC ACR (SLE International Collaborating Clinics/American College of Rheumatology Damage Index for SLE. At the same time, the β2M serum level, ESR (erythrocyte sedimentation rate, anti-dsDNA (anti-double-stranded DNA and C3 and C4 complement fractions were determined.RESULTS: β2M levels correlated positively with SLEDAI (P = 0.02 and ESR (P = 0.0009 and negatively with C3 (P = 0.007. Patients who were positive for anti-dsDNA had higher β2M serum levels (P = 0.009.CONCLUSION: β2M levels are elevated in SLE patients with active disease.

  7. The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus

    Science.gov (United States)

    Ünlü, Ozan; Zuily, Stephane; Erkan, Doruk

    2016-01-01

    Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage. The use of low-dose aspirin (LDA) is controversial for primary thrombosis and pregnancy morbidity prevention because of the lack of strong prospective controlled data. Similarly, the use of anticoagulation is controversial for patients with an aPL-related nephropathy. Until further studies are available, physicians should discuss the risk/benefits of LDA or anticoagulation as well as the available literature with patients. PMID:27708976

  8. Neuropsychiatric manifestations of systemic lupus erythematosus: Iranian experience

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    Haghighi Afshin

    2010-01-01

    Full Text Available Aims: To evaluate the prevalence and characteristics of different neurological and psychiatric presentations in patients admitted to hospital with systemic lupus erythematosus (SLE. Materials and Methods: In this retrospective hospital-based study, we examined the medical records of patients with SLE who were referred to the hospitals affiliated to Shiraz University of Medical Sciences from 1995 to 2005. All patients of SLE who had clinical neurological or psychiatric features were included in this study. The patients′ demographic data, findings on general examination, neuropsychiatric manifestations, and the results of laboratory investigations and imaging studies were recorded. Clinical manifestations were classified according to the American College of Rheumatology (ACR case definitions. Results: Of the 407 study patients, 11.3% had neuropsychiatric complications. The most frequent findings were seizure (63%, headache (60%, and decreased level of consciousness (50%. Cerebrovascular disease (28.3%, seizure disorder (26.5%, and acute confusional state (19.6% were the most prevalent syndromes. Conclusion: The prevalence and nature of different neurological presentations of SLE in Iranian patients has some similarities to that seen in other populations, as well as some differences. Ethnic and environmental factors may contribute to these differences.

  9. Tolerogenic probiotics: potential immunoregulators in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Esmaeili, Seyed-Alireza; Mahmoudi, Mahmoud; Momtazi, Amir Abbas; Sahebkar, Amirhossein; Doulabi, Hassan; Rastin, Maryam

    2017-08-01

    Probiotics are commensal or nonpathogenic microbes that colonize the gastrointestinal tract and confer beneficial effects on the host through several mechanisms such as competitive exclusion, anti-bacterial effects, and modulation of immune responses. There is growing evidence supporting the immunomodulatory ability of some probiotics. Several experimental and clinical studies have been shown beneficial effect of some probiotic bacteria, particularly Lactobacillus and Bifidobacteria strains, on inflammatory and autoimmune diseases. Systemic lupus erythematosus (SLE) is an autoimmune disease that is mainly characterized by immune intolerance towards self-antigens. Some immunomodulatory probiotics have been found to regulate immune responses via tolerogenic mechanisms. Dendritic and T regulatory (Treg) cells, IL-6, IFN-γ, IL-17, and IL-23 can be considered as the most determinant dysregulated mediators in tolerogenic status. As demonstrated by documented experimental and clinical trials on inflammatory and autoimmune diseases, a number of probiotic bacterial strains can restore tolerance in host through modification of such dysregulated mediators. Since there are limited reports regarding to impact of probiotic supplementation in SLE patients, the preset review was aimed to suggest a number of probiotics bacteria, mainly from Bifidobacteria and Lactobacillus strains that are able to ameliorate immune responses. The aim was followed through literature survey on immunoregulatory probiotics that can restore tolerance and also modulate the important dysregulated pro/anti-inflammatory cytokines contributing to the pathogenesis of SLE. © 2016 Wiley Periodicals, Inc.

  10. Management of cardiovascular complications in systemic lupus erythematosus

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    Skamra, Carly; Ramsey-Goldman, Rosalind

    2010-01-01

    Cardiovascular disease (CVD) is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Patients with SLE have an excess risk compared with the general population; this is particularly pronounced in younger women with SLE who have an excess risk of over 50-fold compared with population controls. There is a higher prevalence of subclinical atherosclerosis in patients with SLE compared with controls, as demonstrated by a variety of imaging modalities discussed in this review. The causality of the excess risk of CVD and subclinical atherosclerosis is multifactorial in patients with SLE. While traditional risk factors play a role, after controlling for the traditional Framingham risk factors, the excess risk is still 7.5-fold greater than the general population. This review will also cover novel cardiovascular risk factors and some SLE-specific variables that contribute to CVD risk. This review discusses the risk factor modification and the evidence available for treatment of these risk factors in SLE. There have not yet been any published randomized, controlled trials in patients with SLE with respect to CVD risk factor modifications. Thus, the treatment and management recommendations are based largely on published guidelines for other populations at high risk for CVD. PMID:20305727

  11. Aging and Systemic Lupus Erythematosus - Immunosenescence and Beyond.

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    van den Hoogen, Lucas Laurens; Sims, Gary Patrick; van Roon, Joel Adrianus Gijsbert; Fritsch-Stork, Ruth Dorothea Elisabeth

    2015-01-01

    The lifespan of humans has increased drastically over the last decades; considerable effort has been applied to delineate the mechanisms behind aging in order to find strategies for longevity. As the benefits of the gained knowledge might extend to diseases, where accelerated aging is suspected, the role of aging in the systemic autoimmune disease Systemic Lupus Erythematosus (SLE) is of particular interest. In this review the immunological similarities of SLE and aging are analyzed on three levels: the clinical, the cellular and the molecular, in order to find possible common pathological mechanisms. Common clinical features (e.g. increased infection rates, incidence of tumors and cardiovascular diseases) of SLE-patients and elderly individuals and shared characteristics of immuno-senescence and SLE are identified. These similarities are strongest in the adaptive immune system, where terminally differentiated T-cells and an immunological risk profile are found in both conditions. Also the aging innate immune system has overlapping features with SLE, exemplified by a generally lowered phagocytic capacity. However, great disparities between the aging immune system and SLE become apparent on a closer look, affecting numbers, phenotype and function of most immune cells, ranging from NETosis by granulocytes to the mechanisms underlying abnormal IL-2 production by T-cells. On the molecular level, also the increased presence of aging mechanisms like telomere attrition, DNA damage, autophagy and the characteristics of the mTOR pathway in SLE, possibly contributing to the shared changes on the cellular and clinical level are elaborated. The possible implications thereof concern existing (hydroxychloroquine, rapamycine, Glucocorticoids) as well as novel therapeutic strategies targeting more specific pathways which might rapidly reach the clinical arena. Overall a differential view on the similarities of aging and SLE and possible consequences is presented.

  12. B-cell targeted therapies in systemic lupus erythematosus: successes and challenges.

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    Harvey, Philip R; Gordon, Caroline

    2013-04-01

    Systemic lupus erythematosus is a multisystem autoimmune disease characterized by the formation of autoantibodies that target a variety of self antigens. B cells are fundamental to the development of these antibodies and are a target for intervention in the disease. This review discusses four therapies that target B cells by inducing B-cell depletion, reduction in B-cell proliferation and differentiation, or modulation of B-cell function. Rituximab is an anti-CD20 chimeric monoclonal antibody that depletes B cells but not plasma cells. Systematic reviews of open label studies, particularly in lupus patients refractory to conventional therapy, have suggested that rituximab can be an effective treatment for non-renal lupus and lupus nephritis. However, randomized, double-blind, controlled trials comparing rituximab with placebo in addition to standard of care therapy for non-renal lupus and lupus nephritis over 12 months failed to demonstrate efficacy using the planned primary endpoints, although there were some post-hoc analyses suggesting that rituximab may have beneficial effects that would be worthy of further study as no significant toxicity has been demonstrated. Treatment with belimumab, a humanized monoclonal antibody targeted against B lymphocyte stimulator (BLys), was more efficacious than placebo and had no significant increase in adverse events in two non-renal, phase III lupus trials when given in addition to standard of care therapy for 52 weeks. Belimumab is licensed for the management of lupus in the US and in Europe. Atacicept is a humanized fusion protein that binds BLys and APRIL (a proliferation-inducing ligand) that might be more effective than belimumab in the management of lupus. Unfortunately a phase II/III trial of atacicept in lupus nephritis had to be stopped due to the development of low immunoglobulin levels and pneumonias in some patients. However, in retrospect these complications may have been due to concomitant treatment with

  13. Pregnancy Outcomes in Chinese Patients with Systemic Lupus Erythematosus (SLE: A Retrospective Study of 109 Pregnancies.

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    Ming Ku

    Full Text Available Systemic lupus erythematosus (SLE is a multisystem autoimmune disease that primarily affects women during their reproductive years. The interaction between SLE and pregnancy remains debated. The objective of this study was to analyze the fetal and maternal outcomes of Chinese women with SLE. A total of 109 pregnancies in 83 SLE patients from June 2004 to June 2014 at a tertiary university hospital were reviewed retrospectively. Patients' characteristics, clinical and laboratory data during pregnancy were obtained from electronic medical records. After exclusion of elective abortions, the live birth rate was 61.5%. Significantly, APS (antiphospholipid syndrome, disease activity, hypertension, hypocomplementemia, thrombocytopenia, and anemia during pregnancy were more commonly observed in fetal loss pregnancies than in live birth pregnancies. Compared to the 64 women with a history of SLE, 19 women with new-onset lupus during pregnancy had worse pregnancy outcome. Furthermore, the 64 patients with a history of SLE were divided into lupus nephritis group and SLE group (non-renal involvement. We found that the lupus nephritis group had worse maternal outcome than the SLE group. We conclude that new-onset lupus during pregnancy predicts both adverse maternal and fetal outcomes, while a history of lupus nephritis predicts adverse maternal outcomes. It is essential to provide SLE women with progestational counseling and regular multispecialty care during pregnancy.

  14. Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus

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    Ali Ghavidel

    2015-08-01

    Full Text Available Introduction: Hepatic venous outflow block or Budd-Chiari syndrome is a severe liver disease with a 3 years survival rate of 50%. Several conditions have been implicated as a cause of Budd-Chiari syndrome, including myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, the presence of lupus anti-coagulant, oral contraceptives, pregnancy, and others. In a small number of cases, Budd-Chiari syndrome is associated with the presence of lupus anticoagulant. Anticardiolipin antibodies (ACA are similar to lupus anti-coagulant antiphospholipid antibodies (APLAs, which have been described in patients with recurrent arterial and venous thrombosis, thrombocytopenia, fetal loss, or miscarriage. Case Report: A 23-year-old woman is reported with Budd-Chiari syndrome in whom lupus anticoagulant and anticardiolipin antibodies were shown; 9 months after diagnosis of systemic lupus erythematosus (SLE treatment with steroids admitted with gastrointestinal problems, abdominal pain and ascites and treated oral anticoagulants induced a considerable improvement. This treatment was continued after 1 year, but interruption was followed by redevelopment of ascites. Further treatment with anticoagulants was continued for 5 years with noticeable improvement. Conclusion: Patients with Budd-Chiari syndrome should be tested for lupus anticoagulants and anticardiolipin antibodies, Budd-Chiari syndrome resulting from this cause may have a good response to treatment with oral anticoagulants; this treatment should be maintained permanently, and pregnancy in such patients may initiate serious difficulties. The condition of the patient at follow-up was good.

  15. Predictors of poor sleep quality in patients with systemic lupus erythematosus.

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    Inoue, M; Shiozawa, K; Yoshihara, R; Yamane, T; Shima, Y; Hirano, T; Makimoto, K

    2017-05-01

    Sleep problems are common in patients with systemic lupus erythematosus (SLE). This study aimed to examine the following: (1) predictors of sleep quality and (2) fluctuations in sleep quality in patients with SLE. Patients with SLE were recruited from three rheumatology centers in Japan. We collected demographic and clinical data and data on sleep quality as measured by the Pittsburgh Sleep Quality Index (PSQI), the Medical Outcome Study Short Form-12, and the Lupus Patient Reported Outcome Tool (LupusPRO). Fluctuations in sleep quality were examined by administering the PSQI a second time after a 2-week interval. We used multiple linear regression analysis to predict sleep quality. Of 205 patients who completed the survey, 62.9% showed poor sleep quality. The largest fluctuation in sleep quality was for "waking in the middle of the night or early morning." "LupusPRO pain/vitality" was a major predictor of poor sleep. The other significant predictors were mostly LupusPRO subscales and clinical variables and SF-12 subscales were mostly non-predictive. The majority of the participants had poor sleep quality. A lupus-specific QoL scale is important for understanding poor sleep quality in SLE patients. Symptom management appeared to play a key role in improving sleep quality.

  16. Pregnancy Outcomes in Chinese Patients with Systemic Lupus Erythematosus (SLE): A Retrospective Study of 109 Pregnancies.

    Science.gov (United States)

    Ku, Ming; Guo, Shuiming; Shang, Weifeng; Li, Qing; Zeng, Rui; Han, Min; Ge, Shuwang; Xu, Gang

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that primarily affects women during their reproductive years. The interaction between SLE and pregnancy remains debated. The objective of this study was to analyze the fetal and maternal outcomes of Chinese women with SLE. A total of 109 pregnancies in 83 SLE patients from June 2004 to June 2014 at a tertiary university hospital were reviewed retrospectively. Patients' characteristics, clinical and laboratory data during pregnancy were obtained from electronic medical records. After exclusion of elective abortions, the live birth rate was 61.5%. Significantly, APS (antiphospholipid syndrome), disease activity, hypertension, hypocomplementemia, thrombocytopenia, and anemia during pregnancy were more commonly observed in fetal loss pregnancies than in live birth pregnancies. Compared to the 64 women with a history of SLE, 19 women with new-onset lupus during pregnancy had worse pregnancy outcome. Furthermore, the 64 patients with a history of SLE were divided into lupus nephritis group and SLE group (non-renal involvement). We found that the lupus nephritis group had worse maternal outcome than the SLE group. We conclude that new-onset lupus during pregnancy predicts both adverse maternal and fetal outcomes, while a history of lupus nephritis predicts adverse maternal outcomes. It is essential to provide SLE women with progestational counseling and regular multispecialty care during pregnancy.

  17. MR imaging findings suggestive of posterior reversible encephalopathy syndrome in adolescents with systemic lupus erythematosus

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    Muscal, Eyal; De Guzman, Marietta M.; Myones, Barry L. [Texas Children' s Hospital, Baylor College of Medicine and Pediatric Rheumatology Center, Houston, TX (United States); Traipe, Elfrides; Hunter, Jill V. [Texas Children' s Hospital, Baylor College of Medicine and Diagnostic Imaging, Houston, TX (United States); Brey, Robin L. [University of Texas Health Science Center at San Antonio, Department of Neurology, San Antonio, TX (United States)

    2010-07-15

    Endothelial damage, hypertension and cytotoxic medications may serve as risk factors for the posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus. There have been few case reports of these findings in pediatric lupus patients. We describe clinical and neuroimaging findings in children and adolescents with lupus and a PRES diagnosis. We identified all clinically acquired brain MRIs of lupus patients at a tertiary care pediatric hospital (2002-2008). We reviewed clinical features, conventional MRI and diffusion-weighted imaging (DWI) findings of patients with gray- and white-matter changes suggestive of vasogenic edema and PRES. Six pediatric lupus patients presenting with seizures and altered mental status had MRI findings suggestive of PRES. In five children clinical and imaging changes were seen in conjunction with hypertension and active renal disease. MRI abnormalities were diffuse and involved frontal regions in five children. DWI changes reflected increased apparent diffusivity coefficient (unrestricted diffusion in all patients). Clinical and imaging changes significantly improved with antihypertensive and fluid management. MRI changes suggestive of vasogenic edema and PRES may be seen in children with active lupus and hypertension. The differential diagnosis of seizures and altered mental status should include PRES in children, as it does in adults. (orig.)

  18. Linguistic Validation of the LupusQoL for the Assessment of Quality of Life in Iranian Patients with Systemic Lupus Erythematosus

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    Naeimehossadat Hosseini

    2014-01-01

    Full Text Available Objectives. We evaluated the psychometric properties of the Persian LupusQoL for the evaluation of quality of life in Iranian systemic lupus erythematosus (SLE patients. Methods. The LupusQoL was translated to Persian language. Patients with SLE (n=78 completed the LupusQoL and the Short-Form Health Survey (SF-36. Disease activity and cumulative disease damage were assessed with standard indices. The psychometric properties of the scale were evaluated. Results. The Cronbach’s alpha was 0.97 for the total LupusQoL (above 0.8 for subscales. There were strong corrected item-total (r>0.4, item-subscale (r≥0.5, and subscale-total correlations (r>0.6, as well as intersubscale correlations (r>0.5. Patients with active disease and patients with disease damage index of ≥1 had lower scores in domains of planning, emotional health, burden to others, and body image than patients with inactive disease and those with no disease damage, respectively (P0.4. Conclusion. The psychometric characteristics of the Persian version of LupusQoL questionnaire are acceptable in Iranian population. This instrument can be used to evaluate quality of life in Iranian SLE patients.

  19. Survival in systemic lupus erythematosus, 1995-2010

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    Voss, A; Laustrup, H; Hjelmborg, J

    2013-01-01

    ObjectiveThe objective of this paper is to investigate survival and causes of death in a Danish lupus population.MethodsTwo hundred and fifteen SLE patients (94% Caucasians) were followed prospectively for up to 16 years. Thirty-eight patients died. Survival rate and causes of death were analysed......%) and malignancies (13%). Deaths due to infections and active SLE were rare and predominated within the first seven years after diagnosis and before age 40, while cardiovascular deaths prevailed after 20 years' follow-up.ConclusionThis study shows that despite progress in lupus management, including direct access...... to specialized hospital care and increased use of hydroxychloroquine, mortality in lupus patients is still increased. Main causes of death were active disease and infections among the young and newly diagnosed, while cardiovascular deaths prevailed in longstanding disease....

  20. Aplastic anemia associated to systemic lupus erythematosus in an AIDS patient: a case report

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    Leonardo Rodrigues de Oliveira

    2013-01-01

    Full Text Available Aplastic anemia is a bone marrow failure syndrome characterized by peripheral cytopenias and hypocellular bone marrow. Although aplastic anemia is idiopathic in most cases, rheumatic diseases such as systemic lupus erythematosus are recognized as causes of aplastic anemia, with their possible etiological mechanisms being T and B lymphocyte dysfunction and pro-inflammatory cytokines and autoantibody production directed against bone marrow components. In the course of the human immunodeficiency virus infection/acquired immunodeficiency syndrome, the identification of autoantibodies and the occurrence of rheumatic events, in addition to the natural course of systemic lupus erythematosus which is modified by immune changes that are characteristic of human immunodeficiency virus infection/acquired immunodeficiency syndrome, make the diagnosis of systemic lupus erythematosus challenging. This study reports the case of a woman with acquired immunodeficiency syndrome treated with a highly active antiretroviral therapy, who had prolonged cytopenias and hypocellular bone marrow consistent with aplastic anemia. The clinical picture, high autoantibodies titers, and sustained remission of the patient's hematological status through immunosuppression supported the diagnosis of systemic lupus erythematosus-associated aplastic anemia. This is the first report of aplastic anemia concurrent with systemic lupus erythematosus and acquired immunodeficiency syndrome, providing additional evidence that immune dysfunction is a key part of the pathophysiological mechanism of aplastic anemia.

  1. Management of cardiovascular risk in systemic lupus erythematosus: a systematic review.

    Science.gov (United States)

    Andrades, C; Fuego, C; Manrique-Arija, S; Fernández-Nebro, A

    2017-11-01

    Systemic lupus erythematosus is associated with accelerated atherosclerosis and increased risk of cardiovascular complications. The aim of this study was to review the effectiveness of interventions for primary and secondary prevention of cardiovascular events and mortality and to review the effectiveness of interventions for cardiovascular risk factor reduction in systemic lupus erythematosus patients. A systematic review was conducted. Electronic databases Medline and Embase (1961-2015) were searched. Nineteen articles met the inclusion criteria and were selected. Low-calorie and/or low glycaemic index calories may be a useful option for secondary prevention in obese patients with systemic lupus erythematosus, and exercise would be useful in improving the endothelial function measured by flow-mediated dilation in this group of patients. The use of lipid-lowering drugs may improve the lipid profile in patients with systemic lupus erythematosus and hyperlipidaemia, but the effect of this treatment on overall cardiovascular mortality remains unknown. Antiplatelets, anticoagulants, antimalarials and lipid-lowering drugs may be effective in the primary and secondary prevention of major cardiovascular events, such as acute myocardial infarction or stroke. Similarly, lipid-lowering drugs and antimalarial drugs appear to reduce the serum levels of total cholesterol, low-density lipoprotein, glucose, diastolic blood pressure and calcium deposition at the coronary arteries. They may also improve insulin resistance and the level of high-density lipoproteins. It appears that treatment with antihypertensive drugs reduces blood pressure in patients with systemic lupus erythematosus, but the available studies are of low quality.

  2. Cytokines in relation to autoantibodies before onset of symptoms for systemic lupus erythematosus.

    Science.gov (United States)

    Eriksson, C; Rantapää-Dahlqvist, S

    2014-06-01

    A number of cytokines and chemokines were analysed and related to autoantibodies in blood samples pre-dating the onset of symptoms of systemic lupus erythematosus. Thirty-five patients with systemic lupus erythematosus (American College of Rheumatology criteria) were identified as having donated blood samples, prior to symptom onset, to the Biobank of northern Sweden. Altogether, 140 age- and sex-matched controls were also identified. The concentrations of interferon-α, interleukin-4, interleukin-9, interleukin-10, interferon inducible protein-10 and monocyte chemotactic protein-1 were analysed using multiplex technology and related to autoantibodies (ANA, ENA, anti-dsDNA and anti-histone antibodies) analysed from the same blood sample. The interferon-γ inducible protein-10 levels were higher in the pre-symptomatic individuals than in controls (p lupus erythematosus. An increased concentration of interferon-γ inducible protein-10 pre-dated the onset of systemic lupus erythematosus and was related to autoantibodies before the onset of disease. The levels of interferon-γ inducible protein-10 and interferon-α were correlated. These findings support the proposal that the interferon system is important early in the pathogenesis of systemic lupus erythematosus and autoantibody formation. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  3. Neurodevelopmental disorders in children born to mothers with systemic lupus erythematosus.

    Science.gov (United States)

    Vinet, É; Pineau, C A; Clarke, A E; Fombonne, É; Platt, R W; Bernatsky, S

    2014-10-01

    Children born to women with systemic lupus erythematosus seem to have a potentially increased risk of neurodevelopmental disorders compared to children born to healthy women. Recent experimental data suggest in utero exposure to maternal antibodies and cytokines as important risk factors for neurodevelopmental disorders. Interestingly, women with systemic lupus erythematosus display high levels of autoantibodies and cytokines, which have been shown, in animal models, to alter fetal brain development and induce behavioral anomalies in offspring. Furthermore, subjects with systemic lupus erythematosus and neurodevelopmental disorders share a common genetic predisposition, which could impair the fetal immune response to in utero immunologic insults. Moreover, systemic lupus erythematosus pregnancies are at increased risk of adverse obstetrical outcomes and medication exposures, which have been implicated as potential risk factors for neurodevelopmental disorders. In this article, we review the current state of knowledge on neurodevelopmental disorders and their potential determinants in systemic lupus erythematosus offspring. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  4. Schizophrenia or Atypical Lupus Erythematosus with Predominant Psychiatric Manifestations over 25 Years: Case Analysis and Review

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    Axel Mack

    2017-07-01

    Full Text Available We observed a case over 25 years of relapsing–remitting schizophrenic spectrum disorder, varying regarding the main symptomatology between more depressive or more schizoaffective or rather typical schizophrenic syndrome. Diseased phases were repeatedly accompanied by minor skin lesions, which were initially classified as mixed tissue disorder. Psychotic phases were waxing–waning over years. During one later relapse, skin involvement was severe, classified to likely represent an allergic reaction to psychopharmaca; this generalized exanthema remitted rapidly with cortisone treatment and azathioprine. Under continued azathioprine and low dose neuroleptics, the patient remitted completely, appearing psychiatrically healthy for 16 years. When azathioprine was set off due to pregnancy, an extraordinary severe relapse of schizophrenia like psychosis accompanied by most severe skin lesions developed within a few weeks, then requiring 2 years of psychiatric inpatient treatment. Finally, a diagnosis of systemic lupus erythematodes plus neuropsychiatric lupus was made. A single CSF sample in 2013 showed suspicious biomarkers, matching with CSF cytokine profiling in schizophrenic and affective spectrum disorder patients and indicated mild neuroinflammation. Complex immune suppressive treatment was reinitiated short after relapse, but was only partially successful. However, surprisingly the psychosis and skin lesions remitted (in parallel when belimumab was given (add-on. The very details of this complicated, long-term disease course are discussed also with regard to general ideas, in particular with respect to the question if this case of seemingly comorbid schizophrenia with minor autoimmunity signs represented a case of one emerging autoimmune disorder with variant manifestations systemically and within the CNS, though atypically with predominant appearance as a schizophrenia spectrum disorder.

  5. Erythema Multiforme Like Lesions in a Case of Systemic Lupus Erythematosus

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    Aslı Günaydın

    2012-09-01

    Full Text Available Cutaneous involvement may be seen in 70-85% of patients with systemic lupus erythematosus. Erythema multiforme like lesions are considered as non-specific findings in lupus erythematosus and are rarely seen in the systemic form. Seventeen-year-old woman admitted to our clinic with multiple, violet colored, targetoid lesions on the palms and dorsal areas of both hands and dorsal areas of the feet with a 4-month of duration. She was complaining of photosensitivity and hair loss for a few years. Histopathologic examination revealed hyperkeratosis, parakeratosis, single cell necrosis through the epidermis and perivascular mixed cellular infiltration. C3 and C1q were detected at the dermoepidermal junction in immunofluorescence examination. In laboratory, antinuclear antibody was positive. Elevated sedimentation rate, anemia, and hypocomplementemia were detected. Lupus band test was positive. According to these findings, the patient was diagnosed as systemic lupus erythematosus and the lesions on the hand and feet as erythema multiforme like lesions. Methylprednisolone (1mg/kg/day was started. Notable improvement in her symptoms and in the clinical appearance of the lesion was observed. Erythema multiforme like lesions are the cutaneous findings rarely seen in systemic lupus erythematosus. In the present case, since there was no drug use or herpes virus infection in the history. (Turk J Dermatol 2012; 6: 102-5

  6. Erythema Multiforme Like Lesions in a Case of Systemic Lupus Erythematosus - Case Report

    Directory of Open Access Journals (Sweden)

    Aslı Günaydın

    2012-09-01

    Full Text Available Cutaneous involvement may be seen in 70-85% of patients with systemic lupus erythematosus. Erythema multiforme like lesions are considered as non-specific findings in lupus erythematosus and are rarely seen in the systemic form. Seventeen-year-old woman admitted to our clinic with multiple, violet colored, targetoid lesions on the palms and dorsal areas of both hands and dorsal areas of the feet with a 4-month of duration. She was complaining of photosensitivity and hair loss for a few years. Histopathologic examination revealed hyperkeratosis, parakeratosis, single cell necrosis through the epidermis and perivascular mixed cellular infiltration. C3 and C1q were detected at the dermoepidermal junction in immunofluorescence examination. In laboratory, antinuclear antibody was positive. Elevated sedimentation rate, anemia, and hypocomplementemia were detected. Lupus band test was positive. According to these findings, the patient was diagnosed as systemic lupus erythematosus and the lesions on the hand and feet as erythema multiforme like lesions. Methylprednisolone (1mg/kg/day was started. Notable improvement in her symptoms and in the clinical appearance of the lesion was observed. Erythema multiforme like lesions are the cutaneous findings rarely seen in systemic lupus erythematosus. In the present case, since there was no drug use or herpes virus infection in the history.

  7. Risk factors for ovarian failure in patients with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Medeiros M.M.C.

    2001-01-01

    Full Text Available The aim of the present study was to identify the risk factors for ovarian failure in patients with systemic lupus erythematosus. Seventy-one women aged 17 to 45 years with systemic lupus erythematosus were studied. Patients were interviewed and their medical records reviewed. Demographic characteristics, clinical and serologic profiles, and menstrual and obstetric histories were recorded. Disease activity was measured by the systemic lupus erythematosus disease activity index. Serum FSH, LH, estradiol, progesterone, TSH, prolactin, and antimicrosomal and antithyroglobulin antibodies were measured. Patients who developed ovarian failure were compared to those who did not. Ovarian failure occurred in 11 patients (15.5% and nine had premature menopause (11.3%. Cyclophosphamide administration and older patient age were found to be associated with ovarian failure. The cumulative cyclophosphamide dose was significantly higher in patients with ovarian failure than in those without this condition (18.9 vs 9.1 g; P = 0.04. The relative risk for ovarian failure in patients with cumulative cyclophosphamide dose higher than 10 g was 3.2. TSH levels were high in 100% of patients with ovarian failure who had received pulse cyclophosphamide. Ovarian failure, and premature menopause in particular, is common in patients with systemic lupus erythematosus, with the most important risk factors being cyclophosphamide dose and age. Thyroid problems may be another risk factor for ovarian failure in patients with lupus.

  8. Distribution and clinical significance of lupus anticoagulant and anticardiolipin antibody in 349 patients with systemic lupus erythematosus.

    Science.gov (United States)

    Ninomiya, C; Taniguchi, O; Kato, T; Hirano, T; Hashimoto, H; Hirose, S

    1992-02-01

    Samples from 349 patients with systemic lupus erythematosus (SLE) were tested simultaneously for lupus anticoagulant (LAC) and anticardiolipin antibodies (ACL). LAC was detected in 27.2% of 349 SLE patients by a modified mixing kaolin clotting time. ACL was detected in 34.7% by enzyme-linked immunosorbent assay. Only half of the patients who had LAC or ACL were positive for both of them. In addition, isotypes of ACL in these patients were studied. The IgG isotype was detected in 81.8% of 121 patients, and more than half had only the IgG isotype. When clinical features of patients with LAC or ACL were studied, the incidence of thrombosis, fetal loss, and thrombocytopenia were significantly higher in both groups compared with patients without LAC or ACL. In particular, the patients with both LAC and ACL showed the highest risk of fetal loss (89%) during pregnancy. These results indicate that LAC and ACL are detected in partly different groups of SLE patients, but both of these groups are clinically similar.

  9. CXCL13 as a new biomarker of systemic lupus erythematosus and lupus nephritis - from bench to bedside?

    Science.gov (United States)

    Schiffer, L; Worthmann, K; Haller, H; Schiffer, M

    2015-01-01

    Different studies over the last decade have linked the B cell-attracting chemokine CXC ligand 13 (CXCL13) to the autoimmune disease systemic lupus erythematosus (SLE). A pathogenetic role of this chemokine for disease manifestation in SLE was described initially in mouse models for SLE. Mechanisms of CXCL13 actions were also identified in SLE patients. Moreover, various clinical studies have identified CXCL13 serum levels as a useful biomarker in patients with SLE of different ethnicities for disease activity. In addition, CXCL13 seems to be a promising marker for the diagnosis of lupus nephritis, one of the most severe complications of SLE. However, its exact place within the mechanisms that lead to SLE remains to be defined. Further research is needed to resolve more details of the pathomechanism and the signalling pathway of CXCL13 in SLE. Blocking CXCL13 or the signal pathways of CXCL13 is seen as a promising therapeutic approach for SLE and will be addressed in the near future. This review summarizes all papers that linked CXCL13 to SLE and highlights its importance in the pathogenesis and diagnosis of SLE. © 2014 British Society for Immunology.

  10. The African Lupus Genetics Network (ALUGEN) registry: standardized, prospective follow-up studies in African patients with systemic lupus erythematosus.

    Science.gov (United States)

    Hodkinson, B; Mapiye, D; Jayne, D; Kalla, A; Tiffin, N; Okpechi, I

    2016-03-01

    The prevalence and severity of systemic lupus erythematosus (SLE) differs between ethnic groups and geographical regions. Although initially reported as rare, there is growing evidence that SLE is prevalent and runs a severe course in Africa. There is a paucity of prospective studies on African SLE patients. The African Lupus Genetics Network (ALUGEN) is a multicentred framework seeking to prospectively assess outcomes in SLE patients in Africa. Outcomes measured will be death, hospital admission, disease activity flares, and SLE-related damage. We will explore predictors for these outcomes including clinical, serological, socio-demographic, therapeutic and genetic factors. Further, we will investigate comorbidities and health-related quality of life amongst these patients. Data of patients recently (≤ 5 yrs) diagnosed with SLE will be collected at baseline and annual follow-up visits, and captured electronically. The ALUGEN project will facilitate standardized data capture for SLE cases in Africa, allowing participating centres to develop their own SLE registries, and enabling collaboration to enrich our understanding of inter-ethnic and regional variations in disease expression. Comprehensive, high-quality multi-ethnic data on African SLE patients will expand knowledge of the disease and inform clinical practice, in addition to augmenting research capacity and networking links and providing a platform for future biomarker and interventional studies. © The Author(s) 2015.

  11. 17-β Estradiol reduces atherosclerosis without exacerbating lupus in ovariectomized systemic lupus erythematosus-susceptible LDLr−/− mice

    Science.gov (United States)

    Shelton, KA; Cline, JM; Cann, JA

    2013-01-01

    Objective To test the hypothesis that estrogen treatment in a radiation chimera mouse model of systemic lupus erythematosus (SLE) and atherosclerosis will increase SLE-associated atherosclerosis by increasing autoantibody production and inflammation. Methods We used a radiation chimera mouse model in which bone marrow from the polygenic B6.Sle1.2.3 model of SLE was transferred to the low density lipoprotein receptor knock out (LDLr−/−) model of atherosclerosis on a C57BL/6 background (Sle/LDLr−/−). Ovariectomized chimeric mice were treated for 10 weeks with either 5.6 ug/day of 17β-estradiol or placebo; outcomes included atherosclerosis plaque size, anti-dsDNA autoantibody production and renal pathology. Results Mean atherosclerosis plaque size was 67.4 ± 7.6% smaller in the estrogen treated group (patherosclerosis lesion size and either the renal histology score or UP:UC ratio in Sle/LDLr−/− mice. Conclusion These results indicate that 17β-estradiol is atheroprotective within the context of murine SLE independent of changes in serum cholesterol concentration, autoantibody concentration, or renal pathology. The SLE phenotype in Sle/LDLr−/− mice is not exacerbated by exogenous 17β-estradiol administration, and the reduced UP:UC ratio suggests a protective effect against lupus nephritis. PMID:23395521

  12. 17-β Estradiol reduces atherosclerosis without exacerbating lupus in ovariectomized systemic lupus erythematosus-susceptible LDLr(-/-) mice.

    Science.gov (United States)

    Shelton, K A; Cline, J M; Cann, J A

    2013-04-01

    To test the hypothesis that estrogen treatment in a radiation chimera mouse model of systemic lupus erythematosus (SLE) and atherosclerosis will increase SLE-associated atherosclerosis by increasing autoantibody production and inflammation. We used a radiation chimera mouse model in which bone marrow from the polygenic B6.Sle1.2.3 model of SLE was transferred to the low density lipoprotein receptor knock out (LDLr(-/-)) model of atherosclerosis on a C57BL/6 background (Sle/LDLr(-/-)). Ovariectomized chimeric mice were treated for 10 weeks with either 5.6 μg/day of 17β-estradiol or placebo; outcomes included atherosclerosis plaque size, anti-dsDNA autoantibody production and renal pathology. Mean atherosclerosis plaque size was 67.4 ± 7.6% smaller in the estrogen treated group (p atherosclerosis lesion size and either the renal histology score or UP:UC ratio in Sle/LDLr(-/-) mice. These results indicate that 17β-estradiol is atheroprotective within the context of murine SLE independent of changes in serum cholesterol concentration, autoantibody concentration, or renal pathology. The SLE phenotype in Sle/LDLr(-/-) mice is not exacerbated by exogenous 17β-estradiol administration, and the reduced UP:UC ratio suggests a protective effect against lupus nephritis. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  13. Clinical associations of serum interleukin-17 in systemic lupus erythematosus

    Science.gov (United States)

    2013-01-01

    Introduction Serum interleukin (IL)-17 concentrations have been reported to be increased in systemic lupus erythematosus (SLE), but associations with clinical characteristics are not well understood. We characterized clinical associations of serum IL-17 in SLE. Methods We quantified IL-17 in serum samples from 98 SLE patients studied cross-sectionally, and in 246 samples from 75 of these patients followed longitudinally over two years. Disease activity was recorded using the SLE Disease Activity Index (SLEDAI)-2k. Serum IL-6, migration inhibitory factor (MIF), and B cell activating factor of the tumour necrosis factor family (BAFF) were also measured in these samples. Results Serum IL-17 levels were significantly higher in SLE patients compared to healthy donors (P IL-17 and SLEDAI-2k, at baseline or during longitudinal follow-up. However, we observed that SLEDAI-2k was positively correlated with IL-17/IL-6 ratio. Serum IL-17 was significantly increased in SLE patients with central nervous system (CNS) disease (P = 0.0298). A strong correlation was observed between serum IL-17 and IL-6 (r = 0.62, P IL-17 was also observed with serum BAFF (r = 0.64, P IL-17 concentration correlates poorly with SLE disease activity but is significantly elevated in patients with CNS disease. IL-17/IL-6 ratio may be more useful than IL-17 or IL-6 alone to characterize Th17-driven disease, such as SLE. The association of other cytokines with serum IL-17 suggests that IL-17 may drive activation of diverse immune pathways in SLE. PMID:23968496

  14. Disease characterization of systemic lupus erythematosus (SLE) patients in Quebec.

    Science.gov (United States)

    Ng, R; Bernatsky, S; Rahme, E

    2017-08-01

    Objective Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by an array of organ manifestations that can appear during flares and disappear during remissions. The objectives of this study were: (i) to examine SLE manifestation groups longitudinally in an SLE cohort; and (ii) to assess the association between early antimalarial treatment and renal manifestations. Methods Seven SLE manifestation groups-cutaneous, hematologic, lung, musculoskeletal, neuropsychiatric, serositis, renal-were tracked using Kaplan-Meier survival curves in an incident SLE cohort from Quebec health administrative data ( n = 2010). A subgroup with provincial drug insurance coverage was followed over time to examine the association between early antimalarial treatment (within three months after SLE diagnosis) and renal manifestations using a Cox proportional hazards survival model. Results Cutaneous manifestations was the most common manifestation at SLE diagnosis (30.0%, 95% CI: 27.7-32.2%). About two-thirds (66.2%, 95% CI: 63.4-68.9%) of patients had evidence of at least one SLE manifestation at diagnosis, which increased to 87.2% (95% CI: 84.2-90.3%) by the end of follow-up. After adjusting for age, sex, early concomitant systemic steroid therapy, Charlson comorbidity index, primary care visits in the year prior and other SLE manifestations at baseline, no statistically significant association was established between antimalarial therapy and renal manifestations. Conclusion This study provides insight regarding organ manifestations within a population-based sample. Most patients identified with SLE had other diagnostic evidence that supports an underlying diagnosis of SLE. No protective effects for antimalarial agents against renal manifestations could be established in this population-based cohort.

  15. Skin lesions in lupus erythematosus: A marker of systemic involvement

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    Nilay Kanti Das

    2011-01-01

    Full Text Available Background: Lupus erythematosus (LE is an autoimmune disorder with diverse clinical manifestation ranging from mild cutaneous disorder to life-threatening systemic illness (SLE. In some patients, it remains to persist in the skin-limited form while in others it evolves into SLE. Here comes the role of identifying the markers of systemic involvement, which could determine the course and prognosis of the disease. Aim: To identify those manifestations that could be used to identify the activity of the disease SLE. Materials and Methods: An institution based, descriptive, cross-sectional study carried out over 1 year period. Sixty patients (male : female 1 : 4 with cutaneous LE were recruited for the study. The patients were classified in two groups depending on the presence or absence of ARA criteria of SLE. Detailed account of LE-specific and nonspecific lesions were noted. Statistical significance of the results was compared between the two groups using the chi-square test. Results: Among the different cutaneous manifestations, highly significant (P value <0.001 was found between SLE and nonscarring alopecia, photosensitivity, oral ulcer, malar rash (in decreasing order of odds favoring the association with SLE. Dimorphic skin lesions (P value=0.0326 also showed significant association where as discoid lesion (especially localized variant predicted toward a skin limited form of the disease with high probability of not developing SLE (P value <0.0001. No significant association was found between SLE and papulosquamous lesions, Raynaud′s phenomenon or scarring alopecia. Conclusion: Identification of lesions with high degree of association with SLE can alert the physician of the unfavorable prognosis and allow timely intervention and institution of appropriate management strategies.

  16. Acute-Onset Chest Pain in a 17-Year-Old Female Adolescent With Systemic Lupus Erythematosus.

    Science.gov (United States)

    Mangus, Courtney W; Fatusin, Oluwatosin; Ngo, Thuy L

    2017-05-01

    We report the case of a 17-year-old adolescent girl with systemic lupus erythematosus with disseminated pneumococcal infection leading to purulent pericarditis with cardiac tamponade. Although pericarditis is not an uncommon entity in autoimmune diseases such as systemic lupus erythematosus, purulent pericarditis is a rare cause (<1%) of this presentation.

  17. Epstein-Barr virus early antigen diffuse (EBV-EA/D)-directed immunoglobulin A antibodies in systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Draborg, A H; Jørgensen, J M; Müller, H

    2012-01-01

    We sought to determine whether the serological response towards lytic cycle antigens of Epstein-Barr virus (EBV) is altered in systemic lupus erythematosus (SLE) patients.......We sought to determine whether the serological response towards lytic cycle antigens of Epstein-Barr virus (EBV) is altered in systemic lupus erythematosus (SLE) patients....

  18. The classification of glomerulonephritis in systemic lupus erythematosus revisited

    NARCIS (Netherlands)

    Weening, Jan J.; D'Agati, Vivette D.; Schwartz, Melvin M.; Seshan, Surya V.; Alpers, Charles E.; Appel, Gerald B.; Balow, James E.; Bruijn, Jan A.; Cook, Terence; Ferrario, Franco; Fogo, Agnes B.; Ginzler, Ellen M.; Hebert, Lee; Hill, Gary; Hill, Prue; Jennette, J. Charles; Kong, Norella C.; Lesavre, Philippe; Lockshin, Michael; Looi, Lai-Meng; Makino, Hirofumi; Moura, Luiz A.; Nagata, Michio

    2004-01-01

    The currently used classification reflects our understanding of the pathogenesis of the various forms of lupus nephritis, but clinicopathologic studies have revealed the need for improved categorization and terminology. Based on the 1982 classification published under the auspices of the World

  19. Mechanisms of autoantibody production in systemic lupus erythematosus

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    Shuhong eHan

    2015-05-01

    Full Text Available Autoantibodies against a panoply of self-antigens are seen in SLE, but only a few (anti-Sm/RNP, anti-Ro/La, anti-dsDNA are common. The common lupus autoantigens are nucleic-acid complexes and levels of autoantibodies can be extraordinarily high. We explore why that is the case. Lupus is associated with impaired central or peripheral B cell tolerance and increased circulating autoreactive B cells. However, terminal differentiation is necessary for autoantibody production. Nucleic acid components of the major lupus autoantigens are immunostimulatory ligands for TLR7 or TLR9 that promote plasma cell differentiation. We show that the levels of autoantibodies against the U1A protein (part of a ribonucleoprotein are markedly higher than autoantibodies against other antigens, including dsDNA and the non-nucleic acid-associated autoantigens insulin and thyroglobulin. In addition to driving autoantibody production, TLR7 engagement is likely to contribute to the pathogenesis of inflammatory disease in lupus.

  20. Grasping the Existential Experience of Living with Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Larsen, Janni Lisander

    Dette projekt havde til formål at undersøge eksistentielle vilkår hos kvinder med sygdommen, systemisk lupus erythematosus, herunder hvordan disse vilkår blev erfaret gennem et langvarigt sygdomsforløb. Data blev konstrueret gennem 3 konsekutive, personlige interview sessions på dag 0, efter 6 og...

  1. TGF-β1 Gene Polymorphism at Position -800G /A and Systemic Lupus Erythematosus

    OpenAIRE

    S Naeimi

    2016-01-01

    Introduction: Systemic lupus erythematosus (SLE) is a chronic systemic inflammatory autoimmune disease characterized by a breakdown of self-tolerance. Transforming growth factor-β1 is a cytokine produced by both immune and non immune cells, and it has a wide operating range. human TGF-β1 gene is located on chromosome 19q13 . The aim of this study was investigating the TGF-β1 Gene Polymorphism at Position -800G /A and Systemic Lupus Erythematosus the possible difference in two p...

  2. Metabolic syndrome in patients with systemic lupus erythematosus

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    T Y Popkova

    2008-01-01

    Full Text Available Objective. To characterize metabolic syndrome (M S in pts wit h systemic lupus erythematosus (SLE and determine contribution of immune inflammation to the development of MS. Material and methods. 156 females with SLE (mean age 35 years, mean disease duration 99 months were included. Control group consisted of 69 people of comparable age without rheumatic diseases. MS was diagnosed according to ATP III criteria, \\fascular atherosclerotic damage was assessed by carotid sonographic evaluation. Serum cholesterol (CS, triglycerides (TG and high-density lipoprotein (HDLP CS concentration was assessed with colorimetric and photometric methods, hs CRP level — with nephelometric immunoassay. Results. MS was revealed in 29 from 154 (19% pts with SLE and in 5 from 69 (7% controls (p=0,02. MS components (hypertension, TG elevation and a lipoprotein decrease in SLE were significantly more frequent than in control group. TG, HDLP CS and CRP levels in SLE were higher than in control. Thickness of carotid intima-media complex did not differ in SLE and control. Frequency of atherosclerotic plaques (15% and coronary heart disease (14% in SLE was higher than in control (4% and 2% respectively, p=0,01. Pts with SLE and MS were older, had higher disease activity and maximal glucocorticoid dose during disease period (p<0,05. CRP concentration in SLE with MS was significantly higher. Subclinical signs of atherosclerosis in SLE with MS were more frequent than in SLE without MS (p<0,05. Frequency of clinical signs of atherosclerosis did not differ in these groups. Conclusion. Autoimmune inflammation in SLE plays an important role in the development of MS.

  3. Aberrant Epstein–Barr viral infection in systemic lupus erythematosus☆

    Science.gov (United States)

    Poole, Brian D.; Templeton, Amanda K.; Guthridge, Joel M.; Brown, Eric J.; Harley, John B.; James, Judith A.

    2010-01-01

    Serologic association, cross-reactivity of select EBV-specific antibodies with SLE autoantigens, SLE-like autoimmunity after immunization with EBV peptides, increased EB viral load in SLE patients, and SLE-specific alterations in EBV humoral and cellular immunity implicate Epstein–Barr virus (EBV) in the development of systemic lupus erythematosus (SLE). To investigate SLE-specific differences in EBV gene expression, levels of eight EBV genes were compared between SLE patients and controls by using both ex vivo-infected and un-manipulated peripheral blood mononuclear cells (PBMCs). Expression levels of mRNA were significantly greater by Wilcoxen signed rank test in the ex vivo-infected SLE patient-derived cells for 4 of 8 EBV genes, including BLLF1, 3.2-fold (p<0.004); LMP-2, 1.7-fold (p<0.008); EBNA-1, 1.7-fold (p<0.01); and BcRF1, a proposed DNA binding protein, 1.7-fold (p<0.02). The frequency of LMP-1 gene expression was significantly greater by Chi square analysis in the peripheral blood from SLE patients than controls (44% of patients, 10% of controls p<0.05). PBMCs from SLE patients had greater expression of latent genes as well as increased expression of both latent and lytic genes after infection, suggesting that EBV may participate in SLE etiology through several mechanisms. Such altered infection patterns may contribute to the increased levels of EBV and the molecular mimicry seen in sera from SLE patients. PMID:19167523

  4. Systemic lupus erythematosus and thymus persistens: A case report

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    Stević-Carević Silvija

    2017-01-01

    Full Text Available Introduction. Thymus plays an important role in the maturation of T-lymphocytes and in the development of immune tolerance. Its involution comes after puberty. If thymic tissue remains preserved in an advanced age it is considered to be the thymus persistens. According to the available data, 5% of patients with a thymoma have some of the autoimmune disorders. Medical data on the systemic lupus erythematosus (SLE association with the thymus persistens are scarce. Case report. A 29-year-old patient was diagnosed with SLE at the age of 12. She was treated with continuous doses of corticosteroids and an antimalarial drug (chloroquine. After ten years, the first, and then two more recurrences of the disease with the last recurrence in 2011 occurred. The performed laboratory analyses indicated the disease activity. The radiography of thorax showed a change on the right lung, with enlarged mediastinal shadow. Therefore the multislice computed tomography (MSCT of thorax was made. The pathohistology findings confirmed that the change on the right lung was focus of chronic pneumonitis, while the change in mediastinum was thymus persistens. The thymectomy was performed. Due to pneumonitis, the treatment of SLE was continued with corticosteroids, antimalarial drug and pulse doses of cyclophosphamide. The patient received six monthly and six quarterly pulsed doses of the drug. The remission of the disease maintained all the time. Conclusion. The disorder of thymic function should be considered as a possible cause in the development of SLE. Though the effect of thymectomy is difficult to assess, patients should be carefully monitored.

  5. Antiphospholipid antibodies in Chilean patients with systemic lupus erythematosus.

    Science.gov (United States)

    Palomo, Ivan; Pereira, Jaime; Alarcon, Marcelo; Larrain, Ana Maria; Pinochet, Carmen; Vasquez, Marcela; Velez, Maria T; Leon, M; Espinola, Ricardo; Pierangeli, Silvia

    2002-11-01

    Antiphospholipid antibodies (aPLs) are a heterogeneous family of antibodies found in autoimmune disorders, infectious diseases, and other situations. The presence of different aPLs has been associated with various clinical manifestations of the antiphospholipid syndrome (APS). The objective of this study was to investigate the prevalence of aPLs in a group of 90 Chilean patients with systemic lupus erytematosus (SLE) and 90 healthy controls. We measured anticardiolipin antibodies (aCLs), antiphosphatidylserine antibodies (aPSs), anti-beta(2) glycoprotein I antibodies (anti-beta(2)GPIs), and antiprothrombin antibodies (aPTs) with an enzyme-linked immunosorbent technique using "in-house" assays. Fifty-four of 90 SLE patients (60.0%) had some type of aPL. Forty of 90 (44.4%) were positive for aCLs, 9 of 61 (14.8%) had aPSs, 21 of 90 (23.3%) had anti-beta(2)GPIs, and 18 of 90 (20.0%) had aPTs. In the control group, prevalences were as follows: aCLs, 3.3%; aPSs, 1.1%; anti-beta(2)GPIs, 1.1%; aPTs, 2.2%. In most cases, values were in the low-positive range. Of all aPL detected, 29.5% was of the IgG isotype, 37.5% IgM, and 33.0% IgA. We observed a correlation between aCLs and aPSs and of these antibodies with anti-beta(2)GPIs and aPTs but not between anti-beta(2)GPIs and aPTs. Our results show a high prevalence of aPLs in SLE patients. An association between different specificities and isotypes of aPLs was also observed.

  6. Resilience and treatment adhesion in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Faria, Daniella Antunes Pousa; Revoredo, Luciana Silva; Vilar, Maria José; Eulália Maria Chaves, Maia

    2014-01-01

    Systemic Lupus Erythematosus (SLE) is a chronic autoimmune, rheumatic inflammatory disease that can cause significant morbidity with evident psychological impacts and obvious harm to quality-of-life that require the patient to adapt treatment. Assessment of resilience and the self-reported treatment adhesion behaviors of patients with SLE, investigating which of these factors are associated to resilience. Cross-sectional study of 40 women with SLE. A questionnaire with social demographic data, health history and the Wagnild Young Resilience Scale were used. 62.5% followed the medical treatment properly but 55% found it difficult. 27.5% of the patients presented low resilience, 57.5% medium and 15% high resilience. Resilience was associated in the chi-square test (p-value difficulty in following the treatment and stopping some activity because of the disease. In the correlation analysis, resilience was associated with age (-0.3960), number of working hours (0.5533), specialized treatment duration (-0.8103) and disease duration from diagnosis (-0.8014). Patients with high resilience tended to follow treatment correctly, tried to understand the disease and adhered more to the treatment to avoid risks and promote protection factors. Therefore knowledge of resilience in patients with SLE is necessary. It is important that the state takes necessary actions to facilitate access to treatment, to educational programs and to medical support. Awareness and counselling sessions must be initiated to develop and promote individual capacities to learn how to tackle with the disease for which psychological support of family and doctors can play a significant role.

  7. Adherence to treatment in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Prados-Moreno, Sebastián; Sabio, José Mario; Pérez-Mármol, José Manuel; Navarrete-Navarrete, Nuria; Peralta-Ramírez, María Isabel

    2018-01-12

    Non-adherence to treatment is usually a clinical problem in patients with systemic lupus erythematosus (SLE). Increasing the knowledge of predictors of treatment adherence can be meaningful in the clinical setting. The main objective of the present study was to analyse the influence of sociodemographic, clinical and psychological variables on the degree of treatment adherence in a sample of Spanish women with SLE. This is an observational-transversal study. All participants were evaluated for the degree of treatment adherence, their clinical status, psychopathological manifestations, the level of perceived stress and self-efficacy. The sample was divided into two groups (adherent vs non-adherent). The factors associated with a lack of adherence in this sample were analysed by means of logistic regression. This study comprises 72 women with SLE (average age=36.72±12.2 years). Almost 64% of patients with SLE were non-adherent to treatment. The results showed that a low educational level, being unemployed, living with a partner and alcohol abuse were associated with low treatment adherence. There were significant mean differences between groups in psychopathological subscales of somatisation, obsession-compulsion and general psychopathological indices. There were also mean differences between groups for the level of perceived stress. The use of non-steroidal anti-inflammatory drugs, suffering arthrosis and scoring higher in dimensions of psychopathology were significant predictors of treatment adherence, explaining between 35% and 47% of its variability. Including the clinical and psychopathological manifestations as important aspects in the clinical reasoning of health professionals could improve the adherence to treatment of patients with SLE. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  8. Clinical presentations and outcomes of Filipino juvenile systemic lupus erythematosus

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    Dans Leonila F

    2011-02-01

    Full Text Available Abstract Objective Juvenile Systemic Lupus Erythematosus (SLE varies by location and ethnicity. This study describes the clinical, laboratory profile and outcome of juvenile SLE seen at Philippine General Hospital (PGH from 2004-2008. Method Medical charts of all Filipino Juvenile SLE cases admitted at PGH during the 5-year period were reviewed collecting demographic profile, clinical and laboratory manifestations and treatment during disease course. Results Seventy-eight cases of juvenile SLE were reviewed. There were 7 boys and 71 girls. The mean age at diagnosis was 14 years (SD 2.7 with a range of 8-18 years. Fever (52.5% and malar rash (41.0% were the most common features at disease onset. At the time of diagnosis, the most common features were malar rash (65.3%, renal involvement (62.8% and photosensitivity (55.1%. Mucocutaneous (92.3%, renal (71.7% and hematologic (69.2% involvement were the most common features during the entire course of illness. Infection (34.5% and neurologic (19.0% complications were observed most frequently. Corticocosteroid treatment was given in most of the patients in the form of prednisone (97.4% and concomitant methylprednisolone intravenous pulses (29.4%. Nine patients died during the study period. The overall 5-year mortality rate was 11.5%. Infection (77.0% was the most frequent cause of death. Conclusion Malar rash was a common feature at disease onset and at diagnosis among Filipinos with juvenile SLE. Throughout the disease course, renal involvement occurs in 71.7% of patients. Infection was the leading cause of complication and death. The clinical presentations of Filipinos with juvenile SLE were similar to juvenile SLE in other countries.

  9. Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome.

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    Costallat, Guilherme Lavras; Appenzeller, Simone; Costallat, Lilian Tereza Lavras

    2012-07-01

    To review the clinical, laboratory and outcome features of Evans syndrome (ES) in systemic lupus erythematosus (SLE) patients. We reviewed the charts of 953 SLE patients followed up regularly at our service. ES was defined as the presence of hemolytic anemia and thrombocytopenia concomitantly or sequentially. Clinical and laboratory manifestations occurring during the disease course, as well as concomitant diseases and survival was carefully reviewed. We identified ES in 26 of 953 (2.7%) SLE patients. Twenty-three were women with mean age at SLE diagnosis of 25.7 years. Four (15%) patients had disease onset before the age of 16. In the majority of patients (92%), immune thrombocytopenia and AIHA appeared simultaneously at the beginning of SLE. Active features of SLE were a frequent finding concomitant to ES, especially arthritis (77%), malar rash (61.5%), photosensitivity (57.6%), oral ulcers (34.6%), nephritis (73%), serositis (54%), neuropsychiatric (19%) and pulmonary (15%) manifestations. In addition to this multisystemic disease, 34.6% of our patients had an association with another autoimmune disease such as antiphospholipid syndrome. Recurrence of ES was observed in only four (15%) patients. After follow-up time of 8.72 years, 19 patients (73%) were in remission and seven (27%) patients died. ES is a rare manifestation in SLE, occurring in patients with severe multisystemic SLE manifestations. Treatment strategies frequently used in SLE contribute to longer disease remission and less frequent exacerbation than observed in the general population with ES. Copyright © 2011 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  10. Prospective study of neuropsychiatric events in systemic lupus erythematosus.

    Science.gov (United States)

    Hanly, John G; Su, Li; Farewell, Vern; McCurdy, Grace; Fougere, Lisa; Thompson, Kara

    2009-07-01

    To prospectively examine neuropsychiatric (NP) events and their association with health related quality of life (HRQOL) over time in patients with systemic lupus erythematosus (SLE). In an observational cohort study from a single academic center, NP events and their attribution were identified at enrollment and at annual assessments for up to 7 years. NP events were characterized using the American College of Rheumatology case definitions; other variables were global SLE disease activity and cumulative organ damage. The outcomes of NP events were recorded and self-report HRQOL was measured with the mental (MCS) and physical (PCS) component summary scores of the Medical Outcomes Study Short Form-36. There were 209 patients, 88% female and 92% Caucasian, with a mean (standard deviation) age of 43.7 (13.8) years. Followup was available in 175/209 (84%) patients. There were 299 NP events in 132/209 (63%) patients over a mean followup of 3.6 (2.5) years. Thirty-one percent of NP events in 54 patients were attributed to SLE. Multivariate analysis indicated lower MCS scores in patients with NP events compared to those without events (p attribution. The group means for PCS scores were significantly lower in patients with NP events (p attribution. There was no association between HRQOL and cumulative organ damage, nor between NP events and the progression of organ damage. The association of lower HRQOL with NP events over time, which is independent of progression in cumulative organ damage, emphasizes the persistent negative effect of NP events in the lives of patients with SLE.

  11. DUAL THERAPY WITH BIOLOGICALS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

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    A. A. Mesnyankina

    2016-01-01

    Full Text Available Objective: to determine the efficiency of dual anti-B cell therapy in patients with active systemic lupus erythematosus (SLE.Subjects and methods. The clinic of the V.A. Nasonova Research Institute of Rheumatology followed up three patients with significant SLE, who took rituximab (RTM at a dose of 500–1000 mg, three months after completion of RTM infusions they received belimumab (BLM calculated with reference to 10 mg/kg once monthly, a total of 8 infusions. The follow-up lasted 1 year. The efficiency and tolerability of the therapy and the concentrations of autoantibodies, complement, and B-lymphocyte subpopulations were estimated at baseline and then every 3 months.Results and discussion. During dual anti-B cell therapy, there was considerable clinical improvement, no signs of recurrent SLE throughout the follow-up period, as well as normalization of laboratory markers for disease activity (the concentrations of antibodies against native DNA and complement components C3 and CD4 and persistent depletion of autoreactive B lymphocytes, plasma cells in particular. In all patients, the dose of glucocorticoids (GC could be decreased to 7.5 mg/day calculated with reference to prednisolone at 2 months after the therapy was completed.Conclusion. Dual anti-B cell therapy is a novel promising treatment for active SLE. This treatment regimen contributes to rapid suppression of disease activity, to long-term persistence of the obtained effect, and to reduced risk of severe irreversible organ damages, by minimizing the dose of GCs.

  12. Systemic lupus erythematosus induced by anti-tumour necrosis factor alpha therapy: a French national survey.

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    De Bandt, Michel; Sibilia, Jean; Le Loët, Xavier; Prouzeau, Sebastian; Fautrel, Bruno; Marcelli, Christian; Boucquillard, Eric; Siame, Jean Louis; Mariette, Xavier

    2005-01-01

    The development of drug-induced lupus remains a matter of concern in patients treated with anti-tumour necrosis factor (TNF) alpha. The incidence of such adverse effects is unknown. We undertook a retrospective national study to analyse such patients. Between June and October 2003, 866 rheumatology and internal medicine practitioners from all French hospital centres prescribing anti-TNF in rheumatic diseases registered on the website of the 'Club Rhumatismes et Inflammation' were contacted by email to obtain the files of patients with TNF-induced systemic lupus erythematosus. Twenty-two cases were collected, revealing two aspects of these manifestations. Ten patients (six patients receiving infliximab, four patients receiving etanercept) only had anti-DNA antibodies and skin manifestations one could classify as 'limited skin lupus' or 'toxidermia' in a context of autoimmunity, whereas 12 patients (nine patients receiving infliximab, three patients receiving etanercept) had more complete drug-induced lupus with systemic manifestations and at least four American Congress of Rheumatology criteria. One patient had central nervous system manifestations. No patients had lupus nephritis. The signs of lupus occurred within a mean of 9 months (range 3-16 months) in patients treated with infliximab and within a mean of 4 months (range 2-5 months) in patients treated with etanercept. In all cases after diagnosis was determined, anti-TNF was stopped and specific treatment introduced in eight patients: two patients received intravenous methylprednisolone, four patients received oral steroids (15-35 mg/day), and two patients received topical steroids. Lupus manifestations abated within a few weeks (median 8 weeks, standard deviation 3-16) in all patients except one with longer-lasting evolution (6 months). At that time, cautious estimations (unpublished data from Schering Plough Inc. and Wyeth Inc.) indicated that about 7700 patients had been exposed to infliximab and 3000 to

  13. Fatigue in systemic lupus erythematosus: a randomized controlled trial of exercise.

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    Tench, C M; McCarthy, J; McCurdie, I; White, P D; D'Cruz, D P

    2003-09-01

    To test the efficacy of a graded aerobic exercise programme in treating fatigue in systemic lupus erythematosus. Ninety-three patients with systemic lupus erythematosus without active disease in any major organ were randomized, using a minimization protocol, to 12 weeks of graded exercise therapy, relaxation therapy or no intervention. Analysis by intention to treat showed that 16 of the 33 (49%) patients in the exercise group rated themselves as 'much' or 'very much' better compared with eight out of 29 (28%) in the relaxation group and five out of 32 (16%) in the control group (chi2=8.3, df=2, P=0.02). Fatigue improved significantly on one out of three measures after exercise therapy and there was a trend for fatigue to improve on all measures after exercise. These findings support the use of appropriately prescribed graded aerobic exercise in the management of patients with fatigue and systemic lupus erythematosus.

  14. CD154: An Immunoinflammatory Mediator in Systemic Lupus Erythematosus and Rheumatoid Arthritis

    Science.gov (United States)

    Alaaeddine, Nada; Hassan, Ghada S.; Yacoub, Daniel; Mourad, Walid

    2012-01-01

    Systemic lupus erythematosus and rheumatoid arthritis are two major chronic inflammatory autoimmune diseases with significant prevalence rates among the population. Although the etiology of these diseases remains unresolved, several evidences support the key role of CD154/CD40 interactions in initiating and/or propagating these diseases. The discovery of new receptors (αIIbβ3, α5β1, and αMβ2) for CD154 has expanded our understanding about the precise role of this critical immune mediator in the physiopathology of chronic inflammatory autoimmune diseases in general, and in systemic lupus erythematosus and rheumatoid arthritis in particular. This paper presents an overview of the interaction of CD154 with its various receptors and outlines its role in the pathogenesis of systemic lupus erythematosus and rheumatoid arthritis. Moreover, the potential usefulness of various CD154-interfering agents in the treatment and prevention of these diseases is also discussed. PMID:22110533

  15. CD154: An Immunoinflammatory Mediator in Systemic Lupus Erythematosus and Rheumatoid Arthritis

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    Nada Alaaeddine

    2012-01-01

    Full Text Available Systemic lupus erythematosus and rheumatoid arthritis are two major chronic inflammatory autoimmune diseases with significant prevalence rates among the population. Although the etiology of these diseases remains unresolved, several evidences support the key role of CD154/CD40 interactions in initiating and/or propagating these diseases. The discovery of new receptors (αIIbβ3, α5β1, and αMβ2 for CD154 has expanded our understanding about the precise role of this critical immune mediator in the physiopathology of chronic inflammatory autoimmune diseases in general, and in systemic lupus erythematosus and rheumatoid arthritis in particular. This paper presents an overview of the interaction of CD154 with its various receptors and outlines its role in the pathogenesis of systemic lupus erythematosus and rheumatoid arthritis. Moreover, the potential usefulness of various CD154-interfering agents in the treatment and prevention of these diseases is also discussed.

  16. Anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus.

    Science.gov (United States)

    Su, Fang; Xiao, Weiguo; Yang, Pingting; Chen, Qingyan; Sun, Xiaojie; Li, Tienan

    2017-01-01

    The clinical significance of anti-neutrophil cytoplasmic antibodies in patients with new-onset systemic lupus erythematosus, especially in systemic disease accompanied by interstitial lung disease remains to be elucidated. This study was designed to investigate the role of anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus patients. A hundred and seven patients with new-onset SLE were enrolled. Presence of anti-neutrophil cytoplasmic antibodies in the sera was assessed by indirect immunofluorescence as well as enzyme linked immunosorbent assay against proteinase-3 and myeloperoxidase. Clinical features and laboratory parameters of patients were also recorded. All patients were subjected to chest X-ray, chest high-resolution computed tomography and pulmonary function test. Forty-five systemic lupus erythematosus patients (45/107, 42%) were seropositive for anti-neutrophil cytoplasmic antibodies. Compared with anti-neutrophil cytoplasmic antibodies-negative patients, the anti-neutrophil cytoplasmic antibodies-positive patients had significantly higher incidence of renal involvement, anemia, and Raynaud's phenomenon as well as decreased serum level of complement 3/complement 4 and elevated erythrocyte sedimentation rate. In addition, there was a positive correlation between serum anti-neutrophil cytoplasmic antibodies level and disease activity of systemic lupus erythematosus. Furthermore, prevalence of interstitial lung disease in the anti-neutrophil cytoplasmic antibodies -positive patients (25/45, 55.6%) was obviously higher than that in the anti-neutrophil cytoplasmic antibodies-negative patients (15/62, 24.2%). The sample size was limited and the criteria for screening new-onset systemic lupus erythematosus patients might produce bias. The level of anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus patients correlates positively with the disease activity and the prevalence of interstitial lung disease.

  17. Comparison of estimates of body fat content in childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Sinicato, N A; Peres, F A; de Oliveira Peliçari, K; de Oliveira Santos, A; Ramos, C D; Marini, R; Appenzeller, S

    2017-04-01

    Objective We aimed to compare estimates of body fat content with respect to their ability to predict the percentage of body fat, confirmed by dual-energy X-ray absorptiometry scans in childhood-onset systemic lupus erythematosus. Methods We included 64 consecutive childhood-onset systemic lupus erythematosus patients and 64 healthy age and sex-matched controls in a cross-sectional study. Anthropometric data, body mass index and body adiposity index were calculated for all subjects. Childhood-onset systemic lupus erythematosus patients were further assessed for clinical and laboratory childhood-onset systemic lupus erythematosus manifestations and fat mass, lean mass and percentage of body fat evaluated by dual-energy X-ray absorptiometry. Results Elevated waist/hip ratio was observed in childhood-onset systemic lupus erythematosus patients when compared to controls ( p lupus erythematosus patients and controls. Using dual-energy X-ray absorptiometry as gold standard we observed that all indirect estimates of body fat were correlated with whole body fat mass. We observed a correlation between height and cumulative corticosteroid dose adjusted by weight ( r = 0.429, p = 0.005) in childhood-onset systemic lupus erythematosus. On whole body analysis we observed a correlation between lean mass and ACR Damage Index scores ( r = -0.395; p = 0.019); percentage of body fat and adjusted Systemic Lupus Erythematosus Disease Activity Index ( r = 0.402; p = 0.008), disease duration ( r = -0.370; p = 0.012). On trunk analysis we observed a correlation between lean mass and ACR Damage Index ( r = -0.319; p = 0.042); percentage of body fat with adjusted Systemic Lupus Erythematosus Disease Activity Index ( r = 0.402; p = 0.005), disease duration ( r = -0.408; p = 0.005). Conclusions This is the first study analyzing body adiposity index in childhood-onset systemic lupus erythematosus patients. We observed that all indirect

  18. Histological antiphospholipid-associated nephropathy versus lupus nephritis in patients with systemic lupus erythematosus: an observational cross-sectional study with longitudinal follow-up.

    Science.gov (United States)

    Gerhardsson, Jakob; Sundelin, Birgitta; Zickert, Agneta; Padyukov, Leonid; Svenungsson, Elisabet; Gunnarsson, Iva

    2015-04-27

    Renal involvement is a severe complication in systemic lupus erythematosus (SLE). Moreover, a subset of SLE patients develop the anti-phospholipid syndrome (APS), characterised by the occurrence of anti-phospholipid antibodies in combination with macro- and microvascular thrombotic manifestations, including acute and chronic antiphospholipid-associated nephropathy (APLN). Clinical presentations of lupus nephritis and APLN are similar and a renal biopsy is necessary to differentiate between the conditions. Our aim with this study was to investigate the occurrence of histopathological findings consistent with APLN (hAPLN) in renal biopsies from SLE patients and to investigate associations with anti-phospholipid antibody specificities, clinical manifestations, HLA-DRB1 alleles, and long-term renal outcome. Consecutive renal biopsies from 112 SLE patients with renal involvement were investigated and evaluated for findings of hAPLN; in all there were 236 renal biopsies. Data from biopsy reports and clinical information were collected. Autoantibodies against cardiolipin and β2-glycoprotein-1 were measured by enzyme-linked immunosorbent assay. A lupus anticoagulant test was determined with a modified Dilute Russel Viper Venom method. HLA genotyping was performed by sequence-specific primer PCR. Renal outcome was determined at study end. The prevalence of hAPLN was 14.3% among SLE patients with renal involvement. Compared to patients with pure lupus nephritis, occurrence of hAPLN was associated with intima changes (odds ratio (OR) = 24; 95% confidence interval (CI), 3.0 to 189.8; P lupus nephritis patients (median 116 versus 75 μmol/L; P lupus nephritis. hAPLN is a severe and often unrecognized condition in SLE patients with renal involvement. We have demonstrated an increased risk for development of renal impairment and a genetic predisposition in hAPLN patients compared to lupus nephritis patients.

  19. Validity of LupusQoL-China for the assessment of health related quality of life in Chinese patients with systemic lupus erythematosus.

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    Su-li Wang

    Full Text Available OBJECTIVES: To adapt and assess the validity and reliability of LupusQoL for use in Chinese patients with systemic lupus erythematosus (SLE. METHODS: Debriefing interviews of subjects with SLE guided the language modifications of the tool. The process of adaptation proceeded according to the guideline and pre-testing results of LupusQoL-China. 220 SLE patients completed LupusQoL-China and a generic preference-based measurement of health EuroQoL scale (EQ-5D, and 20 patients repeated them after 2 weeks. Internal consistency (ICR and test-retest (TRT reliability, convergent and discriminant validity were examined. Factor analysis and Rasch analysis were performed. RESULTS: The mean (SD age of the 208 subjects with SLE was 33.93 (± 9.19 years. ICR and TRT of the eight domains ranged from 0.811 to 0.965 and 0.836 to 0.974, respectively. The LupusQoL-China domains demonstrated substantial evidence of construct validity when compared with equivalent domains on the EQ-5D (physical health and usual activities r = -0.63, pain and pain/discomfort r = -0.778, emotional health and anxiety/depression r = -0.761, planning and usual activities r = -0.560. Most LupusQoL-China domains could discriminate patients with varied disease activities and end-organ damage (according to SELENA-SLEDAI and SLICC-DI. The principal component analysis revealed six factors, and confirmatory factor analysis result of which is similar to eight factors model. CONCLUSIONS: These results provide evidence that the LupusQoL-China is valid as a disease-specific HRQoL assessment tool for Chinese patients with SLE.

  20. Do subjective cognitive complaints correlate with cognitive impairment in systemic lupus erythematosus? A Danish outpatient study

    DEFF Research Database (Denmark)

    Vogel, A; Bhattacharya, S; Larsen, J L

    2011-01-01

    This study examined the prevalence of cognitive impairment and its association with depressive symptoms and self-reported cognitive complaints in Danish outpatients with systemic lupus erythematosus (SLE). Fifty-seven consecutive female SLE-outpatients were examined with a comprehensive neuropsyc......This study examined the prevalence of cognitive impairment and its association with depressive symptoms and self-reported cognitive complaints in Danish outpatients with systemic lupus erythematosus (SLE). Fifty-seven consecutive female SLE-outpatients were examined with a comprehensive...

  1. Crosscultural validation and reliability of 3 disease activity indices in systemic lupus erythematosus.

    Science.gov (United States)

    Gladman, D D; Goldsmith, C H; Urowitz, M B; Bacon, P; Bombardier, C; Isenberg, D; Kalunian, K; Liang, M H; Maddison, P; Nived, O

    1992-04-01

    Rheumatologists from 4 countries, representing 8 rheumatology centers, tested 3 systemic lupus erythematosus (SLE) disease activity indices: the SLE Disease Activity Index (SLEDAI) from Toronto; the Systemic Lupus Activity Measure (SLAM) from Boston and the British Isles Lupus Assessment Group (BILAG) for their reproducibility and validity in the assessment of real patients. Seven patients representing a spectrum of disease manifestations and activity were each examined by 4 of 7 observers from all centers except Toronto, using a Youden square design. Each observer completed all 3 indices and a category rating scale for disease activity on each of the 4 patients seen. All 3 indices detected differences among patients. There was no detectable observer effect among the 7 observers with each of the 3 indices. There was a detectable order effect with the SLAM. The 3 indices are comparable and reproducible for evaluating disease activity in SLE.

  2. Systemic lupus erythematosus pregnancies: ten-year data from a single centre in Malaysia.

    Science.gov (United States)

    Teh, C L; Wan, S A; Cheong, Y K; Ling, G R

    2016-08-13

    We performed a retrospective study of all systemic lupus erythematosus (SLE) pregnancies during a 10-year period (2006-2015) to describe the clinical features, maternal and foetal outcomes in our centre. There were 115 pregnancies in 86 women with SLE. Our patients had a mean age of 29.1 years (SD 5.80) and a mean disease duration of 44.63 months (SD 41.17). Fifteen patients had antiphospholipid syndrome (APS). Our patients had complicated pregnancies: 26.1% had SLE flares, 13.9% had pre-eclampsia and 45.1% needed caesarean sections. There were 23.3% foetal losses and 25% preterm deliveries in our patients. There was a higher rate of unplanned pregnancies and lupus flare among pregnancies with active SLE at conception. Pregnancies in lupus nephritis have higher incidence of lupus flares during pregnancy but similar maternal and foetal outcomes compared to those without nephritis. The prognostic indicators for adverse foetal outcome in our patients were flare of SLE (HR 4.08 [CI 95% 1.65-10.13, p < 0.01]) and APS (HR 3.07 [CI 95% 1.12-8.42, p < 0.05]) and the prognostic indicator for adverse maternal outcome was hypertension (HR 3.58 [CI 95% 1.30-9.90, p < 0.05]). Lupus pregnancies in our centre remained as high-risk pregnancies with significant maternal and foetal complications. © The Author(s) 2016.

  3. The pathogenesis and diagnosis of systemic lupus erythematosus: still not resolved

    NARCIS (Netherlands)

    Rekvig, O.P.; Vlag, J. van der

    2014-01-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with various clinical manifestations affecting different tissues. A characteristic feature of SLE is the presence of autoantibodies against double-stranded (ds)DNA, histones and nucleosomes, and other chromatin components. SLE is a

  4. Apoptosis-induced acetylation of histones is pathogenic in systemic lupus erythematosus

    NARCIS (Netherlands)

    Dieker, J.W.C.; Fransen, J.H.; Bavel, C.C.A.W. van; Briand, J.P.; Jacobs, C.W.M.; Muller, S.; Berden, J.H.M.; Vlag, J. van der

    2007-01-01

    OBJECTIVE: In systemic lupus erythematosus (SLE), inadequate removal of apoptotic cells may lead to challenge of the immune system with immunogenic self antigens that have been modified during apoptosis. We undertook this study to evaluate whether apoptosis-induced histone modifications are targets

  5. Idiopathic Thrombocytopenic Purpura as Initial Manifestation of Systemic Lupus Erythematosus: A Case Report

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    Fatih Bağcıer

    2016-08-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic autoimmune disease that can affect almost any organ system. Its presentation and course are highly variable. The classic presentation of SLE in a woman of childbearing age is fever, arthritis, and malar rash triad. However, patients may present rare types of manifestations such as idiopathic thrombocytopenic purpura.

  6. Antibodies in systemic lupus antineutrophil cytoplasmic erythematosus: prevalence, disease activity correlations and organ system associations.

    Science.gov (United States)

    Fauzi, A R; Kong, N C T; Chua, M K; Jeyabalan, V; Idris, M N; Azizah, R

    2004-08-01

    Systemic Lupus Erythematosus (SLE) is a disease with multiorgan involvement and multiple autoantibody production including antineutrophil cytoplasmic antibodies (ANCA). Despite its reported prevalence in more than one third of SLE patients, the role of ANCA in the pathogenesis or otherwise in SLE remains unresolved. 131 SLE patients had been previously studied for various serologic parameters of disease activity. Their cumulative organ involvement in the course of their disease had also been determined and the Lupus Activity Index (LAI) calculated. Their stored sera were then screened for the presence of ANCA by two methods viz Indirect immunofluorescence (IIF) and also enzyme-linked immunosorbent assay (ELISA). ANCA was present in 24.8% of these SLE patients. The atypical ANCA pattern was predominant and accounted for an overall of 20.6%. Anti-MPO and anti-PR3 were detected in 1.5% of patients respectively. No association was found between ANCA positivity and disease activity. There was also no association of ANCA with specific organ involvement. Despite the high prevalence of ANCA especially the atypical variant in SLE, they probably represent only one of the wide repertoire of autoantibodies found in this disease. Routine testing for ANCA in lupus patients is therefore not recommended.

  7. Pancreatitis and systemic lupus erythematosus Pancreatitis y lupus eritematoso sistémico

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    J. Lariño Noia

    2009-08-01

    Full Text Available Gastrointestinal symptoms in patients with SLE are common, specifically abdominal pain. However, the rate of pancreatic diseases is much lower and does not reach 5% according to published series in Europe and the USA. This association between SLE and pancreatic disease is basically at the expense of episodes of acute pancreatitis. An association with chronic pancreatitis is much more uncommon, and only four articles have been published showing this relationship. Three cases of SLE-associated pancreatitis are described, and disease onset, etiological factors, and clinical progression are analyzed. A review of the literature and a brief discussion about pathophysiological mechanisms and the role of corticosteroids are also included.Los síntomas gastrointestinales en los pacientes con lupus eritematoso sistémico (LES son comunes, específicamente el dolor abdominal. Sin embargo la tasa de enfermedades pancreáticas es mucho menor, una tasa que no alcanza ni al 5% según las series publicadas en Europa y EE. UU. Esta asociación entre enfermedades pancreáticas y LES es fundamentalmente a expensas de episodios de pancreatitis aguda. La asociación con pancreatitis crónica es muchísimo más infrecuente, teniendo en cuenta que tan sólo cuatro artículos han sido publicados reflejando esta asociación. Describimos tres casos de asociación entre pancreatitis y LES, analizando el debut de la enfermedad, los factores etiológicos y también la evolución clínica. Hemos realizado, además, una breve discusión de la fisiopatología y del papel de corticosteroides, así como una revisión de la literatura.

  8. Construct and criterion validity of the Euro Qol-5D in patients with systemic lupus erythematosus.

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    Su-li Wang

    Full Text Available OBJECTIVE: To investigate the construct and criterion validity of the Euro Qol-5D (EQ-5D, which allows quality-adjusted life-years to be calculated, in patients with systemic lupus erythematosus (SLE. METHODS: Consecutive SLE patients who had been followed at the Renji Hospital, School of Medicine, Shanghai Jiao Tong University were recruited. Cross-sectional correlations of the EQ-5D with equivalent domains in disease-specific health-related quality of life (HRQoL, LupusQol, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI measures, the Systemic Lupus International Collaborating Clinics Damage Index (SDI, and patient characteristics were tested. Discriminant validity to assess the ability to distinguish between patients of different disease severity was assessed. There also were evaluations of ceiling and floor effects. RESULTS: 240 patients were recruited in total. The EQ-5D correlated moderately to strongly with all domains of the LupusQoL (r: 0.44-0.7 apart from intimate relationships (r = 0.25 and body image (r = 0.18. There was moderate negative correlation between EQ-5D and clinical assessment of disease, SLEDAI (r = -0.589 and SDI (r = -0.509. When compared with equivalent domains on LupusQoL, there was good construct validity in EQ-5D (r: 0.631-0.812. EQ-5D could also discriminate patients with varied disease severity (according SLEDAI and SDI. There was no floor effect in EQ-5D but the ceiling effect remains strong (34%. CONCLUSION: Our results provide sufficient evidence that the EQ-5D displays construct and criterion validity for use in SLE patients. Disease-specific measures of HRQoL used alongside may be a better choice.

  9. Construct and criterion validity of the Euro Qol-5D in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Wang, Su-li; Wu, Bin; Zhu, Li-an; Leng, Lin; Bucala, Richard; Lu, Liang-jing

    2014-01-01

    To investigate the construct and criterion validity of the Euro Qol-5D (EQ-5D), which allows quality-adjusted life-years to be calculated, in patients with systemic lupus erythematosus (SLE). Consecutive SLE patients who had been followed at the Renji Hospital, School of Medicine, Shanghai Jiao Tong University were recruited. Cross-sectional correlations of the EQ-5D with equivalent domains in disease-specific health-related quality of life (HRQoL), LupusQol, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) measures, the Systemic Lupus International Collaborating Clinics Damage Index (SDI), and patient characteristics were tested. Discriminant validity to assess the ability to distinguish between patients of different disease severity was assessed. There also were evaluations of ceiling and floor effects. 240 patients were recruited in total. The EQ-5D correlated moderately to strongly with all domains of the LupusQoL (r: 0.44-0.7) apart from intimate relationships (r = 0.25) and body image (r = 0.18). There was moderate negative correlation between EQ-5D and clinical assessment of disease, SLEDAI (r = -0.589) and SDI (r = -0.509). When compared with equivalent domains on LupusQoL, there was good construct validity in EQ-5D (r: 0.631-0.812). EQ-5D could also discriminate patients with varied disease severity (according SLEDAI and SDI). There was no floor effect in EQ-5D but the ceiling effect remains strong (34%). Our results provide sufficient evidence that the EQ-5D displays construct and criterion validity for use in SLE patients. Disease-specific measures of HRQoL used alongside may be a better choice.

  10. Genome-wide association studies in systemic lupus erythematosus: a perspective

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    Cunninghame Graham, Deborah S

    2009-01-01

    Genome-wide association studies (GWAS) have been shown to be a powerful way of identifying novel susceptibility genes in systemic lupus erythematosus (SLE), as demonstrated by a series of publications in the past year. Lupus has been a late-comer to the GWAS community, being preceded by success stories for the GWAS approach in other autoimmune diseases, including type I diabetes, ankylosing spondylitis, rheumatoid arthritis, Crohn's disease and ulcerative colitis. The paper by Suarez-Gestal and colleagues seeks to exploit the wealth of data available from a total of four GWAS in SLE, three in European-American populations and one in a Swedish population. The authors describe replication of ten lupus susceptibility alleles in a Spanish SLE case-control study. PMID:19664177

  11. [Superior sagittal sinus thrombosis in a case of longstanding systemic lupus erythematosus].

    Science.gov (United States)

    Ohsaka, Misuzu; Nonaka, Tadashi; Oka, Shinichi; Minamida, Yoshihiro; Mikami, Takeshi

    2006-01-01

    A 33-year-old female who had been on a steroid treatment for the past 14 years due to systemic lupus erythematosus (SLE) visited our hospital complaining of mild headache. No neurological deficit and no positive serologic tests for lupus anticoagulants (LAC) and anticardiolipin antibodies (aCL) were noted. Only a mild inflammatory change was observed on routine hematological examination. On neuroradiological examination, MRI revealed thickened falx cerebri and tentorium cerebelli, and an empty delta sign. These findings were suggestive of sinus thrombosis of superior sagittal sinus (SSS). Angiograms clearly demonstrated occlusion of the posterior part of superior sagittal sinus and transeverse sinus (TS). Conservative treatment was chosen because of no evidence of intracranial hypertension. There was no deterioration in her general and neurological status during her hospital stay and she was discharged. Longstanding vasculitis and pachymeningitis related to lupus erythematosus might be the probable cause of the sinus thrombosis in this case.

  12. Pregnancy in Systemic Lupus Erythematosus Patients with Nephritis

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    Panagiotis Pateinakis

    2014-07-01

    Full Text Available Pregnancy in patients with lupus nephritis is a challenging clinical situation. Although not absolutely contraindicated, it is associated with increased risk for foetal and maternal complications, including foetal loss, preterm delivery, intrauterine growth retardation, hypertension, pre-eclampsia, nephritis flare, and, rarely, maternal death. The complication rate is further increased in the presence of antiphospholipid antibodies or the antiphospholipid syndrome. Proliferative classes of nephritis (III and IV also appear to confer excess risk for complications. Immunosuppressives such as cyclophosphamide and mycophenolate, and antihypertensives such as angiotensin-converting-enzyme (ACE inhibitors and angiotensin receptor blockers need to be stopped due to teratogenic effects. Agents like corticosteroids, azathioprine, and probably calcineurin inhibitors are considered compatible with gestation. Lupus activity needs to be assessed and carefully monitored. Thrombotic risk due to antiphospholipid antibodies, thrombotic events, or nephrosis needs to be evaluated and managed accordingly, with the use of aspirin and/or unfractioned or low molecular weight heparin. Differentiating between severe pre-eclampsia and lupus nephritis flare might require a renal biopsy, which might not always be feasible, for example after the 32nd gestational week or in a setting of uncontrolled hypertension or thrombocytopaenia. A 6-month history of quiescent disease on non-teratogenic agents seems to be associated with best chance for favourable outcomes. Pregnancy is optimally managed by a multidisciplinary team of experienced specialists, and close monitoring for disease activity during gestation; additionally, follow-up for maternal flare postpartum is also advised.

  13. Oral manifestations of patients with lupus erythematosus.

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    Brennan, Michael T; Valerin, Manuel A; Napeñas, Joel J; Lockhart, Peter B

    2005-01-01

    Lupus erythematosus manifests as cutaneous variants, such as discoid lupus erythematosus or systemic lupus erythematosus. Systemic lupus erythematosus is a multisystem autoimmune disease characterized by general autoantibody production and a wide range of mucocutaneous, renal, neuropsychiatric, cardiovascular, infectious, and hematologic manifestations. This article discusses the prevalence of and considerations for oral mucosal lesions in lupus erythematosus and the impact of the various disease manifestations of systemic lupus erythematosus on dental management.

  14. Severely crusted cheilitis as an initial presentation of systemic lupus erythematosus

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    Wai Man Mandy Chan

    2017-01-01

    Full Text Available Lupus erythematosus (LE is an autoimmune disease which may initially present solely with lip lesions. Due to a wide spectrum of presentation, these features may initially be misdiagnosed as other oral diseases such as lichen planus, erythema multiforme (EM, and actinic cheilitis, leading to a delay in diagnosis and treatment. We discuss a case of severely crusted cheilitis which was initially diagnosed as EM, with subsequent development of subacute cutaneous LE, and progression to systemic LE. We will discuss the clinical and histological features of lupus cheilitis.

  15. Off-label use of rituximab for systemic lupus erythematosus in Europe

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    Ryden-Aulin, Monica; Boumpas, Dimitrios T; Bultink, Irene

    2016-01-01

    Objectives: Rituximab (RTX) is a biological treatment used off-label in patients with systemic lupus erythematosus (SLE). This survey aimed to investigate the off-label use of RTX in Europe and compare the characteristics of patients receiving RTX with those receiving conventional therapy. Methods...... organ manifestations for which either RTX or conventional therapy was initiated were lupus nephritis followed by musculoskeletal and haematological. The reason for treatment was, besides disease control, corticosteroid-sparing for patients treated with conventional therapy. Conclusions: RTX use for SLE...

  16. Necrotizing Polyarteritis Nodosa-like Vasculitis in a Child with Systemic Lupus Erythematosus.

    Science.gov (United States)

    Nada, Ritambhra; Matthews, Joseph L; Bhattad, Sagar; Gupta, Anju; Singh, Surjit

    2017-02-15

    A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema.

  17. Myocardial ischaemia in systemic lupus erythematosus: detection and clinical relevance.

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    Płazak, Wojciech; Gryga, Krzysztof; Sznajd, Jan; Pasowicz, Mieczysław; Musiał, Jacek; Podolec, Piotr

    2011-01-01

    Severe cardiovascular complications are among the most important causes of mortality in systemic lupus erythematosus (SLE) patients. To assess the usefulness of echocardiography, ECG, and coronary artery calcium scoring (CACS) in the detection of myocardial ischaemia in SLE patients compared to single photon emission computerised tomography (SPECT) and to assess their five-year follow-up. In 50 consecutive SLE patients (mean age 39.2 ± 12.9 years, 90% female), clinical assessment, resting and exercise ECG and echocardiography, multidetector computed tomography - based CACS and SPECT studies (Tc-99m sestamibi) were performed. Patients were then followed for five years. SPECT revealed perfusion defects in 25 (50%) patients; persistent defects in 18 (36%) and exercise-induced defects in seven (14%) subjects. No typical ischaemic heart disease clinical symptoms, signs of ischaemia in resting ECG, or left ventricular contractility impairment in echocardiography were observed. Signs of ischaemia in exercise ECG were found in 17 (34%) patients. The CACS ranged from 1 to 843.2 (median 23.15), and coronary calcifications were observed in 12 (24%) patients. Compared to the SPECT study, exercise ECG had 68% sensitivity and 100% specificity in detecting myocardial ischaemia, while CACS had only 28% sensitivity and 58% specificity. During follow-up, one patient who showed myocardial perfusion defects and the highest calcium score (843.2) at baseline, developed CCS II class symptoms of myocardial ischaemia. Coronary angiography was not performed because of severe anaemia; the patient died three months later. In two other patients with perfusion defects and calcium deposits at baseline, CCS I class symptoms were observed; coronary angiography showed only thin calcified coronary plaques that were haemodynamically insignificant. In about half of relatively young, mostly female, SLE patients, SPECT shows myocardial perfusion defects, with coronary calcifications present in one

  18. Association between systemic lupus erythematosus and Helicobacter pylori seronegativity.

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    Sawalha, Amr H; Schmid, Wendi R; Binder, Steven R; Bacino, Debra K; Harley, John B

    2004-08-01

    Helicobacter pylori is a gram negative spiral bacterium that is clearly associated with a variety of gastrointestinal pathologies. A number of non-gastrointestinal diseases have also been associated with H. pylori. We investigated the prevalence of H. pylori seropositivity as part of a larger serologic survey in a group of 466 patients with systemic lupus erythematosus (SLE) and 466 controls. We studied subjects for seropositivity against 5 antigens including mumps, measles, rubella, varicella zoster, and H. pylori. The 466 SLE patients were taken from a total of 290 pedigrees multiplex for SLE and matched to 466 controls for age (+/- 3 yrs), sex, and ethnicity to non-SLE affected individuals, taken mostly from the same collection of pedigrees multiplex for SLE. Assays for seropositivity were performed using a heterogeneous immunoassay technique. Pearson's chi-square was used to test for association of categorical variables and Student t-test for continuous variables. Logistic regression was used to compute the odds ratio for H. pylori seropositivity in patients and controls. There was a significant difference only in H. pylori seropositivity between SLE cases and their controls. The results were not altered by intrafamilial correlation. Subset analysis by race and sex showed that the differences between the African-American female patients with SLE and their matched controls were responsible for this association. Female African-American patients with SLE had a lower prevalence of H. pylori seropositivity compared to controls (38.1% vs 60.2%, OR 0.41, p = 0.0009, 95% CI 0.24-0.69). Of the 113 African-American female SLE patients in the study group, 43 were seropositive for H. pylori. The mean age of onset for SLE was older in the seropositive group (34.4 yrs) compared to the seronegative SLE patients (28.0 yrs) (t = 2.11, p = 0.039). Of 5 serologic tests performed, only the frequency of H. pylori seropositivity was different between SLE cases and their controls

  19. Population-Based Incidence and Prevalence of Systemic Lupus Erythematosus

    Science.gov (United States)

    Somers, Emily C.; Marder, Wendy; Cagnoli, Patricia; Lewis, Emily E.; DeGuire, Peter; Gordon, Caroline; Helmick, Charles G.; Wang, Lu; Wing, Jeffrey J.; Dhar, J. Patricia; Leisen, James; Shaltis, Diane; McCune, W. Joseph

    2014-01-01

    Objective To estimate the incidence and prevalence of systemic lupus erythematosus (SLE) in a sociodemographically diverse southeastern Michigan source population of 2.4 million people. Methods SLE cases fulfilling the American College of Rheumatology classification criteria (primary case definition) or meeting rheumatologist-judged SLE criteria (secondary definition) and residing in Wayne or Washtenaw Counties during 2002–2004 were included. Case finding was performed from 6 source types, including hospitals and private specialists. Age-standardized rates were computed, and capture–recapture was performed to estimate underascertainment of cases. Results The overall age-adjusted incidence and prevalence (ACR definition) per 100,000 persons were 5.5 (95% confidence interval [95% CI] 5.0–6.1) and 72.8 (95% CI 70.8–74.8). Among females, the incidence was 9.3 per 100,000 persons and the prevalence was 128.7 per 100,000 persons. Only 7 cases were estimated to have been missed by capture–recapture, adjustment for which did not materially affect the rates. SLE prevalence was 2.3-fold higher in black persons than in white persons, and 10-fold higher in females than in males. Among incident cases, the mean ± SD age at diagnosis was 39.3 ± 16.6 years. Black SLE patients had a higher proportion of renal disease and end-stage renal disease (ESRD) (40.5% and 15.3%, respectively) as compared to white SLE patients (18.8% and 4.5%, respectively). Black patients with renal disease were diagnosed as having SLE at younger age than white patients with renal disease (mean ± SD 34.4 ± 14.9 years versus 41.9 ± 21.3 years; P = 0.05). Conclusion SLE prevalence was higher than has been described in most other population-based studies and reached 1 in 537 among black female persons. There were substantial racial disparities in the burden of SLE, with black patients experiencing earlier age at diagnosis, >2-fold increases in SLE incidence and prevalence, and increased

  20. Vitamin D Deficiency in Patients with Systemic Lupus Erythematosus

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    Suzan M. Attar

    2013-01-01

    Full Text Available Objectives: Hypovitaminosis D is common in the general population. Many studies that have been conducted to show the association between vitamin D deficiency and systemic lupus erythematosus (SLE reveal that deficiencies in vitamin D are common in this group of patients. Our aim was to study the relationship between 25(OHD and disease activity in patients with SLE.Methods: Retrospective cohort study of patients with SLE who were followed up at King Abdulaziz University Hospital, Jeddah, from January 2007 to November 2010. Demographic and clinical data were recorded and the 25(OHD levels of the patients were measured. Chi square tests, Student’s t-test, ANOVA and Pearson tests were used for data analysis. ANOVA test was followed by Bonferroni correction. A p-value <0.05 was considered significant.Results: Ninety-five patients with SLE were enrolled in the study. The levels of 25(OHD were significantly lower in patients with active SLE (n=41; 43% than in those with inactive disease (n=54; 57%; p=0.04. The mean (SD levels were 22.3 (14 nmol/L for patients with active disease against 25.0 (14 nmol/L for patients with inactive SLE. No correlation was detected between 25(OH D levels and disease activity score evaluated by SLEDAI-2K. By Pearson correlation, a significant negative correlation existed between 25(OH D and anti ds-DNA (r=-0.38; p<0.001; a positive correlation existed between 25(OHD levels and C4 (r=0.25; p=0.25. By chi square testing, azathioprine treatment (OR=3.5, low C4 (OR= 2.23, low C3 (OR=1.92, and active disease (OR=1.6 were associated with 25(OHD deficiency in SLE patients.Conclusion: Vitamin D deficiency is frequent in patients with SLE. Patients with SLE have a higher risk of developing 25(OHD deficiency in the presence of low serum C3 and C4 levels, and high anti-dsDNA levels.

  1. Systemic lupus erythematosus and pregnancy: clinical evolution, maternal and perinatal outcomes and placental findings

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    Fernanda Garanhani de Castro Surita

    2007-03-01

    Full Text Available CONTEXT AND OBJECTIVE: Systemic lupus erythematosus is a chronic disease that is more frequent in women of reproductive age. The relationship between lupus and pregnancy is problematic: maternal and fetal outcomes are worse than in the general population, and the management of flare-ups is difficult during this period. The aim here was to compare the outcomes of 76 pregnancies in 67 women with lupus, according to the occurrence or absence of flare-ups. DESIGN AND SETTING: An observational cohort clinical study evaluating the evolution of pregnant women with lupus who were receiving care at the prenatal outpatient clinic, Centro de Atenção Integral à Saúde da Mulher, Universidade Estadual de Campinas (CAISM/Unicamp, between 1995 and 2002. METHODS: Data were collected on a precoded form. The women were divided into two groups according to the occurrence or absence of flare-ups, as defined by the systemic lupus erythematosus disease activity index (SLEDAI. The presence or absence of flare-ups and renal involvement was considered to be the independent variable and the other results were dependent variables. RESULTS: Flare-ups occurred in 85.3% of cases, and were most significant when there was renal involvement. This was related to greater numbers of women with preeclampsia and poor perinatal outcome. Intrauterine growth restriction was more common in the women with active disease. Placental weight was significantly lower in the women with renal involvement. CONCLUSIONS: Flare-ups and renal involvement in lupus patients during pregnancy are associated with increased maternal and perinatal complications.

  2. Lupus anticoagulant and history of thrombosis are not associated with persistent endothelial cell activation in systemic lupus erythematosus.

    Science.gov (United States)

    Frijns, C J; Derksen, R H; De Groot, P G; Algra, A; Fijnheer, R

    2001-07-01

    Antiphospholipid antibodies (aPL), especially lupus anticoagulant (LAC), characterize systemic lupus erythematosus (SLE) patients at increased risk for arterial and venous thromboembolic complications. It has been reported that purified human anti-phospholipid antibodies cause endothelial cell activation in in vitro experiments. In order to investigate whether increased endothelial cell activation is associated with thromboembolic events in SLE patients with LAC, we measured plasma levels of thrombomodulin (TM), von Willebrand factor (vWf), sP-selectin, vascular cell adhesion molecule-1 (sVCAM-1) and ED1-fibronectin in a study of 76 patients with SLE. Patients were subdivided on the basis of: no history of thrombosis and LAC-negative (n = 22) or LAC-positive (n = 17); positive history of thrombosis and LAC-negative (n = 16) or LAC-positive (n = 21). The median SLE disease activity index (SLEDAI) was 4. Although concentrations of sTM, vWf, sP-selectin and sVCAM-1 were significantly elevated in SLE compared with values in healthy controls, they did not differ between the four groups, between patients with or without history of thrombosis, and between patients with or without LAC. Presence of anticardiolipin antibodies could not explain these negative findings. Adjustment of the concentrations for significantly associated variables, such as age, hypertension, smoking, immunosuppressive treatment and concentrations of creatinine, cholesterol and homocysteine, did not change the main results of the study. Only sTM was significantly lower in patients with both LAC and thrombosis than in patients without both these features after adjustment for serum creatinine concentrations. In conclusion, we did not find an association between endothelial cell activation and presence of LAC or history of thrombosis in SLE.

  3. Relationship between MCP-1 promoter -2518 A/G gene polymorphism (rs1024611) and systemic lupus erythematosus/lupus nephritis.

    Science.gov (United States)

    Zhou, Tian-Biao; Jiang, Zong-Pei; Liang, Meng-Jun; Huang, Ya-Juan

    2015-02-01

    Results from the published studies on the association between monocyte chemoattractant protein-1 (MCP-1) promoter -2518 A/G (rs1024611) gene polymorphism and systemic lupus erythematosus (SLE)/lupus nephritis (LN) are still conflicting. This meta-analysis was performed to evaluate the relationship between MCP-1 A/G gene polymorphism and SLE/LN and to explore whether MCP-1 A allele, AA genotype or GG genotype could become a predictive marker for SLE/LN risk. Association studies were identified from the databases of PubMed, Embase, Cochrane Library and CBM-disc (China Biological Medicine Database) as of 1 January 2014, and eligible investigations were synthesized using meta-analysis method. Results were expressed with odds ratios (OR) for dichotomous data, and 95% confidence intervals (CI) were also calculated. Sixteen investigations were identified for the analysis of association between MCP-1 A/G gene polymorphism and SLE, consisting of 2425 patients with SLE and 2567 controls. In the overall populations, Asians, Caucasian population, the association between MCP-1 A/G gene polymorphism and SLE susceptibility was not found. Interestingly, a trend toward an association between A allele/AA genotype and LN risk was observed in overall populations, although there was no statistical difference. However, this meta-analysis indicated that AA genotype was associated with LN risk in Caucasians (OR = 0.71; 95% CI: 0.54-0.93; p = 0.01). In conclusion, our results indicate that AA homozygous might be a significant genetic molecular marker to predict the SLE patients developing into LN in Caucasians. However, more investigations are required to further clarify this association.

  4. Th-17 related regulatory network in the pathogenesis of Arab patients with systemic lupus erythematosus and lupus nephritis.

    Science.gov (United States)

    AlFadhli, Suad; AlFailakawi, Asma'a; Ghanem, Aqeel A M

    2016-05-01

    The present study aimed to identify the genes involved in the pathogenesis of systemic lupus erythematosus (SLE) in Arabs by investigating a panel of 84 genes related to the t helper (Th)17-related regulatory network and to further explore the expression levels of serum interleukin (IL)-17A and IL-17F in a studied cohort. A comparative analysis of gene expression profile in SLE and lupus nephritis (LN) patients against that of healthy controls (HC) was performed. A quantitative real-time polymerase chain reaction (PCR) (Th17 autoimmunity and inflammation) array analysis was performed in peripheral white blood cells of 66 SLE patients under specific medical treatment and 30 age/gender/ethnically matched healthy controls. Statistical analysis was carried out using the RT(2) Profiler TM PCR Data Analysis tool. The analysis of Th17 pathway revealed 14 genes (IL-17A, IL-17C, IL-17D, IL-17F, IL-18, IL-12RB2, IL-23R, CCL2, CCL20, CXCL5, MMP3, RORC, STAT4 and TRAF6) that are differentially expressed in SLE and HC (fold change [FC] < 2, P < 0.0006). No significant difference in expression profiles was observed between SLE and LN. A significant difference in serum concentration of IL-17A (P = 0.002) and IL-17F (P = 0.002) was observed between SLE (13.91 ± 4.25) and LN (18.26 ± 4.24). Our study is the first to investigate a panel of 84 genes related to Th17 regulatory pathway in Arab SLE subjects and the first to explore the effect of current immunosuppression regimens on Th17 regulatory pathway. It paves the way for understanding the etiology of SLE and autoimmune diseases in general. © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  5. Human Epididymis Protein 4: A Novel Biomarker for Lupus Nephritis and Chronic Kidney Disease in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Yang, Zaixing; Zhang, Zhiyu; Qin, Baodong; Wu, Ping; Zhong, Renqian; Zhou, Lin; Liang, Yan

    2016-11-01

    Human epididymis protein 4 (HE4) is an available tumor biomarker for detecting ovarian cancer. However, it is unknown if serum HE4 could be a novel biomarker for diagnosis of lupus nephritis (LN) and chronic kidney disease (CKD) in patients with systemic lupus erythematosus (SLE). This study enrolled 209 SLE patients, 75 patients with renal dysfunction without SLE and 32 healthy subjects. HE4 concentrations were analyzed by ELISA (enzyme-linked immunosorbent assay; Fujirebio Diagnostics, Sweden). The receiver operating characteristic (ROC) curves were constructed to assess diagnostic accuracy of HE4 for LN or CKD in SLE. Serum HE4 level was significantly higher in SLE patients than that in healthy controls (P < 0.001), especially for those with LN or CKD. It was also higher in patients with renal dysfunction without SLE than healthy controls (P < 0.001), while there was no significant difference between these patients and those with SLE with CKD (P = 0.73). Multivariate analysis showed significant association between increased HE4 and LN or CKD after controlling for confounders. ROC curves showed the cutoff values were 150.1 pM (sensitivity, 76.8%; specificity, 91.1%) for the diagnosis of LN in SLE and 233.9 pM (sensitivity, 92.9%; specificity, 93.5%) for CKD in SLE. Increased serum HE4 level is closely associated with the development of LN or CKD in SLE patients. Furthermore, it can be used as a novel and useful biomarker for diagnosis of LN or CKD. © 2016 Wiley Periodicals, Inc.

  6. Are Toll-Like Receptors and Decoy Receptors Involved in the Immunopathogenesis of Systemic Lupus Erythematosus and Lupus-Like Syndromes?

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    Giuliana Guggino

    2012-01-01

    Full Text Available In this paper we focus our attention on the role of two families of receptors, Toll-like receptors (TLR and decoy receptors (DcR involved in the generation of systemic lupus erythematosus (SLE and lupus-like syndromes in human and mouse models. To date, these molecules were described in several autoimmune disorders such as rheumatoid arthritis, antiphospholipids syndrome, bowel inflammation, and SLE. Here, we summarize the findings of recent investigations on TLR and DcR and their role in the immunopathogenesis of the SLE.

  7. Patient-reported outcome measures in a population of medically indigent patients with systemic lupus erythematosus in Puerto Rico

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    Diana V Rodríguez-Rivera

    2016-09-01

    Full Text Available Objective: To determine patient-reported outcomes measures in indigent patients with systemic lupus erythematosus receiving their healthcare through the Puerto Rico government managed care system and compare these measures with non-indigent patients treated in a private fee-for-service setting. Methods: A cross-sectional study was conducted in a cohort of 98 Puerto Ricans with systemic lupus erythematosus. Patients from the public group (n = 40 were treated in a university-based specialized systemic lupus erythematosus clinic and the private group (n = 58 in a community-based rheumatology practice. Demographic and clinical features and patient-reported outcomes measures per LupusPRO instrument were determined. LupusPRO captures quality-of-life measures in 12 domains. Differences among study groups were examined using chi-square, Fisher’s exact, t-tests, and the Wilcoxon signed-rank test. Results: The mean (standard deviation age of the study population was 44.9 (12.0 years; 94 (95.9% were women. Patients in the public setting were younger and were more likely to have renal disease and elevated anti-double-stranded DNA antibodies, and being treated with azathioprine and cyclophosphamide. Patients from the public sector were more likely to have better quality-of-life measures in the LupusPRO domains of pain/vitality and coping. No significant differences were observed for the domains of lupus symptoms, physical health, emotional health, body image, cognition, procreation, lupus medications, desires/goals, social support, and satisfaction with medical care. Conclusion: Despite having a lower socioeconomic status and worse clinical status, systemic lupus erythematosus patients from the public sector had equal or better patient-reported outcomes measures than those treated in the private setting. This favorable outcome may be associated with the comprehensive healthcare received by these patients in a specialized lupus clinic.

  8. Development and validation of a disease-specific health-related quality of life measure, the LupusQol, for adults with systemic lupus erythematosus.

    Science.gov (United States)

    McElhone, Kathleen; Abbott, Janice; Shelmerdine, Joanna; Bruce, Ian N; Ahmad, Yasmeen; Gordon, Caroline; Peers, Kate; Isenberg, David; Ferenkeh-Koroma, Ada; Griffiths, Bridget; Akil, Mohamed; Maddison, Peter; Teh, Lee-Suan

    2007-08-15

    To develop and validate a disease-specific health-related quality of life (HRQOL) instrument for adults with systemic lupus erythematosus (SLE). The work consisted of 6 stages. Stage 1 included item generation for questionnaire content from semistructured interviews with SLE patients. In stage 2 item selection for the draft questionnaire was performed by thematic analysis of the patient interview transcripts and expert panel agreement. In stage 3 the content validity of the draft questionnaire was assessed by patients completing the questionnaire and providing critical feedback. In stages 4 and 5 construct validity and internal reliability of the 3 versions of the LupusQoL were evaluated using principal component analysis with varimax rotation and Cronbach's alpha coefficients, respectively. In stage 6 discriminatory validity, concurrent validity, and test-retest reliability were evaluated. Stages 1, 2, and 3 resulted in a preliminary instrument containing 63 items. In stage 4, 8 domains were identified. This factor structure, accounting for 82% of the variance, was confirmed in stage 5. The domains and Cronbach's alpha coefficients were physical health (0.94), emotional health (0.94), body image (0.89), pain (0.92), planning (0.93), fatigue (0.88), intimate relationships (0.96), and burden to others (0.94). Discriminant validity was demonstrated for different levels of disease activity (British Isles Lupus Assessment Group Index) and damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index). High correlations (r = 0.71-0.79) between comparable domains of the Short Form 36 and the LupusQoL assured acceptable concurrent validity. Good test-retest reliability (r = 0.72-0.93) was demonstrated. The LupusQoL is a validated SLE-specific HRQOL instrument with 34 items across 8 domains defined by patients as being important.

  9. Incidence of systemic lupus erythematosus in a population-based cohort using revised 1997 American College of Rheumatology and the 2012 Systemic Lupus International Collaborating Clinics classification criteria.

    Science.gov (United States)

    Ungprasert, P; Sagar, V; Crowson, C S; Amin, S; Makol, A; Ernste, F C; Osborn, T G; Moder, K G; Niewold, T B; Maradit-Kremers, H; Ramsey-Goldman, R; Chowdhary, V R

    2017-03-01

    In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) group published a new set of classification criteria for systemic lupus erythematosus (SLE). Studies applying these criteria to real-life scenarios have found either equal or greater sensitivity and equal or lower specificity to the 1997 ACR classification criteria (ACR 97). Nonetheless, there are no studies that have used the SLICC 12 criteria to investigate the incidence of lupus. We used the resource of the Rochester Epidemiology Project to identify incident SLE patients in Olmsted County, Minnesota, from 1993 to 2005, who fulfilled the ACR 97 or SLICC 12 criteria. A total of 58 patients met criteria by SLICC 12 and 44 patients met criteria by ACR 97. The adjusted incidence of 4.9 per 100,000 person-years by SLICC 12 was higher than that by ACR 97 (3.7 per 100,000 person-years, p = 0.04). The median duration from the appearance of first criterion to fulfillment of the criteria was shorter for the SLICC 12 than for ACR 97 (3.9 months vs 8.1 months). The higher incidence by SLICC 12 criteria came primarily from the ability to classify patients with renal-limited disease, the expansion of the immunologic criteria and the expansion of neurologic criteria.

  10. Study of Flare Assessment in Systemic Lupus Erythematosus Based on Paper Patients.

    Science.gov (United States)

    Isenberg, D; Sturgess, J; Allen, E; Aranow, C; Askanase, A; Sang-Cheol, B; Bernatsky, S; Bruce, I; Buyon, J; Cervera, R; Clarke, A; Dooley, Mary Anne; Fortin, P; Ginzler, E; Gladman, D; Hanly, J; Inanc, M; Jacobsen, S; Kamen, D; Khamashta, M; Lim, S; Manzi, S; Nived, O; Peschken, C; Petri, M; Kalunian, K; Rahman, A; Ramsey-Goldman, R; Romero-Diaz, J; Ruiz-Irastorza, G; Sanchez-Guerrero, J; Steinsson, K; Sturfelt, G; Urowitz, M; van Vollenhoven, R; Wallace, D J; Zoma, A; Merrill, J; Gordon, C

    2018-01-01

    To determine the level of agreement of disease flare severity (distinguishing severe, moderate, and mild flare and persistent disease activity) in a large paper-patient exercise involving 988 individual cases of systemic lupus erythematosus. A total of 988 individual lupus case histories were assessed by 3 individual physicians. Complete agreement about the degree of flare (or persistent disease activity) was obtained in 451 cases (46%), and these provided the reference standard for the second part of the study. This component used 3 flare activity instruments (the British Isles Lupus Assessment Group [BILAG] 2004, Safety of Estrogens in Lupus Erythematosus National Assessment [SELENA] flare index [SFI] and the revised SELENA flare index [rSFI]). The 451 patient case histories were distributed to 18 pairs of physicians, carefully randomized in a manner designed to ensure a fair case mix and equal distribution of flare according to severity. The 3-physician assessment of flare matched the level of flare using the 3 indices, with 67% for BILAG 2004, 72% for SFI, and 70% for rSFI. The corresponding weighted kappa coefficients for each instrument were 0.82, 0.59, and 0.74, respectively. We undertook a detailed analysis of the discrepant cases and several factors emerged, including a tendency to score moderate flares as severe and persistent activity as flare, especially when the SFI and rSFI instruments were used. Overscoring was also driven by scoring treatment change as flare, even if there were no new or worsening clinical features. Given the complexity of assessing lupus flare, we were encouraged by the overall results reported. However, the problem of capturing lupus flare accurately is not completely solved. © 2017, The Authors. Arthritis Care & Research published by Wiley Periodicals, Inc. on behalf of American College of Rheumatology.

  11. Specific Psychosocial and Behavioral Outcomes from the Systemic Lupus Erythematosus Self-Help Course.

    Science.gov (United States)

    Braden, Carrie Jo; And Others

    1993-01-01

    Data from 104 participants in the Systemic Lupus Erythematosus Self-Help Course showed that patients had significant increases in enabling skills and use of relaxation/exercise and decreases in depression. Amount of time spent in class was correlated with significant changes over time. (SK)

  12. Replication of recently identified systemic lupus erythematosus genetic associations : a case-control study

    NARCIS (Netherlands)

    Suarez-Gestal, Marian; Calaza, Manuel; Endreffy, Emoeke; Pullmann, Rudolf; Ordi-Ros, Josep; Sebastiani, Gian Domenico; Ruzickova, Sarka; Santos, Maria Jose; Papasteriades, Chryssa; Marchini, Maurizio; Skopouli, Fotini N.; Suarez, Ana; Blanco, Francisco J.; D'Alfonso, Sandra; Bijl, Marc; Carreira, Patricia; Witte, Torsten; Migliaresi, Sergio; Gomez-Reino, Juan J.; Gonzalez, Antonio

    2009-01-01

    Introduction We aimed to replicate association of newly identified systemic lupus erythematosus (SLE) loci. Methods We selected the most associated SNP in 10 SLE loci. These 10 SNPs were analysed in 1,579 patients with SLE and 1,726 controls of European origin by single-base extension. Comparison of

  13. Libman–Sacks endocarditis, and other echocardiographic findings in systemic lupus erythematosus: Case report

    Directory of Open Access Journals (Sweden)

    Mohamed Atef Hamza

    2012-09-01

    Full Text Available Case report of a 19 year-old female patient with systemic lupus erythematosus (SLE who was presented to Ain Shams University Hospital complaining of dyspnea on moderate exertion. Echocardiography showed the presence of sterile vegetation on the mitral valve, Libman–Sacks endocarditis (LSE.

  14. Disease activity patterns in juvenile systemic lupus erythematosus and its relation to early aggressive treatment

    NARCIS (Netherlands)

    Otten, M. H.; Cransberg, K.; van Rossum, M. A. J.; Groothoff, J. W.; Kist-van Holthe, J. E.; ten Cate, R.; van Suijlekom-Smit, L. W. A.

    2010-01-01

    This study aimed to determine disease activity patterns in juvenile systemic lupus erythematosus (jSLE) and its relation to early treatment. All jSLE patients who visited the outpatient departments of three Dutch university hospitals for at least 6 months were included. Data were retrospectively

  15. Fatigue in patients with systemic lupus erythematosus : the role of dehydroepiandrosterone sulphate

    NARCIS (Netherlands)

    Overman, C. L.; Hartkamp, A.; Bossema, E. R.; Bijl, M.; Godaert, G. L. R.; Bijlsma, J. W. J.; Derksen, R. H. W. M.; Geenen, R.

    2012-01-01

    Fatigue is a major problem in systemic lupus erythematosus (SLE), but the physiological substrate of this fatigue is largely unclear. To examine if low levels of dehydroepiandrosterone (DHEA) and its sulphate DHEAS play a role in SLE fatigue, we compared: 1) DHEAS levels and fatigue between 60

  16. Regional specific changes of cerebral metabolism in systemic lupus erythematosus identified by positron emission tomography

    NARCIS (Netherlands)

    de Jong, BM; Pruim, J; Sinnige, LGF; Beintema, KD; Spronk, PE; Bootsma, H; Kallenberg, CGM; van Zomeren, AH; Haaxma-Reiche, H

    1999-01-01

    In order to test the hypothesis whether the pathogenesis of cerebral systemic lupus erythematosus (SLE) may include an immune-mediated deficit in specific vulnerable brain regions, the regional cerebral metabolism in 9 patients with diffuse as well as focal cerebral symptoms was compared with that

  17. Autoantibodies to high mobility group box 1 in patients with Incomplete and Systemic Lupus Erythematosus

    NARCIS (Netherlands)

    Schaper, F.; De Leeuw, K.; Horst, G.; Maas, F.; Beijeren, D.V.; Bijzet, J.; Heeringa, P.; Limburg, P.C.; Westra, J.

    2015-01-01

    Introduction. High Mobility Group Box-1 (HMGB1) is involved in the pathogenesis of Systemic Lupus Erythematosus (SLE). However, the role of autoantibodies to HMGB1 is unclear. Therefore levels of anti-HMGB1 and their reactivity to HMGB1 BoxA and BoxB were examined in association with disease

  18. Bone metabolism in patients with systemic lupus erythematosus. Effect of disease activity and glucocorticoid treatment

    DEFF Research Database (Denmark)

    Hansen, M; Halberg, P; Kollerup, G

    1998-01-01

    The bone metabolism in patients with systemic lupus erythematosus (SLE) has previously been examined, but the results are conflicting. In the present study the bone mineral density (BMD) of the axial and the appendicular skeleton was examined by means of dual energy x-ray absorptiometry. The bone...

  19. Systemic lupus erythematosus in a 7-year-old girl: A first case report ...

    African Journals Online (AJOL)

    Systemic lupus erythematosus is an autoimmune multisystemic inflammatory disease that is rare in children. Though a disease of the black race it is rarely diagnosed in black African children. Only few cases have been report in Nigeria and these were in the south. We report a case of a 7-year-old girl who presented with ...

  20. Validation of the Fatigue Severity Scale in Danish patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Lorentzen, Kristian; Danielsen, Mads Ammitzbøll; Kay, Susan Due

    2014-01-01

    INTRODUCTION: Fatigue is a symptom of systemic lupus erythematosus (SLE), which has a substantial effect on the patients' quality of life and is a parameter that is difficult to quantify. The Fatigue Severity Scale (FSS) is a validated and reliable tool for quantifying fatigue. However, no Danish...

  1. Mannose-binding lectin variant alleles and the risk of arterial thrombosis in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Øhlenschlaeger, Tommy; Garred, Peter; Madsen, Hans O

    2004-01-01

    Cardiovascular disease is an important complication in patients with systemic lupus erythematosus (SLE). Variant alleles of the mannose-binding lectin gene are associated with SLE as well as with severe atherosclerosis. We determined whether mannose-binding lectin variant alleles were associated...

  2. Xylohypha bantiana Multiple Brain Abscesses in a Patient with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Khalid F AlHabib

    2003-01-01

    Full Text Available Xylohypha bantiana is a rare cause of cerebral fungal infection (phaeohyphomycosis. We report on a 72-year-old man who, while taking several immunosuppressive medications for systemic lupus erythematosus, presented with multiple bilateral cerebral abscesses caused by X bantiana. The lesions were not surgically amenable and the patient died two months after discontinuing antifungal therapy.

  3. The shrinking lung syndrome in systemic lupus erythematosus: improvement with corticosteroid therapy

    NARCIS (Netherlands)

    Oud, K. T. M.; Bresser, P.; ten Berge, R. J. M.; Jonkers, R. E.

    2005-01-01

    Respiratory manifestations of systemic lupus erythematosus (SLE) are frequent. The 'shrinking lung syndrome' (SLS) represents a rare complication of SLE. The pathogenesis and therapy of the SLS remains controversial. We report a series of five consecutive cases with the SLS of which we provide a

  4. Concordance of autoimmune disease in a nationwide Danish systemic lupus erythematosus twin cohort

    DEFF Research Database (Denmark)

    Ulff-Møller, Constance Jensina; Svendsen, Anders Jørgen; Viemose, Louise Nørgaard

    2018-01-01

    OBJECTIVE: To determine the concordance of systemic lupus erythematosus (SLE) and co-aggregating autoimmune diseases among Danish twins. METHODS: SLE-affected twins were ascertained by record linkage between the National Patient Register (NPR) and the Danish Twin Registry (DTR). Registered SLE...

  5. Peripheral aneurysm rupture in a patient with inactive systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Engelke, Christoph; Sabharwal, Tarun; Reidy, John F. [Department of Radiology, Guy' s and St. Thomas' Hospital Trust, St. Thomas' Street, London SE1 9RT (United Kingdom); Mohan, Aarthi R. [Department of Chest Medicine, Guy' s and St. Thomas' Hospital Trust, St. Thomas' Street, London SE1 9RT (United Kingdom)

    2002-12-01

    We describe a patient with inactive systemic lupus erythematosus (SLE) presenting with sudden haemothorax, due to a ruptured internal mammary artery (IMA) aneurysm 7 years after the corticosteroid treatment was terminated. The unusual imaging findings and the treatment with embolization are discussed with a view to the role of a regular vascular screening in this patient group. (orig.)

  6. DNA polymorphism of HLA class II genes in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Cowland, J B; Andersen, V; Halberg, P

    1994-01-01

    We investigated the DNA restriction fragment length polymorphism (RFLP) of the major histocompatibility complex (MHC) genes: HLA-DRB, -DQA, -DQB, -DPB in 24 Danish patients with systemic lupus erythematosus (SLE) and in 102 healthy Danes. A highly significant increase of the frequency of the DR3...

  7. Autoantibodies persist in relatives to systemic lupus erythematosus patients during 12 years follow-up

    DEFF Research Database (Denmark)

    Langkilde, Henrik; Voss, A; Heegaard, N

    2017-01-01

    BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease with presence of autoantibodies and characteristic multi-organ involvement. Relatives of SLE patients have an increased risk of autoantibody production and autoimmune diseases. METHODS: In 2001, 226 first degree relatives (FDRs...

  8. Functional variants in the B-cell gene BANK1 are associated with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Kozyrev, Sergey V; Abelson, Anna-Karin; Wojcik, Jerome

    2008-01-01

    Systemic lupus erythematosus (SLE) is a prototypical autoimmune disease characterized by production of autoantibodies and complex genetic inheritance. In a genome-wide scan using 85,042 SNPs, we identified an association between SLE and a nonsynonymous substitution (rs10516487, R61H) in the B...

  9. Serum levels of ficolin-3 (Hakata antigen) in patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Andersen, T.; Munthe-Fog, L.; Garred, P.

    2009-01-01

    OBJECTIVE: Ficolin-3 is a serum protein of putative importance in autoimmunity. Our objective was to investigate any differential expression of ficolin-3 in patients with systemic lupus erythematosus (SLE) or its clinical subsets. METHODS: Serum levels of ficolin-3 (S-ficolin-3) were determined...

  10. Longitudinal study on premature atherosclerosis in patients with systemic lupus erythematosus

    NARCIS (Netherlands)

    de Leeuw, Karina; Smit, Andries J.; de Groot, Eric; van Roon, Arie M.; Kallenberg, Cees G.; Bijl, Marc

    2009-01-01

    Objectives: To determine risk factors of accelerated atherosclerosis and progression of intima-media thickness (IMT) in patients with systemic lupus erythematosus (SLE). Methods: 74 SLE patients, age ranging from 13 to 69 years, and 74 age- and sex-matched controls were included. IMT of the common

  11. Recent Developments in the Role of High-Mobility Group Box 1 in Systemic Lupus Erythematosus

    NARCIS (Netherlands)

    Schaper, Fleur; Westra, Johanna; Bijl, Marc

    2014-01-01

    High-mobility group box 1 (HMGB1) is an important molecule for several nuclear processes. Recently, HMGB1 has gained much attention as a damage-associated molecular pattern (DAMP) and has been implicated in the pathogenesis of several (auto)immune diseases, in particular, systemic lupus

  12. The Systemic Lupus Erythematosus Responder Index (SRI); A new SLE disease activity assessment

    NARCIS (Netherlands)

    Luijten, K. M. A. C.; Tekstra, J.; Bijlsma, J. W. J.; Bijl, M.

    Systemic Lupus Erythematosus (SLE), because of its complex and multisystemic presentation, lacks a reliable and sensitive gold standard for measuring disease activity. In addition, there is no standardized method for defining response to therapy. Several disease activity indices have been developed

  13. Photosensitivity to fluorescent light in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Martin, L; Chalmers, I M

    1983-10-01

    A 50-year-old woman with systemic lupus erythematosus (SLE) developed a severe rash on her back and shoulders after exposure to fluorescent light while she was a hospital inpatient. Even small amounts of ultraviolet irradiation, such as that emitted by a domestic fluorescent tube, may be harmful to some patients with SLE.

  14. Immune response modulation by Vitamin D: role in systemic lupus erythematosus.

    Directory of Open Access Journals (Sweden)

    Mirentxu eIruretagoyena

    2015-10-01

    Full Text Available Vitamin D plays key roles as a natural immune modulator and has been implicated in the pathophysiology of autoimmune diseases, including systemic lupus erythematosus (SLE. This review presents a summary and analysis of the recent literature regarding immunoregulatory effects of vitamin D as well as its importance in SLE development, clinical severity and possible effects of supplementation in disease treatment.

  15. Acute acalculous cholecystitis in systemic lupus erythematosus: a rare initial manifestation.

    Science.gov (United States)

    Manuel, Valdano; Pedro, Gertrudes Maria; Cordeiro, Lemuel Bornelli; de Miranda, Sandra Maria da Rocha Neto

    2016-01-01

    Acute acalculous cholecystitis is a very rare gastrointestinal manifestation in systemic lupus erythematosus and becomes rarer as an initial manifestation. There are only two cases reported. The authors report a 20-year-old black woman that presented acute acalculous cholecystitis revealed by abdominal computed tomography. During hospitalization, she was diagnosed systemic lupus erythematosus. Conservative treatment with antibiotics was performed with complete remission of the symptoms. Corticosteroid was started in ambulatory. Cholecystectomy has been the treatment of choice in acute acalculous cholecystitis as a complication of systemic lupus erythematosus. The patient responded well to conservative treatment, and surgery was not required. This case is unique in the way that corticosteroid was started in ambulatory care. We should not forget that the acute acalculous cholecystitis can be the initial presentation of systemic lupus erythematosus although its occurrence is very rare. Conservative treatment should be considered. Abdominal computed tomography was a determinant exam for better assessment of acute acalculous cholecystitis. Copyright © 2013 Elsevier Editora Ltda. All rights reserved.

  16. Flaccid paresis due to distal renal tubular acidosis preceding systemic lupus erythematosus.

    NARCIS (Netherlands)

    Meulen, C.G. ter; Pieters, G.F.F.M.; Huysmans, F.T.M.

    2002-01-01

    We report a 25-year-old woman presenting with a flaccid paresis due to severe hypokalaemia as a consequence of distal renal tubular acidosis (dRTA). Six years after presentation of dRTA, she developed overt symptoms of systemic lupus erythematosus (SLE). dRTA in SLE is often secondary to an

  17. Soluble TRAIL concentrations are raised in patients with systemic lupus erythematosus

    NARCIS (Netherlands)

    Lub-De Hooge, M.N.; de Vries, E.G.E.; de Jong, S.; Bijl, Marc

    Background: Increased apoptosis may induce autoimmune conditions. Apoptosis is induced by binding of death receptor ligands, members of the tumour necrosis factor (TNF) superfamily, to their cognate receptors. The Fas - Fas ligand pathway has been studied extensively in relation to systemic lupus

  18. Infections Increase Risk of Arterial and Venous Thromboses in Danish Patients with Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Baronaite Hansen, Renata; Jacobsen, Søren

    2014-01-01

    OBJECTIVE: Infections and thromboses are known complications of systemic lupus erythematosus (SLE). We investigated if infectious episodes in patients with SLE were followed by an increased risk of thrombotic events. METHODS: A cohort of 571 patients with prevalent or incident SLE was followed...

  19. HPV infection and vaccination in Systemic Lupus Erythematosus patients: What we really should know

    NARCIS (Netherlands)

    I. Grein (Ingrid); N. Groot (Noortje); Lacerda, M.I. (Marcela Ignacchiti); N.M. Wulffraat (Nico); G. Pileggi (Gecilmara)

    2016-01-01

    textabstractPatients with Systemic Lupus Erythematosus (SLE) are at increased risk for infections. Vaccination is a powerful tool to prevent infections, even in immunocompromised patients. Most non-live vaccines are immunogenic and safe in patients with SLE, even if antibody titres are frequently

  20. HPV infection and vaccination in Systemic Lupus Erythematosus patients : what we really should know

    NARCIS (Netherlands)

    Rotstein Grein, Ingrid Herta; Groot, Noortje; Lacerda, Marcela Ignacchiti; Wulffraat, Nico; Pileggi, Gecilmara

    2016-01-01

    Patients with Systemic Lupus Erythematosus (SLE) are at increased risk for infections. Vaccination is a powerful tool to prevent infections, even in immunocompromised patients. Most non-live vaccines are immunogenic and safe in patients with SLE, even if antibody titres are frequently lower than