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Sample records for lupus erythrematosus sle

  1. Hydroxychloroquine in systemic lupus erythematosus (SLE).

    Science.gov (United States)

    Ponticelli, C; Moroni, G

    2017-03-01

    Hydroxychloroquine (HCQ) is an alkalinizing lysosomatropic drug that accumulates in lysosomes where it inhibits some important functions by increasing the pH. HCQ has proved to be effective in a number of autoimmune diseases including systemic lupus erythematosus (SLE). Areas covered: In this review the mechanisms of action, the efficacy, and the safety of HCQ in the management of patients with SLE have been reviewed. HCQ may reduce the risk of flares, allow the reduction of the dosage of steroids, reduce organ damage, and prevent the thrombotic effects of anti-phospholipid antibodies. The drug is generally safe and may be prescribed to pregnant women. However, some cautions are needed to prevent retinopathy, a rare but serious complication of the prolonged use of HCQ. Expert opinion: HCQ may offer several advantages not only in patients with mild SLE but can also exert important beneficial effects in lupus patients with organ involvement and in pregnant women. The drug has a low cost and few side effects. These characteristics should encourage a larger use of HCQ, also in lupus patients with organ involvement.

  2. Epidemiologi ved systemisk lupus erythematosus (SLE

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    Inge-Margrethe Gilboe

    2009-10-01

    Full Text Available Det er utført få epidemiologiske studier av SLE i Norge. De studier som er utført viser liknende hyppighet og forekomst av SLE i Norge som i de øvrige nordiske land og forekomst av mild sykdom med en lav andel av alvorlig indre organ manifestasjoner. Insidens og prevalens er kartlagt i Nord-Norge visende en lavere insidens enn i de øvrige nordiske land, mens prevalensen er sammenliknbar. Tall fra Sverige tyder på stabil insidens, men økende prevalens, forenelig med økt overlevelse. Norske og nordiske studier viser høy kvinneandel og sammenliknbar alder ved diagnose. Studiene tyder på en høyere alder ved diagnose i Skandinavia enn i USA og England. Ut fra de nordiske studier er overlevelsen ved SLE god, selv om den er lavere enn i den generelle befolkningen. Overlevelse i de nordiske og europeiske land er sammenliknbare.Selv om overlevelsen ved SLE er betydelig bedret over tid utgjorde aktiv SLE og infeksjoner 50% av dødsårsakene i den danske mortalitetsstudien. Aterosklerotisk hjerte-karsykdom var en hyppig dødsårsak i alle studiene. Kardiovaskulær sykdom som hovedårsak til død ved SLE er påvist i den store internasjonale studien hvor pasienter fra Island og Sverige deltok og i andre europeiske og internasjonale studier. Betydelig økt risiko for hjerteinfarkt hos unge SLE-kvinner i Sverige er også påvist tidligere i internasjonale studier og styrker teorien om at SLE per ce er en selvstendig risikofaktor for aterosklerose. Det er ønskelig med flere epidemiologiske studier av SLE i Norge.There has been few studies on the epidemiology of SLE in Norway. The studies that have been conducted show similar frequency and occurence of SLE in Norway in comparison to the other Nordic countries, and mainly occurence of mild disease with only a low proportion suffering severe internal organ involvement. Studies on incidence and prevalence in the northern part of Norway show a lower incidence than in other Nordic countries, but a

  3. Disease characterization of systemic lupus erythematosus (SLE) patients in Quebec.

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    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.

  4. Presence of antibodies against a cell-surface protein, cross-reactive with DNA, in systemic lupus erythrematosus: a marker of the disease

    International Nuclear Information System (INIS)

    Jacob, L.; Lety, M.A.; Choquette, D.; Viard, J.P.; Jacob, F.; Louvard, D.; Bach, J.F.

    1987-01-01

    Antibodies against a cell-surface protein, cross-reactive with double-stranded DNA, were detected in the serum of 25 patients with active human systemic lupus erythematosus (SLE), defined on the basis of the revised American Rheumatism Association classification. Among these sera, two did not display anti-DNA antibodies, as shown by Farr assay, solid-phase radioimmunoassay, and Crithidia luciliae test. Five other SLE patients were consecutively studied in active and remission states. Antibodies against the protein were detected in the serum of the 5 SLE patients when they were in active phase but not in the serum of the same patients in inactive phase of the disease. The anti-protein antibodies were not found in the serum of 10 inactive SLE patients or in the sera of 10 normal human controls, 10 patients with rheumatoid arthritis, 5 patients with scleroderma, and 4 patients with primary sicca syndrome. Taken together, these results strongly suggest that antibodies against this cell-surface protein could provide a better diagnosis marker and activity index than anti-DNA antibodies in SLE

  5. Psychiatric disorders in systemic lupus erythematosus (SLE: a serious SLE-related crime

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    Gabriele Nicolosi

    2013-04-01

    Full Text Available Introduction: Systemic lupus erythematosus (SLE is associated with CNS disorders in 50-90% of all cases. Thus far 19 neuropsychiatric syndromes have been reported in association with SLE, and many others will be added to this list in the future. Long-term observation and use of the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (Text Revision is the solution for a correct diagnosis. Case report: The patient was an 18-year-old woman who had been charged with the attempted murder of an elderly woman in the latter’s home. According to the victim’s testimony, the young woman had entered the victim’s home under pretence and suddently attacked her with a hammer. The young woman denied all knowledge of the event. A few days after her arrest the patient was hospitalized for attempted suicide. The work-up that began with this hospitalization led to the diagnosis of an intermittent explosive disorder secondary to SLE. The authors analyze this case from the psychiatric, medical, and legal points of view. Conclusions: This is the first report of this type of disorder is a patient with SLE. The authors suggest that intermittent explosive disorder should be added to the list of neuropsychiatric syndromes associated with this disease.

  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. SLE disease patterns in a Danish population-based lupus cohort: an 8-year prospective study

    DEFF Research Database (Denmark)

    Laustrup, H; Voss, A; Green, A

    2009-01-01

    In 1995 all systemic lupus erythematosus (SLE) patients in the county of Funen were retrieved from four separate and independent sources as part of an 8-year prospective study to determine the pattern of disease activity and damage accumulation in a community based lupus cohort of predominantly...... Scandinavian ancestry. Incident cases were subsequently identified by surveillance of these sources. Established and new cases underwent annual, structured interviews, clinical examination and blood sampling. The Systemic Lupus Erythematosus Diseases Activity Index SLEDAI and Systemic Lupus International...

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

    African Journals Online (AJOL)

    of this study was to determine the most common features of patients with systemic lupus erythematosus ... Conclusion: Most of the findings correlate with similar studies worldwide. .... Sciences, University of the Free State to conduct the study.

  9. Autoantibodies against Modified Histone Peptides in SLE Patients Are Associated with Disease Activity and Lupus Nephritis

    NARCIS (Netherlands)

    Dieker, J.W.; Berden, J.H.; Bakker, M.A.; Briand, J.P.; Muller, S.; Voll, R.; Sjowall, C.; Herrmann, M.; Hilbrands, L.B.; Vlag, J. van der

    2016-01-01

    Persistent exposure of the immune system to death cell debris leads to autoantibodies against chromatin in patients with systemic lupus erythematosus (SLE). Deposition of anti-chromatin/chromatin complexes can instigate inflammation in multiple organs including the kidney. Previously we identified

  10. Murine Lupus Susceptibility Locus Sle2 Activates DNA-Reactive B Cells through Two Sub-Loci with Distinct Phenotypes

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    Zeumer, Leilani; Sang, Allison; Niu, Haitao; Morel, Laurence

    2010-01-01

    The NZM2410-derived Sle2 lupus susceptibility locus induces an abnormal B cell differentiation which most prominently leads to the expansion of autoreactive B1a cells. We have mapped the expansion of B1a cells to three Sle2 sub-loci, Sle2a, Sle2b, and Sle2c. Sle2 also enhances the breach of B cell tolerance to nuclear antigens in the 56R anti-DNA immunoglobulin transgenic (Tg) model. This study used the Sle2 sub-congenic strains to map the activation of 56R Tg B cells. Sle2c strongly sustained the breach of tolerance and the activation of anti-DNA B cells. The production of Tg-encoded anti-DNA antibodies was more modest in Sle2a expressing mice, but Sle2a was responsible for the recruitment for Tg B cells to the marginal zone, a phenotype that has been found for 56R Tg B cells in mice expressing the whole Sle2 interval. In addition, Sle2a promoted the production of endogenously encoded anti-DNA antibodies. Overall, this study showed that at least two Sle2 genes are involved in the activation of anti-DNA B cells, and excluded more than two-thirds of the Sle2 interval from contributing to this phenotype. This constitutes an important step toward the identification of novel genes that play a critical role in B cell tolerance. PMID:21270826

  11. Barriers And Motivators for Smoking Cessation in Patients With Systemic Lupus Erythematosus (SLE).

    Science.gov (United States)

    Terrell, Deirdra R; Stewart, Lauren M; Tolma, Eleni L; McClain, Rebekah; Vesely, Sara K; James, Judith A

    2015-11-01

    Although studies have shown that smoking is detrimental to the health of patients with systemic lupus erythematosus (SLE), studies regarding barriers and motivators for smoking cessation are lacking. The purpose of this study was to generate hypotheses regarding the barriers and motivators for smoking cessation in SLE patients. This study was based on the theoretical framework of the stages of change model. All participants met SLE classification criteria. Interviews were conducted with 16 current and 10 former smokers. Motivators included: medical reasons, readiness, and concern for others. Barriers included: enjoyment, coping mechanism, and an emotional connection. Participants were unsure of the impact of smoking on their medication and disease, and had mixed feelings regarding the impact on pain. The main motivator for cessation in this population was concern for one's health. Rheumatologists need to include disease specific harms and assess pain management strategies as part of cessation counseling.

  12. Site differences in mild cognitive dysfunction (MCD) among patients with systemic lupus erythematosus (SLE).

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    Kozora, E; Erkan, D; West, S G; Filley, C M; Zhang, L; Ramon, G; Duggan, E; Lockshin, M D

    2013-01-01

    Mild cognitive dysfunction (MCD) is common in patients with systemic lupus erythematosus (MCD-SLE) but few studies have investigated potential site differences. SLE patients from Denver, CO, and New York, NY, were enrolled in two different cognition studies employing similar screening methods. Using the resulting neuropsychological scores, cognitive impairment was calculated using a cognitive impairment index (CII). The rate of MCD-SLE was 24% at the Denver, CO, site and 60% at the New York, NY, site. The mean CII was 2.6 ± 2.3 versus 4.4 ± 2.7, respectively (p = 0.005). The NY participants had a significantly longer disease duration (p = 0.13) and higher American College of Rheumatology SLE criteria scores (p > 0.001). NY participants had a higher frequency of impairment in semantic verbal fluency (p = 0.005), visuomotor speed (p = 0.013), and motor sequencing (p = 0.001). A correlation was found between cognitive impairment and SLE disease duration (p = 0.03). The rate of MCD-SLE was greater in SLE patients from New York, NY, compared to patients in the Denver, CO, area. The greater duration of disease and higher prevalence of medical complications in the NY group might contribute to this difference. Findings suggest that MCD-SLE may differ by site, but future studies that better evaluate site or selection bias are recommended.

  13. Damage in the Multiethnic Malaysian Systemic Lupus Erythematosus (SLE) Cohort: Comparison with Other Cohorts Worldwide

    Science.gov (United States)

    Shaharir, Syahrul Sazliyana; Hussein, Heselynn; Rajalingham, Sakthiswary; Mohamed Said, Mohd Shahrir; Abdul Gafor, Abdul Halim; Mohd, Rozita; Mustafar, Ruslinda

    2016-01-01

    Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease and despite the improvement in the survival in the past few decades, the morbidity due to disease damage remains significant. The objectives of this study were to investigate the disease damagepattern and determine the associated factors of damage in the multi-ethnic Malaysian SLE patients. We consecutively 424SLE patients who attended a consistent follow-up at the National University of Malaysia Medical Centre and Putrajaya Hospital were recruited. Disease damage was assessed using the SLICC/ACR (Systemic Lupus International Collaborating Clinics/American College of Rheumatology) Damage Index (SDI) scores. Information on their demographics and disease characteristics were obtained from the clinical record. Univariate analysis was performed and the best model of independent predictors of disease damage was determined by multivariate logistic regression analysis. A total of 182 patients (42.9%) had disease damage (SDI ≥1). A significantly higher number of Indian patients had disease/organ damage and they predominantly developed steroid-induced diabetes mellitus (SDM). Patients with corticosteroid-induced osteoporosis (CIOP) were more likely to be Malayswhile majority of patients who developed malignancy were Chinese (p<0.05). In the univariate and multivariate analyses, disease damage was significantly associated with age, Indian ethnicity, lower mean cumulative C3 level, neuropsychiatry lupus (NPSLE), and antiphospholipid syndrome (APLS). Patients who had ever and early treatment with hydroxychloroquine(HCQ)were less likely to develop disease damage while more patients who had received oral prednisolone ≥1mg/kg daily over 2 weeks had disease damage (p<0.05). In conclusion, there were inter-ethnic differences in the damage pattern and risks among SLE patients. PMID:27846298

  14. Simultaneous presentation of acute disseminated encephalomyelitis (ADEM) and systemic lupus erythematosus (SLE) after enteroviral infection: can ADEM present as the first manifestation of SLE?

    Science.gov (United States)

    Kim, J-M; Son, C-N; Chang, H W; Kim, S-H

    2015-05-01

    Central Nervous System (CNS) involvement of Systemic Lupus Erythematosus (SLE) includes a broad range of neuropsychiatric syndromes. Acute Disseminated Encephalomyelitis (ADEM) is a demyelinating CNS disorder characterized by encephalopathy and multifocal lesions predominantly involving the white matter on brain magnetic resonance imaging. ADEM associated with SLE has been only rarely reported. We report an unusual case of a 17-year-old girl who developed ADEM after enteroviral infection as the first manifestation of SLE. The authors emphasize that the patient's illness was preceded by enteroviral infection and that ADEM occurred before any other symptoms of SLE, which makes this case unique. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  15. Analysis of systemic lupus erythematosus (SLE) involving the central nervous system by magnetic resonance imaging (MRI)

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    Suzuki, Kimihiro; Hara, Masako; Nakajima, Shinji and others

    1989-04-01

    Involvement of the central nervous system (CNS) commonly occurs in systemic lupus erythematosus (SLE). But definitive diagnosis remains difficult even with computed tomography (CT). In this study, we used the recently developed technique, magnetic resonance imaging (MRI) for CNS lupus and compared it with CT scans. CT was performed with a General Electric 8800 CT/T scanner. MRI was performed using a Mitsubishi Electric MMI-150 S. Ten patients with CNS lupus were divided into 3 groups. Group I included 4 cases with neurological manifestations alone. All lesions seen on CT were also detected by MRI, with greater clarity and extent. Furthermore, MRI depicted several microinfarcts in white matter without symptoms. Group II included 5 cases with psychiatric features alone. MRI detected a thalamic microinfarct in only one case while CT showed no abnormality in all cases. Group III included 1 case with both neurological and psychiatric symptoms. MRI demonstrated a small infarct of midbrain corresponding with neurological symptoms, more clearly than CT. Therefore MRI demonstrates the degree of brain involvement in SLE more accurately than CT. (author).

  16. Analysis of systemic lupus erythematosus (SLE) involving the central nervous system by magnetic resonance imaging (MRI)

    International Nuclear Information System (INIS)

    Suzuki, Kimihiro; Hara, Masako; Nakajima, Shinji

    1989-01-01

    Involvement of the central nervous system (CNS) commonly occurs in systemic lupus erythematosus (SLE). But definitive diagnosis remains difficult even with computed tomography (CT). In this study, we used the recently developed technique, magnetic resonance imaging (MRI) for CNS lupus and compared it with CT scans. CT was performed with a General Electric 8800 CT/T scanner. MRI was performed using a Mitsubishi Electric MMI-150 S. Ten patients with CNS lupus were divided into 3 groups. Group I included 4 cases with neurological manifestations alone. All lesions seen on CT were also detected by MRI, with greater clarity and extent. Furthermore, MRI depicted several microinfarcts in white matter without symptoms. Group II included 5 cases with psychiatric features alone. MRI detected a thalamic microinfarct in only one case while CT showed no abnormality in all cases. Group III included 1 case with both neurological and psychiatric symptoms. MRI demonstrated a small infarct of midbrain corresponding with neurological symptoms, more clearly than CT. Therefore MRI demonstrates the degree of brain involvement in SLE more accurately than CT. (author)

  17. Identification of MAMDC1 as a candidate susceptibility gene for systemic lupus erythematosus (SLE.

    Directory of Open Access Journals (Sweden)

    Anna Hellquist

    2009-12-01

    Full Text Available Systemic lupus erythematosus (SLE is a complex autoimmune disorder with multiple susceptibility genes. We have previously reported suggestive linkage to the chromosomal region 14q21-q23 in Finnish SLE families.Genetic fine mapping of this region in the same family material, together with a large collection of parent affected trios from UK and two independent case-control cohorts from Finland and Sweden, indicated that a novel uncharacterized gene, MAMDC1 (MAM domain containing glycosylphosphatidylinositol anchor 2, also known as MDGA2, MIM 611128, represents a putative susceptibility gene for SLE. In a combined analysis of the whole dataset, significant evidence of association was detected for the MAMDC1 intronic single nucleotide polymorphisms (SNP rs961616 (P -value = 0.001, Odds Ratio (OR = 1.292, 95% CI 1.103-1.513 and rs2297926 (P -value = 0.003, OR = 1.349, 95% CI 1.109-1.640. By Northern blot, real-time PCR (qRT-PCR and immunohistochemical (IHC analyses, we show that MAMDC1 is expressed in several tissues and cell types, and that the corresponding mRNA is up-regulated by the pro-inflammatory cytokines tumour necrosis factor alpha (TNF-alpha and interferon gamma (IFN-gamma in THP-1 monocytes. Based on its homology to known proteins with similar structure, MAMDC1 appears to be a novel member of the adhesion molecules of the immunoglobulin superfamily (IgCAM, which is involved in cell adhesion, migration, and recruitment to inflammatory sites. Remarkably, some IgCAMs have been shown to interact with ITGAM, the product of another SLE susceptibility gene recently discovered in two independent genome wide association (GWA scans.Further studies focused on MAMDC1 and other molecules involved in these pathways might thus provide new insight into the pathogenesis of SLE.

  18. High mobility group box1 (HMGB1) in relation to cutaneous inflammation in systemic lupus erythematosus (SLE)

    NARCIS (Netherlands)

    Abdulahad, D.A.; Westra, J.; Reefman, E.; Zuidersma, E.; Bijzet, J.; Limburg, P.C.; Kallenberg, C.G.M.; Bijl, M.

    2013-01-01

    Photosensitivity is characteristic of systemic lupus erythematosus (SLE). Upon ultraviolet B (UVB) exposure, patients develop inflammatory skin lesions in the vicinity of sunburn cells (SBCs). High mobility group box 1 (HMGB1) is released from apoptotic and activated cells and exerts inflammatory

  19. Behavioral Deficits Are Accompanied by Immunological and Neurochemical Changes in a Mouse Model for Neuropsychiatric Lupus (NP-SLE)

    DEFF Research Database (Denmark)

    Li, Yan; Eskelund, Amanda; Zhou, H

    2015-01-01

    pathophysiological mechanisms responsible for NP-SLE are increased peripheral pro-inflammatory cytokines, subsequent induction of indoleamine-2,3-dioxygenase (IDO) and activation of the kynurenine pathway. In the MRL/MpJ-Faslpr (MRL/lpr) murine model of lupus, depression-like behavior and cognitive dysfunction...

  20. Investigations of a rabbit (Oryctolagus cuniculus model of systemic lupus erythematosus (SLE, BAFF and its receptors.

    Directory of Open Access Journals (Sweden)

    Jiahui Yang

    2009-12-01

    Full Text Available B-cell activation factor belonging to the tumor necrosis factor family (BAFF is a major contributor to survival of B lymphocytes during development and maturation. A relationship between circulating BAFF levels and disease activity has been reported in patients with the autoimmune disease Systemic Lupus Erythematosus (SLE. Clinical trials targeting BAFF or its receptors are currently in progress. In order to further characterize a rabbit (Oryctolagus cuniculus model of SLE, we investigated the expression of BAFF and its receptors in non-inbred, pedigreed rabbits derived from breeding and selection based on autoantibody responses. We immunized rabbits related to previous groups that developed autoantibodies and inflammatory responses after immunizations with peptides synthesized on multiple antigen-branched polylysine backbones. Blood and sera collected before immunization and after boosts were used for health monitoring, analyses of serum autoantibody responses by ELISA and immunofluorescence. Peripheral blood mononuclear cells (PBMC were studied by flow cytometry and were the source of mRNA for quantitative PCR analyses. We hypothesized that BAFF mRNA expression and serum BAFF levels measured indirectly through BAFF receptor binding might increase in autoantibody-producing rabbits. Immunized rabbits developed elevated levels of leucocyte populations, anti-nuclear, anti-dsDNA and other autoantibodies. BR3 mRNA levels in total PBMC decreased and BAFF levels remained low and unchanged in most immunized rabbits. By flow cytometry, percentages of BAFF positive cells decreased. Percentages of transmembrane activator and CAML interactor (TACI decreased in most rabbits from all the immunized groups. The rabbit is an important model for human autoimmune and infectious diseases, and a high quality draft rabbit genome assembly was recently completed. Human disease models developed in non-inbred pedigreed animals are better able to reflect the complexities

  1. Association of anti C1q and ds-DNA levels with the pathogenesis of Lupus Nephritis among SLE patients

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    Zara Sohail

    2018-02-01

    Full Text Available Background: Lupus nephritis (LN is the most common and serious complication associated with SLE and it results in significant morbidity and mortality. It is known by several studies that patients of LN have higher levels of anti-dsDNA and anti-C1q compared with SLE patients without renal involvement. The current study was designed to determine and compare the level of anti-dsDNA and anti-C1q in patients of SLE with and without lupus nephritis in the Pakistani population. This current study was also aimed at providing proof that anti-C1q levels are more prominent in LN/non-LN SLE as compared to anti-dsDNA. This project may help in the determination of results in Pakistan and contribute to the further confirmation of the sensitivity of anti-C1q. Method: The patient samples were collected from Sheikh Zayed hospital, Lahore. These patients were clinically diagnosed by the Rheumatologists as SLE and LN positive on the basis of ACR and SLEDAI scoring criteria. This study was performed and samples were analyzed in the Department of Medical and Laboratory Sciences, Imperial College of Business Study, Lahore on the patient’s serum by ELISA technique. Result: About 38% (12 patients with LN were positive for anti-dsDNA and 31% (9 SLE patients without LN were positive whereas about 38.7% (12 were anti-dsDNA negative in LN cases and 58.6% (17 in SLE without LN. In case of anti-C1q 100% (31 of these LN patients were positive and 93.1% (27 patients SLE without LN showed positive anti C1q results. Only 6.9% (2 patients showed negative results for anti-C1q in LN negative patients Conclusion: The higher levels of anti-C1q suggest that it may be a better diagnostic marker for LN than that of anti-dsDNA and that it can be helpful in the prognosis of SLE patients.

  2. Outcome of lupus nephritis in childhood onset SLE in North and Central India: single-centre experience over 25 years.

    Science.gov (United States)

    Srivastava, P; Abujam, B; Misra, R; Lawrence, A; Agarwal, V; Aggarwal, A

    2016-04-01

    Childhood SLE (cSLE) has a higher prevalence of lupus nephritis (LN), and there are ethnic variations in response to treatment as well as outcome of LN. There are limited data on long-term outcome of LN in cSLE from the Indian subcontinent. Retrospective analysis of case records of patients with cSLE (satisfying revised American College of Rheumatology (ACR) 1997 criteria for diagnosis) and age of onset Collaborating Clinics (SLICC)/ACR damage score was 0.79 ± 1.13. Actuarial ESRD-free survival at five, 10 and 15 years was 91.1%, 79% and 76.2%, and five-, 10- and 15-year renal survival was 93.8%, 87.1% and 84%, respectively. Although multiple factors individually predicted poor outcome (death/ESRD), only raised serum creatinine at onset (R square = 0.65, p ≤ 0.0001) and damage accrual (R square = 0.62, p ≤ 0.0001) remained significant on multivariate analysis. Eleven (8.2%) children died during the follow-up period, and infections were the leading cause of mortality. Long-term outcome of LN in cSLE in our cohort was better than previous reports from India. However, a high rate of major infection still remains the leading cause of mortality. © The Author(s) 2015.

  3. Chinese SLE Treatment and Research group (CSTAR) registry VII: prevalence and clinical significance of serositis in Chinese patients with systemic lupus erythematosus.

    Science.gov (United States)

    Zhao, J; Bai, W; Zhu, P; Zhang, X; Liu, S; Wu, L; Ma, L; Bi, L; Zuo, X; Sun, L; Huang, C; Tian, X; Li, M; Zhao, Y; Zeng, X

    2016-05-01

    To investigate both the prevalence and clinical characteristics of serositis in Chinese patients with systemic lupus erythematosus (SLE) in a large cohort in the Chinese SLE Treatment and Research group (CSTAR) database. A prospective cross-sectional study of patients with SLE was conducted based on the data from the CSTAR registry. Serositis was defined according to the 1999 revised American College of Rheumatology (ACR) criteria for SLE - that is, pleuritis/pleural effusion and/or pericarditis/pericardial effusion detected by echocardiography, chest X-ray or chest computerized tomography (CT) scan. Peritonitis/peritoneal effusion were confirmed by abdominal ultrasonography. We analysed the prevalence and clinical associations of serositis with demographic data, organ involvements, laboratory findings and SLE disease activity. Of 2104 patients with SLE, 345 were diagnosed with serositis. The prevalence of lupus nephritis (LN), interstitial lung disease and pulmonary arterial hypertension, as well as the presence of leukocytopenia, thrombocytopenia, hypocomplementemia and anti-dsDNA antibodies was significantly higher in patients with serositis (P Lupus-related peritonitis had similar clinical manifestations and laboratory profiles as serositis caused by SLE. There is a significant association of nephropathy, interstitial lung disease, pulmonary arterial hypertension, hypocomplementemia, leukocytopenia, thrombocytopenia and elevated anti-dsDNA antibodies with serositis. The results suggest that higher SLE disease activity contributes to serositis development, and should be treated aggressively. © The Author(s) 2016.

  4. Clinical and immunological aspects and outcome of a Brazilian cohort of 414 patients with systemic lupus erythematosus (SLE): comparison between childhood-onset, adult-onset, and late-onset SLE.

    Science.gov (United States)

    das Chagas Medeiros, M M; Bezerra, M Campos; Braga, F N Holanda Ferreira; da Justa Feijão, M R Melo; Gois, A C Rodrigues; Rebouças, V C do Rosário; de Carvalho, T M Amorim Zaranza; Carvalho, L N Solon; Ribeiro, Át Mendes

    2016-04-01

    The clinical expression of systemic lupus erythematosus (SLE) is influenced by genetic and environmental factors and therefore varies between ethnicities. Information on the epidemiology of SLE in Brazil is scarce and practically limited to studies conducted in socioeconomically developed regions (South and Southeast). The objective of this study was to describe the clinical and immunological aspects and outcome of a cohort of patients with SLE treated at a university hospital in northeastern Brazil and compare patterns related to age at onset: childhood (cSLE), adult (aSLE), and late (lSLE). A random sample of 414 records (women: 93.5%) were reviewed. The mean age at SLE onset and the mean disease duration were 28.9 ± 10.9 years and 10.2 ± 6.6 years, respectively. Most patients had aSLE (n = 338; 81.6%), followed by cSLE (n = 60; 14.5%) and lSLE (n = 16; 3.9%). The female/male ratio was 6.5:1 in cSLE and 16.8:1 in aSLE; in lSLE, all patients were female (p = 0.05). During follow-up, the cSLE group presented higher rates of nephritis (70% vs. 52.9% vs. 12.5%; p = 0.0001) and leuko/lymphopenia (61.7% vs. 43.8% vs. 56.2%; p = 0.02). No significant differences were found for anti-dsDNA, anti-Sm, and antiphospholipid antibodies. Treatment with immunosuppressants was significantly more common, and higher doses of prednisone were used, in cSLE. The prevalence of cardiovascular diseases were more frequent in lSLE (p = 0.03). No significant differences were found between the three groups with regard to mean damage accrual (SDI), remission, and mortality. Although cSLE presented higher rates of nephritis and leuko/lymphopenia, more frequent use of immunosuppressants and higher prednisone doses than aSLE and lSLE, the three groups did not differ significantly with regard to damage accrual, remission, and mortality. © The Author(s) 2015.

  5. Characterization of Novel PI3Kδ Inhibitors as Potential Therapeutics for SLE and Lupus Nephritis in Pre-Clinical Studies.

    Science.gov (United States)

    Haselmayer, Philipp; Camps, Montserrat; Muzerelle, Mathilde; El Bawab, Samer; Waltzinger, Caroline; Bruns, Lisa; Abla, Nada; Polokoff, Mark A; Jond-Necand, Carole; Gaudet, Marilène; Benoit, Audrey; Bertschy Meier, Dominique; Martin, Catherine; Gretener, Denise; Lombardi, Maria Stella; Grenningloh, Roland; Ladel, Christoph; Petersen, Jørgen Søberg; Gaillard, Pascale; Ji, Hong

    2014-01-01

    SLE is a complex autoimmune inflammatory disease characterized by pathogenic autoantibody production as a consequence of uncontrolled T-B cell activity and immune-complex deposition in various organs, including kidney, leading to tissue damage and function loss. There is a high unmet need for better treatment options other than corticosteroids and immunosuppressants. Phosphoinositol-3 kinase δ (PI3Kδ) is a promising target in this respect as it is essential in mediating B- and T-cell function in mouse and human. We report the identification of selective PI3Kδ inhibitors that blocked B-, T-, and plasmacytoid dendritic cell activities in human peripheral blood and in primary cell co-cultures (BioMAP(®)) without detecting signs of undesired toxicity. In an IFNα-accelerated mouse SLE model, our PI3Kδ inhibitors blocked nephritis development, whether administered at the onset of autoantibody appearance or the onset of proteinuria. Disease amelioration correlated with normalized immune cell numbers in the spleen, reduced immune-complex deposition as well as reduced inflammation, fibrosis, and tissue damage in the kidney. Improvements were similar to those achieved with a frequently prescribed drug for lupus nephritis, the potent immunosuppressant mycophenolate mofetil. Finally, we established a pharmacodynamics/pharmacokinetic/efficacy model that revealed that a sustained PI3Kδ inhibition of 50% is sufficient to achieve full efficacy in our disease model. These data demonstrate the therapeutic potential of PI3Kδ inhibitors in SLE and lupus nephritis.

  6. Diagnostic significance of the radiologic appearances of the hands in systemic lupus erythematosus (SLE)

    Energy Technology Data Exchange (ETDEWEB)

    Winkler, P; Baenkler, H W; Pfuhl, E; Gutmann, W

    1980-09-01

    40 patients with SLE were assessed, the diagnoses being made according to modified ARA-criteria; the hand films of 25 of these patients were analysed. The most important roentgenologic findings consisted of reducible deformities with mild or absent articular changes in the deformed MCP and PIP joints. As these changes often occur late in the course of SLE, a primary radiological diagnosis was possible in only three cases (8%), whereas hand films provided confirmatory evidence of disease in a further seven patients. These roentgenological characteristics of SLE were found significantly less often in patients with definite RA; this group tended to have positive ANF (antinuclear factors). Antibodies against DNA may be absent, especially in a less active phase of disease. ANF is non-specific, and SLE simulates RA in 10-20% of cases. It is, therefore, very important in individual cases to consider the diagnosis of SLE in the presence of deformity without corresponding articular changes. Direct comparison of both the p.a. and oblique views is essential in the evaluation of reducible deformities, although this point has not been stressed in the literature. Reducible deformities may be overlooked in physical examination, when the patient gives no history of functional disability. The main differential diagnosis is Jaccoud's arthropathy, which is rarely seen in a general hospital. Most of these patients give a history of recurrent rheumatic fever, a minority complain about joint pain or disability, approximately 90% show signs indicating a valvular lesion of the heart, over 75% have a normal ESR and a positive rheumatoid or antinuclear factor is very rarely found. The distinction from SLE or rheumatoid arthritis is therefore easily established on additional non-radiological criteria.

  7. The diagnostic significance of the radiologic appearances of the hands in systemic lupus erythematosus (SLE)

    International Nuclear Information System (INIS)

    Winkler, P.; Baenkler, H.W.; Pfuhl, E.; Gutmann, W.

    1980-01-01

    40 patients with SLE were assessed, the diagnoses being made according to modified ARA-criteria; the hand films of 25 of these patients were analysed. The most important roentgenologic findings consisted of reducible deformities with mild or absent articular changes in the deformed MCP and PIP joints. As these changes often occur late in the course of SLE, a primary radiological diagnosis was possible in only three cases (8%), whereas hand films provided confirmatory evidence of disease in a further seven patients. These roentgenological characteristics of SLE were found significantly less often in patients with definite RA; this group tended to have positive ANF (antinuclear factors). Antibodies against DNA may be absent, especially in a less active phase of disease. ANF is non-specific, and SLE simulates RA in 10-20% of cases. It is, therefore, very important in individual cases to consider the diagnosis of SLE in the presence of deformity without corresponding articular changes. Direct comparison of both the p.a. and oblique views is essential in the evaluation of reducible deformities, although this point has not been stressed in the literature. Reducible deformities may be overlooked in physical examination, when the patient gives no history of functional disability. The main differential diagnosis is Jaccoud's arthropathy, which is rarely seen in a general hospital. Most of these patients give a history of recurrent rheumatic fever, a minority complain about joint pain or disability, approximately 90% show signs indicating a valvular lesion of the heart, over 75% have a normal ESR and a positive rheumatoid or antinuclear factor is very rarely found. The distinction from SLE or rheumatoid arthritis is therefore easily established on additional non-radiological criteria. (orig./MG) [de

  8. Localization of radiolabeled anti-DNA monoclonal antibodies in murine systemic lupus erythematosus (SLE)

    International Nuclear Information System (INIS)

    Wahl, R.; Hahn, B.; Ebling, F.

    1984-01-01

    The diagnosis of SLE can be extremely difficult. This multi-system disease is characterized by the deposition of DNA-anti-DNA antibody (Ab) complexes in many tissues, producing glomerulonephritis and systemic vasculitis. This study evaluates an IGG monoclonal (Mo) Ab directe3d against DNA (MrSSl) for potential radioimmunodiagnosis of SLE. Six 15 wk. old F-1 female hybrids of NZB+NZW mice (an animal SLE model that develops vasculitis and nephritis) were injected with 50 μCl of I-131 MrSSl and 15 μCl of I-125 isotype-matched control mouse myeloma (LPC-1) (non-reactive with DNA). Imaging and tissue distribution were studied. Two animals were also imaged using I-131 LPC Ab. Images at 2 and 9 days showed no clear differences in scan patterns using MrSSl or LPC-1 Ab. Tissue distribution studies at six days, however, showed a significantly higher accumulation of MrSSl in the kidneys vs. control Ab (2.7% vs. 1.8% of injected dose) (p < .04). Similarly, higher levels of MrSS were also seen in the spleen, liver and lungs (p < .03). Blood levels tended to be higher with the specific antibody as well. These differences were not apparent at 3 days post injection. The increased concentration of MrSSl present at 9 days in several organs may be secondary to MrSSl binding to DNA containing immune complexes present in diseased tissues. Blocked clearance by immune complexes or DNA, or differences in electrical charges of the antibodies could be contributing to the higher MrSSl levels seen. Images did not suggest deiodination as responsible. Further studies are necessary to determine if the amount of MrSSl retained by diseased animals is indicative of SLE disease activity

  9. Evaluation and treatment of acute psychosis in children with Systemic Lupus Erythematosus (SLE): consultation-liaison service experiences at a tertiary-care pediatric institution.

    Science.gov (United States)

    Muscal, Eyal; Nadeem, Tania; Li, Xiofan; Mian, Ayesha; Harris, Toi Blakley

    2010-01-01

    Neurological and psychiatric manifestations of systemic lupus erythematosus (SLE) are prevalent in children with SLE. There are few data on the evaluation and management of psychotic features in children with this systemic autoimmune disorder. The authors describe contemporary Child and Adolescent Psychiatry Consultation and Liaison service management of acute psychosis in children with lupus. The authors reviewed the records (2003-2008) of all pediatric SLE inpatients who were administered a traditional or atypical antipsychotic agent. They describe clinical features, initial and discharge mental status examinations, and inpatient psychotropic medication usage. Ten pediatric SLE patients (age 10-19 years) required psychiatric management for psychosis during the review period. Paranoid delusions (70%), visual hallucinations (60%), and auditory hallucinations (60%) were the most common psychotic symptoms documented. All children were initially treated with an antipsychotic medication. Seven children were maintained on an atypical antipsychotic during their hospitalization. Two children had extrapyramidal signs, but no other adverse events were documented. All children were improved at discharge, and 40% had complete resolution of psychosis; 8 of the 10 patients were discharged on a psychotropic medication. Psychotic manifestations associated with severe disease presentations were successfully treated by child psychiatrists. Atypical antipsychotics were well-tolerated and used as an adjunct to immunosuppressive regimens in these patients. Prospective studies are necessary to improve the care of children and adolescents with SLE and severe psychiatric manifestations.

  10. [Depressive disorder in Mexican pediatric patients with systemic lupus erythematosus (SLE)].

    Science.gov (United States)

    Carbajal-Alonso, Hilda Lilian; García-Moreno, Norberta Prisilia; Rodríguez-Arreola, Brenda; Barrera de León, Juan Carlos

    2016-01-01

    To identify the prevalence of depression in Mexican pediatric patients with systemic lupus erythematosus. Analytical transversal study including patients aged 7-16 years with a diagnosis of systemic lupus erythematosus seen at the Pediatric Rheumatology Consultation Service. The disease was classified by means of the MEX-SLEDAI questionnaire. Descriptive statistics with central tendency and dispersion and comparative measurements with chi-squared and Mann-Whitney U tests. Logistic regression and association with odds ratios. SPSS v.21.0 statistical software package. We evaluated 45 patients who presented depression, n=9 (20%), including eight females (89%) and one male (11%), median age 13 years (range, 7-16) in children with depression vs. 13 years (range, 9-14) p=0.941, depression more frequent in schoolchildren. Habitual residence, disease evolution time, and duration of the immunosuppressor did not show a significant difference between both groups. Divorced parents p=0.037. Neuropsychiatric manifestations of lupus presented in 2.2% of all patients and in 100% of patients with depression. Disease activity index (MEX-SLEDAI) did not demonstrate a relationship with the presence of depression. Prevalences in pediatric populations are less that that reported in adults, association with disease activity, evolution time, and immunosuppressor use and duration not found.

  11. Course of Neuropsychiatric Symptoms during Flares of Systemic Lupus Erythematosus (SLE

    Directory of Open Access Journals (Sweden)

    Gareth Garrett

    2017-01-01

    Full Text Available We present the case of a seventeen-year-old girl who presents with an interesting course of neuropsychiatric symptoms during several flares of SLE. The patient was diagnosed at the age of thirteen and has had four flares in total. The latter two flares included cutaneous and neuropsychiatric symptoms. The most recent flare occurred when she was aged seventeen. She had cutaneous symptoms which coincided with an episode of hypomania. Her mental state further deteriorated following steroid treatment. She exhibited affective and psychotic symptoms. Treatment with cyclophosphamide and olanzapine was associated with an improvement in both cutaneous and neuropsychiatric symptoms. Previously aged sixteen the patient had presented with cutaneous symptoms and a moderate depressive episode which was also exacerbated by steroid treatment. The patient’s mood improved when the dose of oral steroids was reduced to a daily dose of 15–20 mg prednisolone.

  12. The combination of two Sle2 lupus-susceptibility loci and Cdkn2c deficiency leads to T cell-mediated pathology in B6.Faslpr mice

    Science.gov (United States)

    Xu, Zhiwei; Croker, Byron P.; Morel, Laurence

    2013-01-01

    The NZM2410 Sle2c1 lupus susceptibility locus is responsible for the expansion of the B1a cell compartment and for the induction of T-cell induced renal and skin pathology on a CD95 deficient (Faslpr)-background. We have previously shown that deficiency in cyclin-dependent kinase inhibitor p18INK4c (p18) was responsible for the B1a cell expansion but was not sufficient to account for the pathology in B6.lpr mice. This study was designed to map the additional Sle2c1 loci responsible for autoimmune pathology when co-expressed with CD95 deficiency. The production, fine-mapping and phenotypic characterization of five recombinant intervals indicated that three interacting sub-loci were responsive for inducting autoimmune pathogenesis in B6.lpr mice. One of these sub-loci corresponds most likely to p18-deficiency. Another major locus mapping to a 2 Mb region at the telomeric end of Sle2c1 is necessary to both renal and skin pathology. Finally, a third locus centromeric to p18 enhances the severity of lupus nephritis. These results provide new insights into the genetic interactions leading to SLE disease presentation, and represent a major step towards the identification of novel susceptibility genes involved in T-cell mediated organ damage. PMID:23698709

  13. Preeclampsia in pregnancies complicated by systemic lupus erythematosus (SLE) nephritis: prophylactic treatment with multidisciplinary approach are important keys to prevent adverse obstetric outcomes.

    Science.gov (United States)

    Mecacci, Federico; Simeone, Serena; Cirami, Calogero Lino; Cozzolino, Mauro; Serena, Caterina; Rambaldi, Marianna Pina; Gallo, Pamela; Emmi, Lorenzo; Cammelli, Daniele; Mello, Giorgio; Matucci Cerinic, Marco

    2017-11-27

    Systemic lupus erythematosus (SLE) commonly affects women of childbearing age. Hypertension, antiphospholipid syndrome, and lupus nephritis are risk factors for adverse maternal/fetal outcome. The aim of this retrospective cohort study is to compare pregnancy outcomes in patients with and without SLE nephritis, using a multidisciplinary approach and a broad prophylaxis protocol. Data were collected from 86 pregnancies complicated by SLE. Twenty-seven women with nephropathy before pregnancy stated as the study group and 59 formed the control group. Each group received a prophylactic treatment based on their clinical characteristics. Results were expressed as mean ± SD, percentage and χ 2 -test (significant values when p 1.2 mg/dL, which was related to a risk 1.25 times higher than the risk observed in patients with serum creatinine approach in a tertiary care center and a broad prophylactic treatment protocol to patients affected by SLE and complicated by nephritis may definitively foster a successful pregnancy.

  14. The Sarawak lupus cohort: clinical features and disease patterns of 633 SLE patients in a single tertiary centre from East Malaysia.

    Science.gov (United States)

    Teh, C L; Ling, G R; Aishah, Wan Sharifah

    2015-01-01

    Systemic lupus erythematosus (SLE) has been well studied in West Malaysian populations but lacking in East Malaysian populations. The aim of this study was to examine the clinical features and disease patterns of patients with SLE in a multiethnic East Malaysian population in Sarawak. All SLE patients who were treated in Sarawak General Hospital were reviewed in a retrospective longitudinal study using a standard protocol from 1 January 2006 to 31 December 2013. There were a total of 633 patients in our study with the female to male ratio of 12:1. Our study patients were of multiethnic origins with predominant Chinese ethnic group. They had a mean age of 36.9 ± 13.2 years and a mean duration of illness of 7.2 ± 6.0 years. The main involvements were haematological (74.2 %), malar rash (64.0 %) and renal (58.6 %). Chinese patients were less likely to have discoid lupus, pleuritis and pericarditis, while Malay patients were more likely to have arthritis. Bidayuh patients were more likely to have oral ulcer. Secondary antiphospholipid syndrome was more common in Chinese. The majority of patients were in clinical remission with low SDI. There were 58 deaths (9.2 %) during 2006-2013 with the main causes of death being flare of disease and infection.

  15. Population differences in SLE susceptibility genes: STAT4 and BLK, but not PXK, are associated with systemic lupus erythematosus in Hong Kong Chinese.

    Science.gov (United States)

    Yang, W; Ng, P; Zhao, M; Hirankarn, N; Lau, C S; Mok, C C; Chan, T M; Wong, R W S; Lee, K W; Mok, M Y; Wong, S N; Avihingsanon, Y; Lee, T L; Ho, M H K; Lee, P P W; Wong, W H S; Lau, Y L

    2009-04-01

    In this study, we compared the association of several newly discovered susceptibility genes for systemic lupus erythematosus (SLE) between populations of European origin and two Asian populations. Using 910 SLE patients and 1440 healthy controls from Chinese living in Hong Kong, and 278 SLE patients and 383 controls in Thailand, we studied association of STAT4, BLK and PXK with the disease. Our data confirmed association of STAT4 (rs7574865, odds ratio (OR) =1.71, P=3.55 x 10(-23)) and BLK (rs13277113, OR=0.77, P=1.34 x 10(-5)) with SLE. It was showed that rs7574865 of STAT4 is also linked to hematologic disorders and potentially some other subphenotypes of the disease. More than one genetic variant in STAT4 were found to be associated with the disease independently in our populations (rs7601754, OR=0.59, P=1.39 x 10(-9), and P=0.00034 when controlling the effect of rs7574865). With the same set of samples, however, our study did not detect any significant disease association for PXK, a risk factor for populations of European origin (rs6445975, joint P=0.36, OR=1.06, 95% confidence interval: 0.93-1.21). Our study indicates that some of the susceptibility genes for this disease may be population specific.

  16. "We Would Still Find Things to Talk About": Assessment of Mentor Perspectives in a Systemic Lupus Erythematosus Intervention to Improve Disease Self-Management, Empowering SLE Patients.

    Science.gov (United States)

    Flournoy-Floyd, Minnjuan; Ortiz, Kasim; Egede, Leonard; Oates, Jim C; Faith, Trevor D; Williams, Edith M

    2018-04-01

    Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disorder with significant disparate impact on African American women. The current study sought to highlight how the Peer Approaches to Lupus Self-management (PALS) intervention worked bi-directionally wherein both women with SLE leading the disease self-management program (mentors), and those participants who served as mentees, were empowered toward greater disease self-efficacy. Data was captured for this study in two formats from the seven mentors participating in the pilot study: 1) mentor logs and 2) mentor interviews with the principle investigator. This information was then analyzed for themes relating to their experience within the study. We found that empowerment was facilitated by mentors taking their mentorship responsibilities seriously and seeking several avenues for collaboratively developing success with their mentees. Mentors reported that although challenges arose, their desire for success resulted in multiple approaches to be flexible and responsive to the needs of their mentees. Additionally, reciprocity was found to be a vital element of the program. Key thematic areas supported our ability to demonstrate the usefulness of a peer mentoring program for SLE disease self-management on evoking empowerment through reciprocal relationships among mentors and mentees within our study population. Furthermore the feedback from PALS participants yielded very rich and contextual information that can be used as a thematic guide for developing and refining evidence-based interventions that seek to incorporate empowerment into disease self-management efforts for women suffering from SLE. Copyright © 2018 National Medical Association. Published by Elsevier Inc. All rights reserved.

  17. A case of brain SLE: MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Myung Soon; Kim, Seung Min [Wonju College of Medicine, Yonsei University, Wonju (Korea, Republic of)

    1992-01-15

    Systemic lupus erythematosus(SLE) is an autoimmune disease characterized by multisystem involvement including central nervous system and various neurologic symptoms. The authors experienced a case of brain SLE and report MRI and other neuroimaging findings.

  18. Complement receptor expression and activation of the complement cascade on B lymphocytes from patients with systemic lupus erythematosus (SLE)

    DEFF Research Database (Denmark)

    Marquart, H V; Svendsen, A; Rasmussen, J M

    1995-01-01

    It has previously been reported that the expression of the complement receptors, CR1 on erythrocytes and blood leucocytes and CR2 on B cells, is reduced in patients with SLE, and that the reduced expression of CR1 on erythrocytes is related to disease activity. We have earlier demonstrated...... that normal B cells are capable of activating the alternative pathway (AP) of complement in a CR2-dependent fashion. In this study we have investigated whether disturbances in this activity may be related to the altered phenotype of SLE B cells. Flow cytometry was used to measure expression of complement...

  19. 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 ...

  20. The effect of Ramadan fasting on quiescent systemic lupus erythematosus (SLE) patients' disease activity, health quality of life and lipid profile: a pilot study.

    Science.gov (United States)

    Goharifar, Hamid; Faezi, Seyedeh Tahereh; Paragomi, Pedram; Montazeri, Ali; Banihashemi, Arash Tehrani; Akhlaghkhah, Maryam; Abdollahi, Bahar Sadeghi; Kamazani, Zahra; Akbarian, Mahmood

    2015-08-01

    SLE is a common autoimmune disease with considerable morbidity. Ramadan fasting is a religious custom Muslims regularly practice. We aimed to evaluate the effect of Ramadan fasting on SLE patients' disease activity, health quality of life and lipid profile. We conducted this case control study as a pilot study in 40 quiescent SLE patients, 21 cases who decided to fast and 19 controls who decided not to have Ramadan fasting between August and November 2009 in lupus unit of Rheumatology Research Center in Tehran University of Medical Sciences, Iran. They were assessed for SLE Disease Activity Index, lipid profile and quality of life with Short-Form 36 (SF-36) Health Survey, 1 day before Ramadan, the day after and 3 months after Ramadan fasting. After 24.1 ± 5.4 (mean ± SD) days of fasting, anti-ds DNA increased for 0.34 ± 0.41 mmol/dL in cases versus 0.07 ± 0.31 in controls (P = 0.026). Likewise C3 increased more dramatically in cases (16.8 ± 17.5 vs. 2.3 ± 13.2 mg/dL, P = 0.006). Three months after fasting, anti-ds DNA was still increased 0.28 ± 0.46 mmol/dL in cases while a 0.02 ± 0.43 mmol/dL drop in controls was detected (P = 0.04). On the contrary, C3 returned to baseline. These changes were not accompanied with significant changes in disease activity and health quality of life. Ramadan fasting had no effect on lipid profile except for delayed total cholesterol decrease in cases in comparison with controls (16.4 ± 29.4 decrease vs. 4.6 ± 23.9 mg/dL decrease, P = 0.018). Ramadan fasting probably has no detrimental effect on SLE patients' disease activity and their quality of life in the quiescent phase of disease.

  1. Fas expression on peripheral blood lymphocytes in systemic lupus erythematosus (SLE) : relation to lymphocyte activation and disease activity

    NARCIS (Netherlands)

    Bijl, M; Horst, G; Limburg, PC; Kallenberg, CGM

    2001-01-01

    Levels of apoptotic lymphocytes have been found to be increased in SLE and persistence of apoptotic cells has been associated with autoantibody production, Increased lymphocyte Fas (CD95) expression due to lymphocyte activation may account for increased Susceptibility to Fas-mediated apoptosis in

  2. Influence of molecular weight of DNA on the determination of anti-DNA antibodies in systemic lupus erythematosus (SLE) sera by radioimmunoassay

    Energy Technology Data Exchange (ETDEWEB)

    Geisert, M; Heicke, B; Metzmann, E; Zahn, R K

    1975-04-01

    Using a radioimmunoassay (RIA) based on the Farr technique with radioactively labeled /sup 3/H-DNA for quantitative measurements of anti-DNA antibodies in sera of patients with systemic lupus erythematosus (SLE), the influence of molecular weight of DNA (ranging from 0.1 x 10/sup 6/ to 22.0 x 10/sup 6/ daltons) on binding and precipitation in this system has been investigated. Comparing our results with mathematical models it follows that one antibody molecule is fixed on the average to a statistical DNA segment of 2 x 10/sup 6/ to 4 x 10/sup 6/ daltons. Furthermore binding capacity of the DNA was found to be independent of the molecular weight, as demonstrated in a double label experiment using /sup 14/C and /sup 3/H-labeled DNA of different size. However, the amount of radioactivity precipitated was found to depend on the molecular weight of the labeled DNA following a non-linear function. It was calculated that a minimal ratio of fixed antibody molecules per a certain size of DNA was necessary for precipitation. The mathematical treatment of the observed non-linear precipitation dependence will be discussed using various statistical models. The results indicate that the quantitative measurements of anti-DNA antibodies with the Farr technique e.g., for diagnosis and control of SLE in clinical immunology is highly dependent on the molecular weight of the labeled DNA used in the assay system and reliable results are only obtained with DNA of a sufficiently high molecular weight. (auth)

  3. 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...

  4. 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....

  5. Genetics Home Reference: systemic lupus erythematosus

    Science.gov (United States)

    ... Twitter Home Health Conditions Systemic lupus erythematosus Systemic lupus erythematosus Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Systemic lupus erythematosus (SLE) is a chronic disease that causes inflammation ...

  6. Association between paraoxonase-1 gene Q192R and L55M polymorphisms in systemic lupus erythematosus (SLE) and anti-phospholipid syndrome (APS) in a population from Cairo of Egypt.

    Science.gov (United States)

    Ibrahim, Alshaymaa Ahmed; El-Lebedy, Dalia; Ashmawy, Ingy; Hady, Maha Abdel

    2017-06-01

    Paraoxonase-1 (PON1) is involved in the oxidative stress process that cause tissue damage observed in systemic lupus erythematosus (SLE) and anti-phospholipid syndrome (APS). The aim of the present study was to investigate the association of PON1 Q192R and L55M polymorphisms with risk of SLE and associated APS among Egyptian sample. The study included 120 SLE patients (45 without APS and 75 with APS) and 120 healthy subjects. PON1 Q192R and L55M polymorphisms were genotyped by real-time PCR. No significant differences in Q192R genotypes or allele frequencies were found between patients and controls (p = 0.5 and 0.1, respectively). The frequency of the 55M allele was significantly higher in SLE patients than in controls (66.6 vs. 43.3%), while the 55L allele was more frequent in controls (56.6%) than in patients (33.3%) (p = 0.03). The LL genotype was more frequent in controls (21.6%) than in patients (10%) while M allele carrier genotypes (LM + MM) were more frequent among patients (90%) than controls (78.3%), p = 0.04. Also, the 55M allele was more frequent in APS patients (73.3%) than in patients without APS (55.6%), p = 0.004. M allele carrier genotypes (LM + MM) was significantly higher among APS patients (95.4%) than in non-APS patients (80%), p = 0.008. Our results indicated that the PON1 L55M polymorphism associated with SLE and associated APS in a population from Cairo of Egypt, while the Q192R polymorphism plays no role in disease susceptibility. A large scale study to assess PON1 polymorphisms, PON1 activity, and markers of oxidative stress interaction is needed to clarify the role of PON-1 polymorphisms in the pathogenesis of SLE and associated APS.

  7. Chinese SLE Treatment and Research Group Registry: III. Association of Autoantibodies with Clinical Manifestations in Chinese Patients with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Jing Li

    2014-01-01

    Full Text Available We investigated the characteristics of Chinese SLE patients by analyzing the association between specific autoantibodies and clinical manifestations of 2104 SLE patients from registry data of CSTAR cohort. Significant (P<0.05 associations were found between anti-Sm antibody, anti-rRNP antibody, and malar rash; between anti-RNP antibody, anti-SSA antibody, and pulmonary arterial hypertension (PAH; between anti-SSB antibody and hematologic involvement; and between anti-dsDNA antibody and nephropathy. APL antibody was associated with hematologic involvement, interstitial lung disease, and a lower prevalence of oral ulcerations (P<0.05. Associations were also found between anti-dsDNA antibody and a lower prevalence of photosensitivity, and between anti-SSA antibody and a lower prevalence of nephropathy (P<0.05. Most of these findings were consistent with other studies in the literature but this study is the first report on the association between anti-SSA and a lower prevalence of nephropathy. The correlations of specific autoantibodies and clinical manifestations could provide clues for physicians to predict organ damages in SLE patients. We suggest that a thorough screening of autoantibodies should be carried out when the diagnosis of SLE is established, and repeated echocardiography annually in SLE patients with anti-RNP or anti-SSA antibody should be performed.

  8. Haemorrhagic SLE In A Young Male

    Directory of Open Access Journals (Sweden)

    Rajagopal R

    2002-01-01

    Full Text Available Systemic lupus erythematous (SLE is a systemic autoimmune disease that tends to occur in early adult life. The peak age of onset of the first symptom or sign in females is about 38 years and later in men, at about 44 years. Females outnumber men in this illness in a ratio of about 8 : 1. Cutaneous lesions in male have not been properly investigated and some studies in male with SLE have shown that the illness may present with atypical skin lesions. A case of SLE in a 20 year male who developed sudden onset of haemorrhagic vesiculobullous butterfly rash is described.

  9. 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)....

  10. THE CAROLINA LUPUS STUDY (CLU)

    Science.gov (United States)

    Carolina Lupus (CLU) Study, an epidemiologic study of risk factors for systemic lupus erythematosus (SLE). SLE is a severe, chronic, systemic autoimmune disease that disproportionately affects women and African-Americans. The CLU Study focuses on measures of endogenous hormone ex...

  11. Prognostic factors in lupus nephritis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Starklint, Henrik; Halberg, Poul

    2006-01-01

    To evaluate the prognostic significance of clinical and renal biopsy findings in an unselected cohort of patients with systemic lupus erythematosus (SLE) and nephritis.......To evaluate the prognostic significance of clinical and renal biopsy findings in an unselected cohort of patients with systemic lupus erythematosus (SLE) and nephritis....

  12. A framework for remission in SLE

    DEFF Research Database (Denmark)

    van Vollenhoven, Ronald; Voskuyl, Alexandre; Bertsias, George

    2017-01-01

    , for example, clinical systemic lupus erythematosus disease activity index (SLEDAI)=0, British Isles lupus assessment group (BILAG) 2004 D/E only, clinical European consensus lupus outcome measure (ECLAM)=0; with routine laboratory assessments included, and supplemented with physician's global assessment.3....... Distinction is made between remission off and on therapy: remission off therapy requires the patient to be on no other treatment for SLE than maintenance antimalarials; and remission on therapy allows patients to be on stable maintenance antimalarials, low-dose corticosteroids (prednisone ≤5 mg...

  13. Protective Role of Ets1 in SLE

    Science.gov (United States)

    2014-07-01

    Purification and differentiation of Th cells were performed according to a published protocol without modi - fication (3). Preparation of Cytoplasmic...RA), systemic lupus erythematosus (SLE), and type 1 diabetes but reduces the risk of Crohn’s disease [7,14-17]. Despite these observations, it is

  14. Auricular Chondritis in a Postpartum Flare of SLE and APS

    Directory of Open Access Journals (Sweden)

    Elodie Ponce

    2014-10-01

    Full Text Available Introduction: Auricular chondritis has been occasionally described in patients with systemic lupus erythematosus (SLE and antiphospholipid syndrome (APS. Materials and methods: We report the case of a woman with a previous history of APS who presented with auricular chondritis with onset of SLE symptoms during the postpartum period. Conclusion: SLE and APS should be taken into consideration in the differential diagnosis of auricular chondritis.

  15. 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....

  16. 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....

  17. 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

    2018-04-01

    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.

  18. Research Progress on Systemic Lupus Erythematosus Complicated with Infection

    Directory of Open Access Journals (Sweden)

    Zhang Weisan

    2015-06-01

    Full Text Available In recent years, in treatment standardization of systemic lupus erythematosus (SLE, infections and serious complications became the leading cause of death related to this disease, exceeding those of renal involvement and lupus encephalopathy. SLE coinfection is mainly related to defects in humoral immunity and cellular immunity, SLE disease activity, and doses of hormone and immune inhibitors.

  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. Pain and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    M. Di Franco

    2014-06-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease characterized by heterogeneous clinical manifestations involving virtually the entire body. The pain in SLE can have different causes. The SLE classification criteria include mainly the musculoskeletal manifestations of pain, which are commonly reported as initial symptoms of SLE, such as arthralgia, arthritis and/or myalgia. Chronic widespread pain, which is typical of fibromyalgia (FM, is frequently associated with SLE. The aim of this review is to describe widespread pain and fatigue in SLE, and the association of SLE and FM. Although secondary FM is not correlated with the disease activity, it may interfere with the daily activities of SLE patients. Therefore it is necessary to identify its symptoms and treat them promptly to improve the quality of life of patients. In conclusion, it is essential to identify the origin of pain in SLE in order to avoid dangerous over-treatment in patients with co-existing widespread pain and FM.

  1. 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...

  2. 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...

  3. Graviditetskomplikationer hos en patient med systemisk lupus erythematosus og lupus nefritis

    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...

  4. Estudo retrospectivo de 76 fetos de mães com lúpus eritematoso sistêmico (LES Retrospective study of 76 fetus of mother with systemic lupus erythematosus (SLE

    Directory of Open Access Journals (Sweden)

    Marcela Ushida

    2004-10-01

    prevalência de perda fetal (aborto e óbito intra-uterino, entretanto, a freqüência de prematuridade foi maior que a média referida em outros trabalhos. Manifestações hematológicas do lúpus neonatal, como leucopenia e plaquetopenia foram observadas em freqüência maior do que a referida na literatura. Por serem transitórias e assintomáticas, estas alterações podem ter sido subdiagnosticadas.OBJECTIVE: to evaluate the frequency of neonatal lupus, prematurity, fetal loss, and low weight in pregnancies of SLE patients attended at a tertiary health service. MEHTODS: it is a retrospective study evaluating all SLE patients attended at obstetric center of Hospital São Paulo/Unifesp/EPM from November, 1991 to April, 2003. The mother and children's clinical and laboratory data were obtained reviewing medical records. RESULTS: sixty women and 75 pregnancies were identified. The mother age average during the pregnancies was 27.1±6.1 years old and the median of disease duration was 48 months. Two patients needed to be submitted to dialysis during the pregnancy. Sixty-three patients used prednisone, 4 received methilprednisolone pulsetherapy and 2 received azathioprine during the pregnancy. During the first trimester of pregnancy, 6 patients received diphosphate chloroquine and 2 received hidroxychloroquine. Two patients unknowing pregnancy received pulse of cyclophosphamide and another one had used methotrexate and both presented spontaneous abortion. It was observed 13 cases of intrauterine death and 7 abortions. Preterm birth occurred in 57% of pregnancies and the average duration of gestation was 35 weeks. The mean weight of the newborns was 2,332±961g (ranging from 525 to 3,620g. Five cases of neonatal lupus were identified (8,9%. One with congenital heart block (CHB had intrauterine death at the 29th week of gestation, had 3 babies with thrombocytopenia associated with anti-Ro/SSA antibodies and one with neutropenia associated with anti-RNP antibody. Excepting

  5. 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...

  6. Systemisk lupus erythematosus og graviditet

    DEFF Research Database (Denmark)

    Schreiber, Karen; Lykke, Jacob Alexander; Nielsen, Henriette Svarre

    2016-01-01

    Systemic lupus erythematosus (SLE) is a complex autoimmune disease which most often affects women of childbearing age. Pregnancy is therefore an important issue for the patient and the responsible physician. Pregnancy outcomes in women with SLE has improved significantly over the latest decades...

  7. A framework for remission in SLE : Consensus findings from a large international task force on definitions of remission in SLE (DORIS)

    NARCIS (Netherlands)

    van Vollenhoven, Ronald F.; Voskuyl, Alexandre E.; Bertsias, George K.; Aranow, Cynthia; Aringer, Martin; Arnaud, Laurent; Askanase, Anca; Balazova, Petra; Bonfa, Eloisa; Bootsma, Hendrika; Boumpas, Dimitrios T.; Bruce, Ian N.; Cervera, Ricard; Clarke, Ann; Coney, Cindy; Costedoat-Chalumeau, Nathalie; Czirjak, Laszlo; Derksen, Ronald; Doria, Andrea; Doerner, Thomas; Fischer-Betz, Rebecca; Fritsch-Stork, Ruth; Gordon, Caroline; Graninger, Winfried; Gyori, Noemi; Houssiau, Frederic A.; Isenberg, David A.; Jacobsen, Soren; Jayne, David; Kuhn, Annegret; Le Guern, Veronique; Lerstrom, Kirsten; Levy, Roger; Machado-Ribeiro, Francinne; Mariette, Xavier; Missaykeh, Jamil; Morand, Eric; Mosca, Marta; Inanc, Murat; Navarra, Sandra; Neumann, Irmgard; Olesinska, Marzena; Petri, Michelle; Rahman, Anisur; Rekvig, Ole Petter; Rovensky, Jozef; Shoenfeld, Yehuda; Smolen, Josef S.; Tincani, Angela; Urowitz, Murray; van Leeuw, Bernadette; Vasconcelos, Carlos; Voss, Anne; Werth, Victoria P.; Zakharova, Helena; Zoma, Asad; Schneider, Matthias; Ward, Michael

    Objectives Treat-to-target recommendations have identified `remission' as a target in systemic lupus erythematosus (SLE), but recognise that there is no universally accepted definition for this. Therefore, we initiated a process to achieve consensus on potential definitions for remission in SLE.

  8. 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

  9. Systemic Lupus Erythematosus (Lupus)

    Science.gov (United States)

    ... Lupus) English Español 繁體中文 한국어 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, ...

  10. 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....

  11. Kutan lupus erythematosus

    DEFF Research Database (Denmark)

    Sandreva, Tatjana; Voss, Anne; Bygum, Anette

    2016-01-01

    Cutaneous lupus erythematosus (LE) is an autoimmune disease. The most common clinical forms are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE) and discoid LE (DLE). Cutaneous LE, mainly ACLE, can be the first sign of systemic LE (SLE). DLE and SCLE are less associated with development of...... hydroxychloroquine....

  12. Kutan lupus erythematosus

    DEFF Research Database (Denmark)

    Sandreva, Tatjana; Voss, Anne; Bygum, Anette

    2016-01-01

    Cutaneous lupus erythematosus (LE) is an autoimmune disease. The most common clinical forms are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE) and discoid LE (DLE). Cutaneous LE, mainly ACLE, can be the first sign of systemic LE (SLE). DLE and SCLE are less associated with development...

  13. Gastrointestinal system involvement in systemic lupus erythematosus.

    Science.gov (United States)

    Li, Z; Xu, D; Wang, Z; Wang, Y; Zhang, S; Li, M; Zeng, X

    2017-10-01

    Systemic lupus erythematosus (SLE) is a multisystem disorder which can affect the gastrointestinal (GI) system. Although GI symptoms can manifest in 50% of patients with SLE, these have barely been reviewed due to difficulty in identifying different causes. This study aims to clarify clinical characteristics, diagnosis and treatment of the four major SLE-related GI system complications: protein-losing enteropathy (PLE), intestinal pseudo-obstruction (IPO), hepatic involvement and pancreatitis. It is a systematic review using MEDLINE and EMBASE databases and the major search terms were SLE, PLE, IPO, hepatitis and pancreatitis. A total of 125 articles were chosen for our study. SLE-related PLE was characterized by edema and hypoalbuminemia, with Technetium 99m labeled human albumin scintigraphy ( 99m Tc HAS) and alpha-1-antitrypsin fecal clearance test commonly used as diagnostic test. The most common site of protein leakage was the small intestine and the least common site was the stomach. More than half of SLE-related IPO patients had ureterohydronephrosis, and sometimes they manifested as interstitial cystitis and hepatobiliary dilatation. Lupus hepatitis and SLE accompanied by autoimmune hepatitis (SLE-AIH overlap) shared similar clinical manifestations but had different autoantibodies and histopathological features, and positive anti-ribosome P antibody highly indicated the diagnosis of lupus hepatitis. Lupus pancreatitis was usually accompanied by high SLE activity with a relatively high mortality rate. Early diagnosis and timely intervention were crucial, and administration of corticosteroids and immunosuppressants was effective for most of the patients.

  14. Haematological manifestations of lupus

    Science.gov (United States)

    Fayyaz, Anum; Igoe, Ann; Kurien, Biji T; Danda, Debashish; James, Judith A; Stafford, Haraldine A; Scofield, R Hal

    2015-01-01

    Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. While related to disease activity, there is no specific therapy for lymphopenia. Severe lymphopenia may require the use of prophylactic therapy to prevent select opportunistic infections. Isolated idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation, haemolytic anaemia, the antiphospholipid syndrome and renal disease. Thrombocytopenia can be regarded as an important prognostic indicator of survival in patients with SLE. Medical, surgical and biological treatment modalities are reviewed for this manifestation. First-line therapy remains glucocorticoids. Through our review, we conclude glucocorticoids do produce a response in majority of patients initially, but sustained response to therapy is unlikely. Glucocorticoids are used as first-line therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line therapy. Rituximab is promising in refractory and non-responding AIHA. TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of TTP and SLE, and a few patients with SLE have concurrent

  15. [Side effects of the HMG-CoA reductase inhibitors (statins). Lupus erythematosus induced by Atorvastatin therapy].

    Science.gov (United States)

    Hydzik, Piotr; Szpak, Dorota

    2011-01-01

    The paper describes the case of 56 years old woman admitted to the Toxicology Department because of skin lesions, joint and muscle pain and elevated activity of transaminases and creatine phosfokinase as well in biochemical analysis. The symptoms occurred after 6 days of the Atorvastatin therapy. The clinical picture indicated side effects of the hipolipemic therapy, but the presence of the skin lesions suggested drug induced collagenosis (lupus erythrematosus, dermatomyositis). Immunological studies confirmed association with antinuclear antibodies (ANA) and anti-Mi-2 autoantibodies in the serum. Immunosuppressive therapy was ordered with clinical and biochemical improvement.

  16. Renal Tubular Function in Systemic Lupus Erythematosus*

    African Journals Online (AJOL)

    immune' diseases such as. Sjogren's syndrome,'" systemic lupus erythematosus. (SLE),3 alveolitis' and chronic active hepatitis.' The reported abnormalities of renal tubular function include impairment of acid excretion and urinary concentration.

  17. Systemisk lupus erythematosus i Fyns Amt

    DEFF Research Database (Denmark)

    Voss, Anne B.; Green, Anders; Junker, Peter

    1999-01-01

    The incidence and prevalence of systemic lupus erythematosus (SLE) has never been investigated in Denmark, whereas international studies have reached divergent results. In the study patients were ascertained from diagnosis-based registers of inpatients and outpatients, notifications from physicians...

  18. Appearance of Systemic Lupus Erythematosus in Patients with Myasthenia Gravis following Thymectomy: Two Case Reports

    OpenAIRE

    Park, Mi-Jeong; Kim, Yun-A; Lee, Shin-Seok; Kim, Byeong-Chae; Kim, Myeong-Kyu; Cho, Ki-Hyun

    2004-01-01

    We report two cases of systemic lupus erythematosus (SLE) in myasthenia gravis (MG) patients who had undergone thymectomy. SLE developed in the patients 3 months or 13 yr after thymectomy, and polyarthritis was the main clinical manifestation of SLE. Both patients fulfilled at least four of the revised criteria for the classification of SLE. In this report, we describe two postthymectomy lupus patients and perform a comparative review of previous cases.

  19. Hypoparathyroidism associated with systemic lupus erythematosus.

    Science.gov (United States)

    Gazarian, M; Laxer, R M; Kooh, S W; Silverman, E D

    1995-11-01

    We describe a 15-year-old girl with systemic lupus erythematosus (SLE) who presented with hypocalcemia and a generalized seizure in the setting of an intercurrent illness and active central nervous system lupus. She was subsequently found to have idiopathic hypoparathyroidism. The association of SLE with hypoparathyroidism is extremely rare and this case represents the first pediatric report of this rare association. We suggest there may be a common underlying pathophysiological process linking these diseases.

  20. 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.

  1. Autoantibodies in SLE: Specificities, Isotypes and Receptors

    Directory of Open Access Journals (Sweden)

    Barbara Dema

    2016-01-01

    Full Text Available Systemic Lupus Erythematosus (SLE is characterized by a wide spectrum of auto-antibodies which recognize several cellular components. The production of these self-reactive antibodies fluctuates during the course of the disease and the involvement of different antibody-secreting cell populations are considered highly relevant for the disease pathogenesis. These cells are developed and stimulated through different ways leading to the secretion of a variety of isotypes, affinities and idiotypes. Each of them has a particular mechanism of action binding to a specific antigen and recognized by distinct receptors. The effector responses triggered lead to a chronic tissue inflammation. DsDNA autoantibodies are the most studied as well as the first in being characterized for its pathogenic role in Lupus nephritis. However, others are of growing interest since they have been associated with other organ-specific damage, such as anti-NMDAR antibodies in neuropsychiatric clinical manifestations or anti-β2GP1 antibodies in vascular symptomatology. In this review, we describe the different auto-antibodies reported to be involved in SLE. How autoantibody isotypes and affinity-binding to their antigen might result in different pathogenic responses is also discussed.

  2. How do women with lupus manage fatigue?

    DEFF Research Database (Denmark)

    Kier, Anne Ørnholt; Midtgaard, Julie; Hougaard, Karin Sørig

    2016-01-01

    Objective: Half of patients with systemic lupus erythematosus (SLE) consider fatigue to be the most disabling disease symptom. To develop and promote strategies to prevent and control fatigue, this study aimed to describe how women with SLE manage the experience of fatigue. Methods: Four focus...

  3. The frequency and outcome of lupus nephritis

    DEFF Research Database (Denmark)

    Hanly, John G; O'Keeffe, Aidan G; Su, Li

    2016-01-01

    OBJECTIVE: To determine nephritis outcomes in a prospective multi-ethnic/racial SLE inception cohort. METHODS: Patients in the Systemic Lupus International Collaborating Clinics inception cohort (≤15 months of SLE diagnosis) were assessed annually for estimated glomerular filtration rate (e...

  4. Ocular findings in systemic lupus erythemato

    Directory of Open Access Journals (Sweden)

    Samir S. Shoughy

    2016-04-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic multisystem autoimmune disease. Ocular complications occur in up to one-third of patients with SLE. The ocular findings may represent the initial manifestation of the disease and may lead to severe ocular morbidity and loss of vision. Early diagnosis and prompt management of patients with SLE are mandatory and require collaboration between the ophthalmologist and the rheumatologist.

  5. Ocular findings in systemic lupus erythematosus.

    Science.gov (United States)

    Shoughy, Samir S; Tabbara, Khalid F

    2016-01-01

    Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease. Ocular complications occur in up to one-third of patients with SLE. The ocular findings may represent the initial manifestation of the disease and may lead to severe ocular morbidity and loss of vision. Early diagnosis and prompt management of patients with SLE are mandatory and require collaboration between the ophthalmologist and the rheumatologist.

  6. Pregnancy in women with systemic lupus erythematosus.

    Science.gov (United States)

    Kiss, Emese; Bhattoa, Harjit P; Bettembuk, Peter; Balogh, Adam; Szegedi, Gyula

    2002-03-10

    Systemic lupus erythematosus (SLE) is an autoimmune disorder which may be affected by hormonal changes, such as those of pregnancy. Women with SLE have increased adverse pregnancy outcomes. A retrospective analysis of the gynecologic and immunologic case history of 140 women with SLE and the outcome of 263 pregnancies in 99 women with SLE. In patients diagnosed with SLE, the proportion of pregnancies ending with live birth at term decreased to one-third compared with three quarters in those without a diagnosis of SLE and the incidence of pre-term deliveries and spontaneous abortions increased by 6.8 and 4.7 times, respectively. When SLE was associated with secondary antiphospholipid (APL) syndrome, and lupus anticoagulant (LA) or beta2-glycoprotein antibodies were present, a further increase in the incidence of pregnancy loss was observed. Pregnancy did not cause a flare-up of SLE in all cases, the disease remained stable in about 30% of the patients. Lupus was mild in the majority of the women who carried out their pregnancy to term. We also observed mothers with active SLE who successfully carried out pregnancies to term. These findings accord with previous literature and should inform rheumatologists, obstetricians and neonatologists who guide patients in their reproductive decisions.

  7. Children born to SLE and APS mothers.

    Science.gov (United States)

    Nalli, C; Iodice, A; Andreoli, L; Lojacono, A; Motta, M; Fazzi, E; Tincani, A

    2014-10-01

    Systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS) are autoimmune diseases that affect women of childbearing age. Pregnancies in these patients carry several complications such as prematurity. Maternal IgG antiphospholipid antibodies (aPL) can cross the placenta but they don't generally cause any neonatal thrombotic event. Because of the incompleteness of the fetal blood-brain barrier, aPL could theoretically reach the fetal brain. Whether this can have an effect on brain development is still under investigation. Some studies performed in children of patients with SLE and/or APS showed an increased number of learning disabilities without impairment in intelligence level. The objectives of this article are to evaluate the neurodevelopment outcome in 30 children (median age 9 years) born to mothers with SLE and/or APS with IgG anti-beta2-glycoprotein I during the third trimester of pregnancy and found positive for the same antibodies at birth. A neurological physical exam was performed in all children. We submitted some questionnaires to the mothers: the Child Behavior CheckList (CBCL) and a homemade set of questions obtained by a team composed of rheumatologists and pediatric neurologists. Intellectual functioning was determined by the Wechsler scale for corrected age. In all children neurological physical exam and intelligence levels were found to be normal but mild behavior disorders and history of neurological manifestations were shown in three children. Offspring of patients with SLE and/or APS are generally healthy. We and others observed the occurrence of minor neurological disorders that might be related to maternal disease or to prematurity. The limited number of the available data on this sensitive issue supports the need for further studies. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  8. Familial occurrence of autoimmune diseases and autoantibodies in a Caucasian population of patients with systemic lupus erythematosus

    NARCIS (Netherlands)

    Corporaal, S.; Bijl, Marc; Kallenberg, Cees

    To determine the prevalence of autoimmune diseases and autoantibodies in relatives of Caucasian patients with systemic lupus erythematosus (SLE) we questioned 118 patients for the prevalence of autoimmune diseases in their relatives. Multicase SLE families were selected for further investigation:

  9. Independent association of glucocorticoids with damage accrual in SLE.

    Science.gov (United States)

    Apostolopoulos, Diane; Kandane-Rathnayake, Rangi; Raghunath, Sudha; Hoi, Alberta; Nikpour, Mandana; Morand, Eric F

    2016-01-01

    To determine factors associated with damage accrual in a prospective cohort of patients with SLE. Patients with SLE who attended the Lupus Clinic at Monash Health, Australia, between 2007 and 2013 were studied. Clinical variables included disease activity (Systemic Lupus Erythematosus Disease Activity Index-2K, SLEDAI-2K), time-adjusted mean SLEDAI, cumulative glucocorticoid dose and organ damage (Systemic Lupus International Collaborating Clinics Damage Index (SDI)). Multivariate logistic regression analyses were performed to identify factors associated with damage accrual. A total of 162 patients were observed over a median (IQR) 3.6 (2.0-4.7) years. Seventy-five per cent (n=121) of patients received glucocorticoids. Damage accrual was significantly more frequent in glucocorticoid-exposed patients (42% vs 15%, p<0.01). Higher glucocorticoid exposure was independently associated with overall damage accrual after controlling for factors including ethnicity and disease activity and was significant at time-adjusted mean doses above 4.42 mg prednisolone/day; the OR of damage accrual in patients in the highest quartile of cumulative glucocorticoid exposure was over 10. Glucocorticoid exposure was independently associated with damage accrual in glucocorticoid-related and non-glucocorticoid related domains of the SDI. Glucocorticoid use is independently associated with the accrual of damage in SLE, including in non-glucocorticoid related domains.

  10. Type I interferon signature in systemic lupus erythematosus.

    Science.gov (United States)

    Bezalel, Shira; Guri, Keren Mahlab; Elbirt, Daniel; Asher, Ilan; Sthoeger, Zev Moshe

    2014-04-01

    Type I interferons (IFN) are primarily regarded as an inhibitor of viral replication. However, type I IFN, mainly IFNalpha, plays a major role in activation of both the innate and adaptive immune systems. Systemic lupus erythematosus (SLE) is a chronic, multi-systemic, inflammatory autoimmune disease with undefined etiology. SLE is characterized by dysregulation of both the innate and the adaptive immune systems. An increased expression of type I IFN-regulated genes, termed IFN signature, has been reported in patients with SLE. We review here the role of IFNalpha in the pathogenesis and course of SLE and the possible role of IFNalpha inhibition as a novel treatment for lupus patients.

  11. Imunidade na gestação normal e na paciente com lúpus eritematoso sistêmico (LES Immunity in the normal pregnancy and in the patient with systemic lupus erythematosus (SLE

    Directory of Open Access Journals (Sweden)

    Alessandra Cardoso Pereira

    2005-06-01

    Full Text Available A gravidez é uma condição fisiológica na qual ocorrem várias mudanças imunoendócrinas com a finalidade de facilitar a imunossupressão e a tolerância aos antígenos paternos e fetais. Na gravidez humana normal existe uma relativa supressão de citocinas tipo Th1 na resposta dos linfócitos, levando a uma prevalência na resposta do tipo Th2. No LES, onde prevalece a resposta imune do tipo Th2, a gravidez pode estar relacionada com a ativação da doença. Este artigo é uma revisão dessas alterações relacionadas com a resposta imune durante a gestação normal e na da paciente com LES.Pregnancy is a physiologic condition where several immuno-endocrine changes take place with the aim of facilitating immunosuppression and tolerance towards paternal and fetal antigens. In normal human pregnancy there is a relative suppression of Th1 type cytokines in response to lymphocytes, leading to a Th2 type response. In SLE, where a Th2 type response prevails, pregnancy may exacerbate the disease. This article reviews the alterations related to the immune response during normal pregnancy and in SLE patients.

  12. RNA-Seq for enrichment and analysis of IRF5 transcript expression in SLE.

    Directory of Open Access Journals (Sweden)

    Rivka C Stone

    Full Text Available Polymorphisms in the interferon regulatory factor 5 (IRF5 gene have been consistently replicated and shown to confer risk for or protection from the development of systemic lupus erythematosus (SLE. IRF5 expression is significantly upregulated in SLE patients and upregulation associates with IRF5-SLE risk haplotypes. IRF5 alternative splicing has also been shown to be elevated in SLE patients. Given that human IRF5 exists as multiple alternatively spliced transcripts with distinct function(s, it is important to determine whether the IRF5 transcript profile expressed in healthy donor immune cells is different from that expressed in SLE patients. Moreover, it is not currently known whether an IRF5-SLE risk haplotype defines the profile of IRF5 transcripts expressed. Using standard molecular cloning techniques, we identified and isolated 14 new differentially spliced IRF5 transcript variants from purified monocytes of healthy donors and SLE patients to generate an IRF5 variant transcriptome. Next-generation sequencing was then used to perform in-depth and quantitative analysis of full-length IRF5 transcript expression in primary immune cells of SLE patients and healthy donors by next-generation sequencing. Evidence for additional alternatively spliced transcripts was obtained from de novo junction discovery. Data from these studies support the overall complexity of IRF5 alternative splicing in SLE. Results from next-generation sequencing correlated with cloning and gave similar abundance rankings in SLE patients thus supporting the use of this new technology for in-depth single gene transcript profiling. Results from this study provide the first proof that 1 SLE patients express an IRF5 transcript signature that is distinct from healthy donors, 2 an IRF5-SLE risk haplotype defines the top four most abundant IRF5 transcripts expressed in SLE patients, and 3 an IRF5 transcript signature enables clustering of SLE patients with the H2 risk haplotype.

  13. Hair and Scalp Changes in Cutaneous and Systemic Lupus Erythematosus.

    Science.gov (United States)

    Udompanich, Siriorn; Chanprapaph, Kumutnart; Suchonwanit, Poonkiat

    2018-06-09

    Cutaneous and systemic lupus erythematosus (SLE) commonly involves the hair and scalp. Alopecia can result from direct activity of disease on the scalp or from the state of physical stress in the form of telogen effluvium. Discoid lupus erythematosus and lupus panniculitis/profundus are known to cause scarring alopecia, while accumulation of recent studies has shown that non-scarring alopecia in SLE may have different subtypes, comprising lupus erythematosus-specific and lupus erythematosus-nonspecific changes on histology. This review aims to summarize the clinical pattern, trichoscopic, histopathological, and direct immunofluorescence features of different types of alopecia in cutaneous and systemic lupus erythematosus, as well as exploring their relationship with SLE disease activity.

  14. 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.

  15. Systemic lupus erythematosus: Clinical and experimental aspects

    International Nuclear Information System (INIS)

    Smolen, J.S.

    1987-01-01

    This text covers questions related to the history, etiology, pathogenesis, clinical aspects and therapy of systematic lupus erythematosus (SLE). Both animal models and human SLE are considered. With regard to basic science, concise information on cellular immunology, autoantibodies, viral aspects and molecular biology in SLE is provided. Clinical topics then deal with medical, dermatologic, neurologic, radiologic, pathologic, and therapeutic aspects. The book not only presents the most recent information on clinical and experimental insights, but also looks at future aspects related to the diagnosis and therapy of SLE

  16. Epidemiology of cutaneous lupus erythematosus and the associated risk of systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Petersen, M Prütz; Möller, S; Bygum, A

    2018-01-01

    Objectives The objectives of this paper are to describe the epidemiology of cutaneous lupus erythematosus (CLE) and its subtypes in Denmark, and to investigate the probability of receiving a subsequent diagnosis of systemic lupus erythematosus (SLE) and the related time course. Methods A nationwide...

  17. 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...

  18. 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....

  19. Functional promoter haplotypes of the human FAS gene are associated with the phenotype of SLE characterized by thrombocytopenia

    DEFF Research Database (Denmark)

    Nolsøe, R L; Kelly, J A; Pociot, F

    2005-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by production of autoantibodies against intracellular antigens and tissue injury. Defective apoptosis of activated immune cells leads to the development of autoantibodies in SLE. FasL initiated apoptosis is central...... for peripheral tolerance. Fas deficiencies in humans and mice predispose toward systemic autoimmunity. SLE is conferred by many genes. The genetic effects may be concentrated by familial clustering or by stratifying of subphenotypes. We have tested polymorphisms and haplotypes in FAS and FASL for association...... to SLE or subphenotypes in 126 multiplex American SLE pedigrees and found association of the FAS codon214 AC(C/T) as well as the FAS-670G>A'-codon214 AC(C/T)' haplotype to thrombocytopenia in SLE. Furthermore we have functionally characterized the FAS/FASL promoter polymorphisms associated with SLE...

  20. 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...

  1. Immunomodulators in SLE: Clinical evidence and immunologic actions.

    Science.gov (United States)

    Durcan, L; Petri, M

    2016-11-01

    Systemic lupus erythematosus (SLE) is a potentially fatal autoimmune disease. Current treatment strategies rely heavily on corticosteroids, which are in turn responsible for a significant burden of morbidity, and immunosuppressives which are limited by suboptimal efficacy, increased infections and malignancies. There are significant deficiencies in our immunosuppressive armamentarium, making immunomodulatory therapies crucial, offering the opportunity to prevent disease flare and the subsequent accrual of damage. Currently available immunomodulators include prasterone (synthetic dehydroeipandrosterone), vitamin D, hydroxychloroquine and belimumab. These therapies, acting via numerous cellular and cytokine pathways, have been shown to modify the aberrant immune responses associated with SLE without overt immunosuppression. Vitamin D is important in SLE and supplementation appears to have a positive impact on disease activity particularly proteinuria. Belimumab has specific immunomodulatory properties and is an effective therapy in those with specific serological and clinical characteristics predictive of response. Hydroxychloroquine is a crucial background medication in SLE with actions in many molecular pathways. It has disease specific effects in reducing flare, treating cutaneous disease and inflammatory arthralgias in addition to other effects such as reduced thrombosis, increased longevity, improved lipids, better glycemic control and blood pressure. Dehydroeipandrosterone is also an immunomodulator in SLE which can have positive effects on disease activity and has bone protective properties. This review outlines the immunologic actions of these drugs and the clinical evidence supporting their use. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. Leptin levels in patients with systemic lupus erythematosus inversely correlate with regulatory T cell frequency.

    Science.gov (United States)

    Wang, X; Qiao, Y; Yang, L; Song, S; Han, Y; Tian, Y; Ding, M; Jin, H; Shao, F; Liu, A

    2017-11-01

    Leptin levels are increased in patients with systemic lupus erythematosus (SLE) but little is known on how this correlates with several disease characteristics including the frequency of regulatory T cells (Tregs). Here we compared serum leptin levels with frequency of circulating Tregs in 47 lupus patients vs. 25 healthy matched controls. Correlations with lupus disease activity were also analyzed, as well as Treg proliferation potential. It was found that leptin was remarkably increased in SLE patients as compared to controls, particularly in SLE patients with moderate and severe active SLE, and the increase correlated with disease activity. Importantly, increased leptin in lupus patients inversely correlated with the frequency of Tregs but not in controls, and leptin neutralization resulted in the expansion of Tregs ex vivo. Thus, hyperleptinemia in lupus patients correlates directly with disease activity and inversely with Treg frequency. The finding that leptin inhibition expands Tregs in SLE suggests possible inhibition of this molecule for an enhanced Treg function in the disease.

  3. Increased risk of depression in patients with cutaneous lupus erythematosus and systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Hesselvig, J H; Egeberg, A; Kofoed, K

    2018-01-01

    BACKGROUND: Reported prevalences of depression in patients with systemic lupus erythematosus (SLE) range widely, while the prevalence of depression in cutaneous lupus erythematosus (CLE) remains severely understudied. OBJECTIVES: To examine whether patients with SLE or CLE have increased risk...... of primary and secondary care, analyses of risk for depression and antidepressant use were performed in Cox regression models adjusted for age, sex, socio-economic status, smoking, alcohol abuse, prior depression, and prior antidepressant use. RESULTS: A total of 3,489 patients with lupus erythematosus were...

  4. Autoantibodies against protective molecules-C1q, C-reactive protein, serum amyloid P, mannose-binding lectin, and apolipoprotein A1 - Prevalence in systemic lupus erythematosus

    NARCIS (Netherlands)

    Shoenfeld, Yehuda; Szyper-Kravitz, Martine; Witte, Torsten; Doria, Andrea; Tsutsumi, Akito; Tatsuya, Abe; Dayer, Jean-Michel; Roux-Lombard, Pascale; Fontao, Lionel; Kallenberg, Cees G. M.; Bijl, Marc; Matthias, Torsten; Fraser, Abigail; Zandman-Goddard, Gisele; Blank, Miri; Gilburd, Boris; Meroni, Pier Luigi; Shoenfeld, Y; Gershwin, ME

    2007-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of several autoantibodies. Among the multiple factors involved in SLE development, apoptotic defects and impaired clearance of cellular debris have gained considerable interest, as they contribute to

  5. Depression in systemic lupus erythematosus, dependent on or independent of severity of disease

    NARCIS (Netherlands)

    van Exel, E.; Jacobs, J.; Korswagen, L.A.; Voskuyl, A.E.; Stek, M.L.; Dekker, J.; Bultink, I.E.M.

    2013-01-01

    Objectives: To estimate the prevalence of depression in subjects with systemic lupus erythematosus (SLE) in relation to the general population and to unravel the relation between depression and SLE disease characteristics. Methods: One hundred and two subjects with SLE (mean age 44.4 years) were

  6. 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...

  7. Disease activity assessment in SLE: do we have the right instruments?

    OpenAIRE

    Petri, Michelle

    2007-01-01

    No new therapy has been approved for systemic lupus erythematosus (SLE) in decades. Interest in SLE by pharmaceutical and biotechnology companies has increased, leading to multiple clinical trials. Unfortunately, we have now compiled quite a long list of “failed” trials. If this was due to the fact that the studied therapy did not work in SLE, we could accept it and move on. Of concern, however, is that many of the “failed” treatments had a strong “signal” of efficacy, often in subgroup analy...

  8. Biomarkers for Lupus Nephritis: A Critical Appraisal

    Directory of Open Access Journals (Sweden)

    Chi Chiu Mok

    2010-01-01

    Full Text Available Kidney disease is one of the most serious manifestations of systemic lupus erythematosus (SLE. Despite the improvement in the medical care of SLE in the past two decades, the prognosis of lupus nephritis remains unsatisfactory. Besides exploring more effective but less toxic treatment modalities that will further improve the remission rate, early detection and treatment of renal activity may spare patients from intensive immunosuppressive therapies and reduce renal damage. Conventional clinical parameters such as creatinine clearance, proteinuria, urine sediments, anti-dsDNA, and complement levels are not sensitive or specific enough for detecting ongoing disease activity in the lupus kidneys and early relapse of nephritis. Thus, novel biomarkers are necessary to enhance the diagnostic accuracy and sensitivity of lupus renal disease, prognostic stratification, monitoring of treatment response, and detection of early renal flares. This paper reviews promising biomarkers that have recently been evaluated in longitudinal studies of lupus nephritis.

  9. 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.

  10. 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...

  11. The LupusQoL and associations with demographics and clinical measurements in patients with systemic lupus erythematosus.

    Science.gov (United States)

    McElhone, Kathleen; Castelino, Madhura; Abbott, Janice; Bruce, Ian N; Ahmad, Yasmeen; Shelmerdine, Joanna; Peers, Kate; Isenberg, David; Ferenkeh-Koroma, Ada; Griffiths, Bridget; Akil, Mohammed; Maddison, Peter; Gordon, Caroline; Teh, Lee-Suan

    2010-11-01

    Having developed and validated a disease-specific health-related quality of life (HRQOL) measure for patients with systemic lupus erythematosus (SLE), the LupusQoL, we determined its relationship to demographic and clinical measurements in a group of patients with SLE. A group of 322 outpatients completed the LupusQoL. Demographic (age, sex, marital status, ethnicity) and clinical variables (disease duration, disease activity, damage) were recorded. Associations between the 8 LupusQoL domains and age, disease duration, disease activity, and damage were explored using Spearman's correlation coefficients. Differences in LupusQoL scores were examined for sex and marital status using the Mann-Whitney U test. Ethnic groups were compared using ANOVA. All domains of LupusQoL were impaired, with fatigue (56.3) being the worst affected and body image (80.0) the least. The correlations between the LupusQoL domain scores and age (r = -0.01 to -0.22) and disease duration (r = 0 to 0.16) were absent or weak. Similarly, there were no significant differences in the LupusQoL scores regarding sex, marital status, or the 3 main ethnic groups (Black-Caribbean, Asian, White). Although there were statistically significant correlations between the scores of the LupusQoL domains and some scores of the British Isles Lupus Assessment Group index (r = -0.22 to 0.09) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (r = -0.29 to 0.21), these were weak. HRQOL was impaired in this cohort of outpatients with SLE as assessed by the validated lupus-specific LupusQoL. There were no clinically important associations between the 8 domains of the LupusQoL and clinical or demographic variables in this group of patients. Thus, the LupusQoL is a relatively independent outcome measure in patients with SLE.

  12. Central nervous system systemic lupus erythematosus in children

    International Nuclear Information System (INIS)

    Osborn, A.G.; Boyer, R.S.

    1989-01-01

    Ischemic neurologic events and neuropsychiatric disorders occur in approximately 70% of patients with systematic lupus erythematosus (SLE). The CT and MR findings in adults with central nervous system (CNS) SLE have been described, but to the authors' knowledge no pediatric series has been reported. The MR and CT findings in four children with CNS SLE are compared with those reported in adults. Large infarcts are less frequent in children than in adults with CNS SLE, while multiple small infarctions and white matter lesions are more common. These findings in children who have no obvious source of emboli, intracardiac shunt, or history of trauma should raise the suspicion of SLE

  13. [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.

  14. Mucormycosis in systemic lupus erythematosus.

    Science.gov (United States)

    Mok, Chi Chiu; Que, Tak Lun; Tsui, Edmund Yik Kong; Lam, Wing Yin

    2003-10-01

    To describe a case of mucormycosis in systemic lupus erythematosus (SLE) and to review other patients reported in the English literature. A Medline search for articles about mucormycosis in SLE published between 1970 and 2002 was performed by using the key words "lupus," "mucormycosis," "zygomycosis," "Mucorales," "Rhizopus," and "Mucor." Cases were pooled for analysis, and the mycology, diagnosis, treatment, and outcome of mucormycosis in SLE was reviewed. Eight cases of mucormycosis in SLE were identified (female:male = 7:1). The mean age at the time of infection was 31.8 +/- 7.6 years and the mean duration of SLE was 6.3 +/- 3.9 years. All except 1 patient had active lupus and all were receiving high-dose corticosteroids. Concomitant cytotoxic agents were used in 4 patients. Additional predisposing factors for opportunistic infection included hypocomplementemia, nephrotic syndrome, uremia, leukopenia, and diabetes mellitus. The disseminated form of mucormycosis was the most common presentation and the diagnosis often was made only at autopsy (63%). For cases with positive culture results, Rhizopus was the causative species. In 4 patients, manifestations of the fungal infection mimicked those of active SLE. The overall mortality of mucormycosis was very high (88%) and, in most cases, was probably a function of delayed diagnosis and treatment. The cutaneous form appeared to have the best prognosis with combined medical and surgical treatment. Mucormycosis is a rare but usually fatal fungal infection in SLE. Judicious use of immunosuppressive agents, a high index of suspicion, early diagnosis, and combination treatment with amphotericin B and surgical debridement may improve the prognosis of this serious infection.

  15. Genomics of Systemic Lupus Erythematosus: Insights Gained by Studying Monogenic Young-Onset Systemic Lupus Erythematosus.

    Science.gov (United States)

    Hiraki, Linda T; Silverman, Earl D

    2017-08-01

    Systemic lupus erythematosus (SLE) is a systemic, autoimmune, multisystem disease with a heterogeneous clinical phenotype. Genome-wide association studies have identified multiple susceptibility loci, but these explain a fraction of the estimated heritability. This is partly because within the broad spectrum of SLE are monogenic diseases that tend to cluster in patients with young age of onset, and in families. This article highlights insights into the pathogenesis of SLE provided by these monogenic diseases. It examines genetic causes of complement deficiency, abnormal interferon production, and abnormalities of tolerance, resulting in monogenic SLE with overlapping clinical features, autoantibodies, and shared inflammatory pathways. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Lupus erythematosus cell preparation, antinuclear factor and antideoxyribonucleic acid antibody incongruity in systemic lupus erythematosus.

    Science.gov (United States)

    Chee, Y C

    1983-01-01

    'Total antinuclear antibody' (ANF) is detected by the fluorescent antinuclear antibody technique which is a screening test, positive in 99% of systemic lupus erythematosus (SLE) sera. The LE factor (positive in 75% of SLE sera), like the anti-DNA antibody, is an antinuclear antibody but directed against DNA-histone. ANF-negative SLE is a clinical entity with absence of these antibodies. A false negative ANF, in the presence of high titre anti-DNA antibody and/or LE cells, is illustrated in two cases of SLE. Postulated mechanisms for this phenomenon are interference in ANF detection by rheumatoid factor, and the prozone effect on the immunofluorescent tests.

  17. 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

  18. Health-related quality of life in patients with systemic lupus erythematosus: development and validation of a lupus specific symptom checklist

    NARCIS (Netherlands)

    Grootscholten, C.; Ligtenberg, G.; Derksen, R. H. W. M.; Schreurs, K. M. G.; de Glas-Vos, J. W.; Hagen, E. C.; van den Wall Bake, A. W. L.; Huizinga, T. W. J.; van den Hoogen, F. H. J.; Bijl, M.; van Houwelingen, J. C.; Snoek, F. J.; Berden, J. H. M.

    2003-01-01

    Reliable and sensitive measures are needed to evaluate the quality of life (QoL) in patients with systemic lupus erythematosus (SLE). No lupus specific questionnaires are available. This study describes the development and validation of a disease-specific questionnaire for lupus patients, which

  19. OSTEOPOROSIS IN SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    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.

  20. 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 patien...... generation of MPs may partake in the pathology of SLE and that new diagnostic, monitoring, and treatment strategies targeting these processes may be advantageous....

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

    OpenAIRE

    Sibel Serin; Kevser Kutlu Tatar; Tayyibe Saler

    2014-01-01

    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...

  2. 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

  3. 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.

  4. Evaluation of TRAF6 in a large multiancestral lupus cohort

    DEFF Research Database (Denmark)

    Namjou, Bahram; Choi, Chan-Bum; Harley, Isaac T W

    2012-01-01

    Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease with significant immune system aberrations resulting from complex heritable genetics as well as environmental factors. We undertook to study the role of TRAF6 as a candidate gene for SLE, since it plays a major role in several...

  5. Pulmonary arterial hypertension as a manifestation of lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Stark, P; Sargent, E N; Boylen, T; Jaramillo, D

    1987-08-01

    We present five patients with systemic lupus erythematosus (SLE) who developed pulmonary arterial hypertension and cor pulmonale in the course of their disease. The clinical features, as well as, the radiological manifestations of this rare manifestation of SLE are discussed. A vasculitic process is the most likely cause of this complication. Therapy is ineffective and the prognosis is poor.

  6. 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...

  7. Neuropsychiatric Symptoms in Systemic Lupus Erythematosus - Case Report

    Directory of Open Access Journals (Sweden)

    Sandra Almeida

    2014-10-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic inflammatory autoimmune disease of unknown etiology, with episodic course. It is characterized by periods of relative quiescence and periods of exacerbations which may involve any organ or system. About a young woman with a clinical delirium state, we revised the clinical neuropsychiatric features of SLE in the literature.

  8. Renal cell apoptosis in human lupus nephritis: a histological study

    DEFF Research Database (Denmark)

    Faurschou, M; Penkowa, Milena; Andersen, C B

    2009-01-01

    Nuclear autoantigens from apoptotic cells are believed to drive the immunological response in systemic lupus erythematosus (SLE). Conflicting data exist as to the possible renal origin of apoptotic cells in SLE patients with nephritis. We assessed the level of renal cell apoptosis in kidney...

  9. 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 ...

  10. Pulmonary arterial hypertension as a manifestation of lupus erythematosus

    International Nuclear Information System (INIS)

    Stark, P.; Jaramillo, D.

    1987-01-01

    We present five patients with systemic lupus erythematosus (SLE) who developed pulmonary arterial hypertension and cor pulmonale in the course of their disease. The clinical features, as well as, the radiological manifestations of this rare manifestation of SLE are discussed. A vasculitic process is the most likely cause of this complication. Therapy is ineffective and the prognosis is poor. (orig.) [de

  11. 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...

  12. Systemic Lupus Erythematosus: Definitions, Contexts, Conflicts, Enigmas

    Science.gov (United States)

    Rekvig, Ole Petter

    2018-01-01

    Systemic lupus erythematosus (SLE) is an inadequately defined syndrome. Etiology and pathogenesis remain largely unknown. SLE is on the other hand a seminal syndrome that has challenged immunologists, biologists, genetics, and clinicians to solve its nature. The syndrome is characterized by multiple, etiologically unlinked manifestations. Unexpectedly, they seem to occur in different stochastically linked clusters, although single gene defects may promote a smaller spectrum of symptoms/criteria typical for SLE. There is no known inner coherence of parameters (criteria) making up the disease. These parameters are, nevertheless, implemented in The American College of Rheumatology (ACR) and The Systemic Lupus Collaborating Clinics (SLICC) criteria to classify SLE. Still, SLE is an abstraction since the ACR or SLICC criteria allow us to define hundreds of different clinical SLE phenotypes. This is a major point of the present discussion and uses “The anti-dsDNA antibody” as an example related to the problematic search for biomarkers for SLE. The following discussion will show how problematic this is: the disease is defined through non-coherent classification criteria, its complexity is recognized and accepted, its pathogenesis is plural and poorly understood. Therapy is focused on dominant symptoms or organ manifestations, and not on the syndrome itself. From basic scientific evidences, we can add substantial amount of data that are not sufficiently considered in clinical medicine, which may change the paradigms linked to what “The Anti-DNA antibody” is—and is not—in context of the imperfectly defined syndrome SLE. PMID:29545801

  13. Retracted Association of STAT4 gene polymorphism with systemic lupus erythematosus / lupus nephritis risk.

    Science.gov (United States)

    Zhou, Tian-Biao; Jiang, Zong-Pei; Qin, Yuan-Han; Zhou, Jia-Fan

    2014-04-16

    The association of STAT4 gene polymorphism with systemic lupus erythematosus (SLE) / lupus nephritis (LN) results from the published studies is still conflicting. This meta-analysis was performed to evaluate the relationship between STAT4 rs7574865, rs16833431, rs11889341, rs8179673, rs10168266, rs7582694, rs3821236, rs7601754 gene polymorphism and SLE / LN, and to explore whether STAT4 gene polymorphism 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 September 1, 2013, and eligible investigations were synthesized using meta-analysis method. 24 investigations were identified for the analysis of association between STAT4 gene polymorphism and SLE, consisting of 31190 patients with SLE and 43940 controls. In STAT4 rs7574865, there was a marked association between T allele or TT genotype and SLE susceptibility (T: OR=1.53, 95% CI: 1.30-1.79, Prs7574865 gene polymorphism was not associated with the LN risk. Our results indicate that T allele or TT homozygous is a significant risk genetic molecular marker to predict the SLE susceptibility and GG genotype is a valuable marker to against the SLE risk, but the association was not found for LN. However, more investigations are required to further clarify the association of the T allele or TT homozygous with SLE / LN susceptibility. This article is protected by copyright. All rights reserved.

  14. Computerized tomography data on CNS affection in systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Ivanova, M.M.; Bliznyuk, O.I.; Todua, F.I.; Tumanova, A.A.

    1989-01-01

    Computed tomography (CT) of the brain was employed in 40 patients with systemic lupus erythematosus (SLE). Clinical cerebral pathology was obvious in 30 and absent in 10 patients. By CT cerebral symptoms were divided of 4 groups. Clinical symptom complexes of CNS defects and SLE were reflected on definite CT images correlated with focal damage to the brain. CT picture of enlarged subarachnoid space, ventricles and basal cisterns can be observed in SLE patients without neurological symptoms. This indicated likely subclinical cerebral affection

  15. 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....

  16. Autoantibody to MDM2: A Potential Serological Marker of Systemic Lupus Erythematosus

    OpenAIRE

    Liu, Yuan; Dai, Liping; Liu, Weihong; Shi, Guixiu; Zhang, Jianying

    2015-01-01

    Introduction. Systemic lupus erythematosus (SLE) is one of the systemic autoimmune diseases characterized by the polyclonal autoantibody production. The human homologue of the mouse double minute 2 (MDM2) is well known as the negative regulator of p53. MDM2 has been reported to be overexpressed in SLE animal model and to promote SLE. Since abnormally expressed proteins can induce autoimmune response, anti-MDM2 autoantibody was examined in SLE patients. Methods. Anti-MDM2 antibody in sera from...

  17. 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.

  18. Hyperthyroidism association with SLE, lessons from real-life data--A case-control study.

    Science.gov (United States)

    Watad, Abdulla; Cohen, Arnon D; Comaneshter, Doron; Tekes-Manova, Dorit; Amital, Howard

    2016-01-01

    Despite the frequently encountered association between thyroid disease and systemic lupus erythematosus (SLE) is well known, it is of surprise that only several reports compromised of small population size support this observation. To investigate the association of comorbid SLE and hyperthyroidism. Using the database of the largest health maintenance organization (HMO) in Israel, the Clalit Health Services, we searched for the co-existence of SLE and hyperthyroidism. Patients with SLE were compared with age- and sex-matched controls regarding the prevalence of hyperthyroidism in a case-control study. Chi-square and t-tests were used for univariate analysis and a logistic regression model was used for multivariate analysis. The study included 5018 patients with SLE and 25,090 age- and sex- matched controls. The prevalence of hyperthyroidism in patients with SLE was increased compared with the prevalence in controls (2.59% and 0.91%, respectively, p hyperthyroidism (odds ratio 2.52, 95% confidence interval 2.028-3.137). Patients with SLE have a greater prevalence of hyperthyroidism than matched controls. Therefore, physicians treating patients with SLE should be aware of this possibility of this thyroid dysfunction.

  19. Healthcare Utilization and Costs of Systemic Lupus Erythematosus in Medicaid

    Directory of Open Access Journals (Sweden)

    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.

  20. [A case of anti-LKM 1 positive autoimmune hepatitis accompanied by systemic lupus erythematosus].

    Science.gov (United States)

    Choi, Dae Han; Kim, Hae Kyung; Park, Tae Il; John, Byung Min; Kang, Sung Hwan; Lee, Yoon Serk; Kim, Tae Hyun; Lee, Uh Joo; Lee, Tae Seung; Yoon, Gwi Ok

    2008-03-01

    Overlap of autoimmune hepatitis and systemic lupus erythematosus (SLE) is a comparatively rare condition. Although both autoimmune hepatitis and SLE can share common autoimmune features such as polyarthralgia, hypergammaglobulinemia and positive ANA, it has been considered as two different entities. We report a case of anti-LKM1 positive autoimmune hepatitis who developed SLE two years later. The presence of interface hepatitis with lymphoplasma cell infiltrates and rosette formation points to the autoimmune hepatitis rather than SLE hepatitis. Autoimmune hepatitis is infrequently accompanied by SLE, therefore, it could be recommended to investigate for SLE in patients with autoimmune hepatitis.

  1. Increased serum ß2-microglobulin is associated with clinical and immunological markers of disease activity in systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Hermansen, M-L F; Hummelshøj, L; Lundsgaard, Dorte

    2012-01-01

    The objective of this study was to explore the relationship between serum levels of ß2-microglobulin (ß2MG), which some studies suggest reflect disease activity in systemic lupus erythematosus (SLE), and various clinical and immunological markers of disease activity in SLE. Twenty-six SLE patients...

  2. Nitrated nucleosome levels and neuropsychiatric events in systemic lupus erythematosus;

    DEFF Research Database (Denmark)

    Ferreira, Isabel; Croca, Sara; Raimondo, Maria Gabriella

    2017-01-01

    BACKGROUND: In patients with systemic lupus erythematosus (SLE) there is no serological test that will reliably distinguish neuropsychiatric (NP) events due to active SLE from those due to other causes. Previously we showed that serum levels of nitrated nucleosomes (NN) were elevated in a small...... number of patients with NPSLE. Here we measured serum NN in samples from a larger population of patients with SLE and NP events to see whether elevated serum NN could be a marker for NPSLE. METHODS: We obtained serum samples from patients in the Systemic Lupus International Collaborative Clinics (SLICC...

  3. Multiple Autoantibodies Display Association with Lymphopenia, Proteinuria, and Cellular Casts in a Large, Ethnically Diverse SLE Patient Cohort

    Directory of Open Access Journals (Sweden)

    Rufei Lu

    2012-01-01

    Full Text Available Purpose. This study evaluates high-throughput autoantibody screening and determines associated systemic lupus erythematosus (SLE clinical features in a large lupus cohort. Methods. Clinical and demographic information, along with serum samples, were obtained from each SLE study participant after appropriate informed consent. Serum samples were screened for 10 distinct SLE autoantibody specificities and examined for association with SLE ACR criteria and subcriteria using conditional logistic regression analysis. Results. In European-American SLE patients, autoantibodies against 52 kD Ro and RNP 68 are independently enriched in patients with lymphopenia, anti-La, and anti-ribosomal P are increased in patients with malar rash, and anti-dsDNA and anti-Sm are enriched in patients with proteinuria. In African-American SLE patients, cellular casts associate with autoantibodies against dsDNA, Sm, and Sm/nRNP. Conclusion. Using a high-throughput, bead-based method of autoantibody detection, anti-dsDNA is significantly enriched in patienets with SLE ACR renal criteria as has been previously described. However, lymphopenia is associated with several distinct autoantibody specificities. These findings offer meaningful information to allow clinicians and clinical investigators to understand which autoantibodies correlate with select SLE clinical manifestations across common racial groups using this novel methodology which is expanding in clinical use.

  4. Thrombotic thrombocytopenic purpura and deep vein thrombosis as the presenting manifestations of systemic lupus erythematosus: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Mohammad AH Mashhadi

    2011-01-01

    Full Text Available Systemic lupus erythematosus (SLE, is sometimes complicated by the rare fatal syndrome, Thrombotic thrombocyto-penic purpura (TTP, but the occurrence of TTP as the initial manifestation of SLE is very rare. Since they have similar-ities in some features, the differentiation of TTP from SLE may be missed. SLE patients are also more prone to throm-botic events. Here we report a case with TTP and deep vein thrombosis as the presenting symptoms of SLE.

  5. Coronary, Carotid, and Lower-extremity Atherosclerosis and Their Interrelationship in Danish Patients with Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Kay, Susan Due; Poulsen, Mikael Kjaer; Diederichsen, Axel Cosmus Pyndt

    2015-01-01

    OBJECTIVE: Atherosclerosis is highly prevalent among patients with systemic lupus erythematosus (SLE), but has been demonstrated predominantly in non-European SLE cohorts and few investigations have included more than 1 imaging modality. We aimed to investigate the prevalence of atherosclerosis...... regression model, age (p Systemic Lupus International Collaborating Clinics (SLICC; p = 0.008) were significant independent risk factors for atherosclerosis at any vascular territory. CONCLUSION: Atherosclerosis is highly prevalent among Danish patients with SLE...

  6. 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

  7. 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.

  8. Pulmonary manifestations of systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Yang, Kee Hyuk; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Joo, Kyung Bin; Hahm, Chang Kok; Lee, Seung Ro

    2004-01-01

    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

  9. Brain MR imaging of systemic lupus erythematodes

    International Nuclear Information System (INIS)

    Kobayashi, Satoshi; Suzuki, Masayuki; Ueda, Fumiaki; Arai, Kazunori; Kobayashi, Takeshi; Kadoya, Masumi; Matsui, Osamu; Takashima, Tsutomu

    1996-01-01

    Brain MR imaging of 13 patients with systemic lupus erythematodus (SLE) were reviewed. Two major findings was obtained. One was deep white matter hyperintensity (DWMH) and periventricular hyperintensity (PVH), the other was cerebral infarction. In comparison with the same age group, relatively severe brain atrophy was also observed. It was thought that these findings were induced from the vasculitis caused by SLE. However, the influence of the steroid therapy could not be excluded. No definite correlation between MR findings and clinical symptoms were seen. In conclusion, when we interpret brain MR imaging of the patients with SLE, special attention should be paid to their age. (author)

  10. Epidemiology of systemic lupus erythematosus: an update.

    Science.gov (United States)

    Stojan, George; Petri, Michelle

    2018-03-01

    Systemic lupus erythematosus (SLE) is the prototypical systemic autoimmune disease with a significant disease burden across the world among different ethnic, racial, and age groups. The pathophysiological understanding of SLE is constantly evolving and with it, the need for a better definition of the disease itself, for understanding the risk among the different affected populations, and for identifying the factors responsible for the damage accrual through the years. More accurate estimates of incidence and prevalence of SLE among different ethnicities and minority groups not only in the USA, but also in Europe, Middle East, and Asia have provided new insights into the disease burden around the world. Despite advances in treatment, mortality among SLE patients remains high with significant ethnic and geographic variations. Sex, race, and ethnicity significantly affect SLE incidence, prevalence, and mortality.

  11. Acute macular neuroretinopathy associated with systemic lupus erythematosus.

    Science.gov (United States)

    Lee, D H; Lee, S C; Kim, M

    2016-04-01

    Acute macular neuroretinopathy (AMN) is a rare disorder that presents with abrupt visual change with wedge-shaped or flower-like lesions pointing towards the fovea. Ischemic insults to the retinal capillary plexus may be important for development of this disease. While many case reports have been published on AMN, none have described AMN in association with systemic lupus erythematosus (SLE). Here, we report a case of AMN associated with newly-diagnosed SLE. We speculate that in patients with lupus flares, immune complex-mediated vascular injury and microvascular thrombosis may disrupt the deep retinal capillary network, causing ischemic damages to the outer retina and leading to the development of AMN. AMN can develop in patients with lupus flares, and must be considered as an SLE-associated ophthalmologic complication. To the best of our knowledge, this is the first case report of AMN associated with SLE. © The Author(s) 2015.

  12. Recent insights into the genetic basis of systemic lupus erythematosus

    OpenAIRE

    Moser, Kathy L.; Kelly, Jennifer A.; Lessard, Christopher J.; Harley, John B.

    2009-01-01

    Genetic variation was first shown to be part of the cause of systemic lupus erythematosus (SLE or lupus) in the 1970s with associations in the human leukocyte antigen (HLA) region. Almost four decades later, and with the help of increasingly powerful genetic approaches, more than 25 genes are now known to contribute to the mechanisms that predispose individuals to lupus. Over half of these loci have been discovered in the past two years, underscoring the extraordinary success of recent genome...

  13. Neurological Disease in Lupus: Toward a Personalized Medicine Approach.

    Science.gov (United States)

    McGlasson, Sarah; Wiseman, Stewart; Wardlaw, Joanna; Dhaun, Neeraj; Hunt, David P J

    2018-01-01

    The brain and nervous system are important targets for immune-mediated damage in systemic lupus erythematosus (SLE), resulting in a complex spectrum of neurological syndromes. Defining nervous system disease in lupus poses significant challenges. Among the difficulties to be addressed are a diversity of clinical manifestations and a lack of understanding of their mechanistic basis. However, despite these challenges, progress has been made in the identification of pathways which contribute to neurological disease in SLE. Understanding the molecular pathogenesis of neurological disease in lupus will inform both classification and approaches to clinical trials.

  14. Renal cell apoptosis in human lupus nephritis: a histological study

    DEFF Research Database (Denmark)

    Faurschou, M; Penkowa, Milena; Andersen, C B

    2009-01-01

    Nuclear autoantigens from apoptotic cells are believed to drive the immunological response in systemic lupus erythematosus (SLE). Conflicting data exist as to the possible renal origin of apoptotic cells in SLE patients with nephritis. We assessed the level of renal cell apoptosis in kidney...... biopsies from 35 patients with lupus nephritis by means of terminal deoxynucleotidyl-transferase (TdT)-mediated deoxyuridine triphosphate (dUTP)-digoxigenin nick end labeling (TUNEL). Five samples of normal kidney tissue served as control specimens. We did not observe apoptotic glomerular cells in any...... cells constitute a quantitatively important source of auto-antibody-inducing nuclear auto-antigens in human lupus nephritis....

  15. Neurological Disease in Lupus: Toward a Personalized Medicine Approach

    Science.gov (United States)

    McGlasson, Sarah; Wiseman, Stewart; Wardlaw, Joanna; Dhaun, Neeraj; Hunt, David P. J.

    2018-01-01

    The brain and nervous system are important targets for immune-mediated damage in systemic lupus erythematosus (SLE), resulting in a complex spectrum of neurological syndromes. Defining nervous system disease in lupus poses significant challenges. Among the difficulties to be addressed are a diversity of clinical manifestations and a lack of understanding of their mechanistic basis. However, despite these challenges, progress has been made in the identification of pathways which contribute to neurological disease in SLE. Understanding the molecular pathogenesis of neurological disease in lupus will inform both classification and approaches to clinical trials. PMID:29928273

  16. The management of pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Ardoin, Stacy P; Schanberg, Laura E

    2005-12-01

    Most children and adolescents with systemic lupus erythematosus (SLE) now survive into adulthood, leading the pediatric rheumatology community to focus on preventing long-term complications of SLE, including atherosclerosis, obesity, and osteoporosis, and their treatment. Unfortunately, because of the paucity of data in pediatric SLE, little is known about epidemiology, long-term outcome, and optimal treatment. Most research focuses on adults with SLE, but pediatric SLE differs significantly from adult SLE in many aspects, including disease expression, approaches to pharmacologic intervention, management of treatment toxicity, and psychosocial issues. Children and adolescents with SLE require specialized, multidisciplinary care. Treatment can be optimized by early recognition of disease flares and complications, minimizing medication toxicity, educating families about prevention, promoting school performance, addressing concerns about reproductive health, and negotiating the transition to adult-centered medical care. Developmentally appropriate concerns about pain, appearance, and peers often affect treatment adherence and must be addressed by the health-care team. Research in pediatric SLE is desperately needed and provides a unique opportunity to understand how developmental immunology and the hormonal changes associated with puberty affect the pathophysiology of SLE.

  17. [The association of fibromyalgia and systemic lupus erythematosus change the presentation and severity of both diseases?].

    Science.gov (United States)

    de Araújo, Ana Luiza P Kasemodel; Paliares, Isabella Cristina; de Araújo, Maria Izabel P Kasemodel; Novo, Neil Ferreira; Cadaval, Ricardo Augusto M; Martinez, José Eduardo

    2015-01-01

    The association of fibromyalgia (FM) and systemic lupus erythematosus (SLE) have been investigated, with conflicting results regarding the impact of a condition on the other. To determine the frequency of FM in a sample of patients with SLE treated at the Hospital Complex of Sorocaba (CHS) and the impact of FM in SLE activity and quality of life, as well as of SLE in FM. Descriptive and correlational study. Patients who met the American College of Rheumatology (ACR) criteria for SLE and/or FM were included. The total sample was divided into three groups: FM/SLE (patients with association of SLE and FM), SLE (SLE patients only) and FM (FM patients only). The following variables were used: Fibromyalgia Impact Questionnaire (FIQ), activity index of SLE (SLEDAI), Indices of Diagnostic Criteria for Fibromyalgia 2010 (SSI end GPI) and SF-36. The prevalence of patients with FM among SLE patients was 12%. FIQ showed no difference between groups, indicating that SLE did not affect the impact caused by FM alone. The presence of FM in SLE patients did not influence the clinical activity of this disease. A strong impact of FM on the quality of life in patients with SLE was observed; the opposite was not observed. The prevalence of FM observed in SLE patients is 12%. The presence of FM adversely affects the quality of life of patients with SLE. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  18. [Systemic lupus erythematodes].

    Science.gov (United States)

    Lukác, J; Rovenský, J; Lukácová, O; Kozáková, D

    2006-01-01

    Systemic lupus erythematodes (SLE) is chronic autoimmune disease, characteristic by production of autoantibodies against different autoantigens. Etiopathogenesis in not precise determinated, but genetic, immunologic, hormonal factors or influence of environment are assumed. It manifests by various symptoms and it can affect whichever organ or system in the body. Clinical manifestation are due chronic inflammation in the tissues, which is caused first of all by deposit of immunocomplex and by cytotoxic damage. At the last decades the mortality of patients with SLE is markly lower and their live is prolong. In spite of this diagnostic, to follow up and therapy of this disease is complicated and it requires the colaboration of more branches of medicine.

  19. 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 within......% CI 2.38 to 23.57) remained significant risk factors. CONCLUSIONS: In some patients with lupus, MI may develop even before the diagnosis of SLE or shortly thereafter, suggesting that there may be a link between autoimmune inflammation and atherosclerosis....

  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. Systemic lupus erythematosus and myelofibrosis: A case report and revision of literature

    Directory of Open Access Journals (Sweden)

    F. Del Porto

    Full Text Available Blood cytopenia represents one of the diagnostic criteria for systemic lupus erythematosus (SLE and may occur as the first symptom of the disease. Antibody-mediated peripheral destruction of blood cells is the main cause of cytopenia observed in patients affected by SLE, however, inflammatory anemia, nutritional deficiencies, immunosuppressive therapy and, more rarely, myelofibrosis (MF have also been documented. In the literature, 45 cases of autoimmune MF (AIMF and SLE have been previously reported. Here the 46th case of a 43-year-old female with a SLE and an underhand cytopenia, with a review of the literature. Keywords: Systemic lupus erythematosus, Autoimmune myelofibrosis, Blood cytopenia

  2. Estrogen in cardiovascular disease during systemic lupus erythematosus.

    Science.gov (United States)

    Gilbert, Emily L; Ryan, Michael J

    2014-12-01

    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. PubMed was used to search for articles with terms related to estradiol and SLE. The references of retrieved publications were also reviewed. 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 cardiovascular risk factors in

  3. 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

  4. The Pathogenesis of Lupus Nephritis

    Science.gov (United States)

    Lech, Maciej

    2013-01-01

    Lupus nephritis is an immune complex GN that develops as a frequent complication of SLE. The pathogenesis of lupus nephritis involves a variety of pathogenic mechanisms. The extrarenal etiology of systemic lupus is based on multiple combinations of genetic variants that compromise those mechanisms normally assuring immune tolerance to nuclear autoantigens. This loss of tolerance becomes clinically detectable by the presence of antinuclear antibodies. In addition, nucleic acids released from netting or apoptotic neutrophils activate innate and adaptive immunity via viral nucleic acid-specific Toll-like receptors. Therefore, many clinical manifestations of systemic lupus resemble those of viral infection. In lupus, endogenous nuclear particles trigger IFN-α signaling just like viral particles during viral infection. As such, dendritic cells, T helper cells, B cells, and plasma cells all contribute to the aberrant polyclonal autoimmunity. The intrarenal etiology of lupus nephritis involves antibody binding to multiple intrarenal autoantigens rather than the deposition of circulating immune complexes. Tertiary lymphoid tissue formation and local antibody production add to intrarenal complement activation as renal immunopathology progresses. Here we provide an update on the pathogenic mechanisms that lead to lupus nephritis and provide the rationale for the latest and novel treatment strategies. PMID:23929771

  5. 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.

  6. 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.

  7. Genetic Association of CD247 (CD3ζ) with SLE in a Large-Scale Multiethnic Study

    OpenAIRE

    Martins, Madalena; Williams, Adrienne H.; Comeau, Mary; Marion, Miranda; Ziegler, Julie T.; Freedman, Barry I.; Merrill, Joan T.; Glenn, Stuart B.; Kelly, Jennifer A.; Sivils, Kathy M.; James, Judith A.; Guthridge, Joel M.; Alarcón-Riquelme, Marta E.; Bae, Sang-Cheol; Kim, Jae-Hoon

    2015-01-01

    A classic T-cell phenotype in systemic lupus erythematosus (SLE) is the downregulation and replacement of the CD3ζ chain that alters T-cell receptor signaling. However, genetic associations with SLE in the human CD247 locus that encodes CD3ζ are not well established and require replication in independent cohorts. Our aim was therefore to examine, localize and validate CD247-SLE association in a large multiethnic population. We typed 44 contiguous CD247 single-nucleotide polymorphisms (SNPs) i...

  8. Lack of recording of systemic lupus erythematosus in the death certificates of lupus patients.

    Science.gov (United States)

    Calvo-Alén, J; Alarcón, G S; Campbell, R; Fernández, M; Reveille, J D; Cooper, G S

    2005-09-01

    To determine to what extent the diagnosis of systemic lupus erythematosus (SLE) in deceased lupus patients is under-reported in death certificates, and the patient characteristics associated with such an occurrence. The death certificates of 76 of the 81 deceased SLE patients from two US lupus cohorts (LUMINA for Lupus in Minorities: Nature vs Nurture and CLU for Carolina Lupus Study), including 570 and 265 patients, respectively, were obtained from the Offices of Vital Statistics of the states where the patients died (Alabama, Georgia, North Carolina, South Carolina, Tennessee and Texas). Both cohorts included patients with SLE as per the American College of Rheumatology criteria, aged > or =16 yr, and disease duration at enrolment of < or =5 yr. The median duration of follow-up in each cohort at the time of these analyses ranged from 38.1 to 53.0 months. Standard univariable analyses were performed comparing patients with SLE recorded anywhere in the death certificate and those without it. A multivariable logistic regression model was performed to identify the variables independently associated with not recording SLE in death certificates. In 30 (40%) death certificates, SLE was not recorded anywhere in the death certificate. In univariable analyses, older age was associated with lack of recording of SLE in death certificates [mean age (standard deviation) 50.9 (15.6) years and 39.1 (18.6) yr among those for whom SLE was omitted and included on the death certificates, respectively, P = 0.005]. Patients without health insurance, those dying of a cardiovascular event and those of Caucasian ethnicity were also more likely to be in the non-recorded group. In the multivariable analysis, variables independently associated with not recording SLE as cause of death were older age [odds ratio = (95% confidence interval) 1.043 (1.005-1.083 per yr increase); P = 0.023] and lack of health insurance [4.649 (1.152-18.768); P = 0.031]. A high proportion of SLE diagnoses are not

  9. Screening for cognitive impairment in SLE using the Self-Administered Gerocognitive Exam.

    Science.gov (United States)

    Meara, A; Davidson, N; Steigelman, H; Zhao, S; Brock, G; Jarjour, W N; Rovin, B H; Madhoun, H; Parikh, S; Hebert, L; Ayoub, I; Ardoin, S P

    2018-01-01

    Objective Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can affect the central nervous system in multiple ways, including causing cognitive dysfunction. Cognitive dysfunction is a common complaint of SLE patients yet diagnosis is challenging, time consuming, and costly. This study evaluated the Self-Administered Gerocognitive Exam (SAGE) as a screening test for cognitive impairment in a cohort of SLE patients. Methods A total of 118 SLE patients completed the SAGE. Providers completed the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborative Clinics Damage Index (SLICC-DI). SAGE scores were grouped into normal (>16) and abnormal (≤16) categories. Univariate and multivariate analyses were performed. Results Of the 118 participants, 21(18%) scored ≤16 on the SAGE instrument. In univariate analysis, race, ethnicity, household income, and SLICC-DI scores were associated with the SAGE ( p SAGE score was independently associated with higher SLICC-DI score (odds ratio (OR) = 1.44, 95% confidence intervals 1.04-1.99, p = 0.03)), Hispanic ethnicity (OR = 43.4, 95% CI 3.1-601, p = 0.005), and lower household income (OR = 11.9 for ≤$15,000 vs >$50,000, 95% CI 2.45-57, p = 0.002). Conclusions In SLE patients, this study demonstrates an independent relationship between neurocognitive impairment (as measured by the SAGE) and higher lupus-related damage, as measured by the SLICC-DI, and lower household income. Abnormal SAGE scores were also associated with Hispanic ethnicity. A language barrier could explain this because the SAGE instrument was conducted in English only. The SAGE was feasible to measure in the clinic setting.

  10. The useful application of anti-ds DNA antibody radioimmunoassay in the diagnosis of male SLE patients

    International Nuclear Information System (INIS)

    Alshaikhly, A.W.A.R.

    1995-01-01

    From the period of 1994 to 1990, the anti double stranded deoxyribonucleic acid radioimmunoassay (Anti-ds DNA RIA) were extensively applied to confirm diagnosis of Systemic Lupus Erythematosus (SLE) disease in male patients. 54 patients sera with definite diagnosis of SLE, 30 patients sera with Rheumatoid Arthritis (RA), 20 patients sera with other Rheumatic or Collagen vascular diseases (ORD), 5 patients sera of non specific arthritis (NSA) and 55 healthy male individual sera were included. The 54 male SLE patients were diagnosed after screening more than 2000 sera from male patients with various Rheumatic diseases who overlapping clinical symptoms with SLE. The results of this study are confirming that the anti-ds DNA RIA is proved to be a simple, sensitive, specific and useful technique among various assays used for diagnosis purposes. The results also pointed out that the prevalence of SLE disease in males is much lower than females. 4 tabs

  11. The useful application of anti-ds DNA antibody radioimmunoassay in the diagnosis of male SLE patients

    Energy Technology Data Exchange (ETDEWEB)

    Alshaikhly, A W.A.R. [Al-Rasheed Hospital, Baghdad, (Iraq)

    1995-10-01

    From the period of 1994 to 1990, the anti double stranded deoxyribonucleic acid radioimmunoassay (Anti-ds DNA RIA) were extensively applied to confirm diagnosis of Systemic Lupus Erythematosus (SLE) disease in male patients. 54 patients sera with definite diagnosis of SLE, 30 patients sera with Rheumatoid Arthritis (RA), 20 patients sera with other Rheumatic or Collagen vascular diseases (ORD), 5 patients sera of non specific arthritis (NSA) and 55 healthy male individual sera were included. The 54 male SLE patients were diagnosed after screening more than 2000 sera from male patients with various Rheumatic diseases who overlapping clinical symptoms with SLE. The results of this study are confirming that the anti-ds DNA RIA is proved to be a simple, sensitive, specific and useful technique among various assays used for diagnosis purposes. The results also pointed out that the prevalence of SLE disease in males is much lower than females. 4 tabs.

  12. Decreased SAP expression in T cells from patients with SLE contributes to early signaling abnormalities and reduced IL-2 production

    Science.gov (United States)

    Karampetsou, Maria P.; Comte, Denis; Kis-Toth, Katalin; Terhorst, Cox; Kyttaris, Vasileios C.; Tsokos, George C.

    2016-01-01

    T cells from patients with systemic lupus erythematosus (SLE) display a number of functions including increased early signaling events following engagement of the T cell receptor (TCR). Signaling lymphocytic activation molecule family (SLAMF) cell surface receptors and the X-chromosome-defined signaling lymphocytic activation molecule-associated protein (SAP) adaptor are important in the development of several immunocyte lineages and modulating immune response. Here we present evidence that SAP protein levels are decreased in T cells and in their main subsets isolated from 32 women and 3 men with SLE independently of disease activity. In SLE T cells the SAP protein is also subject to increased degradation by a caspase-3. Forced expression of SAP in SLE T cells simultaneously heightened IL-2 production, calcium (Ca2+) responses and tyrosine phosphorylation of a number of proteins. Exposure of normal T cells to SLE serum IgG, known to contain anti-CD3/TCR antibodies, resulted in SAP downregulation. We conclude that SLE T cells display reduced levels of the adaptor protein SAP probably as a result of continuous T cell activation and degradation by caspase-3. Restoration of SAP levels in SLE T cells corrects the overexcitable lupus T cell phenotype. PMID:27183584

  13. Environmental Factors, Toxicants and Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Anselm Mak

    2014-09-01

    Full Text Available Systemic lupus erythematosus (SLE is an immune-complex-mediated multi-systemic autoimmune condition of multifactorial etiology, which mainly affects young women. It is currently believed that the onset of SLE and lupus flares are triggered by various environmental factors in genetically susceptible individuals. Various environmental agents and toxicants, such as cigarette smoke, alcohol, occupationally- and non-occupationally-related chemicals, ultraviolet light, infections, sex hormones and certain medications and vaccines, have been implicated to induce SLE onset or flares in a number case series, case-control and population-based cohort studies and very few randomized controlled trials. Here, we will describe some of these recognized environmental lupus triggering and perpetuating factors and explain how these factors potentially bias the immune system towards autoimmunity through their interactions with genetic and epigenetic alterations. Further in-depth exploration of how potentially important environmental factors mechanistically interact with the immune system and the genome, which trigger the onset of SLE and lupus flares, will certainly be one of the plausible steps to prevent the onset and to decelerate the progress of the disease.

  14. 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.

  15. On lupus, vitamin D and leukopenia.

    Science.gov (United States)

    Simioni, Juliana A; Heimovski, Flavia; Skare, Thelma L

    2016-01-01

    Immune regulation is among the noncalcemic effects of vitamin D. So, this vitamin may play a role in autoimmune diseases such as systemic lupus erythematosus (SLE). To study the prevalence of vitamin D deficiency in SLE and its association with clinical, serological and treatment profile as well as with disease activity. Serum OH vitamin D3 levels were measured in 153 SLE patients and 85 controls. Data on clinical, serological and treatment profile of lupus patients were obtained through chart review. Blood cell count and SLEDAI (SLE disease activity index) were measured simultaneously with vitamin D determination. SLE patients have lower levels of vitamin D than controls (p=0.03). In univariate analysis serum vitamin D was associated with leukopenia (p=0.02), use of cyclophosphamide (p=0.007) and methotrexate (p=0.03). A negative correlation was verified with prednisone dose (p=0.003). No association was found with disease activity measured by SLEDAI (p=0.88). In a multiple regression study only leukopenia remained as an independent association (B=4.04; p=0.02). A negative correlation of serum vitamin level with granulocyte (p=0.01) was also found, but not with lymphocyte count (p=0.33). SLE patients have more deficiency of vitamin D than controls. This deficiency is not associated with disease activity but with leucopenia (granulocytopenia). Copyright © 2015 Elsevier Editora Ltda. All rights reserved.

  16. Serious Infections among Adult Medicaid Beneficiaries with Systemic Lupus Erythematosus and Lupus Nephritis

    Science.gov (United States)

    Feldman, Candace H.; Hiraki, Linda T.; Winkelmayer, Wolfgang C.; Marty, Francisco M.; Franklin, Jessica M.; Kim, Seoyoung C.; Costenbader, Karen H.

    2015-01-01

    Objective While serious infections are significant causes of morbidity and mortality in systemic lupus erythematosus (SLE), the epidemiology in a nationwide cohort of SLE and lupus nephritis (LN) patients has not been examined. Methods Using the Medicaid Analytic eXtract (MAX) database, 2000-2006, we identified patients 18-64 years with SLE and a subset with LN. We ascertained hospitalized serious infections using validated algorithms, and 30-day mortality rates. We used Poisson regression to calculate infection incidence rates (IR), and multivariable Cox proportional hazards models to calculate hazard ratios (HR) for first infection, adjusted for sociodemographics, medication use, and a SLE-specific risk adjustment index. Results We identified 33,565 patients with SLE and 7,113 with LN. There were 9,078 serious infections in 5,078 SLE patients and 3,494 infections in 1,825 LN patients. The infection IR per 100 person-years was 10.8 in SLE and 23.9 in LN. In adjusted models, in SLE, we observed increased risks of infection among males compared to females (HR 1.33, 95% CI 1.20-1.47), in Blacks compared to Whites (HR 1.14, 95% CI 1.06-1.21), and glucocorticoid users (HR 1.51, 95% CI 1.43-1.61) and immunosuppressive users (HR 1.11, 95% CI 1.03-1.20) compared with non-users. Hydroxychloroquine users had a reduced risk of infection compared to non-users (HR 0.73, 95% CI 0.68-0.77). The 30-day mortality rate per 1,000 person-years among those hospitalized with infections was 21.4 in SLE and 38.7 in LN. Conclusion In this diverse, nationwide cohort of SLE patients, we observed a substantial burden of serious infections with many subsequent deaths. PMID:25772621

  17. Coexistence of systemic lupus erythematosus and multiple sclerosis. A case report and literature review.

    Science.gov (United States)

    Jácome Sánchez, Elisa Carolina; García Castillo, María Ariana; González, Victor Paredes; Guillén López, Fernando; Correa Díaz, Edgar Patricio

    2018-01-01

    Multiple sclerosis (MS) and systemic lupus erythematous (SLE) are autoimmune diseases, the coexistence of which is uncommon in patients. Owing to the rarity of this condition, the distinction between MS and SLE is a diagnostic challenge for neurologists. We present a case report in which MS and SLE were present in the same patient. There are few case reports in the world on the association between MS and SLE. The following case report is the first of its kind in which both MS and SLE are present in a patient from a country with low prevalence of MS such as Ecuador.

  18. Different types of headache in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Badry, Reda; Gamal, Rania M

    2015-05-01

    Headache in patients with systemic lupus erythematosus (SLE) is considered a common neurological finding, although the relationship is unclear. In this study, we aimed to evaluate frequency and characteristics of different types of headache in patients with SLE. 40 SLE patients were chosen from those referred to the out patient clinic using the American College of Rheumatology (ACR) criteria for the diagnosis of SLE. Headache classification was done regarding the ICD-II criteria in the patients. Headache severity was assessed by visual analog scale (VAS), and subjects with VAS ≥4 were included in the study. 30 patients out of 40 SLE patients (75%) have different headache types: tension type in 37.5% (n = 15) and migraine in 30% (n = 12), cluster 2.5% (n = 1), and intracranial hypertension 5% (n = 2) of all patients. Headache is frequent in SLE especially tension and migraine types, but overall, it is not associated with disease activity.

  19. 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...

  20. Hydrodynamic Limit of Multiple SLE

    Science.gov (United States)

    Hotta, Ikkei; Katori, Makoto

    2018-04-01

    Recently del Monaco and Schleißinger addressed an interesting problem whether one can take the limit of multiple Schramm-Loewner evolution (SLE) as the number of slits N goes to infinity. When the N slits grow from points on the real line R in a simultaneous way and go to infinity within the upper half plane H, an ordinary differential equation describing time evolution of the conformal map g_t(z) was derived in the N → ∞ limit, which is coupled with a complex Burgers equation in the inviscid limit. It is well known that the complex Burgers equation governs the hydrodynamic limit of the Dyson model defined on R studied in random matrix theory, and when all particles start from the origin, the solution of this Burgers equation is given by the Stieltjes transformation of the measure which follows a time-dependent version of Wigner's semicircle law. In the present paper, first we study the hydrodynamic limit of the multiple SLE in the case that all slits start from the origin. We show that the time-dependent version of Wigner's semicircle law determines the time evolution of the SLE hull, K_t \\subset H\\cup R, in this hydrodynamic limit. Next we consider the situation such that a half number of the slits start from a>0 and another half of slits start from -a exact solutions, we will discuss the universal long-term behavior of the multiple SLE and its hull K_t in the hydrodynamic limit.

  1. Lupus nephritis management guidelines compared.

    Science.gov (United States)

    Wilhelmus, Suzanne; Bajema, Ingeborg M; Bertsias, George K; Boumpas, Dimitrios T; Gordon, Caroline; Lightstone, Liz; Tesar, Vladimir; Jayne, David R

    2016-06-01

    In the past years, many (randomized) trials have been performed comparing the treatment strategies for lupus nephritis. In 2012, these data were incorporated in six different guidelines for treating lupus nephritis. These guidelines are European, American and internationally based, with one separate guideline for children. They offer information on different aspects of the management of lupus nephritis including induction and maintenance treatment of the different histological classes, adjunctive treatment, monitoring of the patient, definitions of response and relapse, indications for (repeat) renal biopsy, and additional challenges such as the presence of vascular complications, the pregnant SLE patient, treatment in children and adolescents and considerations about end-stage renal disease and transplantation. In this review, we summarize the guidelines, determine the common ground between them, highlight the differences and discuss recent literature. © The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  2. Soroprevalência e genótipos do vírus da hepatite C em pacientes com lúpus eritematoso sistêmico (LES em Goiânia, Brasil Hepatitis C virus seroprevalence and genotypes in patients with systemic lupus erythematosus (SLE in Goiânia, Brazil

    Directory of Open Access Journals (Sweden)

    Vitalina de Souza Barbosa

    2005-08-01

    (HCV infection is a source of concern in rheumatology because of its extrahepatic manifestations. Many studies have reported association between HCV infection and rheumatological manifestations such as: musculoskeletal pain, essential mixed cryoglobulinemia, rheumatoid arthritis, Sjögren's syndrome, vasculitis, glomerulonephritis, Raynaud's phenomenon, polyarteritis nodosa, myositis, autoantibody and other connective tissue diseases. In previous studies developed in our region, prevalences of 0.9%, 1.4%, 1.8% and 2.0% were detected among pregnant women, blood donors, leprosy patients and health professionals, respectively. OBJECTIVE: to investigate the prevalence of hepatitis C virus infection among patients with systemic lupus erythematosus (SLE in Goiânia, Brazil. METHODS: 175 patients were interviewed and had blood samples tested for HCV antibodies (anti-HCV by a third generation enzyme linked immunosorbant assay (ELISA. RNA-HCV was detected by polymerase chain reaction (PCR with primers complementary the 5' non-coding region of the HCV genoma, in all anti-HCV positive serum samples and genotyped by a line probe assay. RESULTS: an overall HCV infection prevalence of 2.3% (4/175 was found. Genotyping of RNA-HCV positive samples revealed HCV type 1 in 3 (75% and type 3 in 1 (25% patient. Clinical course was favorable in all HCV positive patients, except one, who died due to renal insuficiency related to lupus nephritis. CONCLUSIONS: anti-HCV prevalence among patients with SLE was slitghly higher than the prevanlence observed in pregnant women, healthy blood donors and leprosy patients, and similar to health professionals.

  3. 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/biochemistry, serol......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...

  4. Central nervous system lupus erythematosus in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Yokota, Shumpei; Kimura, Kazue; Yoshida, Naotaka; Mitsuda, Toshihiro; Ibe, Masa-aki; Shimizu, Hiroko (Yokohama City Univ. (Japan). Faculty of Medicine)

    1989-12-01

    Clinical features of central nervous system (CNS) invlvement in childhood systemic lupus erythematosus (SLE) was investigated. Neuropsychiatric manifestations including seizures, chorea, headache, overt psychosis, tremor, increase of muscle spastisity, and disturbed memory were found in 47% of 15 patients with SLE. There was a well correlatin between CNS abnormalities and SLE disease activity judged by serum complement levels and anti-nuclear antibody and anti-DNA antibody titers. The administration of Prednisolon was effective for the treatment of these CNS abnormalities and steroid psychosis was rare in the present study. EEG abnormalities involving diffuse slowing and slowing bursts were found in 73% of the patients. Cranial CT scan revealed basel ganglia calcifications in 2 patients, and marked brain atrophy in 3 patients. This study indicated that in the long term following of SLE children CNS abnormalities need to be serially checked by EEG and cranial CT scans as well as serological investigations. (author).

  5. 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.

  6. Central nervous system lupus erythematosus in childhood

    International Nuclear Information System (INIS)

    Yokota, Shumpei; Kimura, Kazue; Yoshida, Naotaka; Mitsuda, Toshihiro; Ibe, Masa-aki; Shimizu, Hiroko

    1989-01-01

    Clinical features of central nervous system (CNS) invlvement in childhood systemic lupus erythematosus (SLE) was investigated. Neuropsychiatric manifestations including seizures, chorea, headache, overt psychosis, tremor, increase of muscle spastisity, and disturbed memory were found in 47% of 15 patients with SLE. There was a well correlatin between CNS abnormalities and SLE disease activity judged by serum complement levels and anti-nuclear antibody and anti-DNA antibody titers. The administration of Prednisolon was effective for the treatment of these CNS abnormalities and steroid psychosis was rare in the present study. EEG abnormalities involving diffuse slowing and slowing bursts were found in 73% of the patients. Cranial CT scan revealed basel ganglia calcifications in 2 patients, and marked brain atrophy in 3 patients. This study indicated that in the long term following of SLE children CNS abnormalities need to be serially checked by EEG and cranial CT scans as well as serological investigations. (author)

  7. The involvement of galectin-3 in skin injury in systemic lupus erythematosus patients.

    Science.gov (United States)

    Shi, Z; Meng, Z; Han, Y; Cao, C; Tan, G; Wang, L

    2018-04-01

    Objective Our previous research suggested that anti-galectin-3 antibody was highly associated with the development of lupus skin lesions in systemic lupus erythematosus (SLE). In this study we aimed to investigate the involvement of galectin-3 in SLE skin damage. Methods The study consisted of 49 patients with SLE, 16 with dermatomyositis and 11 with systemic scleroderma and 20 healthy controls. Galectin-3 was examined by ELISA and immunohistochemical staining in serum and skin, respectively. Results Serum galectin-3 was significantly higher in patients with SLE than in those with dermatomyositis ( P  0.05). As for subtypes of skin lesions in SLE, galectin-3 expression was lower in chronic cutaneous lupus erythematosus than in acute cutaneous lupus erythematosus ( P = 0.0439). Conclusion Serum galectin-3 is unlikely to play a role in the pathogenesis of lupus skin damage, but can be a potential biomarker for the measurement of SLE disease activity. Galectin-3 is greatly reduced in patients with lupus lesions compared with healthy controls, which may contribute to the recruitment of inflammatory cells in the skin.

  8. 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...

  9. Impaired Cytokine Responses to Epstein-Barr Virus Antigens in Systemic Lupus Erythematosus Patients

    DEFF Research Database (Denmark)

    Draborg, Anette Holck; Sandhu, Noreen; Larsen, Nanna

    2016-01-01

    We analyzed cytokine responses against latent and lytic Epstein-Barr virus (EBV) antigens in systemic lupus erythematosus (SLE) patients and healthy controls (HCs) to obtain an overview of the distinctive immune regulatory response in SLE patients and to expand the previously determined impaired...

  10. Urinary albumin and beta 2-microglobulin excretion rates in patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Parving, H H; Sørensen, S F; Mogensen, C E

    1980-01-01

    The daily urinary albumin and beta 2-microglobulin excretion rates were measured with sensitive radioimmunoassays in 14 patients with systemic lupus erythematosus (SLE). The duration of SLE ranged from 0.5 to 18 years, mean 10 years. The mean age was 37 years. All patients except 5 received...

  11. Cutaneous lupus erythematosus and the risk of deep venous thrombosis and pulmonary embolism

    DEFF Research Database (Denmark)

    Ahlehoff, O; Wu, Jashin J; Raunsø, Jakob

    2017-01-01

    Background Venous thromboembolism (VTE) is a major public health concern. Lupus erythematosus (LE) is a chronic autoimmune disease ranging from localized cutaneous disease (CLE) to systemic involvement (SLE). Patients with SLE have an increased risk of venous thromboembolism (VTE), but little...

  12. 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

  13. 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

  14. Safety and efficacy of influenza vaccination in systemic lupus erythematosus patients with quiescent disease

    NARCIS (Netherlands)

    Holvast, A; Huckriede, A; Wilschut, J; Horst, G; De Vries, JJC; Benne, CA; Kallenberg, CGM; Bijl, M

    Objective: to assess the safety and efficacy of influenza vaccination in patients with systemic lupus erythematosus (SLE), and to evaluate the influence of immunosuppressive drugs on the immune response. Methods: SLE patients (n = 56) and healthy controls (n = 18) were studied. All patients had

  15. ِAssessment of lupus nephritis by measuring urinary retinol binding ...

    African Journals Online (AJOL)

    Ehab

    Background: Renal disease is a common manifestation of systemic lupus erythematosus (SLE). Both glomerular and ... Objective: The study was aimed to assess the urinary RPB level in SLE patients with and without evidence of .... Twenty-one patients were receiving prednisone (1-2 mg/Kg/day) either alone (n= 13) or.

  16. Imaging of systemic lupus erythematosus. Part II: Gastrointestinal, renal, and musculoskeletal manifestations

    International Nuclear Information System (INIS)

    Goh, Y.P.; Naidoo, P.; Ngian, G.-S.

    2013-01-01

    Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that has a relapsing and remitting course. It has a wide range of presentations with various organ manifestations. In this review, we have compiled the radiological findings of gastrointestinal, renal, and musculoskeletal manifestations of SLE.

  17. 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.

  18. 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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  20. 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...

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

    International Nuclear Information System (INIS)

    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. (orig.)

  2. Are Toll-Like Receptors and Decoy Receptors Involved in the Immunopathogenesis of Systemic Lupus Erythematosus and Lupus-Like Syndromes?

    Directory of Open Access Journals (Sweden)

    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.

  3. Hemorrhage listerial encephalitis in a patient with systemic lupus erythematosis: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hyun Gyu; Lee, Ji Young; Lee, Young Jun; Kim, Young Seo; Kim, Hyun Young; Sung, Won Jae [Hanyang University Hospital, Hanyang University College of Medicine, Seoul (Korea, Republic of)

    2017-05-15

    A 31-year-old female with systemic lupus erythematosus (SLE) presented with fever, headache, seizures and mental status changes. Brain MRI showed T2 hyperintense lesions in the cerebellum and frontal white matter and a lesion in the cerebellum exhibited hemorrhagic changes and peripheral ring enhancement. The MRI features of listerial encephalitis are difficult to differentiate from those of neuropsychiatric SLE and various other diseases. Here, we report a case of hemorrhagic listerial encephalitis in a patient with SLE.

  4. Non-Hodgkin's lymphoma in patients with systemic lupus erythematosus: 2 case reports

    Energy Technology Data Exchange (ETDEWEB)

    Ferri, M. [Hamilton Health Sciences Corp., Dept. of Radiology, Hamilton, Ontario (Canada); Mar, C.; Bhatia, R.S. [Memorial Univ. of Newfoundland, Health Sciences Centre, Discipline of Radiology, St. John' s Newfoundland (Canada)

    2002-04-01

    The association between autoimmune rheumatic diseases and malignancy, and between lymphoproliferative disorders and systemic lupus erythematosus (SLE), in particular, has been documented. Although the imaging features of pulmonary lymphoma and of pulmonary manifestations of SLE have been described separately, the imaging features of the 2 together have not been demonstrated. We present the cases of 2 patients with SLE presenting with non-Hodgkin's lymphoma (NHL). (author)

  5. Non-Hodgkin's lymphoma in patients with systemic lupus erythematosus: 2 case reports

    International Nuclear Information System (INIS)

    Ferri, M.; Mar, C.; Bhatia, R.S.

    2002-01-01

    The association between autoimmune rheumatic diseases and malignancy, and between lymphoproliferative disorders and systemic lupus erythematosus (SLE), in particular, has been documented. Although the imaging features of pulmonary lymphoma and of pulmonary manifestations of SLE have been described separately, the imaging features of the 2 together have not been demonstrated. We present the cases of 2 patients with SLE presenting with non-Hodgkin's lymphoma (NHL). (author)

  6. Bilateral acute lupus pneumonitis in a case of rhupus syndrome

    Directory of Open Access Journals (Sweden)

    Supriya Sarkar

    2012-01-01

    Full Text Available Rhupus syndrome, the overlap of rheumatoid arthritis (RA and systemic lupus erythematosus (SLE, is an extremely uncommon condition. Organ damages found due to SLE are usually mild in rhupus. Lupus pneumonitis in rhupus syndrome has not been reported worldwide. We are reporting a 23-year-old female with bilateral symmetric erosive arthritis, oral ulcer, alopecia, polyserositis, anemia, leucopenia, positive RA-factor, anti nuclear antibody (ANA and anti ds-DNA. She presented with acute onset dyspnea, high fever, chest pain, tachycardia, tachypnea, hypoxia and respiratory alkalosis. High resolution computed tomography (HRCT-thorax showed bilateral, basal consolidation with air bronchogram. Repeated sputum and single broncho alveolar lavage (BAL fluid examination revealed no organism or Hemosiderin-laden macrophage. The diagnosis of rhupus was confirmed by combined manifestations of RA and SLE, and the diagnosis of acute lupus pneumonitis was established by clinico-radiological picture and by excluding other possibilities.

  7. Lupus Nephritis

    Science.gov (United States)

    ... in men and most often strikes during the child-bearing years. Nine out of 10 people who have lupus are women. Lupus is also more common in people of African or Asian background. African Americans and Asian Americans ...

  8. Mitral Valve Surgery in Patients with Systemic Lupus Erythematosus

    Science.gov (United States)

    Hekmat, Manouchehr; Ghorbani, Mohsen; Ghaderi, Hamid; Majidi, Masoud; Beheshti, Mahmood

    2014-01-01

    Valvular heart disease is the common cardiac manifestation of systemic lupus erythematosus (SLE) with a tendency for mitral valve regurgitation. In this study we report a case of mitral valve replacement for mitral stenosis caused by Libman-Sacks endocarditis in the setting of SLE. In addition, we provide a systematic review of the literature on mitral valve surgery in the presence of Libman-Sacks endocarditis because its challenge on surgical options continues. Surgical decision depends on structural involvement of mitral valve and presence of active lupus nephritis and antiphospholipid antibody syndrome. Review of the literature has also shown that outcome is good in most SLE patients who have undergone valvular surgery, but association of antiphospholipid antibody syndrome with SLE has negative impact on the outcome. PMID:25401131

  9. Pleuro-pulmonary abnormalities in patients with systemic lupus erythematosus assessment with high resolution computed tomography, chest radiography and pulmonary function tests

    Energy Technology Data Exchange (ETDEWEB)

    Sant, S.M.; Doran, M.; Fenelon, H.M.; Breatnach, E.S. [University of Michigan (United States)

    1998-04-01

    The objective has to assess the nature of pleuro-pulmonary abnormalities, with particular reference to interstitial lung disease (ILD), in patients with systemic lupus erythematosus (SLE) In conclusion, HRCT is more sensitive than PFTs or CXR in the evaluation of pleuro-pulmonary disease in SLE. We report an unusually high prevalence of HRCT appearances suggestive of ILD in patients with SLE. subclinical lung disease is common in patients with SLE. (author)

  10. Pleuro-pulmonary abnormalities in patients with systemic lupus erythematosus assessment with high resolution computed tomography, chest radiography and pulmonary function tests

    International Nuclear Information System (INIS)

    Sant, S.M.; Doran, M.; Fenelon, H.M.; Breatnach, E.S.

    1998-01-01

    The objective has to assess the nature of pleuro-pulmonary abnormalities, with particular reference to interstitial lung disease (ILD), in patients with systemic lupus erythematosus (SLE) In conclusion, HRCT is more sensitive than PFTs or CXR in the evaluation of pleuro-pulmonary disease in SLE. We report an unusually high prevalence of HRCT appearances suggestive of ILD in patients with SLE. subclinical lung disease is common in patients with SLE. (author)

  11. Molecular pathology of systemic lupus erythematosus in Asians

    OpenAIRE

    Chai, Hwa Chia

    2017-01-01

    Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease affecting various parts of the body. Polymorphisms in genes involved in toll-like receptor (TLR)/interferon (IFN) signalling pathways have been reported previously to be associated with SLE in many populations. This study aimed to investigate the role of seven single nucleotide polymorphisms (SNPs) within TNFAIP3 (rs2230936 and rs3757173), STAT4 (rs7574865, rs10168266, and rs7601754), and IRF5 (rs4728142 and rs729302), that...

  12. Current and emerging treatment options in the management of lupus

    Science.gov (United States)

    Jordan, Natasha; D’Cruz, David

    2016-01-01

    Systemic lupus erythematosus (SLE) is a complex autoimmune disease with variable clinical manifestations. While the clearest guidelines for the treatment of SLE exist in the context of lupus nephritis, patients with other lupus manifestations such as neuropsychiatric, hematologic, musculoskeletal, and severe cutaneous lupus frequently require immunosuppression and/or biologic therapy. Conventional immunosuppressive agents such as mycophenolate mofetil, azathioprine, and cyclophosphamide are widely used in the management of SLE with current more rationalized treatment regimens optimizing the use of these agents while minimizing potential toxicity. The advent of biologic therapies has advanced the treatment of SLE particularly in patients with refractory disease. The CD20 monoclonal antibody rituximab and the anti-BLyS agent belimumab are now widely in use in clinical practice. Several other biologic agents are in ongoing clinical trials. While immunosuppressive and biologic agents are the foundation of inflammatory disease control in SLE, the importance of managing comorbidities such as cardiovascular risk factors, bone health, and minimizing susceptibility to infection should not be neglected. PMID:27529058

  13. A framework for remission in SLE: consensus findings from a large international task force on definitions of remission in SLE (DORIS).

    Science.gov (United States)

    van Vollenhoven, Ronald; Voskuyl, Alexandre; Bertsias, George; Aranow, Cynthia; Aringer, Martin; Arnaud, Laurent; Askanase, Anca; Balážová, Petra; Bonfa, Eloisa; Bootsma, Hendrika; Boumpas, Dimitrios; Bruce, Ian; Cervera, Ricard; Clarke, Ann; Coney, Cindy; Costedoat-Chalumeau, Nathalie; Czirják, László; Derksen, Ronald; Doria, Andrea; Dörner, Thomas; Fischer-Betz, Rebecca; Fritsch-Stork, Ruth; Gordon, Caroline; Graninger, Winfried; Györi, Noémi; Houssiau, Frédéric; Isenberg, David; Jacobsen, Soren; Jayne, David; Kuhn, Annegret; Le Guern, Veronique; Lerstrøm, Kirsten; Levy, Roger; Machado-Ribeiro, Francinne; Mariette, Xavier; Missaykeh, Jamil; Morand, Eric; Mosca, Marta; Inanc, Murat; Navarra, Sandra; Neumann, Irmgard; Olesinska, Marzena; Petri, Michelle; Rahman, Anisur; Rekvig, Ole Petter; Rovensky, Jozef; Shoenfeld, Yehuda; Smolen, Josef; Tincani, Angela; Urowitz, Murray; van Leeuw, Bernadette; Vasconcelos, Carlos; Voss, Anne; Werth, Victoria P; Zakharova, Helena; Zoma, Asad; Schneider, Matthias; Ward, Michael

    2017-03-01

    Treat-to-target recommendations have identified 'remission' as a target in systemic lupus erythematosus (SLE), but recognise that there is no universally accepted definition for this. Therefore, we initiated a process to achieve consensus on potential definitions for remission in SLE. An international task force of 60 specialists and patient representatives participated in preparatory exercises, a face-to-face meeting and follow-up electronic voting. The level for agreement was set at 90%. The task force agreed on eight key statements regarding remission in SLE and three principles to guide the further development of remission definitions:1. Definitions of remission will be worded as follows: remission in SLE is a durable state characterised by …………………. (reference to symptoms, signs, routine labs).2. For defining remission, a validated index must be used, for example, clinical systemic lupus erythematosus disease activity index (SLEDAI)=0, British Isles lupus assessment group (BILAG) 2004 D/E only, clinical European consensus lupus outcome measure (ECLAM)=0; with routine laboratory assessments included, and supplemented with physician's global assessment.3. Distinction is made between remission off and on therapy: remission off therapy requires the patient to be on no other treatment for SLE than maintenance antimalarials; and remission on therapy allows patients to be on stable maintenance antimalarials, low-dose corticosteroids (prednisone ≤5 mg/day), maintenance immunosuppressives and/or maintenance biologics.The task force also agreed that the most appropriate outcomes (dependent variables) for testing the prognostic value (construct validity) of potential remission definitions are: death, damage, flares and measures of health-related quality of life. The work of this international task force provides a framework for testing different definitions of remission against long-term outcomes. Published by the BMJ Publishing Group Limited. For

  14. 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...

  15. Gastrointestinal symptomatology as first manifestation of systemic erythematous lupus

    Directory of Open Access Journals (Sweden)

    Kovačević Zoran

    2009-01-01

    Full Text Available Background. Systemic lupus erithematodes (SLE is chronic, often febrile, multisystemic disease unknown origin and relapsing course which affects connective tissue of the skin, joints, kidney and serous membranes. Gastrointestinal manifestations are rarely the first sign of systemic lupus erythematosus. Case report. We presented a female patient, 35 years old, whose first symptoms of SLE were paralitic ileus (chronic intestinal pseudo-obstruction and polyserositis (pleural effusion and ascites. Except for high parameters of inflammation, leucopenia and thrombocytopenia, all immunological and laboratory tests for SLE were negative in the onset of the disease. During next six months the patient had clinical signs of paralitic ileus several times and was twice operated with progressive malabsorptive syndrome. The full picture of SLE was manifested seven months later associated with lupus nephritis. Treatment with cyclophosphamide, corticosteroids and total parenteral nutrition (30 days induced stable remission of the disease. Conclusion. The SLE can be initially manifested with gastroenterological symptoms without any other clinical and immunologic parameters of the disease. If in patients with SLE and gastrointestinal tract involvement malabsorption syndrom is developed, a treatment success depends on both immunosupressive therapy and total parenteral nutrition.

  16. What Social Workers in Health Care Should Know about Lupus: A Structural Equation Model

    Science.gov (United States)

    Auerbach, Charles; Beckerman, Nancy L.

    2011-01-01

    This article reports on findings from a cross-sectional study (N = 378) of patients living with systemic lupus erythematosus (SLE). The purpose of this study was to identify and clarify the unique psychosocial challenges for those living with lupus. The specific analysis will help to develop a model to determine how different factors influence SLE…

  17. Renal expression of polyomavirus large T antigen is associated with nephritis in human systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Fenton, Kristin Andreassen; Mjelle, Janne Erikke; Jacobsen, Søren

    2008-01-01

    ) that these complexes bound induced anti-nucleosome antibodies and finally (iv) that they associated with glomerular membranes as immune complexes. This process may be relevant for human lupus nephritis, since productive polyomavirus infection is associated with this organ manifestation. Here, we compare nephritis...... to the evolution of lupus nephritis in human SLE....

  18. [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.

  19. Ficolins and the lectin pathway of complement in patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Hein, Estrid; Nielsen, Louise Aas; Nielsen, Christoffer T

    2015-01-01

    The complement system plays a pathophysiological role in systemic lupus erythematosus (SLE). This study aims to investigate whether an association exists between the ficolins that are part of the lectin complement pathway and SLE. EDTA plasma samples from 68 Danish SLE patients and 29 healthy...... Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index] (SDI) (Rho=0.27, P=0.026). The Ficolin-1 concentration was also associated with the occurrence of arterial (P=0.0053) but not venous thrombosis (P=0.42). Finally, deposition of C4, C3 and TCC...

  20. Systemic lupus erythematosus in Spanish males: a study of the Spanish Rheumatology Society Lupus Registry (RELESSER) cohort.

    Science.gov (United States)

    Riveros Frutos, A; Casas, I; Rúa-Figueroa, I; López-Longo, F J; Calvo-Alén, J; Galindo, M; Fernández-Nebro, A; Pego-Reigosa, J M; Olivé Marqués, A

    2017-06-01

    Objective The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. Methods A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). Results Mean ages (18-92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women). Male/female ratio was 1/9. Age of onset of symptoms and age at diagnosis were higher in men than in women ( p lupus nephritis was more common in men, being present in 155 (44.8%) of males versus 933 (29%) of females ( p  50 years had a higher mortality (odds ratios 3.6 and 2.1, respectively). Furthermore, SLE patients who developed pulmonary hemorrhage, pulmonary hypertension, psychiatric involvement, complement deficiency, and hemophagocytic syndrome also had higher mortality, regardless of gender. Conclusion Patients with SLE over the age of 50 years have an increased risk of mortality. In Caucasians, age at diagnosis and symptom onset is higher in men than in women. The diagnostic delay is shorter in men. Male SLE patients present more cardiovascular comorbidities, and also more serositis, adenopathies, splenomegaly, renal involvement, convulsion, thrombosis, and lupus anticoagulant positivity than women.

  1. 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 ...

  2. Systemic Lupus Erythematosus Associated with Extreme Hypertriglyceridemia

    Directory of Open Access Journals (Sweden)

    Chin-Sung Huang

    2008-04-01

    Full Text Available Only a few cases of hypertriglyceridemia in patients with systemic lupus erythematosus (SLE have been reported. We report a case of a 13-year-old girl suffering from SLE associated with severe hypertriglyceridemia. The persistent hypertriglyceridemia was extremely well tolerated. As a result of steroid treatment, serum triglycerides fell dramatically from a high of 5601 mg/dL to 75 mg/dL despite the patient switching to a free diet. We considered the presence of an autoantibody to lipoprotein lipase and commenced immunosuppression. The role of steroids in completely correcting deficient lipoprotein lipase activity is discussed.

  3. 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...

  4. 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.

  5. The pathway of estradiol-induced apoptosis in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Rastin, Maryam; Hatef, Mohammad Reza; Tabasi, Nafisseh; Mahmoudi, Mahmoud

    2012-03-01

    Systemic lupus erythematosus (SLE) is a disease with unknown etiology. The pathologic role of sex hormones and apoptosis in SLE has often been discussed. We studied the effects of estradiol in the pathway of induced apoptosis in Iranian SLE patients. T lymphocytes from 35 SLE patients and 20 age-matched controls were isolated and cultured in the presence of 10(-8) M 17-β estradiol. The expression levels of Fas, Fas ligand (FasL), Bcl-2, caspase-8, and caspase-9 mRNAs were determined semiquantitatively in comparison to the expression level of beta actin RNA. Estradiol exposure did not have any significant effects on the expression levels of Fas, Bcl-2, and caspase-9 in SLE patients and controls. However, the expression levels of FasL and caspase-8 were significantly increased in SLE patients, but not in controls. This suggests the probable involvement of extrinsic apoptosis pathway in estradiol-induced apoptosis in SLE.

  6. Immunoregulation of NKT Cells in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Chen, Junwei; Wu, Meng; Wang, Jing; Li, Xiaofeng

    2015-01-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with different variety of clinical manifestations. Natural killer T (NKT) cells are innate lymphocytes that play a regulatory role during broad range of immune responses. A number of studies demonstrated that the quantity and quality of invariant NKT (iNKT) cells showed marked defects in SLE patients in comparison to healthy controls. This finding suggests that iNKT cells may play a regulatory role in the occurrence and development of this disease. In this review, we mainly summarized the most recent findings about the behavior of NKT cells in SLE patients and mouse models, as well as how NKT cells affect the proportion of T helper cells and the production of autoreactive antibodies in the progress of SLE. This will help people better understand the role of NKT cells in the development of SLE and improve the therapy strategy.

  7. 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

  8. Periodontal disease in Chinese patients with systemic lupus erythematosus.

    Science.gov (United States)

    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.

  9. Splenectomy increases the subsequent risk of systemic lupus erythematosus.

    Science.gov (United States)

    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.

  10. 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.

  11. B cell signature during inactive systemic lupus is heterogeneous: toward a biological dissection of lupus.

    Directory of Open Access Journals (Sweden)

    Jean-Claude Garaud

    Full Text Available Systemic lupus erythematosous (SLE is an autoimmune disease with an important clinical and biological heterogeneity. B lymphocytes appear central to the development of SLE which is characterized by the production of a large variety of autoantibodies and hypergammaglobulinemia. In mice, immature B cells from spontaneous lupus prone animals are able to produce autoantibodies when transferred into immunodeficient mice, strongly suggesting the existence of intrinsic B cell defects during lupus. In order to approach these defects in humans, we compared the peripheral B cell transcriptomas of quiescent lupus patients to normal B cell transcriptomas. When the statistical analysis is performed on the entire group of patients, the differences between patients and controls appear quite weak with only 14 mRNA genes having a false discovery rate ranging between 11 and 17%, with 6 underexpressed genes (PMEPA1, TLR10, TRAF3IP2, LDOC1L, CD1C and EGR1. However, unforced hierarchical clustering of the microarrays reveals a subgroup of lupus patients distinct from both the controls and the other lupus patients. This subgroup has no detectable clinical or immunological phenotypic peculiarity compared to the other patients, but is characterized by 1/an IL-4 signature and 2/the abnormal expression of a large set of genes with an extremely low false discovery rate, mainly pointing to the biological function of the endoplasmic reticulum, and more precisely to genes implicated in the Unfolded Protein Response, suggesting that B cells entered an incomplete BLIMP1 dependent plasmacytic differentiation which was undetectable by immunophenotyping. Thus, this microarray analysis of B cells during quiescent lupus suggests that, despite a similar lupus phenotype, different biological roads can lead to human lupus.

  12. Systemic lupus erythematosus in a male patient

    Science.gov (United States)

    Sibarani, H.; Zubir, Z.

    2018-03-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder with a broad spectrum of clinical presentations. Female to male ratio is approximately 9:1.A 20 years old male was admitted to HAM Hospital 3 months ago with chief complaint pain in both knees joint. After anamneses, physical examination and laboratory test the patient was diagnosed with systemic lupus erythematosus. The patient tested positive for ANA and anti-ds-DNA antibody test. The patient was with giving non-biologic DMARDS @myfortic 360mg, methylprednisolone, chloroquine and other symptomatic drugs.

  13. Prevalence, incidence, and demographics of systemic lupus erythematosus and lupus nephritis from 2000 to 2004 among children in the US Medicaid beneficiary population.

    Science.gov (United States)

    Hiraki, Linda T; Feldman, Candace H; Liu, Jun; Alarcón, Graciela S; Fischer, Michael A; Winkelmayer, Wolfgang C; Costenbader, Karen H

    2012-08-01

    To investigate the nationwide prevalence, incidence, and sociodemographics of systemic lupus erythematosus (SLE) and lupus nephritis among children in the US Medicaid beneficiary population. Children ages 3 years to Classification of Diseases, Ninth Revision [ICD-9] code of 710.0 for SLE, each >30 days apart) were identified from the US Medicaid Analytic eXtract database from 2000 to 2004. This database contains all inpatient and outpatient Medicaid claims for 47 US states and the District of Columbia. Lupus nephritis was identified from ≥2 ICD-9 billing codes for glomerulonephritis, proteinuria, or renal failure, each recorded >30 days apart. The prevalence and incidence of SLE and lupus nephritis were calculated among Medicaid-enrolled children overall and within sociodemographic groups. Of the 30,420,597 Medicaid-enrolled children during these years, 2,959 were identified as having SLE. The prevalence of SLE was 9.73 (95% confidence interval [95% CI] 9.38-10.08) per 100,000 Medicaid-enrolled children. Among the children with SLE, 84% were female, 40% were African American, 25% were Hispanic, 21% were White, and 42% resided in the South region of the US. Moreover, of the children with SLE, 1,106 (37%) had lupus nephritis, representing a prevalence of 3.64 (95% CI 3.43-3.86) per 100,000 children. The average annual incidence of SLE was 2.22 cases (95% CI 2.05-2.40) and that of lupus nephritis was 0.72 cases (95% CI 0.63-0.83) per 100,000 Medicaid enrollees per year. The prevalence and incidence rates of SLE and lupus nephritis increased with age, were higher in girls than in boys, and were higher in all non-White racial/ethnic groups. In the current study, the prevalence and incidence rates of SLE among Medicaid-enrolled children in the US are high compared to studies in other populations. In addition, these data represent the first population-based estimates of the prevalence and incidence of lupus nephritis in the US to date. Copyright © 2012 by the American

  14. Linking susceptibility genes and pathogenesis mechanisms using mouse models of systemic lupus erythematosus

    Science.gov (United States)

    Crampton, Steve P.; Morawski, Peter A.; Bolland, Silvia

    2014-01-01

    Systemic lupus erythematosus (SLE) represents a challenging autoimmune disease from a clinical perspective because of its varied forms of presentation. Although broad-spectrum steroids remain the standard treatment for SLE, they have many side effects and only provide temporary relief from the symptoms of the disease. Thus, gaining a deeper understanding of the genetic traits and biological pathways that confer susceptibility to SLE will help in the design of more targeted and effective therapeutics. Both human genome-wide association studies (GWAS) and investigations using a variety of mouse models of SLE have been valuable for the identification of the genes and pathways involved in pathogenesis. In this Review, we link human susceptibility genes for SLE with biological pathways characterized in mouse models of lupus, and discuss how the mechanistic insights gained could advance drug discovery for the disease. PMID:25147296

  15. Linking susceptibility genes and pathogenesis mechanisms using mouse models of systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Steve P. Crampton

    2014-09-01

    Full Text Available Systemic lupus erythematosus (SLE represents a challenging autoimmune disease from a clinical perspective because of its varied forms of presentation. Although broad-spectrum steroids remain the standard treatment for SLE, they have many side effects and only provide temporary relief from the symptoms of the disease. Thus, gaining a deeper understanding of the genetic traits and biological pathways that confer susceptibility to SLE will help in the design of more targeted and effective therapeutics. Both human genome-wide association studies (GWAS and investigations using a variety of mouse models of SLE have been valuable for the identification of the genes and pathways involved in pathogenesis. In this Review, we link human susceptibility genes for SLE with biological pathways characterized in mouse models of lupus, and discuss how the mechanistic insights gained could advance drug discovery for the disease.

  16. Significance of abnormal myocardial perfusion scintigraphy in young adult patients with SLE

    International Nuclear Information System (INIS)

    Zakavi, S.R.; Kakhki, V.R.D.; Sadeghi, R.; Jokar, M.H.; Khazaei, G.

    2009-01-01

    Detection of subclinical coronary artery disease (CAD) is a potential challenge in patients with systemic lupus erythematosus (SLE) and it is suggested that myocardial perfusion single photon emission computerized tomography (SPECT) is more sensitive than exercise test in this setting. However, the significance of perfusion abnormalities in SLE patients is not well known. In this study, we evaluated the prognostic significance of myocardial perfusion defects in patients with SLE. Patients with proven diagnosis of SLE admitted to the hospital due to noncardiac problems with no history of CAD were studied. All patients underwent 99m Tc-methoxyisobutylisonitrile (MIBI) myocardial perfusion scan using dipyridamole as pharmacological stress. All patients were followed up by reviewing patients file in lupus clinic and any minor or major cardiac events were recorded. Eighteen female and two male patients with mean age of 28.2±12.05 years were included. Six patients had mild reversible perfusion defects with mean summed difference score of 2.5±1.0. Pattern of reverse redistribution (reverse fill-in) was noted in three patients. Eleven patients had normal myocardial perfusion. Hypokinesia was noted in three patients on gated images. One patient with abnormal perfusion died 21 days after imaging due to on-cardiac cause. Nineteen patients were followed for a mean time of 39.2±16.0 months. No major or minor cardiac events were noted during follow-up. Three patients (one with abnormal perfusion) had at least one readmission during follow-up period. Our study showed that myocardial perfusion abnormalities are fairly frequent in SLE patients but the defects are generally mild and do not advocate an adverse prognosis. (author)

  17. 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

  18. 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  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.

  19. Reduced response to Epstein-Barr virus antigens by T-cells in systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Draborg, Anette Holck; Jacobsen, Søren; Westergaard, Marie

    2014-01-01

    OBJECTIVE: Epstein-Barr virus (EBV) has for long been associated with systemic lupus erythematosus (SLE). In this study, we investigated the levels of latent and lytic antigen EBV-specific T-cells and antibodies in SLE patients. METHODS: T cells were analyzed by flow cytometry and antibodies were...

  20. The significance of platelet-associated immunoglobulin G in non-thrombocytopenic patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Sørensen, P G; Mickley, H; Fristed, P

    1985-01-01

    The possible pathogenetic significance of platelet-associated immunoglobulin G in systemic lupus erythematosus (SLE) has been studied, using a semiquantitative immunofluorescence technique. The study included 22 patients suffering from SLE during the period 1973-81. Thirteen patients had various ...

  1. Comprehensive approach to study complement C4 in systemic lupus erythematosus: Gene polymorphisms, protein levels and functional activity

    NARCIS (Netherlands)

    Tsang-A-Sjoe, M. W. P.; Bultink, I. E. M.; Korswagen, L. A.; van der Horst, A. [=Anneke; Rensink, I.; de Boer, M.; Hamann, D.; Voskuyl, A. E.; Wouters, D.

    2017-01-01

    Genetic variation of the genes encoding complement component C4 is strongly associated with systemic lupus erythematosus (SLE), a chronic multi-organ auto-immune disease. This study examined C4 and its isotypes on a genetic, protein, and functional level in 140 SLE patients and 104 healthy controls.

  2. Primary Adrenal Insufficiency (Addison's Disease) Associated with Systemic Lupus Erythematosus: A Rare Occurrence.

    Science.gov (United States)

    Godswill, Okwuonu Chimezie; Odigie, Ojeh-Oziegbe

    2014-10-01

    Coexistence of Addison's disease and systemic lupus erythematosus (SLE) is a rare occurrence with only few reported cases in the literature. We describe a 29-year-old woman who presented to us with clinical features of acute Addisonian crisis and SLE. Laboratory investigations were confirmatory of Addison's disease in a background of SLE. The patient made remarkable improvement on administration of steroids as replacement therapy for adrenal insufficiency and treatment of SLE. Clinicians need to have a high-index of suspicion of this possible coexistence in order to avoid the associated deleterious hemodynamic and metabolic consequences.

  3. Kluver–Bucy syndrome in one case with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Hsiu-Fen Lin

    2011-04-01

    Full Text Available Kluver–Bucy syndrome (KBS is a collection of neuropsychiatric symptoms, including visual agnosia (prosopagnosia, hypermetamorphosis, placidity, hypersexuality, and hyperorality. Although neuropsychiatric manifestation is prevalent in cases with systemic lupus erythematosus (SLE, only one literature reported a case with SLE that had KBS previously. In this article, a 37-year-old woman with SLE who developed KBS and other neuropsychiatric symptoms is presented. Brain imaging proved the relevant structural lesion. The possible explanation of pathogenesis of KBS in SLE is discussed.

  4. The selective value of computed tomography of the brain in Cerebritis due to systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Gaylis, N.B.; Altman, R.D.; Ostrov, S.; Quencer, R.

    1982-01-01

    Systemic lupus erythematosus (SLE) and steroid effects on the brain were measured by computed tomography (CT). Of 14 patients with SLE cerebritis, 10 (71%) had marked cortical atrophy and 4 (29%) minimal atrophy. None were normal by CT. Controls included 22 patients with SLE without cerebritis receiving cortiocosteroids; this group had normal CT scans in 16 (73%) and minimal cortical atrophy in the remaining 6 (27%). Follow-up CT on 5 patients with cerebritis was unchanged. CT of the brain is a minimally invasive technique for documenting SLE cerebritis. CT may also help differentiate cerebritis from the neuropsychiatric side effects of corticosteroids

  5. Endothelial dysfunction is associated with activation of the type i interferon system and platelets in patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Tydén, Helena; Lood, Christian; Gullstrand, Birgitta

    2017-01-01

    Objectives Endothelial dysfunction may be connected to cardiovascular disease (CVD) in systemic lupus erythematosus (SLE). Type I interferons (IFNs) are central in SLE pathogenesis and are suggested to induce both endothelial dysfunction and platelet activation. In this study, we investigated...... with activation of platelets and the type I IFN system. We suggest that an interplay between the type I IFN system, injured endothelium and activated platelets may contribute to development of CVD in SLE....

  6. Selective involvement of the amygdala in systemic lupus erythematosus.

    Directory of Open Access Journals (Sweden)

    Bart J Emmer

    2006-12-01

    Full Text Available BACKGROUND: Antibodies specifically affect the amygdala in a mouse model of systemic lupus erythematosus (SLE. The aim of our study was to investigate whether there is also specific involvement of the amygdala in human SLE. METHODS AND FINDINGS: We analyzed a group of 37 patients with neuropsychiatric SLE (NP-SLE, 21 patients with SLE, and a group of 12 healthy control participants with diffusion weighted imaging (DWI. In addition, in a subset of eight patients, plasma was available to determine their anti-NMDAR antibody status. From the structural magnetic resonance imaging data, the amygdala and the hippocampus were segmented, as well as the white and gray matter, and the apparent diffusion coefficient (ADC was retrieved. ADC values between controls, patients with SLE, and patients with NP-SLE were tested using analysis of variance with post-hoc Bonferroni correction. No differences were found in the gray or white matter segments. The average ADC in the amygdala of patients with NP-SLE and SLE (940 x 10(-6 mm2/s; p = 0.006 and 949 x 10(-6 mm2/s; p = 0.019, respectively was lower than in healthy control participants (1152 x 10(-6 mm2/s. Mann-Whitney analysis revealed that the average ADC in the amygdala of patients with anti-NMDAR antibodies (n = 4; 802 x 10(-6 mm2/s was lower (p = 0.029 than the average ADC of patients without anti-NMDAR antibodies (n = 4; 979 x 10(-6 mm2/s and also lower (p = 0.001 than in healthy control participants. CONCLUSIONS: This is the first study to our knowledge to observe damage in the amygdala in patients with SLE. Patients with SLE with anti-NMDAR antibodies had more severe damage in the amygdala compared to SLE patients without anti-NMDAR antibodies.

  7. Metabolic syndrome in patients with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    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.

  8. Hashimoto thyroiditis, anti-thyroid antibodies and systemic lupus erythematosus.

    Science.gov (United States)

    Posselt, Rayana T; Coelho, Vinícius N; Skare, Thelma L

    2018-01-01

    To study the prevalence of Hashimoto thyroiditis (HT), anti-thyroid autoantibodies (anti-thyroglobulin or TgAb and thyroperoxidase or TPOAb) in systemic lupus erythematosus (SLE) patients. To analyze if associated HT, TgAb and/or TPOAb influence clinical or serological profiles, disease activity and/or its cumulative damage. Three hundred and one SLE patients and 141 controls were studied for thyroid stimulating hormone, thyroxin, TgAb and TPOAb by chemiluminescence and immunometric assays. Patients' charts were reviewed for serological and clinical profiles. Activity was measured by SLE Disease Activity Index and cumulative damage by Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for SLE. SLE patients were divided into: (i) with HT; (ii) with anti-thyroid antibodies but without HT; and (iii) without HT and without anti-thyroid antibodies, and were then compared. Furthermore, SLE patients were compared according to the number of positive anti-thyroid antibodies. Hashimoto thyroiditis prevalence in SLE was 12.6% and 5.6% in controls (P = 0.02; odds ratio = 2.4; 95% CI = 1.09-5.2). Lupus patients with HT had less malar rash (P = 0.02) and more anti-Sm (P = 0.04). Anti-Sm was more common in those with two anti-thyroid antibodies than in those with one or negative. The presence of HT or the number of positive autoantibodies did not associate either with disease activity (P = 0.95) or with cumulative damage (P = 0.98). There is a two-fold increased risk of HT in SLE patients. Anti-Sm antibodies favor this association and also double antibody positivity. Disease activity and cumulative damage are not related to HT or with autoantibodies. © 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

  9. 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...

  10. 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.

  11. Lupus and leprosy: beyond the coincidence.

    Science.gov (United States)

    Ribeiro, F M; Gomez, V E; Albuquerque, E M N; Klumb, E M; Shoenfeld, Y

    2015-02-01

    Systemic lupus erythematous (SLE) is an autoimmune disease that presents an increased susceptibility to infections which may trigger reactivation. Disease flares have been mostly associated with parvovirus B19, cytomegalovirus, EBV and Mycobacterium tuberculosis infections, but it is probable that many other agents may also induce innate and adaptive immune system stimulation including the production of autoantibodies as ANA, anti nDNA and anti-ß2-GPI mainly in lepromatous leprosy. Mycobacterium leprae not only may determine symptoms that mimic lupus flares, including autoantibodies production, but could also act as a trigger for lupus reactivation; however, its association is still not fully explored. As demonstrated for tuberculosis, it is quite possible that molecular mimicry may also be involved in the interface of these two diseases. Some studies reported shared epitopes among idiotypes derived from 8E7 and TH9 lepromatous antibodies and those obtained from SLE patients, and it could partially explain the triggering phenomenon of SLE caused by M. leprae. We report and discuss three Brazilian patients whose disease was inactive and presented disease flares concurrently with the diagnosis of leprosy.

  12. Micromanagement of lupus autoimmunity by microRNAs

    NARCIS (Netherlands)

    Shen, N.

    2012-01-01

    De observaties die Nan Shen deed in zijn promotieonderzoek verschaffen nieuwe inzichten in het ontstaan van de auto-immuunziekte systemische lupus erythematodes (SLE). Ook dragen ze bij aan De observaties die Nan Shen deed in zijn promotieonderzoek verschaffen nieuwe inzichten in het ontstaan van de

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

    African Journals Online (AJOL)

    Systemic lupus erythematosus (SLE) is a chronic, recurrent multi- systemic ... present with many years' history of nonspecific symptoms that are frequently attributed to other ... the assumed diagnoses of peptic ulcer disease, reflux oesophagitis, gastritis and ... The jugular venous pressure was not elevated. The apex beat ...

  14. 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 ...

  15. 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...

  16. Autoantibody Profiling in Lupus Patients using Synthetic Nucleic Acids

    DEFF Research Database (Denmark)

    Klecka, Martin; Thybo, Christina; Macaubas, Claudia

    2018-01-01

    Autoantibodies to nuclear components of cells (antinuclear antibodies, ANA), including DNA (a-DNA), are widely used in the diagnosis and subtyping of certain autoimmune diseases, including systemic lupus erythematosus (SLE). Despite clinical use over decades, precise, reproducible measurement of a...

  17. Aquaporin-4-autoimmunity in patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Asgari, Nasrin; Jarius, Sven; Laustrup, Helle

    2018-01-01

    BACKGROUND: Serum immunoglobulin G targeting the astrocyte water channel aquaporin-4 (AQP4) in the central nervous system (CNS) is a biomarker for neuromyelitis optica spectrum disease (NMOSD). Co-existence of NMOSD with systemic lupus erythematosus (SLE) putatively suggests susceptibility...

  18. 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...

  19. Tofacitinib Ameliorates Murine Lupus and Its Associated Vascular Dysfunction.

    Science.gov (United States)

    Furumoto, Yasuko; Smith, Carolyne K; Blanco, Luz; Zhao, Wenpu; Brooks, Stephen R; Thacker, Seth G; Abdalrahman, Zarzour; Sciumè, Giuseppe; Tsai, Wanxia L; Trier, Anna M; Nunez, Leti; Mast, Laurel; Hoffmann, Victoria; Remaley, Alan T; O'Shea, John J; Kaplan, Mariana J; Gadina, Massimo

    2017-01-01

    Dysregulation of innate and adaptive immune responses contributes to the pathogenesis of systemic lupus erythematosus (SLE) and its associated premature vascular damage. No drug to date targets both systemic inflammatory disease and the cardiovascular complications of SLE. Tofacitinib is a JAK inhibitor that blocks signaling downstream of multiple cytokines implicated in lupus pathogenesis. While clinical trials have shown that tofacitinib exhibits significant clinical efficacy in various autoimmune diseases, its role in SLE and the associated vascular pathology remains to be characterized. MRL/lpr lupus-prone mice were administered tofacitinib or vehicle by gavage for 6 weeks (therapeutic arm) or 8 weeks (preventive arm). Nephritis, skin inflammation, serum levels of autoantibodies and cytokines, mononuclear cell phenotype and gene expression, neutrophil extracellular traps (NETs) release, endothelium-dependent vasorelaxation, and endothelial differentiation were compared in treated and untreated mice. Treatment with tofacitinib led to significant improvement in measures of disease activity, including nephritis, skin inflammation, and autoantibody production. In addition, tofacitinib treatment reduced serum levels of proinflammatory cytokines and interferon responses in splenocytes and kidney tissue. Tofacitinib also modulated the formation of NETs and significantly increased endothelium-dependent vasorelaxation and endothelial differentiation. The drug was effective in both preventive and therapeutic strategies. Tofacitinib modulates the innate and adaptive immune responses, ameliorates murine lupus, and improves vascular function. These results indicate that JAK inhibitors have the potential to be beneficial in SLE and its associated vascular damage. © 2016, American College of Rheumatology.

  20. Evidence-based treatment of systemic lupus erythematosus and its ...

    African Journals Online (AJOL)

    Risk of congenital heart block in infants. ↓ Number and severity of lupus flares. TG = triglycerides; VLDL = very lowdensity lipoproteins; TC = total cholesterol; .... immunosuppressive therapy, whereas ischaemic events may require anticoagulation. Moreover, SLE is a wellknown risk factor for depres sion, anxiety and fatigue ...

  1. Lupus nephritis

    African Journals Online (AJOL)

    1991-03-02

    Mar 2, 1991 ... A recent large Australian series! on lupus nephritis emphasises .... patent capillary lumina and thickened capillary walls. ... (Australia). 135. 27. 58. 15. This study (RSA). 51. 33. 63. 4 have been documented.2-5 All included patients with lupus nephritis, but while the series from Natal' gave a detailed list.

  2. Protein-losing enteropathy in systemic lupus erythematosus: 12 years experience from a Chinese academic center.

    Science.gov (United States)

    Chen, Zhen; Li, Meng-Tao; Xu, Dong; Yang, Hong; Li, Jing; Zhao, Jiu-Liang; Zhang, Heng-Hui; Han, Shao-Mei; Xu, Tao; Zeng, Xiao-Feng

    2014-01-01

    Protein-losing enteropathy (PLE) is a complication in some systemic lupus erythematosus (SLE) patients that is often misdiagnosed. With this study, we provide insight into clinical characteristics, laboratory characteristics, diagnostic tests, risk factors, treatment, and prognosis of the disease. A retrospective, case-control study was performed in 44 patients with SLE-related PLE (PLE group) and 88 patients with active SLE (control group) admitted to our care from January 2000-January 2012. Risk factors for SLE-related PLE were examined, and we analyzed the accuracy of single and combined laboratory characteristics in discriminating SLE-related PLE from active SLE. Serum albumin and C3 levels were measured as outcome during and after treatment with corticosteroids and immunosuppressive agents. The PLE group had lower mean serum albumin and 24-hour urine protein levels, higher mean total plasma cholesterol levels, and greater frequencies of anti-SSA and SSB seropositivity compared with the control group. Anti-SSA seropositivity, hypoalbuminemia, and hypercholesterolemia were independent risk factors for SLE-related PLE. The simultaneous presence of serum albumin (PLE. SLE-related PLE should be considered when an SLE patient presents with generalized edema, anti-SSA antibody seropositivity, hypercholesterolemia, severe hypoalbuminemia, and low 24-hour urine protein levels. Aggressive treatment for lupus might improve prognosis.

  3. Predisposition to Cervical Atypia in Systemic Lupus Erythematosus: A Clinical and Cytopathological Study

    Directory of Open Access Journals (Sweden)

    Hend Hilal Al-Sherbeni

    2015-01-01

    Full Text Available Introduction. Systemic lupus erythematosus (SLE is a complex disease with variable presentations, course, and prognosis. The female genital tract may be a potential target organ in SLE since cervical inflammation may be associated with disease activity. An increase in cervical dysplasia, a precursor of cervical cancer, has been reported in females with SLE. Aim of the Work. This work aimed to study the prevalence of abnormal cervicovaginal smears in patients with systemic lupus erythematosus (SLE and to correlate abnormal smear findings with exposure to infection with human papilloma virus (HPV in SLE patients. Patients and Methods. Thirty-two patients with SLE, fulfilling the 1997 revised criteria for the classification of SLE, were included in this study. They were subjected to full history taking, clinical examination, laboratory investigations, and cervicovaginal smearing. Twenty healthy subjects not known to suffer from any rheumatological disease were used as controls, and they were subjected to cervicovaginal smearing. Results. Four out of 32 SLE patients showed abnormal Pap smears (12.5% compared to none showing any cervical changes in the control group (0%. Among these 4 patients, 3 were having ASCU and one was having LSIL (HPV. Conclusion. Cervicovaginal smearing is an easy, economic, safe, repeatable, and noninvasive technique for screening and early detection of cervical neoplastic lesions in SLE.

  4. Inverse Association of Parkinson Disease With Systemic Lupus Erythematosus: A Nationwide Population-based Study.

    Science.gov (United States)

    Liu, Feng-Cheng; Huang, Wen-Yen; Lin, Te-Yu; Shen, Chih-Hao; Chou, Yu-Ching; Lin, Cheng-Li; Lin, Kuen-Tze; Kao, Chia-Hung

    2015-11-01

    The effects of the inflammatory mediators involved in systemic lupus erythematous (SLE) on subsequent Parkinson disease have been reported, but no relevant studies have focused on the association between the 2 diseases. This nationwide population-based study evaluated the risk of Parkinson disease in patients with SLE.We identified 12,817 patients in the Taiwan National Health Insurance database diagnosed with SLE between 2000 and 2010 and compared the incidence rate of Parkinson disease among these patients with that among 51,268 randomly selected age and sex-matched non-SLE patients. A Cox multivariable proportional-hazards model was used to evaluate the risk factors of Parkinson disease in the SLE cohort.We observed an inverse association between a diagnosis of SLE and the risk of subsequent Parkinson disease, with the crude hazard ratio (HR) being 0.60 (95% confidence interval 0.45-0.79) and adjusted HR being 0.68 (95% confidence interval 0.51-0.90). The cumulative incidence of Parkinson disease was 0.83% lower in the SLE cohort than in the non-SLE cohort. The adjusted HR of Parkinson disease decreased as the follow-up duration increased and was decreased among older lupus patients with comorbidity.We determined that patients with SLE had a decreased risk of subsequent Parkinson disease. Further research is required to elucidate the underlying mechanism.

  5. Systemic lupus erythematosus and thyroid disease - Experience in a single medical center in Taiwan.

    Science.gov (United States)

    Liu, Yu-Chuan; Lin, Wen-Ya; Tsai, Ming-Chin; Fu, Lin-Shien

    2017-06-28

    To investigate the association of systemic lupus erythematosus (SLE) with thyroid diseases in a medical center in central Taiwan. This is a retrospective cohort of 2796 SLE patients in a tertiary referral medical center from 2000 to 2013. We screened SLE by catastrophic illness registration from national insurance bureau; and thyroid diseases by ICD 9 codes, then confirmed by thyroid function test, auto-antibody, medical and/or surgical intervention. We compared the rate of hyperthyroidism, hypothyroidism and autoimmune thyroid disease (AITD) in SLE patients and the 11,184 match controls. We calculated the rate of these thyroid diseases and positive antibodies to thyroglobulin (ATGAb), thyroid peroxidase (TPOAb) in SLE patients grouped by the presence of overlap syndrome and anti-dsDNA antibody. We also compared the association of thyroid diseases to severe SLE conditions, including renal, central nervous system (CNS) involvement, and thrombocytopenia. Compared to the matched controls, the cumulative incidence of thyroid disease, including hyperthyroidism, hypothyroidism and AITD, were all higher in SLE patients (p hyperthyroidism. SLE patients with thyroid diseases also carry higher risk for severe complications such as renal involvement (p = 0.024) central nervous system involvement (p hyperthyroidism, hypothyroidism, and AITD than the matched control. Among lupus patients, the risks of thyroid diseases are even higher in the presence of overlap syndrome. SLE patients with thyroid diseases had higher risk of renal and CNS involvement. Copyright © 2017. Published by Elsevier B.V.

  6. Subclinical impairment of ovarian reserve in systemic lupus erythematosus patients with normal menstruation not using alkylating therapy.

    Science.gov (United States)

    Ma, Wenhong; Zhan, Zhongping; Liang, Xiaoyan; Chen, Jianhui; Huang, Xingfang; Liao, Caiyun

    2013-12-01

    Disease activity is a major factor in menstrual disorders in systemic lupus erythematosus (SLE) patients not receiving alkylating therapy. However, the ovarian reserve of SLE women with normal menstruation is still unclear. Twenty-three SLE patients naïve to cytotoxic agents (SLE group) and nineteen SLE patients receiving current or previous cyclophosphamide (CTX) therapy (without other cytotoxic agents; SLE-CTX group) were enrolled. Twenty-one age-matched healthy women served as controls. All patients and controls had a regular menstrual cycle. Basal hormone levels, including follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol (E2), and anti-Müllerian hormone (AMH), and antral follicle count (AFC) were analyzed in the two study groups and compared with the control group. No significant differences were found between the SLE, SLE-CTX, and control groups in age, body mass index (BMI), and basal FSH and LH levels. The E2 (P=0.023) levels were high and the AMH (P=0.000) values and AFC (P=0.001) were significantly lower in the SLE and SLE-CTX groups compared to control. However, these values were similar between the SLE and SLE-CTX groups. SLE patients not receiving alkylating therapy who had normal menstruation and short illness duration still had an impaired ovarian reserve.

  7. Cross-cultural validation of a disease-specific patient-reported outcome measure for lupus in Philippines.

    Science.gov (United States)

    Navarra, S V; Tanangunan, R M D V; Mikolaitis-Preuss, R A; Kosinski, M; Block, J A; Jolly, M

    2013-03-01

    LupusPRO is a disease-targeted patient-reported outcome measure that was developed and validated among US patients with systemic lupus erythematosus (SLE). We report the cross-cultural validation results of the LupusPRO English-language version among Filipino SLE patients. The 43-item LupusPRO was pretested in 15 SLE individuals, then administered to 106 SLE patients, along with short-form SF36 and the EQ5D visual analogue scale. A mail/drop-back LupusPRO and change in health status item survey were returned within two to three days. Demographics, clinical and serological characteristics, disease activity and damage measured by PGA, SELENA-SLEDAI, LFA Flare, and SLICC-ACR SLE damage index (SDI) were collected. Internal consistency reliability (ICR), test-retest reliability (TRT), convergent validity (corresponding SF36 domains) and criterion validity (against general health and disease activity measures) were tested. Reported p values are two tailed. A total of 121 Filipino SLE subjects (95% women, median age 31.0 ± 16 years) with at least a high school level of English instruction participated. Median (IQR) PGA, SLEDAI and SDI were 0.0 (1.0), 2.0 (10) and 0 (1), respectively. ICR exceeded 0.7 for all domains except the lupus symptoms domain. TRT was greater than 0.85 for all LupusPRO domains. Convergent and criterion validity were observed against corresponding SF36 domains and disease activity measures. The tool was well received by patients. Confirmatory factor analysis showed good fit. English LupusPRO has fair psychometric properties among SLE patients in the Philippines, and is now available for inclusion in clinical trials and longitudinal studies to test responsiveness to change.

  8. Pathogenic inflammation and its therapeutic targeting in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Timothy Andrew Gottschalk

    2015-10-01

    Full Text Available 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 immuno-suppression 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

  9. 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

  10. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  11. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Burns, Natalie S.; Stevens, Anne M.; Iyer, Ramesh S.

    2014-01-01

    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. 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

  13. Toll-like receptor activation in the pathogenesis of lupus nephritis.

    Science.gov (United States)

    Lorenz, Georg; Lech, Maciej; Anders, Hans-Joachim

    2017-12-01

    The pathogenesis of systemic lupus erythematosus (SLE) and lupus nephritis is complex but no longer enigmatic. Much progress has been made to on the polygenetic origin of lupus in identifying gene variants that permit the loss of tolerance against nuclear autoantigens. Along the same line in about 50% of lupus patients additional genetic weaknesses promote immune complex glomerulonephritis and filtration barrier dysfunction. Here we briefly summarize the pathogenesis of SLE with a focus on loss of tolerance and the role of toll-like receptors in the "pseudo"-antiviral immunity concept of systemic lupus. In addition, we discuss the local role of Toll-like receptors in intrarenal inflammation and kidney remodeling. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Molecular characterization of circulating plasma cells in patients with active systemic lupus erythematosus.

    Directory of Open Access Journals (Sweden)

    Patricia L Lugar

    Full Text Available Systemic lupus erythematosus (SLE is a generalized autoimmune disease characterized by abnormal B cell activation and the occurrence of increased frequencies of circulating plasma cells (PC. The molecular characteristics and nature of circulating PC and B cells in SLE have not been completely characterized. Microarray analysis of gene expression was used to characterize circulating PC in subjects with active SLE. Flow cytometry was used to sort PC and comparator B cell populations from active SLE blood, normal blood and normal tonsil. The gene expression profiles of the sorted B cell populations were then compared. SLE PC exhibited a similar gene expression signature as tonsil PC. The differences in gene expression between SLE PC and normal tonsil PC and tonsil plasmablasts (PB suggest a mature Ig secreting cell phenotype in the former population. Despite this, SLE PC differed in expression of about half the genes from previously published gene expression profiles of normal bone marrow PC, indicating that these cells had not achieved a fully mature status. Abnormal expression of several genes, including CXCR4 and S1P(1, suggests a mechanism for the persistence of SLE PC in the circulation. All SLE B cell populations revealed an interferon (IFN gene signature previously only reported in unseparated SLE peripheral blood mononuclear cells. These data indicate that SLE PC are a unique population of Ig secreting cells with a gene expression profile indicative of a mature, but not fully differentiated phenotype.

  15. The SLE heart in scintigraphic diagnostics

    International Nuclear Information System (INIS)

    Rottensteiner, J.

    2001-01-01

    Investigation of SLE-heart of 14 patients with 201Ti-SPECT and 18 FDG-SPECT and comparison with clinical and laboratory parameters. Results: SLE-patients with unspecific heart symptoms show in spite of sufficiently perfusion in 201Ti-SPECT considerable defects in 18-FDG. Interpretation: cause of the defects in FDG in a disturbed glucose metabolism independent of perfusion. (boteke)

  16. Frequency and Type of Hepatic and Gastrointestinal Involvement in Juvenile Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Leila Tahernia

    2017-01-01

    Full Text Available Background. Systemic lupus erythematosus (SLE is a frequent rheumatology disorder among children. Since hepatic involvement is a common systemic manifestation in lupus, the frequency and type of hepatic involvement were determined in pediatric cases of SLE admitted to Children’s Medical Hospital from 2005 to 2014. Methods and Patients. In this observational case-series study, 138 pediatric cases of SLE were admitted in Children’s Medical Center (a pediatric rheumatology referral center in Tehran, Iran enrolled from 2005 to 2014 and the outcomes, frequency, and type of hepatic involvement were assessed among them. Results. Hepatic involvement was reported in 48.55% of total SLE patients. Aspartate aminotransferase (AST, alanine aminotransferase (ALT, and both enzymes higher than normal upper limits were detected in 8.7%, 5%, and 34.7% of lupus patients, respectively. Increased level of liver enzymes was categorized as less than 100, between 100 and 1000, and more than 1000 levels in 23.1%, 23.1%, and 2.1% of cases. The only gastrointestinal involvement in lupus patients contributing to hepatic involvement was gastrointestinal bleeding. Rising in liver enzymes was detected mostly in lupus patients without gastrointestinal bleeding (52.2% without versus 25.8% with gastrointestinal bleeding, P=0.007. Conclusion. Approximately half of the pediatric patients suffering from SLE have hepatic involvement. No significant correlation was observed between various organs involvement and abnormal level of liver enzymes.

  17. Discoid Lupus Erythematosus

    Science.gov (United States)

    ... Name: Category: Share: Yes No, Keep Private Discoid Lupus Erythematosus Share | Discoid lupus erythematosus (DLE) is a chronic skin condition of sores ... diagnosis because other conditions can look like discoid lupus erythematosus. If the skin biopsy shows discoid lupus erythematosus, ...

  18. Genetically determined serum levels of mannose-binding lectin correlate negatively with common carotid intima-media thickness in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Troelsen, Lone N; Garred, Peter; Christiansen, Buris

    2010-01-01

    Patients with systemic lupus erythematosus (SLE) have excess cardiovascular morbidity and mortality due to accelerated atherosclerosis that cannot be attributed to traditional cardiovascular risk factors alone. Variant alleles of the mannose-binding lectin gene (MBL2) causing low serum...

  19. Diminished ability of erythrocytes from patients with systemic lupus erythematosus to limit opsonized immune complex deposition on leukocytes and activation of granulocytes

    DEFF Research Database (Denmark)

    Nielsen, C H; Rasmussen, J M; Voss, A

    1998-01-01

    OBJECTIVE: To compare the ability of normal erythrocytes and erythrocytes from systemic lupus erythematosus (SLE) patients to bind immune complexes (IC), thereby inhibiting IC deposition on polymorphonuclear leukocytes (PMN) and the consequent induction of a PMN respiratory burst (RB). METHODS...

  20. Endovascular repair as a sole treatment in multiple aneurysms in patient with SLE

    International Nuclear Information System (INIS)

    Dineva, S.; Al-Amin, M.; Demetriou, S.; Tsetis, D.

    2013-01-01

    Full text: Introduction: Most aneurysms are local manifestations of systemic disease. For patients over 65 years the incidence of aneurysm of the abdominal aorta (AAA) is approximately 5-6% in men and 1-2 % for women. The presence of both the AAA and aneurysms in other location is even rarer, and this percentage is likely increase further in patients with systemic lupus erythematosus (SLE). What you will learn: We present a rare clinical case of endovascular treatment of multifocal aneurysm including post catheterization pseudoaneurysm. The patient is a 73 years old woman with a history of SLE and age-related comorbidity. Originally an endovascular treatment of aneurysms of the abdominal aorta and right common iliac artery was used. Two years later a successfully endovascular treatment of aneurysm of the right renal artery was conducted, which however is complicated by the formation of a pseudoaneurysm in access through the left femoral artery. The late one is again treated endovascular by placement of a covered stent after failure of percutaneous injection of 1000 UI thrombin. Discussion: Adult patients with a long history of SLE are unsuitable candidates for surgical treatment of aneurysmal disease, especially in its multifocal form. In our case we have taken multistep successful endovascular procedures, including technically hard placing of the stent at the site of the right renal aneurysms, and post catheterization pseudoaneurysm. Conclusion: Multifocal aneurysmal vascular changes due to macroangiopathia in SLE can be treated alone by endovascular means in multi-stages procedures

  1. Outcome of total hip arthroplasty for avascular necrosis of the femoral head in systemic lupus erythematosus.

    Science.gov (United States)

    Woo, Min Su; Kang, Joon Soon; Moon, Kyoung Ho

    2014-12-01

    This study evaluated the result of total hip arthroplasty (THA) for avascular necrosis of the femur head (AVNFH) in systemic lupus erythematosus (SLE) patients. Nineteen THAs were performed on 13 patients with SLE. The results of these patients were compared with the results of the control group (19 patients) who had THR due to AVNFH with none-SLE conditions. The Harris hip score increased from a preoperative average of 65.3 points to 94.9 at the most recent follow-up. In the control group, the mean HHS was 67.2 preoperatively and 96.1 postoperatively at the last follow-up. No significant difference was found between SLE patients and non-SLE patients who underwent hip arthroplasty. In conclusion, THA is an acceptable treatment for achieving functional improvement in patients who had SLE and AVNFH. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Demyelinating syndrome in SLE: review of different disease subtypes and report of a case series

    Directory of Open Access Journals (Sweden)

    E. Chessa

    2017-12-01

    Full Text Available Demyelinating syndrome (DS is a rare manifestation of systemic lupus erythematosus (SLE (1% with high clinical heterogeneity and potentially severe prognosis. It can represent a diagnostic and therapeutic challenge for clinicians. A recent study described 5 different patterns of demyelinating disease presentation, characterised by specific clinical, laboratory and brain and spine magnetic resonance imaging abnormalities: 1 neuromyelitis optica; 2 neuromyelitis optica spectrum disorders; 3 DS prevalently involving the brain; 4 DS prevalently involving the brainstem; 5 clinically isolated syndrome. In this review we briefly discuss typical characteristics of each DS presentation in SLE and we describe 5 illustrative clinical cases, one for each subset of DS, considering both diagnostic and therapeutic options.

  3. Survival in systemic lupus erythematosus, 1995-2010

    DEFF Research Database (Denmark)

    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....

  4. Altered lipoproteins in patients with systemic lupus erythematosus are associated with augmented oxidative stress: a potential role in atherosclerosis.

    Science.gov (United States)

    Park, Jin Kyun; Kim, Jae-Yong; Moon, Jin Young; Ahn, Eun Young; Lee, Eun Young; Lee, Eun Bong; Cho, Kyung-Hyun; Song, Yeong Wook

    2016-12-30

    To examine the structural and oxidative properties of lipoproteins from patients with systemic lupus erythematosus (SLE). The lipid profiles of 35 SLE patients and 15 healthy controls (HCs) were compared. Oxidation status, susceptibility to oxidation, and structural integrity of low-density lipoprotein (LDL) were determined by measuring malondialdehyde (MDA), de novo formation of conjugated dienes in the presence of CuSO 4 , and mobility on gel electrophoresis, respectively. In vitro foam cell formation and the oxidative potential in zebrafish embryos were examined. LDL levels in SLE patients and HCs were similar (p = 0.277). LDL from SLE patients was more fragmented than that from HCs. In addition, LDL from SLE patients was more oxidized than LDL from HCs (p Lipoproteins from SLE patients exhibited greater oxidative potential, which might contribute to accelerated atherosclerosis in SLE.

  5. Systemic lupus erythematosus and risk of preterm birth: a systematic review and meta-analysis of observational studies.

    Science.gov (United States)

    Wei, S; Lai, K; Yang, Z; Zeng, K

    2017-05-01

    We performed a meta-analysis to identify the association between systemic lupus erythematosus (SLE) and preterm birth. In this study, we studied the effects of SLE, SLE disease activity, a history of nephritis and active nephritis on preterm birth. Searches were conducted before 20 May 2016 of PubMed, Embase, Medline and Cochrane Library of literature and article reference lists. Eleven observational case-control studies and thirteen cohort studies met the inclusion criteria. The pooled relative risk (RR) for the risk of preterm birth in SLE patients versus controls was 2.05 (95% confidence interval (CI): 1.72-3.32); for active SLE patients versus inactive was 2.98 (95% CI: 2.32-3.83); for SLE patients with a history of lupus nephritis versus those without nephritis it was 1.62 (95% CI: 1.35-1.95); and for SLE patients with active nephritis versus those with quiescent nephritis it was 1.78 (95% CI: 1.17-2.70). In summary, this study identified a significant association in the above results. This association was more significant in active SLE patients versus inactive. With respect to SLE itself, active inflammation (such as disease activity) may be more hazardous for the management of the pregnancy. This suggests that it is essential to control disease activity in order to achieve a better outcome of SLE pregnancy.

  6. Prognostic implications of active discoid lupus erythematosus and malar rash at the time of diagnosis of systemic lupus erythematosus: Results from a prospective cohort study.

    Science.gov (United States)

    Drucker, A M; Su, J; Mussani, F; Siddha, S K; Gladman, D D; Urowitz, M B

    2016-04-01

    Cutaneous lupus erythematosus (CLE) may have prognostic implications for systemic lupus erythematosus (SLE). We aimed to determine the impact of discoid lupus erythematosus (DLE) and malar rash on SLE disease activity. Data were analyzed from the Toronto Lupus Clinic prospective cohort study. We compared SLE patients with active DLE or malar rash at SLE diagnosis to SLE patients who never developed CLE. Outcomes were assessed at one and five years, including Adjusted Mean Systemic Lupus Erythematosus Disease Activity Index 2000 (AMS). A total of 524 SLE patients (284 without CLE, 65 with DLE, and 175 with malar rash) were included. Mean AMS scores in patients without CLE at one and five years were 5.96 ± 5.06 and 4.00 ± 3.52, which did not differ significantly from scores at one (6.93 ± 5.31, p = 0.17) and five years (4.29 ± 2.62, p = 0.63) in the DLE group. In patients with malar rash, AMS scores at one (8.30 ± 6.80, p < 0.001) and five years (5.23 ± 3.06, p = 0.004) were higher than controls without CLE. Malar rash may be a marker of more severe systemic disease over time, while DLE has no significant impact on general SLE disease activity. © The Author(s) 2015.

  7. Preferential binding to Elk-1 by SLE-associated IL10 risk allele upregulates IL10 expression.

    Directory of Open Access Journals (Sweden)

    Daisuke Sakurai

    Full Text Available Immunoregulatory cytokine interleukin-10 (IL-10 is elevated in sera from patients with systemic lupus erythematosus (SLE correlating with disease activity. The established association of IL10 with SLE and other autoimmune diseases led us to fine map causal variant(s and to explore underlying mechanisms. We assessed 19 tag SNPs, covering the IL10 gene cluster including IL19, IL20 and IL24, for association with SLE in 15,533 case and control subjects from four ancestries. The previously reported IL10 variant, rs3024505 located at 1 kb downstream of IL10, exhibited the strongest association signal and was confirmed for association with SLE in European American (EA (P = 2.7×10⁻⁸, OR = 1.30, but not in non-EA ancestries. SNP imputation conducted in EA dataset identified three additional SLE-associated SNPs tagged by rs3024505 (rs3122605, rs3024493 and rs3024495 located at 9.2 kb upstream, intron 3 and 4 of IL10, respectively, and SLE-risk alleles of these SNPs were dose-dependently associated with elevated levels of IL10 mRNA in PBMCs and circulating IL-10 protein in SLE patients and controls. Using nuclear extracts of peripheral blood cells from SLE patients for electrophoretic mobility shift assays, we identified specific binding of transcription factor Elk-1 to oligodeoxynucleotides containing the risk (G allele of rs3122605, suggesting rs3122605 as the most likely causal variant regulating IL10 expression. Elk-1 is known to be activated by phosphorylation and nuclear localization to induce transcription. Of interest, phosphorylated Elk-1 (p-Elk-1 detected only in nuclear extracts of SLE PBMCs appeared to increase with disease activity. Co-expression levels of p-Elk-1 and IL-10 were elevated in SLE T, B cells and monocytes, associated with increased disease activity in SLE B cells, and were best downregulated by ERK inhibitor. Taken together, our data suggest that preferential binding of activated Elk-1 to the IL10 rs3122605-G allele

  8. [Cardiac tamponade disclosing systemic lupus erythematosus].

    Science.gov (United States)

    Nour-Eddine, M; Bennis, A; Soulami, S; Chraibi, N

    1996-02-01

    Cardiac tamponade secondary to systemic lupus erythematosus is rare and has a very serious prognosis. The authors report a case of cardiac tamponade confirmed by echocardiography, which constituted the presenting sign of systemic lupus erythematosus in a 20-year-old patient, who required emergency pericardial aspiration. The diagnosis of systemic lupus erythematosus was established on the basis of the combination of pericardial involvement, non-erosive arthritis, leukopenia with lymphopenia, presence of LE cells and anti-native DNA antibodies and positive antinuclear antibody titre of 1/2560. The clinical course was favourable in response to 3 months of corticosteroid treatment. The possibility of SLE should be considered in any case of cardiac tamponade in a young patient in which the aetiology is not explained.

  9. Burden of lupus on work: Issues in the employment of individuals with lupus.

    Science.gov (United States)

    Agarwal, Neelam; Kumar, Vinod

    2016-10-17

    Systemic lupus erythematosus (SLE) or Lupus is one of the leading causes of work disability in the United States, accounting for about 20% of the more than estimated 1.5 million Americans with a work disability. The symptoms of lupus can have a profound impact on the person's employment. Impacts of lupus are more pronounced among young and middle-adulthood. Studies have shown that loss in work hours cost the nation nearly $13 billion annually. The loss also impacts the individual's work, quality of life, self-management, and self-efficacy. In this article, the author describes the financial burden of lupus. The article also describes the substantial impact of lupus on employment outcomes for individuals living with the condition. The author also reviews major signs and symptoms of disease and their impact on employment. Findings from this research can be used to identify various accommodations and strategies for individuals to prevent flare-ups. The paper presents innovative strategies that include early interventions and how employers andco-workers can provide helpful support that includes job accommodations to individuals with lupus.

  10. Pediatric patient with systemic lupus erythematosus & congenital acquired immunodeficiency syndrome: An unusual case and a review of the literature

    Directory of Open Access Journals (Sweden)

    Rezaee Fariba

    2008-05-01

    Full Text Available Abstract The coexistence of systemic lupus erythematosus (SLE in patients with congenital human immunodeficiency virus (HIV infection is rare. This is a case report of a child diagnosed with SLE at nine years of age. She initially did well on non-steroidal anti-inflammatory agents, hydroxychloroquine, and steroids. She then discontinued her anti-lupus medications and was lost to follow-up. At 13 years of age, her lupus symptoms had resolved and she presented with intermittent fevers, cachexia, myalgias, arthralgias, and respiratory symptoms. Through subsequent investigations, the patient was ultimately diagnosed with congenitally acquired immunodeficiency syndrome (AIDS.

  11. Association of mannose-binding lectin gene variation with disease severity and infections in a population-based cohort of systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Garred, P; Voss, A; Madsen, H O

    2001-01-01

    This study describes the importance of mannose-binding lectin (MBL) variant alleles for systemic lupus erythematosus (SLE) and accompanying infections in a population-based cohort. MBL alleles were determined in 99 SLE patients recruited from a representative Danish region. Patients were classified...... according to the 1982 revised ACR criteria as definite SLE (D-SLE) (n = 77) fulfilling > or =4 criteria and incomplete SLE (I-SLE) (n = 22) with 0.99, respectively). A meta-analysis of eight previously published studies suggested that the presence of MBL variant alleles confer a 1.6 times overall increased...... risk for D-SLE (P disease activity (SLEDAI-index) in a 2-year follow-up period (P = 0.02) and had an increased risk of acquiring complicating infections in general (P = 0.03) and respiratory infections in particular (P = 0.0006). Only in SLE patients...

  12. Increased IgG on cell-derived plasma microparticles in systemic lupus erythematosus is associated with autoantibodies and complement activation

    DEFF Research Database (Denmark)

    Nielsen, Christoffer T; Østergaard, Ole; Stener, Line

    2012-01-01

    To quantify immunoglobulin and C1q on circulating cell-derived microparticles (MPs) in patients with systemic lupus erythematosus (SLE) and to determine whether immunoglobulin and C1q levels are correlated with clinical and serologic parameters.......To quantify immunoglobulin and C1q on circulating cell-derived microparticles (MPs) in patients with systemic lupus erythematosus (SLE) and to determine whether immunoglobulin and C1q levels are correlated with clinical and serologic parameters....

  13. Validation of Systemic Lupus Erythematosus Diagnosis as the Primary Cause of Renal Failure in the US Renal Data System.

    Science.gov (United States)

    Broder, Anna; Mowrey, Wenzhu B; Izmirly, Peter; Costenbader, Karen H

    2017-04-01

    Using American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC) criteria for systemic lupus erythematosus (SLE) classification as gold standards, we determined sensitivity, specificity, positive and negative predictive values (PPV and NPV) of having SLE denoted as the primary cause of end-stage renal disease (ESRD) in the US Renal Data System (USRDS). ESRD patients were identified by International Classification of Diseases, Ninth Revision codes in electronic medical records of 1 large tertiary care center, Montefiore Hospital, from 2006 to 2012. Clinical data were extracted and reviewed to establish SLE diagnosis. Data were linked by social security number, name, and date of birth to the USRDS, where primary causes of ESRD were ascertained. Of 7,396 ESRD patients at Montefiore, 97 met ACR/SLICC SLE criteria, and 86 had SLE by record only. Among the 97 SLE patients, the attributed causes of ESRD in the USRDS were 77 SLE and 12 with other causes (unspecified glomerulonephritis, hypertension, scleroderma), and 8 missing. Sensitivity, specificity, PPV, and NPV for SLE in the USRDS were 79%, 99.9%, 93%, and 99.7%, respectively. Of the 60 patients with biopsy-proven lupus nephritis, 44 (73%) had SLE as primary ESRD cause in the USRDS. Attribution of the primary ESRD causes among SLE patients with ACR/SLICC criteria differed by race, ethnicity, and transplant status. The diagnosis of SLE as the primary cause of ESRD in the USRDS has good sensitivity, and excellent specificity, PPV, and NPV. Nationwide access to medical records and biopsy reports may significantly improve sensitivity of SLE diagnosis. © 2016, American College of Rheumatology.

  14. Humor in systemic lupus erythematosus.

    Science.gov (United States)

    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.

  15. Validity of LupusQoL-China for the assessment of health related quality of life in Chinese patients with systemic lupus erythematosus.

    Directory of Open Access Journals (Sweden)

    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.

  16. Dicty_cDB: SLE232 [Dicty_cDB

    Lifescience Database Archive (English)

    Full Text Available SL (Link to library) SLE232 (Link to dictyBase) - - - Contig-U16255-1 SLE232F (Link... to Original site) SLE232F 614 - - - - - - Show SLE232 Library SL (Link to library) Clone ID SLE232 (Link to dict...yBase) Atlas ID - NBRP ID - dictyBase ID - Link to Contig Contig-U16255-1 Original site URL http://dict...PSSGFTDFIPSNATCSSLNCNAQQMSCKYVQQACHETSCCPDIPQC QIPATGGGPATGSATGQGTSGGTPGSCDKVNCPNGYICTIVNQLAVCVSPSSSSSSSSST ...CHETSCCPDIPQC QIPATGGGPATGSATGQGTSGGTPGSCDKVNCPNGYICTIVNQLAVCVSPSSSSSSSSST TGSHTTTGGSTTGSHTTTGGSTTGSHTTTGGSA

  17. Patients' perceptions of the effects of systemic lupus erythematosus on health, function, income, and interpersonal relationships : A comparison with Wegener's granulomatosis

    NARCIS (Netherlands)

    Boomsma, MM; Bijl, M; Stegeman, CA; Kallenberg, CGM; Hoffman, GS; Tervaert, JWC

    2002-01-01

    Objective. To describe the patients' perceptions of the effects of systemic lupus erythematosus (SLE) and Wegener's granulomatosis (WG) on health, function, income, and interpersonal relationships. Methods. 114 patients with SLE, and 79 patients with WG completed a self-administered questionnaire.

  18. The substantial burden of systemic lupus erythematosus on the productivity and careers of patients: a European patient-driven online survey.

    Science.gov (United States)

    Gordon, Caroline; Isenberg, David; Lerstrøm, Kirsten; Norton, Yvonne; Nikaï, Enkeleida; Pushparajah, Daphnee S; Schneider, Matthias

    2013-12-01

    The objective of this study was to explore the burden of SLE and its effect on patients' lives. The Lupus European Online (LEO) survey included patient-designed questions on demographics, SLE diagnosis, and the impact of SLE on careers. Three SLE-specific patient-reported outcome (PRO) questionnaires were also completed: the Lupus Quality of Life (LupusQoL), the Fatigue Severity Scale (FSS), and the Work Productivity and Activity Impairment (WPAI)-Lupus v2.0. The survey was available online in five languages from May through August 2010. All self-identified SLE participants were eligible to respond. Survey results were analysed using descriptive statistics. Multivariate linear regression explored factors contributing to impaired productivity. Of the 2070 European SLE patients completing the survey, 93.1% were women, 86.7% were aged Productivity was impaired across all WPAI domains, both at work and in general activities. Fatigue, an inability to plan and reduced physical health were significantly associated with impaired productivity. Patients whose careers were affected by SLE had worse health-related quality of life, more fatigue and worse productivity than patients whose careers were not affected. LEO survey respondents reported that SLE negatively affects their daily lives, productivity and career choices.

  19. Prevalence, severity, and clinical features of acute and chronic pancreatitis in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Wang, Qiang; Shen, Min; Leng, Xiaomei; Zeng, Xiaofeng; Zhang, Fengchun; Qian, Jiaming

    2016-10-01

    Pancreatitis is a rare, life-threatening complication of systemic lupus erythematosus (SLE). This study aimed to describe the clinical features of acute pancreatitis (AP) and chronic pancreatitis (CP) in patients with SLE. Data of patients who fulfilled the revised criteria of the American Rheumatism Association for diagnosis of SLE were retrospectively analyzed. SLE activity was graded according to the SLE Disease Activity Index. Logistic regression analysis was conducted to find out independent associations. Survival rates were estimated by using Kaplan-Meier plots. This study included 5665 SLE patients admitted between January 1983 and January 2014, of whom 52 patients were diagnosed with pancreatitis. Pancreatitis prevalence in SLE patients was 0.92 % (52/5665). AP (0.8 %, 46/5665) was more prevalent than CP (0.1 %, 6/5665), presented mostly during active SLE, and affected more organs. Hypertriglyceridemia occurred in 76.9 % of AP patients and in none of the CP patients. AP patients were divided into severe (n = 10) or mild (n = 20) cases. The average triglyceride level in severe AP cases was higher than that in mild AP cases (P = 0.006), and the mortality rate of lupus-associated AP was 32.6 % (15/46). Concomitant infections and thrombocytopenia were independently associated with poor prognosis (P risk factors for poor prognosis.

  20. Comparison of the Sensitivity to Change of the 36-Item Short Form Health Survey and the Lupus Quality of Life Measure Using Various Definitions of Minimum Clinically Important Differences in Patients With Active Systemic Lupus Erythematosus.

    Science.gov (United States)

    Nantes, Stephanie G; Strand, Vibeke; Su, Jiandong; Touma, Zahi

    2018-01-01

    The Medical Outcomes Study Short Form 36 (SF-36) and Lupus Quality of Life (LupusQoL) are health-related quality of life questionnaires used in systemic lupus erythematosus (SLE). We first determined the hypothesis-testing construct validity of the SF-36 and LupusQoL against disease activity in patients with active SLE and then compared the sensitivity to change of SF-36 and LupusQoL domains according to different definitions of minimum clinically important differences (MCIDs) for improvement and worsening in the current cohort. Seventy-eight clinically active SLE patients concurrently completed both questionnaires at their baseline and followup visits. Questionnaire domain scores were correlated with the SLE Disease Activity Index 2000 (SLEDAI-2K) and evaluated for floor/ceiling effects. The sensitivity to change of domains in each questionnaire was analyzed first, according to the various MCID definitions and, second, by clinically meaningful changes in disease activity. The magnitudes of change in each domain score between the baseline and followup visit were evaluated using standardized response means. In the 78 patients, the mean ± SD SLEDAI-2K scores were 9.7 ± 4.8 at baseline and 8.8 ± 5.1 at followup. SF-36/LupusQoL domain scores did not correlate with disease activity. The SF-36 showed floor effects, and ceiling effects were evident in both questionnaires. All domains of both questionnaires showed sensitivity to change over time. Specific domains that reflected worsening or improvement differed according to differing MCID definitions. In SLE patients with active disease, both the SF-36 and LupusQoL are sensitive to change, reflecting both improvement and worsening. More importantly, the LupusQoL SLE-specific domains (planning, burden to others, body image, and intimate relationships) were largely responsive to change. © 2017, American College of Rheumatology.

  1. Computerized tomography data on CNS affection in systemic lupus erythematosus. Porazhenie tsentral'noj nervnoj sistemy pri sistemnoj krasnoj volchanke po dannym komp'yuternoj tomografii

    Energy Technology Data Exchange (ETDEWEB)

    Ivanova, M M; Bliznyuk, O I; Todua, F I; Tumanova, A A

    1989-01-01

    Computed tomography (CT) of the brain was employed in 40 patients with systemic lupus erythematosus (SLE). Clinical cerebral pathology was obvious in 30 and absent in 10 patients. By CT cerebral symptoms were divided of 4 groups. Clinical symptom complexes of CNS defects and SLE were reflected on definite CT images correlated with focal damage to the brain. CT picture of enlarged subarachnoid space, ventricles and basal cisterns can be observed in SLE patients without neurological symptoms. This indicated likely subclinical cerebral affection.

  2. Male lupus: a diagnosis often delayed--a case series and review of the literature.

    LENUS (Irish Health Repository)

    Ambrose, N L

    2012-02-01

    INTRODUCTION: Systemic lupus erythematosus (SLE) is an auto-immune disease that is characterised by autoantibody production. Male lupus is rare, apart from at either end of the age spectrum. AIM: In this series, we review the histories of six male lupus patients attending our service. RESULTS: Our patients presented in middle age and tended to develop haematological abnormalities, renal involvement and neurological manifestations which preceded the onset of their skin and joint complaints. Our patients accrued damage rapidly and overall did badly. They tended to respond sub-optimally to standard treatments. These cases highlight the need an increased awareness that male SLE patients present with a wide variety of symptoms, and that they accrue damage quickly. There is a need for timely diagnosis and appropriate initiation of treatment. This may help avoid preventable organ damage and increase the survival of men with SLE.

  3. 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.

  4. Circular RNAs and systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Li, Lian-Ju; Huang, Qing; Pan, Hai-Feng; Ye, Dong-Qing

    2016-01-01

    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.

  5. Association of STAT4 rs7574865 with susceptibility to systemic lupus erythematosus in Iranian population.

    Science.gov (United States)

    Mirkazemi, Sedigheh; Akbarian, Mahmoud; Jamshidi, Ahmad Reza; Mansouri, Reza; Ghoroghi, Shima; Salimi, Yahya; Tahmasebi, Zahra; Mahmoudi, Mahdi

    2013-12-01

    Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease with complex genetic inheritance that affecting different organs and systems. STAT4 has been newly identified as a susceptible gene in the development of SLE. According to recent studies, STAT4 has been associated with SLE in various populations. We investigated whether STAT4 single nucleotide polymorphisms (SNPs) were associated with susceptibility and clinical features of SLE in Iranian patients. The study group comprised 280 patients with SLE and 281 sex-, age-, and ethnicity-matched healthy controls of Iranian ancestry. Two SNPs (rs7574865 and rs7601754) were genotyped using the TaqMan MGB Allelic Discrimination method. Our results showed a significant association between rs7574865 T allele (odds ratio (OR) = 1.50, 95 % CI = 1.18-1.92, P = 0.002) and susceptibility to SLE. The rs7574865TT genotype (P = 0.02, OR = 1.94, 95 % CI = 1.74-3.19) and GT genotype (P = 0.008, OR = 1.71, 95 % CI = 1.19-2.45) showed a significant association with the risk of SLE in the Iranian population. We concluded that STAT4 rs7574865 is associated with SLE susceptibility in the Iranian population and this SNP might be a factor in the pathogenesis of SLE. However, further studies are required to investigate the mechanism by which polymorphisms in this gene lead to SLE.

  6. The Incidence and Prevalence of Systemic Lupus Erythematosus in San Francisco County, California: The California Lupus Surveillance Project.

    Science.gov (United States)

    Dall'Era, Maria; Cisternas, Miriam G; Snipes, Kurt; Herrinton, Lisa J; Gordon, Caroline; Helmick, Charles G

    2017-10-01

    Estimates of the incidence and prevalence of systemic lupus erythematosus (SLE) in the US have varied widely. The purpose of this study was to conduct the California Lupus Surveillance Project (CLSP) to determine credible estimates of SLE incidence and prevalence, with a special focus on Hispanics and Asians. The CLSP, which is funded by the Centers for Disease Control and Prevention, is a population-based registry of individuals with SLE residing in San Francisco County, CA, from January 1, 2007 through December 31, 2009. Data sources included hospitals, rheumatologists, nephrologists, commercial laboratories, and a state hospital discharge database. We abstracted medical records to ascertain SLE cases, which we defined as patients who met ≥4 of the 11 American College of Rheumatology classification criteria for SLE. We estimated crude and age-standardized incidence and prevalence, which were stratified by sex and race/ethnicity. The overall age-standardized annual incidence rate was 4.6 per 100,000 person-years. The average annual period prevalence was 84.8 per 100,000 persons. The age-standardized incidence rate in women and men was 8.6 and 0.7 per 100,000 person-years, respectively. This rate was highest among black women (30.5), followed by Hispanic women (8.9), Asian women (7.2), and white women (5.3). The age-standardized prevalence in women per 100,000 persons was 458.1 in blacks, 177.9 in Hispanics, 149.7 in Asians, and 109.8 in whites. Capture-recapture modeling estimated 33 additional incident cases and 147 additional prevalent cases. Comprehensive methods that include intensive case-finding provide more credible estimates of SLE in Hispanics and Asians, and confirm racial and ethnic disparities in SLE. The disease burden of SLE is highest in black women, followed by Hispanic women, Asian women, and white women. © 2017, American College of Rheumatology.

  7. Speckle tracking echocardiography in acute lupus myocarditis: comparison to conventional echocardiography

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    Riëtte Du Toit

    2017-07-01

    Full Text Available Aims: Lupus myocarditis occurs in 5–10% of patients with systemic lupus erythematosus (SLE. No single feature is diagnostic of lupus myocarditis. Speckle tracking echocardiography (STE can detect subclinical left ventricular dysfunction in SLE patients, with limited research on its utility in clinical lupus myocarditis. We report on STE in comparison to conventional echocardiography in patients with clinical lupus myocarditis. Methods and results: A retrospective study was done at a tertiary referral hospital in South Africa. SLE patients with lupus myocarditis were included and compared to healthy controls. Echocardiographic images were reanalyzed, including global longitudinal strain through STE. A poor echocardiographic outcome was defined as final left ventricular ejection fraction (LVEF <40%. 28 SLE patients fulfilled the criteria. Global longitudinal strain correlated with global (LVEF: r = −0.808; P = 0.001 and regional (wall motion score: r = 0.715; P < 0.001 function. In patients presenting with a LVEF ≥50%, global longitudinal strain (P = 0.023, wall motion score (P = 0.005 and diastolic function (P = 0.004 were significantly impaired vs controls. Following treatment, LVEF (35–47% (P = 0.023 and wall motion score (1.88–1.5 (P = 0.017 improved but not global longitudinal strain. Initial LVEF (34%; P = 0.046 and global longitudinal strain (−9.5%; P = 0.095 were lower in patients with a final LVEF <40%. Conclusions: This is the first known report on STE in a series of patients with clinical lupus myocarditis. Global longitudinal strain correlated with regional and global left ventricular function. Global longitudinal strain, wall motion score and diastolic parameters may be more sensitive markers of lupus myocarditis in patients presenting with a preserved LVEF ≥50%. A poor initial LVEF and global longitudinal strain were associated with a persistent LVEF <40%. Echocardiography is a non-invasive tool with diagnostic and

  8. Effective Self-Management Interventions for Patients With Lupus: Potential Impact of Peer Mentoring.

    Science.gov (United States)

    Williams, Edith M; Egede, Leonard; Faith, Trevor; Oates, James

    2017-06-01

    Systemic lupus erythematosus (SLE) is associated with significant mortality, morbidity and cost for the individual patient and society. In the United States, African Americans (AAs) have 3-4 times greater prevalence of lupus, risk of developing lupus at an earlier age and lupus-related disease activity, organ damage and mortality compared with whites. Evidence-based self-management interventions that incorporate both social support and health education have reduced pain, improved function and delayed disability among patients with lupus. However, AAs and women are still disproportionately affected by lupus. This article presents the argument that peer mentoring may be an especially effective intervention approach for AA women with SLE. SLE peers with a track record of success in lupus management and have a personal perspective that clinicians often lack. This commonality and credibility can establish trust, increase communication and, in turn, decrease disparities in healthcare outcomes. Copyright © 2017 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  9. Cortical Thickness and Episodic Memory Impairment in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Bizzo, Bernardo Canedo; Sanchez, Tiago Arruda; Tukamoto, Gustavo; Zimmermann, Nicolle; Netto, Tania Maria; Gasparetto, Emerson Leandro

    2017-01-01

    The purpose of this study was to investigate differences in brain cortical thickness of systemic lupus erythematosus (SLE) patients with and without episodic memory impairment and healthy controls. We studied 51 patients divided in 2 groups (SLE with episodic memory deficit, n = 17; SLE without episodic memory deficit, n = 34) by the Rey Auditory Verbal Learning Test and 34 healthy controls. Groups were paired based on sex, age, education, Mini-Mental State Examination score, and accumulation of disease burden. Cortical thickness from magnetic resonance imaging scans was determined using the FreeSurfer software package. SLE patients with episodic memory deficits presented reduced cortical thickness in the left supramarginal cortex and superior temporal gyrus when compared to the control group and in the right superior frontal, caudal, and rostral middle frontal and precentral gyri when compared to the SLE group without episodic memory impairment considering time since diagnosis of SLE as covaried. There were no significant differences in the cortical thickness between the SLE without episodic memory and control groups. Different memory-related cortical regions thinning were found in the episodic memory deficit group when individually compared to the groups of patients without memory impairment and healthy controls. Copyright © 2016 by the American Society of Neuroimaging.

  10. 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.

  11. Recent insights into the genetic basis of systemic lupus erythematosus.

    Science.gov (United States)

    Moser, K L; Kelly, J A; Lessard, C J; Harley, J B

    2009-07-01

    Genetic variation was first shown to be important in systemic lupus erythematosus (SLE or lupus) in the 1970s with associations in the human leukocyte antigen region. Almost four decades later, and with the help of increasingly powerful genetic approaches, more than 25 genes are now known to contribute to the mechanisms that predispose individuals to lupus. Over half of these loci have been discovered in the past 2 years, underscoring the extraordinary success of genome-wide association approaches in SLE. Well-established risk factors include alleles in the major histocompatibility complex region (multiple genes), IRF5, ITGAM, STAT4, BLK, BANK1, PDCD1, PTPN22, TNFSF4, TNFAIP3, SPP1, some of the Fcgamma receptors, and deficiencies in several complement components, including C1q, C4 and C2. As reviewed here, many susceptibility genes fall into key pathways that are consistent with previous studies implicating immune complexes, host immune signal transduction and interferon pathways in the pathogenesis of SLE. Other loci have no known function or apparent immunological role and have the potential to reveal novel disease mechanisms. Certainly, as our understanding of the genetic etiology of SLE continues to mature, important new opportunities will emerge for developing more effective diagnostic and clinical management tools for this complex autoimmune disease.

  12. Prevalence and associated factors of resting electrocardiogram abnormalities among systemic lupus erythematosus patients without cardiovascular disease

    OpenAIRE

    Al Rayes, Hanan; Harvey, Paula J.; Gladman, Dafna D.; Su, Jiandong; Sabapathy, Arthy; Urowitz, Murray B.; Touma, Zahi

    2017-01-01

    Background Electrocardiogram (ECG) cardiovascular disease (CVD) abnormalities (ECG-CVD) are predictive of subsequent CVD events in the general population. Systemic lupus erythematosus (SLE) patients are vulnerable to CVD. We aimed to determine the prevalence of ECG-CVD in SLE patients and to examine the risk factors associated with ECG-CVD. Methods A 12-lead resting supine ECG was performed on consecutive adult patients attending the clinic. One cardiologist interpreted the ECGs. ECG-CVD were...

  13. Breast vasculitis in association with breast gigantism in a pregnant patient with systemic lupus erythematosus.

    Science.gov (United States)

    Propper, D J; Reid, D M; Stankler, L; Eastmond, C J

    1991-01-01

    A 24 year old woman with systemic lupus erythematosus (SLE) developed widespread necrotic skin ulceration and gigantism of both breasts during an exacerbation of SLE in the last trimester of her second pregnancy. Over the remainder of the pregnancy the ulceration was only controlled by high dose corticosteroids. After parturition, however, it was possible to reduce the steroid dose without recurrence of the ulceration. Images PMID:1888201

  14. Renal tubular dysfunction presenting as recurrent hypokalemic periodic quadriparesis in systemic lupus erythematosus

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    D Prasad

    2014-01-01

    Full Text Available We report recurrent hypokalemic periodic quadriparesis in a 30-year-old woman. Patient had also symptoms of multiple large and small joint pain, recurrent oral ulceration, photosensitivity and hair loss that were persisting since last 6 months and investigations revealed systemic lupus erythematosus (SLE with distal tubular acidosis. Our patient was successfully treated with oral potassium chloride, sodium bicarbonate, hydroxychloroquine and a short course of steroids. Thus, tubular dysfunction should be carefully assessed in patients with SLE.

  15. 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

  16. Thin-section chest CT findings in systemic lupus erythematosus with antiphospholipid syndrome: A comparison with systemic lupus erythematosus without antiphospholipid syndrome

    International Nuclear Information System (INIS)

    Oki, Hodaka; Aoki, Takatoshi; Saito, Kazuyoshi; Yamashita, Yoshiko; Hanamiya, Mai; Hayashida, Yoshiko; Tanaka, Yoshiya; Korogi, Yukunori

    2012-01-01

    Purpose: To assess thin-section chest CT findings in systemic lupus erythematosus (SLE) with antiphospholipid syndrome (APS), in comparison with SLE without APS. Materials and methods: We retrospectively reviewed the medical records and thin-section CT findings of 17 consecutive patients with an established diagnosis of SLE with APS, comparing with 37 consecutive SLE patients without APS, between 2004 and 2008, and patients who had other autoimmune disease, such as Sjögren syndrome, were excluded. No significant differences were seen between the two groups in age, gender, smoking habits, or history of steroid pulse and biological therapy. CT images of 2 mm thickness obtained with a 16- or 64-detector row CT were retrospectively evaluated by two radiologists in consensus on ultra high-resolution gray-scale monitors. Results: The frequency of thin-section CT abnormalities was higher in SLE with APS group (82%) than in SLE without APS group (43%). Ground-glass opacity (59%), architectural distortion (47%), reticulation (41%), enlarged peripheral pulmonary artery (29%), and mosaic attenuation (29%) were significantly more common in the SLE with APS group than in the SLE without APS group (Fisher's exact test, p < 0.01). Conclusion: SLE patients with APS have increased prevalence of thin-section chest CT abnormalities than those without APS.

  17. Pathogenic and Epiphenomenal Anti-DNA Antibodies in SLE

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    Mirjana Pavlovic

    2010-01-01

    Full Text Available The discoveries of natural and the development of manufactured highly efficient catalytic antibodies (abzymes opens the door to many practical applications. One of the most fascinating is the use of such antibodies in human therapy and prevention (vaccination, of cancer, AIDS, autoimmune diseases. A special entity of naturally occurring DNA hydrolytic anti-DNA antibodies is emerging within past decades linked to autoimmune and lymphoproliferative disorders, such as systemic lupus erythematosus (SLE, multiple sclerosis (MS, Sjogren Syndrome (SS, B - Chronic lymphocytic leucosis (B-CLL, and Multiple Myeloma (MM. The origin of the antibodies is unknown. The underlying mechanisms of these activities are suggested to be penetration into the living cells and translocation in the nucleus, with recognition of the specific binding sites at particular (ss or ds DNA. There are controversies in the literature whether hydrolysis is a sequence-specific event. The interplay between anti-DNA antibodies and DNA is not yet elucidated. This molecular “twist” also suggests that anti-DNA antibodies with DNA hydrolytic capacity could be the organism's immune response to a microbial attack, with microbial DNA, or specific genes within microbial DNA sequence, as a target for neutralization. The catalytic antibody-based approach can become a key tool in selective chemotherapeutic strategies.

  18. Genotoxic effect and antigen binding characteristics of SLE auto-antibodies to peroxynitrite-modified human DNA.

    Science.gov (United States)

    Khan, Md Asad; Alam, Khursheed; Mehdi, Syed Hassan; Rizvi, M Moshahid A

    2017-12-01

    Systemic lupus erythematosus (SLE) is an inflammatory autoimmune disease characterized by auto-antibodies against native deoxyribonucleic acid after modification and is one of the reasons for the development of SLE. Here, we have evaluated the structural perturbations in human placental DNA by peroxynitrite using spectroscopy, thermal denaturation and high-performance liquid chromatography (HPLC). Peroxynitrite is a powerful potent bi-functional oxidative/nitrative agent that is produced both endogenously and exogenously. In experimental animals, the peroxynitrite-modified DNA was found to be highly immunogenic. The induced antibodies showed cross-reactions with different types of DNA and nitrogen bases that were modified with peroxynitrite by inhibition ELISA. The antibody activity was inhibited by approximately 89% with its immunogen as the inhibitor. The antigen-antibodies interaction between induced antibodies with peroxynitrite-modified DNA showed retarded mobility as compared to the native form. Furthermore, significantly increased binding was also observed in SLE autoantibodies with peroxynitrite-modified DNA than native form. Moreover, DNA isolated from lymphocyte of SLE patients revealed significant recognition of anti-peroxynitrite-modified DNA immunoglobulin G (IgG). Our data indicates that DNA modified with peroxynitrite presents unique antigenic determinants that may induce autoantibody response in SLE. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. High-density genotyping of immune-related loci identifies new SLE risk variants in individuals with Asian ancestry.

    Science.gov (United States)

    Sun, Celi; Molineros, Julio E; Looger, Loren L; Zhou, Xu-Jie; Kim, Kwangwoo; Okada, Yukinori; Ma, Jianyang; Qi, Yuan-Yuan; Kim-Howard, Xana; Motghare, Prasenjeet; Bhattarai, Krishna; Adler, Adam; Bang, So-Young; Lee, Hye-Soon; Kim, Tae-Hwan; Kang, Young Mo; Suh, Chang-Hee; Chung, Won Tae; Park, Yong-Beom; Choe, Jung-Yoon; Shim, Seung Cheol; Kochi, Yuta; Suzuki, Akari; Kubo, Michiaki; Sumida, Takayuki; Yamamoto, Kazuhiko; Lee, Shin-Seok; Kim, Young Jin; Han, Bok-Ghee; Dozmorov, Mikhail; Kaufman, Kenneth M; Wren, Jonathan D; Harley, John B; Shen, Nan; Chua, Kek Heng; Zhang, Hong; Bae, Sang-Cheol; Nath, Swapan K

    2016-03-01

    Systemic lupus erythematosus (SLE) has a strong but incompletely understood genetic architecture. We conducted an association study with replication in 4,478 SLE cases and 12,656 controls from six East Asian cohorts to identify new SLE susceptibility loci and better localize known loci. We identified ten new loci and confirmed 20 known loci with genome-wide significance. Among the new loci, the most significant locus was GTF2IRD1-GTF2I at 7q11.23 (rs73366469, Pmeta = 3.75 × 10(-117), odds ratio (OR) = 2.38), followed by DEF6, IL12B, TCF7, TERT, CD226, PCNXL3, RASGRP1, SYNGR1 and SIGLEC6. We identified the most likely functional variants at each locus by analyzing epigenetic marks and gene expression data. Ten candidate variants are known to alter gene expression in cis or in trans. Enrichment analysis highlights the importance of these loci in B cell and T cell biology. The new loci, together with previously known loci, increase the explained heritability of SLE to 24%. The new loci share functional and ontological characteristics with previously reported loci and are possible drug targets for SLE therapeutics.

  20. The STAT4 SLE risk allele rs7574865[T] is associated with increased IL-12-induced IFN-γ production in T cells from patients with SLE.

    Science.gov (United States)

    Hagberg, Niklas; Joelsson, Martin; Leonard, Dag; Reid, Sarah; Eloranta, Maija-Leena; Mo, John; Nilsson, Magnus K; Syvänen, Ann-Christine; Bryceson, Yenan T; Rönnblom, Lars

    2018-02-23

    Genetic variants in the transcription factor STAT4 are associated with increased susceptibility to systemic lupus erythematosus (SLE) and a more severe disease phenotype. This study aimed to clarify how the SLE-associated intronic STAT4 risk allele rs7574865[T] affects the function of immune cells in SLE. Peripheral blood mononuclear cells (PBMCs) were isolated from 52 genotyped patients with SLE. Phosphorylation of STAT4 (pSTAT4) and STAT1 (pSTAT1) in response to interferon (IFN)-α, IFN-γ or interleukin (IL)-12, total levels of STAT4, STAT1 and T-bet, and frequency of IFN-γ + cells on IL-12 stimulation were determined by flow cytometry in subsets of immune cells before and after preactivation of cells with phytohaemagglutinin (PHA) and IL-2. Cellular responses and phenotypes were correlated to STAT4 risk allele carriership. Janus kinase inhibitors (JAKi) selective for TYK2 (TYK2i) or JAK2 (JAK2i) were evaluated for inhibition of IL-12 or IFN-γ-induced activation of SLE PBMCs. In resting PBMCs, the STAT4 risk allele was neither associated with total levels of STAT4 or STAT1, nor cytokine-induced pSTAT4 or pSTAT1. Following PHA/IL-2 activation, CD8 + T cells from STAT4 risk allele carriers displayed increased levels of STAT4 resulting in increased pSTAT4 in response to IL-12 and IFN-α, and an augmented IL-12-induced IFN-γ production in CD8 + and CD4 + T cells. The TYK2i and the JAK2i efficiently blocked IL-12 and IFN-γ-induced activation of PBMCs from STAT4 risk patients, respectively. T cells from patients with SLE carrying the STAT4 risk allele rs7574865[T] display an augmented response to IL-12 and IFN-α. This subset of patients may benefit from JAKi treatment. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  1. Tir8/Sigirr prevents murine lupus by suppressing the immunostimulatory effects of lupus autoantigens

    Science.gov (United States)

    Lech, Maciej; Kulkarni, Onkar P.; Pfeiffer, Stephanie; Savarese, Emina; Krug, Anne; Garlanda, Cecilia; Mantovani, Alberto; Anders, Hans-Joachim

    2008-01-01

    The Sigirr gene (also known as Tir8) encodes for an orphan receptor of the Toll-like receptor (TLR)/interleukin 1 receptor family that inhibits TLR-mediated pathogen recognition in dendritic cells. Here, we show that Sigirr also inhibits the activation of dendritic cells and B cells upon exposure to RNA and DNA lupus autoantigens. To evaluate the functional role of Sigirr in the pathogenesis of systemic lupus erythematosus (SLE), we generated Sigirr-deficient C57BL/6-lpr/lpr mice. These mice developed a progressive lymphoproliferative syndrome followed by severe autoimmune lung disease and lupus nephritis within 6 mo of age as compared with the minor abnormalities observed in C57BL/6-lpr/lpr mice. Lack of Sigirr was associated with enhanced activation of dendritic cells and increased expression of multiple proinflammatory and antiapoptotic mediators. In the absence of Sigirr, CD4 T cell numbers were increased and CD4+CD25+ T cell numbers were reduced. Furthermore, lack of Sigirr enhanced the activation and proliferation of B cells, including the production of autoantibodies against multiple nuclear lupus autoantigens. These data identify Sigirr as a novel SLE susceptibility gene in mice. PMID:18644972

  2. Carotid intima-media thickness and arterial stiffness in pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Su-Angka, N; Khositseth, A; Vilaiyuk, S; Tangnararatchakit, K; Prangwatanagul, W

    2017-08-01

    Objectives The carotid intima-media thickness (CIMT) and carotid arterial stiffness index (CASI) act as the surrogate markers of atherosclerosis. We aim to assess CIMT and CASI in pediatric systemic lupus erythematosus (SLE). Methods Patients ≤ 20 years old fulfilling diagnostic criteria for SLE were enrolled. Patients with active smoking, coronary heart disease, cerebrovascular disease, arterial thrombosis, family history of hypercholesterolemia, chronic liver disease, or other chronic severe diseases were excluded. The patients were categorized into four groups: active SLE, age- and sex-matched control (control A), inactive SLE, and age- and sex-matched control (control I), according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). All subjects underwent ultrasound of carotid arteries to evaluate CIMT and CASI. Results One hundred and two SLE patients (26 active and 76 inactive) and one hundred and three healthy controls (26 control A and 77 control I) were enrolled. The median CIMT in all groups were not significantly different (0.43, 0.41-0.44; 0.43, 0.41-0.44; 0.42, 0.41-0.43; and 0.42, 0.41-0.43 mm, respectively).The CASI in active SLE (13.5, 11.4-17.3) was significantly higher than in control A (8.2, 7.2-9.2) ( p < 0.0001), whereas CASI in inactive SLE (12.7, 10.9-15.7) was significantly higher than in control I (8.9, 7.6-9.8). However, the CASI in active and inactive SLE was not significantly different. Conclusions The higher CASI in active and inactive pediatric SLE, implying functional change of carotid arteries, may be early evidence of increased atherosclerosis in pediatric SLE. This functional dysfunction has been found both in inactive and active SLE.

  3. Lupus Flare: An Uncommon Presentation of Disseminated Gonorrhea

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    Uyen To

    2014-01-01

    Full Text Available Gonorrhea is one of the most common sexually transmitted diseases in the US with 700,000 annual cases. Although most cases of gonorrhea are localized, approximately 0.5–3% become disseminated. Here we discuss a rare case of a patient with systemic lupus erythematosus (SLE who developed septic shock from disseminated gonorrhea infection (DGI. Our patient is a 24-year-old woman with SLE, mixed connective tissue disease with cutaneous vasculitis, and lupus nephritis who presented with several weeks of malaise and generalized body aches associated with a diffuse rash along her fingers, palms, and trunk. Infectious workup was unrevealing with the exception of a positive gonorrhea test obtained from a cervical swab. Given her symptoms of tenosynovitis, the appearance of her skin lesions, and her positive gonorrhea test, she was diagnosed with septic shock secondary to DGI. With antibiotic treatment, the patient reported a dramatic improvement of the pain in her swollen joints and her rash receded. Patients diagnosed with SLE carry an increased risk of gonorrhea regardless of whether or not they are being treated for their SLE. Although it is well-documented that SLE is associated with severe DGI, few describe it resulting in overt septic shock.

  4. Serum markers thrombophilia in pregnant women with Systemic Lupus Erythematosus

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    Vanessa Marcon de Oliveira

    Full Text Available Abstract Objectives: to determine the frequency of serum markers for hereditary and acquired thrombophilia and their association with pregnancy in women with Systemic Lupus Erythematosus (SLE. Methods: a case-control study was conducted among 25 pregnant women with SLE (study group and 32 pregnant women without known disease and with at least one previous pregnancy (control group. The presence of antiphospholipid antibodies and hereditary thrombophilia were examined in both groups. We used the y2 Test with Yates correction or Fisher's Exact Test to verify the associations and calculate the relative risk. Results: thrombophilia was present in 72.0% of pregnant women with SLE and in 6.0% of patients in the control group. A significant association was found between the presence of SLE and serum markers for hereditary thrombophilia / antiphospholipid antibodies (p<0.05. The relative risks for antiphospholipid antibodies were 13.20 (ICR95%= 1.81 - 96.46 in pregnant women with SLE, 7.26 (CI95%= 1.77 - 29.86 for the presence of serum markers of hereditary thrombophilia and 7.92 (CI95%= 2.62 - 3.94 for the presence of hereditary thrombophilia and/or antiphospholipid antibodies. Conclusions: the identification of markers for hereditary and/or acquired thrombophilia in pregnant women with lupus may be clinically useful to determine which patients have a higher risk of obstetric complications.

  5. Numerous Fusiform and Saccular Cerebral Aneurysms in Central Nervous System Lupus Presenting with Ischemic Stroke.

    Science.gov (United States)

    Majidi, Shahram; Leon Guerrero, Christopher R; Gandhy, Shreya; Burger, Kathleen M; Sigounas, Dimitri

    2017-07-01

    Central nervous system (CNS) involvement occurs in up to 50% of patients with systemic lupus erythematosus (SLE). Cerebral aneurysm formation is a rare complication of CNS lupus. The majority of these patients present with subarachnoid hemorrhage. We report a patient with an active SLE flare who presented with a recurrent ischemic stroke and was found to have numerous unruptured fusiform and saccular aneurysms in multiple vascular territories. He was treated with high-dose steroid and rituximab along with aspirin and blood pressure control for stroke prevention. Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  6. Off-label use of rituximab for systemic lupus erythematosus in Europe

    DEFF Research Database (Denmark)

    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...

  7. Purified umbilical cord derived mesenchymal stem cell treatment in a case of systemic lupus erythematosus.

    Science.gov (United States)

    Phillips, Christopher D; Wongsaisri, Pornpatcharin; Htut, Thein; Grossman, Terry

    2017-12-01

    Systemic lupus erythematosus (SLE) is a multiple organ system autoimmune disorder for which there is no known cure. We report a case of a young adult lady with SLE and Sjogren's with diagnostic and clinical resolution following purified umbilical cord derived mesenchymal stem cell (MSC) and globulin component protein macrophage activating factor (GcMAF) therapy in a combined multidisciplinary integrative medicine protocol. Our patient had complete reversal of all clinical and laboratory markers. We recommend a prospective randomized double blind study to assess the sustained efficacy of MSC and GcMAF in the treatment of autoimmune connective tissue diseases such as systemic lupus erythematosus.

  8. Purtscher-like retinopathy in systemic lupus erythematosus.

    Science.gov (United States)

    Wu, Chan; Dai, Rongping; Dong, Fangtian; Wang, Qian

    2014-12-01

    To investigate clinical characteristics of Purtscher-like retinopathy and its clinical implications among patients with systemic lupus erythematosus (SLE). Observational case series. setting: Tertiary medical center. patient population: Patients with SLE who were diagnosed with Purtscher-like retinopathy between 2002 and 2013. observation procedures: Assessment and follow-up in the ophthalmology department. main outcome measure: Visual acuity and funduscopic examination at presentation and at 6 month follow-up, with analysis of the association between Purtscher-like retinopathy and other systemic involvement of SLE and overall disease activity. Among 5688 patients with SLE evaluated, 8 cases of Purtscher-like retinopathy were diagnosed. Typical fundus abnormalities included Purtscher flecken, cotton-wool spots, retinal hemorrhages, macular edema, optic disk swelling, and a pseudo-cherry red spot. Fluorescein angiography abnormalities included areas of capillary nonperfusion corresponding to the retinal whitening, late leakage, peripapillary staining, precapillary occlusion, and slower filling of vessels. The prevalence of central nervous system lupus was significantly higher among those with Purtscher-like retinopathy (6/8) than among 240 patients randomly sampled from those without Purtscher-like retinopathy. A very high SLE Disease Activity Index (≥20) was present in all 8 patients with Purtscher-like retinopathy. All patients received corticosteroids combined with immunosuppressants. For the majority of patients, optic atrophy developed during follow-up with persistent low visual acuity. As a rare and severe ophthalmic complication of SLE, Purtscher-like retinopathy was associated with central nervous system lupus and highly active disease. Visual acuity recovery was usually poor despite prompt treatment. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. 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.

  10. 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.

  11. Genetic polymorphisms of dsRNA ligating pattern recognition receptors TLR3, MDA5, and RIG-I. Association with systemic lupus erythematosus and clinical phenotypes

    DEFF Research Database (Denmark)

    Enevold, C; Kjaer, Lasse; Nielsen, Claus Henrik

    2014-01-01

    This study aimed to demonstrate possible associations between genetic polymorphisms in Toll-like receptor 3, interferon induced with helicase C domain 1 (IFIH1) and DEAD (Asp-Glu-Ala-Asp) box polypeptide 58 and systemic lupus erythematosus (SLE), including the phenotypes lupus nephritis and malar...

  12. The Involvement of MicroRNAs in Modulation of Innate and Adaptive Immunity in Systemic Lupus Erythematosus and Lupus Nephritis

    Science.gov (United States)

    Köhler, Paulina; von Rauchhaupt, Ekaterina

    2018-01-01

    Noncoding RNAs (ncRNAs), including microRNAs (miRNAs), represent a family of RNA molecules that do not translate into protein. Nevertheless, they have the ability to regulate gene expression and play an essential role in immune cell differentiation and function. MicroRNAs were found to be differentially expressed in various tissues, and changes in their expression have been associated with several pathological processes. Yet, their roles in systemic lupus erythematosus (SLE) and lupus nephritis (LN) remain to be elucidated. Both SLE and LN are characterized by a complex dysfunction of the innate and adaptive immunity. Recently, significant findings have been made in understanding SLE through the use of genetic variant identification and expression pattern analysis and mouse models, as well as epigenetic analyses. Abnormalities in immune cell responses, cytokine and chemokine production, cell activation, and apoptosis have been linked to a unique expression pattern of a number of miRNAs that have been implicated in the immune pathogenesis of this autoimmune disease. The recent evidence that significantly increased the understanding of the pathogenesis of SLE drives a renewed interest in efficient therapy targets. This review aims at providing an overview of the current state of research on the expression and role of miRNAs in the immune pathogenesis of SLE and LN. PMID:29854836

  13. The Involvement of MicroRNAs in Modulation of Innate and Adaptive Immunity in Systemic Lupus Erythematosus and Lupus Nephritis.

    Science.gov (United States)

    Honarpisheh, Mohsen; Köhler, Paulina; von Rauchhaupt, Ekaterina; Lech, Maciej

    2018-01-01

    Noncoding RNAs (ncRNAs), including microRNAs (miRNAs), represent a family of RNA molecules that do not translate into protein. Nevertheless, they have the ability to regulate gene expression and play an essential role in immune cell differentiation and function. MicroRNAs were found to be differentially expressed in various tissues, and changes in their expression have been associated with several pathological processes. Yet, their roles in systemic lupus erythematosus (SLE) and lupus nephritis (LN) remain to be elucidated. Both SLE and LN are characterized by a complex dysfunction of the innate and adaptive immunity. Recently, significant findings have been made in understanding SLE through the use of genetic variant identification and expression pattern analysis and mouse models, as well as epigenetic analyses. Abnormalities in immune cell responses, cytokine and chemokine production, cell activation, and apoptosis have been linked to a unique expression pattern of a number of miRNAs that have been implicated in the immune pathogenesis of this autoimmune disease. The recent evidence that significantly increased the understanding of the pathogenesis of SLE drives a renewed interest in efficient therapy targets. This review aims at providing an overview of the current state of research on the expression and role of miRNAs in the immune pathogenesis of SLE and LN.

  14. The relationship between increased levels of Anti-dsDNA with clinical manifestation in patients with SLE in Haji Adam Malik General Hospital Medan

    Science.gov (United States)

    Marpaung, B.; Patrick, J.

    2018-03-01

    Systemic Lupus Erythematosus (SLE) is an autoimmune rheumatic disease characterized by widespread inflammation and affects any organism the body. Many autoimmune diseases result in autoantibody production, but Anti-dsDNA antibodies are highly specific to SLE. Previous study found that Anti-dsDNA antibodies are associated with severe clinical manifestations of lupus. The aim of this study was to examine the relationship between anti-dsDNA level with clinical features and laboratory findings in SLE patients. This cross-sectional study was conducted in Hospital Haji Adam Malik Medan in May-October 2016.We examine anti-dsDNA, clinical features and kidney laboratory profile in all patient. Data were statistically analyzed.81 SLE patients with median level of anti-dsDNA 294 (6.1-1317). There was no significant relationship between increased level of Anti-dsDNA with clinical manifestations (p>0.05). There were significant relationships between increased level of Anti-dsDNA with renal impairment (p=0.049), urea level (p=0.016), urine protein (p=0.042) and hematology disorder (p=0.005). Arthritis is the most frequent clinical manifestation (96.3%) followed by malar rash (77.8%). Elevated anti-dsDNA level was not related with clinical manifestations but there was significant relationship with hematology disorder, urea, creatinine, and proteinuria in SLE patents.

  15. Macrophage Activation Syndrome as Initial Presentation of Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Say-Tin Yeap

    2008-04-01

    Full Text Available Macrophage activation syndrome (MAS is known to be a severe and potentially life-threatening complication of rheumatic disorder, especially systemic juvenile rheumatoid arthritis. It is very rare for MAS to be an initial presentation of systemic lupus erythematosus (SLE. Here, we report a 14-year-old girl in whom MAS developed as an initial presentation of SLE. With early diagnosis and administration of cyclosporine A, she had a fair outcome. Further testing showed positive anti-dsDNA about 8 months later.

  16. Cardiac manifestation's history in the systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Iglesias Gamarra, Antonio; Rondon, Federico; Restrepo, Jose Felix

    2001-01-01

    In this paper it is broadly and in depth reviewed the cardiac manifestation's history of systemic lupus erythematosus (SLE), since an historical analysis of clinical manifestations both in pre and post corticosteroids period. The way how the heart and the cardiovascular system's functions have been studied by clinical and semiological views are showed, through clinical manifestations such as myocarditis pericarditis, endocarditis, rhythm alterations, etc, and the evolution of laboratory methods used to its study as well as immunologic prognostic markers and risk factors for coronary disease in SLE

  17. Angiotensin-converting enzyme (ACE) gene insertion/deletion polymorphism is not a risk factor for hypertension in SLE nephritis.

    Science.gov (United States)

    Negi, Vir S; Devaraju, Panneer; Gulati, Reena

    2015-09-01

    SLE is a systemic autoimmune disease with high prevalence of hypertension. Around 40-75 % of SLE patients develop nephritis, a major cause of hypertension and mortality. Angiotensin-converting enzyme (ACE) maintains the blood pressure and blood volume homeostasis. An insertion/deletion (I/D) polymorphism in intron 16 of ACE gene was reported to influence the development of hypertension, nephritis, and cardiovascular diseases in different ethnic populations. Despite compelling evidence for the high prevalence of hypertension in individuals with SLE, underlying factors for its development are not well studied. With this background, we analyzed the influence of ACE insertion/deletion polymorphism on susceptibility to SLE, development of nephritis and hypertension, other clinical features and autoantibody phenotype in South Indian SLE patients. Three hundred patients with SLE and 460 age and sex similar ethnicity matched individuals were included as patients and healthy controls, respectively. The ACE gene insertion/deletion polymorphism was analyzed by PCR. Insertion (I) and deletion (D) alleles were observed to be equally distributed among patients (57 and 43 %) and controls (59 and 41 %), respectively. The mutant (D) allele did not confer significant risk for SLE (II vs. ID: p = 0.4, OR 1.15, 95 % CI 0.8-1.6; II vs. DD: p = 0.34, OR 1.22, 95 % CI 0.8-1.85). There was no association of the ACE genotype or the allele with development of lupus nephritis (II vs. ID: p = 0.19, OR 1.41, 95 % CI 0.84-2.36; II vs. DD: p = 0.41, OR 0.74, 95 % CI 0.38-1.41) or hypertension (II vs. ID: p = 0.85, OR 0.9, 95 % CI 0.43-1.8; II vs. DD: p = 0.66, OR 1.217, 95 % CI 0.5-2.8). The presence of mutant allele (D) was not found to influence any clinical features or autoantibody phenotype. The insertion/deletion polymorphism of the ACE gene is not a genetic risk factor for SLE and does not influence development of hypertension or lupus nephritis in South Indian

  18. Systemic lupus erythaematosus in a multiethnic US cohort (LUMINA) LIII: disease expression and outcome in acute onset lupus.

    Science.gov (United States)

    Bertoli, A M; Vilá, L M; Reveille, J D; Alarcón, G S

    2008-04-01

    To determine the features associated with acute onset systemic lupus erythaematosus (SLE). A total of 631 SLE patients from LUMINA (for "lupus in minority populations: nature vs nurture"), a multiethnic (Hispanics, African-Americans and Caucasians) cohort, were studied. Acute disease onset was defined as the accrual of > or = 4 American College of Rheumatology (ACR) criteria for the classification of SLE in < or = 4 weeks. Socioeconomic demographic features, clinical manifestations, disease activity, damage accrual, mortality, autoantibodies, HLA class II and FCGR alleles, behavioural/psychological variables were compared between patients with acute and insidious disease onset by univariable (chi(2) and Student t test) and multivariable (stepwise logistic regression) analyses. A total of 94 (15%) patients had acute disease onset. In the multivariable analysis, patients with acute onset lupus had more renal involvement (odds ratio (OR) = 1.845, 95% CI 1.076-3.162; p = 0.026) and higher disease activity (OR = 1.057, 95% CI 1.005-1.112; p = 0.030). By contrast, age (OR = 0.976, 95% CI 0.956-0.997; p = 0.025), education (OR = 0.901, 95% CI 0.827-0.983, p = 0.019), health insurance (OR = 0.423, 95% CI 0.249-0.718; p = 0.001) and skin involvement (OR = 0.346, 95% CI 0.142-0.843; p = 0.019) were negatively associated with acute onset lupus. No differences were found regarding the serological, genetic and behavioural/psychological features; this was also the case for damage accrual and mortality. Patients with acute onset lupus seem to be younger, have a lower socio-economic status and display more severe disease in terms of clinical manifestations and disease activity. However, intermediate (damage) and long-term (mortality) outcomes appear not to be influenced by the type of disease onset in SLE.

  19. Type I Interferon in the Pathogenesis of Lupus

    Science.gov (United States)

    Crow, Mary K.

    2014-01-01

    Investigations of patients with systemic lupus erythematosus (SLE) have applied insights from studies of the innate immune response to define type I interferon (IFN-I), with IFN-α the dominant mediator, as central to the pathogenesis of this prototype systemic autoimmune disease. Genetic association data identify regulators of nucleic acid degradation and components of TLR-independent, endosomal TLR-dependent, and IFN-I signaling pathways as contributors to lupus disease susceptibility. Together with a gene expression signature characterized by IFNI-induced gene transcripts in lupus blood and tissue, those data support the conclusion that many of the immunologic and pathologic features of this disease are a consequence of a persistent self-directed immune reaction driven by IFN-I and mimicking a sustained anti-virus response. This expanding knowledge of the role of IFN-I and the innate immune response suggests candidate therapeutic targets that are being tested in lupus patients. PMID:24907379

  20. High Health Care Utilization Preceding Diagnosis of Systemic Lupus Erythematosus in Youth.

    Science.gov (United States)

    Chang, Joyce C; Mandell, David S; Knight, Andrea M

    2017-12-01

    Childhood-onset systemic lupus erythematosus (SLE) is associated with high risk for organ damage, which may be mitigated by early diagnosis and treatment. We characterized health care utilization for youth in the year preceding SLE diagnosis compared to controls. Using Clinformatics ™ DataMart (OptumInsight, Eden Prairie, MN) de-identified administrative data from 2000 to 2013, we identified 682 youth ages 10-24 years with new-onset SLE (≥3 International Classification of Diseases, Ninth Revision (ICD-9) codes for SLE 710.0, each >30 days apart), and 1,364 age and sex-matched healthy controls. We compared the incidence of ambulatory, emergency, and inpatient visits 12 months before SLE diagnosis, and frequency of primary diagnoses. We examined subject characteristics associated with utilization preceding SLE diagnosis. Youth with SLE had significantly more visits in the year preceding diagnosis than controls across ambulatory (incidence rate ratio (IRR) 2.48, p<0.001), emergency (IRR 3.42, p<0.001) and inpatient settings (IRR 3.02, p<0.001). The most frequent acute care diagnoses and median days to SLE diagnosis were: venous thromboembolism (313, interquartile range (IQR) 18-356), thrombocytopenia (278, IQR 39-354), chest pain (73, IQR 29.5-168), fever (52, IQR 17-166), and acute kidney failure (14, IQR 5-168). Having a psychiatric diagnosis prior to SLE diagnosis was strongly associated with increased utilization across all settings. Youth with SLE have high health care utilization throughout the year preceding SLE diagnosis. Examining variable diagnostic trajectories of youth presenting for acute care preceding SLE diagnosis, and increased attention to psychiatric morbidity may help improve care for youth with new-onset SLE. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  1. Annual incidence and standardized incidence ratio of cerebrovascular accidents in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Mok, C C; Ho, L Y; To, C H

    2009-01-01

    To study the annual incidence and standardized incidence ratio (SIR) of cerebrovascular accident (CVA) in patients with systemic lupus erythematosus (SLE). The annual incidence of CVA from 1999 to 2007 in a longitudinal cohort of SLE patients was calculated each year and compared with that of the regional population within the same study period. Age-specific SIRs and outcome of CVA in SLE patients were also studied. In 2007, there were 490 SLE patients in our cohort. The mean annual incidence of CVA between 1999 and 2007 was 6.45/1000 patients and no obvious trend over time was observed. Of the 20 CVAs in patients with SLE, 18 (90%) were ischaemic stroke whereas two (10%) were haemorrhagic stroke. The mean SIR of all types of CVA in SLE patients was 2.02 [95% confidence interval (CI) 1.30-3.81; p = 0.002]. The SIR of ischaemic stroke decreased with age and the stroke incidence was no longer significantly higher than that of the population in patients aged >or= 60 years. Haemorrhagic stroke occurred mainly in younger SLE patients. The duration of hospitalization and the mortality rate for CVA was non-significantly higher in SLE than in non-SLE patients. The incidence of CVA in SLE remained constant over the 8 years between 1999 and 2007. Younger SLE patients are at substantially increased risk of CVA compared to age-matched population. The duration of hospitalization and the mortality rate for CVA are similar in SLE and non-SLE patients.

  2. Cytokines in systemic lupus erythematosus: far beyond Th1/Th2 dualism lupus: cytokine profiles.

    Science.gov (United States)

    Guimarães, Poliana Macedo; Scavuzzi, Bruna Miglioranza; Stadtlober, Nicole Perugini; Franchi Santos, Lorena Flor da Rosa; Lozovoy, Marcell Alysson Batisti; Iriyoda, Tatiana Mayumi Veiga; Costa, Neide Tomimura; Reiche, Edna Maria Vissoci; Maes, Michael; Dichi, Isaias; Simão, Andréa Name Colado

    2017-10-01

    The aims of this study were to delineate cytokine profiles of systemic lupus erythematosus (SLE), construct prediction models for diagnosis and disease activity using those profiles, and to examine the associations between TNFB Ncol polymorphism, body mass index (BMI) and vitamin D levels with cytokine levels. Two hundred SLE patients and 196 healthy controls participated in this case-control study. Plasma cytokines levels of tumor necrosis factor (TNF)-α, interferon (IFN)-γ, interleukin (IL)-1β, IL- 4, IL-6, IL-10, IL-12 and IL-17 were measured and cytokines profiles were computed. IL-6, IL-12, IL-17, IFN-γ and IL-10 levels were significantly higher in SLE, while IL-4 was lower in SLE. The Th1/Th2 and Th1+Th17/Th2 profiles were significantly higher in SLE than in healthy controls, whereas there were no significant differences in the proinflammatory cytokine profile (TNFα+IL-6+IL-1β). In total, 90.4% of all subjects were correctly classified using Th1+Th17 profile and IL-10 (positively associated) and IL-4 (negatively associated) as predictor variables (sensitivity=66.7% and specificity=96.9%). In all, 20.9% of the variance in the SLE Disease Activity Index was predicted by the Th1+Th17/Th2 ratio, IL-10 and BMI (all positively) and proinflammatory profile (inversely associated). B1/B1 genotype is accompanied by increased IL-17 and Th17/Th2 ratio, while B1/B2 genotype is accompanied by higher IL-4 and IFNγ values. 25-OH vitamin D was inversely associated with IFN-γ levels. SLE is accompanied by Th1, Th17 and Treg profile and lowered IL-4 production. Lowered vitamin D levels and B1/B1 genotype, but not BMI, contribute to changes in cytokines profiles. Future treatments should target Th1, Th2 and Th17 profiles rather than inflammatory cytokines.

  3. Evaluation of cerebral perfusion in patients with neuropsychiatric systemic lupus erythematosus using 123I-IMP SPECT

    International Nuclear Information System (INIS)

    Yoshida, Atsuko; Shishido, Fumio; Kato, Kazuo; Watanabe, Hiroshi; Seino, Osamu

    2007-01-01

    In the course of systemic lupus erythematosus (SLE), central nervous system (CNS) complications occur at a high frequency. An accurate diagnosis of CNS lupus, differentiated from secondary CNS involvement, is difficult. CNS lupus is indicative of advancing primary disease and is treated by steroid pulse therapy or increased dosage of steroids. In contrast, if symptoms are caused by secondary CNS complications, it is possible to observe or treat these complications using symptomatic therapy. We examined whether quantitative cerebral blood flow (CBF) measured using cerebral perfusion single photon emission computed tomography (SPECT) can be used to differentiate CNS lupus from secondary CNS involvement. We divided 18 SLE patients with CNS symptoms into a CNS lupus group and a non-CNS lupus group, and then compared the mean cerebral blood flow (mCBF) of each group of patients. SPECT was performed with N-isopropyl-p-[ 123 I] iodoamphetamine (IMP), with quantitation carried out by table look-up and autoradiographic methods. The mCBF of both groups was decreased; however, the mCBF of patients with CNS lupus was significantly lower than that of non-CNS lupus patients. Quantitative CBF may provide a useful tool to distinguish CNS lupus from non-CNS lupus. (author)

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

    Directory of Open Access Journals (Sweden)

    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.

  5. Successful treatment of massive ascites due to lupus peritonitis with hydroxychloroquine in old- onset lupus erythematosus.

    Science.gov (United States)

    Hammami, Sonia; Bdioui, Fethia; Ouaz, Afef; Loghmari, Hichem; Mahjoub, Sylvia; Saffar, Hamouda

    2014-01-01

    Systemic lupus erythematous (SLE) is an auto-immune disease with multiple organ involvements that occurs mainly in young women. Literature data suggest that serositis is more frequent in late-onset SLE. However, peritoneal serositis with massive ascites is an extremely rare manifestation. We report a case of old-onset lupus peritonitis treated successfully by Hydroxychloroquine. A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis. Physical examination showed only massive ascites. Laboratory investigations showed leucopenia: 3100/mm3, lymphopenia: 840/mm3 and trace protein (0.03 g/24 h). Ascitic fluid contained 170 cells mm(3) (67% lymphocytes), 46 g/L protein, but no malignant cells. The main etiologies of exudative ascites were excluded. She had markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL) with hypo-complementemia (C3 levl was at 67 mg/dL). Antibody against the Smith antigen was also positive. Relying on these findings, the patient was diagnosed with SLE and treated with Hydroxychloroquine 200 mg daily in combination with diuretics. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take chloroquine. This case was characterized by old age of onset of SLE, the extremely rare initial presentation with lupus peritonitis and massive painful ascites with dramatic response to only hydroxychloroquine treatment.

  6. Depression and ways of coping in SLE induced arthritis patient: a case study

    International Nuclear Information System (INIS)

    Farah, D.; Nezam, N.; Naz, H.

    2012-01-01

    Systemic lupus erythematosus is a systemic autoimmune disease (autoimmune connective tissue disease) that most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys and nervous system. One of the outcomes of SLE is arthritis which is a group of conditions involving damage to the joints of the body. One of the most common types is rheumatoid arthritis which is an autoimmune immune disease. This SLE induced arthritis has many impacts on every aspect of life including biological, psychological, social and - financial domains. In most of the cases arthritis is associated With depression and anxiety. It is envisaged that people with arthritis suffer more from depression as compared to general population. The present study was designed to evaluate depression using lung Self Rating Scale and coping using two scales: Ways of Coping and Coping Self Efficacy in a 27 years old female participant with SLE induced Arthritis. The results of the two paradigms and detailed case history revealed that the level of depression lies in the normal range that coincides with the use of effective coping strategies. Thus the participant's positive outlook enabled to improve the quality of life. (author)

  7. Discoid Lupus Erythematosus

    Science.gov (United States)

    ... Name: Category: Share: Yes No, Keep Private Discoid Lupus Erythematosus Share | Discoid lupus erythematosus (DLE) is a chronic skin condition of ... occur. A small percentage of patients with discoid lupus can develop disease of the internal organs, which ...

  8. 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....... Another four co-twins had other autoimmune disease, corresponding to a probandwise concordance of any autoimmune disease of 50.0% in MZ (95% CI: 21.5-78.5) and 23.1% in DZ twins (95% CI: 8.18-50.3). CONCLUSION: Population-based Danish data suggest that SLE twin concordance is lower than previously...... reported, but still point to the importance of both genetic and environmental factors, and indicate a substantial co-aggregation of other autoimmune diseases in SLE twins....

  9. Lack of Association between an Interleukin-I Receptor Antagonist Gene Polymorphism and Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Victor A. Danis

    1994-01-01

    Full Text Available Non-MHC linked genes may contribute to genetic predisposition to the development of systemic lupus erythematosus. The possibility that cytokine genes may be involved was raised by the observation of increased frequency in expression of an uncommon allele of an interleukin-I receptor antagonist gene polymorphism and SLE in a recent U.K. study. We have not been able to show any significant differences in expression of this allele in SLE patients as a whole or in any patient subgroups. Our results actually show a slight decrease in the expression of this allele in SLE patients compared with healthy controls and in SLE patients with malar rash compared with SLE patients without malar rash.

  10. Dysregulation of Cell Death and Its Epigenetic Mechanisms in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Haijing Wu

    2016-12-01

    Full Text Available Systemic lupus erythematosus (SLE is a systemic autoimmune disease involving multiple organs and tissues, which is characterized by the presence of excessive anti-nuclear autoantibodies. The pathogenesis of SLE has been intensively studied but remains far from clear. Increasing evidence has shown that the genetic susceptibilities and environmental factors-induced abnormalities in immune cells, dysregulation of apoptosis, and defects in the clearance of apoptotic materials contribute to the development of SLE. As the main source of auto-antigens, aberrant cell death may play a critical role in the pathogenesis of SLE. In this review, we summarize up-to-date research progress on different levels of cell death—including increasing rate of apoptosis, necrosis, autophagy and defects in clearance of dying cells—and discuss the possible underlying mechanisms, especially epigenetic modifications, which may provide new insight in the potential development of therapeutic strategies for SLE.

  11. Lupus nephritis

    African Journals Online (AJOL)

    1991-03-02

    Mar 2, 1991 ... will benefit from immunosuppressive therapy. Our study found hypertension, which persisted at the most recent follow-up, to be associated with a poor outcome, as was poor renal function both at biopsy and follow-up. Leaker er al. I found that survival in lupus nephritis is unaffected by age, sex, nephrotic ...

  12. Lupus panniculitis

    International Nuclear Information System (INIS)

    Mondello, Eduardo; Vega, Alejandro de la; Eyheremendy, EduardoP.

    2002-01-01

    Lupus panniculitis (LP) is a benign entity. To our knowledge LP has not been reported in the radiology literature. Our objective is to describe imaging findings as previous articles have not focused on this aspect. Computed Tomography and MR imaging demonstrate lipoatrophy and isolated areas with inflammatory activity in the subcutaneous tissue. (author)

  13. Depression Risk in Young Adults With Juvenile- and Adult-Onset Lupus: Twelve Years of Followup.

    Science.gov (United States)

    Knight, Andrea M; Trupin, Laura; Katz, Patricia; Yelin, Edward; Lawson, Erica F

    2018-03-01

    To compare major depression risk among young adults with juvenile-onset and adult-onset systemic lupus erythematosus (SLE), and to determine demographic and health-related predictors of depression. Young adults with SLE ages 18-45 years (n = 546) in the Lupus Outcomes Study completed annual telephone surveys from 2002-2015, including assessment of depression using the Center for Epidemiologic Studies Depression Scale (CES-D), and self-report measures of sociodemographics and health characteristics. Juvenile-onset SLE was defined as age adult-onset SLE. Older age, lower educational attainment, and physical function, higher disease activity, and a history of smoking were associated with an increased depression risk. Juvenile-onset SLE patients had a higher risk of major depression across all educational groups. Young adults with SLE, particularly those with juvenile-onset disease, are at high risk for major depression, which is associated with increased disease activity, poorer physical functioning, and lower educational attainment. Early depression intervention in young adults with SLE has the potential to improve both medical and psychosocial outcomes. © 2017, American College of Rheumatology.

  14. IgA nephropathy in systemic lupus erythematosus patients: case report and literature review

    Directory of Open Access Journals (Sweden)

    Leonardo Sales da Silva

    2016-06-01

    Full Text Available Abstract Systemic erythematosus lupus (SLE is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non‐lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN, the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1,280, nuclear fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non‐SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in the short and long term.

  15. Brain MR imaging in systemic lupus erythematous

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hyun Ae; Chang, Kee Hyun; Han, Moon Hee; Lee, Kyung Hwon; Kim, Sung Kwon; Lee, Jung Sang [Seoul National University College of Medicine, Seoul (Korea, Republic of); Cha, Sang Hoon [Chungbuk National University College of Medicine, Chungju (Korea, Republic of)

    1992-09-15

    To present MR imaging findings of intracranial lesions in systemic lupus erythematosus(SLE), a retrospective study was performed on MR images of 33 SLE patients with neurologic symptoms and signs. MR imaging was performed on either a 0.5 T (21 patients) or 2.0 T unit (12 patients), using T1-weighted, proton-density-weighted, and T2-weighted spin echo sequences in all patients. In seven patients, post-contrast T1-weighted images were also obtained after administration of gadopentetate dimeglumine. The main MR findings consisted of focal lesions suggesting ischemia/infarct (15 patients), diffuse brain atrophy (8), and findings associated with infection (4). The MR findings were normal in 11 patients (33%). The focal lesions suggesting ischemia/infarcts presumably secondary to vasculitis were distributed in the cortex or subcortical white matter (7 patients), deep periventricular white matter (3), or in both areas (5). Most of the focal lesions were multiple and small in size. The findings associated with infection were variable and included communicating hydrocephalus, meningeal enhancement, granuloma, etc. MR findings of SLE were non-specific and therefore clinical correlation is needed when evaluating SLE in MR.

  16. [Role of anti c-mpl antibody in systemic lupus erythematosus with thrombocytopenia].

    Science.gov (United States)

    Yang, Tuo; Huang, Ci Bo; Lai, Bei; Zhao, Li Ke; Chen, Ying Juan; Zhao, Yue Tao; Zhang, Chun Mei; Zeng, Xiao Feng

    2012-04-18

    To determine whether anti-thrompoietin receptor (TPO-R, c-mpl) antibody contributes to thrombocytopenia in systemic lupus erytematosus (SLE) and explore the pathogenic role of this antibody. Sera from 24 SLE patients with thrombocytopenia, 27 SLE patients having normal platelet counts with a history of thrombocytopenia, 18 SLE patients with neither thrombocytopenia nor post thrombocytopenia and 18 healthy controls were collected. Anti c-mpl antibodies were detected by an indirected ELISA assay. The serum TPO levels were measured by an ELISA assay. Clinical findings, autoantibody profiles, and SLEDAI were evaluated. Serum anti c-mpl antibodies were detected in 18.8% of the SLE patientis. The frequency of this antibody in SLE with thrombocytopenia, SLE with a history of thrombocytopenia and SLE without thrombocytopenia were of no difference (P=0.600). In the patients with anti c-mpl antibodies, their platelet counts were decreased(P=0.025) and serum TPO levels elevated(P=0.038) than those in the patients without, while there were no differences between the two groups in C3, C4, ESR, CRP level, the frequency of ANA, dsDNA, ANCA and SLEDAI. Anti c-mpl antibody contributes to SLE-associated thrombocytopenia by functionally blocking an interaction between thrombopoietin and c-mpl, which might inhibit TPO-dependent megakaryocyte proliferation and differentiation.

  17. Human papilloma virus and lupus: the virus, the vaccine and the disease.

    Science.gov (United States)

    Segal, Yahel; Calabrò, Michele; Kanduc, Darja; Shoenfeld, Yehuda

    2017-07-01

    Systemic lupus erythematosus (SLE) is a well known, widespread autoimmune disease, involving multiple organ systems, with a multifaceted, widely unmapped etiopathogenesis. Recently, a new aspect of morbidity has been described among SLE patients: infection with human papilloma virus (HPV). We set out to review data regarding the intricate relationship between the two and attempt to determine whether HPV may pose as a contributing factor to the development of SLE. We relate to epidemiological, molecular and clinical data. We have found evidence in all these fields suggesting HPV to be involved in the pathogenesis of SLE: increased prevalence of HPV infection among SLE patients; vast molecular homology between viral peptides and human proteins associated with SLE; several reports of SLE development post-HPV vaccination. Our findings suggest a possible involvement of HPV infection in the induction of SLE, via a mechanism of immune cross-reaction due to molecular homology. We review clinical, epidemiological and molecular data suggesting involvement of HPV infection in the pathogenesis of SLE. We suggest that these findings may justify the development of new HPV vaccines containing viral peptides that bear no homology to the human proteome, in order to avoid possible adverse immune cross-reactivity.

  18. HLA-G genotype and HLA-G expression in systemic lupus erythematosus: HLA-G as a putative susceptibility gene in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Rizzo, R; Hviid, T V F; Govoni, M

    2008-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease mainly mediated by the deposit of immune complexes and defects in T lymphocytes and antigen-presenting cells along with a high production of T-helper 2 cytokines. A tolerance-inducible function of nonclassical class Ib human leukocyte...

  19. Defective B cell response to T-dependent immunization in lupus-prone mice

    Science.gov (United States)

    Niu, Haitao; Sobel, Eric S.; Morel, Laurence

    2009-01-01

    Lupus anti-nuclear Abs show the characteristics of Ag-driven T cell-dependent (TD) humoral responses. If autoAgs elicit the same response as exogenous Ags, lupus should enhance humoral responses to immunization. Blunted responses to various immunizations have, however, been reported in a significant portion of lupus patients. In this study, we show that lupus-prone B6.Sle1.Sle2.Sle3 (B6.TC) mice produce significantly less Ab in response to TD immunization than congenic controls, while producing significantly more total Ig. This blunted Ab response to TD Ag could be reconstituted with B6.TC B and CD4+ T cells. Multiple defects were found in the B6.TC response to NP-KLH as compared to total Ig, including a smaller percentage of B cells participating to the NP-response, a reduced entry into germinal centers, and highly defective production of NP-specific long-lived plasma cells in the bone marrow. B6.TC plasma cells expressed reduced levels of FcγRIIb, which suggests that reduced apoptosis in resident plasma cells prevents the establishment of newly-formed NP-specific plasma cells in bone marrow niches. Overall, these results show that lupus-prone mice responded differently to auto- and exogenous antigens and suggest that low FcγRIIb, hypergammaglobulinemia and high autoantibody production would be predictive of a poor response to immunization in lupus patients. PMID:18924209

  20. Understanding lupus nephritis: diagnosis, management, and treatment options

    Directory of Open Access Journals (Sweden)

    Mok CC

    2012-05-01

    Full Text Available Chi Chiu MokDepartment of Medicine, Tuen Mun Hospital and Center for Assessment and Treatment of Rheumatic Diseases, Pok Oi Hospital, Hong Kong, ChinaAbstract: Systemic lupus erythematosus (SLE predominantly affects women in their reproductive years. Renal disease (glomerulonephritis is one of the most frequent and serious manifestations of SLE. Of the various histological types of lupus glomerulonephritis, diffuse proliferative nephritis carries the worst prognosis. Combined with high-dose prednisone, mycophenolate mofetil (MMF has emerged as a first-line immunosuppressive treatment, although data regarding the efficacy of MMF on the long-term preservation of renal function are forthcoming. Cyclophosphamide is reserved for more severe forms of lupus nephritis, such as crescentic glomerulonephritis with rapidly deteriorating renal function, patients with significant renal function impairment at presentation, and refractory renal disease. Evidence for the calcineurin inhibitors in the treatment of lupus nephritis is weaker, and it concerns patients who are intolerant or recalcitrant to other agents. While further controlled trials are mandatory, B cell modulation therapies, such as rituximab, belimumab and epratuzumab are confined to refractory disease. Non-immunosuppressive measures, such as angiotensin-converting enzyme inhibitors, vigorous blood pressure control, prevention and treatment of hyperlipidemia and osteoporosis, are equally important.Keywords: lupus, nephritis, nephropathy, glomerulonephritis, treatment, therapy, women

  1. Protein-losing enteropathy in systemic lupus erythematosus: 12 years experience from a Chinese academic center.

    Directory of Open Access Journals (Sweden)

    Zhen Chen

    Full Text Available Protein-losing enteropathy (PLE is a complication in some systemic lupus erythematosus (SLE patients that is often misdiagnosed. With this study, we provide insight into clinical characteristics, laboratory characteristics, diagnostic tests, risk factors, treatment, and prognosis of the disease.A retrospective, case-control study was performed in 44 patients with SLE-related PLE (PLE group and 88 patients with active SLE (control group admitted to our care from January 2000-January 2012. Risk factors for SLE-related PLE were examined, and we analyzed the accuracy of single and combined laboratory characteristics in discriminating SLE-related PLE from active SLE. Serum albumin and C3 levels were measured as outcome during and after treatment with corticosteroids and immunosuppressive agents.The PLE group had lower mean serum albumin and 24-hour urine protein levels, higher mean total plasma cholesterol levels, and greater frequencies of anti-SSA and SSB seropositivity compared with the control group. Anti-SSA seropositivity, hypoalbuminemia, and hypercholesterolemia were independent risk factors for SLE-related PLE. The simultaneous presence of serum albumin (<22 g/l and 24-hour urine protein (<0.8 g/24 h had high specificity, positive predictive value, negative predictive value, and positive likelihood ratio, a low negative likelihood ratio and no significant reduction in sensitivity. High dosage of glucocorticosteroid combined with cyclophosphomide were mostly prescribed for SLE-related PLE.SLE-related PLE should be considered when an SLE patient presents with generalized edema, anti-SSA antibody seropositivity, hypercholesterolemia, severe hypoalbuminemia, and low 24-hour urine protein levels. Aggressive treatment for lupus might improve prognosis.

  2. 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.

  3. Interferon regulatory factor 5 gene polymorphism in Egyptian children with systemic lupus erythematosus.

    Science.gov (United States)

    Hammad, A; Mossad, Y M; Nasef, N; Eid, R

    2017-07-01

    Background Increased expression of interferon-inducible genes is implicated in the pathogenesis of systemic lupus erythematosus (SLE). Interferon regulatory factor 5 (IRF5) is one of the transcription factors regulating interferon and was proved to be implicated in the pathogenesis of SLE in different populations. Objectives The objective of this study was to investigate the correlation between polymorphisms of the IRF5 gene and SLE susceptibility in a cohort of Egyptian children and to investigate their association with clinico-pathological features, especially lupus nephritis. Subjects and methods Typing of interferon regulatory factor 5 rs10954213, rs2004640 and rs2280714 polymorphisms were done using polymerase chain reaction-restriction fragment length polymorphism for 100 children with SLE and 100 matched healthy controls. Results Children with SLE had more frequent T allele and TT genotype of rs2004640 ( P c  = 0.003 and 0.024, respectively) compared to controls. Patients with nephritis had more frequent T allele of rs2004640 compared to controls ( P c  = 0.003). However the allele and genotype frequencies of the three studied polymorphisms did not show any difference in patients with nephritis in comparison to those without nephritis. Haplotype GTA of rs10954213, rs2004640 and rs2280714, respectively, was more frequent in lupus patients in comparison to controls ( p = 0.01) while the haplotype GGG was more frequent in controls than lupus patients ( p = 0.011). Conclusion The rs2004640 T allele and TT genotype and GTA haplotype of rs rs10954213, rs2004640, and rs2280714, respectively, can be considered as risk factors for the development of SLE. The presence of the rs2004640 T allele increases the risk of nephritis development in Egyptian children with SLE.

  4. Oral candidiasis in systemic lupus erythematosus.

    Science.gov (United States)

    Fangtham, M; Magder, L S; Petri, M A

    2014-06-01

    We assessed the frequency of oral candidiasis and the association between demographic variables, disease-related variables, corticosteroid treatment, other treatments and the occurrence of oral candidiasis in the Hopkins Lupus Cohort. In this large prospective cohort study of 2258 patients with systemic lupus erythematosus (SLE), demographic and clinical associates of oral candidiasis were estimated by univariate, multivariate and within-person regression models. There were 53,548 cohort visits. Oral candidiasis was diagnosed at 675 visits (1.25%) in 325 (14%) of the patients. In the multivariate analyses, oral candidiasis was associated with African-American ethnicity, SELENA-SLEDAI disease activity, high white blood cell count, a history of bacterial infection, prednisone use and immunosuppressive use. The urine protein by urine dip stick was higher in SLE patients with oral candidiasis. Considering only patients who had candidiasis at some visits in a 'within-person' analysis, candidiasis was more frequent in visits with higher SELENA-SLEDAI disease activity, high white blood cell count, proteinuria by urine dip stick, a history of bacterial infection and prednisone use. The use of hydroxychloroquine was associated with a lower risk of oral candidiasis, but was not statistically significant (p = 0.50) in the within-person analysis models. This study identified multiple risk factors for oral candidiasis in SLE. Inspection of the oral cavity for signs of oral candidiasis is recommended especially in SLE patients with active disease, proteinuria, high white blood cell count, taking prednisone, immunosuppressive drugs or antibiotics. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  5. Prevalence of human herpesvirus 8 infection in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Sun Shipeng

    2011-05-01

    Full Text Available Abstract Background For decades, scientists have tried to understand the environmental factors involved in the development of systemic lupus erythematosus (SLE, in which viral infections was included. Previous studies have identified Epstein-Barr virus (EBV to incite SLE. Human herpesvirus 8 (HHV-8, another member of the gammaherpesvirus family, shares a lot in common with EBV. The characteristics of HHV-8 make it a well-suited candidate to trigger SLE. Results In the present study, serum samples from patients (n = 108 with diagnosed SLE and matched controls (n = 122 were collected, and the prevalence of HHV-8 was compared by a virus-specific nested PCR and a whole virus enzyme-linked immunoassay (EIA. There was significant difference in the prevalence of HHV-8 DNA between SLE patients and healthy controls (11 of 107 vs 1 of 122, p = 0.001; significant difference was also found in the detection of HHV-8 antibodies (19 of 107 vs 2 of 122, p We also detected the antibodies to Epstein-Barr virus viral capsid antigen (EBV-VCA and Epstein-Barr nuclear antigen-1 (EBNA-1. Both patients and controls showed high seroprevalence with no significant difference (106 of 107 vs 119 of 122, p = 0.625. Conclusion Our finding indicated that there might be an association between HHV-8 and the development of SLE.

  6. Acquired Von Willebrand’s Syndrome in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  8. SLE local martingales in logarithmic representations

    International Nuclear Information System (INIS)

    Kytölä, Kalle

    2009-01-01

    A space of local martingales of SLE-type growth processes forms a representation of Virasoro algebra, but apart from a few simplest cases, not much is known about this representation. The purpose of this paper is to exhibit examples of representations where L 0 is not diagonalizable—a phenomenon characteristic of logarithmic conformal field theory. Furthermore, we observe that the local martingales bear a close relation to the fusion product of the boundary changing fields. Our examples reproduce first of all many familiar logarithmic representations at certain rational values of the central charge. In particular we discuss the case of SLE κ=6 describing the exploration path in critical percolation and its relation to the question of operator content of the appropriate conformal field theory of zero central charge. In this case one encounters logarithms in a probabilistically transparent way, through conditioning on a crossing event. But we also observe that some quite natural SLE variants exhibit logarithmic behavior at all values of κ, thus at all central charges and not only at specific rational values

  9. Clinical significance of nailfold capillaroscopy in systemic lupus erythematosus: correlation with endothelial cell activation markers and disease activity.

    Science.gov (United States)

    Kuryliszyn-Moskal, A; Ciolkiewicz, M; Klimiuk, P A; Sierakowski, S

    2009-01-01

    To evaluate whether nailfold capillaroscopy (NC) changes are associated with the main serum endothelial cell activation markers and the disease activity of systemic lupus erythematosus (SLE). Serum levels of vascular endothelial growth factor (VEGF), endothelin-1 (ET-1), soluble E-selectin (sE-selectin), and soluble thrombomodulin (sTM) were determined by an enzyme-linked immunosorbent assay (ELISA) in 80 SLE patients and 33 healthy controls. Nailfold capillary abnormalities were seen in 74 out of 80 (92.5%) SLE patients. A normal capillaroscopic pattern or mild changes were found in 33 (41.25%) and moderate/severe abnormalities in 47 (58.75%) of all SLE patients. In SLE patients a capillaroscopic score >1 was more frequently associated with the presence of internal organ involvement (p 1 and controls. SLE patients with severe/moderate capillaroscopic abnormalities showed significantly higher VEGF serum levels than patients with mild changes (p < 0.001). Moreover, there was a significant positive correlation between the severity of capillaroscopic changes and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) (p < 0.005) as well as between capillaroscopic score and VEGF serum levels (p < 0.001). Our findings confirm the usefulness of NC as a non-invasive technique for the evaluation of microvascular involvement in SLE patients. A relationship between changes in NC, endothelial cell activation markers and clinical features of SLE suggest an important role for microvascular abnormalities in clinical manifestation of the disease.

  10. Echolalia as a novel manifestation of neuropsychiatric systemic lupus erythematosus.

    Science.gov (United States)

    Zapor, M; Murphy, F T; Enzenauer, R

    2001-01-01

    "That tongue of yours, by which I have been tricked, shall have its power curtailed and enjoy the briefest use of speech." With these words, Hera, of Greek mythology, deprived the nymph Echo of spontaneous speech, constraining her instead to merely repeating the words of others. Echolalia, which derives from the word "echo," is disordered speech in which an individual persistently repeats what is heard. Echolalia has been described in patients with a number of neuropsychiatric illnesses including autism and Tourette's syndrome. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a heterogeneous disease with protean manifestations that may occur in approximately 25% to 50% of patients with systemic lupus erythematosus (SLE). Although the most common manifestations include cognitive dysfunction (50%) and seizures (20%), NPSLE may also present as peripheral neuropathy (15%), psychosis (10%), or other central nervous system abnormalities. We report the case of a 57-year-old woman with SLE and echolalia.

  11. Dicty_cDB: SLE110 [Dicty_cDB

    Lifescience Database Archive (English)

    Full Text Available SL (Link to library) SLE110 (Link to dictyBase) - - - Contig-U16520-1 SLE110E (Link... to Original site) - - - - - - SLE110E 237 Show SLE110 Library SL (Link to library) Clone ID SLE110 (Link to dict...yBase) Atlas ID - NBRP ID - dictyBase ID - Link to Contig Contig-U16520-1 Original site URL http://dict...NLSEDVNLEEFVMSKDDLSGADIKAICTESGLLALRERRMRVTYXDFKKAKEKVLYR KTAGAPEGLYM*kkknqnq Translated Amino Acid sequence... (All Frames) Frame A: AKMNLSEDVNLEEFVMSKDDLSGADIKAICTESGLLALRERRMRVTYXDFKKAKEKVL

  12. Terbinafine-induced subacute cutaneous lupus erythematosus in two patients with systemic lupus erythematosus successfully treated with topical corticosteroids.

    Science.gov (United States)

    Kalińska-Bienias, Agnieszka; Kowalewski, Cezary; Woźniak, Katarzyna

    2013-08-01

    So far in the literature there have been reported only 5 patients with a recognized and well-documented history of systemic lupus erythematosus (SLE) who developed SCLE after terbinafine introduction. Here we report two women suffering from SLE who developed SCLE after initiation of oral terbinafine for onychomycosis. Skin lesions in both of them were extensive, located on the trunk, and upper and lower extremities. No exacerbation of SLE symptoms was observed at that time. Despite severe skin lesions, patients revealed good response to topical corticosteroids within a few weeks. The systemic review of the literature and our experience on terbinafine-induced SCLE developing in patients with SLE allowed to create a description for this special subset: a) terbinafine-induced SCLE usually develop in 1-8 weeks after terbinafine introduction, b) skin lesions are usually severe, disseminated including lower extremities, c) patients present Ro/SS-A La/SS-B antibodies, but anti-histone antibodies are rarely observed, d) exacerbation of SLE symptoms is rather not observed, e) eruptions clear within 2-8 weeks, f) withdrawal of terbinafine and topical corticosteroids should be considered as a first-line therapy in these cases, g) terbinafine should be carefully used in patients suffering from SLE.

  13. Interleukin-6 promotes systemic lupus erythematosus progression with Treg suppression approach in a murine systemic lupus erythematosus model.

    Science.gov (United States)

    Mao, Xiaoli; Wu, Yunyun; Diao, Huitian; Hao, Jianlei; Tian, Gaofei; Jia, Zhenghu; Li, Zheng; Xiong, Sidong; Wu, Zhenzhou; Wang, Puyue; Zhao, Liqing; Yin, Zhinan

    2014-11-01

    Our aim is to reveal the role of interleukin 6 (IL-6) in the pathogenesis of systemic lupus erythematosus (SLE) in a murine model of SLE. Normal female C57BL/6 mice were immunized with syngeneic-activated lymphocyte-derived DNA (ALD-DNA) to induce SLE. Non-immunized mice were used as control. SLE-associated markers, including anti-double-stranded DNA (anti-dsDNA) Abs, urine protein, and kidney histopathology, were assayed to ensure the induction of the disease. Compared with control mice, ALD-DNA immunized mice exhibited high levels of anti-dsDNA Abs, IL-6 expression in vivo and in vitro. We also found that IL-6 knockout (IL-6KO) mice were resistant to ALD-DNA-induced SLE. The activation of CD4(+) T cells in immunized IL-6KO mice was lower than in immunized wild-type (Wt) mice. Intracellular cytokine staining showed that Foxp3 expression in immunized IL-6KO mice was higher than in immunized Wt mice, which might be associated with the disease severity. We further discovered that ALD-DNA-stimulated dendritic cells supernatants could result in higher IL-6 and TNF-α expression and could suppress Foxp3 expression. In addition, blocking IL-6 could up-regulate Foxp3 expression. Therefore, our findings show that IL-6 promotes the progression of SLE via suppressing Treg differentiation.

  14. Variation in STAT4 is associated with systemic lupus erythematosus in Chinese Northern Han population.

    Science.gov (United States)

    Su, Yin; Zhao, Yi; Liu, Xu; Guo, Jian-Ping; Jiang, Quan; Liu, Xiang-Yuan; Zhang, Feng-Chun; Zheng, Yi; Li, Xiao-Xia; Song, Hui; Huang, Ci-Bo; Huang, Yan-Hong; Wang, Tian; Pan, Si-Si; Li, Chun; Liu, Xia; Zhu, Lei; Zhang, Chun-Fang; Li, Zhan-Guo

    2010-11-01

    Recent studies have identified signal transducer and activator of transcription 4 (STAT4) as a susceptibility gene for systemic lupus erythematosus (SLE) in different populations. In order to examine whether the allele distribution of the single nucleotide polymorphism (SNP) in gene STAT4 rs7574865 in patients with SLE is different from those of healthy controls in Chinese Northern Han population, we investigated whether the variants of STAT4 rs7574865 were associated with any specific clinical features of SLE. We genotyped SNPs in STAT4 rs7574865 in 252 patients with SLE and 497 healthy controls. All subjects were from the Northern part of Chinese Han population. The genotypes in rs7574865 were determined by polymerase chain reaction (PCR) and consequence direct sequencing of PCR products in the DNA samples. There was a significant difference in distribution of the SNPs in rs7574865 between the SLE patients and healthy controls. Compared with healthy controls, there was a significant correlation between TT genotypes in rs7574865 and the risk of SLE when GG genotype was used as a reference genotype after adjusting for gender and age. The frequency of T allele in the SLE patients was strongly significantly higher than that of healthy controls. Furthermore, there was a significant difference in the distribution of SNP in rs7574865 between male and female SLE patients, when compared with healthy controls. The frequency of T allele in rs7574865 in male patients was significantly higher than that of male healthy controls or female patients. There was no significant correlation between the frequencies of T allele in STAT4 rs7574865 and the clinical features of SLE. The SNP rs7574865 in STAT4 is strongly associated with risk of SLE in the Chinese Northern Han population. The TT genotype and T allele in STAT4 rs7574869 are susceptibility factors for SLE, especially for male SLE patients.

  15. Lupus anticoagulant, disease activity and low complement in the first trimester are predictive of pregnancy loss.

    Science.gov (United States)

    Mankee, Anil; Petri, Michelle; Magder, Laurence S

    2015-01-01

    Multiple factors, including proteinuria, antiphospholipid syndrome, thrombocytopenia and hypertension, are predictive of pregnancy loss in systemic lupus erythematosus (SLE). In the PROMISSE study of predictors of pregnancy loss, only a battery of lupus anticoagulant tests was predictive of a composite of adverse pregnancy outcomes. We examined the predictive value of one baseline lupus anticoagulant test (dilute Russell viper venom time) with pregnancy loss in women with SLE. From the Hopkins Lupus Cohort, there were 202 pregnancies from 175 different women after excluding twin pregnancies and pregnancies for which we did not have a first trimester assessment of lupus anticoagulant. We determined the percentage of women who had a pregnancy loss in groups defined by potential risk factors. The lupus anticoagulant was determined by dilute Russell viper venom time with appropriate mixing and confirmatory testing. Generalised estimating equations were used to calculate p values, accounting for repeated pregnancies in the same woman. The age at pregnancy was 40 (3%). 55% were Caucasian and 34% African-American. Among those with lupus anticoagulant during the first trimester, 6/16 (38%) experienced a pregnancy loss compared with only 16/186 (9%) of other pregnancies (p=0.003). In addition, those with low complement or higher disease activity had a higher rate of pregnancy loss than those without (p=0.049 and 0.005, respectively). In contrast, there was no association between elevated anticardiolipin in the first trimester and pregnancy loss. The strongest predictor of pregnancy loss in SLE in the first trimester is the lupus anticoagulant. In addition, moderate disease activity by the physician global assessment and low complement measured in the first trimester were predictive of pregnancy loss. These data suggest that treatment of the lupus anticoagulant could be considered, even in the absence of history of pregnancy loss.

  16. Effects of obesity on health-related quality of life in juvenile-onset systemic lupus erythematosus.

    Science.gov (United States)

    Mina, R; Klein-Gitelman, M S; Nelson, S; Eberhard, B A; Higgins, G; Singer, N G; Onel, K; Tucker, L; O'Neil, K M; Punaro, M; Levy, D M; Haines, K; Ying, J; Brunner, H I

    2015-02-01

    This study evaluated the effects of obesity on health-related quality of life (HRQOL) measures in juvenile-onset systemic lupus erythematosus (jSLE). Obesity was defined as a body mass index (BMI) ≥ 95 th percentile according to the Sex-specific Center for Disease Control BMI-For-Age Charts and determined in a multicenter cohort of jSLE patients. In this secondary analysis, the domain and summary scores of the Pediatric Quality of Life (PedsQL) Inventory and the Child Health Questionnaire (CHQ) of obese jSLE patients were compared to those of non-obese jSLE patients as well as historical obese and non-obese healthy controls. Mixed-effects modeling was performed to evaluate the relationship between obesity and HRQOL measures. Among the 202 jSLE patients, 25% (n = 51) were obese. Obesity had a significant negative impact on HRQOL in jSLE, even after adjusting for differences in current corticosteroid use, disease activity, disease damage, gender and race between groups. Obese jSLE patients had lower physical functioning compared to non-obese jSLE patients, and to non-obese and obese healthy controls. Compared to their non-obese counterparts, obese jSLE patients also had worse school functioning, more pain, worse social functioning and emotional functioning. Parents of obese jSLE patients worry more. The CHQ scores for obese jSLE patients were also worse compared to non-obese jSLE patients in several other domains. Our study demonstrates the detrimental effects of obesity on patient-reported outcomes in jSLE. This supports the importance of weight management for the therapeutic plan of jSLE. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  17. 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...

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

    International Nuclear Information System (INIS)

    Nagaoka, Shohei; Tani, Kenji; Ishigatsubo, Yoshiaki

    1988-01-01

    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. (author)

  19. Autoantibody Profiling in Lupus Patients using Synthetic Nucleic Acids

    DEFF Research Database (Denmark)

    Klecka, Martin; Thybo, Christina; Macaubas, Claudia

    2018-01-01

    specificity and reproducibility. Applying the ELISA tests to serological studies of pediatric and adult SLE, we identified novel clinical correlations. We also observed preferential recognition of a specific synthetic antigen by antibodies in SLE sera. We determined the probable basis for this finding using...... computational analyses, providing valuable structural information for future development of DNA antigens. Synthetic nucleic acid molecules offer the opportunity to standardize assays and to dissect antibody-antigen interactions.......Autoantibodies to nuclear components of cells (antinuclear antibodies, ANA), including DNA (a-DNA), are widely used in the diagnosis and subtyping of certain autoimmune diseases, including systemic lupus erythematosus (SLE). Despite clinical use over decades, precise, reproducible measurement of a...

  20. 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.

  1. 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.

  2. 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.

  3. Association between systemic lupus erythematosus and multiple sclerosis: lupoid sclerosis

    International Nuclear Information System (INIS)

    Medina, Yimy F; Martinez, Jose B; Fernandez, Andres R; Quintana, Gerardo; Restrepo, Jose Felix; Rondon, Federico; Gamarra, Antonio Iglesias

    2010-01-01

    Multiple sclerosis (MS) and Systemic Lupus Erythematosus (SLE) with/without antiphospholipid syndrome are autoimmune illnesses. It has been described in many occasions the association of these two illnesses and the clinical picture of MS with characteristics of laboratory of SLE. When they affect to the central nervous system they can make it in a defined form for each illness or they can also make it in interposed or combined form of the two illnesses what has been called lupoid sclerosis; making that in some cases difficult the differentiation of the two illnesses and therefore to address the treatment. We present four cases of lupoid sclerosis, discuss the clinical and laboratory characteristics of this entity and we make a differentiation of the multiple sclerosis with the neurological affectation of SLE especially for images and laboratory results.

  4. Regulatory T-Cell-Associated Cytokines in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Akiko Okamoto

    2011-01-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease characterized by autoantibody production, complement activation, and immune complex deposition, resulting in tissue and organ damage. An understanding of the mechanisms responsible for homeostatic control of inflammation, which involve both innate and adoptive immune responses, will enable the development of novel therapies for SLE. Regulatory T cells (Treg play critical roles in the induction of peripheral tolerance to self- and foreign antigens. Naturally occurring CD4+CD25+ Treg, which characteristically express the transcription factor forkhead box protein P3 (Foxp3, have been intensively studied because their deficiency abrogates self-tolerance and causes autoimmune disease. Moreover, regulatory cytokines such as interleukin-10 (IL-10 also play a central role in controlling inflammatory processes. This paper focuses on Tregs and Treg-associated cytokines which might regulate the pathogenesis of SLE and, hence, have clinical applications.

  5. 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.

  6. 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

  7. 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.

  8. Differential genetic associations for systemic lupus erythematosus based on anti-dsDNA autoantibody production.

    Directory of Open Access Journals (Sweden)

    Sharon A Chung

    2011-03-01

    Full Text Available Systemic lupus erythematosus (SLE is a clinically heterogeneous, systemic autoimmune disease characterized by autoantibody formation. Previously published genome-wide association studies (GWAS have investigated SLE as a single phenotype. Therefore, we conducted a GWAS to identify genetic factors associated with anti-dsDNA autoantibody production, a SLE-related autoantibody with diagnostic and clinical importance. Using two independent datasets, over 400,000 single nucleotide polymorphisms (SNPs were studied in a total of 1,717 SLE cases and 4,813 healthy controls. Anti-dsDNA autoantibody positive (anti-dsDNA +, n = 811 and anti-dsDNA autoantibody negative (anti-dsDNA -, n = 906 SLE cases were compared to healthy controls and to each other to identify SNPs associated specifically with these SLE subtypes. SNPs in the previously identified SLE susceptibility loci STAT4, IRF5, ITGAM, and the major histocompatibility complex were strongly associated with anti-dsDNA + SLE. Far fewer and weaker associations were observed for anti-dsDNA - SLE. For example, rs7574865 in STAT4 had an OR for anti-dsDNA + SLE of 1.77 (95% CI 1.57-1.99, p = 2.0E-20 compared to an OR for anti-dsDNA - SLE of 1.26 (95% CI 1.12-1.41, p = 2.4E-04, with p(heterogeneity<0.0005. SNPs in the SLE susceptibility loci BANK1, KIAA1542, and UBE2L3 showed evidence of association with anti-dsDNA + SLE and were not associated with anti-dsDNA - SLE. In conclusion, we identified differential genetic associations with SLE based on anti-dsDNA autoantibody production. Many previously identified SLE susceptibility loci may confer disease risk through their role in autoantibody production and be more accurately described as autoantibody propensity loci. Lack of strong SNP associations may suggest that other types of genetic variation or non-genetic factors such as environmental exposures have a greater impact on susceptibility to anti-dsDNA - SLE.

  9. Association of the interferon signature metric with serological disease manifestations but not global activity scores in multiple cohorts of patients with SLE

    Science.gov (United States)

    Kennedy, William P; Maciuca, Romeo; Wolslegel, Kristen; Tew, Wei; Abbas, Alexander R; Chaivorapol, Christina; Morimoto, Alyssa; McBride, Jacqueline M; Brunetta, Paul; Richardson, Bruce C; Davis, John C; Behrens, Timothy W; Townsend, Michael J

    2015-01-01

    Objectives The interferon (IFN) signature (IS) in patients with systemic lupus erythematosus (SLE) includes over 100 genes induced by type I IFN pathway activation. We developed a method to quantify the IS using three genes—the IS metric (ISM)—and characterised the clinical characteristics of patients with SLE with different ISM status from multiple clinical trials. Methods Blood microarray expression data from a training cohort of patients with SLE confirmed the presence of the IS and identified surrogate genes. We assayed these genes in a quantitative PCR (qPCR) assay, yielding an ISM from the IS. The association of ISM status with clinical disease characteristics was assessed in patients with extrarenal lupus and lupus nephritis from four clinical trials. Results Three genes, HERC5, EPSTI and CMPK2, correlated well with the IS (p>0.96), and composed the ISM qPCR assay. Using the 95th centile for healthy control data, patients with SLE from different studies were classified into two ISM subsets—ISM-Low and ISM-High—that are longitudinally stable over 36 weeks. Significant associations were identified between ISM-High status and higher titres of anti-dsDNA antibodies, presence of anti extractable nuclear antigen autoantibodies, elevated serum B cell activating factor of the tumour necrosis factor family (BAFF) levels, and hypocomplementaemia. However, measures of overall clinical disease activity were similar for ISM-High and ISM-Low groups. Conclusions The ISM is an IS biomarker that divides patients with SLE into two subpopulations—ISM-High and ISM-Low—with differing serological manifestations. The ISM does not distinguish between high and low disease activity, but may have utility in identifying patients more likely to respond to treatment(s) targeting IFN-α. Clinicaltrials.gov registration number NCT00962832. PMID:25861459

  10. Quality-of-life measurements in multiethnic patients with systemic lupus erythematosus: cross-cultural issues.

    Science.gov (United States)

    Toloza, Sergio M A; Jolly, Meenakshi; Alarcón, Graciela S

    2010-08-01

    Although the survival rate for systemic lupus erythematosus (SLE) has improved dramatically during the past 50 years, the quality of life of patients afflicted with this disease remains poor. Currently existent measures of disease activity and damage in SLE do not capture the patient's perspective and health-related quality of life (HRQoL). Most studies in SLE pertaining to HRQoL are from developed Western societies, with only a few from others. These studies have been conducted predominantly in women and using the Medical Outcomes Survey Short Form 36, a generic HRQoL instrument that has been shown not to be sensitive to change in lupus. Existent lupus-specific HRQoL measures have not yet been used in SLE clinical trials. New HRQoL research tools are currently undergoing validation in different countries, languages, and cultural settings, which may help dissect the underlying role of socioeconomic status and specific disease-related features that impact SLE-related quality of life.

  11. Children & Teens (with Lupus)

    Science.gov (United States)

    ... nine. blog Lupus at school: A guide for parents and kids Advice for communicating with your child's school about their lupus and ... teens on adjusting to life with lupus For teens, living with lupus can require some major ... in school Advice from parents and education experts on 504 and Individualized Education ...

  12. Typing TREX1 gene in patients with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    M. Fredi

    2015-06-01

    Full Text Available An impaired expression of interferon-α regulated genes has been reported in patients with either systemic lupus erythematosus (SLE or Aicardi-Goutières syndrome (AGS, a rare monogenic encephalopathy with onset in infancy. One of mutations causing AGS is located in the TREX1 gene on chromosome 3. Heterozygous mutations in TREX1 were reported in SLE patients. TREX1 is a DNA exonuclease with specificity for ssDNA. An impairment of its activity may result in the accumulation of nucleid acid. A recent study described a significant association between a haplotype including several common single nucleotide polymorphisms (SNPs of TREX1 and neurological manifestations in European SLE patients. Fifty-one SLE patients were screened for TREX1 gene, and the corresponding data were collected from clinical charts. A novel heterozygous variant (p.Asp130Asn was identified in one patient and in none of 150 controls. A missense variation was located in one of the three active sites of the gene and was classified as probably damaging. Variations of SNP rs11797 were detected in 33 SLE patients and a variation of rs3135944 in one. A significantly higher rate of the minor allele (T nucleotide of SNP rs11797 was found in SLE patients with neuropsychiatric manifestations [12/16 (75% vs 28/86 (32.5% O=0.002, odds ratio=6.42 95% confidence interval (1.7-26.2]. Only 1 out of 8 patients (12.5% with neuropsychiatric SLE carried the wild-type form in homozygosity. Although we analyzed a small number of patients, we found a novel variation of TREX1, which may be pathogenic. The polymorphism of rs11797 was more frequent in SLE patients with neurological manifestations.

  13. Oxidative stress and fatigue in systemic lupus erythematosus.

    Science.gov (United States)

    Segal, B M; Thomas, W; Zhu, X; Diebes, A; McElvain, G; Baechler, E; Gross, M

    2012-08-01

    The objective of this study is to investigate the relationship of oxidative stress to fatigue in systemic lupus erythematosus (SLE). Patients with a confirmed diagnosis of SLE by ACR criteria and healthy controls completed validated questionnaires to assess depression and fatigue. Fatigue was measured with the Fatigue Severity Scale (FSS) and the Profile of Fatigue (Prof-F). Visual analogue scales (VAS) were also used to assess fatigue and pain. Depression was measured with the Center for Epidemiologic Studies Depression Scale (CES-D). Plasma F(2)-isoprostane was measured with gas chromatography/mass spectroscopy to assess oxidative stress. Evaluation included medical record review, physical exam and calculation of body mass index (BMI), disease activity (SLEDAI) and damage (SLICC) in the SLE patients. Seventy-one SLE patients with low disease activity (mean SLEDAI = 1.62 standard error (SE) 0.37, range 0-8) were compared to 51 controls. Fatigue-limiting physical activity (defined as FSS ≥ 4) was present in 56% of patients and 12% of controls. F(2)-isoprostane was higher in SLE patients with fatigue compared to not-fatigued SLE subjects (p = .0076) who were otherwise similar in ethnicity, disease activity and cardiovascular risk factors. Plasma F(2)-isoprostane was strongly correlated with FSS and Profile of Somatic Fatigue (Prof-S) (p fatigue (p = .005), CES-D (p = .008) and with BMI (p = .0001.) In a multivariate model, F(2)-isoprostane was a significant predictor of FSS after adjustment for age, BMI, pain and depression (p = .0002). Fatigue in SLE patients with low disease activity is associated with increased F(2)-isoprostane. F2-isoprostane could provide a useful biomarker to explore mitochondrial function and the regulation of oxidative pathways in patients with SLE in whom fatigue is a debilitating symptom.

  14. 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.)

  15. Frequency of dental caries in active and inactive systemic lupus erythematous patients: salivary and bacterial factors.

    Science.gov (United States)

    Loyola Rodriguez, J P; Galvan Torres, L J; Martinez Martinez, R E; Abud Mendoza, C; Medina Solis, C E; Ramos Coronel, S; Garcia Cortes, J O; Domínguez Pérez, R A

    2016-10-01

    The objective of this study was to determine dental caries frequency and to analyze salivary and bacterial factors associated with active and inactive systemic lupus erythematous (SLE) patients. Also, a proposal to identify dental caries by a surface, teeth, and the patient was developed. A cross-sectional, blinded study that included 60 SLE patients divided into two groups of 30 subjects each, according to the Activity Index for Diagnosis of Systemic Lupus Erythematous (SLEDAI). The decayed, missing, and filled teeth (DMFT) index and Integrative Dental Caries Index (IDCI) were used for analyzing dental caries. The saliva variables recorded were: flow, pH, and buffer capacity. The DNA copies of Streptococcus mutans and Streptococcus sobrinus were estimated by real-time PCR. The caries frequency was 85% for SLE subjects (73.3% for inactive systemic lupus erythematous (ISLE) and 100% for active systemic lupus erythematous (ASLE)); DMFT for the SLE group was 12.6 ± 5.7 and the IDCI was (9.8 ± 5.9). The ASLE group showed a salivary flow of 0.65 compared with 0.97 ml/1 min from the ISLE group; all variables mentioned above showed a statistical difference (p dental caries in epidemiological studies. © The Author(s) 2016.

  16. 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.

  17. Libman–Sacks endocarditis, and other echocardiographic findings in systemic lupus erythematosus: Case report

    OpenAIRE

    Hamza, Mohamed Atef; Allam, Lamyaa

    2012-01-01

    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).

  18. 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...

  19. Vitamin D levels in women with systemic lupus erythematosus and fibromyalgia

    NARCIS (Netherlands)

    Huisman, A. M.; White, K. P.; Algra, A.; Harth, M.; Vieth, R.; Jacobs, J. W.; Bijlsma, J. W.; Bell, D. A.

    2001-01-01

    Many patients with systemic lupus erythematosus (SLE) and fibromyalgia (FM) may spend less time exposed to the sun than healthy individuals and thus might have low vitamin D levels. It is known that hydroxychloroquine (HCQ) inhibits conversion of 25(OH)- to 1,25(OH)2-vitamin D both in vitro and in

  20. Lupus-related single nucleotide polymorphisms and risk of diffuse large B-cell lymphoma

    NARCIS (Netherlands)

    Bernatsky, Sasha; Velásquez García, Héctor A; Spinelli, John; Gaffney, Patrick; Smedby, Karin E; Ramsey-Goldman, Rosalind; Wang, Sophia S.; Adami, Hans-Olov; Albanes, Demetrius; Angelucci, Emanuele; Ansell, Stephen M.; Asmann, Yan W.; Becker, Nikolaus; Benavente, Yolanda; Berndt, Sonja I.; Bertrand, Kimberly A.; Birmann, Brenda M.; Boeing, Heiner; Boffetta, Paolo; Bracci, Paige M.; Brennan, Paul; Brooks-Wilson, Angela R.; Cerhan, James R.; Chanock, Stephen J.; Clavel, Jacqueline; Conde, Lucia; Cotenbader, Karen H; Cox, David G; Cozen, Wendy; Crouch, Simon; De Roos, Anneclaire J.; De Sanjose, Silvia; Di Lollo, Simonetta; Diver, W. Ryan; Dogan, Ahmet; Foretova, Lenka; Ghesquières, Hervé; Giles, Graham G.; Glimelius, Bengt; Habermann, Thomas M.; Haioun, Corinne; Hartge, Patricia; Hjalgrim, Henrik; Holford, Theodore R.; Holly, Elizabeth A.; Jackson, Rebecca D.; Kaaks, Rudolph; Kane, Eleanor; Kelly, Rachel S.; Klein, Robert J.; Kraft, Peter; Kricker, Anne; Lan, Qing; Lawrence, Charles; Liebow, Mark; Lightfoot, Tracy; Link, Brian K.; Maynadie, Marc; McKay, James; Melbye, Mads; Molina, Thierry Jo; Monnereau, Alain; Morton, Lindsay M.; Nieters, Alexandra; North, Kari E.; Novak, Anne J.; Offit, Kenneth; Purdue, Mark P.; Rais, Marco; Riby, Jacques; Roman, Eve; Rothman, Nathaniel; Salles, Gilles; Severi, Gianluca; Severson, Richard K.; Skibola, Christine F.; Slager, Susan L.; Smith, Alex; Smith, Martyn T.; Southey, Melissa C.; Staines, Anthony; Teras, Lauren R.; Thompson, Carrie A.; Tilly, Hervé; Tinker, Lesley F.; Tjonneland, Anne; Turner, Jenny; Vajdic, Claire M.; Vermeulen, Roel C H; Vijai, Joseph; Vineis, Paolo; Virtamo, Jarmo; Wang, Zhaoming; Weinstein, Stephanie; Witzig, Thomas E.; Zelenetz, Andrew; Zeleniuch-Jacquotte, Anne; Zhang, Yawei; Zheng, Tongzhang; Zucca, Mariagrazia; Clarke, Ann E

    2017-01-01

    Objective: Determinants of the increased risk of diffuse large B-cell lymphoma (DLBCL) in SLE are unclear. Using data from a recent lymphoma genome-wide association study (GWAS), we assessed whether certain lupus-related single nucleotide polymorphisms (SNPs) were also associated with DLBCL.

  1. Circulating microRNA expression profiles associated with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Carlsen, Anting Liu; Schetter, Aaron J; Nielsen, Christoffer

    2013-01-01

    OBJECTIVE: To evaluate the specificity of expression patterns of cell-free, circulating microRNAs in systemic lupus erythematosus (SLE). METHODS: Total RNA was purified from plasma and 45 different specific mature microRNAs were determined using quantitative reverse transcription polymerase chain...

  2. 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...

  3. 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...

  4. 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...

  5. Ocular manifestations of systematic lupus erythematosus in children

    International Nuclear Information System (INIS)

    Al-Mayouf, Sulaiman M.; Al-Hemidan, Amal I.

    2003-01-01

    To determine the prevalence and spectrum of ocular manifestations in children with systematic lupus erythematosus (SLE) and to examine the correlation of the ocular manifestations with disease activity , other organ involvement and the presence of circulating of autoantibodies. This study was performed at King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia from June 2002 to November 2002. It included detailed eye examination, measuring circulating autoantibodies (antinuclear,anti phospholipid antibodies) and circulation of SLE disease activity index (SLEDAI). 52 consecutive children (45 females) with SLE completed the evaluation .The mean age of the patients was 11.3 years and the mean SLEDAI was 9.5 Thirty patients (57.7%) had the disease for more than 1 year. 18 patients(34.6%) had ocular manifestations.7 patients had abnormal . Schirmer's test. 5 patients had ratinal vascular lesions. 1 patient had bilateral iridocyclitis. 3 patients had unilateral optic neuropathy and 11 patients had visual field defects.Fisher extract test revealed positive correlation between optic neuropathy and central nervous system(CNS) involvement. There was no correlation among other variables; probably due to sample size. Ocular manifestations including sight threatening complications are not rare in children with SLE.Optic neuropathy had a strong prediction for CNS lupus. (author)

  6. Expression patterns of signaling lymphocytic activation molecule family members in peripheral blood mononuclear cell subsets in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Karampetsou, Maria P; Comte, Denis; Kis-Toth, Katalin; Kyttaris, Vasileios C; Tsokos, George C

    2017-01-01

    Genome-wide linkage analysis studies (GWAS) studies in systemic lupus erythematosus (SLE) identified the 1q23 region on human chromosome 1, containing the Signaling Lymphocytic Activation Molecule Family (SLAMF) cluster of genes, as a lupus susceptibility locus. The SLAMF molecules (SLAMF1-7) are immunoregulatory receptors expressed predominantly on hematopoietic cells. Activation of cells of the adaptive immune system is aberrant in SLE and dysregulated expression of certain SLAMF molecules has been reported. We examined the expression of SLAMF1-7 on peripheral blood T cells, B cells, monocytes, and their respective differentiated subsets, in patients with SLE and healthy controls in a systematic manner. SLAMF1 levels were increased on both T cell and B cells and their differentiated subpopulations in patients with SLE. SLAMF2 was increased on SLE CD4+ and CD8+ T cells. The frequency of SLAMF4+ and SLAMF7+ central memory and effector memory CD8+ T cells was reduced in SLE patients. Naïve CD4+ and CD8+ SLE T cells showed a slight increase in SLAMF3 levels. No differences were seen in the expression of SLAMF5 and SLAMF6 among SLE patients and healthy controls. Overall, the expression of various SLAMF receptors is dysregulated in SLE and may contribute to the immunopathogenesis of the disease.

  7. Differences in disease features between childhood-onset and adult-onset systemic lupus erythematosus patients presenting with acute abdominal pain.

    Science.gov (United States)

    Tu, Yu-Ling; Yeh, Kuo-Wei; Chen, Li-Chen; Yao, Tsung-Chieh; Ou, Liang-Shiou; Lee, Wen-I; Huang, Jing-Long

    2011-04-01

    Abdominal pain in systemic lupus erythematosus (SLE) patients has rarely been analyzed in pediatric populations. We planned to investigate the potential differences between childhood-onset and adult-onset SLE patients who were hospitalized because of acute abdominal pain. A retrospective study including 23 childhood-onset SLE patients with 38 admissions and 88 adult-onset SLE patients with 108 admissions from 1999 to 2008 were conducted in our hospital. All of them had the chief complaint of diffuse abdominal pain. The etiologies of acute abdominal pain in adult-onset SLE patients were more diverse than childhood-onset SLE patients. The most common cause of acute abdominal pain in SLE patients was lupus mesenteric vasculitis (LMV) (18.5%), followed by acute gastroenteritis (14.4%), pancreatitis (10.3%), appendicitis (7.5%), and cholecystitis (6.2%). Compared with adults, children were admitted more often due to LMV (31.6% versus 13.9%; P = 0.016), had more frequently recurrent episodes (39.1% versus 14.8%; P = 0.009), and were more often treated with immunosuppressive agents (31.6% versus 7.4%; P abdominal pain should be considered in SLE patients. LMV is the most common cause of acute abdomen in childhood-onset SLE patients with low mortality and morbidity provided by prompt diagnosis and timely administration of high-dose intravenous corticosteroids after excluding real surgical abdomen. Crown Copyright © 2011. Published by Elsevier Inc. All rights reserved.

  8. CLINICAL SIGNIFICANCE OF ANTIPHOSPHOLIPID ANTIBODIES AND GENE MUTATIONS IN HEMOSTASIS OF CHILDREN WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND JUVENILE DERMATOMYOSITIS

    Directory of Open Access Journals (Sweden)

    O.A. Solntseva

    2006-01-01

    Full Text Available Thrombophilia in children with diffuse connective tissue disorders as systemic lupus erythematosus (SLE and juvenile dermatomyositis (JDM could arise from various causes including peripherial blood circulation of antiphospholipid antibodies (APH and genetic mutations in the system of hemostasis. Thrombosis is a serious and prognostically unfavorable complication that has negative impact on the underlying disease course. The study included 96 children, 65 of them had diagnosed SLE and the other 31 had JDM. The Elisa method was used to detect antiphospholipid antibodies, coagulation method was used to detect lupus anticoagulant (LAC and antibodies to cardiolipins (anticl, ?2:glycoprotein 1 (anti ? 2 gp 1 and prothrombin (APT. The PCR method (DNA diagnostics was used to detect DNA mutations as factor resistance to of activated protein c (Leiden 5,10 methylen tetrahydrofolate reductase (MTHFR gene polymorphism. The incidence of APL antibodies was registered in 61.5% patients with SLE and in 32.2% of patients with JDM. Ac ligg, anti ?2 gp 1 Igg were clinically significant in thrombotic events in patients with SLE and JDM, and so was LAC in patients with SLE. The prevalence of the hemostasis system mutations is concordant with reported data. Conclusion thrombophilia is frequently associated with APH antibodies or combination of APH antibodies with genetic abnormalities. Sole genetic mutations are salient in patients with JDM.Key words: thrombophilia, systemic lupus erythematosus, juvenile dermatomyositis, antiphospholipid antibodies, lupus anticoagulant, leiden, prothrombin, methylentet rahydrofolate reductase.

  9. White matter lesions and brain atrophy in systemic lupus erythematosus patients: correlation to cognitive dysfunction in a cohort of systemic lupus erythematosus patients using different definition models for neuropsychiatric systemic lupus erythematosus.

    Science.gov (United States)

    Cannerfelt, B; Nystedt, J; Jönsen, A; Lätt, J; van Westen, D; Lilja, A; Bengtsson, A; Nilsson, P; Mårtensson, J; Sundgren, P C

    2018-06-01

    Aim The aim of this study was to evaluate the extent of white matter lesions, atrophy of the hippocampus and corpus callosum, and their correlation with cognitive dysfunction (CD), in patients diagnosed with systemic lupus erythematosus (SLE). Methods Seventy SLE patients and 25 healthy individuals (HIs) were included in the study. To evaluate the different SLE and neuropsychiatric SLE (NPSLE) definition schemes, patients were grouped both according to the American College of Rheumatology (ACR) definition, as well as the more stringent ACR-Systemic Lupus International Collaborating Clinics definition. Patients and HIs underwent a 3 Tesla brain MRI and a standardized neuropsychological test. MRI data were evaluated for number and volume of white matter lesions and atrophy of the hippocampus and corpus callosum. Differences between groups and subgroups were evaluated for significance. Number and volume of white matter lesions and atrophy of the hippocampus and corpus callosum were correlated to cognitive dysfunction. Results The total volume of white matter lesions was significantly larger in SLE patients compared to HIs ( p = 0.004). However, no significant differences were seen between the different SLE subgroups. Atrophy of the bilateral hippocampus was significantly more pronounced in patients with NPSLE compared to those with non-NPSLE (right: p = 0.010; left p = 0.023). Significant negative correlations between cognitive test scores on verbal memory and number and volume of white matter lesions were present. Conclusion SLE patients have a significantly larger volume of white matter lesions on MRI compared to HIs and the degree of white matter lesion volume correlates to cognitive dysfunction, specifically to verbal memory. No significant differences in the number or volume of white matter lesions were identified between subgroups of SLE patients regardless of the definition model used.

  10. Hemorrhagic Tamponade as Initial Manifestation of Systemic Lupus with Subsequent Refractory and Progressive Lupus Myocarditis Resulting in Cardiomyopathy and Mitral Regurgitation

    Directory of Open Access Journals (Sweden)

    Nicole Marijanovich

    2018-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a heterogeneous autoimmune disease with a wide range of clinical and serological manifestations. Cardiac disease among patients with SLE is common and can involve the pericardium, myocardium, valves, conduction system, and coronary arteries. We are reporting a case of SLE in a young woman that is unique is unique in that initial symptoms consisted of pericarditis and hemorrhagic tamponade which remained progressive and resistant to aggressive immunosuppressive treatment and led to severe cardiomyopathy (ejection fraction of 25% and severe (+4 mitral regurgitation. Her immunosuppressive treatment included hydroxychloroquine, high-dose steroids, intravenous immunoglobulins, azathioprine, and mycophenolate mofetil. Her disease progression was felt to be due to underlying uncontrolled SLE because the complement levels remained persistently low throughout the entire course and PET Myocardial Perfusion and Viability study showed stable persistent active inflammation. Eventually, she was treated with cyclophosphamide which led to improvement in ejection fraction to 55% with only mild mitral regurgitation.

  11. Population Pharmacokinetics of Hydroxychloroquine in Japanese Patients With Cutaneous or Systemic Lupus Erythematosus.

    Science.gov (United States)

    Morita, Shigemichi; Takahashi, Toshiya; Yoshida, Yasushi; Yokota, Naohisa

    2016-04-01

    Hydroxychloroquine (HCQ) is an effective treatment for patients with cutaneous lupus erythematosus (CLE) or systemic lupus erythematosus (SLE) and has been used for these patients in more than 70 nations. However, in Japan, HCQ has not been approved for CLE or SLE. To establish an appropriate therapeutic regimen and to clarify the pharmacokinetics (PK) of HCQ in Japanese patients with CLE with or without SLE (CLE/SLE), a population pharmacokinetic (PopPK) analysis was performed. In a clinical study of Japanese patients with a diagnosis of CLE irrespective of the presence of SLE, blood and plasma drug concentration-time data receiving multiple oral doses of HCQ sulfate (200-400 mg daily) were analyzed using nonlinear mixed-effects model software. The blood and plasma concentrations of HCQ were analyzed using a high-performance liquid chromatography tandem mass spectrometry method. Model evaluation and validation were performed using goodness-of-fit (GOF) plots, visual predictive check, and a bootstrap. The PopPKs of HCQ in the blood and plasma of 90 Japanese patients with CLE/SLE were well described by a 1-compartment model with first-order absorption and absorption lag time. Body weight was a significant (P < 0.001) covariate of oral clearance of HCQ. The final model was assessed using GOF plots, a bootstrap, and visual predictive check, and this model was appropriate. Simulations based on the final model suggested that the recommended daily doses of HCQ sulfate (200-400 mg) based on the ideal body weight in Japanese patients with CLE/SLE were in the similar concentration ranges. The PopPK models derived from both blood and plasma HCQ concentrations of Japanese patients with CLE/SLE were developed and validated. Based on this study, the dosage regimens of HCQ sulfate for Japanese patients with CLE/SLE should be calculated using the individual ideal body weight.

  12. Evaluation of early cardiac dysfunction in patients with systemic lupus erythematosus with or without anticardiolipin antibodies.

    Science.gov (United States)

    Barutcu, A; Aksu, F; Ozcelik, F; Barutcu, C A E; Umit, G E; Pamuk, O N; Altun, A

    2015-09-01

    The aim of this study was to use transthoracic Doppler echocardiographic (TTE) imaging methods to identify cardiac dysfunction, an indicator of subclinical atherosclerosis in asymptomatic systemic lupus erythematosus (SLE) patients in terms of cardiac effects. This study involved 80 patients: a study group (n = 50) and control group (n = 30). They were categorized into four subgroups: anticardiolipin antibodies (aCL) (+) (n = 14) and aCL (-) (n = 36); systemic lupus erythematosus disease activity index (SLEDAI) ≥ 6 (n = 15) and SLEDAI 5 years group compared with the disease period <5 years group (p < 0.01, p < 0.05, respectively). Carrying out regular scans with TTE image of SLE patients is important in order to identify early cardiac involvement during monitoring and treatment. Identifying early cardiac involvement in SLE may lead to a reduction in mortality and morbidity rates. © The Author(s) 2015.

  13. Advances in our understanding of immunization and vaccines for patients with systemic lupus erythematosus.

    Science.gov (United States)

    Bragazzi, Nicola Luigi; Watad, Abdulla; Sharif, Kassem; Adawi, Mohammad; Aljadeff, Gali; Amital, Howard; Shoenfeld, Yehuda

    2017-10-01

    Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease. In SLE, immune system dysfunction is postulated to result by virtue of the disease itself as well as by the impact of treatment modalities employed. A myriad of immune dysregulations occur including complement system dysfunction among others. Infectious agents are known to complicate the disease course in close to 25-45% of SLE patients. Areas covered: In this review a discussion of the immunogenicity and safety of viral and bacterial vaccinations in SLE was performed. The search included ISI Web of Science (WoS), Scopus, MEDLINE/PubMed, Google-Scholar, DOAJ, EbscoHOST, Scirus, Science Direct, Cochrane Library and ProQuest. Proper string made up of a key-words including 'SLE', 'vaccination', 'safety' and 'efficacy' was used. Expert commentary: Vaccination of SLE patients is proven to be immunogenic. Concerns regarding vaccine safety are postulated, yet no direct relationship between vaccination and disease exacerbation were established. While live virus vaccines are generally contraindicated in immunosuppressive states, generally live attenuated vaccinations are recommended in SLE patients on a case-to-case basis. In SLE patients, clinical parameters such as vaccination during disease exacerbations have not been intensively studied and therefore while apparently safe, vaccination is generally recommended while disease is quiescent.

  14. Episodic memory impairment in systemic lupus erythematosus: involvement of thalamic structures.

    Science.gov (United States)

    Zimmermann, Nicolle; Corrêa, Diogo Goulart; Netto, Tania Maria; Kubo, Tadeu; Pereira, Denis Batista; Fonseca, Rochele Paz; Gasparetto, Emerson Leandro

    2015-02-01

    Episodic memory deficits in systemic lupus erythematosus (SLE) have been frequently reported in the literature; however, little is known about the neural correlates of these deficits. We investigated differences in the volumes of different brain structures of SLE patients with and without episodic memory impairments diagnosed by the Rey Auditory Verbal Learning Test (RAVLT). Groups were paired based on age, education, sex, Mini Mental State Examination score, accumulation of disease burden (SLICC), and focused attention dimension score. Patients underwent magnetic resonance imaging (MRI). Cortical volumetric reconstruction and segmentation of the MR images were performed with the FreeSurfer software program. SLE patients with episodic memory deficits presented shorter time of diagnosis than SLE patients without episodic memory deficits. ANOVA revealed that SLE patients with episodic memory deficits had a larger third ventricle volume than SLE patients without episodic memory deficits and controls. Additionally, covariance analysis indicated group effects on the bilateral thalamus and on the third ventricle. Our findings indicate that episodic memory may be impaired in SLE patients with normal hippocampal volume. In addition, the thalamus may undergo volumetric changes associated with episodic memory loss in SLE.

  15. An evaluation of vitamin D status in individuals with systemic lupus erythematosus.

    Science.gov (United States)

    Breslin, Leanne C; Magee, Pamela J; Wallace, Julie M W; McSorley, Emeir M

    2011-11-01

    Systemic lupus erythematosus (SLE) is a multi-system inflammatory disease where genetic susceptibility coupled with largely undefined environmental factors is reported to underlie the aetiology of the disease. One such factor is low vitamin D status. The primary source of vitamin D is endogenous synthesis following exposure of the skin to UVB light. Photosensitivity, sunlight avoidance and the use of sun protection factor in combination with medications prescribed to treat the symptoms of the disease, puts SLE patients at increased risk of vitamin D deficiency. Decreased conversion of 25-hydroxyvitamin D to the metabolically active form, 1,25-dihydroxyvitamin D3, is possible, due to renal impairment common in SLE putting additional stress on vitamin D metabolism. The majority of studies have identified low 25-hydroxyvitamin D in SLE patients, albeit using varying cut-offs (importance of optimal vitamin D status in SLE, critically evaluates research carried out to date that has investigated vitamin D in SLE, and highlights the need for a well-designed observational study that controls for diet, medication use, dietary supplements, UV exposure and seasonality, that uses sensitive methods for measuring vitamin D status and disease activity in SLE to conclusively establish the role of vitamin D in SLE.

  16. Do clinical manifestations of Systemic Lupus Erythematosus in Pakistan correlate with rest of Asia?

    Science.gov (United States)

    Rabbani, Malik Anas; Siddiqui, Bilal Karim; Tahir, Muhammad Hammad; Ahmad, Bushra; Shamim, A; Majid, Shahid; Ali, Syed Sohail; Shah, Syed Mansoor Ahmed; Ahmad, Aasim

    2006-05-01

    Systemic Lupus Erythematosus (SLE) is known to be different among people with different racial, geographical and socio-economic back grounds. Asia has diverse ethnic groups broadly, Orientals in the East and Southeast Asia, Indians in South Asia and Arabs in the Middle East. These regions differ significantly from the Caucasians with reference to SLE. The purpose of this study was, therefore, to delineate the clinical pattern and disease course in Pakistani patients with SLE and compare it with Asian data. Patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatism Association admitted at the Aga Khan University Hospital between 1986 and 2001 were studied by means of a retrospective review of their records. The results were compared with various studies in different regions of Asia. Demographically, it was seen that SLE is a disease predominantly of females in their third decade, which is generally consistent with Asian data. There was less cutaneous manifestations, arthritis, serositis, haematological and renal involvement compared to various regions in Asia. The neurological manifestations of SLE, however, place Pakistani patients in the middle of a spectrum between South Asians and other Asian races. This study has shown that the clinical characteristics of SLE patients in our country may be different to those of other Asian races. Although our population is similar to South Asians, but clinical manifestations of our SLE patients are considerably different, suggesting some unknown etiology. Further studies are required to confirm the above results and to find statistically sounder associations.

  17. HMGB1 Promotes Systemic Lupus Erythematosus by Enhancing Macrophage Inflammatory Response

    Directory of Open Access Journals (Sweden)

    Mudan Lu

    2015-01-01

    Full Text Available Background/Purpose. HMGB1, which may act as a proinflammatory mediator, has been proposed to contribute to the pathogenesis of multiple chronic inflammatory and autoimmune diseases including systemic lupus erythematosus (SLE; however, the precise mechanism of HMGB1 in the pathogenic process of SLE remains obscure. Method. The expression of HMGB1 was measured by ELISA and western blot. The ELISA was also applied to detect proinflammatory cytokines levels. Furthermore, nephritic pathology was evaluated by H&E staining of renal tissues. Results. In this study, we found that HMGB1 levels were significantly increased and correlated with SLE disease activity in both clinical patients and murine model. Furthermore, gain- and loss-of-function analysis showed that HMGB1 exacerbated the severity of SLE. Of note, the HMGB1 levels were found to be associated with the levels of proinflammatory cytokines such as TNF-α and IL-6 in SLE patients. Further study demonstrated that increased HMGB1 expression deteriorated the severity of SLE via enhancing macrophage inflammatory response. Moreover, we found that receptor of advanced glycation end products played a critical role in HMGB1-mediated macrophage inflammatory response. Conclusion. These findings suggested that HMGB1 might be a risk factor for SLE, and manipulation of HMGB1 signaling might provide a therapeutic strategy for SLE.

  18. PTPN22 gene polymorphisms in autoimmune diseases with special reference to systemic lupus erythematosus disease susceptibility

    Directory of Open Access Journals (Sweden)

    Pradhan V

    2010-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a prototype autoimmune disease. SLE is a result of one or more immune mechanisms, like autoantibody production, complement activation, multiple inflammation and immune complex deposition leading to organ tissue damage. SLE affected patients are susceptible to common and opportunistic infections. There are several reports suggesting that Mycobacterium tuberculosis infection precipitates SLE in patients from endemic areas. Genetic factors and environmental factors also play an important role in the overall susceptibility to SLE pathophysiology. Recently, protein tyrosine phosphatase, non-receptor type 22 (PTPN22 gene, has been found to be associated with several autoimmune diseases like SLE, Grave′s disease and Hashimoto thyroiditis. The missense R620W polymorphism, rs 2476601, in PTPN22 gene at the nucleotide 1858 in codon 620 (620Arg > Trp has been associated with autoimmune diseases. The PTPN22 locus is also found to be responsible for development of pulmonary tuberculosis in certain populations. The PTPN22 1858C/T gene locus will be ideal to look for SLE susceptibility to tuberculosis in the Indian population. In this review, we focus on human PTPN22 gene structure and function as well as the association of PTPN22 gene polymorphisms with SLE susceptibility

  19. Influence of psychological stress on headache in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Vargas-Hitos, José Antonio; Sabio, José Mario; Martínez-Egea, Isabel; Jiménez-Jáimez, Enrique; Rodríguez-Guzmán, Manuel; Navarrete-Navarrete, Nuria; López-Lozano, Esther; Romero-Alegría, Ángela; de la Calle, Cristina; Jáimez-Gámiz, Laura; Baños-Piñero, Pilar; Nebrera-Navarro, Fernando; Fidalgo, Alba; Caminal, Luis; de Ramón Garrido, Enrique; Ortego-Centeno, Norberto; Expósito, Manuela; Zamora-Pasadas, Mónica; Jiménez-Alonso, Juan

    2014-03-01

    To compare the prevalence and disability of headache in patients with systemic lupus erythematosus (SLE) with the general population and to assess the role of chronic psychological stress (CPS) in headache development. One hundred seventy patients with SLE and 102 control subjects matched for age, sex, and level of education were included in this multicenter, cross-sectional study. CPS, headache-related disability, and chronic analgesic intake (CAI) were evaluated in all participants. No statistical differences in the prevalence of headache between both groups were observed but headache disability was significantly higher in patients with SLE. In addition, a higher average score in the Cohen Perceived Stress Scale (CPSS) and a higher prevalence of patients with CAI were observed in patients with SLE. In multivariate analysis, CPSS score was positively (OR 1.09; 95% CI: 1.03-1.14; p = 0.001) and CAI negatively (OR 0.43; 95% CI: 0.19-0.99; p = 0.049) associated with headache in patients with SLE. Despite the prevalence of headache in patients with SLE and the general population being similar, headache-related disability may be higher in patients with SLE. Moreover, CPS might play a role in the pathogenesis of SLE headache, whereas CAI might have a protective effect against it.

  20. Increased serum level of prolactin is related to autoantibody production in systemic lupus erythematosus.

    Science.gov (United States)

    Yang, J; Li, Q; Yang, X; Li, M

    2016-04-01

    Prolactin (PRL) is known to aid effector B cells and augment autoimmunity, but the role of PRL in systemic lupus erythematosus (SLE) is not fully elucidated. The aim of this study was to determine the correlation between the serum levels of PRL and autoantibody production in SLE. Blood levels of PRL, anti-double-stranded DNA (ds-DNA) antibody, immunoglobulin M (IgM) and immunoglobulin G (IgG) were determined in samples from 30 adult patients with SLE and 25 healthy controls. The relationships between the serum level of PRL and SLE disease activity, as well as the titres of the ds-DNA antibody, IgM and IgG were determined. The serum level of PRL was higher in the SLE patients than in the healthy controls. PRL concentration increased during SLE flares-ups and decreased following disease remission. There was a positive correlation between the PRL concentration and serum levels of IgM, IgG and ds-DNA antibody titre. These data suggest that the serum level of PRL was closely related to the antibody production and disease activity of SLE patients. PRL concentration was dramatically reduced upon the remission of disease activity, indicating that PRL levels might be a promising predictor of SLE disease severity. © The Author(s) 2015.

  1. Rationale and design of the screening of pulmonary hypertension in systemic lupus erythematosus (SOPHIE) study.

    Science.gov (United States)

    Huang, Duo; Cheng, Yang-Yang; Chan, Pak-Hei; Hai, Jojo; Yiu, Kai-Hang; Tse, Hung-Fat; Wong, Ka-Lam; Fan, Katherine; Li, Ying Wah; Ng, Woon-Leung; Yim, Cheuk-Wan; Wong, Cheuk-Hon John; Tam, Lai-Shan; Wong, Priscilla C H; Wong, Chi-Yuen; Ho, Chup-Hei; Leung, Alexander M H; Mok, Chi-Chiu; Lam, Ho; Lau, Chak-Sing; Cheung, Tommy; Ho, Carmen; Law, Sharon W Y; Chan, Esther W; Yin, Li-Xue; Yue, Wen-Sheng; Mok, Toi Meng; Evora, Mario Alberto; Siu, Chung-Wah

    2018-01-01

    Current guideline-recommended screening for pulmonary hypertension in patients with systemic sclerosis has not been evaluated in systemic lupus erythematosus (SLE), which is disproportionately prevalent in Asians. This multicentre, cross-sectional screening study aims to study the prevalence of pulmonary hypertension among SLE patients using these guidelines, and identify independent predictors and develop a prediction model for pulmonary hypertension in SLE patients. SLE patients from participating centres will undergo an echocardiography- and biomarker-based pulmonary hypertension screening procedure as in the DETECT study. Standard right heart catheterisation will be provided to patients with intermediate or high echocardiographic probability of pulmonary hypertension. Those with low echocardiographic probability will rescreen within 1 year. The primary measure will be the diagnosis and types of pulmonary hypertension and prevalence of pulmonary hypertension in SLE patients. The secondary measures will be the predictors and prediction models for pulmonary hypertension in SLE patients. The estimated sample size is approximately 895 participants. The results of the SOPHIE study will be an important contribution to the literature of SLE-related pulmonary hypertension and may be immediately translatable to real clinical practice. Ultimately, this study will provide the necessary evidence for establishing universal guidelines for screening of pulmonary hypertension in SLE patients.

  2. Systemic lupus erythematosus in an African Caribbean population: incidence, clinical manifestations, and survival in the Barbados National Lupus Registry.

    Science.gov (United States)

    Flower, Cindy; Hennis, Anselm J M; Hambleton, Ian R; Nicholson, George D; Liang, Matthew H

    2012-08-01

    To assess the epidemiology, clinical features, and outcomes of systemic lupus erythematosus (SLE) in the predominantly African Caribbean population of Barbados. A national registry of all patients diagnosed with SLE was established in 2007. Complete case ascertainment was facilitated by collaboration with the island's sole rheumatology service, medical practitioners, and the lupus advocacy group. Informed consent was required for inclusion. Between January 1, 2000 and December 31, 2009, there were 183 new cases of SLE (98% African Caribbean) affecting 172 women and 11 men for unadjusted annual incidence rates of 12.21 (95% confidence interval [95% CI] 10.46-14.18) and 0.84 (95% CI 0.42-1.51) per 100,000 person-years, respectively. Excluding pediatric cases (ages <18 years), the unadjusted incidence rate among women was 15.14 per 100,000 person-years. The principal presenting manifestations were arthritis (84%), nephritis (47%), pleuritis (41.5%), malar rash (36.4%), and discoid lesions (33.1%). Antinuclear antibody positivity was 95%. The overall 5-year survival rate was 79.9% (95% CI 69.6-87.1), decreasing to 68% in patients with nephritis. A total of 226 persons with SLE were alive at the end of the study for point prevalences of 152.6 (95% CI 132.8-174.5) and 10.1 (95% CI 5.4-17.2) per 100,000 among women and men, respectively. Rates of SLE in Barbadian women are among the highest reported to date, with clinical manifestations similar to African American women and high mortality. Further study of this population and similar populations of West African descent might assist our understanding of environmental, genetic, and health care issues underpinning disparities in SLE. Copyright © 2012 by the American College of Rheumatology.

  3. Improving B-cell depletion in systemic lupus erythematosus and rheumatoid arthritis.

    Science.gov (United States)

    Mota, Pedro; Reddy, Venkat; Isenberg, David

    2017-07-01

    Rituximab-based B-cell depletion (BCD) therapy is effective in refractory rheumatoid arthritis (RA) and although used to treat patients with refractory systemic lupus erythematosus (SLE) in routine clinical practice, rituximab failed to meet the primary endpoints in two large randomised controlled trials (RCTs) of non-renal (EXPLORER) and renal (LUNAR) SLE. Areas covered: We review how BCD could be improved to achieve better clinical responses in RA and SLE. Insights into the variability in clinical response to BCD in RA and SLE may help develop new therapeutic strategies. To this end, a literature search was performed using the following terms: rheumatoid arthritis, systemic erythematosus lupus, rituximab and B-cell depletion. Expert commentary: Poor trial design may have, at least partly, contributed to the apparent lack of response to BCD in the two RCTs of patients with SLE. Enhanced B-cell depletion and/or sequential therapy with belimumab may improve clinical response at least in some patients with SLE.

  4. Seasonal distribution of active systemic lupus erythematosus and its correlation with meteorological factors

    Directory of Open Access Journals (Sweden)

    Zhang Hua-Li

    2011-01-01

    Full Text Available OBJECTIVE: To explore the characteristics of seasonal distribution of active systemic lupus erythematosus (SLE and the influences of meteorological factors including temperature and humidity on active systemic lupus erythematosus. METHODS: The characteristics of seasonal distribution of active SLE and its correlation with meteorological factors were retrospectively analyzed in 640 patients living in the city of Zhanjiang, China and had active SLE between January 1997 and December 2006. RESULTS: In winter, when there are weaker ultraviolet (UV rays, the ratio of patients with active SLE to total inpatients was 3.89 %o, which is significantly higher than in other seasons with stronger UV rays, including 2.17 %o in spring, 1.87 0 in summer and 2.12 0 in autumn. The number of patients with active SLE had significant negative correlation with mean temperature and was not significantly related to mean humidity. CONCLUSION: Active SLE has the characteristics of seasonal distribution and is associated with temperature. The mechanism remains to be further studied.

  5. Impact of Childbearing Decisions on Family Size of Korean Women with Systemic Lupus Erythematosus.

    Science.gov (United States)

    Kim, In Je; Kim, Hyoun-Ah; Suh, Chang-Hee; Park, Yong-Wook; Lee, Hye-Soon; Bang, So-Young; Bae, Sang-Cheol; Kang, Young Mo; Lee, Won Kyung; Park, Hyesook; Lee, Jisoo

    2016-05-01

    Systemic lupus erythematosus (SLE) predominantly affects women in their reproductive years and has a significant impact on childbearing. We investigated the influence of personal decision on family size among Korean women with SLE and factors that affect the decisions. A case-control study comparing childbearing history and decisions of 112 SLE patients and 135 controls was performed. Women with SLE participating in the Network for Lupus Clinical Research in South Korea and matching controls between ages of 18-45, who are/were married or living with a partner were included. Data regarding socio-demographics, reproductive history, and childbearing decisions were collected through a survey using a standardized questionnaire and medical record review. More women with SLE reported at least one pregnancy (85.7% vs. 71.9%, P = 0.009) or at least one live birth (85.7% vs. 71.9%, P = 0.003) compared with controls. Mean number of pregnancies was significantly higher (2.4 ± 1.6 vs. 1.4 ± 1.3, P family size and childbearing decisions among Korean women with SLE.

  6. Retinal vasculopathy in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Gao, N; Li, M T; Li, Y H; Zhang, S H; Dai, R P; Zhang, S Z; Zhao, L D; Wang, L; Zhang, F C; Zhao, Y; Zeng, X F

    2017-10-01

    A retrospective case control study was conducted in the Peking Union Medical College Hospital. Medical records were reviewed for demographic data, clinical features, laboratory results, systemic lupus erythematosus (SLE) disease activity evaluations, and ophthalmic examinations to investigate the clinical characteristics and significance of retinal vasculopathy (RV) in Chinese patients with systemic lupus erythematosus. The prevalence of RV was approximately 0.66% (35/5298) in SLE patients. A total of 60 eyes were involved. The ocular presentations included decrease of visual acuity (48/60, 80%), visual field loss (7/60, 11.7%), and diplopia (3/60, 5%). Ophthalmic fundoscopic examination revealed cotton-wool spots (30/60, 50%), retinal vascular attenuation (31/60, 51.6%), and hemorrhages (41/60, 68.3%). Retinal angiogram showed that 72.7% (16/22) eyes had vaso-occlusion. The ophthalmic episodes could occur at any stage of SLE duration, with a median of 12 months (0-168 months) following SLE onset. Twenty-one (35%) eyes did not recover, or even worsened, during hospital stay. RV was found to be significantly associated with neuropsychiatric lesions (51.4% vs. 21.3%, p = .005) and hematological disturbance (62.9% vs. 34.3%, p = .005). SLE patients with RV had significantly higher SLE disease activity index scores than controls (19.9 ± 0.9 vs. 10.2 ± 0.7, p < .001). An inverse association of anti-SSA antibody with RV was detected (34.3% vs. 67.1%, p = .001). Nervous system disturbance (odds ratio (OR) = 4.340, 95% confidence interval (CI) 1.438, 13.094, p = .009) and leukocytopenia (OR = 6.385, 95% CI 1.916, 21.278, p = .003) were independent risk factors, while anti-SSA antibody positivity (OR = 0.249, 95% CI 0.087, 0.710, p = .009) was a protective factor for RV in SLE patients. In certain cases, RV is a threatening condition for SLE patients presenting with clinical ocular manifestations. Ophthalmo

  7. Metabolic syndrome in Iranian patients with systemic lupus erythematosus and its determinants.

    Science.gov (United States)

    Fatemi, Alimohammad; Ghanbarian, Azadeh; Sayedbonakdar, Zahra; Kazemi, Mehdi; Smiley, Abbas

    2018-01-05

    The aim of this study was to determine the prevalence of metabolic syndrome (MetS) in Iranian patients with systemic lupus erythematosus (SLE) and its determinants. In a cross-sectional study, 98 patients with SLE and 95 controls were enrolled. Prevalence of MetS was determined based on American Heart Association and National Heart, Lung, and Blood Institute (AHA/NHLBI) and 2009 harmonizing criteria. In addition, demographic features and lupus characteristics such as disease duration, pharmacological treatment, laboratory data, SLE disease activity index (SLEDAI), and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage index (SDI) were recorded. The predictors of MetS were obtained by backward stepwise regression analysis. Using AHA/NHLBI, MetS was observed in 35 (35.7%) patients and 28 (29.8%) controls (P = 0.4). Using harmonizing criteria, MetS was observed in 37 (37.7%) patients and 33 (35.1%) controls (P = 0.7). There was no difference in frequency distribution of MetS components between the patients and the controls. In multivariate regression analysis, low C3, blood urea nitrogen (BUN), and body mass index were independent determinants of MetS in lupus patients. BUN, low C3, and body mass index were the major determinants of MetS in lupus patients.

  8. Role of MHC-Linked Susceptibility Genes in the Pathogenesis of Human and Murine Lupus

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    Manfred Relle

    2012-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic autoimmune disease characterized by the production of autoantibodies against nuclear antigens and a systemic inflammation that can damage a broad spectrum of organs. SLE patients suffer from a wide variety of symptoms, which can affect virtually almost any tissue. As lupus is difficult to diagnose, the worldwide prevalence of SLE can only be roughly estimated to range from 10 and 200 cases per 100,000 individuals with dramatic differences depending on gender, ethnicity, and location. Although the treatment of this disease has been significantly ameliorated by new therapies, improved conventional drug therapy options, and a trained expert eye, the underlying pathogenesis of lupus still remain widely unknown. The complex etiology reflects the complex genetic background of the disease, which is also not well understood yet. However, in the past few years advances in lupus genetics have been made, notably with the publication of genome-wide association studies (GWAS in humans and the identification of susceptibility genes and loci in mice. This paper reviews the role of MHC-linked susceptibility genes in the pathogenesis of systemic lupus erythematosus.

  9. A Comparitive Study of Anticardiolipin Antibodies among Systemic Lupus Erythematosus Patients from Western and Eastern India.

    Science.gov (United States)

    Doley, D; Kakati, S; Saikia, L; Rajadhyaksha, A; Nadkar, Milind; Khadilkar, P; Patwardhan, M; Pradhan, V

    2017-03-01

    Anti-phospholipid antibodies (APA) like anticardiolipin antibodies (ACA) are important cause of venous and arterial thrombosis and other occlusive vascular diseases. Prevalence of these antibodies in SLE patients at the time of diagnosis is not known in Indian SLE patients. This study was conducted to evaluate the prevalence of ACA in SLE patients from Eastern and Western India and to correlate them with disease activity. Seventy SLE patients from Assam Medical College, Dibrugarh, Assam and 85 SLE patients from Rheumatology Department, KEM Hospital, Mumbai were studied. SLE disease activity was evaluated by SLE Disease Activity Index (SLEDAI) score at the time of evaluation. All patients studied were in an active stage of disease. Demographic data showed significant variations in the clinical manifestations of SLE between two regions. Renal manifestations were higher (42.9%) among SLE patients from Eastern region as compared with 37.6% patients from Western region. These patients were categorized as Lupus Nephritis (LN) and patients that did not show any renal manifestations were categories as non-LN. ACA to IgG and IgM subclasses were tested by ELISA. IgGACA positivity was 20%, 12.9% and IgM-ACA positivity was 18.6%, 12.9% where asIgG + IgM ACA positivity as found in 12.9%, 3.5% patients respectively among SLE patients from Eastern and Western India. ACA positivity was higher among LN patients from Eastern India whereas the same was higher among non-LN patients from Western India. Hence detection of ACA alongwith associated clinical manifestations were helpful to evaluate their possible association with disease severity in SLE patients. A long term follow up of patients having ACA antibodies without thrombotic event is needed to detect their possible thrombotic event in future along with their clinical presentation from these two different geographic regions from India.

  10. Association of STAT4 polymorphism with severe renal insufficiency in lupus nephritis.

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    Karin Bolin

    Full Text Available Lupus nephritis is a cause of significant morbidity in systemic lupus erythematosus (SLE and its genetic background has not been completely clarified. The aim of this investigation was to analyze single nucleotide polymorphisms (SNPs for association with lupus nephritis, its severe form proliferative nephritis and renal outcome, in two Swedish cohorts. Cohort I (n = 567 SLE cases, n = 512 controls was previously genotyped for 5676 SNPs and cohort II (n = 145 SLE cases, n = 619 controls was genotyped for SNPs in STAT4, IRF5, TNIP1 and BLK. Case-control and case-only association analyses for patients with lupus nephritis, proliferative nephritis and severe renal insufficiency were performed. In the case-control analysis of cohort I, four highly linked SNPs in STAT4 were associated with lupus nephritis with genome wide significance with p = 3.7 × 10(-9, OR 2.20 for the best SNP rs11889341. Strong signals of association between IRF5 and an HLA-DR3 SNP marker were also detected in the lupus nephritis case versus healthy control analysis (p <0.0001. An additional six genes showed an association with lupus nephritis with p <0.001 (PMS2, TNIP1, CARD11, ITGAM, BLK and IRAK1. In the case-only meta-analysis of the two cohorts, the STAT4 SNP rs7582694 was associated with severe renal insufficiency with p = 1.6 × 10(-3 and OR 2.22. We conclude that genetic variations in STAT4 predispose to lupus nephritis and a worse outcome with severe renal insufficiency.

  11. Differential Genetic Associations for Systemic Lupus Erythematosus Based on Anti–dsDNA Autoantibody Production

    Science.gov (United States)

    Chung, Sharon A.; Taylor, Kimberly E.; Graham, Robert R.; Nititham, Joanne; Lee, Annette T.; Ortmann, Ward A.; Jacob, Chaim O.; Alarcón-Riquelme, Marta E.; Tsao, Betty P.; Harley, John B.; Gaffney, Patrick M.; Moser, Kathy L.; Petri, Michelle; Demirci, F. Yesim; Kamboh, M. Ilyas; Manzi, Susan; Gregersen, Peter K.; Langefeld, Carl D.; Behrens, Timothy W.; Criswell, Lindsey A.

    2011-01-01

    Systemic lupus erythematosus (SLE) is a clinically heterogeneous, systemic autoimmune disease characterized by autoantibody formation. Previously published genome-wide association studies (GWAS) have investigated SLE as a single phenotype. Therefore, we conducted a GWAS to identify genetic factors associated with anti–dsDNA autoantibody production, a SLE–related autoantibody with diagnostic and clinical importance. Using two independent datasets, over 400,000 single nucleotide polymorphisms (SNPs) were studied in a total of 1,717 SLE cases and 4,813 healthy controls. Anti–dsDNA autoantibody positive (anti–dsDNA +, n = 811) and anti–dsDNA autoantibody negative (anti–dsDNA –, n = 906) SLE cases were compared to healthy controls and to each other to identify SNPs associated specifically with these SLE subtypes. SNPs in the previously identified SLE susceptibility loci STAT4, IRF5, ITGAM, and the major histocompatibility complex were strongly associated with anti–dsDNA + SLE. Far fewer and weaker associations were observed for anti–dsDNA – SLE. For example, rs7574865 in STAT4 had an OR for anti–dsDNA + SLE of 1.77 (95% CI 1.57–1.99, p = 2.0E-20) compared to an OR for anti–dsDNA – SLE of 1.26 (95% CI 1.12–1.41, p = 2.4E-04), with pheterogeneity<0.0005. SNPs in the SLE susceptibility loci BANK1, KIAA1542, and UBE2L3 showed evidence of association with anti–dsDNA + SLE and were not associated with anti–dsDNA – SLE. In conclusion, we identified differential genetic associations with SLE based on anti–dsDNA autoantibody production. Many previously identified SLE susceptibility loci may confer disease risk through their role in autoantibody production and be more accurately described as autoantibody propensity loci. Lack of strong SNP associations may suggest that other types of genetic variation or non-genetic factors such as environmental exposures have a greater impact on susceptibility to anti–dsDNA – SLE. PMID

  12. Maternal, Fetal and Neonatal Outcomes in Pregnant Women with Systemic Lupus Erythematosus: A Comprehensive Review Study

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    Fatemeh Zahra Karimi

    2017-11-01

    Full Text Available Background:Systemic lupus erythematosus (SLE is an autoimmune disease with multiple organ involvement and periods of relapse and remission that mainly affects young women of childbearing age. In this regard the reproductive health is an important issue. Although diagnosis, treatment and management of pregnancy in SLE women have been improved recently, but the main concern is effects of SLE on maternal, fetal and neonatal outcomes. This study aimed to investigate the maternal, fetal and neonatal outcomes in pregnant women with SLE. Materials and Methods: The databases of PubMed, Medline, Scopus and Web of Science as well as domestic database (Persian such as SID, Magiran, Irandoc, and Google Scholar were searched with using keywords such as" Systemic lupus erythematosus"; "Pregnancy"; "Neonatal lupus"; "maternal, fetus or neonatal outcome";  and equivalent Persian words. Included were all Persian and English articles, published between 2000 and May 2017. Finally, a total of 77 studies were included. Results: Adverse perinatal outcomes increase in pregnancies with lupus. Outcomes include respiratory, cardiovascular, blood and skin disorders in mothers; stillbirth, spontaneous, and recurrent abortion in fetuses and neonatal lupus, prematurity, intrauterine growth restriction (IUGR, and small for gestational age (SGA in neonates, respectively. Conclusion: Pregnant women with SLE are at high risk due to increased complications for both mother and fetus. It seems broad control of the women before fertilization, so that they be at full remission in the beginning of pregnancy and the disease activity be in complete control, it can help to improve outcomes of pregnancy and so better results can be expected.

  13. Failure to thrive and nephrocalcinosis due to distal renal tubular acidosis: A rare presentation of pediatric lupus nephritis.

    Science.gov (United States)

    Nandi, Madhumita; Das, Mrinal Kanti; Nandi, Sukanta

    2016-01-01

    A 9-year-old female child was initially diagnosed of having nephrocalcinosis with distal renal tubular acidosis (dRTA) while investigating for short stature. She later on developed features of nephrotic syndrome (NS) while on treatment for RTA. Investigation for the cause of NS revealed very strong serological evidence in favor of systemic lupus erythematosus (SLE). Histopathological confirmation could not be done due to bilateral severely contracted kidneys. There are a few case reports of dRTA as the presentation of SLE, but nephrocalcinosis with dRTA with subsequent manifestation of SLE has hitherto not been reported in literature.

  14. Integrative medicine for managing the symptoms of lupus nephritis

    Science.gov (United States)

    Choi, Tae-Young; Jun, Ji Hee; Lee, Myeong Soo

    2018-01-01

    Abstract Background: Integrative medicine is claimed to improve symptoms of lupus nephritis. No systematic reviews have been performed for the application of integrative medicine for lupus nephritis on patients with systemic lupus erythematosus (SLE). Thus, this review will aim to evaluate the current evidence on the efficacy of integrative medicine for the management of lupus nephritis in patients with SLE. Methods and analyses: The following electronic databases will be searched for studies published from their dates of inception February 2018: Medline, EMBASE and the Cochrane Central Register of Controlled Trials (CENTRAL), as well as 6 Korean medical databases (Korea Med, the Oriental Medicine Advanced Search Integrated System [OASIS], DBpia, the Korean Medical Database [KM base], the Research Information Service System [RISS], and the Korean Studies Information Services System [KISS]), and 1 Chinese medical database (the China National Knowledge Infrastructure [CNKI]). Study selection, data extraction, and assessment will be performed independently by 2 researchers. The risk of bias (ROB) will be assessed using the Cochrane ROB tool. Dissemination: This systematic review will be published in a peer-reviewed journal and disseminated both electronically and in print. The review will be updated to inform and guide healthcare practice and policy. Trial registration number: PROSPERO 2018 CRD42018085205 PMID:29595669

  15. Epidemiology and sociodemographics of systemic lupus erythematosus and lupus nephritis among US adults with Medicaid coverage, 2000-2004.

    Science.gov (United States)

    Feldman, Candace H; Hiraki, Linda T; Liu, Jun; Fischer, Michael A; Solomon, Daniel H; Alarcón, Graciela S; Winkelmayer, Wolfgang C; Costenbader, Karen H

    2013-03-01

    Systemic lupus erythematosus (SLE) and lupus nephritis (LN) disproportionately affect individuals who are members of racial/ethnic minority groups and individuals of lower socioeconomic status (SES). This study was undertaken to investigate the epidemiology and sociodemographics of SLE and LN in the low-income US Medicaid population. We utilized Medicaid Analytic eXtract data, with billing claims from 47 states and Washington, DC, for 23.9 million individuals ages 18-65 years who were enrolled in Medicaid for >3 months in 2000-2004. Individuals with SLE (≥3 visits >30 days apart with an International Classification of Diseases, Ninth Revision [ICD-9] code of 710.0) and with LN (≥2 visits with an ICD-9 code for glomerulonephritis, proteinuria, or renal failure) were identified. We calculated SLE and LN prevalence and incidence, stratified by sociodemographic category, and adjusted for number of American College of Rheumatology (ACR) member rheumatologists in the state and SES using a validated composite of US Census variables. We identified 34,339 individuals with SLE (prevalence 143.7 per 100,000) and 7,388 (21.5%) with LN (prevalence 30.9 per 100,000). SLE prevalence was 6 times higher among women, nearly double in African American compared to white women, and highest in the US South. LN prevalence was higher among all racial/ethnic minority groups compared to whites. The areas with lowest SES had the highest prevalence; areas with the fewest ACR rheumatologists had the lowest prevalence. SLE incidence was 23.2 per 100,000 person-years and LN incidence was 6.9 per 100,000 person-years, with similar sociodemographic trends. In this nationwide Medicaid population, there was sociodemographic variation in SLE and LN prevalence and incidence. Understanding the increased burden of SLE and its complications in this low-income population has implications for resource allocation and access to subspecialty care. Copyright © 2013 by the American College of Rheumatology.

  16. Critical Role of CD8 T Cells in Mediating Sex-Based Differences in a Murine Model of Lupus

    Science.gov (United States)

    2009-08-21

    with the disease. Exposure to certain chemicals has also been linked to SLE. Occupational exposure to crystalline silica has been shown to increase...Pathogenesis of Systemic Lupus Erythematosus. In Kelley’s Textbook of Rheumatology , 8th ed., 8th ed. G. S. Firestein, ed. W.B. Saunders...lupus erythematosus: alterations associated with prednisolone therapy . Scand J Immunol 31:139-145. 70. Ettinger, R., G. P. Sims, A. M. Fairhurst, R

  17. A multi-group confirmatory factor analyses of the LupusPRO between southern California and Filipino samples of patients with systemic lupus erythematosus.

    Science.gov (United States)

    Azizoddin, D R; Olmstead, R; Cost, C; Jolly, M; Ayeroff, J; Racaza, G; Sumner, L A; Ormseth, S; Weisman, M; Nicassio, P M

    2017-08-01

    Introduction Systemic lupus erythematosus (SLE) leads to a range of biopsychosocial health outcomes through an unpredictable and complex disease path. The LupusPRO is a comprehensive, self-report measure developed specifically for populations with SLE, which assesses both health-related quality of life and non-health related quality of life. Given its increasingly widespread use, additional research is needed to evaluate the psychometric integrity of the LupusPRO across diverse populations. The objectives of this study were to evaluate the performance of the LupusPRO in two divergent patient samples and the model fit between both samples. Methods Two diverse samples with SLE included 136 patients from an ethnically-diverse, urban region in southern California and 100 from an ethnically-homogenous, rural region in Manila, Philippines. All patients met the ACR classification criteria for SLE. Confirmatory factor analysis (CFAs) were conducted in each sample separately and combined to provide evidence of the factorial integrity of the 12 subscales in the LupusPRO. Results Demographic analyses indicated significant differences in age, disease activity and duration, education, income, insurance, and medication use between groups. Results of the separate CFAs indicated moderate fit to the data for the hypothesized 12-factor model for both the Manila and southern California groups, respectively [χ 2 (794) = 1283.32, p < 0.001, Comparative Fit Index (CFI) = 0.793; χ 2 (794) =1398.44, p < 0.001, CFI = 0.858]. When the factor structures of the LupusPRO in the southern California and Manila groups were constrained to be equal between the two groups, findings revealed that the factor structures of measured variables fit the two groups reasonably well [χ 2  (1697) = 2950.413, df = 1697, p < 0.000; CFI = 0.811]. After removing seven constraints and eight correlations suggested by the Lagrange multiplier test, the model fit improved

  18. Hypoparathyroidism in a patient with systemic lupus erythematosus coexisted with ankylosing spondylitis: a case report and review of literature.

    Science.gov (United States)

    Jiang, Lindi; Dai, Xiaomin; Liu, Jun; Ma, Lili; Yu, Fei

    2010-12-01

    Hypoparathyroidism is rare in patients with systemic lupus erythematosus (SLE). Here we describe a case of SLE coexisted with hypoparathyroidism and ankylosing spondylitis with definite diagnosis, and also give a review of past five cases of SLE with hypoparathyroidism. We find that hypoparathyroidism is easily ignored by subtle manifestations despite of its significant complications. More attention should be paid to clues to hypocalcemia, symptoms of central nervous system and prolonged QT interval on electrocardiogram. The three diseases may be coexistent of genetically determined markers. The cause of hypoparathyroidism in SLE patient is not clear. It may be independent of SLE. Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  19. Clinical and diagnostic significance of activity of enzymes participating in endoergic reactions of patients systemic lupus erythematosus and systemic sclerosis

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    LA Zborovskaya

    2004-01-01

    Full Text Available Objective. To improve quality of diagnosis of systemic lupus erythematosus (SLE and systemic sclerosis (SS. Material and methods. 30 pts with SLE and 30 with SS were included. Besides complex clinical, instrumental and laboratory examination activity and isoenzymes of succinate dehydrogenase (SDG, fumarate hydrase (FH, malate dehydrogenase (MDG, cytochrome oxidase (CO were evaluated trice (at admission, after two weeks and at discharge with original methods. 30 healthy persons were included in the control group. Results. SLE and SS pts had significant changes of energy metabolism enzymes depended on clinical features of the disease. Enzyme indices at minimal activity of SLE and SS were more informative than most of traditional laboratory tests. Comparative analysis of enzyme indices in SLE and SS pts revealed some features with along with clinical, instrumental and traditional laboratory data should be consider in diagnosis of these diseases. Enzyme indices correlated with changes of pts clinical state what allow to use them as criteria of treatment efficacy.

  20. Further Evidence of Subphenotype Association with Systemic Lupus Erythematosus Susceptibility Loci: A European Cases Only Study

    Science.gov (United States)

    Alonso-Perez, Elisa; Suarez-Gestal, Marian; Calaza, Manuel; Ordi-Ros, Josep; Balada, Eva; Bijl, Marc; Papasteriades, Chryssa; Carreira, Patricia; Skopouli, Fotini N.; Witte, Torsten; Endreffy, Emöke; Marchini, Maurizio; Migliaresi, Sergio; Sebastiani, Gian Domenico; Santos, Maria Jose; Suarez, Ana; Blanco, Francisco J.; Barizzone, Nadia; Pullmann, Rudolf; Ruzickova, Sarka; Lauwerys, Bernard R.; Gomez-Reino, Juan J.; Gonzalez, Antonio

    2012-01-01

    Introduction Systemic Lupus Erythematosus (SLE) shows a spectrum of clinical manifestations that complicate its diagnosis, treatment and research. This variability is likely related with environmental exposures and genetic factors among which known SLE susceptibility loci are prime candidates. The first published analyses seem to indicate that this is the case for some of them, but results are still inconclusive and we aimed to further explore this question. Methods European SLE patients, 1444, recruited at 17 centres from 10 countries were analyzed. Genotypes for 26 SLE associated SNPs were compared between patients with and without each of 11 clinical features: ten of the American College of Rheumatology (ACR) classification criteria (except ANAs) and age of disease onset. These analyses were adjusted for centre of recruitment, top ancestry informative markers, gender and time of follow-up. Overlap of samples with previous studies was excluded for assessing replication. Results There were three new associations: the SNPs in XKR6 and in FAM167A-BLK were associated with lupus nephritis (OR = 0.76 and 1.30, Pcorr = 0.007 and 0.03, respectively) and the SNP of MECP2, which is in chromosome X, with earlier age of disease onset in men. The previously reported association of STAT4 with early age of disease onset was replicated. Some other results were suggestive of the presence of additional associations. Together, the association signals provided support to some previous findings and to the characterization of lupus nephritis, autoantibodies and age of disease onset as the clinical features more associated with SLE loci. Conclusion Some of the SLE loci shape the disease phenotype in addition to increase susceptibility to SLE. This influence is more prominent for some clinical features than for others. However, results are only partially consistent between studies and subphenotype specific GWAS are needed to unravel their genetic component. PMID:23049788

  1. Nocturnal Hypertension and Attenuated Nocturnal Blood Pressure Dipping is Common in Pediatric Lupus

    Science.gov (United States)

    Campbell, J. Fallon; Swartz, Sarah J.; Wenderfer, Scott E.

    2015-01-01

    Hypertension is an important manifestation of systemic lupus erythematosus (SLE) but reports of prevalence vary between 20-70% in published reports of adult and pediatric patients. For both children and adults with SLE, the clinical diagnosis and management of hypertension has traditionally been based on guidelines developed for the general population. In clinical trials, the criteria used for defining participants with hypertension are mostly undefined. As a first step towards formally assessing the blood pressure (BP) patterns of children diagnosed with SLE, 24-hr ambulatory BP monitoring data was analyzed on clinic patients who presented with prehypertension or stage I hypertension. In this pediatric SLE cohort (n=10), 20% met daytime criteria for a diagnosis of hypertension. Patterns of BP elevation varied widely with white coat, masked, isolated systolic, and diastolic nocturnal hypertension all identified. Nocturnal hypertension was detected in 60% and attenuated nocturnal BP dipping in 90% of both hypertensive and normotensive SLE patients. In SLE patients, the median nighttime systolic and diastolic loads were 25% and 15.5% compared with median daily loads of 12.5% and 11.5%. Daytime and nighttime systolic and diastolic BP load and nocturnal dipping was compared to a control population consisting of 85 non-SLE patients under 21 years old with prehypertension or stage 1 hypertension presenting to hypertension clinic. Median systolic BP dipped 5.3 mmHg in SLE patients compared to 11.9 mmHg in non-lupus ( p-value = 0.001). Median diastolic BP dipped 12.9 mmHg versus 18.5 mmHg in non-lupus ( p-value = 0.003). Patterns of BP dysregulation in pediatric SLE merit further exploration. Children with or without SLE displaying prehypertensive or stage 1 casual BP measurements had similar rates of hypertension by ambulatory BP monitoring. However, regardless of BP diagnosis, and independent of kidney involvement, there was an increased proportion with attenuated

  2. Identification of unique microRNA signature associated with lupus nephritis.

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    Jeannie L Te

    2010-05-01

    Full Text Available MicroRNAs (miRNA have emerged as an important new class of modulators of gene expression. In this study we investigated miRNA that are differentially expressed in lupus nephritis. Microarray technology was used to investigate differentially expressed miRNA in peripheral blood mononuclear cells (PBMCs and Epstein-Barr Virus (EBV-transformed cell lines obtained from lupus nephritis affected patients and unaffected controls. TaqMan-based stem-loop real-time polymerase chain reaction was used for validation. Microarray analysis of miRNA expressed in both African American (AA and European American (EA derived lupus nephritis samples revealed 29 and 50 differentially expressed miRNA, respectively, of 850 tested. There were 18 miRNA that were differentially expressed in both racial groups. When samples from both racial groups and different specimen types were considered, there were 5 primary miRNA that were differentially expressed. We have identified 5 miRNA; hsa-miR-371-5P, hsa-miR-423-5P, hsa-miR-638, hsa-miR-1224-3P and hsa-miR-663 that were differentially expressed in lupus nephritis across different racial groups and all specimen types tested. Hsa-miR-371-5P, hsa-miR-1224-3P and hsa-miR-423-5P, are reported here for the first time to be associated with lupus nephritis. Our work establishes EBV-transformed B cell lines as a useful model for the discovery of miRNA as biomarkers for SLE. Based on these findings, we postulate that these differentially expressed miRNA may be potential novel biomarkers for SLE as well as help elucidate pathogenic mechanisms of lupus nephritis. The investigation of miRNA profiles in SLE may lead to the discovery and development of novel methods to diagnosis, treat and prevent SLE.

  3. 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.

  4. Systemic lupus erythematosus and the Crithidia luciliae immunofluorescent test

    International Nuclear Information System (INIS)

    Whitehouse, I.J.; Fehr, K.; Wagenhaeuser, F.J.

    1983-01-01

    A comparative study of the Crithidia luciliae immunofluorescence (CL-IF) assay and an adapted Farr radioimmunoassay (RIA), for the measurement of antibodies to native deoxyribonucleic acid, was performed using forty-two sera from patients with systematic lupus erythematosus (SLE) and another forty-two from patients with rheumatoid arthritis. Both assays were specific for SLE. The CL-IF assay was statistically significantly more sensitive than the adapted RIA assay. This significant difference was due to greater sensitivity of the CL-IF assay in the cases of sera from patients with SLE of slight activity. Additional advantages of the CL-IF assay were its use to classify the immunoglobulin types of the antibodies (most commonly IgG or IgM) and to measure complement-fixing antibodies to native deoxyribonucleic acid; it affords a simple method of selecting and following SLE patients at risk of developing severe renal disease. These advantages plus the simplicity and inexpensiveness of the CL-IF assay make it a useful tool, especially for use in small laboratories, for the study of antibodies to native deoxyribonucleic acid in patients with SLE. (orig.) [de

  5. Cigarette smoking and the pathogenesis of systemic lupus erythematosus.

    Science.gov (United States)

    Speyer, Cameron B; Costenbader, Karen H

    2018-06-01

    Systemic lupus erythematosus (SLE) is a multi-system inflammatory autoimmune disease of incompletely understood etiology. It is thought that environmental exposures 'trigger' or accelerate the disease in genetically-predisposed individuals. Areas covered: Substantial epidemiological evidence exists to support the association between cigarette smoking and the risk of incident SLE. Recent evidence points to current smoking as the specific risk factor, with decreasing risk 5 years after smoking cessation, and the greatest risk for disease characterized by the presence of SLE-specific autoantibodies. Research has begun to search for possible explanations for the temporal nature of the relationship between current smoking and autoantibody positive-SLE. Here we review potential biologic mechanisms linking smoking and SLE risk, including effects upon T and B cells, inflammatory cytokines, oxidative stress, and the formation of short-lived DNA adducts. Expert commentary: The directions for future research in this field include studies of gene-environment interactions, epigenetics, metabolomics and putative biologic mechanisms.

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

    Science.gov (United States)

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

    2017-10-25

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

  7. Ultraviolet-A1 irradiation therapy for systemic lupus erythematosus.

    Science.gov (United States)

    McGrath, H

    2017-10-01

    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 these

  8. 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

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

    Directory of Open Access Journals (Sweden)

    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

  10. [CD4 lymphocytopenia in systemic lupus erythematosus].

    Science.gov (United States)

    Ferreira, Sofia; Vasconcelos, Júlia; Marinho, António; Farinha, Fátima; Almeida, Isabel; Correia, João; Barbosa, Paulo; Mendonça, Teresa; Vasconcelos, Carlos

    2009-01-01

    Systemic Lupus Erythematosus (SLE) is an inflammatory chronic disease characterized by the presence of autoantibodies, immunocomplex production and organ injury. Several alterations of the immune system have been described, namely of CD4 T cells, with particular focus on regulatory subgroup. Quantify peripheral CD4 T cells in a population of patients with SLE and correlate it with lupus activity, affected organs, therapeutics and infections. Retrospective study involving all SLE patients seen in the clinical immunology outpatient clinic of the Hospital Geral Santo António, Porto that has done some peripheral blood flow cytometry study. Twenty-nine patients have been evaluated, 16 were taking glucocorticoids and six immunossupressors. The mean SLEDAI at the study time was nine and the ECLAM was three. Thirty-one percent of the patients had leukopenia, 76% lymphocytopenia and the same number CD4 depletion. Fifty-five percent of the patients had CD4 levels lower than 500/mm3, 31% lower than 200/mm3. All patients with SLEDAI > or = 20 and ECLAM > or = 4 had CD4 counts inferior to 500/mm3 and all patients with inactive disease had CD4 superior to 500/mm3. There have been three opportunistic infections: cryptococcal meningitis, pulmonary aspergilosis, Pneumocystis jirovecii pneumonia, all in patients with CD4 counts lower than 500/mm3. Decreased CD4 T cells counts have been very common in this study population. There is an inverse relation between CD4 cells counts and disease activity. Opportunistic infections occurred in patients with severe CD4 depletion.

  11. 46-Year Trends in Systemic Lupus Erythematosus Mortality in the United States, 1968 to 2013: A Nationwide Population-Based Study.

    Science.gov (United States)

    Yen, Eric Y; Shaheen, Magda; Woo, Jennifer M P; Mercer, Neil; Li, Ning; McCurdy, Deborah K; Karlamangla, Arun; Singh, Ram R

    2017-12-05

    No large population-based studies have been done on systemic lupus erythematosus (SLE) mortality trends in the United States. To identify secular trends and population characteristics associated with SLE mortality. Population-based study using a national mortality database and census data. United States. All U.S. residents, 1968 through 2013. Joinpoint trend analysis of annual age-standardized mortality rates (ASMRs) for SLE and non-SLE causes by sex, race/ethnicity, and geographic region; multiple logistic regression analysis to determine independent associations of demographic variables and period with SLE mortality. There were 50 249 SLE deaths and 100 851 288 non-SLE deaths from 1968 through 2013. Over this period, the SLE ASMR decreased less than the non-SLE ASMR, with a 34.6% cumulative increase in the ratio of the former to the latter. The non-SLE ASMR decreased every year starting in 1968, whereas the SLE ASMR decreased between 1968 and 1975, increased between 1975 and 1999, and decreased thereafter. Similar patterns were seen in both sexes, among black persons, and in the South. However, statistically significant increases in the SLE ASMR did not occur among white persons over the 46-year period. Females, black persons, and residents of the South had higher SLE ASMRs and larger cumulative increases in the ratio of the SLE to the non-SLE ASMR (31.4%, 62.5%, and 58.6%, respectively) than males, other racial/ethnic groups, and residents of other regions, respectively. Multiple logistic regression showed independent associations of sex, race, and region with SLE mortality risk and revealed significant racial/ethnic differences in associations of SLE mortality with sex and region. Underreporting of SLE on death certificates may have resulted in underestimates of SLE ASMRs. Accuracy of coding on death certificates is difficult to ascertain. Rates of SLE mortality have decreased since 1968 but remain high relative to non-SLE mortality, and significant sex

  12. 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

  13. High resolution CT findings of pleuropulmonary lnvolvement in systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Kun Sik; Kim, Jung Sik; Suh, Soo Jhi; Lee, Sung Moon; Sohn, Seok Ho; Park, Sung Bae; Kim, Hyun Chul [Keimyung University College of Medicine, Deagu (Korea, Republic of)

    1993-09-15

    To evaluate the high resolution computed tomography (HRCT) findings of pleuropulmonary involvement in systemic lupus erythematosus (SLE), we analyzed HRCT findings of 12 patients of clinically confirmed SLE with respiratory symptoms. In four patients, HRCT findings before and after chemotherapy were compared. The common HRCT findings were ground glass opacity (100%), bronchial wall thickening (66%), patch parenchymal opacity (58%), septal or intralobular line thickening (58%), micronodule (58%), central core prominence (41%), small pleural effusion (91%), and pericardial effusion (33%). Follow up HRCT obtained after treatment showed significant improvement of pleural effusion (4/4), pericardial effusion (3/3), pericardial thickening (1/1), patch opacity (2/2), and ground glass opacity (2/4). But bronchial wall thickening (2/2) and micronodule (2/2) were not improved. Although there are no pathognomonic HRCT findings in SLE, bilateral small pleural effusion, ground glass opacity, subpleural patchy opacity, and micronodule are common and suggestive findings in the pleuropulmonary involvement of SLE.

  14. Relationship between higher cortical dysfunction and the findings of magnetic resonance imaging in systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Maeshima, Etsuko; Maeshima, Shinichiro; Yamada, Yoichi; Yukawa, Susumu [Wakayama Medical Coll. (Japan)

    1996-04-01

    The relationship between systemic lupus erythematosus (SLE) and organic lesions was investigated by magnetic resonance imaging (MRI) to clarify the etiology of higher cortical dysfunction in SLE. The subjects were 10 patients with SLE, and higher cortical dysfunction was observed in 8 (80%) of the 10 patients. Five (82.5%) of the 8 patients showed abnormal MRI findings. The findings of higher cortical dysfunction were consistent with the MRI findings in 1 of the 5 patients, but not in the remaining four. MRI revealed no lesion despite the presence of higher cortical dysfunction in three patients. These results suggest that the association of organic changes and functional changes in cerebral nerve cells is important for etiology of higher cortical dysfunction in SLE. (author).

  15. Topics on vitamin D in systemic lupus erythematosus: analysis of evidence and critical literature review.

    Science.gov (United States)

    Marinho, António; Taveira, Mariana; Vasconcelos, Carlos

    2017-04-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with multiorgan inflammation, linked to the loss of immune tolerance to self-antigens and the production of a diversity of autoantibodies. The phenotype and progression of SLE have been linked to a combination of environmental, genetic, and hormonal factors. One such environmental factor is vitamin D, a vital hormone with well-established effects on mineral metabolism, skeletal health, and effects on cardiovascular system. The purpose of this article is to make the analysis of evidence and literature review of the pleomorphic effects of Vitamin D in SLE. The article is structured in topics of interest based in the authors' opinion and summarizes the evidence of studies and trials of vitamin D in SLE.

  16. Symptoms of shrinking lung syndrome reveal systemic lupus erythematosus in a 12-year-old girl.

    Science.gov (United States)

    Meinicke, Holger; Heinzmann, Andrea; Geiger, Julia; Berner, Reinhard; Hufnagel, Markus

    2013-12-01

    While pleuropulmonary involvement in systemic lupus erythematosus (SLE) is a common occurrence, shrinking lung syndrome (SLS) is a rare complication of SLE, particularly in children. We report on a teenager girl with a primary SLE diagnosis, which was based upon clinical, imaging, lung-function and histological findings ascertained to be compatible with SLS. Following a pneumonia, the patient developed inflammatory residues in the lower lobes, an event that probably caused diaphragmatic immobility and subsequently led to SLS. Treatment response to steroids, cyclophosphamide and hydroxychloroquine in this case was excellent, and efficacy was more profound than previously has been reported in the literature with respect to pediatric patients. This case report argues that prognosis of SLS in SLE is likely to be favorable when the diagnosis is made early and the disease is treated appropriately. © 2012 Wiley Periodicals, Inc.

  17. Neuromyelitis optica spectrum disorder in patient with systemic lupus erythematosus - our experience

    Directory of Open Access Journals (Sweden)

    Božić Ksenija

    2018-01-01

    Full Text Available Introduction. Neuromyelitis optica spectrum disorder (NMOSD is a rare demyelinating immune-mediated central nervous system disease. It is extremely rare to occur in patients with systemic lupus erythematosus (SLE, and it represents a diagnostic and therapeutic challenge. Case report. A 38-year-old Caucasian woman with medical history of SLE and new onset of flaccid paraparesis, fecal and urinary incontinence, persistent nausea and vomiting was admitted to our hospital. Based on the clinical presentation, magnetic resonance imaging findings and positive aquaporin 4 (AQP4 antibodies, a NMOSD with coexisting SLE were diagnosed. Pulse-doses of cyclophosphamide and glucocorticoids were efficient in patient treatment. Conclusion. In a patient with SLE and symptoms of longitudinal extensive transverse myelitis and/or optic neuritis and area postrema syndrome, assessment of AQP4 antibodies is neccessary for diagnosing NMOSD. Accurate diagnosis, and timely and long-term administration of immunosuppressive therapy are crucial for favorable outcome of these two coexisting diseases.

  18. Hand deforming arthropathy (Jaccoud’s syndrome in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    A. P. Zhornyak

    2005-01-01

    Full Text Available Objective. To characterize Jaccoud’s syndrome - hand deforming arthropathy (HDA in systemic lupus erythematosus (SLE. Material and vethods. Analysis of 235 case histories of pts with SLE followed up in the Institute of Rheumatology of RAMS from 1982 to 2002 was performed. 26 from them had HDA according to D. Alarcon-Segovia criteria (1988. SLE activity was determined according to V.A Nassonova and SLEDAI-I scale. Results. 26 from 235 SLE pts (11 % had H DA. More than in 25% from them this damage formed during the first year of the disease and included nonerosive joint damage with the development of finger deformities such as "swan neck", "boutonniere", Z-deformity of thumb, muscle atrophia and ulnar deviation. Longer treatment with glucocorticoids put off HDA development (r=0,64, p=0,0003

  19. Systemic Lupus Erythematosus in Primary Care: An Update and Practical Messages for the General Practitioner

    Science.gov (United States)

    Gergianaki, Irini; Bertsias, George

    2018-01-01

    Systemic Lupus Erythematosus (SLE) is a complex chronic autoimmune disease that manifests a wide range of organ involvement. Traditionally, the diagnosis and management of SLE is provided at secondary and tertiary centers to ensure prompt initiation of treatment, adequate control of flares and prevention of irreversible organ damage. Notwithstanding, the role of primary care in SLE is also emerging as there are still significant unmet needs such as the diagnostic delay at the community level and the high burden of therapy- and disease-related comorbidities. In the present review, we summarize practical messages for primary care physicians and general practitioners (GPs) concerning early diagnosis and proper referral of patients with SLE. In addition, we discuss the main comorbidities complicating the disease course and the recommended preventative measures, and we also provide an update on the role and current educational needs of GPs regarding the disease. PMID:29896474

  20. Relationship between higher cortical dysfunction and the findings of magnetic resonance imaging in systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Maeshima, Etsuko; Maeshima, Shinichiro; Yamada, Yoichi; Yukawa, Susumu

    1996-01-01

    The relationship between systemic lupus erythematosus (SLE) and organic lesions was investigated by magnetic resonance imaging (MRI) to clarify the etiology of higher cortical dysfunction in SLE. The subjects were 10 patients with SLE, and higher cortical dysfunction was observed in 8 (80%) of the 10 patients. Five (82.5%) of the 8 patients showed abnormal MRI findings. The findings of higher cortical dysfunction were consistent with the MRI findings in 1 of the 5 patients, but not in the remaining four. MRI revealed no lesion despite the presence of higher cortical dysfunction in three patients. These results suggest that the association of organic changes and functional changes in cerebral nerve cells is important for etiology of higher cortical dysfunction in SLE. (author)

  1. Education, employment, absenteeism, and work disability in women with systemic lupus erythematosus.

    Science.gov (United States)

    Ekblom-Kullberg, S; Kautiainen, H; Alha, P; Leirisalo-Repo, M; Julkunen, H

    2015-01-01

    To study education, employment, absenteeism, and work disability (WD) in women with systemic lupus erythematosus (SLE) compared to population controls. The study included 181 women of working age with SLE (mean age 44.0 years, disease duration 12.7 years) and 549 female population controls matched for age living in the same metropolitan area of Helsinki. Data regarding education, employment, absenteeism, and WD in patients and controls were obtained by questionnaire and personal interview. Basic education, vocational, or academic degrees and occupational categories in patients with SLE were similar to those in controls. In total, 62% of the patients were employed, compared to 77% of the controls (p Absenteeism and work disability are, however, 2-3 times more common than in controls. Less than half of the patients were on permanent disability pension due to SLE 20 years after diagnosis of the disease.

  2. Incidence and prevalence of systemic lupus erythematosus among Arab and Chaldean Americans in southeastern Michigan: the Michigan Lupus Epidemiology and Surveillance Program.

    Science.gov (United States)

    Housey, Michelle; DeGuire, Peter; Lyon-Callo, Sarah; Wang, Lu; Marder, Wendy; McCune, W Joseph; Helmick, Charles G; Gordon, Caroline; Dhar, J Patricia; Leisen, James; Somers, Emily C

    2015-05-01

    We assessed the burden of systemic lupus erythematosus (SLE) among Arab and Chaldean Americans residing in southeast Michigan. For those meeting SLE criteria from the Michigan Lupus Epidemiology and Surveillance Registry, we determined Arab or Chaldean ethnicity by links with demographic data from birth certificates and with a database of Arab and Chaldean names. We compared prevalence and incidence of SLE for Arab and Chaldean Americans with estimates for non-Arab and non-Chaldean American Whites and Blacks. We classified 54 individuals with SLE as Arab and Chaldean Americans. The age-adjusted incidence and prevalence estimates for Arab and Chaldean Americans were 7.6 and 62.6 per 100 000, respectively. Arab and Chaldean Americans had a 2.1-fold excess SLE incidence compared with non-Arab and non-Chaldean American Whites. Arab and Chaldean American women had both significantly higher incidence rates (5.0-fold increase) and prevalence estimates (7.4-fold increase) than did Arab and Chaldean American men. Recognizing that Arab and Chaldean Americans experience different disease burdens from Whites is a first step toward earlier diagnosis and designing targeted interventions. Better methods of assigning ethnicity would improve research in this population.

  3. Clinical outcomes of hydronephrosis in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Hong, Seokchan; Kim, Yong-Gil; Ahn, Soo Min; Bae, Seung-Hyeon; Lim, Doo-Ho; Kim, Jeong Kon; Lee, Chang-Keun; Yoo, Bin

    2016-12-01

    Hydronephrosis is a rare complication of systemic lupus erythematosus (SLE). Bladder and/or gastrointestinal involvement in SLE are associated with development of hydronephrosis, but the management and treatment outcomes of hydronephrosis are largely unknown. Therefore, we investigated the clinical manifestations and factors associated with the treatment response in patients with SLE complicated by hydronephrosis. A retrospective analysis was performed of all 634 SLE patients who underwent computed tomography and/or ultrasonography between January 1998 and December 2013. We reviewed the clinical characteristics and treatment outcomes of patients with SLE-associated hydronephrosis. Hydronephrosis was identified in 15 patients with SLE complicated by cystitis and/or enteritis. All patients were treated initially with moderate to high doses of corticosteroids. A follow-up imaging study showed that 11 (73.3%) of 15 patients experienced improvements in hydronephrosis, and urinary obstruction was resolved without urological intervention in the majority of these patients (8/11, 72.7%). The four patients who experienced no improvement in hydronephrosis were older than those who responded to treatment (median age [interquartile range]; 43.0 [37.5-53.0] years vs. 28.0 [21.0-38.5] years; P = 0.026). In addition, delayed treatment (≥ 1 month after onset of symptoms) with corticosteroids was more frequently observed in the non-responding patients than in the responding patients (P = 0.011). Our findings suggest that treatment with corticosteroids alone leads to favorable outcomes in patients with SLE-associated hydronephrosis, except when treatment is delayed, particularly in elderly patients. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  4. Galectin-3 binding protein links circulating microparticles with electron dense glomerular deposits in lupus nephritis.

    Science.gov (United States)

    Nielsen, C T; Østergaard, O; Rekvig, O P; Sturfelt, G; Jacobsen, S; Heegaard, N H H

    2015-10-01

    A high level of galectin-3-binding protein (G3BP) appears to distinguish circulating cell-derived microparticles in systemic lupus erythematosus (SLE). The aim of this study is to characterize the population of G3BP-positive microparticles from SLE patients compared to healthy controls, explore putative clinical correlates, and examine if G3BP is present in immune complex deposits in kidney biopsies from patients with lupus nephritis. Numbers of annexin V-binding and G3BP-exposing plasma microparticles from 56 SLE patients and 36 healthy controls were determined by flow cytometry. Quantitation of microparticle-associated G3BP, C1q and immunoglobulins was obtained by liquid chromatography tandem mass spectrometry (LC-MS/MS). Correlations between microparticle-G3BP data and clinical parameters were analyzed. Co-localization of G3BP with in vivo-bound IgG was examined in kidney biopsies from one non-SLE control and from patients with class IV (n = 2) and class V (n = 1) lupus nephritis using co-localization immune electron microscopy. Microparticle-G3BP, microparticle-C1q and microparticle-immunoglobulins were significantly (P microparticle populations could be discerned by flow cytometry, including two subpopulations that were significantly increased in SLE samples (P = 0.01 and P = 0.0002, respectively). No associations of G3BP-positive microparticles with clinical manifestations or disease activity were found. Immune electron microscopy showed co-localization of G3BP with in vivo-bound IgG in glomerular electron dense immune complex deposits in all lupus nephritis biopsies. Both circulating microparticle-G3BP numbers as well as G3BP expression are increased in SLE patients corroborating G3BP being a feature of SLE microparticles. By demonstrating G3BP co-localized with deposited immune complexes in lupus nephritis, the study supports cell-derived microparticles as a major autoantigen source and provides a new understanding of the origin of

  5. Serum IP-10 is useful for identifying renal and overall disease activity in pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Zhang, Chen-Xing; Cai, Li; Shao, Kang; Wu, Jing; Zhou, Wei; Cao, Lan-Fang; Chen, Tong-Xin

    2018-05-01

    Traditional serological biomarkers often fail to assess systemic lupus erythematosus (SLE) disease activity and discriminate lupus nephritis (LN). The aim of this study was to identify novel markers for evaluating renal and overall disease activity in Chinese patients with pediatric systemic lupus erythematosus (pSLE). The study included 46 patients with pSLE (35 girls, 11 boys; average age 13.3 ± 2.6 years) and 31 matched healthy controls (22 girls, 9 boys; average age 12.3 ± 2.4 years). The SLE Disease Activity Index (SLEDAI) and renal SLEDAI were used to assess disease activity. Nine different soluble mediators in plasma, including tumor necrosis factor alpha (TNF-α), platelet-derived growth factor-BB (PDGF-BB), interferon (IFN) gamma inducible protein 10 (IP-10), interleukin (IL)-1β, IFN-γ, IL-17A, IL-2, Fas and Fas ligand, were measured by Luminex assay and compared between patients with active and inactive pSLE as well as between patients with pSLE with active and inactive renal disease. Receiver operating characteristic curve analysis was used to measure the discrimination accuracy. Of the 46 patients with pSLE, 30 (65.2%) had LN. These patients had significantly elevated levels of serum TNF-α, PDGF-BB, IP-10 and Fas. The serum levels of IP-10 were also significantly higher in patients with active pSLE. We found that IP-10 was also more sensitive and specific than conventional laboratory parameters, including anti-double-stranded DNA and complement components C3 and C4, for distinguishing active lupus from quiescent lupus. The serum level of IP-10 was also significantly increased in children with pSLE with active renal disease relative to those with inactive renal disease. There was also a positive correlation between serum IP-10 levels and renal SLEDAI scores as well as with 24 h urine protein. Serum IP-10 is useful for identifying renal and overall disease activity in children with pSLE.

  6. 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

  7. Patient-reported outcome measures for systemic lupus erythematosus clinical trials: a review of content validity, face validity and psychometric performance.

    Science.gov (United States)

    Holloway, Laura; Humphrey, Louise; Heron, Louise; Pilling, Claire; Kitchen, Helen; Højbjerre, Lise; Strandberg-Larsen, Martin; Hansen, Brian Bekker

    2014-07-22

    Despite overall progress in treatment of autoimmune diseases, patients with systemic lupus erythematosus (SLE) experience many inflammatory symptoms representing an unmet medical need. This study aimed to create a conceptual model of the humanistic and economic burden of SLE, and review the patient-reported outcomes (PROs) used to measure such concepts in SLE clinical trials. A conceptual model for SLE was developed from structured review of published articles from 2007 to August 2013 identified from literature databases (MEDLINE, EMBASE, PsycINFO, EconLit) plus other sources (PROLabels, FDA/EMA websites, Clinicaltrials.gov). PROs targeting key symptoms/impacts were identified from the literature. They were reviewed in the context of available guidance and assessed for face and content validity and psychometric properties to determine appropriateness for use in SLE trials. The conceptual model identified fatigue, pain, cognition, daily activities, emotional well-being, physical/social functioning and work productivity as key SLE concepts. Of the 68 articles reviewed, 38 reported PRO data. From these and the other sources, 15 PROs were selected for review, including SLE-specific health-related quality of life (HRQoL) measures (n = 5), work productivity (n = 1), and generic measures of fatigue (n = 3), pain (n = 2), depression (n = 2) and HRQoL (n = 2). The Functional Assessment of Chronic Illness Therapy - Fatigue Scale (FACIT-Fatigue), Brief Pain Inventory (BPI-SF) and LupusQoL demonstrated the strongest face validity, conceptual coverage and psychometric properties measuring key concepts in the conceptual model. All PROs reviewed, except for three Lupus-specific measures, lacked qualitative SLE patient involvement during development. The Hospital Anxiety and Depression Scale (HADS), Short Form [36 item] Health Survey version 2 (SF-36v2), EuroQoL 5-dimensions (EQ-5D-3L and EQ-5D-5L) and Work Productivity and Activity Impairment Questionnaire: Lupus (WPAI:Lupus

  8. Patient-reported outcome measures for systemic lupus erythematosus clinical trials: a review of content validity, face validity and psychometric performance

    Science.gov (United States)

    2014-01-01

    Background Despite overall progress in treatment of autoimmune diseases, patients with systemic lupus erythematosus (SLE) experience many inflammatory symptoms representing an unmet medical need. This study aimed to create a conceptual model of the humanistic and economic burden of SLE, and review the patient-reported outcomes (PROs) used to measure such concepts in SLE clinical trials. Methods A conceptual model for SLE was developed from structured review of published articles from 2007 to August 2013 identified from literature databases (MEDLINE, EMBASE, PsycINFO, EconLit) plus other sources (PROLabels, FDA/EMA websites, Clinicaltrials.gov). PROs targeting key symptoms/impacts were identified from the literature. They were reviewed in the context of available guidance and assessed for face and content validity and psychometric properties to determine appropriateness for use in SLE trials. Results The conceptual model identified fatigue, pain, cognition, daily activities, emotional well-being, physical/social functioning and work productivity as key SLE concepts. Of the 68 articles reviewed, 38 reported PRO data. From these and the other sources, 15 PROs were selected for review, including SLE-specific health-related quality of life (HRQoL) measures (n = 5), work productivity (n = 1), and generic measures of fatigue (n = 3), pain (n = 2), depression (n = 2) and HRQoL (n = 2). The Functional Assessment of Chronic Illness Therapy - Fatigue Scale (FACIT-Fatigue), Brief Pain Inventory (BPI-SF) and LupusQoL demonstrated the strongest face validity, conceptual coverage and psychometric properties measuring key concepts in the conceptual model. All PROs reviewed, except for three Lupus-specific measures, lacked qualitative SLE patient involvement during development. The Hospital Anxiety and Depression Scale (HADS), Short Form [36 item] Health Survey version 2 (SF-36v2), EuroQoL 5-dimensions (EQ-5D-3L and EQ-5D-5L) and Work Productivity and

  9. Features, Treatment, and Outcomes of Macrophage Activation Syndrome in Childhood-Onset Systemic Lupus Erythematosus.

    Science.gov (United States)

    Borgia, R Ezequiel; Gerstein, Maya; Levy, Deborah M; Silverman, Earl D; Hiraki, Linda T

    2018-04-01

    To describe the features and treatment of macrophage activation syndrome (MAS) in a single-center cohort of patients with childhood-onset systemic lupus erythematosus (SLE), and to compare childhood-onset SLE manifestations and outcomes between those with and those without MAS. We included all patients with childhood-onset SLE followed up at The Hospital for Sick Children from 2002 to 2012, and identified those also diagnosed as having MAS. Demographic, clinical, and laboratory features of MAS and SLE, medication use, hospital and pediatric intensive care unit (PICU) admissions, as well as damage indices and mortality data were extracted from the Lupus database. Student's t-tests and Fisher's exact tests were used to compare continuous and categorical variables, respectively. We calculated incidence rate ratios of hospital and PICU admissions comparing patients with and those without MAS, using Poisson models. Kaplan-Meier survival analysis was used to examine the time to disease damage accrual. Of the 403 patients with childhood-onset SLE, 38 (9%) had MAS. The majority (68%) had concomitant MAS and SLE diagnoses. Fever was the most common MAS clinical feature. The frequency of renal and central nervous system disease, hospital admissions, the average daily dose of steroids, and time to disease damage were similar between those with and those without MAS. We observed a higher mortality rate among those with MAS (5%) than those without MAS (0.2%) (P = 0.02). MAS was most likely to develop concomitantly with childhood-onset SLE diagnosis. The majority of the MAS patients were successfully treated with corticosteroids with no MAS relapses. Although the numbers were small, there was a higher risk of death associated with MAS compared to SLE without MAS. © 2018, American College of Rheumatology.

  10. Perceptions of racism in healthcare among patients with systemic lupus erythematosus: a cross-sectional study

    Science.gov (United States)

    Vina, Ernest R; Hausmann, Leslie R M; Utset, Tammy O; Masi, Christopher M; Liang, Kimberly P; Kwoh, C Kent

    2015-01-01

    Background Racial disparities in the clinical outcomes of systemic lupus erythematosus (SLE) exist. Perceived racial discrimination may contribute to disparities in health. Objectives To determine if perceived racism in healthcare differs by race among patients with SLE and to evaluate its contribution to racial disparities in SLE-related outcomes. Methods 163 African–American (AA) and 180 white (WH) patients with SLE were enrolled. Structured interviews and chart reviews were done to determine perceptions of racism, SLE-related outcomes (Systemic Lupus International Collaborating Clinics (SLICC) Damage Index, SLE Disease Activity, Center for Epidemiologic Studies-Depression (CES-D)), and other variables that may affect perceptions of racism. Serial hierarchical multivariable logistic regression models were conducted. Race-stratified analyses were also performed. Results 56.0% of AA patients compared with 32.8% of WH patients had high perceptions of discrimination in healthcare (pracism. The odds of having greater disease damage (SLICC damage index ≥2) were higher in AA patients than in WH patients (crude OR 1.55 (95% CI 1.01 to 2.38)). The odds of having moderate to severe depression (CES-D ≥17) were also higher in AA patients than in WH patients (crude OR 1.94 (95% CI 1.26 to 2.98)). When adjusted for sociodemographic and clinical characteristics, racial disparities in disease damage and depression were no longer significant. Among AA patients, higher perceived racism was associated with having moderate to severe depression (adjusted OR 1.23 (95% CI 1.05 to 1.43)) even after adjusting for sociodemographic and clinical variables. Conclusions Perceptions of racism in healthcare were more common in AA patients than in WH patients with SLE and were associated with depression. Interventions aimed at modifiable factors (eg, trust in providers) may reduce higher perceptions of race-based discrimination in SLE. PMID:26322238

  11. Effect of hydroxychloroquine treatment on pro-inflammatory cytokines and disease activity in SLE patients: data from LUMINA (LXXV), a multiethnic US cohort

    Science.gov (United States)

    Willis, R; Seif, AM; McGwin, G; Martinez-Martinez, LA; González, EB; Dang, N; Papalardo, E; Liu, J; Vilá, LM; Reveille, JD; Alarcón, GS; Pierangeli, SS

    2013-01-01

    Objective We sought to determine the effect of hydroxychloroquine therapy on the levels proinflammatory/prothrombotic markers and disease activity scores in patients with systemic lupus erythematosus (SLE) in a multiethnic, multi-center cohort (LUMINA). Methods Plasma/serum samples from SLE patients (n=35) were evaluated at baseline and after hydroxychloroquine treatment. Disease activity was assessed using SLAM-R scores. Interferon (IFN)-α2, interleukin (IL)-1β, IL-6, IL-8, inducible protein (IP)-10, monocyte chemotactic protein-1, tumor necrosis factor (TNF)-α and soluble CD40 ligand (sCD40L) levels were determined by a multiplex immunoassay. Anticardiolipin antibodies were evaluated using ELISA assays. Thirty-two frequency-matched plasma/serum samples from healthy donors were used as controls. Results Levels of IL-6, IP-10, sCD40L, IFN-α and TNF-α were significantly elevated in SLE patients versus controls. There was a positive but moderate correlation between SLAM-R scores at baseline and levels of IFN-α (p=0.0546). Hydroxychloroquine therapy resulted in a significant decrease in SLAM-R scores (p=0.0157), and the decrease in SLAM-R after hydroxychloroquine therapy strongly correlated with decreases in IFN-α (p=0.0087). Conclusions Hydroxychloroquine therapy resulted in significant clinical improvement in SLE patients, which strongly correlated with reductions in IFN-α levels. This indicates an important role for the inhibition of endogenous TLR activation in the action of hydroxychloroquine in SLE and provides additional evidence for the importance of type I interferons in the pathogenesis of SLE. This study underscores the use of hydroxychloroquine in the treatment of SLE. PMID:22343096

  12. Reduced response to Epstein–Barr virus antigens by T-cells in systemic lupus erythematosus patients

    Science.gov (United States)

    Draborg, Anette Holck; Jacobsen, Søren; Westergaard, Marie; Mortensen, Shila; Larsen, Janni Lisander; Houen, Gunnar; Duus, Karen

    2014-01-01

    Objective Epstein–Barr virus (EBV) has for long been associated with systemic lupus erythematosus (SLE). In this study, we investigated the levels of latent and lytic antigen EBV-specific T-cells and antibodies in SLE patients. Methods T cells were analyzed by flow cytometry and antibodies were analyzed by enzyme-linked immunosorbent assay. Results SLE patients showed a significantly reduced number of activated (CD69) T-cells upon ex vivo stimulation with EBV nuclear antigen (EBNA) 1 or EBV early antigen diffuse (EBV-EA/D) in whole blood samples compared with healthy controls. Also, a reduced number of T-cells from SLE patients were found to produce interferon-γ upon stimulation with these antigens. Importantly, responses to a superantigen were normal in SLE patients. Compared with healthy controls, SLE patients had fewer EBV-specific T-cells but higher titres of antibodies against EBV. Furthermore, an inverse correlation was revealed between the number of lytic antigen EBV-specific T-cells and disease activity of the SLE patients, with high-activity SLE patients having fewer T-cells than low-activity SLE patients. Conclusions These results indicate a limited or a defective EBV-specific T-cell response in SLE patients, which may suggest poor control of EBV infection in SLE with an immune reaction shift towards a humoral response in an attempt to control viral reactivation. A role for decreased control of EBV as a contributing agent in the development or exacerbation of SLE is proposed. PMID:25396062

  13. Downregulation of TIM-3 mRNA expression in peripheral blood mononuclear cells from patients with systemic lupus erythematosus

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    Cai, X.Z. [Central Laboratory, First Affiliated Hospital, China Medical University, Shenyang (China); Department of Immunology, College of Basic Medical Sciences, China Medical University, Shenyang (China); Huang, W.Y.; Qiao, Y.; Chen, Y.; Du, S.Y.; Chen, D.; Yu, S. [Central Laboratory, First Affiliated Hospital, China Medical University, Shenyang (China); Liu, N. [Department of Nephrology, First Affiliated Hospital, China Medical University, Shenyang (China); Dou, L.Y. [Central Laboratory, First Affiliated Hospital, China Medical University, Shenyang (China); Jiang, Y. [Central Laboratory, First Affiliated Hospital, China Medical University, Shenyang (China); Department of Immunology, College of Basic Medical Sciences, China Medical University, Shenyang (China); Department of Dermatology, First Affiliated Hospital, China Medical University, Shenyang (China)

    2014-10-17

    The T-cell immunoglobulin and mucin domain (TIM) family is associated with autoimmune diseases, but its expression level in the immune cells of systemic lupus erythematosus (SLE) patients is not known. The aim of this study was to investigate whether the expression of TIM-3 mRNA is associated with pathogenesis of SLE. Quantitative real-time reverse transcription-polymerase chain reaction analysis (qRT-PCR) was used to determine TIM-1, TIM-3, and TIM-4 mRNA expression in peripheral blood mononuclear cells (PBMCs) from 132 patients with SLE and 62 healthy controls. The PBMC surface protein expression of TIMs in PBMCs from 20 SLE patients and 15 healthy controls was assayed by flow cytometry. Only TIM-3 mRNA expression decreased significantly in SLE patients compared with healthy controls (P<0.001). No significant differences in TIM family protein expression were observed in leukocytes from SLE patients and healthy controls (P>0.05). SLE patients with lupus nephritis (LN) had a significantly lower expression of TIM-3 mRNA than those without LN (P=0.001). There was no significant difference in the expression of TIM-3 mRNA within different classes of LN (P>0.05). Correlation of TIM-3 mRNA expression with serum IgA was highly significant (r=0.425, P=0.004), but was weakly correlated with total serum protein (r{sub s}=0.283, P=0.049) and serum albumin (r{sub s}=0.297, P=0.047). TIM-3 mRNA expression was weakly correlated with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI; r{sub s}=-0.272, P=0.032). Our results suggest that below-normal expression of TIM-3 mRNA in PBMC may be involved in the pathogenesis of SLE.

  14. Joint ultrasound baseline abnormalities predict a specific long-term clinical outcome in systemic lupus erythematosus patients.

    Science.gov (United States)

    Corzo, P; Salman-Monte, T C; Torrente-Segarra, V; Polino, L; Mojal, S; Carbonell-Abelló, J

    2017-06-01

    Objective To describe long-term clinical and serological outcome in all systemic lupus erythematosus (SLE) domains in SLE patients with hand arthralgia (HA) and joint ultrasound (JUS) inflammatory abnormalities, and to compare them with asymptomatic SLE patients with normal JUS. Methods SLE patients with HA who presented JUS inflammatory abnormalities ('cases') and SLE patients without HA who did not exhibit JUS abnormalities at baseline ('controls') were included. All SLE clinical and serological domain involvement data were collected. End follow-up clinical activity and damage scores (systemic lupus erythematosus disease activity index (SLEDAI), Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR)) were recorded. JUS inflammatory abnormalities were defined based on the Proceedings of the Seventh International Consensus Conference on Outcome Measures in Rheumatology Clinical Trials (OMERACT-7) definitions. Statistical analyses were carried out to compare 'cases' and 'controls'. Results A total of 35 patients were recruited. The 'cases', n = 18/35, had a higher incidence of musculoskeletal involvement (arthralgia and/or arthritis) through the follow-up period (38.9% vs 0%, p = 0.008) and received more hydroxychloroquine (61.1% vs 25.0%, p = 0.034) and methotrexate (27.8% vs 0%, p = 0.046) compared to 'controls', n = 17/35. Other comparisons did not reveal any statistical differences. Conclusions We found SLE patients with arthralgia who presented JUS inflammatory abnormalities received more hydroxychloroquine and methotrexate, mainly due to persistent musculoskeletal involvement over time. JUS appears to be a useful technique for predicting worse musculoskeletal outcome in SLE patients.

  15. Real-Time PCR of cytomegalovirus and Epstein-Barr virus in adult Egyptian patients with systemic lupus erythematosus.

    Science.gov (United States)

    Mohamed, Aly E; Hasen, Amany M; Mohammed, Ghada F A; Elmaraghy, Nermin N

    2015-05-01

    Infections may act as environmental triggers for induction of systemic lupus erythematosus (SLE). We sought to explore the relative frequencies of Epstein-Barr virus (EBV) and human cytomegalovirus (CMV) in adult Egyptian patients with SLE and their correlation with disease activity and damage. Thirty-three consecutive adult patients satisfying the 1997 American College of Rheumatology (ACR) Classification Criteria for SLE and 30 healthy controls were included in this case-control study. All patients were subjected to complete clinical and laboratory evaluation to determine the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR). Sera from both groups were analyzed for immunoglobulin M (IgM) and IgG antibodies against CMV and EBV. Qualitative real time polymerase chain reaction (PCR) for both viruses was performed for all SLE patients. Almost all SLE patients 32/33 (96.9%) were positive for IgG anti-CMV antibodies versus 20/30 in the control group (66.6%) (P = 0.002). All SLE patients were positive for IgG anti-EBV antibodies compared to 25 in the control group (100% vs. 83.3%, P = 0.02). CMV and EBV DNA were detected by PCR in 30.3% and 51.5% of SLE patients, respectively. A statistically significant lower SLEDAI was found in PCR positive patients for EBV compared to negative patients (9.6 ± 5.2 vs. 13.1 ± 3.1, respectively P = 0.041). Adult Egyptian patients with SLE had higher frequencies of anti-CMV and EBV IgG compared to healthy controls. Furthermore, our single point assessment of SLEDAI suggested that exposure to EBV infection might be associated with a lower disease activity. © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  16. Downregulation of TIM-3 mRNA expression in peripheral blood mononuclear cells from patients with systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Cai, X.Z.; Huang, W.Y.; Qiao, Y.; Chen, Y.; Du, S.Y.; Chen, D.; Yu, S.; Liu, N.; Dou, L.Y.; Jiang, Y.

    2014-01-01

    The T-cell immunoglobulin and mucin domain (TIM) family is associated with autoimmune diseases, but its expression level in the immune cells of systemic lupus erythematosus (SLE) patients is not known. The aim of this study was to investigate whether the expression of TIM-3 mRNA is associated with pathogenesis of SLE. Quantitative real-time reverse transcription-polymerase chain reaction analysis (qRT-PCR) was used to determine TIM-1, TIM-3, and TIM-4 mRNA expression in peripheral blood mononuclear cells (PBMCs) from 132 patients with SLE and 62 healthy controls. The PBMC surface protein expression of TIMs in PBMCs from 20 SLE patients and 15 healthy controls was assayed by flow cytometry. Only TIM-3 mRNA expression decreased significantly in SLE patients compared with healthy controls (P<0.001). No significant differences in TIM family protein expression were observed in leukocytes from SLE patients and healthy controls (P>0.05). SLE patients with lupus nephritis (LN) had a significantly lower expression of TIM-3 mRNA than those without LN (P=0.001). There was no significant difference in the expression of TIM-3 mRNA within different classes of LN (P>0.05). Correlation of TIM-3 mRNA expression with serum IgA was highly significant (r=0.425, P=0.004), but was weakly correlated with total serum protein (r s =0.283, P=0.049) and serum albumin (r s =0.297, P=0.047). TIM-3 mRNA expression was weakly correlated with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI; r s =-0.272, P=0.032). Our results suggest that below-normal expression of TIM-3 mRNA in PBMC may be involved in the pathogenesis of SLE

  17. Living with Lupus

    Science.gov (United States)

    ... How Lupus Affects the Body Lupus and the Workplace Mental Health and Wellbeing Overlapping Diseases Reproductive Health Self-Advocacy Treatment Options all resource types Videos Articles Downloadable PDFs Slideshow Q & A List Personal Stories ...

  18. Lupus enteritis: from clinical findings to therapeutic management

    Science.gov (United States)

    2013-01-01

    Lupus enteritis is a rare and poorly understood cause of abdominal pain in patients with systemic lupus erythematosus (SLE). In this study, we report a series of 7 new patients with this rare condition who were referred to French tertiary care centers and perform a systematic literature review of SLE cases fulfilling the revised ACR criteria, with evidence for small bowel involvement, excluding those with infectious enteritis. We describe the characteristics of 143 previously published and 7 new cases. Clinical symptoms mostly included abdominal pain (97%), vomiting (42%), diarrhea (32%) and fever (20%). Laboratory features mostly reflected lupus activity: low complement levels (88%), anemia (52%), leukocytopenia or lymphocytopenia (40%) and thrombocytopenia (21%). Median CRP level was 2.0 mg/dL (range 0–8.2 mg/dL). Proteinuria was present in 47% of cases. Imaging studies revealed bowel wall edema (95%), ascites (78%), the characteristic target sign (71%), mesenteric abnormalities (71%) and bowel dilatation (24%). Only 9 patients (6%) had histologically confirmed vasculitis. All patients received corticosteroids as a first-line therapy, with additional immunosuppressants administered either from the initial episode or only in case of relapse (recurrence rate: 25%). Seven percent developed intestinal necrosis or perforation, yielding a mortality rate of 2.7%. Altogether, lupus enteritis is a poorly known cause of abdominal pain in SLE patients, with distinct clinical and therapeutic features. The disease may evolve to intestinal necrosis and perforation if untreated. Adding with this an excellent steroid responsiveness, timely diagnosis becomes primordial for the adequate management of this rare entity. PMID:23642042

  19. The association between systemic lupus erythematosus and valvular heart disease: an extensive data analysis.

    Science.gov (United States)

    Watad, Abdulla; Tiosano, Shmuel; Grysman, Noam; Comaneshter, Doron; Cohen, Arnon D; Shoenfeld, Yehuda; Amital, Howard

    2017-05-01

    Association between antiphospholipid syndrome in systemic lupus erythematosus (SLE) and valvular heart disease (VHD) is well reported, but relatively few studies have been carried out to establish the linkage between VHD and SLE itself. We aimed to investigate link between VHD and SLE and to evaluate the association of diverse factors with VHD among these patients in a large-scale population-based study. We used the databases of the largest state-mandated health service organization in Israel. All SLE patients were included (n = 5018) as well as their age and sex-matched controls (n = 25 090), creating a cross-sectional population-based study. Medical records of all subjects were analysed for documented VHD and the presence of antiphospholipid antibodies (aPLs). A logistic regression model was carried out to evaluate the diverse factors including SLE and aPLs as independent risk factors for VHD. Valvular heart disease were found to be more frequent among SLE group when compared to controls (aortic stenosis, 1·08% vs. 0·35% respectively, P < 0·001; aortic insufficiency, 1·32% vs. 0·29% respectively, P < 0·001; mitral stenosis, 0·74% vs. 0·21% respectively, P < 0·001; mitral insufficiency, 1·91% vs. 0·39% respectively, P < 0·001). Male sex, hypertension, aPLs and SLE were found to be significant independent risk factors for VHD. All VHD are more prevalent among SLE patients when compared to controls. SLE and aPLs are independent risk factor for VHD (OR of 2·46 and 1·7, respectively). Physicians must be aware of such significant association, and routine echocardiography should be considered in SLE patients regardless of their aPL status. © 2017 Stichting European Society for Clinical Investigation Journal Foundation.

  20. Prevalence of systemic lupus erythematosus among patients of hypothyroidism in a tertiary care center

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    Rudrajit Paul

    2012-01-01

    Full Text Available Context: Hypothyroidism is a common public health problem in India. With iodine sufficiency, autoimmune thyroiditis is becoming the most important etiology of hypothyroidism. Often, thyroiditis is associated with other systemic autoimmune diseases. Aims: We undertook thisobservational study to find the prevalence of systemic lupus erythematosus (SLE amongst the hypothyroid patients at our Institution. Settings and Design: This is probably the first study of its kind from India. Materials and Methods: 185 patients with diagnosed hypothyroidism were included and screening for SLE was done by standard epidemiological criteria. Majority of the patients (63.8% were young adults (20-40 years. Statistical Analysis Used: Two by two contingency tables were analyzed by Chi-square test or Fisher′s exact test as needed. Logistic regression model was used considering the presence of SLE as a dependent variable. Results: Eleven (5.94% patients were found to have SLE. However, anti nuclear factor was positive in 145 cases (78.4%. Of the patients with SLE, 8 (72.7% were found to be anti TPO positive, but the titers of ANF and anti TPO did not correlate. Presence of discoid rash, haematological criteria and presence of antibodies like anti-dsDNA were significantly correlated with the presence of SLE in hypothyroid patients. Presence of ANF was also correlated with the grade of goiter (r=0.62; P<0.05. Also four patients with SLE had a positive family history (OR=9.37. Logistic regression model showed anti-TPO has OR=1.54 (P=0.02 for the development of SLE. Conclusions: Prevalence of SLE in hypothyroid patients is high compared to the general population, especially, as thyroiditis is very common in SLE.

  1. Time trend and risk factors of avascular bone necrosis in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Tse, Sau Mei; Mok, Chi Chiu

    2017-06-01

    Objectives The objective of this paper is to study the time trend and risk factors of avascular bone necrosis (AVN) in patients with systemic lupus erythematosus (SLE). Methods Between 1999 and 2014, patients who fulfilled the ACR criteria for SLE and developed symptomatic AVN were identified from our cohort database and compared with those without AVN, matched for age, sex and SLE duration. The standardized incidence ratios (SIRs) of AVN in different SLE age groups were calculated from data derived from our hospital registry and population census. Risk factors for AVN were studied by logistic regression, adjusted by a propensity score for ever use of high-dose glucocorticoids (GCs). Results Fifty-five SLE patients with AVN and 220 SLE patients without AVN were studied. There were 104 AVN sites involved, with the hips being most commonly affected (82%). The point prevalence of AVN in our SLE cohort was 7.4%. The SIRs of AVN in our SLE patients were 131 (86.6-199; p < 0.001) and 56.0 (34.3-91.4; p < 0.001), respectively, in the periods 1995-2004 and 2005-2014. In both decades, the age-stratified SIR was highest in the youngest age group (<19 years). AVN patients were more likely to be treated with GCs and had received a significantly higher cumulative dose of prednisolone since SLE diagnosis (16.5 vs 10.7 grams; p = 0.001). The SLE damage score (excluding AVN) was also significantly higher in AVN than non-AVN patients (2.5 vs 0.4; p < 0.001). Logistic regression revealed that preceding septic arthritis of the involved joint (odds ratio (OR) 17.7 (1.5-205); p = 0.02), cushingoid body habitus (OR 2.4 (1.1-5.2); p = 0.04), LDL cholesterol level (OR 1.4 (1.0-1.9); p = 0.04), maximum daily dose of prednisolone (OR 6.4 (1.2-33.3); p = 0.03) and cumulative dose of prednisolone received in the first six months of the first lupus flare (OR 1.3 (1.0-1.8); p = 0.046) were independently associated with AVN. Conclusions AVN is prevalent in SLE

  2. Clinical characteristics and outcomes of the meningitides in systemic lupus erythematosus.

    Science.gov (United States)

    Baizabal-Carvallo, José Fidel; Delgadillo-Márquez, German; Estañol, Bruno; García-Ramos, Guillermo

    2009-01-01

    The meningitides are rare but well-identified complications in patients with systemic lupus erythematosus (SLE). To determine the clinical characteristics, risk factors, prevalence and outcomes of the meningitides (septic and aseptic) in patients with SLE. From January 1988 to December 2006, we identified patients with SLE and septic or aseptic meningitis. We identified 25 episodes of meningitis in 23 patients with SLE, from a total of 1,411 SLE patients (1.63%); in 15 out of 25 episodes, a microorganism was identified. Mycobacterium tuberculosis, Listeria monocytogenes and Criptococcus neoformans represented the main microorganisms. In 10 episodes, aseptic meningitis was diagnosed. Lymphopenia, steroid use, chronic damage and systemic activity of SLE were frequent in both kinds of meningitis. Although the clinical presentation did not differ significantly, patients with septic meningitis had more residual neurological deficits (p = 0.04). Meningitis was observed in about 1.6% of the patients with SLE; in 40% of the cases, no microorganism could be isolated. A residual neurological deficit was more common in patients with septic meningitis. Copyright (c) 2008 S. Karger AG, Basel.

  3. Hypothalamic-mediated model for systemic lupus erythematosis: relation to hemispheric chemical dominance.

    Science.gov (United States)

    Kurup, Ravi Kumar; Kurup, Parameswara Achutha

    2003-11-01

    The isoprenoid pathway including endogenous digoxin was assessed in systemic lupus erythematosis (SLE). All the patients with SLE were right-handed/left hemispheric dominant by the dichotic listening test. This was also studied for comparison in patients with right hemispheric and left hemispheric dominance. The isoprenoid pathway was upregulated with increased digoxin synthesis in patients with SLE and in those with right hemispheric dominance. In this group of patients (i) the tryptophan catabolites were increased and the tyrosine catabolites reduced, (ii) the dolichol and glycoconjugate levels were elevated, (iii) lysosomal stability was reduced, (iv) ubiquinone levels were low and free radical levels increased, and (v) the membrane cholesterol:phospholipid ratios were increased and membrane glycoconjugates reduced. On the other hand, in patients with left hemispheric dominance the reverse patterns were obtained. The biochemical patterns obtained in SLE is similar to those obtained in left-handed/right hemispheric chemically dominant individuals. But all the patients with SLE were right-handed/left hemispheric dominant by the dichotic listening test. Hemispheric chemical dominance has no correlation with handedness or the dichotic listening test. SLE occurs in right hemispheric chemically dominant individuals, and is a reflection of altered brain function. The role of the isoprenoid pathway in the pathogenesis of SLE and its relation to hemispheric dominance is discussed.

  4. Neuropsychiatric symptoms in systemic lupus erythematosus: impact on quality of life.

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    Monahan, R C; Beaart-van de Voorde, L J J; Steup-Beekman, G M; Magro-Checa, C; Huizinga, T W J; Hoekman, J; Kaptein, A A

    2017-10-01

    Objective Assess quality of life in patients with systemic lupus erythematosus (SLE) presenting with neuropsychiatric symptoms (neuropsychiatric SLE, NPSLE). Methods Quality of life was assessed using the Short-Form 36 item Health Survey (SF-36) in patients visiting the Leiden NPSLE clinic at baseline and at follow-up. SF-36 subscales and summary scores were calculated and compared with quality of life of the general Dutch population and patients with other chronic diseases. Results At baseline, quality of life was assessed in 248 SLE patients, of whom 98 had NPSLE (39.7%). Follow-up data were available for 104 patients (42%), of whom 64 had NPSLE (61.5%). SLE patients presenting neuropsychiatric symptoms showed a significantly reduced quality of life in all subscales of the SF-36. Quality of life at follow-up showed a significant improvement in physical functioning role ( p = 0.001), social functioning ( p = 0.007), vitality ( p = 0.023), mental health ( p = 0.014) and mental component score ( p = 0.042) in patients with neuropsychiatric symptoms not attributed to SLE, but no significant improvement was seen in patients with NPSLE. Conclusion Quality of life is significantly reduced in patients with SLE presenting neuropsychiatric symptoms compared with the general population and patients with other chronic diseases. Quality of life remains considerably impaired at follow-up. Our results illustrate the need for biopsychosocial care in patients with SLE and neuropsychiatric symptoms.

  5. The coexistence of antiphospholipid syndrome and systemic lupus erythematosus in Colombians.

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    Juan-Sebastian Franco

    Full Text Available OBJECTIVES: To examine the prevalence and associated factors related to the coexistence of antiphospholipid syndrome (APS and systemic lupus erythematosus (SLE in a cohort of Colombian patients with SLE, and to discuss the coexistence of APS with other autoimmune diseases (ADs. METHOD: A total of 376 patients with SLE were assessed for the presence of the following: 1 confirmed APS; 2 positivity for antiphospholipid (aPL antibodies without a prior thromboembolic nor obstetric event; and 3 SLE patients without APS nor positivity for aPL antibodies. Comparisons between groups 1 and 3 were evaluated by bivariate and multivariate analysis. RESULTS: Although the prevalence of aPL antibodies was 54%, APS was present in just 9.3% of SLE patients. In our series, besides cardiovascular disease (AOR 3.38, 95% CI 1.11-10.96, p = 0.035, pulmonary involvement (AOR 5.06, 95% CI 1.56-16.74, p = 0.007 and positivity for rheumatoid factor (AOR 4.68, 95%IC 1.63-14.98, p = 0.006 were factors significantly associated with APS-SLE. APS also may coexist with rheumatoid arthritis, Sjögren's syndrome, autoimmune thyroid diseases, systemic sclerosis, systemic vasculitis, dermatopolymyositis, primary biliary cirrhosis and autoimmune hepatitis. CONCLUSIONS: APS is a systemic AD that may coexist with other ADs, the most common being SLE. Awareness of this polyautoimmunity should be addressed promptly to establish strategies for controlling modifiable risk factors in those patients.

  6. Expression of BAFF and BR3 in patients with systemic lupus erythematosus

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    J.H. Duan

    2016-03-01

    Full Text Available The objective of this study was to examine the relationship between the expression of B cell activating factor (BAFF and BAFF receptor in patients with disease activity of systemic lupus erythematosus (SLE. Real-time RT-PCR was used to examine BAFF mRNA expression in peripheral blood monocytes of active and stable SLE patients and healthy controls. The percentage of BAFF receptor 3 (BR3 on B lymphocytes was measured by flow cytometry. Soluble BAFF levels in serum were assayed by ELISA. Microalbumin levels were assayed by an automatic immune analysis machine. BAFF mRNA and soluble BAFF levels were highest in the active SLE group, followed by the stable SLE group, and controls (P<0.01. The percentage of BR3 on B lymphocytes was downregulated in the active SLE group compared with the stable SLE group and controls (P<0.01. BAFF mRNA levels and soluble BAFF levels were higher in patients who were positive for proteinuria than in those who were negative (P<0.01. The percentage of BR3 on B lymphocytes was lower in patients who were positive for proteinuria than in those who were negative (P<0.01. The BAFF/BR3 axis may be over-activated in SLE patients. BAFF and BR3 levels may be useful parameters for evaluating treatment.

  7. Regional cerebral glucose metabolism in systemic lupus erythematosus patients with major depressive disorder.

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    Saito, Tomoyuki; Tamura, Maasa; Chiba, Yuhei; Katsuse, Omi; Suda, Akira; Kamada, Ayuko; Ikura, Takahiro; Abe, Kie; Ogawa, Matsuyoshi; Minegishi, Kaoru; Yoshimi, Ryusuke; Kirino, Yohei; Ihata, Atsushi; Hirayasu, Yoshio

    2017-08-15

    Depression is frequently observed in patients with systemic lupus erythematosus (SLE). Neuropsychiatric SLE (NPSLE) patients often exhibit cerebral hypometabolism, but the association between cerebral metabolism and depression remains unclear. To elucidate the features of cerebral metabolism in SLE patients with depression, we performed brain 18F-fluoro-d-glucose positron emission tomography (FDG-PET) on SLE patients with and without major depressive disorder. We performed brain FDG-PET on 20 SLE subjects (5 male, 15 female). The subjects were divided into two groups: subjects with major depressive disorder (DSLE) and subjects without major depressive disorder (non-DSLE). Cerebral glucose metabolism was analyzed using the three-dimensional stereotactic surface projection (3D-SSP) program. Regional metabolism was evaluated by stereotactic extraction estimation (SEE), in which the whole brain was divided into segments. Every SLE subject exhibited cerebral hypometabolism, in contrast to the normal healthy subjects. Regional analysis revealed a significantly lower ER in the left medial frontal gyrus (p=0.0055) and the right medial frontal gyrus (p=0.0022) in the DSLE group than in the non-DSLE group. Hypometabolism in the medial frontal gyrus may be related to major depressive disorder in SLE. Larger studies are needed to clarify this relationship. Copyright © 2017 Elsevier B.V. All rights reserved.

  8. Functional relevance for associations between genetic variants and systemic lupus erythematosus.

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    Fei-Yan Deng

    Full Text Available Systemic lupus erythematosus (SLE is a serious prototype autoimmune disease characterized by chronic inflammation, auto-antibody production and multi-organ damage. Recent association studies have identified a long list of loci that were associated with SLE with relatively high statistical power. However, most of them only established the statistical associations of genetic markers and SLE at the DNA level without supporting evidence of functional relevance. Here, using publically available datasets, we performed integrative analyses (gene relationship across implicated loci analysis, differential gene expression analysis and functional annotation clustering analysis and combined with expression quantitative trait loci (eQTLs results to dissect functional mechanisms underlying the associations for SLE. We found that 14 SNPs, which were significantly associated with SLE in previous studies, have cis-regulation effects on four eQTL genes (HLA-DQA1, HLA-DQB1, HLA-DQB2, and IRF5 that were also differentially expressed in SLE-related cell groups. The functional evidence, taken together, suggested the functional mechanisms underlying the associations of 14 SNPs and SLE. The study may serve as an example of mining publically available datasets and results in validation of significant disease-association results. Utilization of public data resources for integrative analyses may provide novel insights into the molecular genetic mechanisms underlying human diseases.

  9. Cognitive dysfunction and functional magnetic resonance imaging in systemic lupus erythematosus.

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    Barraclough, M; Elliott, R; McKie, S; Parker, B; Bruce, I N

    2015-10-01

    Cognitive dysfunction is a common aspect of systemic lupus erythematosus (SLE) and is increasingly reported as a problem by patients. In many cases the exact cause is unclear. Limited correlations between specific autoantibodies or structural brain abnormalities and cognitive dysfunction in SLE have been reported. It may be that the most appropriate biomarkers have yet to be found. Functional magnetic resonance imaging (fMRI) is a technique used in many other conditions and provides sensitive measures of brain functionality during cognitive tasks. It is now beginning to be employed in SLE studies. These studies have shown that patients with SLE often perform similarly to healthy controls in terms of behavioural measures on cognitive tasks. However, SLE patients appear to employ compensatory brain mechanisms, such as increased response in fronto-parietal regions, to maintain adequate cognitive performance. As there have been only a few studies using fMRI in SLE to investigate cognitive dysfunction, many questions remain unanswered. Further research could, however, help to identify biomarkers for cognitive dysfunction in SLE. © The Author(s) 2015.

  10. An update on diet and nutritional factors in systemic lupus erythematosus management.

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    Aparicio-Soto, Marina; Sánchez-Hidalgo, Marina; Alarcón-de-la-Lastra, Catalina

    2017-06-01

    Systemic lupus erythematosus (SLE) is a chronic inflammatory and autoimmune disease characterised by multiple organ involvement and a large number of complications. SLE management remains complicated owing to the biological heterogeneity between patients and the lack of safe and specific targeted therapies. There is evidence that dietary factors can contribute to the geoepidemiology of autoimmune diseases such as SLE. Thus, diet therapy could be a promising approach in SLE owing to both its potential prophylactic effects, without the side effects of classical pharmacology, and its contribution to reducing co-morbidities and improving quality of life in patients with SLE. However, the question arises as to whether nutrients could ameliorate or exacerbate SLE and how they could modulate inflammation and immune function at a molecular level. The present review summarises preclinical and clinical experiences to provide the reader with an update of the positive and negative aspects of macro- and micronutrients and other nutritional factors, including dietary phenols, on SLE, focusing on the mechanisms of action involved.

  11. A meta-analysis of the association of STAT4 polymorphism with systemic lupus erythematosus.

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    Yuan, Hui; Feng, Jin-Bao; Pan, Hai-Feng; Qiu, Li-Xin; Li, Lian-Hong; Zhang, Ning; Ye, Dong-Qing

    2010-06-01

    STAT4 has been newly identified as a susceptibility gene for systemic lupus erythematosus (SLE) in recent reports. To more precisely estimate the association between STAT4 polymorphism and SLE risk, a meta-analysis was performed. Studies on the association of STAT4 rs7574865 or rs7601754 with SLE were fully considered and carefully selected using three electronic databases (PubMed, Embase, and Web of Science). A total of 17 comparisons from 8 relevant studies involving 7,381 patients and 11,431 controls were included to analyze the association between STAT4 rs7574865 and SLE risk. The pooled OR for the minor T allele of STAT4 rs7574865 was 1.65 (95% CI 1.56-1.75, P rs7574865 with SLE susceptibility was similar in populations of European or Asian origin, although significant differences in the minor T allele frequencies were observed in the two population controls. As for rs7601754, there were five comparisons from four relevant studies involving 2,498 patients and 4,825 controls in this meta-analysis. The pooled OR for the minor C allele of STAT4 rs7601754 was 0.67 (95% CI 0.59-0.75, P rs7574865 polymorphism as a susceptibility factor for SLE in populations of European and Asian origin. Our results also suggest that STAT4 rs7601754 polymorphism might be associated with SLE risk.

  12. Lupus Foundation of America

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    ... Store Read About Our $3.8M Commitment to Stem Cell Research. Learn More Committed to Advancing Research on Lupus ... person with lupus? Get Answers Latest News & Stories Research News | Nov. 16, 2017 Major Lupus Stem Cell Study Receives Funding $3.8 million committed by ...

  13. Anti-pentraxin 3 auto-antibodies might be protective in lupus nephritis: a large cohort study.

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    Yuan, Mo; Tan, Ying; Pang, Yun; Li, Yong-Zhe; Song, Yan; Yu, Feng; Zhao, Ming-Hui

    2017-11-01

    Anti-pentraxin 3 (PTX3) auto-antibodies were found to be associated with the absence of renal involvement in systemic lupus erythematosus (SLE). This study is to investigate the prevalence of anti-PTX3 auto-antibodies and their clinical significance based on a large Chinese lupus nephritis cohort. One hundred and ninety-six active lupus nephritis patients, 150 SLE patients without clinical renal involvement, and 100 healthy controls were enrolled. Serum anti-PTX3 auto-antibodies and PTX3 levels were screened by enzyme-linked immunosorbent assay (ELISA). The associations between anti-PTX3 auto-antibodies and clinicopathological parameters in lupus nephritis were further analyzed. Anti-PTX3 auto-antibodies were less prevalent in active lupus nephritis patients compared with SLE without renal involvement (19.4% (38/196) versus 40.7% (61/150), p auto-antibodies were negatively correlated with proteinuria in lupus nephritis (r = -.143, p = .047). The levels of proteinuria, serum creatinine, and the prevalence of thrombotic microangiopathy were significantly higher in patients with higher PTX3 levels (≥3.207 ng/ml) and without anti-PTX3 auto-antibodies compared with patients with lower PTX3 levels (auto-antibodies (4.79 (3.39-8.28) versus 3.95 (1.78-7.0), p = .03; 168.84 ± 153.63 versus 101.44 ± 47.36, p = .01; 34.1% (14/41) versus 0% (0/9), p = .04; respectively). Anti-PTX3 auto-antibodies were less prevalent in active lupus nephritis patients compared with SLE without renal involvement and associated with less severe renal damage, especially with the combined evaluation of serum PTX3 levels.

  14. [PAL-1 5G/4G polymorphism in patients with systemic lupus erythematosus].

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    Savov, A; Andonova, S; Tanev, D; Robeva, R; Marincheva, Ts; Tomova, A; Kumanov, Ph; Rashkov, R; Kolarov, Zl

    2014-01-01

    Systemic lupus erythematosus (SLE) is a connective tissue disease affecting predominantly women that has been widely associated with obstetric complications. Inherited thrombophilias are significant risk factors for pregnancy loss, but their role in patients with SLE, and especially in those without concomitant secondary antiphospholipid syndrome (APS) has not been clarified. The aim of the present study was to study PAI-1 5G/4G polymorphism in women with lupus. A total of 103 SLE patients as well as 69 healthy volunteers were genotyped for PAI-1 5G/4G (rs1799889). No significant differences in the PAI-1 5G/4G genotype prevalence between patients and controls were found. After exclusion of the women with secondary APS, the frequency of pregnancies and spontaneous abortions, as well as the number of live births were similar in the studied patients with different PAI-1 genotype (p> 0.05). PAI-1 5G/4G polymorphism was not significantly related to any of the lupus ACR criteria or disease activity (p > 0.05), but it could influence the platelet number in the studied patients (263.52 ± 91.10 [5G/5G genotype] versus 210.12 ± 71.79 [4G/4G genotype], p = 0.023). In conclusion, our results showed that PAI-1 4G/5G polymorphism did not worsen the reproductive outcome in SLE women without secondary APS.

  15. Outcome of pregnancy in patients with inactive systemic lupus erythromatosus and minimal proteinuria

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    Alshohaib Saad

    2009-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a multisystem disease. This study was under-taken to assess the outcome of pregnancies in patients with inactive SLE. We prospectively studied 20 female patients with diagnosis of stable class IV Lupus nephritis followed up at King Abdul Aziz University Hospital, in Jeddah, Saudi Arabia between 1998 and 2008. Before each pregnancy all the patients had their blood pressure, serum creatinine, creatinine clearance, serology for SLE and 24-hour urine protein excretion measured and then repeated at monthly intervals during the pregnancy. Statistical analysis was performed using the Wilcoxon signed-rank test. Despite having negative antinuclear antibody (ANA significant complications were observed during pregnancy. The daily proteinuria during 34-36 weeks′ gestation was significantly higher (P< 0.05 than during 32 weeks. Two patients had abortions one stillbirth and 2 required termination of the pregnancy; one due to severe hypertension, and other due to renal impairment. One patient developed HELLP (hemolysis, elevated liver enzymes, low platelets syndrome. 14 patients had a successful preg-nancy, including 4 requiring a cesarian section. In conclusion, although no clinical evidence of lupus disease activity was demonstrated pre-conception proteinuria significantly increased during pregnancy along with maternal and fetal complications. Pregnant females with diagnosis of SLE need a multidisciplinary care during the pregnancy and post-partum period.

  16. Decreased Daily Melatonin Levels in Women with Systemic Lupus Erythematosus - A Short Report

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    Ralitsa Robeva

    2013-09-01

    Full Text Available Background: The neuroendocrine system is known to influence immunity, but the precise interactions between different hormones and autoimmune disorders remain obscure. Aims: The present study aimed to investigate the role of daily serum melatonin concentrations in the development of systemic lupus erythematosus (SLE in women. Study Design: Case-control study Methods: One-hundred and eleven SLE female patients and 46 healthy women were included in the study. Daily serum melatonin levels were investigated in all participants. Results: SLE patients showed significantly lower daily melatonin levels in comparison to healthy women during the short photoperiod (17.75±7.13 pg/mL [16.05] vs. 21.63±6.60 pg/mL [20.10], p=0.012. Hormone concentrations were inversely related to the SLE activity index (SLEDAI (r= -0.268, p=0.004, but they did not correlate to any particular American College Rheumatology (ACR criterion (p>0.05 for all. Conclusion: Daily melatonin levels were decreased in women with systemic lupus erythematosus and correlated inversely to the activity of the autoimmune disease. Further studies are needed to clarify the importance of the pineal and extrapineal melatonin secretion in patients with systemic lupus erythematosus as well as the interrelations between hormones and autoimmunity.

  17. Burden of illness in systemic lupus erythematosus: results from a UK patient and carer online survey.

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    Kent, T; Davidson, A; Newman, D; Buck, G; D'Cruz, D

    2017-09-01

    Objective The objective of this study was to assess the impact of systemic lupus erythematosus (SLE) on patients and carers. Methods Adults with SLE and carers of SLE patients completed a UK-specific online survey covering many aspects of the disease. Surveys were developed in collaboration with an NHS lupus unit and a lupus patient organization. Results A total of 121 patients and 31 carers completed the surveys. Of the 70% of patients initially misdiagnosed with another condition, 59% received treatment for the misdiagnosis. Fatigue was the most debilitating symptom, experienced daily by 79% of patients. The proportion of patients not reporting flares to healthcare providers varied with flare severity: mild flares (43%), moderate flares (15%) and severe flares (5%). Most patients (89%) reported reduced ability to socialize, and 76% had changed employment; of these, 52% stopped working completely. Over one-half (52%) of carers in paid employment missed time from work, and 55% of carers reported a worsened financial status. Most carers (87%) experienced interference with social activities. Conclusion SLE is commonly misdiagnosed and has a considerable impact on the physical, social and financial status of patients and carers. Increased awareness of the disease among healthcare providers and employers of patients and their carers is needed.

  18. Reduced ADAMTS13 activity is associated with thrombotic risk in systemic lupus erythematosus.

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    Martin-Rodriguez, S; Reverter, J C; Tàssies, D; Espinosa, G; Heras, M; Pino, M; Escolar, G; Diaz-Ricart, M

    2015-10-01

    Severe deficiency of ADAMTS13 activity leads to von Willebrand factor (VWF) ultralarge multimers with high affinity for platelets, causing thrombotic thrombocytopenic purpura. Other pathological conditions with moderate ADAMTS13 activity exhibit a thrombotic risk. We examined the ADAMTS13 activity in systemic lupus erythematosus (SLE) and its value as a thrombotic biomarker. ADAMTS13 activity, VWF antigen and multimeric structure, and vascular cell adhesion molecule 1 (VCAM-1) were measured in plasma samples from 50 SLE patients and 50 healthy donors. Disease activity (systemic lupus erythematosus disease activity index; SLEDAI) and organ damage (systemic lupus international collaborating clinics) scores, thrombotic events, antiphospholipid syndrome (APS) and antiphospholipid antibodies (aPLs) were registered. SLE patients showed decreased ADAMTS13 activity and high VWF levels compared with controls (66 ± 27% vs. 101 ± 8%, P 60%, 60-40% and <40%), comparative analysis showed significant association between ADAMTS13 activity and SLEDAI (P < 0.05), presence of aPLs (P < 0.001), APS (P < 0.01) and thrombotic events (P < 0.01). Reduced ADAMTS13 activity together with increased VWF levels were especially notable in patients with active disease and with aPLs. ADAMTS13 activity, in combination with other laboratory parameters, could constitute a potential prognostic biomarker of thrombotic risk in SLE. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  19. Genetic similarities and differences between discoid and systemic lupus erythematosus patients within the Polish population

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    Katarzyna Skonieczna

    2017-05-01

    Full Text Available Introduction: Many studies have shown that some SNPs might be a risk factor for systemic lupus erythematosus (SLE, but little is known about potential susceptibility loci of the skin types of the disease. Discoid lupus erythematosus (DLE is the most common form of the cutaneous lupus erythematosus. Nevertheless, a genetic contribution to DLE is not fully recognized. Aim: We aimed to analyze three SNPs located in the STAT4 (rs7574865, ITGAM (rs1143679 and TNXB (rs1150754 genes in both DLE and SLE patients from Poland. Material and methods: SNPs were genotyped using real-time polymerase chain reaction (PCR. Statistical significance of the differences between patient and control groups in both allele and genotype frequencies were calculated using two tailed Fisher’s exact test. The correction for multiple testing by the Bonferroni adjustment and odds ratio were also calculated. Results : For the first time, we have shown that the polymorphisms located in the STAT4 (rs7574865, but not in the ITGAM (rs1143679 nor the TNXB (rs1150754 genes, might be associated with the development of DLE within the Polish population. The variation of the three investigated SNPs was found to be associated with SLE in our dataset. Conclusions : The results of our study suggest differences in the molecular background between DLE and SLE within the Polish population.

  20. Genetic similarities and differences between discoid and systemic lupus erythematosus patients within the Polish population.

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    Skonieczna, Katarzyna; Czajkowski, Rafał; Kaszewski, Sebastian; Gawrych, Mariusz; Jakubowska, Aneta; Grzybowski, Tomasz

    2017-06-01

    Many studies have shown that some SNPs might be a risk factor for systemic lupus erythematosus (SLE), but little is known about potential susceptibility loci of the skin types of the disease. Discoid lupus erythematosus (DLE) is the most common form of the cutaneous lupus erythematosus. Nevertheless, a genetic contribution to DLE is not fully recognized. We aimed to analyze three SNPs located in the STAT4 (rs7574865), ITGAM (rs1143679) and TNXB (rs1150754) genes in both DLE and SLE patients from Poland. SNPs were genotyped using real-time polymerase chain reaction (PCR). Statistical significance of the differences between patient and control groups in both allele and genotype frequencies were calculated using two tailed Fisher's exact test. The correction for multiple testing by the Bonferroni adjustment and odds ratio were also calculated. For the first time, we have shown that the polymorphisms located in the STAT4 (rs7574865), but not in the ITGAM (rs1143679) nor the TNXB (rs1150754) genes, might be associated with the development of DLE within the Polish population. The variation of the three investigated SNPs was found to be associated with SLE in our dataset. The results of our study suggest differences in the molecular background between DLE and SLE within the Polish population.