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Sample records for negative systemic lupus

  1. RP105-Negative B Cells in Systemic Lupus Erythematosus

    PubMed Central

    Koarada, Syuichi; Tada, Yoshifumi

    2012-01-01

    Systemic lupus erythematosus (SLE) is a multisystem disease characterized by B cells producing autoantibodies against nuclear proteins and DNA, especially anti-double-strand DNA (dsDNA) antibodies. RP105 (CD180), the toll-like receptor- (TLR-) associated molecule, is expressed on normal B cells. However, RP105-negative B cells increase in peripheral blood from patients with active SLE. RP105 may regulate B-cell activation, and RP105-negative B cells produce autoantibodies and take part in pathophysiology of SLE. It is possible that targeting RP105-negative B cells is one of the treatments of SLE. In this paper, we discuss the RP105 biology and clinical significance in SLE. PMID:21941580

  2. Antinuclear antibody-negative systemic lupus erythematosus in a case with pregnancy.

    PubMed

    Xie, Qibing; Liu, Yi; Yang, Nanping; Yin, Geng

    2012-10-01

    We described a 30-year-old pregnant woman developing antinuclear antibodies (ANA)-negative systemic lupus erythematosus (SLE) accompanied with arthritis, malar rash, lymphopenia, autoimmune hemolytic anemia, pericardial and pleural effusion, proteinuria and seizures. All serum autoantibodies except anti-Ro antibody were negative. An artificially induced abortion was performed to prevent SLE deterioration. Steroid and cyclophosphamide therapy were effective for this patient. During the 2-year follow-up period, her ANA and other SLE-related autoantibodies in serum remained negative. This case suggests that ANA may not be required in the pathogenesis of SLE, even in the case with pregnancy, and ANA-negative SLE may also have a dangerous clinical course.

  3. Hypersensitivity to molybdenum as a possible trigger of ANA-negative systemic lupus erythematosus.

    PubMed Central

    Federmann, M; Morell, B; Graetz, G; Wyss, M; Elsner, P; von Thiessen, R; Wüthrich, B; Grob, D

    1994-01-01

    After implantation of two metal plates a 24 year old woman developed fever of unknown origin and successively more symptoms of an ANA-negative systemic lupus erythematosus (SLE). These symptoms resolved after removal of the plates and recurred during patch testing of the metal components, which showed a reaction to molybdenum. A lymphocyte transformation test indicated a delayed-type hypersensitivity to molybdenum. Subsequent progressive flare ups of SLE appeared without molybdenum reexposure. This is the first report suggesting the existence of a hypersensitivity to molybdenum, which may act as another environmental trigger for SLE. PMID:8037499

  4. Systemic lupus erythematosus.

    PubMed

    Pearce, Lynne

    2016-06-22

    Essential facts Systemic lupus erythematosus (SLE) is a rare, chronic autoimmune disease that causes inflammation in various parts of the body. Its annual incidence is estimated at one in 20,000. Although there is no cure, early diagnosis and appropriate treatment can help to control the symptoms, preventing damage to vital organs. In addition, there are other types of lupus that affect the skin, including discoid lupus erythematosus and subacute cutaneous lupus erythematosus.

  5. Lupus

    MedlinePlus

    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 ...

  6. The influence of clinical manifestations and treatment on satisfaction with life together with positive and negative emotions in systemic lupus erythematosus patients.

    PubMed

    Kulczycka, Lilianna; Sysa-Jędrzejowska, Anna; Robak, Ewa

    2011-01-01

    The aim was to determine the satisfaction with life together with positive and negative emotions in systemic lupus erythematosus (SLE) patients, and correlate them with clinical manifestations of the disease and method of treatment. The study included 83 SLE patients. Satisfaction with life was measured using the Satisfaction with Life Scale. Positive and negative aspects were assessed using the Positive and Negative Affects Schedule. Other data were collected from patients at the time of measurement. Satisfaction with life as well as positive and negative emotions are connected with both the patient clinical condition and mode of therapy. There are correlations of these parameters with the number of medicines used and clinical manifestations of the disease. Systemic lupus erythematosus as a long-lasting and incurable disease has an impact not only on the patient quality of life but also on satisfaction with life and patient emotions. That is why it is very important to measure all these parameters to improve patient compliance.

  7. An anti-nuclear antibody-negative patient with systemic lupus erythematosus (SLE) accompanied with anti-ribosomal P antibody (anti-P).

    PubMed

    Sugisaki, Kota; Takeda, Isao; Kanno, Takashi; Nogai, Syoji; Abe, Kunio; Sakuma, Hideo; Kasukawa, Reiji

    2002-11-01

    We report a case of an anti-nuclear antibody (ANA)-negative patient with systemic lupus erythematosus (SLE) accompanied with anti-phospholipid antibody syndrome (APS) and lupus nephritis (LN). Histological examination of placenta obtained by an artificially-induced abortion revealed multiple thromboses in the placental villi. Histology of biopsied kidney tissue revealed minimal change with deposits of immunoglobulin and complement. Anti-ribosomal P antibodies (anti-P) and lupus anticoagulant (LAC) were positive and anti-double stranded DNA antibody (anti-DNA) showed only a slightly positive titer in her serum. The intensity of proteinuria of the patient was correlated with the anti-P, but not anti-DNA titers.

  8. Neuropsychiatric Systemic Lupus Erythematosus

    PubMed Central

    Popescu, Alexandra; Kao, Amy H

    2011-01-01

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

  9. Negative anti-C1q antibody titers may influence therapeutic decisions and reduce the number of renal biopsies in systemic lupus erythematosus.

    PubMed

    Moura, Carlos Geraldo Guerreiro de; Mangueira, Cristóvão Luis Pitangueira; Cruz, Luzia Arlinda Sampaio; Cruz, Constança Margarida Sampaio

    2011-01-01

    In a cross-sectional study involving 62 patients with systemic lupus erythematosus (SLE), we found that patients with biopsy-proven lupus nephritis (LN) had higher titers of anti-C1q antibodies than active SLE without nephritis patients. Anti-C1q was associated with a negative predictive value of 94.59%, a positive predictive value of 52%, a sensitivity of 86.66% and a specificity of 74.47% for the diagnosis of LN. We conclude that high titers of anti-C1q antibodies are strongly associated with the presence of active LN, and the negative predictive value of this test for diagnosing LN is very high; therefore, it can influence therapeutic decisions and reduce the number of renal biopsies in patients with SLE.

  10. Biomarkers for systemic lupus erythematosus.

    PubMed

    Ahearn, Joseph M; Liu, Chau-Ching; Kao, Amy H; Manzi, Susan

    2012-04-01

    The urgent need for lupus biomarkers was demonstrated in September 2011 during a Workshop sponsored by the Food and Drug Administration: Potential Biomarkers Predictive of Disease Flare. After 2 days of discussion and more than 2 dozen presentations from thought leaders in both industry and academia, it became apparent that highly sought biomarkers to predict lupus flare have not yet been identified. Even short of the elusive biomarker of flare, few biomarkers for systemic lupus erythematosus (SLE) diagnosis, monitoring, and stratification have been validated and employed for making clinical decisions. This lack of reliable, specific biomarkers for SLE hampers proper clinical management of patients with SLE and impedes development of new lupus therapeutics. As such, the intensity of investigation to identify lupus biomarkers is climbing a steep trajectory, lending cautious optimism that a validated panel of biomarkers for lupus diagnosis, monitoring, stratification, and prediction of flare may soon be in hand.

  11. [Systemic lupus erythematosus and weakness].

    PubMed

    Vinagre, Filipe; Santos, Maria José; da Silva, José Canas

    2006-01-01

    We report a case of a 13-year old young girl, with Juvenile Systemic Lupus Erythematosus and recent onset of muscle weakness. Investigations lead to the diagnosis of Myasthenia Gravis. The most important causes of muscle weakness in lupus patients are discussed.

  12. Male only Systemic Lupus

    PubMed Central

    Aggarwal, Rachna; Namjou, Bahram; Li, Shibo; D'Souza, Anil; Tsao, Betty P; Bruner, Ben; James, Judith A.; Scofield, R. Hal

    2010-01-01

    Systemic lupus erythematosus (SLE) is more common among women than men with a ratio of about 10 to 1. We undertook this study to describe familial male SLE within a large cohort of familial SLE. SLE families (two or more patients) were obtained from the Lupus Multiplex Registry and Repository. Genomic DNA and blood samples were obtained using standard methods. Autoantibodies were determined by multiple methods. Medical records were abstracted for SLE clinical data. Fluorescent in situ hybridization (FISH) was performed with X and Y centromere specific probes, and a probe specific for the toll-like receptor 7 gene on the X chromosome. Among 523 SLE families, we found five families in which all the SLE patients were male. FISH found no yaa gene equivalent in these families. SLE-unaffected primary female relatives from the five families with only-male SLE patients had a statistically increased rate of positive ANA compared to SLE-unaffected female relatives in other families. White men with SLE were 5 times more likely to have an offspring with SLE than were White women with SLE but there was no difference in this likelihood among Black men. These data suggest genetic susceptibility factors that act only in men. PMID:20472921

  13. An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy.

    PubMed

    Caltik, Aysun; Demircin, Gülay; Bülbül, Mehmet; Erdogan, Ozlem; Akyüz, Sare G; Arda, Nilüfer

    2013-01-01

    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that may affect any organ of the body. We report here an unusual case of seronegative SLE presented as vasculitis with rash, lower gastrointestinal system bleeding and acute renal failure. The patient was a 13-year-old boy, with abdominal distention, pretibial edema, arthritis and petechia on bilateral ankles. He had deteriorated renal functions (creatinine 1.65 mg/dl), hypoalbuminemia (1.6 g/dl) and hypocomplementemia with nephrotic range proteinuria and hematuria. He developed pleural effusion and peritonitis. Serum ANA, anti dsDNA, p ANCA, c ANCA, anticardiolipin IgM and IgG titers were negative. A renal biopsy was performed which revealed diffuse proliferative glomerulonephritis with full-house staining pattern in immunofluorescent microscopic examination suggesting Class IV Lupus Nephritis. He was administered a total of six courses of monthly intravenous pulse methyl prednisolone, dipyridamole, oral cyclophosphamide followed by azothiopirine and oral prednisolone therapy. The renal functions and serum albumin levels turned normal but peritonitis persisted and disappeared after the third pulse steroid therapy. In conclusion, we presented this patient to remind the possibility of SLE in such seronegative patients with unusual findings in order to avoid the delay in the management of this disease with high mortality and morbidity if not treated. Full-house nephropathy is an important clue especially for the diagnosis of ANA negative SLE.

  14. Systemic lupus erythematosus.

    PubMed

    Shaikh, Maliha F; Jordan, Natasha; D'Cruz, David P

    2017-02-01

    Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease that is highly heterogeneous in its presentation. This can pose significant challenges for physicians responsible for the diagnosis and treatment of such patients. SLE arises from a combination of genetic, epigenetic and environmental factors. Pathologically, the disease is primarily driven by loss of immune tolerance and abnormal B- and T-cell function. Major organ involvement may lead to significant morbidity and mortality. Classification criteria for SLE have been developed largely for research purposes; however, these are also widely used in clinical practice. Antinuclear antibodies are the hallmark serological feature, occurring in over 95% of patients with SLE at some point during their disease. The mainstay of treatment is antimalarial drugs such as hydroxychloroquine, combined with corticosteroids and conventional immunosuppressive drugs. An increasing understanding of pathogenesis has facilitated a move towards the development of targeted biologic therapies, with the introduction of rituximab and belimumab into clinical practice.

  15. MTOR ACTIVATION TRIGGERS IL-4 PRODUCTION AND NECROTIC DEATH OF DOUBLE-NEGATIVE T CELLS IN PATIENTS WITH SYSTEMIC LUPUS ERYHTHEMATOSUS

    PubMed Central

    Lai, Zhi-Wei; Borsuk, Rebecca; Shadakshari, Ashwini; Yu, Jianghong; Dawood, Maha; Garcia, Ricardo; Francis, Lisa; Tily, Hajra; Bartos, Adam; Faraone, Stephen V.; Phillips, Paul; Perl, Andras

    2013-01-01

    The mechanistic target of rapamycin (mTOR) is recognized as a sensor of mitochondrial dysfunction and effector of T-cell lineage development, however, its role in autoimmunity, including systemic lupus erythematosus, remains unclear. Here, we prospectively evaluated mitochondrial dysfunction and mTOR activation in PBL relative to SLE disease activity index (SLEDAI) during 274 visits of 59 patients and 54 matched healthy subjects. Partial least square-discriminant analysis identified 15 of 212 parameters that accounted for 70.2% of the total variance and discriminated lupus and control samples (p<0.0005); increased mitochondrial mass of CD3+/CD4−/CD8− double-negative (DN) T cells (p=1.1×10−22) and FoxP3 depletion in CD4+/CD25+ T cells were top contributors (p=6.7×10−7). Prominent necrosis and mTOR activation were noted in DN T cells during 15 visits characterized by flares (SLEDAI increase ≥4) relative to 61 visits of remission (SLEDAI decrease ≥4). mTOR activation in DN T cells was also noted at pre-flare visits of SLE patients relative to those of stable disease or healthy controls. DN lupus T cells showed increased production of IL-4, which correlated with depletion of CD25+/CD19+B cells. Rapamycin treatment in vivo blocked the IL-4 production and necrosis of DN T cells, increased the expression of FoxP3 in CD25+/CD4+T cells, and expanded CD25+/CD19+ B cells. These results identify mTOR activation to be a trigger of IL-4 production and necrotic death of DN T cells in patients with SLE. PMID:23913957

  16. Systemic lupus erythematosus.

    PubMed

    Kaul, Arvind; Gordon, Caroline; Crow, Mary K; Touma, Zahi; Urowitz, Murray B; van Vollenhoven, Ronald; Ruiz-Irastorza, Guillermo; Hughes, Graham

    2016-06-16

    Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. Women of childbearing age and certain racial groups are typically predisposed to developing the condition. Rare, inherited, single-gene complement deficiencies are strongly associated with SLE, but the disease is inherited in a polygenic manner in most patients. Genetic interactions with environmental factors, particularly UV light exposure, Epstein-Barr virus infection and hormonal factors, might initiate the disease, resulting in immune dysregulation at the level of cytokines, T cells, B cells and macrophages. Diagnosis is primarily clinical and remains challenging because of the heterogeneity of SLE. Classification criteria have aided clinical trials, but, despite this, only one drug (that is, belimumab) has been approved for use in SLE in the past 60 years. The 10-year mortality has improved and toxic adverse effects of older medications such as cyclophosphamide and glucocorticoids have been partially offset by newer drugs such as mycophenolate mofetil and glucocorticoid-sparing regimes. However, further improvements have been hampered by the adverse effects of renal and neuropsychiatric involvement and late diagnosis. Adding to this burden is the increased risk of premature cardiovascular disease in SLE together with the risk of infection made worse by immunosuppressive therapy. Challenges remain with treatment-resistant disease and symptoms such as fatigue. Newer therapies may bring hope of better outcomes, and the refinement to stem cell and genetic techniques might offer a cure in the future.

  17. Lupus anticoagulant: clinical significance in anticardiolipin positive patients with systemic lupus erythematosus.

    PubMed

    McHugh, N J; Moye, D A; James, I E; Sampson, M; Maddison, P J

    1991-08-01

    The significance of anticardiolipin antibodies and the lupus anticoagulant was studied in 58 consecutive patients with systemic lupus erythematosus. On 85 occasions serum IgG and IgM anticardiolipin antibodies were measured by an enzyme linked immunosorbent assay (ELISA), and simultaneous plasma samples tested for lupus anticoagulant activity. The most significant association with clinical events (previous thrombosis or thrombocytopenia occurring in 11/58 patients) was with prolonged tissue thromboplastin inhibition time (TTIT) followed by prolonged kaolin cephalin clotting time (KCCT) then raised IgG anticardiolipin antibody concentrations and dilute Russell's viper venom time. Although IgG anticardiolipin antibodies or KCCT were the most sensitive tests in identifying this group, the TTIT was the most specific (98%). Nine patients were IgG anticardiolipin antibody positive and lupus anticoagulant negative, of whom one had thrombocytopenia but none had thrombosis. The presence of a lupus anticoagulant in anticardiolipin antibody positive patients increases specificity for certain adverse clinical events.

  18. Systemic lupus erythematosus and Klinefelter's syndrome.

    PubMed Central

    French, M A; Hughes, P

    1983-01-01

    A case of Klinefelter's syndrome presenting with systemic lupus erythematosus while receiving androgen replacement therapy is described. The association of systemic lupus erythematosus with Klinefelter's syndrome is discussed, particularly in terms of the effect of sex hormones. PMID:6882046

  19. Systemic lupus erythematosus presenting as fulminant lupus pneumonitis: a rare case report.

    PubMed

    Aggarwal, H K; Jain, D; Mittal, A; Rao, A; Yadav, R K; Jain, P

    2016-06-23

    We report a case of 19 year-old female patient diagnosed as systemic lupus erythematosus (SLE) presented with fever and diffuse cutaneous lesions. During the hospital stay she had acute pneumonia, pleural effusion and respiratory failure, which required intensive care unit (ICU) care and mechanical ventilator support. A fulminant course of the disease, decreased values of complement levels and positive antinuclear antibodies (ANA) in pleural fluid and repeated negative sputum for acid-fast bacillus, blood cultures enabled diagnosis of fulminant lupus pneumonitis. Fulminant lupus pneumonitis is a rare but potentially life threatening complication of SLE. Management requires involvement of multiple specialties and rigorous efforts in reviving the patient.

  20. Systemic lupus erythematosus and Raynaud's phenomenon*

    PubMed Central

    Heimovski, Flavia Emilie; Simioni, Juliana A.; Skare, Thelma Larocca

    2015-01-01

    BACKGROUND Patients with systemic lupus erythematosus seem to belong to different serological and clinical subgroups of the disease. Genetic background can cause the appearance of these subgroups. OBJECTIVE To determine whether Brazilian patients who have systemic lupus erythematosus and Raynaud's phenomenon differ from those who do not. METHODS Retrospective analysis of 373 medical records of systemic lupus erythematosus patients studied for demographic, clinical and serological data. A comparative analysis was performed of individuals with and without RP. RESULTS There was a positive association between Raynaud's phenomenon and age at diagnosis (p=0.02), presence of anti-Sm (p=0.01) antibodies and anti-RNP (p<0.0001). Furthermore, a negative association was found between Raynaud's phenomenon and hemolysis (p=0.01), serositis (p=0.01), glomerulonephritis (p=0.0004) and IgM aCL (p=0.004) antibodies. CONCLUSION Raynaud's phenomenon patients appear to belong to a systemic lupus erythematosus subset with a spectrum of clinical manifestations located in a more benign pole of the disease. PMID:26734864

  1. Systemic Lupus Erythematosus and Pregnancy.

    PubMed

    Lateef, Aisha; Petri, Michelle

    2017-05-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a strong female predilection. Pregnancy remains a commonly encountered but high-risk situation in this setting. Both maternal and fetal mortality and morbidity are still significantly increased despite improvements in outcomes. Maternal morbidity includes higher risk of disease flares, preeclampsia and other pregnancy-related complications. Fetal issues include higher rates of preterm birth, intrauterine growth restriction, and neonatal lupus syndromes. Treatment options during pregnancy are also limited and maternal benefit has to be weighed against fetal risk. A coordinated approach, with close monitoring by a multidisciplinary team, is essential for optimal outcomes.

  2. Childhood systemic lupus erythematosus and neonatal lupus syndrome.

    PubMed

    Sandborg, C I

    1998-09-01

    Systemic lupus erythematosus in children can present with a wide spectrum of disease manifestations. Significant organ system involvement appears to be more severe in children than in adults. Central nervous system disease continues to be difficult to diagnose because of the lack of sensitive and specific diagnostic tests. Renal function is the major determinant of long-term prognosis and management in children with lupus. Identification of patients who are most at risk for progression of renal disease and aggressive treatment, including corticosteroids and immunosuppressive agents, are indicated. Genetic susceptibility studies in lupus reveal multiple contributions from HLA and non-HLA genes. Current concepts regarding apoptosis and DNA-protein complexes and autoreactive T-cell help for anti-DNA antibody production suggest novel directions for therapies. New understandings of the pathogenesis of neonatal lupus syndrome and congenital heart block reveals important information about prospective monitoring and management of mothers and fetuses at risk.

  3. [Systemic lupus erythematosus and pregnancy].

    PubMed

    Diniz-da-Costa, Teresa; Centeno, Mónica; Pinto, Luísa; Marques, Aurora; Mendes-Graça, Luís

    2012-01-01

    Systemic lupus erythematosus is a chronic inflammatory disease, resulting from an auto-immune dysfunction. The etiology of this disease is unknown. It frequently occurs in women of childbearing age. Pregnancy in patients with systemic lupus erythematosus may be associated with several complications (maternal, obstetrical and fetal). The prognosis for both mother and child is better when systemic lupus erythematosus has been quiescent for at least six months before pregnancy. Thus, preconceptional assessment and management is crucial for helping women to achieve a period of disease remission before pregnancy as well as for allowing an adjustment of therapy. Maternal health and fetal development should be closely monitored during pregnancy. These patients should be surveilled by a multidisciplinary team (obstetrician, rheumatologist or internist, nephrologist if necessary and a pediatrician), in a tertiary care hospital. Antiphospholipid syndrome, positivity for anti-SSA/Ro or anti-SSB/LA antibodies, hypertension or renal involvement are associated with an increase of adverse pregnancy outcomes. In this article the authors review the main aspects of Systemic lupus erythematosus (SLE) and pregnancy.

  4. Dyslipidemia in systemic lupus erythematosus.

    PubMed

    Szabó, Melinda Zsuzsanna; Szodoray, Peter; Kiss, Emese

    2017-02-07

    Cardiovascular disease is one of the major causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Accelerated atherosclerosis is related to traditional (age, hypertension, diabetes mellitus, dyslipidemia, obesity, smoking, and positive family history) and non-traditional, disease-related factors. Traditional risk factors are still more prominent in patients with lupus, as both hypertension and hypercholesterinemia were independently associated with premature atherosclerosis in several SLE cohorts. In this work, the authors summarize the epidemiology of dyslipidemia in lupus patients and review the latest results in the pathogenesis of lipid abnormalities. The prevalence of dyslipidemia, with elevations in total cholesterol (TC), low-density lipoprotein (LDL), triglyceride (TG), and apolipoprotein B (ApoB), and a reduction in low-density lipoprotein (LDL) levels are about 30% at the diagnosis of SLE rising to 60% after 3 years. Multiple pathogenetic mechanism is included, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) can suppress HDL and increase TG, auto-antibodies can cause the injury of the endothelium, lipoprotein lipase (LPL) activity can be reduced by circulating inflammatory mediators and antibodies, and increased oxidative stress may trigger a wide range of pro-atherogenic lipid modifications. As a major risk factor, dyslipidemia should be treated aggressively to minimize the risk of atherosclerosis and cardiovascular events. Randomized controlled trials with statins are controversial in the detention of atherosclerosis progression, but can be favorable by inhibiting immune activation that is the arterial wall and by decreasing lupus activity.

  5. 75 FR 35492 - Guidance for Industry on Lupus Nephritis Caused By Systemic Lupus Erythematosus-Developing...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-06-22

    ... availability of a guidance for industry entitled ``Lupus Nephritis Caused By Systemic Lupus Erythematosus... nephritis (LN) caused by systemic lupus erythematosus (SLE). This guidance finalizes the parts of the draft guidance entitled ``Systemic Lupus Erythematosus--Developing Drugs for Treatment'' (the draft...

  6. [Systemic lupus erythematosus during pregnancy].

    PubMed

    Christensen, H

    1996-01-22

    Systemic lupus erythematosus (SLE) is a connective tissue disease most commonly affecting women of a fertile age. It is recommended that pregnancy is planned during periods with remission or low activity of he disease because it seems that SLE is more likely to be exacerbated by pregnancy when the disease is active at the time of conception. Presence of circulating anti-phospholipid antibodies is associated with a poor prognostic outcome for the foetus and increases the risk of complications related to pregnancy.

  7. Manifestations of Systemic Lupus Erythematosus

    PubMed Central

    COJOCARU, Manole; COJOCARU, Inimioara Mihaela; SILOSI, Isabela; VRABIE, Camelia Doina

    2011-01-01

    ABSTRACT Systemic lupus erythematosus (SLE) is a chronic, multifaceted autoimmune inflammatory disease that can affect any part of the body. SLE is a disease of unknown aetiology with a variety of presenting features and manifestations. Interest in the disease has been stimulated in recent years, and improved methods of diagnosis have resulted in a significant increase in the number of cases recognized. It is apparent that it can no longer be regarded as a rare disease. The majority of the pathology in SLE is related to deposits of immune complexes in various organs, which triggers complement and other mediators of inflammation. Symptoms vary from person to person, and may come and go, depend on what part of the body is affected, can be mild, moderate, or severe. Diagnosis can be difficult because lupus mimics many other diseases; it requires clinical and serologic criteria. PMID:22879850

  8. 77 FR 38305 - Guidance for Industry on Lupus Nephritis Caused by Systemic Lupus Erythematosus-Developing...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-06-27

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

  9. [Management of systemic lupus erythematosus].

    PubMed

    Aringer, M; Schneider, M

    2016-11-01

    In the last few decades a number of small, often largely unrecognized steps have fundamentally changed the management of systemic lupus erythematosus (SLE). The current goal is to stop all disease activity without long-term use of more than 5 mg prednisolone per day. Remission, i.e. absence of activity in the SLE activity score of choice, is the defined target in the treat to target approach. The essential basic measures include life-long hydroxychloroquine as well as protection from sunlight (UV) and vitamin D substitution. Patients suffering from SLE need more vaccinations than the healthy population and control of risk factors for atherosclerosis is critical for long-term survival. Methotrexate is on par with azathioprine. If disease activity cannot be controlled in this way, belimumab is an approved therapeutic option. Cyclophosphamide is still used but only in life-threatening situations, such as lupus nephritis or central nervous system (CNS) vasculitis and in drastically reduced doses. Alternatively, off-label mycophenolate mofetil (MMF) can be used particularly for lupus nephritis and off-label rituximab in refractory disease courses. Numerous novel approaches are being tested in controlled trials and it is hoped that new drugs will be available for SLE patients within a few years.

  10. Infections and systemic lupus erythematosus

    PubMed Central

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

    2016-01-01

    ABSTRACT Objective To determine the incidence of infections in a population of systemic lupus erythematosus individuals and the characteristics of infections regarding original site, as well as to study the possible associations between infections and treatment. Methods An analytical retrospective study using data from medical charts of systemic lupus erythematosus patients from a single university hospital. A total of 144 patients followed up for five years were included. Data collected comprised age of patients and age at onset of lupus, sex and ethnicity, disease duration before the study period, medications, cumulative dose of prednisone, occurrence of infections and their original site. Results The most frequent infections were urinary tract infections (correlated to use of prednisone − p<0.0001 and cyclophosphamide − p=0.045), upper airways infections (correlated to use of prednisone − p=0.0004, mycophenolate mofetil − p=0.0005, and cyclosporine − p=0.025), and pneumonia (associated to prednisone − p=0.017). Conclusion Prednisone was the drug more often associated with presence of infections, pointing to the need for a more judicious management of this drug. PMID:27074234

  11. Lupus

    MedlinePlus

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Lupus KidsHealth > For Teens > Lupus Print A A A ... dad that she might have lupus. What Is Lupus? Lupus (pronounced: LOO-pus) is a disease that ...

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

    PubMed

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

    2011-03-01

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

  13. [Ballism in systemic lupus erythematosus].

    PubMed

    Havsager, A M; Carstensen, N C

    1991-08-12

    A case of systemic lupus erythematosus (SLE) with ballism is presented. The movement disorders, as a group, account for approximately 2% of the neurological and psychiatric symptoms of central nervous system (CNS) involvement in SLE, and more than any of the other neuro-psychiatric manifestations they tend to precede the diagnosis of SLE. Ballism and other rare movement disorders are not included in the 1982 revised American Rheumatism Association criteria for SLE. Being aware of the correlation between CNS disease and more severe SLE, one should assess therapeutic intervention critically.

  14. Acute transverse myelopathy complicating systemic lupus erythematosus.

    PubMed Central

    Propper, D J; Bucknall, R C

    1989-01-01

    A sixteen year old girl with systemic lupus erythematosus developed acute transverse myelopathy. She was treated with high dose steroids, cyclophosphamide, and plasma exchange and regained partial neurological function. Previous descriptions of transverse myelopathy complicating systemic lupus erythematosus are reviewed, with particular reference to the efficacy of high dose steroid treatment. PMID:2662918

  15. Belimumab in Systemic Lupus Erythematosus

    PubMed Central

    Srivastava, Ankita

    2016-01-01

    Belimumab is the only approved biological agent for the treatment of systemic lupus erythematosus (SLE). It is a fully humanized IgG1γ monoclonal antibody directed against soluble B lymphocyte stimulator (BLyS). It is indicated as an add-on therapy for the treatment of adult patients with active, autoantibody-positive SLE, who are receiving standard therapy. Belimumab is generally well-tolerated, common adverse effects include infections, infusion reactions, hypersensitivity, headache, nausea, and fatigue. Psychiatric events including suicidal tendency, progressive multifocal leukoencephalopathy and malignancies too have been reported. Apart from SLE, the drug is also being tried for other autoimmune disorders. PMID:27688447

  16. [Systemic lupus erythematosus and pregnancy].

    PubMed

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

    2016-08-01

    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, and current research initiatives aim towards further improvement. Pregnant women with SLE are still considered being at various levels of risk. In order to achieve the best possible outcomes for mother and child, joint care in specialised multidisciplinary teams including rheumatologists and obstetricians is recommended.

  17. Systemic lupus erythematosus and exercise.

    PubMed

    Ayán, C; Martín, V

    2007-01-01

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

  18. Breastfeeding in mothers with systemic lupus erythematosus.

    PubMed

    Noviani, M; Wasserman, S; Clowse, M E B

    2016-08-01

    Breastfeeding is known to improve the well-being of a mother and her infant, and about half of all new mothers breastfeed, but it is unknown how breastfeeding is pursued in systemic lupus erythematosus (SLE; lupus) patients. We sought to determine the rate of breastfeeding and the factors influencing this among women with lupus. In addition, we reassessed the current safety data in lactation of lupus medications. Data were collected from lupus patients enrolled in a prospective registry who fulfilled the 2012 SLICC criteria, had a live birth, and for whom postpartum breastfeeding status was known. Data included physician assessments of lupus activity and medications, breastfeeding intentions during pregnancy and practice following pregnancy. The safety of medications in breastfed infants was assessed through a comprehensive review of LactMed, a national database about medications in lactation. A total of 51 pregnancies in 84 women with lupus were included in the study. Half of the lupus patients (n = 25, 49%) chose to breastfeed. The rate of breastfeeding was not significantly affected by socioeconomic factors. In contrast, low postpartum lupus activity, term delivery, and a plan to breastfeed early in pregnancy were significantly associated with breastfeeding in lupus patients. In reviewing the most up-to-date data, the majority of lupus medications appear to have very minimal transfer into breast milk and are likely compatible with breastfeeding. Half of women with lupus breastfed and most desire to breastfeed. Hydroxychloroquine, azathioprine, methotrexate, and prednisone have very limited transfer into breast milk and may be continued while breastfeeding. © The Author(s) 2016.

  19. Large artery inflammation in systemic lupus erythematosus.

    PubMed

    Sokalski, D G; Copsey Spring, T R; Roberts, W N

    2013-08-01

    A 23-year-old African-American woman with a history of recurrent pneumonias presented to the hospital with 2 weeks of shortness of breath, chest pain, fevers, and lightheadedness. The histologic diagnosis proved to be lupus aortitis. Optimal Framingham risk factor management by itself may not be a completely successful approach in diminishing the extra risk of atherosclerosis conferred by systemic lupus erythematosus (SLE). Therefore it remains possible that important modifiable cardiovascular risk factors may include low-grade SLE disease activity in medium-sized vessels. The implication of the idea that subclinical vessel inflammation is widespread in patients with lupus-and that this inflammation confers a significant part of the patients' risk of accelerated atherosclerosis-might be a lowering of the threshold for aggressive disease-modifying treatment of lupus, essentially a "treat-to-target" approach to systemic lupus.

  20. Gastrointestinal system involvement in systemic lupus erythematosus.

    PubMed

    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.

  1. Childhood-onset bullous systemic lupus erythematosus.

    PubMed

    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.

  2. IFN-α Negatively Regulates the Expression of Endothelial Nitric Oxide Synthase and Nitric Oxide Production: Implications for Systemic Lupus Erythematosus.

    PubMed

    Buie, Joy Jones; Renaud, Ludivine L; Muise-Helmericks, Robin; Oates, Jim C

    2017-09-15

    Systemic lupus erythematosus (SLE) is a known risk factor for endothelial dysfunction. Murine and human lupus studies revealed a role for IFN-α in vascular abnormalities associated with impaired blood vessel dilation. However, the impact of IFN-α on mediators that induce vasodilation and modulate inflammation, including endothelial NO synthase (eNOS) and NO bioavailability, are unknown. The objectives of this study were to determine how IFN-α promotes endothelial dysfunction in SLE, focusing on its regulation of eNOS and NO production in endothelial cells. We demonstrate that IFN-α promotes an endothelial dysfunction signature in HUVECs that is characterized by transcription suppression and mRNA instability of eNOS complemented by upregulation of MCP1 and VCAM1 These changes are associated with IFN-inducible gene expression. IFN-α impairs insulin-mediated NO production, and altered gene expression resulted from eNOS instability, possibly due to enhanced miR-155 expression. IFN-α significantly impaired NO production in insulin-stimulated HUVECs. IFN-α treatment also led to enhanced neutrophil adhesion. Our study introduces a novel pathway by which IFN-α serves as a proatherogenic mediator through repression of eNOS-dependent pathways. This could promote the development of endothelial dysfunction and cardiovascular disease in SLE. Copyright © 2017 by The American Association of Immunologists, Inc.

  3. Systemic lupus erythematosus: an update.

    PubMed

    Golder, Vera; Hoi, Alberta

    2017-03-20

    Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease predominantly affecting women of childbearing age. New classification criteria for SLE have greater sensitivity and therefore improve the diagnostic certainty for some patients, especially those who may previously have been labelled as having undifferentiated symptoms. Uncontrolled disease activity leads to irreversible end-organ damage, which in turn increases the risk of premature death; early and sustained control of disease activity can usually be achieved by conventional immunosuppressant therapy. The development of biological therapy lags behind that for other rheumatic diseases, with belimumab being the only targeted therapy approved by the Therapeutic Goods Administration. "Treat-to-target" concepts are changing trial design and clinical practice, with evidence-based definition of response criteria in the form of remission and low disease activity now on the horizon. While new therapies are awaited, research should also focus on optimising the use of current therapy and improving the quality of care of patients with SLE.

  4. Humor in systemic lupus erythematosus.

    PubMed

    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.

  5. [Cerebral infarction in systemic lupus erythematosus].

    PubMed

    Overbeck, S; Wermuth, L

    1989-02-13

    The case-history of a man aged 31 years with systemic lupus erythematosus and cerebral infarction is presented. Although patients with active disease are young, cerebral infarcts are strikingly frequent among them.

  6. Mucormycosis complications in systemic lupus erythematosus.

    PubMed

    Arce-Salinas, C A; Pérez-Silva, E

    2010-07-01

    This case involved a 75-year-old woman with systemic lupus erythematosus. Two months previously, she had a flare that was treated successfully by increasing the dosages of prednisone and azathioprine. A sudden onset of ocular pain, diplopia, and loss of vision suggestive of optical neuritis or vascular involvement confused the issue, and rhinocerebral zygomycosis was demonstrated later. We review the presentations of this fungal infection in patients with systemic lupus erythematosus with emphasis on its initial features.

  7. Renal biopsy in patients with systemic lupus erythematosus: Not just lupus glomerulonephritis!

    PubMed

    Howell, David N

    2017-01-01

    Kidney biopsy is a mainstay in the diagnosis and management of renal disease in patients with systemic lupus erythematosus. Though biopsies from patients with lupus typically show various forms of immune complex glomerulonephritis, other pathologies are occasionally encountered, including unusual lupus-related nephropathies, other forms of autoimmune disease, and occasional renal disorders without any direct connection with lupus or autoimmunity. Electron microscopy is a powerful tool for detecting and classifying these unusual conditions, which frequently have important therapeutic and prognostic implications.

  8. Total lymphoid irradiation in refractory systemic lupus erythematosus

    SciTech Connect

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

    1986-07-01

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

  9. Periodontitis and systemic lupus erythematosus.

    PubMed

    Sete, Manuela Rubim Camara; Figueredo, Carlos Marcelo da Silva; Sztajnbok, Flavio

    2016-01-01

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

  10. Osteonecrosis in systemic lupus erythematosus.

    PubMed

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

    2015-01-01

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

  11. Central nervous system manifestations of neonatal lupus: a systematic review.

    PubMed

    Chen, C C; Lin, K-L; Chen, C-L; Wong, A May-Kuen; Huang, J-L

    2013-12-01

    Neonatal lupus is a rare and acquired autoimmune disease. Central nervous system abnormalities are potential manifestations in neonatal lupus. Through a systematic literature review, we analyzed the clinical features of previously reported neonatal lupus cases where central nervous system abnormalities had been identified. Most reported neonatal lupus patients with central nervous system involvement were neuroimaging-determined and asymptomatic. Only seven neonatal lupus cases were identified as having a symptomatic central nervous system abnormality which caused physical disability or required neurosurgery. A high percentage of these neurosymptomatic neonatal lupus patients had experienced a transient cutaneous skin rash and had no maternal history of autoimmune disease before pregnancy.

  12. Breast cancer in systemic lupus.

    PubMed

    Bernatsky, S; Ramsey-Goldman, R; Petri, M; Urowitz, M B; Gladman, D D; Fortin, P F; Ginzler, E; Romero-Diaz, J; Peschken, C; Jacobsen, S; Hanly, J G; Gordon, C; Nived, O; Yelin, E H; Isenberg, D; Rahman, A; Bae, S-C; Joseph, L; Witte, T; Ruiz-Irastorza, G; Aranow, C; Kamen, D; Sturfeldt, G; Foulkes, W D; Hansen, J E; St Pierre, Y; Raymer, P Chrétien; Tessier-Cloutier, B; Clarke, A E

    2017-03-01

    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 exposures over time. In univariate and multivariate models, the principal factor associated with breast cancers was older age at cohort entry. Conclusions There was little evidence that breast cancer risk in this SLE sample was strongly driven by any of the clinical factors that we studied. Further search for factors that determine the lower risk of breast cancer in SLE may be warranted.

  13. [Systemic lupus erythematosus and pregnancy].

    PubMed

    Schwarz, P; Halberg, P

    1989-04-24

    The literature about pregnancies in patients with systemic lupus erythematosus (SLE) was reviewed. Information about 1,164 pregnancies was compiled. Fifty-four per cent of the pregnancies were normal, 15 per cent of the babies were premature, and 29 per cent of the pregnancies resulted in fetal wastage. In a background population of 50,000 women the corresponding figures were 80, 4 and 16 per cent, respectively. The neonatal mortality of babies borne by women with SLE was 2 per cent. About one third of the patients had an exacerbation of their disease, mostly post-partum or during the last trimester of the pregnancy. The risk of a exacerbation for patients with active disease at the time of conception was double that of patients with inactive disease. Therapeutic abortions were followed by improvement as well as deterioration of the disease. Two per cent of the patients died during pregnancy or post-partum. Cardiolipin-antibody may be a marker of fetal wastage and Ro-antibody may be a marker of fetal heart block in babies borne by women with SLE. Pregnancy was previously considered inadvisable in all patients with SLE. During recent years, the attitude to this problem has been less rigid. However the patients should be fully informed about the risk of exacerbation, fetal wastage, and premature delivery and patients with active disease should be advised to postpone pregnancy until a remission has been achieved.

  14. [Systemic lupus erythematosus and pregnancy].

    PubMed

    Basheva, S; Nikolov, A; Stoilov, R; Stoilov, N

    2012-01-01

    Connective-tissue disorders, also referred to as collagen-vascular disorders, are characterized by autoantibody-mediated connective-tissue abnormalities. These are also called immune-complex diseases because many involve deposition of immune complexes in specific organ or tissue sites. Some of these disorders are characterized by sterile inflammation, especially of the skin, joints, blood vessels, and kidneys, and are referred to as rheumatic diseases. For inexplicable reasons, many rheumatic diseases primarily affect women. Another major category of connective-tissue diseases includes inherited disorders of bone, skin, cartilage, blood vessels. Examples include Marfan syndrome, osteogenesis imperfecta, and Ehlers-Danlos syndrome. Lupus erythematosus (LE) is the main and most important disease in the group of systemic connective tissue diseases. It is heterogeneous, multiple organs autoimmune inflammatory disease with complex pathogenesis, which is the result of interaction between the susceptible genes and environmental factors that lead to abnormal immune response. In this review will consider: its incidence, pathogenesis, clinical forms and clinical features and diagnosis set based on generally accepted clinical criteria developed by the American College of Rheumatology (ACR), the course of pregnancy in patients suffering from LE, the most common complications of LE during pregnancy and antiphospholipid syndrome as part of LE.

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

    PubMed

    Chanprapaph, K; Sawatwarakul, S; Vachiramon, V

    2017-01-01

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

  16. Recurrent podocytopathy in a patient with systemic lupus erythematosus

    PubMed Central

    Paramalingam, Shereen; Wong, Daniel D; Dogra, Gursharan K; Nossent, Johannes C

    2017-01-01

    Podocytopathy in systemic lupus erythematosus is characterised by diffuse foot process effacement without significant peripheral capillary wall immune deposits as seen on electron microscopy. Lupus podocytopathy falls outside the scope of the current International Society of Nephrology and the Renal Pathology Society classification of lupus nephritis. We present a case of relapsing podocytopathy with nephrotic syndrome occurring simultaneously with two extra-renal and serological disease flares, which makes it likely that podocytopathy was related to systemic lupus erythematosus activity. This case adds to the growing body of evidence that lupus podocytopathy must be considered in the differential diagnosis of systemic lupus erythematosus patients presenting with nephrotic syndrome. PMID:28321309

  17. Recurrent podocytopathy in a patient with systemic lupus erythematosus.

    PubMed

    Paramalingam, Shereen; Wong, Daniel D; Dogra, Gursharan K; Nossent, Johannes C

    2017-01-01

    Podocytopathy in systemic lupus erythematosus is characterised by diffuse foot process effacement without significant peripheral capillary wall immune deposits as seen on electron microscopy. Lupus podocytopathy falls outside the scope of the current International Society of Nephrology and the Renal Pathology Society classification of lupus nephritis. We present a case of relapsing podocytopathy with nephrotic syndrome occurring simultaneously with two extra-renal and serological disease flares, which makes it likely that podocytopathy was related to systemic lupus erythematosus activity. This case adds to the growing body of evidence that lupus podocytopathy must be considered in the differential diagnosis of systemic lupus erythematosus patients presenting with nephrotic syndrome.

  18. Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome.

    PubMed

    Murtaza, Ghulam; Iskandar, Joy; Humphrey, Tara; Adhikari, Sujeen; Kuruvilla, Aneesh

    2017-04-01

    Libman-Sacks endocarditis is characterized by sterile and verrucous lesions that predominantly affect the aortic and mitral valves. In most cases, patients do not have significant valvular dysfunction. However, patients with significant valvular dysfunction may present with serious complications such as cardiac failure, arrhythmias, and thromboembolic events. Recently, association of Libman-Sacks endocarditis with antiphospholipid antibody syndrome (APS) has been made. APS is most commonly defined by venous and arterial thrombosis, recurrent pregnancy loss, and thrombocytopenia. While the syndrome can be a primary syndrome, it is usually secondary to systemic lupus erythematosus. Catastrophic antiphospholipid syndrome (CAPS) can be a life-threatening presentation of APS and can occur in 1% of patients with antiphospholipid syndrome. We present a very rare case of a young female patient with lupus-negative Libman-Sacks endocarditis complicated by CAPS.

  19. The lupus band test in systemic lupus erythematosus patients

    PubMed Central

    Reich, Adam; Marcinow, Katarzyna; Bialynicki-Birula, Rafal

    2011-01-01

    The lupus band test (LBT) is a diagnostic procedure that is used to detect deposits of immunoglobulins and complement components along the dermoepidermal junction in patients with lupus erythematosus (LE). The LBT is positive in about 70%–80% of sun-exposed non-lesional skin specimens obtained from patients with systemic LE (SLE), and in about 55% of SLE cases if sun-protected nonlesional skin is analyzed. In patients with cutaneous LE only, the lesional skin usually shows a positive LBT. The LBT helps in differentiating LE from other similar skin conditions and may also be helpful in making the diagnosis of SLE in subjects with no specific cutaneous lesions. Furthermore, a positive LBT may be applied as a prognostic parameter for LE patients. However, the correct interpretation of this test requires detailed knowledge of the site of the biopsy, deposit components, morphology and brightness of the immunofluorescent band, and other associated serologic findings, as well as the response to treatment. It must be emphasized that LBT is a laboratory procedure that should always be interpreted in conjunction with clinical findings and other serological and immunopathological parameters. PMID:21339940

  20. Lupus Nephritis Susceptibility Loci in Women with Systemic Lupus Erythematosus

    PubMed Central

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

    2014-01-01

    Lupus nephritis is a manifestation of SLE resulting from glomerular immune complex deposition and inflammation. Lupus nephritis demonstrates familial aggregation and accounts for significant morbidity and mortality. We completed a meta-analysis of three genome-wide association studies of SLE to identify lupus nephritis–predisposing loci. Through genotyping and imputation, >1.6 million markers were assessed in 2000 unrelated women of European descent with SLE (588 patients with lupus nephritis and 1412 patients with lupus without nephritis). Tests of association were computed using logistic regression adjusting for population substructure. The strongest evidence for association was observed outside the MHC and included markers localized to 4q11-q13 (PDGFRA, GSX2; P=4.5×10−7), 16p12 (SLC5A11; P=5.1×10−7), 6p22 (ID4; P=7.4×10−7), and 8q24.12 (HAS2, SNTB1; P=1.1×10−6). Both HLA-DR2 and HLA-DR3, two well established lupus susceptibility loci, showed evidence of association with lupus nephritis (P=0.06 and P=3.7×10−5, respectively). Within the class I region, rs9263871 (C6orf15-HCG22) had the strongest evidence of association with lupus nephritis independent of HLA-DR2 and HLA-DR3 (P=8.5×10−6). Consistent with a functional role in lupus nephritis, intra-renal mRNA levels of PDGFRA and associated pathway members showed significant enrichment in patients with lupus nephritis (n=32) compared with controls (n=15). Results from this large-scale genome-wide investigation of lupus nephritis provide evidence of multiple biologically relevant lupus nephritis susceptibility loci. PMID:24925725

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

    PubMed

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

    2014-12-01

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

  2. Pyomyositis in childhood-systemic lupus erythematosus.

    PubMed

    Blay, Gabriela; Ferriani, Mariana P L; Buscatti, Izabel M; França, Camila M P; Campos, Lucia M A; Silva, Clovis A

    2016-01-01

    Pyomyositis is a pyogenic infection of skeletal muscle that arises from hematogenous spread and usually presents with localized abscess. This muscle infection has been rarely reported in adult-onset systemic lupus erythematous and, to the best of our knowledge, has not been diagnosed in pediatric lupus population. Among our childhood-onset systemic lupus erythematous population, including 289 patients, one presented pyomyositis. This patient was diagnosed with childhood-onset systemic lupus erythematous at the age of 10 years-old. After six years, while being treated with prednisone, azathioprine and hydroxychloroquine, she was hospitalized due to a 30-day history of insidious pain in the left thigh and no apparent trauma or fever were reported. Her physical examination showed muscle tenderness and woody induration. Laboratory tests revealed anemia, increased acute phase reactants and normal muscle enzymes. Computer tomography of the left thigh showed collection on the middle third of the vastus intermedius, suggesting purulent stage of pyomyositis. Treatment with broad-spectrum antibiotic was initiated, leading to a complete clinical resolution. In conclusion, we described the first case of pyomyositis during childhood in pediatric lupus population. This report reinforces that the presence of localized muscle pain in immunocompromised patients, even without elevation of muscle enzymes, should raise the suspicion of pyomyositis. A prompt antibiotic therapy is strongly recommended.

  3. Intravenous immunoglobulin therapy and systemic lupus erythematosus.

    PubMed

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

    2005-12-01

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

  4. Spontaneous ureteral rupture in a patient with systemic lupus erythematosus

    SciTech Connect

    Benson, C.H.; Pennebaker, J.B.; Harisdangkul, V.; Songcharoen, S.

    1983-08-01

    A patient with known systemic lupus erythematosus had fever and symptoms of a lower urinary tract infection. Bone scintigraphy showed left ureteral perforation and necrosis with no demonstrable nephrolithiasis. It is speculated that this episode was due to lupus vasculitis.

  5. Lupus - resources

    MedlinePlus

    Resources - lupus ... The following organizations are good resources for information on systemic lupus erythematosus : The Lupus Foundation of America -- www.lupus.org The National Institute of Arthritis and Musculoskeletal ...

  6. Validation of the LupusPRO in Chinese patients from Hong Kong with systemic lupus erythematosus.

    PubMed

    Mok, Chi Chiu; Kosinski, Mark; Ho, Ling Yin; Chan, Kar Li; Jolly, Meenakshi

    2015-02-01

    LupusPRO is a disease-targeted, patient-reported outcome (PRO) measure that was developed and validated for assessment of quality of life in US patients with systemic lupus erythematosus (SLE). We present results of adapting the LupusPRO into Chinese and testing its psychometric properties in Chinese patients with SLE. LupusPRO was translated into "traditional" Chinese, followed by pretesting among native Cantonese Chinese speakers. The translation version was revised based on the feedback obtained. The Chinese language LupusPRO tool was administered along with a generic PRO tool (the Short Form 36 health survey [SF-36]) to ethnic Chinese SLE patients. At the same time, demographic information, clinical data, disease activity (Safety of Estrogens in Lupus Erythematosus National Assessment [SELENA] version of the Systemic Lupus Erythematosus Disease Activity Index [SLEDAI]), and damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI]) were obtained. We performed confirmatory factor analysis of the Chinese LupusPRO and evaluated internal consistency reliability, as well as convergent and criterion validity. Among the 463 SLE patients (95% women) with a mean ± SD age of 42.3 ± 13.5 years, the mean ± SD physician global assessment score was 0.48 ± 0.45, the mean ± SD SELENA-SLEDAI score was 2.9 ± 3.0, and the mean ± SD SDI score was 0.7 ± 1.2. Results of factor analysis conformed to the original LupusPRO model with only minor modifications. The reliability of the LupusPRO domains ranged from 0.60-0.94. LupusPRO domains had correlations as expected with the corresponding SF-36 domains. A significant but weak correlation with disease activity was noted for criterion validity as expected. The Chinese language LupusPRO has fair psychometric properties and may be used in SLE clinical trials. Copyright © 2015 by the American College of Rheumatology.

  7. [Pregnancy and systemic lupus erythematosus: compatible?].

    PubMed

    Jason, M; von Frenckell, C; Emonts, P

    2012-11-01

    Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that predominantly occurs in women of childbearing age. The risk of obstetric complications in lupus parturients is significant. In addition, pregnancy may be associated with flares of the disease requiring immunosuppressive therapy. For these reasons, SLE pregnancies are considered high risk and involve careful collaboration of the obstetrician and rheumatologist. Through the latter and medical advances including a better and better understanding of the binomial mother-child, most pregnancies end in a success.

  8. [Serious bleeding in systemic lupus erythematosus complicated by lupus anticoagulant-hypoprothrombinaemia syndrome].

    PubMed

    Engelsen, Jytte; Nielsen, Susan M; Thorsen, Sixtus

    2006-01-30

    Severe hemorrhagic diathesis due to lupus anticoagulant complicated by hypoprothrombinaemia resulting from prothrombin autoantibodies is a rare disorder and is often associated with systemic lupus erythematosus (SLE). We report a case in which a 15-year-old girl with SLE developed marked haemorrhagic manifestations due to menorrhagia and nosebleeds. The acute bleeding episode was treated with SAGM, tranexamic acid and recombinant factor VIIa. Lupus anticoagulant, cardiolipin antibodies and antiprothrombin antibodies were successfully depressed within weeks after corticosteroid therapy was begun.

  9. Lupus vulgaris in a patient with systemic lupus erythematosus and persistent IgG deficiency.

    PubMed

    Düzgün, N; Duman, M; Sonel, B; Peksari, Y; Erdem, C; Tokgöz, G

    1997-01-01

    We present the case of a patient with juvenile onset systemic lupus erythematosus (SLE) who developed a persistent, acquired hypogammaglobulinaemia with IgG deficiency. The hypogammaglobulinaemia was probably a complication of high dose corticosteroid treatment. The serum IgG level remained subnormal despite intravenous immunoglobulin therapy. Lupus vulgaris, which developed on the nasal cartilage in this patient with SLE, is not an expected finding. This patient is probably the first reported case of SLE associated with lupus vulgaris.

  10. Antiphospholipid syndrome nephropathy in systemic lupus erythematosus.

    PubMed

    Daugas, Eric; Nochy, Dominique; Huong, Du Le Thi; Duhaut, Pierre; Beaufils, Hélène; Caudwell, Valérie; Bariety, Jean; Piette, Jean-Charles; Hill, Gary

    2002-01-01

    In the course of the antiphospholipid syndrome (APS), the existence of vaso-occlusive lesions capable of affecting numerous organs is now well established. The renal involvement attributable to primary APS, APS nephropathy (APSN), corresponds to vaso-occlusive lesions of the intrarenal vessels, associating side-by-side, acute thromboses with chronic arterial and arteriolar lesions, leading to zones of cortical ischemic atrophy. A retrospective study of 114 lupus patients undergoing renal biopsy was undertaken to determine the following: (1) if APSN can be found in the course of systemic lupus erythematosus (SLE); (2) if certain clinical and biologic factors can permit the prediction of the presence of APSN; and (3) if APSN is a superadded renal morbidity factor in lupus patients. This study shows the following: (1) APSN occurs in SLE (32% of patients with renal biopsies) in addition to, and independently of, lupus nephritis; (2) APSN is statistically associated with lupus anticoagulant but not with anticardiolipin antibodies; (3) APSN is associated with extrarenal APS, mainly arterial thromboses and obstetrical fetal loss, but not with the venous thromboses of APS; (4) APSN is an independent risk factor, over and above lupus nephritis, that contributes to an elevated prevalence of hypertension, elevated serum creatinine, and increased interstitial fibrosis. Thus, it seems likely that, because of its associations with hypertension, elevated serum creatinine, and increased interstitial fibrosis, APSN may worsen the prognosis in these patients. APSN may also have therapeutic significance in that its recognition should permit a better balance between immunosuppressor and antithrombotic and/or vasoprotective therapy. Finally, this study suggests that APSN should be considered as an element to be included in the classification criteria of APS.

  11. Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

    PubMed Central

    Her, Min Young; Song, Joo Yeon

    2009-01-01

    Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE. PMID:19270838

  12. Hypocomplementemic urticarial vasculitis in systemic lupus erythematosus.

    PubMed

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

    2009-02-01

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

  13. Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus.

    PubMed Central

    Simeon-Aznar, C P; Cuenca-Luque, R; Fonollosa-Pla, V; Bosch-Gil, J A

    1992-01-01

    The case of a patient admitted with thrombotic thrombocytopenic purpura nine years after developing systemic lupus erythematosus (SLE) is reported. Thrombotic thrombocytopenic purpura associated with SLE has been described on other occasions, but in most patients the diagnosis of SLE precedes that of thrombotic thrombocytopenic purpura. The unusual sequence and the chronological separation of the two diseases is emphasised. PMID:1575591

  14. Clinical significance of the mixing test in laboratory diagnoses of lupus anticoagulant: the fate of the mixing test in integrated lupus anticoagulant test systems.

    PubMed

    Hong, Sung Kuk; Hwang, Sang Mee; Kim, Ji-Eun; Kim, Hyun Kyung

    2012-12-01

    The mixing test is used to determine the presence of inhibitors in laboratory diagnoses of lupus anticoagulant. Updated international guidelines state that an integrated lupus anticoagulant test system does not require the mixing test; an appraisal of the mixing tests in integrated lupus anticoagulant test systems is, therefore, required. We investigated the clinical relevance of mixing tests by using the best cutoff value of the mixing test through thrombotic risk analysis. A retrospective analysis was performed on 525 specimens with positive screening tests by using two integrated lupus anticoagulant tests: diluted Russell's Viper venom (dRVVT) and silica clotting time. The diagnostic performance of two interpretation formulas (percentage correction, Rosner index) was assessed, and the thrombotic risk of a subgroup based on the mixing results was investigated. Finally, the thrombotic risk of lupus anticoagulant positivity based on the integrated lupus anticoagulant test system procedures was assessed for the appraisal of mixing test exclusion in integrated lupus anticoagulant test systems. The best cutoff values of mixing test interpretation methods based on dRVVT were as follows: 60.1% for percentage correction and 15.7 for Rosner index. There was no substantial difference in the thrombotic risk between percentage correction and the Rosner index. The mixing-positive group showed a higher lupus anticoagulant titer and higher thrombotic risk than the mixing-negative group. However, even the mixing-negative group carried a significant risk of thrombosis. Finally, lupus anticoagulant positivity determined by the updated two-step procedure (screening and confirmation tests) showed higher thrombotic risk than that determined by the traditional three-step procedure (screening, mixing, and confirmation tests). Although a positive mixing result can predict a high risk of thrombosis, negative mixing results are also associated with a substantial thrombotic risk. The

  15. ANA-Negative Lupus Presenting with Heart Failure and Severe Valvular Dysfunction: Case Report and Review of the Literature.

    PubMed

    Hoang, Vu; Addison, Daniel; Lakkis, Nasser; Tabbaa, Rashed

    2015-01-01

    Antinuclear antibody (ANA) negative lupus is an important subset of the systemic lupus erythematosus (SLE) disease spectrum. Since the introduction of human cell line for ANA assay, the occurrence of true ANA-negative SLE has been a rare clinical phenomenon. The nature of cardiac involvement in ANA-negative SLE is not well understood, although any cardiac involvement, including valvular dysfunction, should be considered as a presenting manifestation of SLE irrespective of serology status. Early recognition and intervention appears to be associated with decreased morbidity. The following report describes our first case of ANA-negative SLE with an initial presentation of severe cardiac valvular dysfunction and heart failure. It also characterizes the spectrum of disease severity in ANA-negative SLE and demonstrates how aggressive SLE therapy can improve cardiac disease.

  16. Kawasaki disease and juvenile systemic lupus erythematosus.

    PubMed

    Diniz, J C; Almeida, R T; Aikawa, N E; Sallum, A M E; Sakane, P T; Silva, C A

    2012-01-01

    Kawasaki disease (KD) is a common vasculitis in childhood. To the authors' knowledge, only one case of juvenile systemic lupus erythematosus (JSLE)-like onset mimicking KD and another case of KD and JSLE association have previously been described. However, the prevalence of this association of the two diseases was not reported. Therefore, over 27 consecutive years, 5419 patients were followed at the Pediatric Rheumatology Unit and 271 (5%) of them met the ACR classification criteria for JSLE. Two (0.7%) of them were female. These also had KD according to European League against Rheumatism / Paediatric Rheumatology European Society (EULAR/PReS) consensus criteria and are described in this report. One case was a 13-year-old who presented all six KD criteria. Echocardiogram showed pericardial effusion, dilatation and tortuosity of right and left coronary, and her symptoms promptly improved after treatment with intravenous immunoglobulin (IVIG). Lupus diagnosis was established a few days later. Another case was a 4-year-old who had also met all six KD criteria, with improvement after IVIG, and lupus diagnosis was made 1 year later. In conclusion, the frequency of the association between these two autoimmune diseases was rare. The occurrence of a second autoimmune systemic disease in a patient with a history of KD should also be considered. Furthermore, the initial presentation of lupus may mimic KD.

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

    PubMed

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

    2013-05-22

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

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

    PubMed Central

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

    2013-01-01

    A middle aged female patient presented with generalised palpable purpura associated with intense pruritus along with subconjunctival haemorrhage and orbital inflammation. There was extensive dermographism. Other systemic examinations were within normal limits. Haematological profile was normal except raised D-dimer. Skin biopsy revealed the presence of leucocytoclastic vasculitis. Antinuclear antibody was positive in a titre of 1 : 160, but antidouble-stranded DNA was negative. Urine examination revealed haematuria and proteinuria. Complement C3, C4 and C1q levels were decreased with the presence of anti-C1q antibody. There was a diagnostic dilemma between systemic lupus erythematosus and hypocomplementaemic urticarial vasculitis syndrome. However, as the patient did not fulfil the American College of Rheumatology criteria for systemic lupus erythematosus, but fulfilled all the criteria for hypocomplementaemic urticarial vasculitis syndrome, the case was finally diagnosed as hypocomplementaemic urticarial vasculitis syndrome and treated accordingly with favourable outcome. PMID:23704433

  19. [Modern therapy for systemic lupus erythematosus].

    PubMed

    Fischer-Betz, R; Schneider, M

    2007-12-01

    Even though no new treatment for systemic lupus erythematosus (SLE) has been approved in over 40 years, the treatment possibilities have expanded decisively in this time. The available evidence for most forms of therapy is rather thin, yet there is a wide consensus on an individual immunosuppressive therapy that is adjusted to the illness activity and severity. Various new substances, e.g. other immunosuppressants and anti-cell therapies have been tested, mostly in open studies. Considering the experience in other indications, mycophenolate mofetil and rituximab have a very promising potential to be approved for therapy of SLE. Aside from these, "old medications" such as antimalarial drugs still possess a high value. Besides the effective suppression of the illness activity, this is especially decisive for the improvement of the long-term prognosis, the treatment of co-morbidities and secondary prevention and the avoidance of negative effects of the illness and therapy, such as infections, osteoporosis, and premature arteriosclerosis. As these "simple" measures often remain disregarded in modern therapy, they will be focused on in this overview on the current treatment concepts for SLE and these aspects will be discussed in greater depth. With regard to new future therapies, we refer the reader to other current publications.

  20. Biotherapies in systemic lupus erythematosus: New targets.

    PubMed

    Lazaro, Estibaliz; Scherlinger, Marc; Truchetet, Marie-Elise; Chiche, Laurent; Schaeverbeke, Thierry; Blanco, Patrick; Richez, Christophe

    2016-09-20

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a polymorphic presentation. The variability in the clinical expression and severity of SLE makes new treatments both essential and challenging to develop. Several biotherapies targeting different pathophysiological pathways have been developed over the past 15 years. The results of Phase II trials were encouraging but rarely borne out by Phase III trials. Recent data, which are discussed in detail in this review, allowed belimumab - a monoclonal antibody against BLyS (B-lymphocyte stimulator) - to become the first biotherapy approved for use in SLE. Other molecules targeting B cells include the two anti-BLyS antibodies tabalumab and blisibimod; atacicept, which targets both BLyS and APRIL (a proliferation-inducing ligand); and the monoclonal antibody to CD22 epratuzumab. The rekindling of interest in the B-cell pathway has also driven new clinical research into rituximab, a monoclonal antibody targeting CD20 with evaluations of new strategies. A new and promising approach is the use of inhibitors of the type 1 interferon (IFN) pathway, of which the most promising is anifrolumab, a monoclonal antibody targeting the type 1 IFN receptor. In this review, we discuss study findings and their clinical relevance, present the most promising targets, and analyze possible explanations to negative results, such as inappropriate patient selection and treatment response criteria or the erratic use of high-dose glucocorticoid therapy.

  1. Epigenetics and Systemic Lupus Erythematosus: Unmet Needs.

    PubMed

    Meroni, Pier Luigi; Penatti, Alessandra Emiliana

    2016-06-01

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

  2. Systemic lupus erythematosus: the face of Asia.

    PubMed

    Feng, Pao-Hsii

    2007-06-01

    This review documents the remarkable progress systemic lupus erythematosus (SLE) has made in the past 40 years especially in Asia. It provides a kaleidoscope in terms of prevalence, ethnic and regional variations, disease manifestation, treatment strategies, and outcome. From a seminal paper on the use of intravenous cyclophosphamide in 1964 from Singapore to the use of mycophenolate mofetil in 2000 from Hong Kong and Guangzhou, the prognosis of lupus has changed dramatically in the last few decades. With more targeted therapies and better translational research, this progress is set to continue in the coming years. From an acute fulminating illness, lupus has now evolved to one with a chronic, relapsing course. The main causes of morbidity and mortality are now either treatment-related or patient-related rather than the disease itself. The present time is one of unprecedented growth of new therapeutic approaches and reevaluation of past treatment modalities. With improving socioeconomic conditions in the region, we anticipate further rapid progress in disease outcome. Although living with the wolf is still an ordeal for our patients, optimism has now replaced nihilism in the lupus world. There is light at the end of the tunnel.

  3. Genome-Wide Association Study in African-Americans with Systemic Lupus Erythematosus

    DTIC Science & Technology

    2013-09-01

    Americans with Systemic Lupus Erythematosus PRINCIPAL INVESTIGATOR: John Harley, M.D., Ph.D...SUPPLEMENTARY NOTES 14. ABSTRACT Systemic lupus erythematosus ( lupus ) is a potentially deadly systemic autoimmune disease that disproportionately... Systemic lupus erythematosus ( lupus ) is a potentially deadly systemic autoimmune disease that disproportionately afflicts women and African

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

    PubMed Central

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

    2014-01-01

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

  5. [Pregnancy complications in a patient with systemic lupus erythematosus and lupus nephritis].

    PubMed

    Bisgaard, Helene; Jacobsen, Søren; Tvede, Niels; Langhoff-Roos, Jens

    2014-07-14

    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 and that pregnant women with SLE should be followed in a multidisciplinary setting.

  6. The deleterious role of basophils in systemic lupus erythematosus

    PubMed Central

    Pellefigues, Christophe; Charles, Nicolas

    2013-01-01

    Systemic lupus erythematosus is a complex autoimmune disease of multifactorial origins. All compartments of the immune system appear to be affected, at least in some way, and to contribute to disease pathogenesis. Due to an escape from negative selection autoreactive T and B cells accumulate in SLE patients leading to the production of autoantibodies mainly raised against nuclear components and their subsequent deposition into target organs. We recently showed that basophils, in an IgE and IL-4 dependent manner, contribute to SLE pathogenesis by amplifying autoantibody production. Here, we summarize what we have learned about the deleterious role of basophils in lupus both in a mouse model and in SLE patients. We discuss which possible pathways could be involved in basophil activation and recruitment to secondary lymphoid organs during SLE, and how basophils may amplify autoantibody production. PMID:24209595

  7. Seizure disorders in Systemic Lupus Erythematosus

    PubMed Central

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

    2015-01-01

    Objective To describe the frequency, attribution, outcome and predictors of seizures in SLE Methods The Systemic Lupus International Collaborating Clinics (SLICC) performed a prospective inception cohort study. Demographic variables, global SLE disease activity (SLEDAI-2K), cumulative organ damage (SLICC/ACR Damage Index (SDI)) and neuropsychiatric events were recorded at enrollment and annually. Lupus anticoagulant, anticardiolipin, anti-β2 glycoprotein-I, anti-ribosomal P and anti-NR2 glutamate receptor antibodies were measured at enrollment. Physician outcomes of seizures were recorded. Patient outcomes were derived from the SF-36 mental (MCS) and physical (PCS) component summary scores. Statistical analyses included Cox and linear regressions. Results The cohort was 89.4% female with a mean follow up of 3.5±2.9 years. 75/1631 (4.6%) had ≥1 seizure, the majority around the time of SLE diagnosis. Multivariate analysis indicated a higher risk of seizures with African race/ethnicity (HR(CI):1.97 (1.07–3.63); p=0.03) and lower education status (1.97 (1.21–3.19); p<0.01). Higher damage scores (without NP variables) were associated with an increased risk of subsequent seizures (SDI=1:3.93 (1.46–10.55)); SDI=2 or 3:1.57 (0.32–7.65); SDI≥4:7.86 (0.89–69.06); p=0.03). There was an association with disease activity but not with autoantibodies. Seizures attributed to SLE frequently resolved (59/78(76%)) in the absence of anti-seizure drugs. There was no significant impact on the MCS or PCS scores. Anti-malarial drugs in absence of immunosuppressive agents were associated with reduced seizure risk (0.07(0.01–0.66); p=0.03). Conclusion Seizures occurred close to SLE diagnosis, in patients with African race/ethnicity, lower educational status and cumulative organ damage. Most seizures resolved without a negative impact on health-related quality of life. Anti-malarial drugs were associated with a protective effect. PMID:22492779

  8. What is new in systemic lupus erythematosus.

    PubMed

    Rúa-Figueroa Fernández de Larrinoa, Iñigo

    2015-01-01

    Systemic lupus erythematosus is a heterogeneous rheumatic systemic disease with extremely varied clinical manifestations and a diverse pathogenesis, as illustrated in this review on the most relevant new knowledge related to the disease. Topics such as anemia, pathogenesis, cardiovascular risk assessment, antiphospholipid syndrome, prediction of damage and recent advances in treatment, including tolerogenic and biological agents, are discussed. Relevant contributions regarding classical therapies such as corticosteroid and antimalarials and their optimal use, as well as the roll of vitamin D, are also referred.

  9. Lupus cystitis in Korean patients with systemic lupus erythematosus: risk factors and clinical outcomes.

    PubMed

    Koh, J H; Lee, J; Jung, S M; Ju, J H; Park, S-H; Kim, H-Y; Kwok, S-K

    2015-10-01

    This study was performed to investigate the clinical characteristics of lupus cystitis and determine the risk factors and clinical outcomes of lupus cystitis in patients with systemic lupus erythematosus (SLE). We retrospectively reviewed 1064 patients at Seoul St. Mary's Hospital in Seoul, Korea, from 1998 to 2013. Twenty-four patients had lupus cystitis. Lupus cystitis was defined as unexplained ureteritis and/or cystitis as detected by imaging studies, cystoscopy, or bladder histopathology without urinary microorganisms or stones. Three-fourths of patients with lupus cystitis had concurrent lupus mesenteric vasculitis (LMV). The initial symptoms were gastrointestinal in nature for most patients (79.2%). High-dose methylprednisolone was initially administered to most patients (91.7%) with lupus cystitis. Two patients (8.3%) died of urinary tract infections. Sixty-five age- and sex-matched patients with SLE who were admitted with other manifestations were included as the control group. Patients with lupus cystitis showed a lower C3 level (p = 0.031), higher SLE Disease Activity Index score (p = 0.006), and higher ESR (p = 0.05) upon admission; more frequently had a history of LMV prior to admission (p < 0.001); and less frequently had a history of neuropsychiatric lupus (p = 0.031) than did patients with SLE but without lupus cystitis. The occurrence of lupus cystitis was associated with a history of LMV (OR, 21.794; 95% CI, 4.061-116.963). The median follow-up period was 3.4 years, and the cumulative one-year mortality rate was 20%. Complications developed in 33.3% of patients with lupus cystitis and were related to survival (log-rank p = 0.021). Our results suggest that the possibility of lupus cystitis should be considered when a patient with SLE and history of LMV presents with gastrointestinal symptoms or lower urinary tract symptoms. Development of complications in patients with lupus cystitis can be fatal. Thus, intensive treatment

  10. Central nervous system systemic lupus erythematosus mimicking progressive multifocal leucoencephalopathy.

    PubMed Central

    Kaye, B R; Neuwelt, C M; London, S S; DeArmond, S J

    1992-01-01

    The case is reported of a patient with central nervous system systemic lupus erythematosus (SLE) with features of progressive multifocal leucoencephalopathy (PML) seen clinically and by magnetic resonance imaging. A brain biopsy sample showed microinfarcts. The use of magnetic resonance imaging and IgG synthesis rates in evaluating central nervous system lupus, the co-occurrence of SLE and PML, and the differentiation of these entities by magnetic resonance imaging and by histology are considered. Images PMID:1444628

  11. Therapeutic potential of SIGIRR in systemic lupus erythematosus.

    PubMed

    Wang, Chao; Feng, Chen-Chen; Pan, Hai-Feng; Wang, De-Guang; Ye, Dong-Qing

    2013-08-01

    Single immunoglobulin IL-1-related receptor (SIGIRR), which is also known as Toll/interleukin-1 receptor 8, is a member of the interleukin-1 receptor (IL-1R) family. Different from other typical IL-1R superfamily members, SIGIRR seems to exert negatively modulates in immune responses. Several previous studies demonstrated that SIGIRR influences chronic inflammatory or autoimmune diseases, such as intestinal inflammation, rheumatoid arthritis and psoriatic arthritis. Recent work has explored the role of SIGIRR in systemic lupus erythematosus (SLE), for example, the role of SIGIRR protects the mice from hydrocarbon oil-induced lupus has been reported. These results indicate that SIGIRR may represent a novel target for the treatment of SLE. In this review, we will discuss the SIGIRR and the therapeutic potential of modulating the pathway in SLE.

  12. Novel therapeutic agents for systemic lupus erythematosus.

    PubMed

    Gescuk, Bryan D; Davis, John C

    2002-09-01

    The last significant breakthrough in the treatment of systemic lupus erythematosus (SLE) was the use of cyclophosphamide and methylprednisolone in the treatment of lupus nephritis. Recent advances in immunology, oncology, and endocrinology have resulted in many potential therapies for SLE. These therapies include new immunosuppressants, biologic medications, tolerizing agents, immunoablation techniques, and hormonal medications. Each of these approaches will be discussed in this review. Some therapies are currently in use in clinical rheumatology practice (mycophenolate mofetil) and others are entering phase I trials (anti-BLyS monoclonal antibody). While some of these new therapies target specific inflammatory mechanisms in SLE (anti-CD40L monoclonal antibody), others work by nonspecific inhibition of the immune system (immunoablation).

  13. Systemic lupus, folie a trois and homicide.

    PubMed

    Caribé, André C; Daltro-Oliveira, Renato; Araújo, Ricardo Henrique; Cardoso, Ana Paula; Guimarães, Paulo Barreto; Miranda-Scippa, Angela; Quarantini, Lucas C

    2013-10-01

    Folie a trois is a syndrome characterized by the transfer of delusional ideas from one person to two other persons. This condition rarely ends in the murder of any involved and we are unaware of where the primary case had the diagnosis of acute psychosis in systemic lupus erythematosus (SLE). We present a case report of folie a trois resulting in murder, secondary to acute psychosis in SLE. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. Concomitant systemic lupus erythematosus and ankylosing spondylitis.

    PubMed Central

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

    1990-01-01

    The case is reported of a 42 year old white woman meeting currently used diagnostic criteria for both ankylosing spondylitis and systemic lupus erythematosus (SLE). As found in a previously described similar case of a black man, HLA typing showed antigens associated with both SLE and seronegative spondyloarthropathy. This case thus supports the hypothesis that the two diseases occur together only when this rare combination of HLA antigens is present. Images PMID:2344214

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

    PubMed

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

    2015-02-01

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

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

    PubMed

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

    2016-11-01

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

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

    PubMed

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

    2017-04-01

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

  18. Different Types of Lupus

    MedlinePlus

    ... Twitter Facebook Pinterest Email Print Different types of lupus Lupus Foundation of America February 24, 2017 Resource ... lupus. Learn more about each type below. Systemic Lupus Erythematosus Systemic lupus is the most common form ...

  19. Different Types of Lupus

    MedlinePlus

    ... 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 ...

  20. Systemic lupus erythematosus: Is it one disease?

    PubMed

    Rivas-Larrauri, Francisco; Yamazaki-Nakashimada, Marco Antonio

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystemic disease with a variety of clinical presentations. Monogenic predisposing conditions to the development of this disease have been described. As examples, an impaired expression of interferon-α regulated genes or complement deficiencies have been reported in patients with SLE, with particular clinical presentations. Those defects present particular presentations and a different severity, making an argument that lupus is not a single disease but many. Treatment could be individualized depending on the underlying defect generating the subtype of the disease. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  1. Ultraviolet radiation and systemic lupus erythematosus.

    PubMed

    Barbhaiya, M; Costenbader, K H

    2014-05-01

    Exposure to ultraviolet (UV) radiation is among the environmental factors that have been proposed and studied in association with systemic lupus erythematosus (SLE). While it is known that UV radiation exposure may exacerbate pre-existing lupus, it remains unclear whether UV exposure is a risk factor for the development of SLE. Experimental studies show a significant immunomodulatory role for UV radiation, but strong epidemiologic data regarding its role in triggering SLE onset are lacking. Further studies are needed to assess the role of UV radiation in relation to development of incident SLE, yet they are challenging to design due to difficulties in accurate exposure assessment, the heterogeneous nature of SLE, and the challenge of assessing photosensitivity, a feature of SLE, which often precedes its diagnosis.

  2. Acquired enophthalmos with systemic lupus erythematosus.

    PubMed

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

    2016-01-01

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

  3. Systemic lupus erythematosus in Nepal: A review.

    PubMed

    Kafle, M P; Lee, Vws

    2016-08-01

    Nepal is a small country that is landlocked between India and China. Several ethnic groups live within the 147,181 km(2) of this country. Geographic diversity ranges from the high Himalayas to the flatlands of the Ganges plains. Lupus nephritis (LN), a complication of systemic lupus erythematosus (SLE), is a common kidney problem in Nepal; but the real incidence and prevalence of SLE in Nepal is largely not known. Here, it more commonly affects people (mostly women) living in the southern flatlands, but SLE is reported to be uncommon further south in India. Even though the disease appears to be common, good quality research is uncommon in Nepali literature. This article was written to provide a review of the articles published to date about SLE in Nepal and to discuss the gaps in knowledge that require further evaluation.

  4. Cutaneous Manifestations of Systemic Lupus Erythematosus

    PubMed Central

    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

  5. Living with Lupus (For Parents)

    MedlinePlus

    ... Lupus The three main types of lupus are: Systemic lupus erythematosus, or SLE , which can affect multiple organs in ... which can cause scarring; and subacute cutaneous lupus erythematosus (SCLE) , ... systemic lupus. Drug-induced lupus , which accounts for about ...

  6. Periorbital discoid lupus: a rare localization in a patient with systemic lupus erythematosus.

    PubMed

    Cakici, Ozgur; Karadag, Remzi; Bayramlar, Huseyin; Ozkanli, Seyma; Uzuncakmak, Tugba Kevser; Karadag, Ayse Serap

    2016-01-01

    A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area..

  7. Periorbital discoid lupus: a rare localization in a patient with systemic lupus erythematosus*

    PubMed Central

    Cakici, Ozgur; Karadag, Remzi; Bayramlar, Huseyin; Ozkanli, Seyma; Uzuncakmak, Tugba Kevser; Karadag, Ayse Serap

    2016-01-01

    A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area.. PMID:28300917

  8. Estrogen therapy in systemic lupus erythematosus.

    PubMed

    Askanase, Anca D

    2004-01-01

    Given the female preponderance of systemic lupus erythematosus (SLE) in humans, the adverse effects of female gender and sex hormones in murine lupus, and numerous reports (retrospective, often anecdotal and uncontrolled) that describe a temporal association between estrogen exposure and development or exacerbation of SLE, it is tempting to accept that estrogens and SLE simply do not mix. While there are valid concerns regarding the use of exogenous estrogens in women with SLE, there are also potential health benefits to be considered. Oral contraceptives (OCs) offer effective birth control and may be bone protective in corticosteroid-treated patients. Recent studies, albeit retrospective, suggest that OCs are well tolerated in patients with SLE. Several salutary effects of postmenopausal estrogens assume particular importance in SLE where the risks of osteoporosis, exaggerated by menopause (natural or cyclophosphamide-induced) and corticosteroids, are substantial. However, the results of the Women's Health Initiative trial significantly limit the use of hormone replacement therapy in the general population, and raise particular concern for SLE patients. Other exogenous hormones (clomifene, gonadotropins, gonadotropin-releasing hormones) may be used to elevate levels of endogenous estrogen and to stimulate ovulation in patients with diminished fertility. Patients with inactive or stable/moderate disease and at low risk for thrombosis may benefit from OCs and other hormonal therapies without a change in lupus activity. Large prospective, double-blind, placebo-controlled studies inclusive of all ethnic groups should provide the basis for more definitive recommendations.

  9. [A case of systemic lupus erythematosus presenting with jaundice and lupus pneumonia].

    PubMed

    Kawai, Takako; Tominaga, Sizuo; Okouchi, Akiko; Kudo, Makoto; Katoh, Kiyoshi; Shoda, Masataka; Fujino, Masayuki A

    2005-02-01

    A 27-year-old Japanese woman was referred to our hospital for acute hepatitis in April 2002. She had been suffering from low grade fever and fatigue for a week. She also presented with dyspnea. On admission, ALT and AST were 857 U/l and 473 U/l respectively. Urine protein was 2 g/day. Chest radiograph showed bilateral infiltrative shadow and pleural effusion. She developed jaundice and her level of total bilirubin was increased to 9.6 mg/dl on May 9. Antibodies to hepatitis viruses were not detected. Testing for antimitochondrial antibodies, antismooth muscle antibodies, and antiribosomal P antibodies showed all negative. However, antinuclear antibodies were positive at titer 1:160 and anti-double stranded DNA antibodies were 130 U/ml. A diagnosis of systemic lupus erythematosus was made and oral administration of 60 mg/day prednisolon was started on May 10. Serum levels of ALT, AST and bilirubin were reduced to within normal range and pulmonary lesions were also improved. We conclude that this is a rare case of systemic lupus erythematosus presenting with acute hepatitis and jaundice.

  10. Respiratory manifestations of systemic lupus erythematosus: old and new concepts.

    PubMed

    Pego-Reigosa, José María; Medeiros, Dina A; Isenberg, David A

    2009-08-01

    The respiratory system is commonly involved in systemic lupus erythematosus. Lung disorders are classified as primary (due to lupus) and secondary to other conditions. Pleuritis and pulmonary infections are the most prevalent respiratory manifestations of each type. Other infrequent manifestations include interstitial lung disease, acute lupus pneumonitis, diffuse alveolar haemorrhage, pulmonary arterial hypertension, acute reversible hypoxaemia and shrinking lung syndrome. Even when current diagnostic tests contribute to an earlier diagnosis, the treatment of these manifestations is based on clinical experience and small series. Larger controlled trials of the different therapies in the treatment of those lung manifestations of lupus are needed. Overall malignancy is little increased in lupus, but lung cancer and non-Hodgkin's lymphoma are among the most frequent types of cancer found in these patients. As survival in lupus patients has improved over recent decades, avoiding pulmonary damage emerges as an important objective.

  11. Elevated sacroilac joint uptake ratios in systemic lupus erythematosus

    SciTech Connect

    De Smet, A.A.; Mahmood, T.; Robinson, R.G.; Lindsley, H.B.

    1984-08-01

    Sacroiliac joint radiographs and radionuclide sacroiliac joint uptake ratios were obtained on 14 patients with active systemic lupus erythematosus. Elevated joint ratios were found unilaterally in two patients and bilaterally in seven patients when their lupus was active. In patients whose disease became quiescent, the uptake ratios returned to normal. Two patients had persistently elevated ratios with continued clinical and laboratory evidence of active lupus. Mild sacroiliac joint sclerosis and erosions were detected on pelvic radiographs in these same two patients. Elevated quantitative sacroiliac joint uptake ratios may occur as a manifestation of active systemic lupus erythematosus.

  12. Toe walking as a presenting sign of systemic lupus erythematosus.

    PubMed

    Basiaga, M; Sherry, D

    2015-10-01

    Toe walking is a previously unreported presentation of systemic lupus erythematosus (SLE). We describe a patient who presented with profound multisystem involvement that was preceded by one month of toe walking and multiple flexion contractures without arthritis. Her lupus is now under control after aggressive therapy, yet she continues to struggle with tendinopathy despite continued physical and occupational therapy. Lupus should be considered in the appropriate clinical context in children who have new-onset contractures due to tight tendons.

  13. Cardiovascular risk assessment and treatment in systemic lupus erythematosus.

    PubMed

    Elliott, Jennifer R; Manzi, Susan

    2009-08-01

    With improved treatment modalities and survival rates, patients with systemic lupus erythematosus live longer and their co-morbidities have become more apparent. Of great concern is cardiovascular disease, which has become a leading cause of death. Lupus patients prematurely develop atherosclerosis, which likely arises from an interaction among traditional cardiovascular risk factors, factors specific to lupus itself and inflammatory mediators. Despite these findings, lupus patients are not always adequately evaluated for traditional risk factors, many of which are treatable and reversible. We propose that lupus patients be assessed and managed regarding cardiovascular risk factors in the same manner as patients with known cardiovascular disease. As a result, preventive cardiology should be considered an essential component of the care for patients with lupus.

  14. Pregnancy and contraception in systemic and cutaneous lupus erythematosus.

    PubMed

    Guettrot-Imbert, G; Morel, N; Le Guern, V; Plu-Bureau, G; Frances, C; Costedoat-Chalumeau, N

    2016-10-01

    A causal link has long been described between estrogen and systemic lupus erythematosus activity. Contraceptive and pregnancy management is now common for lupus patients, but pregnancy continues to be associated with higher maternal and fetal mortality/morbidity in systemic lupus erythematosus patients than among the general population. Potential complications include lupus flares, obstetric complications (fetal loss, in utero growth retardation, premature birth) and neonatal lupus syndrome. Association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetric complications. Anti-SSA and/or anti-SSB antibodies put fetuses at risk for neonatal lupus. Improving the outcome of such pregnancies depends upon optimal systematic planning of pregnancy at a preconception counseling visit coupled with a multidisciplinary approach. Absence of lupus activity, use of appropriate medication during pregnancy based on the patient's medical history and risk factors, and regular monitoring constitute the best tools for achieving a favorable outcome in such high-risk pregnancies. The aim of this review is to provide an update on the management of contraception and pregnancy in systemic lupus erythematosus, cutaneous lupus and/or antiphospholipid syndrome in order to reduce the risk of complications and to ensure the best maternal and fetal prognosis. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  15. Cardiac tamponade as an initial manifestation of systemic lupus erythematosus.

    PubMed

    Carrion, Diego M; Carrion, Andres F

    2012-06-12

    Clinical manifestations of pericardial disease may precede other signs and symptoms associated with systemic lupus erythematosus. Although pericardial effusion is one of the most common cardiac problems in patients with systemic lupus erythematosus, haemodynamically significant effusions manifesting as cardiac tamponade are rare and require prompt diagnosis and treatment.

  16. Anticytokine therapies in systemic lupus erythematosus

    PubMed Central

    Cava, Antonio La

    2010-01-01

    The dysfunctional immune response that characterizes systemic lupus erythematosus (SLE) associates with an unbalanced production of soluble mediators that are crucial in promoting and sustaining chronic inflammation. The successful use of biologics in several autoimmune diseases has led to studies in SLE aimed at contrasting the proinflammatory responses that contribute to tissue and organ damage in the disease. Several approaches have been developed and tested as potential therapeutic agents in SLE in preclinical studies and in clinical trials. This article provides an overview on antibody-based approaches in SLE that, although preliminary, have the potential to expand the current therapeutic possibilities in the disease. PMID:20636010

  17. Collapsing glomerulopathy in systemic lupus erythematosus.

    PubMed

    Abadeer, Kerolos; Alsaad, Ali A; Geiger, Xochiquetzal J; Porter, Ivan E

    2017-02-27

    Collapsing glomerulopathy (CG) is a rare disease that can be associated with multiple other disorders. It usually leads to poor prognosis with a high percentage of patients progressing to end-stage renal disease. In this article, we illustrate a clinical case of CG associated with systemic lupus erythematosus that had a prompt response to mycophenolate and prednisone. The condition started after sudden cessation of the already established mycophenolate treatment regimen. The patient then presented with acute kidney injury due to kidney biopsy-proven CG. In that circumstance, we hypothesised that mycophenolate may play a role in prevention and development of CG.

  18. Management of Systemic Lupus Erythematosus During Pregnancy.

    PubMed

    Sammaritano, Lisa R

    2017-01-14

    Reproductive issues including contraception, fertility, and pregnancy are important components of the comprehensive care of women with systemic lupus erythematosus (SLE). SLE pregnancies are complicated due to risk for maternal disease exacerbation and potential for fetal and neonatal complications. Pre-pregnancy assessment is important to identify patients with severe disease-related damage who should avoid pregnancy, counsel patients to conceive when disease has been stable and inactive on appropriate medications, and assess relevant risk factors including renal disease, antiphospholipid antibody, and anti-Ro/SS-A and anti-La/SS-B antibodies. With careful planning, monitoring, and care, most women with SLE can anticipate a successful pregnancy.

  19. Dendritic cells in systemic lupus erythematosus.

    PubMed

    Seitz, Heather M; Matsushima, Glenn K

    2010-04-01

    Systemic lupus erythematosus (SLE) persists as a chronic inflammatory autoimmune disease and is characterized by the production of autoantibodies and immune complexes that affect multiple organs. The underlying mechanism that triggers and sustains disease are complex and involve certain susceptibility genes and environmental factors. There have been several immune mediators linked to SLE including cytokines and chemokines that have been reviewed elsewhere [ 1-3 ]. A number of articles have reviewed the role of B cells and T cells in SLE [ 4-10 ]. Here, we focus on the role of dendritic cells (DC) and innate immune factors that may regulate autoreactive B cells.

  20. Renal vascular lesions in systemic lupus erythematosus.

    PubMed

    Katz, S M; Korn, S; Umlas, S L; DeHoratius, R J

    1990-01-01

    In the past, necrotizing vasculitis has been considered to be one of the dominant intrarenal vascular abnormalities in systemic lupus erythematosus (SLE). To test the validity of this statement, 70 consecutive renal biopsies from patients with SLE were reviewed. Light microscopy (LM) and immunofluorescence (IF) studies documented abnormalities, including thrombosis and nephrosclerosis, in 30 patients (43 percent), but no cellular infiltration of the vessel walls or other evidence of acute necrotizing vasculitis was seen. It is concluded that while intrarenal vasculopathy with thrombosis and nephrosclerosis is a common finding in SLE, our data and recently published studies suggest that acute necrotizing vasculitis occurs rarely, if at all, in SLE nephritis.

  1. Shrinking lung syndrome in systemic lupus erythematosus

    PubMed Central

    Borrell, Helena; Narváez, Javier; Alegre, Juan José; Castellví, Ivan; Mitjavila, Francesca; Aparicio, María; Armengol, Eulàlia; Molina-Molina, María; Nolla, Joan M.

    2016-01-01

    Abstract Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965–2015). These 80 cases, together with our 9 patients, form the basis of the present analysis. The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases. An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful. There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength. The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild

  2. Hypogammaglobulinemia in pediatric systemic lupus erythematosus.

    PubMed

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

    2013-11-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease typically associated with elevated serum immunoglobulin G (IgG). Hypogammaglobulinemia in SLE patients has been attributed to immunosuppressive treatment or a transient effect associated with nephrotic syndrome. We retrospectively reviewed pediatric SLE patients from a single institution to identify patients with hypogammaglobulinemia and risk factors for hypogammaglobulinemia. A total of 116 pediatric SLE cases from 1997 to 2011 were reviewed and patients with hypogammaglobulinemia (IgG < 500 mg/dl) were identified. The two cohorts were evaluated for association with age, sex, presence of lupus nephritis at SLE diagnosis, disease activity at diagnosis, initial IgG level, and drug treatment. Eighty-six patients were included in our study, with a median age of 15 years and a median follow-up of 39.5 months. Seven percent (six of 86) of patients had hypogammaglobulinemia with a median onset of 27 months (0-72 months) after SLE diagnosis. Significant associations were noted for white race (p value 0.029), male sex (p value 0.009), and the presence of lupus nephritis at SLE diagnosis (p value 0.004). Use of immunosuppressive treatment did not show a statistical association with hypogammaglobulinemia, although two of the patients with hypogammaglobulinemia did receive rituximab. Most patients with hypogammaglobulinemia received intravenous immunoglobulin (IVIG) replacement therapy because of infections and/or concern for infection. Measurement of immunoglobulin levels during treatment in SLE could help identify patients with hypogammaglobulinemia who might require more aggressive follow-up to monitor for increased risk of infection and need for IVIG treatment. A prospective study is needed to validate associated risk factors identified in this study.

  3. Treatment Algorithms in Systemic Lupus Erythematosus.

    PubMed

    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

  4. Different Types of Lupus

    MedlinePlus

    ... lupus. Learn more about each type below. Systemic lupus erythematosus Systemic lupus is the most common form of lupus—it’s ... it is likely that these people already had systemic lupus, with the skin rash as their ... lupus erythematosus Drug-induced lupus is a lupus-like disease ...

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

    PubMed

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

    2017-05-31

    Severity in systemic lupus erythematosus may vary from mild to even fatal consequences. There are no biomarkers to predict the disease's prognosis. The Systemic Lupus International Collaborating Clinics/ Systemic Damage Index defines systemic lupus erythematosus disease severity and is found to predict prognosis. To test damage determinants in a single-centre systemic lupus erythematosus cohort. Retrospectively followed systemic lupus erythematosus female patients (defined by the identification of at least four systemic lupus erythematosus American College of Rheumatology criteria - fulfillment 100%, n = 76) over the past five years. Age of onset, ethnicity, disease duration, number of American College of Rheumatology criteria at the end of follow-up, cumulative: renal, neuropsychiatric and articular phenotypes, hypertension, dyslipidaemia, smoking and Systemic Lupus Erythematosus Disease Activity Index 2K were correlated to the presence and degree of irreversible damage (Systemic Lupus International Collaborating Clinics Damage Index). Accumulation of American College of Rheumatology criteria was measured in a sub-group of patients followed from disease onset (within a year of the first symptom ascribed to systemic lupus erythematosus) (n = 39 - 51%); Systemic Lupus Erythematosus Disease Activity Index and Systemic Lupus International Collaborating Clinics Damage Index were performed. Statistical analysis was performed using Chi-square, Wilcoxon Mann-Whitney tests and Spearman correlation rho (Sig. 2-tailed p < 0.05). Systemic Lupus International Collaborating Clinics/Systemic Damage Index > 0 was present in 56.6% and significantly associated to a longer duration, a higher number of American College of Rheumatology criteria and a neuropsychiatric phenotype when compared with those with no damage. The final number of American College of Rheumatology criteria accrued was positively correlated to a higher disease activity over the past five years of follow

  6. Refractory disease in systemic lupus erythematosus.

    PubMed

    Campar, Ana; Farinha, Fátima; Vasconcelos, Carlos

    2011-09-01

    There is no definition or guidelines for refractory disease (RD) in Systemic Lupus Erythematosus (SLE). However, new therapies have been tested mainly in refractory patients. The concept, like the disease, is complex and implies deeper knowledge on the disease pathogenesis and patients' subsets. RD is not included in current activity indices of the disease, what raises the question of how are we monitoring its response to new drugs. In this paper, we analyse some concepts considered important for the global definition of RD in SLE and in some specific organ involvements, excluding lupus nephritis. Management issues will be addressed also. Finally, we review therapeutic options in particular subsets of the disease, namely, cutaneous, articular, haematological and neuropsychiatric lupus. Crucial to the management of a patient suspected to be refractory is an accurate diagnosis, assuring that the persistent clinical manifestations are derived primarily from SLE and not from a concomitant or alternative process. Likewise, certainty about the patient compliance with the therapy prescribed is a frequent unrecognized problem that erroneously might lead to a classification of RD. Therapy of RD for SLE, in general and in most particular involvements, is currently based mainly on the clinician's own experience and judgement, with few randomized trials effectively addressing the issue. In such a heterogeneous disease, consideration of approval of drugs for single-organ indications may pave the way for new therapies. Better biomarkers are needed to add accuracy to the currently used activity indices in order to monitor RD and consolidate its definition. Prospective studies directed to RD in the main SLE involvements are needed to improve our understanding on the management of the disease and foster the development of targeted new drugs. Copyright © 2011 Elsevier B.V. All rights reserved.

  7. Raynaud's phenomenon with oral manifestation in systemic lupus erythematosus.

    PubMed

    da Cunha Bang, F; Lange Wantzin, G; Dahl Christensen, J

    1985-01-01

    A 24-year-old woman with discoid lupus erythematosus developed systemic lupus erythematosus after 6 years. One of the clinical features was Raynaud's phenomenon in the fingers and toes, and furthermore Raynaud's phenomenon appeared in the tongue when exposed to cold and windy weather.

  8. Hydralazine Induced Lupus Syndrome Presenting with Recurrent Pericardial Effusion and a Negative Antinuclear Antibody

    PubMed Central

    Iyer, Praneet; Zijoo, Ritika

    2017-01-01

    Drug induced lupus erythematosus (DIL or DILE) is an autoimmune disorder caused by chronic use of certain drugs. We report a unique case of hydralazine induced lupus syndrome (HILS) with a negative antinuclear antibody in a female patient who was on hydralazine for a period of 1.5–2 years and developed recurrent pericardial effusion as a result of it. Initially her condition was managed with a pericardial window. The recurrence of a massive pericardial effusion necessitated a right hemipericardiectomy. After hydralazine was stopped, she never had any further episodes of pericardial effusion or tamponade. PMID:28194293

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

    PubMed

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

    2017-04-01

    only important predictor. The change in SF-36 MCS was also independently associated with neuropsychiatric systemic lupus erythematosus ( B = 5.783; p < 0.05) and inflammatory neuropsychiatric systemic lupus erythematosus ( B = 11.133; p < 0.001). Disease duration and change in disease activity were the only predictors in both cases. The change in SF-36 PCS was only negatively associated with age. Conclusion Inflammatory neuropsychiatric systemic lupus erythematosus events have better clinical outcome and meaningful improvement in SF-36 MCS than ischaemic neuropsychiatric systemic lupus erythematosus or non-neuropsychiatric systemic lupus erythematosus.

  10. Characteristics of pleural effusions in systemic lupus erythematosus: differential diagnosis of lupus pleuritis.

    PubMed

    Choi, B Y; Yoon, M J; Shin, K; Lee, Y J; Song, Y W

    2015-03-01

    We investigated the clinical characteristics of pleural effusion in systemic lupus erythematosus (SLE). A prospective analysis of 17 SLE patients with pleural effusion (seven lupus pleuritis, eight transudative effusions and two parapneumonic effusions) was performed. Thirty non-SLE patients with pleural effusion were recruited as controls. A pleural fluid ANA titer ≥1:160 was found in 8/17 (47.1%) SLE patients and none of the 30 non-SLE patients (p = 0.0001). Pleural fluid to serum C3 ratios were significantly lower in SLE than in non-SLE (median (minimum-maximum) 0.29 (0.03-0.43) versus 0.52 (0.26-0.73), p = 0.0002). Among SLE patients, pleural fluid ANA titers ≥1:160 were more frequently found in patients with lupus pleuritis than in those with pleural effusion from causes other than lupus itself (85.7% versus 20.0%, p = 0.0152). Serum CRP levels were significantly increased in patients with lupus pleuritis compared with SLE patients with transudative pleural effusion (2.30 (0.30-5.66) versus 0.7 (0.12-1.47) mg/dl, p = 0.0062). In conclusion, pleural fluid ANA titer and serum CRP levels are significantly increased in lupus pleuritis.

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

    PubMed

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

    2015-03-01

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

  12. Lupus

    MedlinePlus

    ... bite or scratch of a wolf ("lupus" is Latin for wolf and "erythematosus" is Latin for red). SLE is the most serious form ... occurs more often in African-American, Asian-American, Latin-American, and Native-American women than in non- ...

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

    PubMed

    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.

  14. Mood Disorders in Systemic Lupus Erythematosus

    PubMed Central

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

    2015-01-01

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

  15. Ocular manifestations in systemic lupus erythematosus.

    PubMed

    Silpa-archa, Sukhum; Lee, Joan J; Foster, C Stephen

    2016-01-01

    Systemic lupus erythematosus (SLE) can involve many parts of the eye, including the eyelid, ocular adnexa, sclera, cornea, uvea, retina and optic nerve. Ocular manifestations of SLE are common and may lead to permanent blindness from the underlying disease or therapeutic side effects. Keratoconjunctivitis sicca is the most common manifestation. However, vision loss may result from involvement of the retina, choroid and optic nerve. Ocular symptoms are correlated to systemic disease activity and can present as an initial manifestation of SLE. The established treatment includes prompt systemic corticosteroids, steroid-sparing immunosuppressive drugs and biological agents. Local ocular therapies are options with promising efficacy. The early recognition of disease and treatment provides reduction of visual morbidity and mortality.

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

    PubMed

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

    2014-06-01

    The expression of connective tissue growth factor mRNA in human kidneys may serve as an early marker for lupus nephritis progression. Therefore, we speculated that connective tissue growth factor may be involved in the pathogenesis of systemic lupus erythematosus and lupus nephritis. In this study, we set out to investigate the associations between serum connective tissue growth factor levels and clinicopathological features of patients with systemic lupus erythematosus and lupus nephritis. Serum samples from patients with non-renal systemic lupus erythematosus, renal biopsy-proven lupus nephritis and healthy control subjects were detected by enzyme-linked immunosorbent assay for serum connective tissue growth factor levels. The associations between connective tissue growth factor levels and clinicopathological features of the patients were further analysed. The levels of serum connective tissue growth factor in patients with non-renal systemic lupus erythematosus and lupus nephritis were both significantly higher than those in the normal control group (34.14 ± 12.17 ng/ml vs. 22.8 ± 3.0 ng/ml, p<0.001; 44.1 ± 46.8 ng/ml vs. 22.8 ± 3.0 ng/ml, p = 0.035, respectively). There was no significant difference of the serum connective tissue growth factor levels between non-renal systemic lupus erythematosus and lupus nephritis group (34.14 ± 12.17 ng/ml vs. 44.1 ± 46.8 ng/ml, p = 0.183). Serum connective tissue growth factor levels were significantly higher in lupus nephritis patients with the following clinical manifestations, including anaemia (51.3 ± 51.4 ng/ml vs. 23.4 ± 9.7 ng/ml, p<0.001) and acute renal failure (85.5 ± 75.0 ng/ml vs. 31.2 ± 21.8 ng/ml, p = 0.002). Serum connective tissue growth factor levels in class IV were significantly higher than that in class II, III and V (57.6 ± 57.5 ng/ml vs. 18.7 ± 6.4 ng/ml, p = 0.019; 57.6 ± 57.5 ng/ml vs. 25.2

  17. Management of pregnancy in systemic lupus erythematosus.

    PubMed

    Lateef, Aisha; Petri, Michelle

    2012-12-01

    Systemic lupus erythematosus (SLE) is an autoantibody-mediated systemic autoimmune disease, predominantly affecting young females. Pregnancy is increasingly common in the setting of SLE, as survival and quality of life of patients improve. Although live births can be achieved in the most cases, pregnancy in patients with SLE remains a high-risk condition. Maternal and fetal mortality and morbidity are considerably increased, compared with the general population. Aberrations in pregnancy-related maternal immune adaptations are likely contributors. Active maternal disease, renal involvement, specific autoantibody subsets and advanced organ damage are predictors of poor outcome. Therapeutic options are limited during pregnancy as maternal benefit has to be weighed against fetal risk. Prevention of preterm birth and refractory pregnancy loss, as well as management of established neonatal heart block remain unmet needs. Further research should address these important issues that affect young patients with SLE and their babies.

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

    PubMed

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

    2015-09-01

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

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

    PubMed

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

    2017-05-25

    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.

  20. Lupus-related advanced liver involvement as the initial presentation of systemic lupus erythematosus.

    PubMed

    Lu, Ming-Chi; Li, Ko-Jen; Hsieh, Song-Chou; Wu, Cheng-Han; Yu, Chia-Li

    2006-12-01

    Systemic lupus erythematosus (SLE), a prototype of systemic autoimmune disease characterized by multiorgan involvement with diverse clinical and serological manifestations, principally affects women in their child-bearing years. Clinically significant hepatic abnormality as the initial presentation of SLE has rarely been reported. Eleven patients with lupus with initial presentation of lupus-related hepatitis were included in this retrospective review. Clinical manifestation, immunological profiles, and risk factors for poor prognosis were analyzed. The most commonly associated clinical manifestations were found to be thrombocytopenia, leukopenia, advancing age, and presence of anti-SSA/Ro antibody and anti-thyroid antibodies. The diagnosis of SLE was delayed due to dominant hepatic abnormalities. Age greater than 50 years and marked hepatic decompensation in accordance with Child classification B and C might suggest poor prognosis (p=0.06). However, the p value was not statistically significant because of the small sample size. Lupus-related hepatitis, particularly in late-onset lupus, is common. In addition, the presence of anti-SSA, anti-thyroglobulin, and anti-microsomal antibodies is indicative of hepatic involvement in patients with SLE.

  1. Systemic lupus erythematosus flare triggered by a spider bite.

    PubMed

    Martín Nares, Eduardo; López Iñiguez, Alvaro; Ontiveros Mercado, Heriberto

    2016-01-01

    Systemic lupus erythematosus is a chronic autoimmune disease with a relapsing and remitting course characterized by disease flares. Flares are a major cause of hospitalization, morbidity and mortality in patients with systemic lupus erythematosus. Some triggers for these exacerbations have been identified, including infections, vaccines, pregnancy, environmental factors such as weather, stress and drugs. We report a patient who presented with a lupus flare with predominantly mucocutaneous, serosal and cardiac involvement after being bitten by a spider and we present the possible mechanisms by which the venom elicited such a reaction. To the best of our knowledge, this is the first such case reported in the literature.

  2. Neuropsychiatric Systemic Lupus Erythematosus: A Diagnostic Conundrum

    PubMed Central

    Joseph, Vivek; Anil, Rahul; Aristy, Sary

    2016-01-01

    A 70-year-old man presented with complaints of rapid cognitive decline and new onset leukopenia. The patient had a 17-year history of refractory seizures. Detailed review of symptoms and investigations revealed the patient met American College of Rheumatology (ACR) diagnostic criteria for systemic lupus erythematosus (SLE). The patient had high titer ANA with a strongly positive dsDNA. Immunosuppressive therapy with hydroxychloroquine and mycophenolate mofetil led to significant improvement in cognition and seizures. Neuropsychiatric SLE should be considered a potential differential diagnosis for patients presenting with seizures or cognitive decline. Moreover, neuropsychiatric manifestations especially seizures are an early event in the disease course of SLE. Hence, we believe that early diagnosis of SLE by neuropsychiatric manifestations will not only lead to better control of CNS symptoms but early immunosuppressive therapy could control the progression of the underlying autoimmune disease. PMID:27635183

  3. [Thyroid involvement in systemic lupus erythematosus].

    PubMed

    Pedersen, L V; Herlin, T

    1995-07-24

    Systemic lupus erythematosus (SLE) is a rare disease in childhood, and is characterized by widespread inflammation of blood vessels and connective tissue. Although the disease affects a number of different organs, thyroid involvement is not included in the classification criteria set of SLE. We describe two cases of irls with SLE who developed thyroiditis with goitre, thyroid autoantibodies, elevated serum TSH and decreased thyroid function tests. One patient had thyroiditis eighteen months before SLE was diagnosed and the other developed thyroiditis six months after the onset of SLE. Recent prospective studies have shown that thyroid involvement in SLE presenting either as hyper- or hypothyroidism is more common among children than adults. We therefore recommend that thyroid function tests should regularly be performed in juvenile SLE patients and, conversely, that child patients with Hashimoto's thyroiditis should be examined for symptoms and serology of SLE.

  4. Atypical systemic lupus erythematosus or Castleman's disease.

    PubMed

    Van de Voorde, K; De Raeve, H; De Block, C E; Van Regenmortel, N; Van Offel, J F; De Clerck, L S; Stevens, W J

    2004-01-01

    Collagen vascular diseases and malignancies have common systemic and immune features. We report a case of a 21 year old female patient with constitutional symptoms, polyserositis, spontaneous rupture of the spleen, leukocytoclastic vasculitis and acute renal failure. The tentative diagnosis of SLE was made because she developed a positive antinuclear factor (1/640), with anti-SSA antibodies and a positive lupus anticoagulans. Two months later a cervical lymphadenopathy occurred while recieving treatment with prednisolone. A lymph node biopsy revealed morphologic features of a SLE, similar to those observed in multicentric Castleman's disease (MCD). MCD is a distinct type of a lymphoproliferative disorder of unknown etiology. The difficulties in differential diagnosis of these two diseases are discussed.

  5. Atherosclerosis risk factors in systemic lupus erythematosus.

    PubMed

    Agarwal, Surabhi; Elliott, Jennifer R; Manzi, Susan

    2009-08-01

    Cardiovascular disease (CVD) has emerged as a leading cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Growing evidence suggests that inflammation plays a key role in the pathogenesis of atherosclerosis from initial endothelial dysfunction to rupture of atheromatous plaques. The increased frequency of atherosclerosis in SLE is likely due to a complex interplay among traditional risk factors, disease-related factors such as medications and disease activity, and inflammatory and immunogenic factors. Identification of these novel risk factors will lead to a better understanding of CVD pathogenesis and may also provide targets for potential treatment strategies. When caring for SLE patients, clinicians should be aware of the increased CVD risk and treat the known modifiable risk factors in addition to controlling disease activity and inflammation.

  6. Systemic lupus erythematosus--2005 annus mirabilis?

    PubMed

    Isenberg, David; Rahman, Anisur

    2006-03-01

    We are about to enter a new era in the treatment of patients with systemic lupus erythematosus (SLE). For the past 40 years hydroxychloroquine sulfate and corticosteroids, together with varying combinations of immunosuppressive drugs, have been the main treatments for SLE. Although effective for many patients, some patients fail to respond to these drugs and even more suffer from major side effects due to the generalized nature of the immunosuppression. In this article we review the remarkable confluence of new therapies ranging from newer immunosuppressive drugs with fewer side effects, such as mycophenolate mofetil, to the more targeted approaches offered by biological agents. These agents have been designed to block molecules such as CD20, CD22 and interleukin-10 that are thought to have an integral part in the development of SLE. This wolf might not yet be about to become extinct but its survival is increasingly under threat!

  7. New therapies for systemic lupus erythematosus

    PubMed Central

    Goldblatt, F; Isenberg, D A

    2005-01-01

    In the past 40 years, prognosis for patients with systemic lupus erythematosus (SLE) has improved, with 10-year survival now approximately 90%. This is due probably to a combination of earlier disease diagnosis and diagnosis of milder disease, due in part to availability of multiple serological tests for SLE, use of steroids and other immunosuppressive agents, and availability of renal dialysis and transplantation. Despite this, however, the potential for significant morbidity and mortality remains in the group of patients with partially responsive or treatment resistant disease. More recently, advancements in the understanding of molecular mechanisms involved in the pathogenesis of SLE have translated to the development of novel therapies, offering possible alternatives to this patient cohort. Discussion of these pharmacological options and ongoing research forms the basis of this review. PMID:15807843

  8. The Avise Lupus Test and Cell-bound Complement Activation Products Aid the Diagnosis of Systemic Lupus Erythematosus

    PubMed Central

    Mossell, James; Goldman, John A.; Barken, Derren; Alexander, Roberta Vezza

    2016-01-01

    Background: Systemic lupus erythematosus (SLE) is a multifaceted disease, and its diagnosis may be challenging. A blood test for the diagnosis of SLE, the Avise Lupus test, has been recently commercialized and validated in clinical studies. Objectives: To evaluate the use of the Avise Lupus test by community rheumatologists. Methods: The study is a longitudinal, case-control, retrospective review of medical charts. Cases had a positive test result, and controls had a negative result; all patients were anti-nuclear antibodies (ANA) positive but negative for SLE-specific autoantibodies. Features of SLE, diagnosis, and medications at two time points were recorded. Results: Twenty of the 23 cases (87%) and 4 of the 23 controls (17%) were diagnosed with SLE (sensitivity=83%; specificity=86%). More cases than controls (43% vs. 17%) fulfilled 4 American College of Rheumatology (ACR) classification criteria of SLE. Sensitivity of the test was significantly higher than the ACR score (83% vs. 42%, p=0.006). A higher percentage of patients who met the classification criteria had elevated cell-bound complement activation products (CB-CAPs) compared to patients who did not. Anti-rheumatic medications were used in a higher percentage of cases than controls (83% vs. 35% at baseline, p=0.002), suggesting that cases were treated more aggressively early on. Conclusion: A positive Avise Lupus test result aids in formulating a SLE diagnosis when diagnosis based on standard-of-care tests and clinical features may be challenging, and impacts patient management. Prospective studies will be performed to better evaluate the clinical utility of the test and of CB-CAPs as biomarkers of SLE. PMID:27867431

  9. Peripheral neuropathy--a rare complication of paediatric systemic lupus erythematosus.

    PubMed

    Sridhar, A V; Gosalakkal, J; Pye, I F; Houtman, P

    2004-01-01

    Peripheral neuropathy is an uncommon complication in paediatric systemic lupus erythematosus (SLE). We report the case of a 10-year-old Chinese girl who developed peripheral neuropathy within 3 months of the onset of SLE and presented with bilateral foot drop and sensory symptoms of both hands and feet. There was no involvement of the central nervous system at the time of presentation. The patient was negative for anticardiolipin antibodies, but positive for lupus anticoagulant. She was treated with intravenous methylprednisolone followed by oral steroids, methotrexate, gabapentin and amitryptyline. Although peripheral neuropathy is a rare complication of paediatric systemic lupus erythematosus, one should be vigilant for this entity as part of the neurological spectrum. It may not be associated with involvement of the central nervous system. Antiphospholipid antibodies may have role in the pathogenesis of SLE associated peripheral neuropathy. We speculate that routine nerve conduction studies may have a role in detecting sub-clinical cases.

  10. Bullous Systemic Lupus Erythematosus Associated with Esophagitis Dissecans Superficialis

    PubMed Central

    Jaffe, Eric A.

    2015-01-01

    Bullous systemic lupus erythematosus is one of the rare autoantibody mediated skin manifestation of systemic lupus erythematosus (SLE) demonstrating subepidermal blistering with neutrophilic infiltrate histologically. We present a case of a 40-year-old Hispanic female who presented with a several months' history of multiple blistering pruritic skin lesions involving the face and trunk, a photosensitive rash over the face and neck, swelling of the right neck lymph node, and joint pain involving her elbows and wrist. Her malady was diagnosed as bullous systemic lupus erythematosus based on the immunological workup and biopsy of her skin lesions. The patient also complained of odynophagia and endoscopy revealed esophagitis dissecans superficialis which is a rare endoscopic finding characterized by sloughing of the esophageal mucosa. The bullous disorders typically associated with esophagitis dissecans superficialis are pemphigus and rarely bullous pemphigoid. However, this is the first reported case of bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis. PMID:25821624

  11. Novel treatments for systemic lupus erythematosus.

    PubMed

    Gayed, Mary; Gordon, Caroline

    2010-11-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that is associated with the production of autoantibodies, and with considerable morbidity and mortality. There has been much interest in developing more specific therapies for this disease, which is currently managed with immunosuppressive drugs, predominantly corticosteroids, azathioprine, methotrexate and cyclophosphamide, in combination with hydroxychloroquine. Mycophenolate mofetil has been demonstrated to be as efficacious as cyclophosphamide in patients with lupus nephritis, and is being used increasingly in the clinic despite not being licensed for this indication. Novel methods of reducing autoantibody formation in SLE include the use of mAbs that modulate and/or deplete B-cells (anti-CD22 and anti-CD20 antibodies, respectively), or that interfere with the stimulatory effects of the soluble factor B-lymphocyte stimulator (anti-BLys antibodies). Alternative approaches include the use of atacicept (Merck Serono), a transmembrane activator and calcium modulator ligand interactor (TACI)-Ig fusion protein, which inhibits B-cell stimulation by binding to BLys and a profileration-inducing ligand (APRIL), or toleragens such as abetimus. Blocking costimulatory molecule interactions, such as the CD40-CD40 ligand interaction with mAbs and the CD28-B7 interaction with a soluble cytotoxic T-lymphocyte antigen 4 (CTLA-4)-IgG1 construct (abatacept), has also been attempted as a therapeutic strategy for SLE. The most promising strategy for a new drug for SLE is belimumab (Human Genome Sciences/GlaxoSmithKline), an anti-BLys antibody, as two phase III clinical trials with this drug recently met their primary endpoints. In this review, these novel approaches to the treatment of SLE, including the potential of targeting cytokine pathways involved in autoimmunity, are discussed.

  12. The Euro-lupus project: epidemiology of systemic lupus erythematosus in Europe.

    PubMed

    Cervera, R; Khamashta, M A; Hughes, G R V

    2009-09-01

    The Euro-lupus project provides updated information on the epidemiologic characteristics of systemic lupus erythematosus (SLE) at the change of the millennium and defines several clinical and immunological prognostic factors. The Euro-lupus cohort is composed of 1000 patients with SLE who have been followed prospectively since 1991. Among other findings, this project has shown that a) the age at onset of the disease, the gender and the autoantibody pattern, among other factors, modify the disease expression and define some specific SLE subsets; b) most of the SLE inflammatory manifestations are less common after long-term evolution of the disease, thus probably reflecting the effect of therapy as well as the progressive remission of the disease in many patients and c) a more prominent role of thrombotic events is becoming evident affecting both morbidity and mortality in SLE.

  13. Recent Advances in the Immunopathogenesis of Systemic Lupus Erythematosus

    PubMed Central

    Bardana, Emil J.; Pirofsky, Bernard

    1975-01-01

    Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory disease having definite etiologic associations with ethnic, genetic, viral and immunologic factors. Its pathologic hallmark, vasculitis, is currently felt to be the end result of an immune-complex mechanism. Several clinical and serologic variants of SLE are recognized including discoid lupus erythematosus (DLE), mixed connective tissue disease (MCTD) and drug-induced equivalents—such as procainamide-induced lupus (PIL). The distinguishing features of these variants as well as their prognosis and therapy are discussed in relation to recent developments in the immunopathogenesis of SLE. PMID:46657

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

    PubMed

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

    2016-09-01

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

  15. Ocular involvement in systemic lupus erythematosus.

    PubMed

    Preble, Janine M; Silpa-archa, Sukhum; Foster, C Stephen

    2015-11-01

    Many patients suffer from the ocular manifestations associated with systemic lupus erythematosus (SLE). Retinal vasculitis and optic neuritis are two of the most vision-threatening complications that can be associated with the disease. Ocular manifestations are often associated with wide-spread systemic inflammation which can be fatal. Thus, immediate recognition and treatment is vital for a positive outcome. There is an array of medications available to ophthalmologists for treating the ocular manifestations of SLE. Treating the underlying systemic disease is crucial, as well as treating the active ocular complications. Recently, more attention has been placed on evaluating biologic agents' efficacy in treating the systemic condition. New therapies continue to emerge that have the potential to provide benefit to patients suffering from SLE. SLE is a serious systemic condition that may first present with ocular manifestations. Thus, it is crucial for ophthalmologists to be equipped with the knowledge to detect and adequately treat the disorder to avoid vision/life-threatening complications. More research is needed to determine which therapy provides the best outcome for patients with limited side-effects.

  16. Necrotizing Polyarteritis Nodosa-like Vasculitis in a Child with Systemic Lupus Erythematosus.

    PubMed

    Nada, Ritambhra; Matthews, Joseph L; Bhattad, Sagar; Gupta, Anju; Singh, Surjit

    2017-02-15

    A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema.

  17. Macrophage subpopulations in systemic lupus erythematosus.

    PubMed

    Orme, Jacob; Mohan, Chandra

    2012-02-01

    Systemic lupus erythematosus (SLE) is a heterogeneous group of autoimmune disorders defined by a consensus of clinical and laboratory criteria. Much of the pathophysiology and therapy of SLE has focused on autoimmune B and T cells of the adaptive immune system. Recently, the role of macrophages, part of the innate immune system, in SLE pathogenesis has gained attention. The field of immunology in general has recently changed in the way that it approaches macrophages. Rather than viewing them as a single, concrete whole, it has become clear that different subpopulations of macrophages contribute to various immune and non-immune processes. Such a nomenclature may provide an ideal framework from which to study macrophage pathogenesis in SLE. Studies suggest that M1 subtype macrophages play an important inflammatory role in SLE pathogenesis. Further, apparently reduced populations of M2a and M2c subtype macrophages may contribute to the lack of anti-inflammatory activity apparent in the disease. M2b subtype macrophages may actually have a role in causing disease directly. Regulatory macrophages have yet to be explored thoroughly in SLE, though the presence of a few of their markers may mean that they are active in suppressing SLE-related inflammation.

  18. Tuberculosis in patients with systemic lupus erythematosus: Spain's situation.

    PubMed

    Arenas Miras, María del Mar; Hidalgo Tenorio, Carmen; Jimenez Alonso, Juan

    2013-01-01

    There has recently been an increase in the incidence of patients with systemic lupus erythematosus (SLE) due mainly to earlier diagnosis, and increased survival. Tuberculosis in our country is one of the most prevalent infectious diseases, and one of the underlying causes would be HIV infection and increased immigration from areas with high tuberculosis prevalence; this phenomenon is truly important in patients with autoimmune diseases, as clinical presentation, severity and prognosis of tuberculosis are often different to that of immunocompetent patients. Studies of tuberculosis in patients with SLE are scarce and inconclusive, with many doubts existing about the performance or non-tuberculous prophylaxis in this population and the absence of a protocol due to lack of conclusive studies. New techniques for diagnosis of tuberculosis (IGRAs) may be useful in this population due to higher sensitivity than Mantoux, helping avoid false negatives. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  19. [Systemic lupus erythematosus and the central nervous system].

    PubMed

    Rojas, E; Orrea Solano, M

    1993-01-01

    The central nervous system (CNS) manifestations of the chronic autoimmune disease systemic lupus erythematous (SLE) are reviewed. SLE-CNS dysfunction is broadly divided into neurologic and psychiatric clinical categories. The distinct clinical entities within these broad categories are fully described. Diagnostic criteria employed to verify the presence of SLE-CNS dysfunction, including laboratory serum and cerebral spinal fluid analyses as well as radiologic and other multimodality diagnostic tools, are compared and contrasted with respect to sensitivity and specificity.

  20. A case of lupus vulgaris successfully treated with antituberculous therapy despite negative PCR and culture.

    PubMed

    Akoglu, Gulsen; Karaduman, Aysen; Boztepe, Gonca; Ozkaya, Ozay; Sahin, Sedef; Erkin, Gul; Kolemen, Fikret

    2005-01-01

    A 14-year-old boy presented with a pink firm plaque with well-defined borders in the right infra-orbital skin area. On diascopy, the infiltrate exhibited a typical apple-jelly appearance. No acid-fast bacilli could be demonstrated. A polymerase chain reaction (PCR) assay did not reveal the presence of mycobacteria in a lesional biopsy sample. Culture of biopsied tissue on Loewenstein-Jensen medium was negative. Although the tuberculosis culture and PCR did not confirm tuberculosis, a diagnosis of lupus vulgaris was made considering the clinical and histopathological findings. After a 9-month antituberculous therapy, the lesion disappeared. We believe that a diagnosis of lupus vulgaris still depends more on clinical and histopathological findings than on tuberculosis culture or PCR.

  1. Intestinal Dysbiosis Associated with Systemic Lupus Erythematosus

    PubMed Central

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

    2014-01-01

    ABSTRACT Systemic lupus erythematosus (SLE) is the prototypical systemic autoimmune disease in humans and is characterized by the presence of hyperactive immune cells and aberrant antibody responses to nuclear and cytoplasmic antigens, including characteristic anti–double-stranded DNA antibodies. We performed a cross-sectional study in order to determine if an SLE-associated gut dysbiosis exists in patients without active disease. A group of 20 SLE patients in remission, for which there was strict inclusion and exclusion criteria, was recruited, and we used an optimized Ion Torrent 16S rRNA gene-based analysis protocol to decipher the fecal microbial profiles of these patients and compare them with those of 20 age- and sex-matched healthy control subjects. We found diversity to be comparable based on Shannon’s index. However, we saw a significantly lower Firmicutes/Bacteroidetes ratio in SLE individuals (median ratio, 1.97) than in healthy subjects (median ratio, 4.86; P < 0.002). A lower Firmicutes/Bacteroidetes ratio in SLE individuals was corroborated by quantitative PCR analysis. Notably, a decrease of some Firmicutes families was also detected. This dysbiosis is reflected, based on in silico functional inference, in an overrepresentation of oxidative phosphorylation and glycan utilization pathways in SLE patient microbiota. PMID:25271284

  2. Metabolic Disturbances Associated with Systemic Lupus Erythematosus

    PubMed Central

    Wu, Tianfu; Xie, Chun; Han, Jie; Ye, Yujin; Weiel, Jim; Li, Quan; Blanco, Irene; Ahn, Chul; Olsen, Nancy; Putterman, Chaim; Saxena, Ramesh; Mohan, Chandra

    2012-01-01

    The metabolic disturbances that underlie systemic lupus erythematosus are currently unknown. A metabolomic study was executed, comparing the sera of 20 SLE patients against that of healthy controls, using LC/MS and GC/MS platforms. Validation of key differences was performed using an independent cohort of 38 SLE patients and orthogonal assays. SLE sera showed evidence of profoundly dampened glycolysis, Krebs cycle, fatty acid β oxidation and amino acid metabolism, alluding to reduced energy biogenesis from all sources. Whereas long-chain fatty acids, including the n3 and n6 essential fatty acids, were significantly reduced, medium chain fatty acids and serum free fatty acids were elevated. The SLE metabolome exhibited profound lipid peroxidation, reflective of oxidative damage. Deficiencies were noted in the cellular anti-oxidant, glutathione, and all methyl group donors, including cysteine, methionine, and choline, as well as phosphocholines. The best discriminators of SLE included elevated lipid peroxidation products, MDA, gamma-glutamyl peptides, GGT, leukotriene B4 and 5-HETE. Importantly, similar elevations were not observed in another chronic inflammatory autoimmune disease, rheumatoid arthritis. To sum, comprehensive profiling of the SLE metabolome reveals evidence of heightened oxidative stress, inflammation, reduced energy generation, altered lipid profiles and a pro-thrombotic state. Resetting the SLE metabolome, either by targeting selected molecules or by supplementing the diet with essential fatty acids, vitamins and methyl group donors offers novel opportunities for disease modulation in this disabling systemic autoimmune ailment. PMID:22723834

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

    PubMed Central

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

    2011-01-01

    Objective Systemic lupus erythematosus is a clinically heterogeneous autoimmune disease. A number of genetic loci that increase lupus susceptibility have been established. This study examines if these genetic loci also contribute to the clinical heterogeneity in lupus. Materials and methods 4001 European-derived, 1547 Hispanic, 1590 African-American and 1191 Asian lupus patients were genotyped for 16 confirmed lupus susceptibility loci. Ancestry informative markers were genotyped to calculate and adjust for admixture. The association between the risk allele in each locus was determined and compared in patients with and without the various clinical manifestations included in the ACR criteria. Results Renal disorder was significantly correlated with the lupus risk allele in ITGAM (p=5.0×10−6, OR 1.25, 95% CI 1.12 to 1.35) and in TNFSF4 (p=0.0013, OR 1.14, 95% CI 1.07 to 1.25). Other significant findings include the association between risk alleles in FCGR2A and malar rash (p=0.0031, OR 1.11, 95% CI 1.17 to 1.33), ITGAM and discoid rash (p=0.0020, OR 1.20, 95% CI 1.06 to 1.33), STAT4 and protection from oral ulcers (p=0.0027, OR 0.89, 95% CI 0.83 to 0.96) and IL21 and haematological disorder (p=0.0027, OR 1.13, 95% CI 1.04 to 1.22). All these associations are significant with a false discovery rate of <0.05 and pass the significance threshold using Bonferroni correction for multiple testing. Conclusion Significant associations were found between lupus clinical manifestations and the FCGR2A, ITGAM, STAT4, TNSF4 and IL21 genes. The findings suggest that genetic profiling might be a useful tool to predict disease manifestations in lupus patients in the future. PMID:21719445

  4. Juvenile systemic lupus erythematosus in Nigeria.

    PubMed

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

    2017-03-01

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

  5. Prolidase deficiency and systemic lupus erythematosus

    PubMed Central

    Shrinath, M; Walter, J; Haeney, M; Couriel, J; Lewis, M; Herrick, A

    1997-01-01

    Accepted 31 January 1997
 Two children with prolidase deficiency, an inborn error of proline metabolism, developed clinical and immunological abnormalities consistent with a diagnosis of systemic lupus erythematosus (SLE). The first child died from septicaemia, and SLE was only diagnosed during his terminal illness. As a result of this diagnosis his cousin, who was already known to have prolidase deficiency, was investigated further and a diagnosis of SLE confirmed. Following treatment with oral prednisolone her clinical condition has improved, although she has a persistently raised erythrocyte sedimentation rate (ESR) and florid facial rash. Both prolidase deficiency and SLE are associated with disturbances in immune function and have clinical features in common. It is likely that prolidase deficiency is a risk factor for the development of SLE. Additionally, patients with SLE should—where there is a family history or presentation in childhood—be specifically investigated for prolidase deficiency, since standard immunological or haematological investigations will not identify the characteristic biochemical abnormalities.

 PMID:9196362

  6. Unmet medical needs in systemic lupus erythematosus

    PubMed Central

    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

  7. Derivation and Validation of Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus

    PubMed Central

    Petri, Michelle; Orbai, Ana-Maria; Alarcón, Graciela S.; Gordon, Caroline; Merrill, Joan T.; Fortin, Paul R.; Bruce, Ian N.; Isenberg, David; Wallace, Daniel J.; Nived, Ola; Sturfelt, Gunnar; Ramsey-Goldman, Rosalind; Bae, Sang-Cheol; Hanly, John G.; Sanchez-Guerrero, Jorge; Clarke, Ann; Aranow, Cynthia; Manzi, Susan; Urowitz, Murray; Gladman, Dafna; Kalunian, Kenneth; Costner, Melissa; Werth, Victoria P.; Zoma, Asad; Bernatsky, Sasha; Ruiz-Irastorza, Guillermo; Khamashta, Munther A.; Jacobsen, Soren; Buyon, Jill P.; Maddison, Peter; Dooley, Mary Anne; van Vollenhoven, Ronald F.; Ginzler, Ellen; Stoll, Thomas; Peschken, Christine; Jorizzo, Joseph L.; Callen, Jeffrey P.; Lim, S. Sam; Fessler, Barri J.; Inanc, Murat; Kamen, Diane L.; Rahman, Anisur; Steinsson, Kristjan; Franks, Andrew G.; Sigler, Lisa; Hameed, Suhail; Fang, Hong; Pham, Ngoc; Brey, Robin; Weisman, Michael H.; McGwin, Gerald; Magder, Laurence S.

    2012-01-01

    Objective The Systemic Lupus Collaborating Clinics (SLICC) revised and validated the American College of Rheumatology (ACR) SLE classification criteria in order to improve clinical relevance, meet stringent methodology requirements and incorporate new knowledge in SLE immunology. Methods The classification criteria were derived from a set of 702 expert-rated patient scenarios. Recursive partitioning was used to derive an initial rule that was simplified and refined based on SLICC physician consensus. SLICC validated the classification criteria in a new validation sample of 690 SLE patients and controls. Results Seventeen criteria were identified. The SLICC criteria for SLE classification requires: 1) Fulfillment of at least four criteria, with at least one clinical criterion AND one immunologic criterion OR 2) Lupus nephritis as the sole clinical criterion in the presence of ANA or anti-dsDNA antibodies. In the derivation set, the SLICC classification criteria resulted in fewer misclassifications than the current ACR classification criteria (49 versus 70, p=0.0082), had greater sensitivity (94% versus 86%, p<0.0001) and equal specificity (92% versus 93%, p=0.39). In the validation set, the SLICC Classification criteria resulted in fewer misclassifications (62 versus 74, p=0.24), had greater sensitivity (97% versus 83%, p<0.0001) but less specificity (84% versus 96%, p<0.0001). Conclusions The new SLICC classification criteria performed well on a large set of patient scenarios rated by experts. They require that at least one clinical criterion and one immunologic criterion be present for a classification of SLE. Biopsy confirmed nephritis compatible with lupus (in the presence of SLE autoantibodies) is sufficient for classification. PMID:22553077

  8. Gastrointestinal system manifestations in juvenile systemic lupus erythematosus.

    PubMed

    Sönmez, Hafize Emine; Karhan, Asuman Nur; Batu, Ezgi Deniz; Bilginer, Yelda; Gümüş, Ersin; Demir, Hülya; Yüce, Aysel; Özen, Seza

    2017-02-16

    Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal anti-inflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

  9. Indications for colonoscopy in patients with systemic lupus erythematosus.

    PubMed

    Iwamuro, Masaya; Okada, Hiroyuki; Kato, Jun; Tanaka, Takehiro; Sada, Ken-Ei; Makino, Hirofumi; Yamamoto, Kazuhide

    2013-01-01

    Systemic lupus erythematosus is a systemic autoimmune disorder that sometimes involves the gastrointestinal tract. The aim of this study is to describe the clinical characteristics of patients with systemic lupus erythematosus with colorectal involvement, and to provide criteria for colonoscopy. Among 288 patients with systemic lupus erythematosus, 29 patients underwent colonoscopy. The clinical backgrounds were comparatively analyzed between the patients with colorectal involvements (n = 11, group A) and the patients without colorectal involvements (n = 18, group B). Endoscopic features were also evaluated in group A patients. The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) of the group A patients was higher than that of the group B patients. Abdominal pain (n = 6) and diarrhea (n = 5) were significantly correlated with the presence of colorectal involvements, and other manifestations in group A patients included visible blood in stools (n = 5) and fever (n = 1). In colonoscopy, discrete ulcers (n = 5), longitudinal ulcers (n = 1), erosions and/or small ulcers (n = 2), edematous mucosa (n = 2), and concurrent ulcerative colitis (n = 1) were identified. Patients with systemic lupus erythematosus with SLEDAI scores > or = 5, or with gastrointestinal symptoms, particularly those who present with abdominal pain or diarrhea should undergo colonoscopy, because these patients are likely to have mucosal damage in the colorectum.

  10. Novel approaches to therapy for systemic lupus erythematosus: update 2005.

    PubMed

    Zandman-Goddard, Gisele; Orbach, Hedi; Shoenfeld, Yehuda

    2005-07-01

    This review covers the major advances in the therapeutic potentials related to systemic lupus erythematosus published in Medline between 2000 and February 2005. Controlled, open and Phase I-III trials were included. Anecdotal reports were excluded. Several trials have defined the role of cyclophosphamide, methotrexate, antimalarials, hormonal treatment and mycophenolate mofetil (Cellcept) in the management of systemic lupus erythematosus. The aims of novel biologics for systemic lupus erythematosus are to target the autoimmune disease at different points: B-cell depletion (rituximab [Rituxan], anti-BLys antibodies [Lymphostat-B]), inhibition of T-B interaction (rituximab), blockade of cytokines (anti-interleukin-10 antibodies), manipulation of idiotypes (intravenous immunoglobulin), tolerance induction to DNA and immunoglobulin-peptides and peptide therapy (abetimus sodium [Riquent]). Low-dose intravenous cyclophosphamide (Euro-Lupus protocol) is as effective as the conventional National Institutes of Health protocol and is also associated with less toxicity. Stem cell transplantation for severe disease induces remission in most patients, however, the relapse rate in a third of patients and the associated morbity and mortality restricts its use to selected patients with life-threatening disease. Intravenous immunoglobulin, although utilized in open trials, is effective and safe for various manifestations of systemic lupus erythematosus. Major advances have been associated with mycophenolate mofetil and rituximab. Mycophenolate mofetil is effective for induction and maintenance therapy of lupus proliferative glomerulonephritis and is associated with fewer adverse events than monthly intravenous cyclophosphamide. Rituximab is a promising agent, and although its utilization is presently limited, it appears to be effective for lupus patients with severe disease.

  11. [B lymphocyte stimulator in systemic lupus erythematosus].

    PubMed

    Mercado, Ulises

    2012-01-01

    The B lymphocyte stimulator (BLyS) is an essential protein for the growth and survival of B cells. BLyS is expressed on monocytes, macrophages, and dendritic cells. BLyS binds to three receptors on B cells: BAFF-R, BCMA, and TACI. BLyS overexpression in mice leads to lupus-like syndrome, but not in all, whereas BLyS deficient mice results in a block of B cell development. High serum levels of BLyS can be detected in patients with lupus and rheumatoid arthritis. BLyS antagonists are an attractive target for treating autoimmune diseases.

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

    PubMed Central

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

    2009-01-01

    Objective To determine the features associated with acute onset systemic lupus erythaematosus (SLE). Methods 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 ≥4 American College of Rheumatology (ACR) criteria for the classification of SLE in ≤4 weeks. Socioeconomic demographic features, clinical manifestations, disease activity, damage accrual, mortally, autoantibodies. HLA class II and FCGR alleles, behavioural/psychological variables were compared between patients with acute and insidious disease onset by univariable (χ2 and Student t test) and multivariable (stepwise logistic regression) analyses. Results 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. Conclusions 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. PMID:17720721

  13. Interarm Blood Pressure Difference in Patients With Systemic Lupus Erythematosus.

    PubMed

    Ye, Yicong; Ding, Faming; Li, Mentao; Yang, Xinglin; Pang, Haiyu; Wang, Qian; Xu, Dong; Kang, Lin; Zeng, Xiaofeng; Zhang, Shuyang

    2015-08-01

    This study was performed to determine the relationship between systemic lupus erythematosus (SLE) and the interarm blood pressure difference (IAD) and to elucidate the role of the IAD as a surrogate marker for early detection of peripheral artery disease (PAD) in patients with SLE. In total, 135 patients with SLE and 135 age- and gender-matched subjects were enrolled. The IAD and risk of an abnormal IAD were compared between the SLE and control groups, and logistic regression analysis was performed to determine the relationship between SLE and an abnormal IAD. The specificity and sensitivity of an IAD of 10 mm Hg or greater for diagnosis of PAD (ankle brachial index of <0.90) were calculated. Both the systolic and diastolic IADs were significantly higher in the SLE group than in the control group (P < 0.001). After adjustment for confounding factors, SLE remained significantly associated with an abnormal IAD (P = 0.039). Both the systolic and diastolic IADs were negatively associated with the ankle brachial index. Using a systolic IAD of 10 mm Hg or greater as the cutoff point, the specificity and sensitivity for PAD were 90% and 41%, respectively. A diastolic IAD of 10 mm Hg or greater exhibited higher specificity (92%), but lower sensitivity (30%). Systemic lupus erythematosus is independently associated with an abnormal IAD, and an IAD of 10 mm Hg or greater predicts PAD with high specificity but low sensitivity. Blood pressure should be measured at least once in both arms in patients with SLE for early detection of asymptomatic PAD.

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

    SciTech Connect

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

    1984-08-01

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

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

    PubMed

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

    2017-04-01

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

  16. Nuclear Pore Protein p62 Autoantibodies in Systemic Lupus Erythematosus

    PubMed Central

    Kraemer, Doris M; Tony, Hans-Peter

    2010-01-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which is classically characterised by a variety of autoantibodies to deoxyribonucleic acid (DNA), ribonucleic acid (RNA), other nuclear and cytoplasmic antigens. Recently several novel autoantibodies against a variety of specific nuclear pore proteins have been described, including the nucleoporin p62. In this paper we evaluate anti-nucleoporin p62 antibodies by western blot analysis in 25 systemic lupus erythematosus patients. Six patients showed antibodies directed against nucleoporin p62. Our data indicate that p62 antibodies could be a useful additional marker in SLE. PMID:20648220

  17. 75 FR 35493 - Guidance for Industry on Systemic Lupus Erythematosus-Developing Medical Products for Treatment...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-06-22

    ... Systemic Lupus Erythematosus--Developing Medical Products for Treatment; Availability AGENCY: Food and Drug... availability of a guidance for industry entitled ``Systemic Lupus Erythematosus--Developing Medical Products..., therapeutic biological products, and medical devices for the treatment of systemic lupus erythematosus......

  18. [Plasma homocysteine levels in systemic lupus erythematosus].

    PubMed

    Martínez-Berriotxoa, Agustín; Ruiz-Irastorza, Guillermo; Egurbide Arberas, María Victoria; Rueda Gutiérrez, Miguel; Aguirre Errasti, Ciriaco

    2003-05-17

    Cardiovascular disease is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). An association between hyperhomocysteinemia and increased cardiovascular risk has been reported. On the other hand, renal failure and deficiency of vitamin B12 and/or folic acid are common causes of hyperhomocysteinemia. The aims of this study were to determine plasma total homocystein (tHcy) concentrations in SLE patients and to analyze the association of plasma tHcy with age, sex, plasma creatinine, vitamin B12, folates and total cholesterol, as well as with other clinical conditions linked to atherothrombosis in SLE patients. Fasting plasma levels of tHcy, vitamin B12, folates, total cholesterol and creatinine were measured in 94 SLE patients (11 males, 83 females) and in a control group of 308 healthy volunteers (122 males, 186 females). A review of the medical records of SLE patients was performed. Plasma tHcy concentrations were higher in patients with SLE (median 10.54 (mol/L) than in controls (median 8.49 (mol/L, p < 0.001). Hyperhomocysteinemia (tHcy >=15 (mol/L) was found in 17.02% SLE patients. In a multivariate analysis, plasma creatinine (p < 0.001), total cholesterol (p = 0.038), male sex (p = 0.003) and smoking (p = 0.001) were associated with higher plasma tHcy concentrations. No associations were found between plasma tHcy and hypertension, SLE duration, prednisone therapy and antiphospholipid antibodies. Plasma tHcy concentrations are higher in SLE patients than in healthy controls. High concentrations of plasma creatinine and total plasma cholesterol, male sex and smoking are associated with a higher concentration of plasma tHcy in SLE. Since the clinical consequences of hyperhomocysteinemia are not well established, routine determination of plasmatic tHcy and supplemental therapy in patients with high levels of tHcy are not recommended.

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

    PubMed

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

    2011-10-01

    Systemic lupus erythematosus is a clinically heterogeneous autoimmune disease. A number of genetic loci that increase lupus susceptibility have been established. This study examines if these genetic loci also contribute to the clinical heterogeneity in lupus. 4001 European-derived, 1547 Hispanic, 1590 African-American and 1191 Asian lupus patients were genotyped for 16 confirmed lupus susceptibility loci. Ancestry informative markers were genotyped to calculate and adjust for admixture. The association between the risk allele in each locus was determined and compared in patients with and without the various clinical manifestations included in the ACR criteria. Renal disorder was significantly correlated with the lupus risk allele in ITGAM (p=5.0 × 10(-6), OR 1.25, 95% CI 1.12 to 1.35) and in TNFSF4 (p=0.0013, OR 1.14, 95% CI 1.07 to 1.25). Other significant findings include the association between risk alleles in FCGR2A and malar rash (p=0.0031, OR 1.11, 95% CI 1.17 to 1.33), ITGAM and discoid rash (p=0.0020, OR 1.20, 95% CI 1.06 to 1.33), STAT4 and protection from oral ulcers (p=0.0027, OR 0.89, 95% CI 0.83 to 0.96) and IL21 and haematological disorder (p=0.0027, OR 1.13, 95% CI 1.04 to 1.22). All these associations are significant with a false discovery rate of <0.05 and pass the significance threshold using Bonferroni correction for multiple testing. Signifi cant associations were found between clinical manifestations and the FCGR2A, ITGAM, STAT4, TNSF4 and IL21 genes. The findings suggest that genetic profiling might be a useful tool to predict disease manifestations in lupus patients in the future.

  20. Ethnicity and mortality from systemic lupus erythematosus in the US

    PubMed Central

    Krishnan, E; Hubert, H B

    2006-01-01

    Objective To study ethnic differences in mortality from systemic lupus erythematosus (lupus) in two large, population‐based datasets. Methods We analysed the national death data (1979–98) from the National Center for Health Statistics (Hyattsville, Maryland, USA) and hospitalisation data (1993–2002) from the Nationwide Inpatient Sample (NIS), the largest hospitalisation database in the US. Results The overall, unadjusted, lupus mortality in the National Center for Health Statistics data was 4.6 per million, whereas the proportion of in‐hospital mortality from the NIS was 2.9%. African‐Americans had disproportionately higher mortality risk than Caucasians (all‐cause mortality relative risk adjusted for age = 1.24 (women), 1.36 (men); lupus mortality relative risk = 3.91 (women), 2.40 (men)). Excess risk was found among in‐hospital deaths (odds ratio adjusted for age = 1.4 (women), 1.3 (men)). Lupus death rates increased overall from 1979 to 98 (p<0.001). The proportional increase was greatest among African‐Americans. Among Caucasian men, death rates declined significantly (p<0.001), but rates did not change substantially for African‐American men. The African‐American:Caucasian mortality ratio rose with time among men, but there was little change among women. In analyses of the NIS data adjusted for age, the in‐hospital mortality risk decreased with time among Caucasian women (p<0.001). Conclusions African‐Americans with lupus have 2–3‐fold higher lupus mortality risk than Caucasians. The magnitude of the risk disparity is disproportionately higher than the disparity in all‐cause mortality. A lupus‐specific biological factor, as opposed to socioeconomic and access‐to‐care factors, may be responsible for this phenomenon. PMID:16627544

  1. Abdominal manifestations in childhood‐onset systemic lupus erythematosus

    PubMed Central

    Richer, O; Ulinski, T; Lemelle, I; Ranchin, B; Loirat, C; Piette, J C; Pillet, P; Quartier, P; Salomon, R; Bader‐Meunier, B

    2007-01-01

    Background Childhood‐onset lupus erythematosus is a rare disorder of unknown origin. Objectives To describe the frequency of gastrointestinal manifestations at presentation of systemic lupus erythematosus SLE and at follow‐up, and discuss the specific causes of these manifestations. Methods Medical records of 201 patients with childhood‐onset SLE followed up in French paediatric nephrological, haematological and rheumatological centres were reviewed and abstracted for gastrointestinal manifestations. Results Gastrointestinal involvement was recorded in 39 (19%) children. The median (range) age at the time of initial gastrointestinal manifestations was 11.3 (4.5–16) years. Gastrointestinal symptoms were present at or occurred within 1 month after diagnosis in 32% patients. Abdominal pain was the most frequent symptom, present in 34 (87%) patients. It was mostly related to lupus involvement, especially ascites (n = 14) and pancreatitis (n = 12), more rarely to treatment‐induced events (n = 1) or infection (n = 1) and never to events unrelated to SLE. Three children with surgical abdomen underwent a laparotomy before SLE was diagnosed, with a final diagnosis of lupus peritonitis and lupus acalculous cholecystitis. C reactive protein values were <40 mg/l in all but two patients who had surgical abdomen. Abdominal ultrasonography and computed tomography scans were abnormal in 58% and 83% of the evaluated patients, respectively. Corticosteroids, associated with intravenous cyclophospamide in eight patients, led to complete remission of gastrointestinal involvement in 30 of 31 treated patients. Conclusion Gastrointestinal involvement is common in children with SLE, and is mainly due to primary lupus involvement. Corticoidsteroid treatment should be promptly considered in children with lupus presenting with abdominal pain after infectious disease; side effects of treatment and intestinal perforation have been excluded. PMID:16818463

  2. Pregnancy implications for systemic lupus erythematosus and the antiphospholipid syndrome.

    PubMed

    Andreoli, Laura; Fredi, Micaela; Nalli, Cecilia; Reggia, Rossella; Lojacono, Andrea; Motta, Mario; Tincani, Angela

    2012-05-01

    Multidisciplinary approach and patient counselling have been the key points in the improvement of the management of pregnancy in women with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Most of these women can have successful pregnancy when thoroughly informed and instructed on several different issues. Disease activity should be in stable remission prior to pregnancy in order to reduce the chance for flare during pregnancy. To this purpose, medications must be modulated: "safe" drugs should be continued throughout pregnancy, embryotoxic/foetotoxic drugs should be withdrawn timely, and beneficial drugs such as low dose aspirin and heparin should be added for prophylaxis of maternal and foetal outcome, especially in the presence of antiphospholipid antibodies. The safety profile of anti-rheumatic drugs during pregnancy and breastfeeding should be kept constantly updated, as new data from inadvertent exposure accumulates and new drugs (especially biological agents) are available. Patients may carry autoantibodies that can negatively affect the baby, being neonatal lupus the prototypical case of passively acquired autoimmunity. Research has been greatly active in this field and more information on risk stratification and management are now available for counselling. The effect of both autoantibodies and drug exposure has been evaluated in the offspring: some concerns about learning disabilities have been raised, but these are treatable conditions that are likely to be overcome. To counsel a woman with SLE/APS during childbearing age means also to deal with contraception. Despite the "preferred choice" - combined oral contraceptive - may not be suitable for most of the patients, other options are available and should be offered and discussed with the patient. Fertility is not generally affected in SLE/APS patients, but those cases who require assisted reproduction techniques should be carefully evaluated and managed.

  3. Risk factors of systemic lupus erythematosus flares during pregnancy.

    PubMed

    Jara, Luis J; Medina, Gabriela; Cruz-Dominguez, Pilar; Navarro, Carmen; Vera-Lastra, Olga; Saavedra, Miguel A

    2014-12-01

    This review examines the risk factors for the development of systemic lupus erythematosus (SLE) flares during pregnancy. In preconception, anti-DNA, hypocomplementemia, previous thrombosis, triple antiphospholipid (aPL) antibody positivity, active lupus nephritis and discontinuation of medications such as hydroxychloroquine and azathioprine are factors associated with pregnancy failure. During pregnancy, SLE flares are associated with aPL antibodies, synergic changes of pregnancy on Th1 and TH2 cytokines, other cytokines and chemokines that interact with hormones such as estrogen and prolactin that amplify the inflammatory effect. From the clinical point of view, SLE activity at pregnancy onset, thrombocytopenia, lupus nephritis, arterial hypertension, aPL syndromes, preeclampsia is associated with lupus flares and fetal complications. In puerperium, the risk factors of flares are similar to pregnancy. Hyperactivity of immune system, autoantibodies, hyperprolactinemia, active lupus nephritis, decrease in TH2 cytokines with increase in TH1 cytokines probably participate in SLE flare. The SLE flares during pregnancy make the difference between an uncomplicated pregnancy and pregnancy with maternal and fetal complications. Therefore, the knowledge of risk factors leads the best treatment strategies to reduce flares and fetal complications in SLE patients.

  4. The dissociation of arterial hypertension and lupus glomerulonephritis in systemic lupus erythematosus.

    PubMed

    Petrin, J; Rozman, B; Dolenc, P; Logar, D; Bozic, B; Vizjak, A; Ferluga, D; Jezersek, P

    1993-06-01

    In spite of several articles questioning the general opinion that arterial hypertension in patients with systemic lupus erythematosus (SLE) is only the consequence of lupus glomerulonephritis (LGN), this still remains the usual pathophysiologic explanation. The purpose of this study was to explore the correlations between hypertension and LGN and to assess the importance of hypertension control for the prognosis of patients. A retrospective analysis of 173 patients with SLE over a period of 14 years was performed. For most of the patients, data were available from regular follow-up visits over an average of 6 years. Our results show a dissociation of hypertension and LGN and an association of hypertension and renal dysfunction. Severe hypertensive renal vascular lesions correlated well with a decrease of renal function. Successful treatment of hypertension is therefore essential in order to prevent deterioration of renal function in patients with LGN.

  5. Postextraction hemorrhage in a young male patient with systemic lupus erythematosus.

    PubMed

    Schwartz, S; Esseltine, D W

    1984-03-01

    A case of a 13-year-old boy with prolonged bleeding after tooth extraction is reported. This was the first manifestation of systemic lupus erythematosus found to be associated with circulating anticoagulants, including the "lupus anticoagulant," and possible hypoprothrombinemia.

  6. Infection in systemic lupus erythematosus: friend or foe?

    PubMed Central

    Francis, Lisa; Perl, Andras

    2010-01-01

    Infectious agents have long been implicated in the pathogenesis of systemic lupus erythematosus. Common viruses, such as the Epstein-Barr virus, transfusion transmitted virus, parvovirus and cytomegalovirus, have an increased prevalence in patients with systemic lupus erythematosus. They may contribute to disease pathogenesis through triggering autoimmunity via structural or functional molecular mimicry, encoding proteins that induce cross-reactive immune responses to self antigens or modulate antigen processing, activation, or apoptosis of B and T cells, macrophages or dendritic cells. Alternatively, some infectious agents, such as malaria, Toxoplasma gondii and Helicobacter pylori, may have a protective effect. Vaccinations may play dual roles by protecting against friend and foe alike. PMID:20209114

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

    PubMed

    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.

  8. Streptococcus pneumoniae necrotizing fasciitis in systemic lupus erythematosus.

    PubMed

    Sánchez, A; Robaina, R; Pérez, G; Cairoli, E

    2016-04-01

    Necrotizing fasciitis is a rapidly progressive destructive soft tissue infection with high mortality. Streptococcus pneumoniae as etiologic agent of necrotizing fasciitis is extremely unusual. The increased susceptibility to Streptococcus pneumoniae infection in patients with systemic lupus erythematosus is probably a multifactorial phenomenon. We report a case of a patient, a 36-year-old Caucasian female with 8-year history of systemic lupus erythematosus who presented a fatal Streptococcus pneumoniae necrotizing fasciitis. The role of computed tomography and the high performance of blood cultures for isolation of the causative microorganism are emphasized. Once diagnosis is suspected, empiric antibiotic treatment must be prescribed and prompt surgical exploration is mandatory.

  9. Kikuchi–Fujimoto disease and systemic lupus erythematosus

    PubMed Central

    Baenas, Diego F; Diehl, Fernando A; Haye Salinas, María J; Riva, Verónica; Diller, Ana; Lemos, Pablo A

    2016-01-01

    Kikuchi–Fujimoto disease, or histiocytic necrotizing lymphadenitis, is an infrequent idiopathic disorder. It has been associated with autoimmune disorders, of which systemic lupus erythematosus is the most outstanding. The basis of its diagnosis relies on the histological examination of lymph nodes, which typically reveals necrosis surrounded by histiocytes with crescentic nucleus, immunoblasts and plasma cells, and absence of neutrophils. We report the case of a 27-year-old Argentinian female patient without any relevant past medical history to demonstrate the correlation between Kikuchi–Fujimoto disease and systemic lupus erythematosus. PMID:27418858

  10. Immunological studies of the placenta in systemic lupus erythematosus.

    PubMed Central

    Grennan, D M; McCormick, J N; Wojtacha, D; Carty, M; Behan, W

    1978-01-01

    An immunological study was made of the placentae from 5 mothers with lupus erythematosus. 3 of the 5 mothers had anti-DNA antibodies in their sera at the time of delivery and in one of these anti-DNA antibodies were detected in the cord blood. This patient had active renal disease and serological evidence suggestive of circulating immune complexes in her blood at the time of delivery. Immunofluorescence studies showed granular deposition of immunoglobulin and C3 on the trophoblast basement membrane similar to that previously described on the glomerular basement membrane in systemic lupus erythematosus. Anti-DNA antibodies were eluted from the placenta in this case. We suggest that immune complex deposition on the trophoblast basement membrane in patients with active systemic lupus erythematosus may play a part in the increased fetal mortality in this disease. Images PMID:348129

  11. Bullous Systemic Lupus Erythematosus and Lupus Nephritis in a Young Girl

    PubMed Central

    Momen, Tooba; Madihi, Yahya

    2016-01-01

    Bullous systemic lupus erythematosus (BSLE) is an autoimmune blistering disease occurring in patients with systemic lupus erythematosus (SLE). It is a rare disease, especially in children. A 14-year-old girl initially presented with fatigue, generalized vesiculobullous skin lesions, and ulcers over the hard palate and oral mucosa. Clinical investigations revealed hematuria and proteinuria, a high erythrocyte sedimentation rate and titer of antinuclear antibody, and anti-double-stranded DNA. Skin biopsy findings were suggestive of BSLE. A renal biopsy confirmed the features of class V lupus nephritis. Based on the clinical features and investigations, a diagnosis of BSLE with nephritis was made. She received methylprednisolone pulse therapy and hydroxychloroquine; however, it did not alleviate the vesiculobullous eruption, so treatment with dapsone started and resulted in the dramatic disappearance of the lesions. Interruption of dapsone due to hemolysis did not aggravate the bullous disease. During follow-up, she had multiple flare-ups of disease and nephritis without rebound of bullous lesions. BSLE is a rare presentation of SLE in children. Differentiating it from other skin bullous diseases and SLE with blister is important for the correct management. The unusual presentation of this disease may delay the diagnosis and therefore requires a high index of clinical suspicion. PMID:27974963

  12. [Systemic lupus erythematous and CD24v].

    PubMed

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

    2015-01-01

    Antecedentes: el lupus eritematoso sistémico es un padecimiento autoinmunitario, de origen multifactorial, con predisposición genética; más de 100 genes participan en su etiopatogenia. El gen de CD24 puede mediar varias funciones, como su actividad coestimuladora en la expansión clonal de las células T. El polimorfismo de un simple nucleótido de CD24, que resulta en un reemplazo no conservador de alanina a valina (CD24v), que precede inmediatamente al sitio de anclaje GPI (posición ω-1), condiciona la pérdida de actividad de CD24. Se ha descrito que CD24v está asociado con esclerosis múltiple y lupus eritematoso sistémico en otras poblaciones. Objetivo: encontrar la existencia de CD24v en pacientes mexicanos con lupus eritematoso sistémico. Material y método: estudio de genotipificación de CD24v en el que se incluyeron 64 sujetos, 32 casos con lupus eritematoso sistémico: 28 mujeres y 4 hombres; y 32 controles: 9 mujeres y 23 hombres; todos eran pacientes con lupus eritematoso sistémico del Centro Médico Nacional 20 de Noviembre, del ISSSTE, atendidos en los servicios de Inmunología Clínica y Reumatología. Resultados: de los casos, 19 pacientes tenían genotipo homocigoto silvestre, 12 con genotipo heterocigotos y sólo un paciente mostró el polimorfismo en estado homocigoto. De los controles, 17 sujetos mostraron genotipos heterocigotos silvestres, 14 eran heterocigotos y sólo en uno se encontró que era homocigoto polimórfico. Se obtuvo una razón de momios de 0.84 y chi cuadrada de 0.17, por lo que no hubo diferencia estadísticamente significativa. Conclusiones: se demostró que no hay diferencia estadísticamente significativa entre pacientes con lupus eritematoso sistémico y controles respecto a la existencia de CD24v.

  13. Ultraviolet-A1 irradiation therapy for systemic lupus erythematosus.

    PubMed

    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

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

    PubMed

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

    2017-05-01

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

  15. Ultraviolet-A1 irradiation therapy for systemic lupus erythematosus

    PubMed Central

    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

  16. Prolactin has a pathogenic role in systemic lupus erythematosus.

    PubMed

    Jara, Luis J; Medina, Gabriela; Saavedra, Miguel A; Vera-Lastra, Olga; Torres-Aguilar, Honorio; Navarro, Carmen; Vazquez Del Mercado, Monica; Espinoza, Luis R

    2017-01-28

    Prolactin, a 23-kDa peptide hormone, is produced by the anterior pituitary gland and extrapituitary sites including the immune cells. Prolactin (PRL) participates in innate and adaptive immune response. PRL stimulates the immune cells by binding to receptor (PRL-R). Binding of PRL to its receptor activates the Janus kinase-signal transducer (JAK-STAT). Activation of these cascades results in endpoints such as immunoestimulator and immunosupressor action. Prolactin belongs to the network of immune-neuroendocrine interaction. Hyperprolactinemia has been found in patients with systemic lupus erythematosus (SLE), and new evidence has confirmed a significant correlation between serum PRL levels and disease activity. PRL participates in activation of SLE during pregnancy and in pathogenesis of lupus nephritis, neuropsychiatric, serosal, hematologic, articular, and cutaneous involvement. Hyperprolactinemia was associated with increase IgG concentrations, anti-DNA antibodies, immune complex, glomerulonephritis, and accelerated mortality in murine lupus. Bromocriptine, a dopamine analog that suppresses PRL secretion, was associated with decreased lupus activity, prolonged lifespan, and restoration of immune competence in experimental model. In clinical trials, bromocriptine and derivative drugs showed beneficial therapeutic effect in treating human lupus, including pregnancy. Taken together, clinical and experimental results leave little doubt that PRL indeed contributes to the pathogenesis and clinical expression of SLE.

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

    PubMed

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

    2016-12-01

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

  18. CXCR5 is critically involved in progression of lupus through regulation of B cell and double-negative T cell trafficking.

    PubMed

    Wiener, A; Schippers, A; Wagner, N; Tacke, F; Ostendorf, T; Honke, N; Tenbrock, K; Ohl, K

    2016-07-01

    The recruitment of immune cells to sites of tissue inflammation is orchestrated by chemokine/chemokine receptor networks. Among these, the CXCL13/CXCR5 axis is thought to be involved critically in systemic lupus erythematosus (SLE) and lupus nephritis pathogenesis. Beyond B cell abnormalities, another hallmark of SLE disease is the occurrence of aberrant T cell responses. In particular, double-negative (DN) T cells are expanded in the peripheral blood of patients with SLE and in lupus-prone mice. DN T cells induce immunoglobulin production, secrete proinflammatory cytokines and infiltrate inflamed tissue, including kidneys. We aimed to investigate how CXCR5 deficiency changes immune cell trafficking in murine lupus. We therefore crossed CXCR5(-/-) mice with B6/lpr mice, a well-established murine lupus model. B cell numbers and B cellular immune responses were diminished in CXCR5-deficient B6/lpr mice. In addition, we observed reduced accumulation of DN T cells in spleen and lymph nodes, paralleled by reduced splenomegaly and lymphadenopathy. In-vivo migration assays revealed reduced migration of CXCR5-deficient DN T cells into lymph nodes, and ex-vivo-activated CXCR5-deficient DN T cells failed to infiltrate kidneys of recipients. Moreover, DN T cells and B cells of CXCR5-deficient B6/lpr mice failed to migrate towards CXCL13 in vitro. We propose that CXCR5 is involved critically in B cell trafficking and germinal cell (GC) formation in murine lupus and in guiding pathogenic DN T cells into lymphoid organs and kidneys, and we therefore describe new pathomechanisms for the CXCL13/CXCR5 axis in SLE.

  19. [Systemic lupus erythematosus. 2. Factors of predictive significance for survival].

    PubMed

    Halberg, P; Bendixen, G; Fugleberg, S; Jørgensen, F; Kriegbaum, N J; Lorenzen, I; Müller, K; Olesen, K M; Rasmussen, E; Ullman, S

    1991-06-10

    Sixty-one of 173 patients with systemic lupus erythematosus followed for a mean of 13.9 years had severe infections which influenced their survival more than could be accounted for by the mortality (20 per cent) caused by the infections. Patients with infections had more SLE manifestations than patients without infections, and they died of lupus manifestations more often than patients without infections. Patients who went into a permanent remission and patients who died of lupus differed most markedly by the rates of infection. The rate of infection was increased more than tenfold in patients treated with high dosages of glucocorticoid compared with patients who received low dosages. Treatment with cytostatics influenced the rate of infections to a moderate degree. Nephropathy also influenced survival but half of the patients with nephropathy maintained a normal plasma creatinine in spite of the long observation period. 16 per cent of the patients with nephropathy died of kidney failure or are receiving chronic hemodialysis.

  20. Acute macular neuroretinopathy associated with systemic lupus erythematosus.

    PubMed

    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.

  1. Radiologic findings in late-onset systemic lupus erythematosus

    SciTech Connect

    Braunstein, E.M.; Weissman, B.N.; Sosman, J.L.; Schur, P.H.

    1983-03-01

    Systemic lupus erythematosus in the elderly has a different clinical and serologic course from that in young patients. Radiographic findings in patients in whom the diagnosis was made after age 50 were compared with findings in younger patients to see if the radiologic patterns are also different. The only significant radiographic difference between the two groups was that the older group had a greater incidence of soft-tissue swelling of the hands and wrists (p < 0.001). There was no significant difference in osteopenia, erosion, soft-tissue calcification, alignment abnormalities, or intrathoracic findings. Of 24 patients over age 50, two developed lymphoma and another developed multiple myeloma. The data agree with clinical observations that there is a higher incidence of arthritis in late-onset lupus, but clinical findings of increased incidence of pleuropericardial disease are not confirmed radiographically. The coincidence of hematologic malignancy with late-onset lupus in this series is noteworthy.

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

    PubMed

    Pagalavan, L; Kan, F K

    2011-03-01

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

  3. [Management of pregnancy in patients with systemic lupus erythematosus].

    PubMed

    Ugarte, A; Villar, I; Ruiz-Irastorza, G

    2012-11-01

    Patients with systemic lupus erythematosus are exposed to a remarkably high number of maternal-fetal complications during pregnancy. Knowledge regarding the reciprocal influence between lupus and pregnancy is the starting point to assure that these patients are correctly monitored. It is also important to carry out comprehensive preconception evaluation to individually evaluate the risk of each patient. The immunological profile, history of nephritis, presence of chronic damage and disease activity are the basic data that will determine the specific individual risk profile. Finally, correct drug management must be assured during this period, based on the safety profile of the different treatments during pregnancy and lactation.

  4. Spontaneous soft tissue haemorrhage in systemic lupus erythematosus.

    PubMed

    Abdulla, M C

    2016-12-31

    Diversity in clinical presentations and complications of systemic lupus erythematosus (SLE) make the diagnosis and management challenging. The mechanisms of haemorrhagic manifestations in SLE have not been well elucidated. A 47-year-old woman with no comorbidities was admitted after suffering fatigue and low grade fever for six months. She had bilateral soft tissue haemorrhage over the forearm and intra retinal haemorrhages. She was assessed and diagnosed as having SLE based on positive antinuclear antibody, strongly positive anti double stranded DNA, thrombocytopenia and low C3 and C4 levels. We describe a case of spontaneous bilateral soft tissue haemorrhage in SLE and discuss the various mechanisms causing bleeding in lupus.

  5. Eyelash Trichomegaly: A Rare Presenting Feature of Systemic Lupus Erythematosus.

    PubMed

    Dalal, Ashish; Sharma, Sudhanshu; Kumar, Ajay; Sharma, Nidhi

    2017-01-01

    An increase in length, curling, pigmentation, or thickness of eyelashes is termed eyelash trichomegaly. It may be inherited as an isolated trait or as a feature of a congenital syndrome such as Oliver-McFarlane syndrome or oculocutaneous albinism. Acquired conditions associated with eyelash trichomegaly include HIV infection, connective tissue disorders, and the administration of drugs such as cyclosporine and topical latanoprost. We hereby report a rare case of acquired eyelash trichomegaly with diffuse hair loss and "lupus hairs" on the scalp in a 16-year-old female diagnosed with systemic lupus erythematosus.

  6. Eyelash Trichomegaly: A Rare Presenting Feature of Systemic Lupus Erythematosus

    PubMed Central

    Dalal, Ashish; Sharma, Sudhanshu; Kumar, Ajay; Sharma, Nidhi

    2017-01-01

    An increase in length, curling, pigmentation, or thickness of eyelashes is termed eyelash trichomegaly. It may be inherited as an isolated trait or as a feature of a congenital syndrome such as Oliver-McFarlane syndrome or oculocutaneous albinism. Acquired conditions associated with eyelash trichomegaly include HIV infection, connective tissue disorders, and the administration of drugs such as cyclosporine and topical latanoprost. We hereby report a rare case of acquired eyelash trichomegaly with diffuse hair loss and “lupus hairs” on the scalp in a 16-year-old female diagnosed with systemic lupus erythematosus. PMID:28839394

  7. Cardiovascular Events in Systemic Lupus Erythematosus

    PubMed Central

    Fernández-Nebro, Antonio; Rúa-Figueroa, Íñigo; López-Longo, Francisco J.; Galindo-Izquierdo, María; Calvo-Alén, Jaime; Olivé-Marqués, Alejandro; Ordóñez-Cañizares, Carmen; Martín-Martínez, María A.; Blanco, Ricardo; Melero-González, Rafael; Ibáñez-Rúan, Jesús; Bernal-Vidal, José Antonio; Tomero-Muriel, Eva; Uriarte-Isacelaya, Esther; Horcada-Rubio, Loreto; Freire-González, Mercedes; Narváez, Javier; Boteanu, Alina L.; Santos-Soler, Gregorio; Andreu, José L.; Pego-Reigosa, José M.

    2015-01-01

    Abstract This article estimates the frequency of cardiovascular (CV) events that occurred after diagnosis in a large Spanish cohort of patients with systemic lupus erythematosus (SLE) and investigates the main risk factors for atherosclerosis. RELESSER is a nationwide multicenter, hospital-based registry of SLE patients. This is a cross-sectional study. Demographic and clinical variables, the presence of traditional risk factors, and CV events were collected. A CV event was defined as a myocardial infarction, angina, stroke, and/or peripheral artery disease. Multiple logistic regression analysis was performed to investigate the possible risk factors for atherosclerosis. From 2011 to 2012, 3658 SLE patients were enrolled. Of these, 374 (10.9%) patients suffered at least a CV event. In 269 (7.4%) patients, the CV events occurred after SLE diagnosis (86.2% women, median [interquartile range] age 54.9 years [43.2–66.1], and SLE duration of 212.0 months [120.8–289.0]). Strokes (5.7%) were the most frequent CV event, followed by ischemic heart disease (3.8%) and peripheral artery disease (2.2%). Multivariate analysis identified age (odds ratio [95% confidence interval], 1.03 [1.02–1.04]), hypertension (1.71 [1.20–2.44]), smoking (1.48 [1.06–2.07]), diabetes (2.2 [1.32–3.74]), dyslipidemia (2.18 [1.54–3.09]), neurolupus (2.42 [1.56–3.75]), valvulopathy (2.44 [1.34–4.26]), serositis (1.54 [1.09–2.18]), antiphospholipid antibodies (1.57 [1.13–2.17]), low complement (1.81 [1.12–2.93]), and azathioprine (1.47 [1.04–2.07]) as risk factors for CV events. We have confirmed that SLE patients suffer a high prevalence of premature CV disease. Both traditional and nontraditional risk factors contribute to this higher prevalence. Although it needs to be verified with future studies, our study also shows—for the first time—an association between diabetes and CV events in SLE patients. PMID:26200625

  8. [Detection of lupus anticoagulants in the routine blood coagulation laboratory exemplified by 36 patients with systemic lupus erythematosus].

    PubMed

    Halbmayer, W M; Haushofer, A; Schratzberger, W; Petera, P; Duschet, P; Fischer, M

    1993-07-15

    36 patients suffering from systemic lupus erythematosus (SLE) were subjected to various screening and confirmation tests for the presence of lupus anticoagulants (LA) which are a risk for thrombosis. In five out of the 36 patients (14%) lupus anticoagulants could be found. Five out of the 36 patients (14%) showed increased antiphospholipid antibody (APA) levels whereby only two of these patients were at the same time LA-positive. The specificity, sensitivity and effectiveness of various tests in respect of LA-demonstrability have been assessed and the results taken as the basis for proposal of a largely automated stepwise diagnostic procedure for LA-determination within the routine coagulation laboratory.

  9. Neonatal lupus in triplet pregnancy of a patient with undifferentiated connective tissue disease evolving to systemic lupus erythematosus.

    PubMed

    Demaestri, M; Sciascia, S; Kuzenko, A; Bergia, R; Barberis, L; Lanza, M G; Bertero, M T

    2009-04-01

    Pregnancy in patients suffering from undifferentiated connective tissue disease (UCTD) represents a risk situation for both the mother and the child. SSA/SSB autoantibodies can determine neonatal lupus (NL) in the foetus, regardless of the maternal disease. Furthermore, pregnancy increases the risk of flares and evolution to differentiated connective tissue disease (CTD). We report an uncommon case in which these complications occurred in a mother and in her foetuses. A 37-year-old woman affected by UCTD developed systemic lupus erythematosus (SLE) after her triplet pregnancy. The only manifestation of neonatal lupus we observed in the three newborns was SSA positivity associated with asymptomatic transient neutropenia.

  10. Probable systemic lupus erythematosus with cell-bound complement activation products (CB-CAPS).

    PubMed

    Lamichhane, D; Weinstein, A

    2016-08-01

    Complement activation is a key feature of systemic lupus erythematosus (SLE). Detection of cell-bound complement activation products (CB-CAPS) occurs more frequently than serum hypocomplementemia in definite lupus. We describe a patient with normocomplementemic probable SLE who did not fulfill ACR classification criteria for lupus, but the diagnosis was supported by the presence of CB-CAPS.

  11. Rhizomelic chondrodysplasia punctata with maternal systemic lupus erythromatosus.

    PubMed

    Roy, Amrita; De, Pranab; Chakraborty, Swapna

    2013-06-08

    We report Rhizomelic Chondrodysplasia Punctata (RDCP), a rare, autosomal recessive disorder with rhizomelic shortening of limbs, congenital cataracts and seizures but without any biochemical abnormality. The mother of the baby developed Systemic Lupus Erythromatosus (SLE) with Ro/SSA antibodies 11 months after delivery. Ro/SSA antibodies may generate calreticulin antibodies causing characteristic skeletal changes.

  12. Is chronic periodontitis premature in systemic lupus erythematosus patients?

    PubMed

    Calderaro, Débora Cerqueira; Ferreira, Gilda Aparecida; Corrêa, Jôice Dias; Mendonça, Santuza Maria Souza; Silva, Tarcília Aparecida; Costa, Fernando Oliveira; Lúcio Teixeira, Antônio

    2017-03-01

    The aim of this study was to compare the frequency and severity of chronic periodontitis (CP) in systemic lupus erythematosus (SLE) patients with individuals without rheumatic diseases. Seventy-five patients with SLE were compared to 75 individuals without rheumatic diseases (control group) matched for age, educational level, and income. The activity of SLE was assessed with the Systemic Lupus Erythematosus Disease Activity Index 2000. Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for Systemic Lupus Erythematosus evaluated SLE-related damage. Dental evaluation included measuring plaque index and parameters of periodontal disease (probing depth, clinical attachment level, and bleeding on probing). Fifty-one (68 %) SLE patients and 42 (56 %) control individuals had CP (p = 0.13). Periodontal status was similar in both groups. Considering only individuals with CP, SLE patients were younger than controls (40.7 ± 9.8 versus 46.14 ± 12.5 years of age, p = 0.02). CP was not associated with activity or therapeutics in SLE patients. Severity of periodontal parameters was similar in SLE patients and control subjects; however, CP occurred earlier in SLE patients.

  13. Chorea in systemic lupus erythematosus: association with antiphospholipid antibodies.

    PubMed Central

    Khamashta, M A; Gil, A; Anciones, B; Lavilla, P; Valencia, M E; Pintado, V; Vázquez, J J

    1988-01-01

    Chorea is a rare manifestation of systemic lupus erythematosus (SLE). In this report the clinical features of two cases of chorea associated with SLE are presented. Of special interest were the raised titres of antiphospholipid antibodies in both cases. The possible pathogenic role of these antibodies is briefly discussed. PMID:3415367

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

    PubMed

    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

  15. Systemic lupus erythematosus: Clinical and experimental aspects

    SciTech Connect

    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. Distinct antibody profile: a clue to primary antiphospholipid syndrome evolving into systemic lupus erythematosus?

    PubMed

    Freire, Paula Vieira; Watanabe, Elisa; dos Santos, Nelita Rocha; Bueno, Cleonice; Bonfá, Eloísa; de Carvalho, Jozélio Freire

    2014-03-01

    We have performed a retrospective study to determine if patients with antiphospholipid syndrome that developed systemic lupus erythematosus (APS/SLE) had distinct clinical and/or serological features. All 80 primary APS (PAPS) patients followed up at our APS unit were included in the study and divided into two groups: 14 APS/SLE and 66 PAPS. Prior or at onset of lupus manifestations, six patients were uniformly negative for lupus and Sjögren autoantibodies, and the other eight patients had persistent positive. In the first year after diagnosis of SLE, three patients remained with negative antibodies, the other seven patients maintained the same antibodies, and four patients developed other antibodies. APS/SLE group had a significant lower mean age at PAPS diagnosis (26.0 ± 8.0 vs. 34.2 ± 11.9 years, p = 0.03) and a longer disease duration (14.0 ± 7.0 vs. 6.0 ± 5.0 years, p < 0.0001). The mean time for PAPS to develop SLE was 5.2 ± 4.3 years. The typical clinical and laboratorial findings of APS did not discriminate both groups of patients. At lupus onset, antinuclear antibodies were more frequently observed in those who evolved to SLE (100 vs. 51.5%, p = 0.0005). Anti-double-stranded DNA (dsDNA), anti-ribosomal P, anti-Ro/SS-A, anti-La/SS-B, and anti-U1RNP antibodies were exclusively found in the APS/SLE patients, whereas anti-Smith (Sm) antibodies were not detected in both groups. The detection of a distinct subgroup of lupus-associated autoantibody in PAPS patients seems to be a hint to overt SLE disease, particularly in those patients with young age at diagnosis.

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

    PubMed

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

    2017-04-01

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

  18. Sensitivity to Change and Minimal Important Differences of the LupusQoL in Patients With Systemic Lupus Erythematosus

    PubMed Central

    McElhone, Kathleen; Abbott, Janice; Sutton, Chris; Mullen, Montana; Lanyon, Peter; Rahman, Anisur; Yee, Chee‐Seng; Akil, Mohammed; Bruce, Ian N.; Ahmad, Yasmeen; Gordon, Caroline

    2016-01-01

    Objective As a health‐related quality of life (HRQOL) measure, the LupusQoL is a reliable and valid measure for adults with systemic lupus erythematosus (SLE). This study evaluates the responsiveness and minimal important differences (MIDs) for the 8 LupusQoL domains. Methods Patients experiencing a flare were recruited from 9 UK centers. At each of the 10 monthly visits, HRQOL (LupusQoL, Short Form 36 health survey [SF‐36]), global rating of change (GRC), and disease activity using the British Isles Lupus Assessment Group 2004 index were assessed. The responsiveness of the LupusQoL and the SF‐36 was evaluated primarily when patients reported an improvement or deterioration on the GRC scale and additionally with changes in physician‐reported disease activity. MIDs were estimated as mean changes when minimal change was reported on the GRC scale. Results A total of 101 patients were recruited. For all LupusQoL domains, mean HRQOL worsened when patients reported deterioration and improved when patients reported an improvement in GRC; SF‐36 domains showed comparable responsiveness. Improvement in some domains of the LupusQoL/SF‐36 was observed with a decrease in disease activity, but when disease activity worsened, there was no significant change. LupusQoL MID estimates for deterioration ranged from −2.4 to −8.7, and for improvement from 3.5 to 7.3; for the SF‐36, the same MID estimates were −2.0 to −11.1 and 2.8 to 10.9, respectively. Conclusion All LupusQoL domains are sensitive to change with patient‐reported deterioration or improvement in health status. For disease activity, some LupusQoL domains showed responsiveness when there was improvement but none for deterioration. LupusQoL items were derived from SLE patients and provide the advantage of disease‐specific domains, important to the patients, not captured by the SF‐36. PMID:26816223

  19. Sensitivity to Change and Minimal Important Differences of the LupusQoL in Patients With Systemic Lupus Erythematosus.

    PubMed

    McElhone, Kathleen; Abbott, Janice; Sutton, Chris; Mullen, Montana; Lanyon, Peter; Rahman, Anisur; Yee, Chee-Seng; Akil, Mohammed; Bruce, Ian N; Ahmad, Yasmeen; Gordon, Caroline; Teh, Lee-Suan

    2016-10-01

    As a health-related quality of life (HRQOL) measure, the LupusQoL is a reliable and valid measure for adults with systemic lupus erythematosus (SLE). This study evaluates the responsiveness and minimal important differences (MIDs) for the 8 LupusQoL domains. Patients experiencing a flare were recruited from 9 UK centers. At each of the 10 monthly visits, HRQOL (LupusQoL, Short Form 36 health survey [SF-36]), global rating of change (GRC), and disease activity using the British Isles Lupus Assessment Group 2004 index were assessed. The responsiveness of the LupusQoL and the SF-36 was evaluated primarily when patients reported an improvement or deterioration on the GRC scale and additionally with changes in physician-reported disease activity. MIDs were estimated as mean changes when minimal change was reported on the GRC scale. A total of 101 patients were recruited. For all LupusQoL domains, mean HRQOL worsened when patients reported deterioration and improved when patients reported an improvement in GRC; SF-36 domains showed comparable responsiveness. Improvement in some domains of the LupusQoL/SF-36 was observed with a decrease in disease activity, but when disease activity worsened, there was no significant change. LupusQoL MID estimates for deterioration ranged from -2.4 to -8.7, and for improvement from 3.5 to 7.3; for the SF-36, the same MID estimates were -2.0 to -11.1 and 2.8 to 10.9, respectively. All LupusQoL domains are sensitive to change with patient-reported deterioration or improvement in health status. For disease activity, some LupusQoL domains showed responsiveness when there was improvement but none for deterioration. LupusQoL items were derived from SLE patients and provide the advantage of disease-specific domains, important to the patients, not captured by the SF-36. © 2016, The Authors. Arthritis Care & Research published by Wiley Periodicals, Inc. on behalf of the American College of Rheumatology.

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

    PubMed

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

    2015-01-01

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

  1. [A case of systemic lupus erythematosus complicated with psoriasis vulgaris].

    PubMed

    Shidara, Kumi; Soejima, Makoto; Shiseki, Mariko; Ohta, Syuji; Nishinarita, Makoto

    2003-12-01

    A 49-years-old female admitted to our hospital because of skin eruptions on the extremities in 1985. She had suffered from polyarthralgia, skin eruptions since 1983. Physical examinations revealed discoid lesion, central nervous system involvement, and polyarthritis. Laboratory tests revealed leukopenia, thrombocytopenia, and hypocomplementemia. Antinuclear antibody, ant-DNA antibody, LE test were positive. From these findings, she was diagnosed as systemic lupus erythematosus (SLE). She developed lupus peritonitis in 1990 and 1994, which was successfully treated by steroid pulse therapy. Since then, the activity of SLE was in good control under administration of prednisolone 10 mg/day. Chilblain lupus was seen from 1993, Raynaud's phenomenon from 1996, and she further developed subcutaneous induration on her chest, back and upper extremities in 1999. Skin biopsy findings were compatible with lupus panniculitis. In 2002, erythematous patches with scales were observed on her right hand and left knee, and these skin lesions were histologically diagnosed as psoriasis vulgaris. An autoimmune response similar to SLE is speculated in psoriasis. We describe a rare case of SLE with various skin lesions including psoriasis vulgaris.

  2. Nature and nurture in systemic lupus erythematosus.

    PubMed

    Maddison, P J

    1999-01-01

    Nowhere across the spectrum of rheumatic and dermatological disease is the interaction of nature and nurture more relevant than in the connective tissue diseases such as SLE. While genetic and environmental factors are clearly involved in both the triggering of the disease and its expression, the interaction is complex with different combinations of factors contributing in different patients. For example, while genetic factors contribute substantially to susceptibility to lupus, this does not follow a simple Mendelian pattern of inheritance and mathematical models suggest that there may be varying contribution from at least four genes with differing inheritances. A variety of candidate genes and environmental factors have been highlighted in SLE but to dissect out the complexity of how these might interact requires the study of patient groups with a better defined clinical and serological phenotype. For example, studies of patients with subacute cutaneous lupus (SCLE) have shown associations with various genes in the MHC region (including HLA, complement and TNF) and suggest that the biological effect of inheriting an extended MHC region may be greater than its individual parts. One can now speculate on how interaction with an environmental factor such as UV light explains pathogenesis.

  3. Systemic lupus erythematosus and thrombotic thrombocytopenia purpura: a refractory case without lupus activity.

    PubMed

    Garcia Boyero, Raimundo; Mas Esteve, Eva; Mas Esteve, Maria; Millá Perseguer, M Magdalena; Marco Buades, Josefa; Beltran Fabregat, Juan; Cañigral Ferrando, Guillermo; Belmonte Serrano, Miguel Angel

    2013-01-01

    The association between systemic lupus erythematosus (SLE) and thrombotic thrombocytopenic purpura (TTP) has been infrequently reported. Usually, patients with TTP have more SLE activity and frequent renal involvement. Here we present a case of TTP associated to low-activity SLE. The absence of renal and major organ involvement increased the difficulty in making the initial diagnosis. ADAMTS13 activity in plasma in this patient was very low, as seen in other similar cases. The evolution of the patient was poor, needing plasma exchanges and immunosuppressive therapy, including the use of rituximab.

  4. The antiphospholipid syndrome in patients with systemic lupus erythematosus.

    PubMed

    Pons-Estel, Guillermo J; Andreoli, Laura; Scanzi, Francesco; Cervera, Ricard; Tincani, Angela

    2017-01-01

    The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL). APS may be associated with other diseases, mainly systemic lupus erythematosus (SLE). The presence or absence of SLE might modify the clinical or serological expression of APS. Apart from the classical manifestations, APS patients with associated SLE more frequently display a clinical profile with arthralgias, arthritis, autoimmune hemolytic anemia, livedo reticularis, epilepsy, glomerular thrombosis, and myocardial infarction. The management of patients with SLE and APS/aPL should include an accurate stratification of vascular risk factors. Low dose aspirin and hydroxychloroquine should be considered as primary prophylaxis. In high risk situations, such as surgery, prolonged immobilization, and puerperium, the prophylaxis should be potentiated with low molecular weight heparin. The challenge of treating patients with a previous vascular event (secondary prophylaxis) is the choice of treatment (anti-platelet agents, anticoagulation with vitamin K antagonists or combined therapy) and its duration, based on individual risk stratification and the site of vascular presentation. The role of novel anticoagulants in APS patients is still to be clearly defined. Novel approaches are needed since the prognosis of SLE patients with APS/aPL is still worse than that of SLE patients with negative aPL. The goal for the future is to improve the outcome of these patients by means of early recognition and optimal preventative treatment.

  5. Epileptic syndrome in systemic lupus erythematosus and neuronal autoantibody associations.

    PubMed

    Kampylafka, E I; Alexopoulos, H; Fouka, P; Moutsopoulos, H M; Dalakas, M C; Tzioufas, A G

    2016-10-01

    We investigated systemic lupus erythematosus (SLE) patients with epilepsy, a major and organic neurological symptom. Our aim was to test patients for the autoimmune epilepsy-associated antibodies anti-GAD, anti-NMDAR, anti-AMPAR1/2, anti-GABABR and anti-VGKC. We tested sera from ten SLE patients with current or previous episodes of epileptic seizures. In addition, sera were tested for staining on primary hippocampal neurons. The patients' clinical and neuroimaging profile, disease activity and accumulated damage scores and therapeutic regimens administered were recorded, and correlations were evaluated. Patients were negative for all anti-neuronal autoantibodies tested, and showed no staining on primary hippocampal cells, which suggests the absence of autoantibodies against neuronal cell surface antigens. Epileptic seizures were all tonic-clonic, and all patients had high disease activity (mean SLE Damage Acticity Index score 19.3 ± 7.3). Six patients had minor or no brain magnetic resonance imaging findings, and three had major findings. 9/10 patients received immunosuppression for 5 ± 4 months, while anti-convulsive treatment was administered to all patients (4.2 ± 3 years). Our results suggest that the majority of SLE-related epileptic seizures cannot be attributed to the action of a single antibody against neuronal antigens. Studies with larger neuropsychiatric SLE populations and stricter inclusion criteria are necessary to verify these findings.

  6. Elevated plasma interleukin-37 levels in systemic lupus erythematosus patients.

    PubMed

    Wu, G-C; Li, H-M; Wang, J-B; Leng, R-X; Wang, D-G; Ye, D-Q

    2016-10-01

    This study aims to evaluate the plasma interleukin (IL)-37 levels in systemic lupus erythematosus (SLE) patients, as well as its association with major clinical and laboratory features. Ninety consecutively selected SLE patients and 78 community-based healthy controls were recruited. Plasma IL-37 levels were detected by enzyme-linked immunosorbent assay (ELISA). The major clinical and laboratory data of SLE patients were also recorded. The results showed that IL-37 level was significantly higher in the plasma of patients with SLE compared with controls (p = 0.028). The correlation of plasma IL-37 levels with major clinical and laboratory data of SLE patients was also analyzed, and the results showed that anti-Sm and anti-RNP were negatively associated with plasma IL-37 levels of SLE patients, while C3 was positively associated with plasma IL-37 levels of SLE patients. No significant associations of IL-37 with other clinical and laboratory parameters were observed (all p > 0.05). In conclusion, elevated plasma IL-37 level and its associations with anti-Sm, anti-RNP and C3 in SLE patients suggest that IL-37 may be implicated in this disease. © The Author(s) 2016.

  7. Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology.

    PubMed

    Berntsson, Shala Ghaderi; Katsarogiannis, Evangelos; Lourenço, Filipa; Moraes-Fontes, Maria Francisca

    2016-01-01

    Progressive multifocal leukoencephalopathy (PML) caused by reactivation of the JC virus (JCV), a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE). We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine) since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions) and JCV isolation from the cerebrospinal fluid (700 copies/ml). IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need.

  8. HPV and systemic lupus erythematosus: a mosaic of potential crossreactions.

    PubMed

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

    2017-04-01

    Etiology, pathogenesis, and immunology of systemic lupus erythematosus (SLE) form a complex, still undeciphered picture that recently has been further made complicated by a new factor of morbidity: human papillomaviruses (HPVs). Indeed, a prevalence of HPV infections has been reported among SLE patients. Searching for molecular mechanisms that might underlie and explain the relationship between HPV infection and SLE, we explored the hypothesis that immune responses following HPV infection may crossreact with proteins that, when altered, associate with SLE. Analyzing HPV L1 proteins and using Epstein-Barr virus (EBV) and human retrovirus (HERV) as controls, we found a vast peptide overlap with human proteins comprehending lupus Ku autoantigen proteins p86 and p70, lupus brain antigen 1 homolog, lupus antigen expressed in neurons and muscles, natural killer cell IgG-like receptors, complement proteins C4-A and C4-B, complement receptor CD19, and others. The multitude and heterogeneity of peptide overlaps not only further support the hypothesis that crossreactivity can represent a primum movens in SLE onset, but also provide a molecular framework to the concept of SLE as "an autoimmune mosaic syndrome." Finally, once more, it emerges the need of using the principle of peptide uniqueness as a new paradigm for safe and efficacious vaccinology.

  9. Flares of systemic lupus erythematosus during pregnancy and the puerperium: prevention, diagnosis and management.

    PubMed

    Stojan, George; Baer, Alan N

    2012-07-01

    Systemic lupus erythematosus is a systemic autoimmune disease that primarily affects women in their reproductive age years. Pregnancy in systemic lupus erythematosus now has favorable outcomes for the majority of women. However, flares of disease activity, preeclampsia, fetal loss, intrauterine growth retardation and preterm birth are established risks of such pregnancies. Active lupus nephritis at the time of conception poses the greatest risk for disease flares and poor obstetric outcomes. Patients should delay conception until their lupus has been in remission for at least 6 months. In addition, certain lupus medications are potentially teratogenic and need to be stopped before conception. The signs and symptoms of a lupus flare may mimic those of normal pregnancy, impeding its recognition during pregnancy. Hydroxychloroquine, low-dose prednisone, pulse intravenous methylprednisolone and azathioprine are commonly used to treat lupus flares during pregnancy.

  10. Echocardiographic findings in asymptomatic systemic lupus erythematosus patients.

    PubMed

    Mohammed, Abdel GaffarA; Alghamdi, Abdulaziz A; ALjahlan, Mohammad A; Al-Homood, Ibrahim A

    2017-03-01

    The aim of this study is to use transthoracic echocardiographic (TTE) imaging methods to identify cardiac dysfunction in asymptomatic systemic lupus erythematosus (SLE) patients and to determine the association between echocardiographic findings and serology. This is a prospective cross-sectional study where 50 patients with confirmed diagnoses of SLE were recruited from rheumatology outpatient clinics. Clinical and serological evaluation to confirm the diagnosis of lupus was done in all patients. Fifty SLE patients, 46 (92%) females and 4 (8%) males, were recruited. Anti-double-stranded DNA (Anti-dsDNA), anticardiolipin, lupus anticoagulant, and anti-β2-glycoproteins were positive in 52.1, 32.6, 13.3, and 15.6%, respectively. Transthoracic echocardiogram revealed mitral regurgitation in 16 patients (32%), pericardial effusion in16 patients (32%), aortic regurgitation in five patients (10%), and tricuspid regurgitation in 10 patients (20%). Eleven patients had left ventricular hypertrophy (22%), and eight patients had ventricular systolic dysfunction (16%). Only four patients had ventricular diastolic dysfunction (8%). A significant association between mitral and tricuspid valve regurgitation and positive anti-dsDNA (p < 0.018, p < 0.006, respectively) was found. Positive anticardiolipin antibodies, lupus anticoagulant, and anti-β 2 glycoprotein antibodies were also associated with mitral valve regurgitation (p values 0.044, 0.006, and 0.023), respectively. Active disease assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was found to be associated with increased risk of mitral valvular leaflet thickening (p value 0.028). Performing regular transthoracic echocardiogram in asymptomatic SLE patients is important for early detection and appropriate treatment of cardiac lesions. Clinically quiescent but serologically active disease and presence of antiphospholipid antibodies were associated with structural heart abnormalities.

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

    PubMed Central

    Jung, Ju-Yang; Suh, Chang-Hee

    2017-01-01

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

  12. [A case report of childhood systemic lupus erythematosus complicated with lupus cystitis].

    PubMed

    Kurosawa, Rumiko; Miyamae, Takako; Imagawa, Tomoyuki; Katakura, Shigeki; Mori, Masaaki; Aihara, Yuhkoh; Yokota, Shumpei

    2006-06-01

    The patient was a 13-year-old girl. In August 2000, she presented with a fever, together with diarrhea, vomiting, arthralgia, nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear antibodies, anti-DNA antibodies and platelet-associated IgG, idiopathic thrombopenic purpura, and systemic lupus erythematosus (SLE) was suspected. From January 2001, when she caught measles, she reported abdominal pain, and urinalysis indicated urinary protein and occult blood, and the left kidney was found hydronephrotic. At the same time left ureter stenosis and dilatation were demonstrated. Symptoms were disappeared by hydration and treatment with NSAIDs, but 2 months later fever and erythematous patches seen on both cheeks led to the proper diagnosis of SLE, and she was admitted to our hospital. Intravenous pyelography revealed hydronephrosis on left kidney, constriction and dilatation of the left ureter, and intracystic endoscopy showed erythema at the orifice of the left ureter. The pathological examination indicated the presence of vasculitis, and finally lupus cystitis was diagnosed. Intravenous cyclophosphamide (IVCY)-pulse therapy was introduced to a total of 8 times over the period of a year, and maintenance therapy with predonisolone and azathioprin was also used. After completion of the IVCY-pulse therapy, the hydronephrosis and constriction of the ureter were disappeared. No side effects of IVCY-pulses were observed, and the patient is now in remission. We reported a case of childhood SLE complicated with lupus cystitis and successfully treated by IVCY-pulse therapy and maintenance predonisolone and azathioprin.

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

    PubMed Central

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

    2015-01-01

    Objective To validate clinical indices of lupus nephritis (LN) activity and damage when used in children against the criterion standard of kidney biopsy findings. Methods In 83 children requiring kidney biopsy the SLE Disease Activity Index Renal Domain (SLEDAI-R); British Isles Lupus Assessment Group index Renal Domain (BILAG-R), Systemic Lupus International Collaborating Clinics Renal Activity (SLICC-RAS) and Damage Index Renal Domain (SDI-R) were measured. Fixed effect and logistic models were done to predict International Society of Nephrology/Renal Pathology Society (ISN/RPS) class; low/moderate vs. high LN-activity [NIH Activity Index (NIH-AI) score: ≤ 10 vs. > 10; Tubulointerstitial Activity Index (TIAI) score: ≤ 5 vs. > 5) or the absence vs. presence of LN chronicity [NIH Chronicity Index (NIH-CI) score: 0 vs. ≥ 1]. Results There were 10, 50 and 23 patients with class I/II, III/IV and V, respectively. Scores of the clinical indices did not differentiate among patients by ISN/RPS class. The SLEDAI-R and SLICC-RAS but not the BILAG-R differed with LN-activity status defined by NIH-AI scores, while only the SLEDAI-R scores differed between LN-activity status based on TIAI scores. The sensitivity and specificity of the SDI-R to capture LN chronicity was 23.5% and 91.7%, respectively. Despite designed to measure LN-activity, SLICC-RAS and SLEDAI-R scores significantly differed with LN chronicity status. Conclusion Current clinical indices of LN fail to discriminate ISN/RPS Class in children. Despite its shortcomings, the SLEDAI-R appears to best for measuring LN activity in a clinical setting. The SDI-R is a poor correlate of LN chronicity. PMID:26213987

  14. Satisfaction with control of systemic lupus erythematosus and lupus nephritis: physician and patient perspectives

    PubMed Central

    Mozaffarian, Neelufar; Lobosco, Steve; Lu, Peng; Roughley, Adam; Alperovich, Gabriela

    2016-01-01

    Purpose Patient satisfaction with disease control of systemic lupus erythematosus (SLE) is an important component of medical management. This analysis evaluated patient and physician satisfaction with disease control of SLE, factors associated with satisfaction/dissatisfaction, and the degree of physician–patient concordance of these parameters. Patients and methods Data were extracted from the US Adelphi Real World Lupus Disease Specific Programme®, a cross-sectional survey of 50 rheumatologists, 25 nephrologists, and their patients with non-nephritis SLE (NNSLE) or lupus nephritis (LN). Results Physicians reported moderate or severe disease activity in 25.0% of patients with NNSLE and in 50.5% of patients with LN, and were satisfied with disease control in 78.6% (132/168) and 73.8% (152/206) of patients, respectively. For patients, 75.8% (75/99) with NNSLE were satisfied with their current treatment, compared with 65.5% (74/113) with LN. Physician–patient agreement (70.7%) on the level of satisfaction was “slight” (kappa =0.1445) for NNSLE; patients were more frequently dissatisfied than physicians with regard to joint tenderness, fatigue, anxiety, pain on movement, malar rash, and photosensitivity. Physician–patient agreement (71.4%) on the level of satisfaction was “fair” (kappa =0.3695) for LN; patients expressed greater dissatisfaction than physicians for headache, photosensitivity, and anxiety, whereas physicians were more dissatisfied with regard to joint swelling, kidney function, and blood pressure control. In general, patients with NNSLE or LN who were dissatisfied (or whose physicians were dissatisfied) were more likely to have joint swelling, joint stiffness, malar rash, hair loss, depression, and fatigue, have moderate or severe disease, or to be currently experiencing disease flare. Conclusion These data highlight the patient and physician dissatisfaction with real-world disease control of SLE. PMID:27784995

  15. Acute herpes simplex virus 1 pneumonitis in a patient with systemic lupus erythematosus.

    PubMed

    Sabugo, Francisca; Espinoza-Araya, Ricardo; Meneses, Manuel F; Cuchacovich, Miguel

    2014-01-01

    A woman with severe and longstanding systemic lupus erythematosus presented with a 1-week history of fever up to 38°C and pain in her right flank. Computed tomography scan of the chest revealed interstitial infiltrates and multiple nodules. Bronchoalveolar lavage did not show any inflammatory cells. Gram stain and cultures for aerobic and anaerobic bacteria, fungi, and Nocardia; acid-fast staining; polymerase chain reaction for tuberculosis, cytomegalovirus, herpesvirus 6, and parvovirus B19; and IF staining for pneumocystic and Legionella antigen were all negative. Transbronchial biopsy was nondiagnostic. Open lung biopsy with polymerase chain reaction and immunohistochemistry analyses revealed herpes simplex virus 1 infection. Acyclovir therapy was initiated and was followed by significant improvement. Herpes simplex virus 1 infection (although unusual) should be considered in patients with systemic lupus erythematosus with an atypical clinical presentation.

  16. Can Cell Bound Complement Activation Products Predict Inherited Complement Deficiency in Systemic Lupus Erythematosus?

    PubMed Central

    Waters, Barry

    2016-01-01

    Activation of the classical pathway complement system has long been implicated in stimulating immune complex mediated tissue destruction in systemic lupus erythematosus (SLE). C3 and C4 complement levels are utilized as part of SLE diagnosis and monitoring criteria. Recently, cell bound complement activation products (CBCAPs) have shown increased sensitivity in diagnosing and monitoring lupus activity, compared to traditional markers. CBCAPs are increasingly utilized in rheumatology practice as additional serological markers in evaluating SLE patients. We report a case of a patient diagnosed with SLE that had chronically low C3 and C4, along with negative CBCAPs. We surmise that the patient has an inherited complement deficiency as the etiology of her SLE and that CBCAPs could be used to predict such deficiency. PMID:28074166

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

    PubMed

    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

    2017-06-26

    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.Immunology and Cell Biology advance online publication, 25 July 2017; doi:10.1038/icb.2017.53.

  18. Anticyclic citrullinated peptide autoantibodies in systemic lupus erythematosus.

    PubMed

    Singh, Usha; Singh, Sangeeta; Singh, Nand K; Verma, Pramod K; Singh, Suman

    2011-06-01

    Systemic lupus erythematosus is an autoimmune disease with protean manifestation. Arthritis is one of the most common manifestations seen in SLE. Anti-CCP Ab is a recently described autoantibody that has been claimed as a most sensitive and specific marker for the diagnosis of RA. Study was performed to see whether anti-CCP2 Ab is positive in lupus arthritis or not. Anti-CCP Ab, ANA, ds DNA, and APLA were estimated by ELISA. Anti-CCP2 Ab was positive in 22 cases (37.93%) of SLE. Mean value of anti-CCP (18.08±16.95 U/ml) was statistically significant (P<0.001) when compared to control (5.07±U/ml). A total of 44 (75.86%) patients with SLE had arthritis. In 29 (50.00%) cases, arthritis resembled RA along with classical features of SLE, while 15 cases (25.86%) had nonspecific lupus arthritis. In 13 cases (44.82%) of RA type lupus arthritis, anti-CCP2 Ab was positive, while only three (20%) nonspecific lupus arthritis cases had elevated anti-CCP. In 14 (24.13) patients with SLE, there was no arthritis, but in this group also (6/14) 42.85% cases had elevated anti-CCP. A total of 11 (50%) patients with duration less than 1 year had more anti-CCP 2 positivity when compared to disease duration between 1 and 3 years (27.27%) and disease duration more than 3 years (22.72%), but specifically, it was not significant. Our study concludes that anti-CCP2 is not a specific antibody for RA, but it is present in autoimmune diseases.

  19. The rate of and risk factors for frequent hospitalization in systemic lupus erythematosus: results from the Korean lupus network registry.

    PubMed

    Lee, J W; Park, D J; Kang, J H; Choi, S E; Yim, Y R; Kim, J E; Lee, K E; Wen, L; Kim, T J; Park, Y W; Sung, Y K; Lee, S S

    2016-11-01

    Objectives The survival rate of patients with systemic lupus erythematosus has improved in the last few decades, but the rate of hospitalization and health care costs for these patients remain higher than in the general population. Thus, we evaluated the rate of hospitalization and associated risk factors in an inception cohort of Korean patients with lupus. Methods Of the 507 patients with systemic lupus erythematosus enrolled in the KORean lupus NETwork, we investigated an inception cohort consisting of 196 patients with systemic lupus erythematosus presenting within 6 months of diagnosis based on the American College of Rheumatology classification criteria. We evaluated the causes of hospitalization, demographic characteristics, and laboratory and clinical data at the time of systemic lupus erythematosus diagnosis of hospitalized patients and during a follow-up period. We calculated the hospitalization rate as the number of total hospitalizations divided by the disease duration, and defined "frequent hospitalization" as hospitalization more than once per year. Results Of the 196 patients, 117 (59.6%) were admitted to hospital a total of 257 times during the 8-year follow-up period. Moreover, 22 (11.2%) patients were hospitalized frequently. The most common reasons for hospitalization included disease flares, infection, and pregnancy-related morbidity. In the univariate regression analysis, malar rash, arthritis, pericarditis, renal involvement, fever, systemic lupus erythematosus disease activity index > 12, hemoglobin level < 10 mg/dl, albumin level < 3.5 mg/dl, and anti-Sjögren's syndrome A positivity were associated with frequent hospitalization. Finally, multivariate analysis showed that arthritis, pericarditis, and anti-Sjögren's syndrome A antibody positivity at the time of diagnosis were risk factors for frequent hospitalization. Conclusions Our results showed that frequent hospitalization occurred in 11.2% of hospitalized patients and

  20. Impact of systemic lupus erythematosus on oral health-related quality of life.

    PubMed

    Corrêa, J D; Branco, L G A; Calderaro, D C; Mendonça, S M S; Travassos, D V; Ferreira, G A; Teixeira, A L; Abreu, L G; Silva, T A

    2017-01-01

    Oral symptoms in systemic lupus erythematosus (SLE) patients are often unexplored and affect the health-related quality of life. The aims of this study were: (a) to evaluate the oral health condition of SLE patients compared to control subjects without rheumatic diseases; (b) to determine the consequences of oral health condition in the quality of life of these two groups. Individuals with SLE ( n = 75) and without SLE ( n = 78) (control group), paired for gender and age, underwent complete oral examination. Sociodemographic and clinical information was obtained, and interviews were conducted using the Brazilian version of the oral health impact profile. The activity and damage of SLE disease were assessed, respectively, by the systemic lupus erythematosus disease activity index 2000 and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. When we analysed the oral health condition and hygiene habits of the participants, SLE patients exhibited an increased number of missing teeth despite their higher frequency of tooth brushing. No significant differences were verified in other habits and clinical parameters evaluated such as smoking, flossing, salivary flux, periodontitis, decayed and filled teeth. Patients with SLE presented with worse oral health-related quality of life than controls ( P = 0.011). The significant difference was on individuals' physical disability ( P = 0.002). The determinant of the negative impact on the oral health-related quality of life was prosthesis wearing ( P < 0.05). Overall, the oral health impact profile score was higher in individuals with moderate SLE damage compared to SLE individuals with no damage ( P = 0.043). Patients with SLE had a negative impact of oral condition on their quality of life. The evaluation of the oral health-related quality of life might be useful to monitor the effects of SLE on oral condition.

  1. A patient with plaque type morphea mimicking systemic lupus erythematosus.

    PubMed

    Wardhana; Datau, E A

    2015-04-01

    Morphea is an uncommon connective tissue disease with the most prominent feature being thickening or fibrosis of the dermal without internal organ involvement. It is also known as a part of localized scleroderma. Based on clinical presentation and depth of tissue involvement, morphea is classified into several forms, and about two thirds of adults with morphea have plaque type. Overproduction of collagen production by fibroblast is the cause of abnormality in morphea, and the hyperactivity mechanism of fibroblast is still unknown, although there are several mechanisms already proposed. Plaque type morphea is actually a benign and self limited. Plaque type morphea that mimicking systemic lupus erythematosus in clinical appearance, such as alopecia and oral mucosal ulcers, is uncommon. A case of plaque type morphea mimicking systemic lupus erythematosus in a 20 year old woman was discussed. The patient was treated with local and systemic immunosuppressant and antioxydant. The patient's condition is improved without any significant side effects.

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

    PubMed

    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.

  3. Genome-Wide Association Study in African-Americans With Systemic Lupus Erythematosus

    DTIC Science & Technology

    2011-09-01

    Americans with Systemic Lupus Erythematosus PRINCIPAL INVESTIGATOR: John Harley, M.D., Ph.D... Systemic Lupus Erythematosus 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) 5d. PROJECT NUMBER John Harley, M.D., Ph.D. 5e. TASK...SUPPLEMENTARY NOTES 14. ABSTRACT Systemic lupus erythematosus is a potentially deadly systemic autoimmune disease that disproportionately afflicts women

  4. Association of calreticulin expression with disease activity and organ damage in systemic lupus erythematosus patients.

    PubMed

    Wang, Yichao; Xie, Jiaogui; Liu, Zhili; Fu, Hongwei; Huo, Qianyu; Gu, Yajun; Liu, Yunde

    2017-05-01

    Measurement of disease activity in patients with systemic lupus erythematosus (SLE) is important for monitoring disease progression and evaluating the therapeutic effects. The severity of organ damage correlates with clinical status and prognosis. Therefore, it is imperative to find an effective biomarker measuring disease activity and organ damage for SLE management. The present study investigated the possibility of serum calreticulin (CRT) in the assessment of disease activity and organ damage in SLE patients. Serum CRT levels from 80 patients with SLE, 55 patients with other autoimmune diseases and 60 healthy controls (HC) were measured by ELISA. Disease activity was assessed using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores. Organ damage was evaluated with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. CRT levels in SLE were significantly higher than that in other autoimmune diseases and HC. CRT was correlated with SLEDAI-2K score (r=0.3345, P=0.0024), and with anti-double-stranded DNA (anti-dsDNA) (r=0.4483, P<0.0001). A significant negative correlation of CRT levels with complement 3 (r=-0.3635, P=0.0009) and complement 4 (r=-0.3507, P=0.0014) was observed in patients with SLE. Furthermore, the patients with SLE and a positive anti-Ro52 result had higher levels of CRT compared with those with a negative anti-Ro52 result (P<0.001). Elevated levels of CRT were also reported among patients with SLE who also indicated the presence of cumulative organ damage. In addition, increased expression of CRT correlated with the presence of lupus nephritis. In conclusion, the results of the current report provided that CRT may be used as a potential biomarker for clinical diagnosis and of prognosis, providing additional information regarding disease activity and organ damage alongside other traditional indices.

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

    PubMed Central

    Xue, Jing; Yang, Jiali; Yang, Lijuan; Zhou, Shaolan; Ji, Chen; Wang, Xuemei; Yu, Nan

    2017-01-01

    An early diagnosis of lupus nephritis (LN) has an important clinical implication in guiding treatments of systemic lupus erythematosus (SLE) in clinical settings. In this study, the concentrations of Wnt-3A, Frizzled-8 (FZD-8), and Dickkopf-1 (DKK-1) of Wnt signaling, as well as their diagnostic values for accessing LN, were evaluated by ELISA in sera and urine of 111 SLE patients (31 with LN and 80 without LN) and 70 healthy cohorts. Significantly more abundances of DKK-1 protein were determined in both of sera and urine of SLE patients compared to healthy cohorts (p < 0.0001); in particular the serum DKK-1 concentration was even higher in LN-SLE patients relative to non-LN SLE subjects (p < 0.0001). Intriguingly, concentrations of above examined proteins in SLE patients showed no correlation between serum and urine. Moreover, a combination of DKK-1 with anti-dsDNA and/or levels of complement C3 and C4 could not increase the specificity and/or sensitivity for identification of patients with LN diseases, but both ROC curve and multiple-factor nonconditional logistic regression analysis showed that serum DKK-1 was considered better positive biomarker for identification of LN in SLE patients. These results imply that serum and/or urine DKK-1 may be a valuable and independent biomarker for identification of SLE patients with LN. PMID:28373995

  6. Complement-fixing properties of antinuclear antibodies distinguish drug-induced lupus from systemic lupus erythematosus.

    PubMed

    Rubin, R L; Teodorescu, M; Beutner, E H; Plunkett, R W

    2004-01-01

    The immunofluorescence antinuclear antibody (ANA) test has been widely used to monitor autoimmune disease, but its value for diagnostic purposes is compromised by low specificity and high prevalence in disease-free individuals. The capacity of autoantibodies to fix serum complement proteins when bound to antigen is an important effector function because this property is associated with acute and chronic inflammatory processes. The current study evaluates the complement-fixing properties of antinuclear antibodies (CANA) in three well-defined and clinically-related patient groups: systemic lupus erythematosus (SLE), drug-induced lupus (DIL) and drug-induced autoimmunity (DIA). Of 20 patients diagnosed with SLE, 90% displayed complement-fixing ANA while this feature was present in only two of 18 patients with DIL and no patients with DIA without associated disease even though the mean ANA titres were similar among these patient groups. CANA was significantly correlated with anti-Sm activity. Because SLE but not DIL or DIA can be a life-threatening disease associated with complement consumption in vivo, these results demonstrate that measurement of CANA is a diagnostically useful tool and may have immunopathologic implications.

  7. Acute lupus pneumonitis followed by intestinal pseudo-obstruction in systemic lupus erythematosus: A case report

    PubMed Central

    JI, CAIHONG; YU, XING; WANG, YONG; SHI, LUFENG

    2016-01-01

    Intestinal pseudo-obstruction (IpsO) and acute lupus pneumonitis (ALP) are uncommon severe complications of systemic lupus erythematosus (SLE). The present study reports the case of a 26-year-old female who presented with abdominal pain, nausea and vomiting as initial symptoms. Computed tomography (CT) scanning revealed the jejunal wall was thickened and streaky, mimicking the presentation of intestinal obstruction. Following emergency surgery, the patient's general condition was aggravated, with evident limb erythematous rashes. A series of laboratory examinations revealed SLE, and combined with patient's medical history IpsO was diagnosed, with a disease Activity Index score of 10. During the therapeutic period, high fever, dyspnea and oxygen saturation (SaO2) reductions were detected, and CT scans indicated lung infiltration, excluding other causes through a comprehensive infectious work-up and a bronchoalveolar lavage examination. ALP was confirmed and treated with high-dose methylprednisolone and gamma globulin supplement. The patient responded well and was discharged in 2 weeks. In the one-year tapering period and after stopping corticosteroids, the patient recovered well with no relapse detected. In conclusion, the manifestation of IpsO in SLE is rare and represents a challenge for the surgeon to establish the correct diagnosis and avoid inappropriate surgical intervention. ALP may be the consequence of emergency surgery, and immediate high-dose glucocorticoid therapy is recommended. PMID:27347044

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

    PubMed

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

    2015-05-01

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

  9. [The main stages in the history of systemic lupus erythematosus].

    PubMed

    Rampudda, M; Marson, P; Pasero, G

    2009-01-01

    Systemic lupus erythematosus can be considered the most characteristic and important among the connective tissue diseases. In this short review the main stages of its history are sketched, from the introduction of the term "lupus", traditionally attributed to Roger Frugardi, in 1230 (but in fact already documented in the 10th century) to the actual knowledge of its clinical and laboratory aspects. Initially considered exclusively of dermatological interest, the first to describe a systemic form with visceral involvement were Moriz Kohn Kaposi and William Osler. Significant contribution was also given by serological diagnosis, and in particular, by the identification of specific markers of disease, such as anti-native DNA and anti-Sm antibodies, allowing early diagnosis and the establishment of an adequate therapy.

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

    PubMed

    Schreiber, K

    2016-04-01

    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 advice against pregnancy in the 1950s has been replaced by a common understanding that women with SLE often have successful pregnancy outcomes, and clinicians therefore advise on pregnancy planning, including possible drug adjustments, timing and close surveillance. The recently published Predictors 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 the PROMISSE cohort will hopefully identify serological biomarkers, possibly genes, and in addition, give valuable information about underlying disease mechanisms. © The Author(s) 2016.

  11. An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome

    PubMed Central

    Sharmeen, Saika; Hussain, Nazia

    2016-01-01

    Hemophagocytic syndrome (HS) or hemophagocytic lymphohistiocytosis (HLH) is an immune mediated phenomenon that can occur in the setting of an autoimmune disease, chronic immunosuppression, malignancy, or infection. It has been more commonly described in the pediatric population and less commonly in adults. We describe a case of a 52-year-old male who presented with a rash. He simultaneously met the Systemic Lupus International Collaborating Clinics (SLICC) criteria for the diagnosis of systemic lupus erythematosus (SLE) and the diagnostic criteria of HS as described in the hemophagocytic lymphohistiocytosis (HLH) 2004 trial. The bone marrow on autopsy showed the presence of abundant hemosiderophages with focal hemophagocytosis. SLE-associated HS might be underdiagnosed due to the overlap in clinical findings. This case represents the importance of prompt diagnosis and treatment of such a potentially fatal clinical syndrome. PMID:26981305

  12. Acquired winged scapula in a patient with systemic lupus erythematosus.

    PubMed

    Delmonte, S; Massone, C; Parodi, A; Rebora, A

    1998-01-01

    A 34-year-old woman presented with a right winged scapula 8 months after developing systemic lupus erythematosus (SLE) with subacute cutaneous manifestations. The patient experienced severe shoulder pain followed by weakness of the right arm in the typically winged scapula fashion. Electromyography of the serratus anterior showed long thoracic nerve palsy. Clinical and laboratory signs did not reveal any associated disease. Paralysis of the long thoracic nerve has never been described before in SLE.

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

    PubMed

    Gupta, Anu; Jogi, Vishal; Goyal, Manoj Kumar; Modi, Manish; Khurana, Dheeraj

    2014-10-01

    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.

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

    PubMed Central

    Gupta, Anu; Jogi, Vishal; Goyal, Manoj Kumar; Modi, Manish; Khurana, Dheeraj

    2014-01-01

    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. PMID:25506165

  15. Aortic valve replacement in a patient with systemic lupus erythematosus

    PubMed Central

    Kansara, Bhuvnesh; Singh, Ajmer; Karlekar, Anil; Mishra, Yugal K

    2013-01-01

    Valvular heart disease in systemic lupus erythematosus (SLE) is associated with substantial morbidity and mortality. Current therapy includes symptomatic measures and valve replacement. SLE can present major challenges because of accrued organ damage, coagulation defects and complex management regimes. The peri-operative goals are to maintain strict asepsis, avoid use of nephrotoxic drugs and thereby renal insult, and to promote early ambulation post-operatively. PMID:23878452

  16. Asymptomatic diffuse "encephalitic" cerebral toxoplasmosis in a woman with systemic lupus erythematosus.

    PubMed

    Murro, Diana; Novo, Jorge; Arvanitis, Leonidas

    2016-07-01

    Classic cerebral toxoplasmosis typically presents with neurologic symptoms such as seizures and mental status changes and histological examination shows focal lesions with necrosis. However, in the diffuse "encephalitic" form, patients are asymptomatic with diffuse, inflammatory, non-necrotic lesions. Asymptomatic diffuse "encephalitic" toxoplasmosis has been reported only in four acquired immunodeficiency syndrome patients and one human immunodeficiency virus (HIV) negative patient with chronic lymphocytic leukemia. We present a 36-year-old HIV-negative woman with systemic lupus erythematosus and lupus nephritis who was on immunosuppression for 9years after cadaveric renal transplant and died from pulmonary hemorrhage and cytomegalovirus pneumonia. Brain autopsy findings revealed multifocal microglial nodules containing Toxoplasma bradyzoites and associated astrogliosis. These nodules were prominent in the cerebellum, midbrain and medulla and also present in the cortex and thalamus. No coagulative necrosis, necrotizing abscesses, or other opportunistic infections were present. The patient had previously exhibited no neurologic symptoms and there was no clinical suspicion for toxoplasmosis. To the best of our knowledge, this is the first case of diffuse, non-necrotizing, "encephalitic" cerebral toxoplasmosis reported in a lupus patient and also the first reported female case.

  17. A fatal case of seronegative, late-onset systemic lupus erythematosus presenting with motor sensory axonal polyneuropathy.

    PubMed

    Anyfantakis, Dimitrios; Symvoulakis, Emmanouil K; Barbounakis, Emmanouil; Kastanakis, Miltiades; Athanasakis, Evangelos; Blevrakis, Evangelos; Kastanakis, Serafeim

    2014-09-01

    Systemic lupus erythematosus is a multisystemic, autoimmune, inflammatory disorder predominantly affecting young females. Its onset may be abrupt or insidious, presenting with a broad range of clinical and immunological features. We report an unusual case of elderly-onset systemic lupus erythematosus in a woman initially diagnosed with discoid lupus, and subsequently admitted to hospital due to a progressive psycho-motor deficit. Electrophysiological measurements suggested a diagnosis of acute motor sensory axonal neuropathy. Unusual clinical features and negative serology led to diagnostic uncertainty. This case report offers information on the course of the disease through the entire chain of the health care delivery (from primary to tertiary). Despite the efforts of the hospital staff, it was not possible to save the life of the woman.

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

    PubMed

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

    2009-01-01

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

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

    PubMed

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

    2016-11-01

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

  20. Novel evidence-based systemic lupus erythematosus responder index.

    PubMed

    Furie, Richard A; Petri, Michelle A; Wallace, Daniel J; Ginzler, Ellen M; Merrill, Joan T; Stohl, William; Chatham, W Winn; Strand, Vibeke; Weinstein, Arthur; Chevrier, Marc R; Zhong, Z John; Freimuth, William W

    2009-09-15

    To describe a new systemic lupus erythematosus (SLE) responder index (SRI) based on a belimumab phase II SLE trial and demonstrate its potential utility in SLE clinical trials. Data from a randomized, double-blind, placebo-controlled study in 449 patients of 3 doses of belimumab (1, 4, 10 mg/kg) or placebo plus standard of care therapy (SOC) over a 56-week period were analyzed. The Safety of Estrogens in Lupus Erythematosus: National Assessment (SELENA) version of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and British Isles Lupus Assessment Group (BILAG) SLE disease activity instruments, the Short Form 36 health survey, and biomarker analyses were used to create a novel SRI. Response to treatment in a subset of 321 serologically active SLE patients (antinuclear antibodies >/=1:80 and/or anti-double-stranded DNA antibodies >/=30 IU/ml) at baseline was retrospectively evaluated using the SRI. SRI response is defined as 1) a >/=4-point reduction in SELENA-SLEDAI score, 2) no new BILAG A or no more than 1 new BILAG B domain score, and 3) no deterioration from baseline in the physician's global assessment by >/=0.3 points. In serologically active patients, the addition of belimumab to SOC resulted in a response in 46% of patients at week 52 compared with 29% of the placebo patients (P = 0.006). SRI responses were independent of baseline autoantibody subtype. This evidence-based evaluation of a large randomized, placebo-controlled trial in SLE resulted in the ability to define a robust responder index based on improvement in disease activity without worsening the overall condition or the development of significant disease activity in new organ systems.

  1. Serum neuron specific enolase - a novel indicator for neuropsychiatric systemic lupus erythematosus?

    PubMed

    Hawro, T; Bogucki, A; Krupińska-Kun, M; Maurer, M; Woźniacka, A

    2015-12-01

    Neuropsychiatric (NP) lupus, a common manifestation of systemic lupus erythematosus (SLE), is still insufficiently understood, in part, because of the lack of specific biomarkers. Neuron specific enolase (NSE), an important neuronal glycolytic enzyme, shows increased serum levels following acute brain injury, and decreased serum levels in several chronic disorders of the nervous system, including multi infarct dementia, multiple sclerosis and depression. The aim of the study was to evaluate serum NSE levels in SLE patients with and without nervous system involvement, and in healthy controls, and to assess the correlation of NSE serum levels of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) with clinical parameters. The study comprised 47 SLE patients and 28 controls. SLE activity was assessed using the Systemic Lupus Activity Measure (SLAM). A neurologist and a psychiatrist examined all patients. NP involvement was diagnosed according to strict NPSLE criteria proposed by Ainiala and coworkers, as modification to American College of Rheumatology (ACR) nomenclature and case definitions. NSE serum levels were determined by use of an immunoassay. Mean NSE serum concentrations in patients with NPSLE were significantly lower than in non-NPSLE patients (6.3 ± 2.6 µg/L vs. 9.7 ± 3.3 µg/L, p < 0.01) and in controls (8.8 ± 3.3 µg/L, p < 0.05). There were significant negative correlations between NSE serum levels and SLE activity (r = -0.42, p < 0.05) and the number of NPSLE manifestations diagnosed (-0.37; p = 0.001). Decreased serum concentrations of NSE may reflect chronic neuronal damage with declined metabolism of the nervous tissue in patients with NPSLE. © The Author(s) 2015.

  2. Systemic lupus erythematosus following HPV immunization or infection?

    PubMed

    Soldevilla, H F; Briones, S F R; Navarra, S V

    2012-02-01

    The link between autoimmunity and infectious agents has been strongly suggested by reports of lupus or lupus-like syndromes following immunization. This report describes three patients with either newly diagnosed systemic lupus erythematosus (SLE) or SLE flare, following vaccination for human papilloma virus (HPV). CASE 1: A 17-year-old female completed two doses of HPV vaccine uneventfully. Two months later, she developed arthralgias with pruritic rashes on both lower extremities, later accompanied by livedo reticularis, bipedal edema with proteinuria, anemia, leucopenia, hypocomplementemia and high titers of anti-nuclear antibody (ANA) and anti-double-stranded DNA (anti-dsDNA). Kidney biopsy showed International Society of Nephrology/Renal Pathology Society Class III lupus nephritis. She was started on high dose steroids followed by pulse cyclophosphamide therapy protocol for lupus nephritis, and subsequently went into remission. CASE 2: A 45-year-old housewife, previously managed for 11 years as having rheumatoid arthritis, had been in clinical remission for a year when she received two doses of HPV immunization. Four months later, she developed fever accompanied by arthritis, malar rash, oral ulcers, recurrent ascites with intestinal pseudo-obstruction, and behavioral changes. Cranial MRI showed vasculitic lesions on the frontal and parietal lobes. Laboratory tests showed anemia with leucopenia, hypocomplementemia, proteinuria, ANA positive at 1:320, and antibodies against dsDNA, Ro/SSA, La/SSB and histone. She improved following pulse methylprednisolone with subsequent oral prednisone combined with hydroxychloroquine. CASE 3: A 58-year-old housewife diagnosed with SLE had been in clinical remission for 8 years when she received two doses of HPV immunization. Three months later, she was admitted to emergency because of a 1-week history of fever, malar rash, easy fatigability, cervical lymph nodes, gross hematuria and pallor. Laboratory exams showed severe

  3. Cardiac tamponade as initial presentation in systemic lupus erythematosus.

    PubMed

    Jawaid, Ambreen; Almas, Aysha

    2014-05-01

    Systemic Lupus Erythematosus (SLE) is one of the many diseases known as 'the great imitators' because it can have diverse presentations and so is misunderstood for other illnesses. This case illustrates a 19 years old girl with SLE who presented as cardiac tamponade which is a rare feature of lupus pericarditis requiring medical and surgical treatment. Even after pericardiocentesis and steroid therapy there was a re-accumulation of the pericardial fluid resulting in cardiac tamponade which led to pericardial window formation. This case draws attention to the need to consider the diagnosis of tamponade in patients with connective tissue disease and dyspnea or hemodynamic compromise. It also outlines the treatment options available so that surgical referral, if needed, can be done timely for this rare but life threatening manifestation of SLE.

  4. [Tapering and termination of immunosuppressive therapy : Systemic lupus erythematosus].

    PubMed

    Aringer, M; Leuchten, N; Fischer-Betz, R

    2017-02-01

    Similar to patients with other rheumatic diseases, patients with systemic lupus erythematosus (SLE) nowadays can also have the desire to terminate immunosuppressive and immunomodulatory medications. In order to provide appropriate advice to patients, the two main issues are the risk of severe adverse events under long-term therapy with any drug and the perceived risk of a flare, in particular of severe flares. The risks of long-term therapy vary greatly between drugs, ranging from severe unacceptable risks with cyclophosphamide and higher dose glucocorticoids to low risks usually outweighed by long-term benefits with hydroxychloroquine. The individual risk of flares is often difficult to estimate but clinical remission and at least 3 years of immunosuppression are recommended for lupus nephritis. The duration of remission can also be shorter in cases of milder forms of disease. This review article tries to put the available evidence into a clinical perspective and to derive concrete recommendations.

  5. Targeting BLyS in systemic lupus erythematosus.

    PubMed

    Liu, Yaoyang; La Cava, Antonio

    2012-05-01

    Systemic lupus erythematosus (SLE) is a chronic disabling autoimmune disease that significantly impacts the quality of life of patients, and can associate with several complications including end-stage renal disease and shortened lifespan. A central component in the pathogenesis of SLE is the B-cell production of autoantibodies to multiple self-antigens. Since, B lymphocyte stimulator (BLyS) plays a key role in the selection, differentiation and survival of most B cells, it has been studied as a therapeutic target in SLE. After a gap of more than fifty years without new drugs being approved for this disease, the human neutralizing anti-BLyS monoclonal antibody belimumab has recently been approved by the FDA for SLE therapy. This review provides an overview on the targeting of BLyS in lupus animal models, the use of belimumab in human SLE, and relevant patents.

  6. Unmet Needs of Patients with Systemic Lupus Erythematosus

    PubMed Central

    Danoff-Burg, Sharon; Friedberg, Fred

    2009-01-01

    The authors' goal was to assess unmet needs of patients with systemic lupus erythematosus (SLE). Participants (N = 112), who were recruited through the mailing list and support group meetings of a Lupus Alliance of America Affiliate, completed a survey based on prior research. All participants perceived at least 1 unmet need. The most frequently reported unmet needs were in the physical symptoms domain. Older patients were more likely than younger patients to have higher levels of unmet needs related to physical and psychological functioning. African American patients were more likely than white patients to have higher levels of unmet needs related to health services and information. Our findings document the high prevalence and variety of unmet needs among these patients, as well as variations among demographic groups. To address unmet needs of SLE patients, targeted referrals to patient educators, mental health professionals, and support organizations are important adjuncts to medical treatment. PMID:19297299

  7. Polyreactive autoantibodies in systemic lupus erythematosus have pathogenic potential

    PubMed Central

    Zhang, Jie; Jacobi, Annett M.; Wang, Tao; Berlin, RoseAnn; Volpe, Bruce; Diamond, Betty

    2009-01-01

    The present study was undertaken to determine whether germline encoded and polyreactive antibodies might be pathogenic and whether the breach of early tolerance checkpoints in Systemic Lupus Erythematosus (SLE) might lead to a population of B cells expressing germline encoded antibodies that become pathogenic merely by class-switching to IgG in a pro-inflammatory milieu. We demonstrate here that IgM, DNA-reactive antibodies obtained from lupus patients that are unmutated and display polyreactivity can bind to isolated glomeruli and exhibit neurotoxic potential. Thus, the IgM polyreactive repertoire in SLE includes antibodies that may acquire pathogenic function merely by undergoing class-switch recombination to become IgG antibodies. PMID:19398190

  8. Presence of hepatitis-associated antigen in systemic lupus erythematosus

    PubMed Central

    Alarcón-Segovia, D.; Fishbein, Eugenia; Díaz-Jouanen, E.

    1972-01-01

    Presence of hepatitis-associated antigen (HAA) was investigated in 504 sera from 116 patients with SLE and was found in 41% of them. HAA was present in at least one serum in 75% of the patients but there were variations in presence and titres in the same patient at different times. Except for a tendency of HAA to appear or rise in titre with lupusi nactivation following corticosteroid or immunosuppresive therapy, there was no correlation between its presence and disease activity, specific organ involvement, antinuclear antibodies or immunoglobulin levels. All but one of twelve lupus patients with recurrent bacterial infections had HAA at high titres. HAA appeared in the serum of a patient upon development of IgA deficiency. HAA antigenaemia in systemic lupus erythematosus seems a consequence rather than a cause of the immunological derangement in this disease. PMID:4538860

  9. Vaccine-preventable infections in Systemic Lupus Erythematosus

    PubMed Central

    Murdaca, Giuseppe; Orsi, Andrea; Spanò, Francesca; Faccio, Valeria; Puppo, Francesco; Durando, Paolo; Icardi, Giancarlo; Ansaldi, Filippo

    2016-01-01

    Systemic Lupus Erythematosus (SLE) is characterized by abnormal autoantibody production and clearance. Infections are among the most important causes of morbidity and mortality in SLE patients; they have an increased frequency of severe bacterial and viral infections possibly due to inherited genetic and immunologic defects and to immunosuppressive therapies. In addition, infectious agents can switch on lupus disease expression and activity. Among the strategies to reduce the risk of infection, vaccination can be considered the most reliable option. Most vaccines are effective and safe in SLE patients, although in certain cases immunogenicity may be sub-optimal and vaccination can trigger a flare. Although these issues are currently unresolved, the risk benefit balance is in favor for vaccination to reduce the risk of infection in SLE patients. In the present review we discuss the preventive strategies currently recommended to reduce bacterial and viral infections in SLE. PMID:26750996

  10. Cavitary pulmonary lesions in systemic lupus erythematosus: an unusual manifestation.

    PubMed

    Dalili, Amir Reza; Lotfi, Reza; Mousavi, Seyedeh Maryam

    2014-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown pathogenesis. The frequency of SLE with cavitary lesion manifestation is very rare and is thought to be due to infection or pulmonary embolism. A 19-year-old female diagnosed with SLE complicated by lupus nephritis and cavitary pulmonary lesion is presented in this case report. Other diseases that can lead to such lesions were ruled out in the patient. The patient improved briefly after the initiation of immunosuppressive therapy, but was unresponsive to supportive treatment due to pneumothorax. Pneumothorax is caused by cavitary lesions and possibly bronchopleural fistulas - these later caused respiratory distress and death. The patient did not show any improvement in the lesions after the initiation of immunosuppressive therapy. This case report suggests that the differential diagnosis of cavitary lung lesions should include SLE.

  11. Cavitary pulmonary lesions in systemic lupus erythematosus: an unusual manifestation

    PubMed Central

    Dalili, Amir Reza; Lotfi, Reza; Mousavi, Seyedeh Maryam

    2014-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown pathogenesis. The frequency of SLE with cavitary lesion manifestation is very rare and is thought to be due to infection or pulmonary embolism. A 19-year-old female diagnosed with SLE complicated by lupus nephritis and cavitary pulmonary lesion is presented in this case report. Other diseases that can lead to such lesions were ruled out in the patient. The patient improved briefly after the initiation of immunosuppressive therapy, but was unresponsive to supportive treatment due to pneumothorax. Pneumothorax is caused by cavitary lesions and possibly bronchopleural fistulas – these later caused respiratory distress and death. The patient did not show any improvement in the lesions after the initiation of immunosuppressive therapy. This case report suggests that the differential diagnosis of cavitary lung lesions should include SLE. PMID:25763160

  12. Immunosuppression in pregnant women with systemic lupus erythematosus.

    PubMed

    Ponticelli, Claudio; Moroni, Gabriella

    2015-05-01

    Most pregnancies are successful in women with systemic lupus erythematosus, particularly if the disease is quiescent and there are no signs of active nephritis. There is no major impact of immunosuppression on maternal outcome. However, high doses of cyclosporine and glucocorticoids are used which may favor development of hypertension or preeclampsia. Some immunosuppressive drugs may exert toxic effects on the fetus. Glucocorticoids may cause small birth weight, and azathioprine and calcineurin inhibitors may be associated with lower birth weight, gestational age and prematurity. Cyclophosphamide may cause fetal malformation when given in the first trimester. Mycophenolate and leflunomide are teratogenic drugs and should be withdrawn before conception in case of programmed pregnancy or should be rapidly discontinued in case of unexpected pregnancy. Option counseling for pregnancy and correct use of immunosuppressive drugs are prerequisites for a successful pregnancy in women with lupus.

  13. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus.

    PubMed

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

    2014-10-01

    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.

  14. [Systemic lupus erythematosus in the pregnant patient. Implications for anesthesia].

    PubMed

    Pastor Tomás, E; Guillén Antón, J; Vaquerizo Gareta, A; Lirola Grajales, P; Martínez García, R; Cuartero Lobera, J

    2001-03-01

    A 28-year-old woman with systemic lupus erythematosus and a history of aseptic meningitis, digestive bleeding due to thrombopenia and deep venous thrombosis underwent elective cesarean for transverse presentation at 35 weeks. Preoperative blood work-up showed an antinuclear antibody titre that was slightly positive and steroid treatment was started. Surgery operation was performed with general anesthesia. The outcome was satisfactory even though serious complications can develop during the management of anesthesia in such patients. Systemic lupus erythematosus is a chronic, multisystemic disease that mainly affects women of childbearing age. Antibodies and immunocomplexes play a fundamental role. Given the multiorgan involvement in this disease, preoperative study of the lupus patient should assess all such involvement, including maternal-fetal risk, as well as consider the drug and anesthetic management to be applied. Among the clinical signs that can affect management of anesthesia are the following: aseptic meningitis, high blood pressure, pericarditis, pneumonitis and recurrent venous thrombosis. Anemia, thrombopenia and significantly altered coagulation events are common.

  15. Orthopedic surgery and its complication in systemic lupus erythematosus

    PubMed Central

    Mak, Anselm

    2014-01-01

    Systemic lupus erythematosus (SLE) is a multi-systemic immune-complex mediated autoimmune condition which chiefly affects women during their prime year. While the management of the condition falls into the specialty of internal medicine, patients with SLE often present with signs and symptoms pertaining to the territory of orthopedic surgery such as tendon rupture, carpal tunnel syndrome, osteonecrosis, osteoporotic fracture and infection including septic arthritis, osteomyelitis and spondylodiscitis. While these orthopedic-related conditions are often debilitating in patients with SLE which necessitate management by orthopedic specialists, a high index of suspicion is necessary in diagnosing these conditions early because lupus patients with potentially severe orthopedic conditions such as osteomyelitis frequently present with mild symptoms and subtle signs such as low grade fever, mild hip pain and back tenderness. Additionally, even if these orthopedic conditions can be recognized, complications as a result of surgical procedures are indeed not uncommon. SLE per se and its various associated pharmacological treatments may pose lupus patients to certain surgical risks if they are not properly attended to and managed prior to, during and after surgery. Concerted effort of management and effective communication among orthopedic specialists and rheumatologists play an integral part in enhancing favorable outcome and reduction in postoperative complications for patients with SLE through thorough pre-operative evaluation, careful peri-operative monitoring and treatment, as well as judicious postoperative care. PMID:24653977

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

    PubMed

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

    2016-07-15

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

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

    PubMed

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

    2017-05-01

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

  18. Infodemiology of systemic lupus erythematous using Google Trends.

    PubMed

    Radin, M; Sciascia, S

    2017-01-01

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

  19. Depressive symptoms and associated factors in systemic lupus erythematosus.

    PubMed

    Karol, David E; Criscione-Schreiber, Lisa G; Lin, Min; Clowse, Megan E B

    2013-01-01

    Depressive symptoms affect anywhere from 11% to 71% of patients with systemic lupus erythematosus (SLE), which may be related to SLE disease activity, other clinical variables, or sociodemographic factors. We aimed to measure the rate of depressive symptoms in our cohort of patients with SLE and to identify modifiable factors associated with depressive symptoms. Patients in our university-based SLE registry completed the Beck Depression Inventory-II (BDI-II), pain scores, and demographic information. Disease activity was measured using the physician's global assessment (PGA) and Selena-SLE disease activity index (Selena-systemic lupus erythematosus disease activity index (SLEDAI)). Patients were identified as having moderate or severe depressive symptoms (BDI-II ≥ 18) or not (BDI-II < 18). Nonparametric tests and χ(2) tests were used as appropriate to compare variables between groups. Fifty-three of 127 people (41.7%) were identified as having moderate or severe depressive symptoms, which were associated with higher pain levels and lower self-reported of current health status. Patients with moderate or severe depressive symptoms were more likely (49%) than those with no or mild depressive symptoms (18%) to have lupus arthritis (P < 0.01). Of the 53 patients with moderate or severe depressive symptoms, only 26 (49.0%) were prescribed antidepressants, and only 8/53 patients (15.0%) were prescribed the maximum dose of antidepressant. This study identified moderate or severe depressive symptoms in 41.7% of our cohort of patients with SLE. The most significant variable associated with these symptoms was pain; improved treatment of pain, and in particular from lupus arthritis, may result in alleviation of depressive symptoms in patients with SLE. Copyright © 2013 The Academy of Psychosomatic Medicine. Published by Elsevier Inc. All rights reserved.

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

    PubMed

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

    2017-04-25

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

  1. Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus.

    PubMed

    Tincani, A; Rebaioli, C B; Taglietti, M; Shoenfeld, Y

    2006-10-01

    Cardiac involvement is one of the main complications substantially contributing to the morbidity and mortality of patients suffering from systemic autoimmune diseases. All the anatomical heart structures can be affected, and multiple pathogenic mechanisms have been reported. Non-organ-specific autoantibodies have been implicated in immune complex formation and deposition as the initial triggers for inflammatory processes responsible for Libman-Sacks verrucous endocarditis, myocarditis and pericarditis. Anti-phospholipid antibodies have been associated with thrombotic events in coronary arteries, heart valve involvement and intra-myocardial vasculopathy in the context of primary and secondary anti-phospholipid syndrome. Antibodies-SSA/Ro and anti-SSB/La antigens play a major pathogenic role in affecting the heart conduction tissue leading to the electrocardiographic abnormalities of the neonatal lupus syndrome and have been closely associated with endocardial fibroelastosis.

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

    PubMed

    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 < 0.001) and plaque index (P = 0.002) were positively associated; Age was the only variable independently associated with 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.

  3. Selected serum cytokines in systemic lupus erythematosus treated with quinagolide.

    PubMed

    Hrycek, A; Cieślik, P; Tustanowski, J; Nowak, S; Jedynak, P

    2001-01-01

    The objective of this study was to determine the effect of quinagolide (Norprolac) on serum level of cytokines in systemic lupus erythematosus (SLE) patients. In 20 SLE patients treated with a low dose of quinagolide, and in 17 healthy persons who constituted the control group, concentration of serum prolactin (PRL), interleukins (ILs), soluble tumor necrosis factor receptors (sTNF Rs) preceded by calculation of disease activity index (SLEDAI) were tested at entry and then after 3 months in 16 patients and after 6 months in 11 patients who completed the study. Serum PRL level was higher (though insignificantly) in the SLE group than in the controls and decreased significantly after 6 months of therapy. A raised SLEDAI score at entry was significantly reduced during therapy but a weak correlation with PRL level was revealed. A significant increase in IL-6 level in SLE group as compared to controls was observed (respectively 14.57 +/- 13.25 and 5.04 +/- 3.35 microg/ml) as well as a significantly decreased level after 6 months of treatment (4.30 +/- 2.51 pg/ml). There was a significant difference between sTNF RI concentration before and after 3 months of quinagolide treatment (respectively 1140.83 +/- 312.08 and 1454.58 +/- 465.54 pg/ml). After 6 months of treatment a statistically significant correlation between concentration of PRL and level of IL-6 and a negative correlation between PRL and sTNF RI was revealed. Quinagolide treatment may have a role in the management of SLE patients.

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

    PubMed

    Knight, Caroline L; Nelson-Piercy, Catherine

    2017-01-01

    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 followup. This article explores the challenges at each stage of pregnancy, discusses the effect of SLE on pregnancy and vice versa, and reviews antirheumatic medications with the latest guidance about their use and safety in pregnancy. Such information is required to effectively and safely manage each stage of pregnancy in women with SLE.

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

    PubMed Central

    Knight, Caroline L; Nelson-Piercy, Catherine

    2017-01-01

    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 followup. This article explores the challenges at each stage of pregnancy, discusses the effect of SLE on pregnancy and vice versa, and reviews antirheumatic medications with the latest guidance about their use and safety in pregnancy. Such information is required to effectively and safely manage each stage of pregnancy in women with SLE

  6. Mercury in Hair Is Inversely Related to Disease Associated Damage in Systemic Lupus Erythematosus

    PubMed Central

    Crowe, William; Doherty, Leanne; Watson, Gene; Armstrong, David; Ball, Elisabeth; Magee, Pamela; Allsopp, Philip; Bell, Aubrey; Strain, J. J.; McSorley, Emeir

    2015-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease, and environmental factors are proposed to exacerbate existing symptoms. One such environmental factor is mercury. The aim of this study was to investigate the relationship between exposure to mercury (Hg) and disease activity and disease associated damage in Total Hg concentrations in hair and urine were measured in 52 SLE patients. Dental amalgams were quantified. Disease activity was assessed using three indexes including the British Isles Lupus Assessment Group Index (BILAG). Disease associated damage was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology SLICC/ACR Damage Index. Pearson’s correlation identified a significant negative correlation between hair Hg and BILAG (r = −0.323, p = 0.029) and SLICC/ACR (r = −0.377, p = 0.038). Multiple regression analysis identified hair Hg as a significant predictor of disease associated damage as determined by SLICC/ACR (β = −0.366, 95% confidence interval (CI): −1.769, −0.155 p = 0.019). Urinary Hg was not related to disease activity or damage. Fish consumption is the primary route of MeHg exposure in humans and the inverse association of hair Hg with disease activity observed here might be explained by the anti-inflammatory effects of n-3 long chain polyunsaturated fatty acids also found in fish. PMID:26703710

  7. Mercury in Hair Is Inversely Related to Disease Associated Damage in Systemic Lupus Erythematosus.

    PubMed

    Crowe, William; Doherty, Leanne; Watson, Gene; Armstrong, David; Ball, Elisabeth; Magee, Pamela; Allsopp, Philip; Bell, Aubrey; Strain, J J; McSorley, Emeir

    2015-12-23

    Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease, and environmental factors are proposed to exacerbate existing symptoms. One such environmental factor is mercury. The aim of this study was to investigate the relationship between exposure to mercury (Hg) and disease activity and disease associated damage in Total Hg concentrations in hair and urine were measured in 52 SLE patients. Dental amalgams were quantified. Disease activity was assessed using three indexes including the British Isles Lupus Assessment Group Index (BILAG). Disease associated damage was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology SLICC/ACR Damage Index. Pearson's correlation identified a significant negative correlation between hair Hg and BILAG (r = -0.323, p = 0.029) and SLICC/ACR (r = -0.377, p = 0.038). Multiple regression analysis identified hair Hg as a significant predictor of disease associated damage as determined by SLICC/ACR (β = -0.366, 95% confidence interval (CI): -1.769, -0.155 p = 0.019). Urinary Hg was not related to disease activity or damage. Fish consumption is the primary route of MeHg exposure in humans and the inverse association of hair Hg with disease activity observed here might be explained by the anti-inflammatory effects of n-3 long chain polyunsaturated fatty acids also found in fish.

  8. Monoclonal Antibodies for Systemic Lupus Erythematosus (SLE) †

    PubMed Central

    Ponticelli, Claudio; Moroni, Gabriella

    2010-01-01

    A number of monoclonal antibodies (mAb) are now under investigation in clinical trials to assess their potential role in Systemic Lupus Erythematosus (SLE). The most frequently used mAb is rituximab, which is directed against CD20, a membrane protein expressed on B lymphocytes. Uncontrolled trials reported an improvement of SLE activity in non-renal patients and other studies even reported an improvement of severe lupus nephritis unresponsive to conventional treatments. However two randomized trials failed to show the superiority of rituximab over conventional treatment in non renal SLE and in lupus nephritis. Preliminary trials reported promising results with epratuzumab, a humanized mAb directed against CD22, and with belimumab, a human mAb that specifically recognizes and inhibits the biological activity of BLyS a cytokine of the tumor-necrosis-factor (TNF) ligand superfamily. Other clinical trials with mAb directed against TNF-alpha, interleukin-10 (Il-10), Il-6, CD154, CD40 ligand, IL-18 or complement component C5 are under way. At present, however, in spite of good results reported by some studies, no firm conclusion on the risk-benefit profile of these mAbs in patients with SLE can be drawn from the available studies. PMID:27713252

  9. Pregnancy-related issues in women with systemic lupus erythematosus.

    PubMed

    Singh, Abha G; Chowdhary, Vaidehi R

    2015-02-01

    While fertility is preserved in females with systemic lupus erythematosus (SLE), it is well established that pregnancy in these patients is associated with adverse maternal and fetal outcomes, including pregnancy loss, pre-eclampsia, preterm delivery and intrauterine growth retardation, as well as neonatal mortality. Mechanisms underlying these adverse outcomes are poorly understood, and better understanding of these would allow development of targeted and personalized treatment strategies. Established risk factors for adverse pregnancy outcomes include active disease within 6 months prior to conception and during pregnancy, active nephritis, maternal hypertension, antiphospholipid antibodies and hypocomplementemia. While intensive monitoring is recommended, the comparative effectiveness of appropriate management strategies is unclear. While current strategies are able to achieve live births in 85-90% of pregnancies, certain aspects such as prevention of preterm birth, treatment of congenital heart block due to neonatal lupus and recurrent pregnancy loss despite best management, remains challenging. Pregnancy is also associated with an increased risk of flare of lupus, particularly in patients with active disease at time of conception or within 6 months prior to conception. Pregnant patients with SLE should be followed in a high-risk obstetric clinic, and care should be closely coordinated between the obstetrician and rheumatologist.

  10. Environmental factors predicting nephritis in systemic lupus erythematosus.

    PubMed Central

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

    1993-01-01

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

  11. The Incidence and Prevalence of Systemic Lupus Erythematosus, 2002–2004: The Georgia Lupus Registry

    PubMed Central

    Lim, S. Sam; Bayakly, A. Rana; Helmick, Charles G.; Gordon, Caroline; Easley, Kirk; Drenkard, Cristina

    2015-01-01

    Objective The Georgia Lupus Registry is a population-based registry designed to improve our ability to estimate incidence and prevalence of systemic lupus erythematosus (SLE) in a large population. Methods Potential cases were identified from multiple sources during the years 2002 through 2004. Cases were defined by the American College of Rheumatology (ACR) Criteria for SLE or a combined definition. Age-standardized rates were determined and stratified by race and sex. With capture-recapture analyses, we estimated the under-ascertainment of cases. Results Using the ACR case definition, the overall crude and age-adjusted incidence rate was 5.6/100,000, with capture-recapture and combined definition rates being slightly higher. The age-adjusted incidence rate for women was >5 times higher (9.2 vs. 1.8) than that for men. Black women had an incidence rate nearly 3 times higher than that for white women with a significantly higher rate in the 30 to 59 years age group. The overall crude and age-adjusted prevalence rates were 74.4 and 73/100,000, respectively. The age-adjusted prevalence rate for women was nearly 9 times higher (127.6 vs. 14.7) than that for men. Black women had very high rates (196.2). A striking difference was seen in the proportion with end-stage renal disease in prevalent cases, with a sevenfold greater involvement among blacks. Conclusion With more complete case finding, our incidence and prevalence rates are among the highest reported in the United States. Results continue to underscore striking gender, age, and racial disparities between blacks and whites. PMID:24504808

  12. Lupus anticoagulant in systemic lupus erythematosus: a clinical and renal pathological study.

    PubMed

    Farrugia, E; Torres, V E; Gastineau, D; Michet, C J; Holley, K E

    1992-11-01

    Circulating lupus anticoagulant (LA) is associated with thrombosis in large and small vessels. To determine how often the presence of LA is associated with thrombosis within the renal microcirculation, 33 patients with systemic lupus erythematosus (SLE), renal dysfunction, and LA were identified over a 25-year period (LA group) and 32 patients with renal SLE but with normal gross coagulation screen were matched for age, sex, and biopsy timing (C group). Prevalences of serositis, neuropsychiatric illness, leukopenia, thrombocytopenia, hemolysis, anti-DS-DNA elevation, and complement reduction were similar. Arthritis was less and biologic false-positive (BFP) syphilis serology more common in LA. More LA patients had thrombotic events (LA 39% v C 13%; P = 0.014); bleeding episodes, including postbiopsy, were similar. At biopsy, hypertension (LA 55%, C 41%), serum creatinine (mean +/- SD: LA 186 +/- 168 mumol/L [2.1 +/- 1.9 mg/dL] v C 150 +/- 168 mumol/L [1.7 +/- 1.9 mg/dL]) and proteinuria (LA 2.6 +/- 3.1 g/24 h v C 3.1 +/- 2.7) were similar. Lesions by World Health Organization (WHO) class, activity, and chronicity indices, as well as immunofluorescence (IF) and electron microscopy (EM) findings, were not significantly different. Occlusive glomerular, arteriolar, and arterial fibrin thrombi, along with varying degrees of renal thrombotic microangiopathy, were seen in five of 33 patients with LA, but zero of 32 C patients (P = 0.053); three of these five patients died soon after biopsy. Overall, mortality was not different between LA and C. We conclude that the majority of patients with SLE, renal dysfunction, and LA exhibit renal morphologic findings indistinguishable from patients without LA. However, a significant minority of LA patients have thrombotic microangiopathy in their biopsy, which is accompanied by a worse prognosis.

  13. [Kikuchi-Fujimoto disease prior to childhood-systemic lupus erythematosus diagnosis].

    PubMed

    Martins, Sofia S; Buscatti, Izabel M; Freire, Pricilla S; Cavalcante, Erica G; Sallum, Adriana M; Campos, Lucia M A; Silva, Clovis A

    2014-01-01

    Kikuchi-Fujimoto disease (KFD) is a self-limiting histiocytic necrotizing lymphadenitis of unknown origin. Of note, KFD was infrequently reported in adult systemic lupus erythematosus (SLE), with rare occurrence in childhood-SLE (C-SLE) patients. To our knowledge, the prevalence of KFD in the paediatric lupus population was not studied. Therefore, in a period of 29 consecutive years, 5,682 patients were followed at our institution and 289 (5%) met the American College of Rheumatology classification criteria for SLE, one had isolated KFD (0.03) and only one had KFD associated to C-SLE diagnoses, which case was reported herein. A 12 year-old female patient had high fever, fatigue and cervical and axillary lymphadenopathy. The antinuclear antibodies (ANA) were negative, with positive IgM and IgG herpes simplex virus type 1 and type 2 serologies. Fluorine-18-fluoro-deoxy-glucose positron emission tomography/computed tomography (PET/CT) imaging demonstrated diffuse lymphadenopathy. The axillary lymph node biopsy showed necrotizing lymphadenitis with histiocytes, without lymphoproliferative disease, compatible with KFD. After 30 days, she presented spontaneous regression and no therapy was required. Nine months later, she developed malar rash, photosensitivity, oral ulcers, lymphopenia and ANA 1:320 (homogeneous nuclear pattern). At that moment the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score was 10 and she was treated with prednisone (1.0mg/kg/day) and hidroxychloroquine showing progressive improvement of hers signs and symptoms. In conclusion, KFD is a benign and rare disease in our paediatric lupus population. We also would like to reinforce the relevance of autoimmune diseases diagnosis during the follow-up of patients with KFD.

  14. Epileptic seizures and EEG features in juvenile systemic lupus erythematosus.

    PubMed

    Vieira-Karuta, Simone Carreiro; Silva, Izabella Celidônio Bertoldo; Liberalesso, Paulo Breno Noronha; Bandeira, Márcia; Janz, Loris; Löhr, Alfredo

    2008-09-01

    Juvenile systemic lupus erythematosus is more incident in female affecting different systems including the central nervous system. The aim of this study was to check the incidence of seizures and electroencephalographic features in these patients. It was analyzed all patients with juvenile systemic lupus erythematosus referred to the Pequeno Príncipe Hospital in Curitiba, PR, Brazil, in the year of 2007. The patients were submitted to EEG and subdivided into two groups according to the presence or absence of epileptic seizures. Mann-Whitney statistical test was used. Forty-nine cases were included, there were 73.45% female, with an age between 3 and 28 years (micro=17.00 years; s=5.01 years). Seizures (13/26.50%) were the most frequent manifestation followed by headache (13/26.50%) and ischemic stroke (6/12.25%). Cerebral vasculites were the most frequent alteration in neuroimage. The abnormalities of EEG were characterized by asymmetry of the electric cerebral activity, diffuse disorganized background activity, focal epileptiform discharges in the right central-temporal region, generalized paroxysmal of 3 Hz spike-waves, and bursts of theta-delta slowness activity in the right parietal-occiptal region. The statistic analysis showed no significantly difference between age of onset of symptoms and the risk of seizures (p 0.675) as well as between time of the disease and the risk of seizures (p 0.436). Neurologic manifestations, in special epileptic seizures, are frequent in systemic lupus erythematosus. Age of onset of symptoms and the time of disease did not increase the risk of epileptic seizures in this disease.

  15. Therapeutic interventions of tissue specific autoimmune onset in systemic lupus erythematosus.

    PubMed

    Dasgupta, Subhajit; Dasgupta, Shaoni

    2016-06-10

    Systemic lupus erythematosus (lupus) is a female predominant autoimmune disease. The auto reactive B cells and T helper cells together are known to develop self-reactive immune responses in different tissues like kidney, bone, cardiovascular and central nervous system. Progression of disease is associated with deposition of immune complex which initiates tissue damage. The therapy for lupus still includes corticosteroids to reduce allergic manifestations and inflammatory immune responses. Recent observations suggested that, mycophenolate mofetil and cyclophosphamide treatment in combination with corticosteroids have benefit in lupus therapy. The prospect of B cell depletion by CD20 targeted monoclonal antibody Rituximab has been demonstrated in lupus patients. The CD52 specific monoclonal antibody Alemtuzumab is another proposition for lupus therapy. The drug Belimumab inhibits B cell activation by altering BAFF/APRIL signal cascade. Recent discovery of the CD22 targeted Epratuzumab also shows therapeutic prospect. The researches on new generation drugs for autoimmune lupus include search for inhibitors of CD40-CD40Ligand interactions, CD86 activation, selective modulation of complement cascades. The choice of inhibitors of transcription factor NF-κBp65 and selective modulators for estrogen receptor alpha are proposed areas of lupus drug discovery research. Keeping a close eye on the mechanisms of disease onset, a comprehensive view is provided on recent therapy of systemic lupus erythematosus.

  16. Risk factors for neuropsychiatric manifestations in children with systemic lupus erythematosus: case-control study.

    PubMed

    Zuniga Zambrano, Yenny Carolina; Guevara Ramos, Juan David; Penagos Vargas, Nathalia Elena; Benitez Ramirez, Diana Carol; Ramirez Rodriguez, Sandra Milena; Vargas Niño, Adriana Carolina; Izquierdo Bello, Alvaro Hernando

    2014-09-01

    Neuropsychiatric symptoms in children with systemic lupus erythematosus cause high morbidity and disability. This study analyzed risk factors associated with neuropsychiatric presentation in patients with systemic lupus erythematosus aged <18 years. A case-control study was performed. Medical record information of patients with a diagnosis of systemic lupus erythematosus who were hospitalized with or without neuropsychiatric symptoms was collected between March 2007 and January 2012. Clinical variables, laboratory examinations, neuroimages, and disease activity (Systemic Erythematosus Lupus Disease Activity Index) and damage (Systemic Lupus International Collaborating Clinics) indices were analyzed. A total of 90 patients were selected, 30 with neuropsychiatric symptoms. The patients' average age was 12.2 years. The most common neuropsychiatric symptoms were seizures, migraine, and depression. The average Systemic Erythematosus Lupus Disease Activity Index was 19.86 (S.D. 10.83) and the average Systemic Lupus International Collaborating Clinics index was 2.02 (S.D. 2.43), with higher values in patients with neuropsychiatric symptoms (P = 0.001). The levels of complement C3 and C4 were significantly higher in patients with a neuropsychiatric disorder (P = 0.003). Lupus anticoagulant was found in 51.5% of patients with neuropsychiatric symptoms (odds ratio, 3.7; 95% confidence interval, 1.3-10.0). Immunosuppression with azathioprine, rituximab, or cyclophosphamide delayed the time to neuropsychiatric systemic lupus erythematosus development by 18.5 months (95% confidence interval, 10.6-26.5) compared to patients who did not receive these agents. The presence of lupus anticoagulant was a risk factor in our patients. The use of immunosuppressants, such as cyclophosphamide, rituximab, and azathioprine, delayed the presentation of neuropsychiatric manifestations of lupus. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; review of literature.

    PubMed

    Rezaieyazdi, Zahra; Sharifi-Doloui, Davood; Hashemzadeh, Kamila; Shirdel, Abbas; Mansouritorghabeh, Hassan

    2012-01-01

    Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE) is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced factor VIII activity and a high titer of FVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, factor VIII inhibitor assay was negative.

  18. Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus: review of literature.

    PubMed

    Rezaieyazdi, Zahra; Sharifi-Doloui, Davood; Hashemzadeh, Kamila; Shirdel, Abbas; Mansouritorghabeh, Hassan

    2011-12-01

    Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE), is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII (fVIII) inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced fVIII activity and a high titre of fVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, fVIII inhibitor assay was negative.

  19. Pharmacological Management of Childhood-Onset Systemic Lupus Erythematosus.

    PubMed

    Thorbinson, Colin; Oni, Louise; Smith, Eve; Midgley, Angela; Beresford, Michael W

    2016-06-01

    Systemic lupus erythematosus (SLE) is a rare, severe, multisystem autoimmune disorder. Childhood-onset SLE (cSLE) follows a more aggressive course with greater associated morbidity and mortality than adult-onset SLE. Its aetiology is yet to be fully elucidated. It is recognised to be the archetypal systemic autoimmune disease, arising from a complex interaction between the innate and adaptive immune systems. Its complexity is reflected by the fact that there has been only one new drug licensed for use in SLE in the last 50 years. However, biologic agents that specifically target aspects of the immune system are emerging. Immunosuppression remains the cornerstone of medical management, with glucocorticoids still playing a leading role. Treatment choices are led by disease severity. Immunosuppressants, including azathioprine and methotrexate, are used in mild to moderate manifestations. Mycophenolate mofetil is widely used for lupus nephritis. Cyclophosphamide remains the first-line treatment for patients with severe organ disease. No biologic therapies have yet been approved for cSLE, although they are being used increasingly as part of routine care of patients with severe lupus nephritis or with neurological and/or haematological involvement. Drugs influencing B cell survival, including belimumab and rituximab, are currently undergoing clinical trials in cSLE. Hydroxychloroquine is indicated for disease manifestations of all severities and can be used as monotherapy in mild disease. However, the management of cSLE is hampered by the lack of a robust evidence base. To date, it has been principally guided by best-practice guidelines, retrospective case series and adapted adult protocols. In this pharmacological review, we provide an overview of current practice for the management of cSLE, together with recent advances in new therapies, including biologic agents.

  20. Purtscher-like retinopathy in systemic lupus erythematosus.

    PubMed

    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.

  1. Structural Brain Network Reorganization in Patients with Neuropsychiatric Systemic Lupus Erythematosus.

    PubMed

    Xu, X; Hui, E S; Mok, M Y; Jian, J; Lau, C S; Mak, H K F

    2017-01-01

    Patients with neuropsychiatric systemic lupus erythematosus have worse outcomes compared with those with systemic lupus erythematosus. A better understanding of the mechanisms of neuropsychiatric systemic lupus erythematosus could potentially improve diagnosis and management. The goal of this study was to investigate the differences in the structural brain network of patients with neuropsychiatric systemic lupus erythematosus compared with patients with systemic lupus erythematosus by using brain connectivity analysis. We recruited 20 subjects for each patient cohort and age-matched healthy controls. The topology and efficiency of the network and the characteristics of various brain hubs were investigated by using brain connectivity analysis of diffusion MR imaging data. There were more extensive reorganizations in the structural brain network of patients with neuropsychiatric systemic lupus erythematosus than in patients with systemic lupus erythematosus. For example, the network of the former had significantly decreased clustering coefficient and local efficiency. They also had significantly lower nodal efficiency in the superior temporal gyrus (P = .046) and middle temporal gyrus (P = .041). Our results hint at a plausible relationship between the neuropsychiatric symptoms and reorganization of the structural brain network of patients with systemic lupus erythematosus. Brain connectivity analysis may be a potential tool to subtype these patients. © 2017 by American Journal of Neuroradiology.

  2. Maternal systemic lupus erythematosus and chondrodysplasia punctata in two sibs: phenocopy or coincidence?

    PubMed Central

    Elçioglu, N; Hall, C M

    1998-01-01

    Two sibs with chondrodysplasia punctata in whom the mother was suffering from systemic lupus erythematosus are presented and the radiological features described. Comparison with other forms of chondrodysplasia punctata with a review of the relevant publications is presented and the possible association with maternal systemic lupus erythematosus is highlighted. Images PMID:9719382

  3. Case report: disseminated dermatophytosis by microsporum gypseum in a systemic lupus erythematosus patient

    PubMed Central

    Macêdo, Danielle Patrícia Cerqueira; Neves, Rejane Pereira; Lopes, Flávia Cadengue

    2008-01-01

    Mycosis is a major contributor to morbidity and mortality in patients with systemic lupus erythematosus and frequent exposition to an infectious source could enhance the development of dermatophytic infections. A case of disseminated dermatophytosis by Microsporum gypseum is reported in a systemic lupus erythematosus (SLE) patient. PMID:24031171

  4. Anti-C1q in systemic lupus erythematosus.

    PubMed

    Stojan, G; Petri, M

    2016-07-01

    C1q is the first component of the classical complement pathway. Both clinically validated in-house ELISA assays as well as commercial ELISA kits are used for detection of anti-C1q antibodies. Anti-C1q autoantibodies can be detected in a wide range of autoimmune diseases and are highly sensitive for hypocomplementemic uticarial vasculitis. In SLE, anti-C1q are strongly associated with proliferative lupus nephritis, and their absence carries a negative predictive value for development of lupus nephritis of close to 100%. Anti-C1q in combination with anti-dsDNA and low complement has the strongest serological association with renal involvement. The anti-C1q titers correlate with global disease activity scores in patients with renal involvement, and higher titers seem to precede renal flares. After the successful treatment of a renal flare, anti-C1q has the tendency to decrease or even become undetectable. The main obstacle to the inclusion of anti-C1q in the classification criteria and clinical management of SLE is the lack of standardized laboratory assays.

  5. Defining Low Disease Activity in Systemic Lupus Erythematosus.

    PubMed

    Polachek, Ari; Gladman, Dafna D; Su, Jiandong; Urowitz, Murray B

    2017-07-01

    To define and identify a group of systemic lupus erythematosus patients with low disease activity (LDA) and to examine whether LDA is similar to patients in remission and different from a high disease activity group (HDA) in short-term outcomes. The LDA group was defined as Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) <3, including only 1 clinical manifestation of rash, alopecia, mucosal ulcers, pleurisy, pericarditis, fever, thrombocytopenia, or leukopenia. The patients could be taking antimalarials. Remission was defined as no clinical manifestation from taking antimalarials alone, and the HDA group was defined as SLEDAI-2K >6. The time frame for inclusion in each group was at least 1 year. Of 620 patients with active disease who were seen between 1970 and 2015, 80 patients (12.9%) fulfilled the criteria for LDA, 191 (30.8%) for remission, and 349 (56.3%) for HDA. The LDA patients with and without positive serology results were similar at baseline and with prior disease characteristics. After 2 years of followup, the LDA and remission groups were similar in their adjusted mean SLEDAI-2K score, organ involvement, The Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) score, mortality, and therapies. After 2 and 4 years of followup, the HDA group had a higher adjusted mean SLEDAI-2K score, more major organ involvement, a higher SDI score, higher mortality, and more therapy compared to the combined LDA/remission groups. LDA and remission groups had similar short-term outcomes, and both had better outcomes and prognosis than the HDA group. LDA may be used as an outcome measure in therapeutic trials or in treat-to-target regimens. © 2016, American College of Rheumatology.

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

    PubMed

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

    2017-01-01

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

  7. Current concepts in immunosuppressive drug therapy of systemic lupus erythematosus.

    PubMed

    Lehman, T J

    1992-04-01

    The routine use of intravenous cyclophosphamide has led to advances in the preservation of renal function and quality of life for patients with systemic lupus erythematosus complicated by diffuse proliferative glomerulonephritis. Most patients receiving 3 years of intravenous cyclophosphamide according to a rigorous protocol experience longterm remission of their disease. However, in some the disease continues to progress or flares recurrently. Early intervention and combined immunosuppressive regimens modeled on protocols developed for the treatment of neoplastic disease appear to offer the greatest likelihood of attaining permanent remission for large numbers of patients. Pilot studies of these regimens are in progress.

  8. Emerging concepts in the molecular pathogenesis of systemic lupus erythematosus.

    PubMed

    Nambiar, Madhusoodana P; Juang, Yuang-Tuang; Tsokos, George C

    2002-01-01

    Systemic lupus erythematosus is a prototypic autoimmune disease that afflicts predominantly women during their child-bearing age. The disease is characterized by the production of autoantibodies and immune complexes in association with a diverse array of clinical manifestations. Investigation into the etiopathogenesis has been directed at identifying the genes that provide susceptibility to the disease, the complex cellular and cytokine aberrations and the biochemical abnormalities that are responsible for them. Understanding the immune cell signaling and gene transcription abnormalities will help us tailor new strategies for efficient biotherapy of the disease.

  9. Abnormalities of T cell signaling in systemic lupus erythematosus

    PubMed Central

    2011-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease resulting from a loss of tolerance to multiple self antigens, and characterized by autoantibody production and inflammatory cell infiltration in target organs, such as the kidneys and brain. T cells are critical players in SLE pathophysiology as they regulate B cell responses and also infiltrate target tissues, leading to tissue damage. Abnormal signaling events link to defective gene transcription and altered cytokine production, contributing to the aberrant phenotype of T cells in SLE. Study of signaling and gene transcription abnormalities in SLE T cells has led to the identification of novel targets for therapy. PMID:21457530

  10. Acquired hemophilia A in a patient with systemic lupus erythematosus.

    PubMed

    Ishikawa, T; Tsukamoto, N; Suto, M; Uchiumi, H; Mitsuhashi, H; Yokohama, A; Maesawa, A; Nojima, Y; Naruse, T

    2001-06-01

    A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia A. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor VIII activity (2.8 %) and the presence of factor VIII inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical.

  11. Systemic lupus erythematosus accompanying with renal tuberculosis: a case report

    PubMed Central

    Li, Tao; Gao, Liang; Chen, Peng; Bu, Si-Yuan; Cao, De-Hong; Yang, Lu; Wei, Qiang

    2015-01-01

    A 26-year-old woman, with a six-year history of well-controlled systemic lupus erythematosus (SLE), complained of urinary frequency and urgency. After failure of commonly-used antibiotic therapy, mycobacterium tuberculosis was cultured from her urine and renal tuberculosis (TB) was diagnosed. However, she underwent right nephrectomy after the combination therapies of prednisone for SLE and anti-tuberculosis treatment for renal TB failed. To our knowledge, SLE accompanying renal TB is rare, and such a rapid deterioration in renal function has never been reported. PMID:26221395

  12. Vaccination of Adult Patients with Systemic Lupus Erythematosus in Portugal

    PubMed Central

    Moraes-Fontes, Maria Francisca; Antunes, Ana Margarida; Gruner, Heidi; Riso, Nuno

    2016-01-01

    In the wake of the Portuguese vaccination program 50th anniversary it seems appropriate to review vaccination in patients with systemic lupus erythematosus. Controversial issues as regards the association between autoimmune diseases, infections, and vaccines are discussed as well as vaccine safety and efficacy issues as regards chronic immunosuppressant (IS) drug therapy. After a brief overview of national policies, specific recommendations are made as regards vaccination for adult patients with SLE with a particular focus on current IS therapy and unmet needs. PMID:27069477

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

    PubMed

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

    2017-02-08

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

  14. [The influence of pregnancy on the systemic lupus erythematosus].

    PubMed

    Basheva, S; Nikolov, A; Monov, C; Shumnalieva, R; Monova, D; Rashkov, R

    2014-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease caused by the interaction between genetic and environment factors which leads to abnormal immune responses. SLE affects more commonly women of childbearing age which raises the following questions--the influence of the disease activity on pregnancy and the influence of pregnancy on disease activity. On the one hand physiological changes occurring during pregnancy could lead to increased SLE activity, on the other hand the latter could mimic SLE activity. Differentiating these manifestations is important for the clinical practice--pregnancy and delivering guidance and SLE therapy.

  15. Broad Concepts in Management of Systemic Lupus Erythematosus.

    PubMed

    Chowdhary, Vaidehi R

    2017-05-01

    Systemic lupus erythematosus is a multisystem autoimmune disease with protean manifestation. Although commonly seen in young women, it can affect men as well as elderly patients. Approach to treatment is multidisciplinary, involves defining the extent of organ involvement, and distinguishing between active manifestations and damage. The mainstay of therapy is judicious use of immunosuppressive medications. Long-term follow-up to address morbidity arising from treatment complications, disease damage, and increased cardiovascular risk is essential. Copyright © 2017 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

  16. Congenital heart block and maternal systemic lupus erythematosus.

    PubMed Central

    Esscher, E; Scott, J S

    1979-01-01

    The association between infants with congenital heart block (CHB) and the presence or later development of maternal systemic lupus erythematosus or other connective-tissue disease (CTD) was reviewed in 67 cases. In 24 cases CHB was diagnosed at or before birth. Of nine necropsies on affected infants, seven showed endomyocardial fibrosis. The results suggest that one in three mothers who deliver babies with CHB have or will develop CTD. The association is probably explained by placental transfer of a maternal antibody. Awareness of the association may lead to prevention of the birth of children with CHB and better neonatal care of affected children. PMID:455010

  17. An Unusual Mimicker of Systemic Lupus Erythematosus: A Case Report

    PubMed Central

    Aluoch, Aloice O; Farbman, Mathew; Gladue, Heather

    2015-01-01

    We present a case of a 47 year-old African American female with 15 pack-years of tobacco use and heavy alcohol use who presented with arthritis and was found to have a positive antinuclear antibodies (ANA), anti double stranded DNA antibodies (anti-dsDNA), and anti-Sjogren’s syndrome-related antigen A and antigen B (anti-SSA and anti-SSB). She was subsequently found to have a lung adenocarcinoma associated with hypertrophic pulmonary osteoarthropathy (HPO). This demonstrates a case of positive antinuclear antibodies and arthritis in a patient with lung adenocarcinoma, which can be falsely diagnosed as systemic lupus erythematosus. PMID:26106457

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

    PubMed Central

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

    2016-01-01

    Alveolar hemorrhage (AH) is a rare syndrome that can often occur in autoimmune diseases, blood clotting disorders, infection or by acute inhalation injury, presenting rapid evolution and high mortality, especially with late diagnosis and treatment. Among the autoimmune diseases, there are reported cases in patients with primary antiphospholipid syndrome (PAPS), vasculitis and systemic lupus erythematosus (SLE). An early diagnosis is an essential tool in the successful management of this complication, requiring aggressive treatment based on vigorous immunosuppression and broad-spectrum antibiotic. We describe here a case of alveolar hemorrhage associated with glomerulonephritis as the open presentation in a patient with SLE. PMID:27994272

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

    PubMed

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

    2016-01-01

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

  20. Does parvovirus infection have a role in systemic lupus erythematosus?

    PubMed

    Hod, Tami; Zandman-Goddard, Giselle; Langevitz, Pnina; Rudnic, Hagit; Grossman, Zehava; Rotman-Pikielny, Pnina; Levy, Yair

    2017-01-23

    We sought to evaluate a possible link between parvovirus B19 infection and the clinical and laboratory expression of systemic lupus erythematosus (SLE). SLE patients were examined to evaluate their clinical status and disease activity. A complete Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score was obtained for each patient. In addition, we determined the level of systemic involvement throughout the course of the disease. Blood levels of IgM and IgG antibodies to parvovirus B19, levels of anti-dsDNA, C3, and C4 were measured. A PCR real-time assay was used to determine the presence of parvovirus B19 genetic material. The viral genome was found in sera of 2 of 51(3.9%) patients with SLE. There was no correlation between viral serology and the clinical and serological parameters of the disease. More SLE patients with secondary antiphospholipid syndrome (APS) had IgG and IgM antibodies to the virus (p < 0.029 and p < 0.018, respectively). These patients also had a higher titer of IgG antibodies to parvovirus B19 compared to SLE patients without APS. In this group of SLE patients, no association was found between parvovirus infection and the presence or activity of SLE. The results of the study suggest an association between parvovirus infection and antibody production directed against phospholipids.

  1. [Neuropsychiatric systemic lupus erythematosus (1st part). Cases definitions and diagnosis and treatment of central nervous system and psychiatric manifestations of systemic lupus erythematosus].

    PubMed

    Lefèvre, G; Zéphir, H; Warembourg, F; Michelin, E; Pruvo, J-P; Hachulla, E; Semah, F; Dubucquoi, S; Lenfant, P; Vermersch, P; Hatron, P-Y; Prin, L; Launay, D

    2012-09-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease, which primarily affects skin and joints. Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself. A classification, published in 1999 by the American College of Rheumatology (ACR) research committee, described 12 CNS syndromes and seven peripheral neurologic syndromes compatible with "neuropsychiatric systemic lupus erythematosus" (NPSLE). Despite this consensus, studies which have been published since 1999 have reported a prevalence of NPSLE varying from 20 to 97 %, which shows the diagnosis difficulty and the heterogeneity of neuropsychiatric manifestations in SLE. In order to understand the limits of this classification, we propose in this first part an exhaustive review of publications describing neuropsychiatric manifestations according to the ACR 1999 classification. We also detail case definitions, prevalence and risk factors, clinical characteristics and diagnosis of each lupus-related psychiatric and CNS manifestation. Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

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

    PubMed

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

    2012-06-01

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

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

    PubMed

    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.

  4. Systemic lupus erythematosus in Asturias, Spain: clinical and serologic features.

    PubMed

    Gómez, Jesús; Suárez, Ana; López, Patricia; Mozo, Lourdes; Díaz, José Bernardino; Gutiérrez, Carmen

    2006-05-01

    Asturias is an autonomous region in the north of Spain with historical and anthropologic peculiarities. In the current report, we examine the main clinical and immunologic features of 363 patients with systemic lupus erythematosus (SLE), virtually the entire population of SLE patients in Asturias. We constructed a database with the clinical and immunologic features of all patients fulfilling the American College of Rheumatology criteria, based on the review of hospital records corresponding to blood samples received for antinuclear antibodies testing since 1992. Arthritis was the most frequently observed main clinical feature and neuropathy was the rarest. Male patients had a disease more frequently characterized by serositis (p<0.05) and neurologic disorder (p<0.01) than females, while children presented malar rash (p<0.05), fever (p<0.05), and kidney involvement (p<0.01) more often than adults. Late-onset patients were characterized by lower frequencies of malar rash (p<0.01), neurologic disorder (p<0.05), alopecia (p<0.01), and lymphadenopathy (p<0.05) than young adults. Numerous direct and inverse significant associations were found among clinical and immunologic features. The most relevant significant associations were neurologic disorder with lupus anticoagulant (p<0.01); kidney involvement with serositis (p<0.01) and DNA antibodies (p<0.05); and thrombosis with DNA antibodies (p<0.05), cardiolipin antibodies (p<0.01), and lupus anticoagulant (p<0.01). A low mortality was found in our series, although kidney involvement (p<0.05) and cardiolipin antibodies (p<0.05) are factors associated with poor survival.

  5. New-onset systemic lupus erythematosus during pregnancy.

    PubMed

    Zhao, Chunmei; Zhao, Jijun; Huang, Yuefang; Wang, Zilian; Wang, Hongyue; Zhang, Hui; Xu, Hanshi; Yang, Niansheng

    2013-06-01

    Few studies have been published focusing on the clinical features of new-onset systemic lupus erythematosus (SLE) during pregnancy. This study examined the clinical characteristics of SLE during pregnancy or puerperium. The clinical characteristics and serological parameters of 48 patients with onset of SLE during pregnancy were retrospectively compared with those of age-matched new-onset SLE patients who were diagnosed in a period of more than 12 months without pregnancy (n = 65) and age-matched preeclampsia patients (n = 48). SLE tended to occur during the first and second trimesters (33 and 42 %, respectively). Lupus nephritis (LN) and severe thrombocytopenia were more commonly seen in new-onset SLE during pregnancy than in patients without pregnancy (68.8 vs 35.4 % and 25 vs 9.2 %, respectively, p < 0.05). However, pregnant patients had lower frequency of fever, arthritis, arthralgia, alopecia, oral ulcer, and hypocomplementemia than the nonpregnant controls (p < 0.05). Compared to LN patients without pregnancy (n = 23), LN patients with pregnancy (n = 33) had more prominent proteinuria and nephrotic syndrome (p < 0.05). On the other hand, when compared to patients with preeclampsia, patients with new-onset SLE during pregnancy had early onset of symptoms during gestation and were characterized by presence of fever, malar lesion, autoantibodies, hypocomplementemia, hyperuricemia, active urinary sediment, and multi-organ involvement. In conclusion, patients with their first onset of lupus during pregnancy generally have more severe disease with higher prevalence of renal and platelet involvement.

  6. Depression, Medication Adherence, and Service Utilization in Systemic Lupus Erythematosus

    PubMed Central

    Julian, Laura J.; Yelin, Edward; Yazdany, Jinoos; Panopalis, Pantelis; Trupin, Laura; Criswell, Lindsey A.; Katz, Patricia

    2009-01-01

    Objective Forgetting to take medications is an important cause of nonadherence. This study evaluated factors associated with forgetting to take medications in a large cohort of persons with systemic lupus erythematosus (SLE) participating in the University of California, San Francisco Lupus Outcomes Study (LOS). Relationships among adherence problems and service utilization (outpatient visits, emergency department visits, and hospitalizations) were also evaluated. Methods The cohort consisted of 834 LOS participants who provided self-reported frequency of forgetting to take medications as directed. Predictors of adherence and service utilization patterns included self-reported sociodemo-graphics, disease-related characteristics (e.g., disease activity, recent SLE flare), and mental health characteristics (Center for Epidemiologic Studies Depression Scale and cognitive function screen). Health care utilization patterns included the presence and quantity of visits to rheumatologists, primary care physicians, other care providers, emergency departments, and hospitalizations. Results Forty-six percent of the LOS cohort reported forgetting to take medications at least some of the time. Depressive symptom severity was a strong predictor of adherence difficulties (odds ratio [OR] 1.04, 95% confidence interval [95% CI] 1.02–1.05; P < 0.0001) after accounting for all other predictors. Persons reporting adherence difficulties had significantly greater numbers of outpatient rheumatology and primary care visits, and were more likely to visit the emergency department (OR 1.45, 95% CI 1.04–2.04; P = 0.03). Conclusion Depression may be an important cause of medication adherence problems, and difficulties with adherence are significantly associated with high-cost service utilization, specifically emergency department visits. In an era of rapidly evolving treatments for lupus, identifying patients at risk for adherence problems may decrease medical expenditures and improve

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

    PubMed

    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

  8. The Systemic Lupus International Collaborating Clinics (SLICC) group - it was 20 years ago today.

    PubMed

    Isenberg, D A; Ramsey-Goldman, R; Gladman, D; Hanly, J G

    2011-11-01

    The Systemic Lupus International Collaborating Clinics (SLICC) group is 20 years old this year (2011). This brief review traces the origins of the group focussing on its more recent history and reviewing some of its major contributions to lupus research during the past two decades.

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

    PubMed Central

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

    2011-01-01

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

  10. Lupus mastitis.

    PubMed

    Cerveira, Isabel; Costa Matos, L; Garrido, António; Oliveira, Elda; Solheiro, Helena; Bastos, Marina; Cortez Vaz, F; Nogueira Martins, F

    2006-10-01

    We report a case of a 28-year-old female with the diagnosis of systemic lupus erythematosus (SLE) referred to our breast pathology consultancy in 2002 due to a left breast nodule. Further investigation revealed bilateral coarse calcifications. Biopsy was consistent with a diagnosis of lupus mastitis.

  11. Myocardial ischaemia in systemic lupus erythematosus: detection and clinical relevance.

    PubMed

    Płazak, Wojciech; Gryga, Krzysztof; Sznajd, Jan; Pasowicz, Mieczysław; Musiał, Jacek; Podolec, Piotr

    2011-01-01

    Severe cardiovascular complications are among the most important causes of mortality in systemic lupus erythematosus (SLE) patients. To assess the usefulness of echocardiography, ECG, and coronary artery calcium scoring (CACS) in the detection of myocardial ischaemia in SLE patients compared to single photon emission computerised tomography (SPECT) and to assess their five-year follow-up. In 50 consecutive SLE patients (mean age 39.2 ± 12.9 years, 90% female), clinical assessment, resting and exercise ECG and echocardiography, multidetector computed tomography - based CACS and SPECT studies (Tc-99m sestamibi) were performed. Patients were then followed for five years. SPECT revealed perfusion defects in 25 (50%) patients; persistent defects in 18 (36%) and exercise-induced defects in seven (14%) subjects. No typical ischaemic heart disease clinical symptoms, signs of ischaemia in resting ECG, or left ventricular contractility impairment in echocardiography were observed. Signs of ischaemia in exercise ECG were found in 17 (34%) patients. The CACS ranged from 1 to 843.2 (median 23.15), and coronary calcifications were observed in 12 (24%) patients. Compared to the SPECT study, exercise ECG had 68% sensitivity and 100% specificity in detecting myocardial ischaemia, while CACS had only 28% sensitivity and 58% specificity. During follow-up, one patient who showed myocardial perfusion defects and the highest calcium score (843.2) at baseline, developed CCS II class symptoms of myocardial ischaemia. Coronary angiography was not performed because of severe anaemia; the patient died three months later. In two other patients with perfusion defects and calcium deposits at baseline, CCS I class symptoms were observed; coronary angiography showed only thin calcified coronary plaques that were haemodynamically insignificant. In about half of relatively young, mostly female, SLE patients, SPECT shows myocardial perfusion defects, with coronary calcifications present in one

  12. Mood Disorders in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study.

    PubMed

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

    2015-07-01

    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). 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 of Rheumatology Damage Index [SDI]), and Short Form 36 subscales, mental and physical component summary scores were collected. Time to event, linear and ordinal regressions, and multi-state models were used as appropriate. Among the 1,827 patients with SLE, 88.9% were female, and 48.9% were Caucasian. The mean ± SD age of the patients was 35.1 ± 13.3 years, disease duration was 5.6 ± 4.8 months, and the length of followup was 4.7 ± 3.5 years. During the course of the study, 863 (47.2%) of the 1,827 patients had 1,627 neuropsychiatric events. Mood disorders occurred in 232 (12.7%) of 1,827 patients, and 98 (38.3%) of 256 mood disorder events were attributed to SLE. The estimated cumulative incidence of any mood disorder after 10 years was 17.7% (95% confidence interval 15.1, 20.2%). A greater risk of mood disorder was associated with concurrent neuropsychiatric events (P ≤ 0.01), and a lower risk 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 (72.4%) were treated with antidepressants. One hundred twenty-six (49.2%) of 256 mood disorders resolved in 117 (50.4%) of 232 patients. Mood disorders, the second most

  13. Aberrant B cell selection and activation in systemic lupus erythematosus.

    PubMed

    Kil, Laurens P; Hendriks, Rudi W

    2013-08-01

    The detrimental role of B lymphocytes in systemic lupus erythematosus (SLE) is evident from the high levels of pathogenic antinuclear autoantibodies (ANAs) found in SLE patients. Affirming this causative role, additional antibody-independent roles of B cells in SLE were appreciated. In recent years, many defects in B cell selection and activation have been identified in murine lupus models and SLE patients that explain the increased emergence and persistence of autoreactive B cells and their lowered activation threshold. Therefore, clinical trials with B cell depletion regimens in SLE patients were initiated but disappointingly the efficacy of B cell depleting agents proved to be limited. Remarkably however, a major breakthrough in SLE therapy was accomplished by blocking B cell survival factors rather then eliminating B cells. This surprising finding indicates that although SLE is a B cell-driven disease, the amplifying crosstalk between B cells and other cells of the immune system likely evokes the observed tolerance breakdown in B cells. Moreover, this implies that intelligent interception of pro-inflammatory loops rather then selectively silencing B cells will be key to the development of new SLE therapies. In this review, we will not only highlight the intrinsic B cell defects that facilitate the persistence of autoreactive B cells and their activation, but in addition we will focus on B cell extrinsic signals derived from T cells and innate immune cells that lower the activation threshold for B cells.

  14. Hearing loss in patients with systemic lupus erythematosus.

    PubMed

    Abbasi, Mahnaz; Yazdi, Zohreh; Kazemifar, Amir Mohammad; Bakhsh, Zahra Zarin

    2013-06-15

    Systemic lupus erythematosus has its unique complications which warrant careful examination and assessment during follow/up visits of patients. The present study was conducted to evaluate prevalence of hearing loss in patients with SLE. At present a case- control study has been performed on 45 patients with SLE in a clinic of a teaching university hospital, Qazvin city, Iran. The patients were examined and evaluated for auditory and hearing problems as well as parameters related to their disease severity and progression. The control group was selected from the same clinic. Five patients (11.1%) complained from hearing loss, 4 patients s (8.9%) complained from otorrhea, 3 patients (6.7%) had tinnitus in research group, moreover twelve patients (26.7%) in case group and 4 patients (8.9%) in control group had sensorineural hearing loss. The difference was found to be statistically significant. No statistical significant relationship was found between severity, age of onset, and duration of the disease, and the lab tests of the patients with hearing loss. The present study implies that patients with systemic lupus erythematosus may develop sensorineural hearing loss during their course of the disease. It is recommended that audiology examination and/or audiometry become a part of routine follow/up studies of the patients.

  15. Abatacept for the treatment of systemic lupus erythematosus.

    PubMed

    Pimentel-Quiroz, Victor R; Ugarte-Gil, Manuel F; Alarcón, Graciela S

    2016-01-01

    Due to improvements in our understanding of the pathogenesis of systemic lupus erythematosus (SLE), several target drugs have been and are being developed. One of the possible targets in SLE is co-stimulation between antigen-presenting cells and T cells. Abatacept is a co-stimulation moderator approved for the treatment of several autoimmune diseases. There is an unmet need for drugs with a better efficacy and safety profile when treating patients with SLE. In this review, the authors discuss the mechanism of action of abatacept including its role in the immune system and glomeruli, and relevant information about its clinical efficacy and safety. Possible explanations for the failure of previous randomized clinical trials are also discussed. Abatacept has demonstrated efficacy in other autoimmune diseases, but in SLE, randomized clinical trials have failed to achieve their primary outcome. Despite these disappointing results and based on its mechanism of action, abatacept seems to have a role in lupus nephritis and arthritis. This should be corroborated with new trials which hopefully will overcome the design pitfalls of the ones conducted to date.

  16. Entheseal involvement in systemic lupus erythematosus: are we missing something?

    PubMed

    Di Matteo, A; Satulu, I; Di Carlo, M; Lato, V; Filippucci, E; Grassi, W

    2017-03-01

    Background Musculoskeletal involvement is extremely common in patients with systemic lupus erythematosus (SLE). Continuing the research initiated in patients with inflammatory arthritis, recent studies have shown the potential role of musculoskeletal ultrasound (MSUS) in the evaluation of clinical and subclinical lupus synovitis. The inflammatory process in SLE is traditionally considered to be localized at synovial tissue areas while enthesis is not included among the possible targets of the disease. Patients and methods Entheses included in the Glasgow Ultrasound Enthesitis Scoring System were scanned in a cohort of 20 SLE patients serving as disease controls in an MSUS study aimed at assessing enthesitis in patients with psoriatic arthritis. We describe in detail four cases with unexpected and unequivocal expressions of MSUS enthesitis according to the OMERACT definition. Three out of four patients had no predisposing factors for enthesopathy. Case no. 2 was treated with a variable-dose prednisone regimen. Results In the four cases MSUS examination revealed relevant grey-scale and power Doppler abnormalities at the entheseal level, most commonly at the distal insertion of the patellar tendon. Signs of clinical enthesitis were detected in only one patient. Conclusions This case series shows for the first time the presence of clearly evident MSUS findings indicative of enthesitis in four out of 20 SLE patients (20%), raising the hypothesis that enthesis could be a missing target in the clinical evaluation of SLE patients. Our case series justifies further investigations for a better evaluation of the prevalence, characteristics and clinical relevance of entheseal involvement in SLE.

  17. Immunogenetics of Systemic Lupus Erythematosus: A Comprehensive Review

    PubMed Central

    Ghodke-Puranik, Yogita; Niewold, Timothy B.

    2015-01-01

    Summary Our understanding of the genetic basis of systemic lupus erythematosus has progressed rapidly in recent years. While many genetic polymorphisms have been associated with disease susceptibility, the next major step involves integrating these genetic polymorphisms into the molecular mechanisms and cellular immunology of the human disease. In this review, we summarize some recent work in this area, including the genetics of the type I IFN response in SLE, including polygenic and monogenic factors, as well as epigenetic influences. Contributions of both HLA and non-HLA polymorphisms to the complex genetics of SLE are reviewed. We also review recent reports of specific gene deficits leading to monogenic SLE-like syndromes. The molecular functions of common SLE-risk variants are reviewed in depth, including regulatory variations in promoter and enhancer elements and coding-change polymorphisms, and studies which are beginning to define the molecular and cellular functions of these polymorphisms in the immune system. We discuss epigenetic influences on lupus, with an emphasis on micro-RNA expression and binding, as well as epigenetic modifications that regulate the expression levels of various genes involved in SLE pathogenesis and the ways epigenetic marks modify SLE susceptibility genes. The work summarized in this review provides a fascinating window into the biology and molecular mechanisms of human SLE. Understanding the functional mechanisms of causal genetic variants underlying the human disease greatly facilitates our ability to translate genetic associations toward personalized care, and may identify new therapeutic targets relevant to human SLE disease mechanisms. PMID:26324017

  18. Pleural and pulmonary involvement in systemic lupus erythematosus.

    PubMed

    Torre, Olga; Harari, Sergio

    2011-01-01

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

  19. Progressive outer retinal necrosis syndrome in the course of systemic lupus erythematosus.

    PubMed

    Turno-Kręcicka, A; Tomczyk-Socha, M; Zimny, A

    2016-12-01

    Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). To date, only a few cases of PORN have been reported in patients, mostly among those who were immunocompromised. To our knowledge, only one case of PORN in a patient with systemic lupus erythematosus (SLE) has been described. We report the case of a 44-year old HIV-negative patient with lupus nephritis, whom was being treated by mycophenolate mophetil (MMF), arechin and prednisone. After 14 months of MMF therapy, the patient revealed PORN symptoms; and several months later, the patient developed Type B primary central nervous system lymphoma (PCNSL). PORN is usually compared to acute retinal necrosis (ARN) syndrome, because of having the same causative agent: varicella zoster virus (VZV). There are also some similarities in clinical findings. Our observation supports the hypothesis that PORN symptoms in HIV-negative patients can be an intermediate form between ARN and PORN, and can vary according to the patient's immune status.

  20. Epidemiology of systemic lupus erythematosus and cutaneous lupus erythematosus in a predominantly white population in the United States.

    PubMed

    Jarukitsopa, Sudumpai; Hoganson, Deana D; Crowson, Cynthia S; Sokumbi, Olayemi; Davis, Mark D; Michet, Clement J; Matteson, Eric L; Maradit Kremers, Hilal; Chowdhary, Vaidehi R

    2015-05-01

    Epidemiologic studies comparing the incidence and prevalence of systemic lupus erythematosus (SLE) and isolated cutaneous lupus erythematosus (CLE) are few. Olmsted County, Minnesota provides a unique setting for such a study owing to resources of the Rochester Epidemiology Project. We sought to describe and compare the incidence and prevalence of SLE and CLE from 1993-2005. SLE cases were identified from review of medical records and fulfilled the 1982 American College of Rheumatology classification criteria. CLE cases included patients with classic discoid lupus erythematosus, subacute CLE, lupus panniculitis, and bullous lupus erythematosus. Age- and sex-adjusted incidence and prevalence were standardized to the 2000 US white population. The age- and sex-adjusted incidence of SLE (2.9 per 100,000; 95% confidence interval [95% CI] 2.0-3.7) was similar to that of CLE (4.2 per 100,000; 95% CI 3.1-5.2, P = 0.10). However, the incidence of CLE was 3 times higher than SLE in men (2.4 versus 0.8 per 100,000; P = 0.009). The age- and sex-adjusted prevalence of CLE on January 1, 2006 was higher than that of SLE (70.4 versus 30.5 per 100,000; P < 0.001). The prevalences of CLE and SLE in women were similar, but the prevalence of CLE was higher in men than in women (56.9 versus 1.6 per 100,000; P < 0.001). The incidence of CLE rose steadily with age and peaked at 60-69 years. The incidences of CLE and SLE are similar, but CLE is more common than SLE in men and in older adults. These findings may reflect differences in genetic or environmental etiology of CLE. © 2015, American College of Rheumatology.

  1. Drug-induced lupus erythematosus

    MedlinePlus

    ... Causes Drug-induced lupus erythematosus is similar to systemic lupus erythematosus (SLE). It is an autoimmune disorder. This means ... 2015:chap 132. Wright B, Bharadwaj S, Abelson A. Systemic lupus erythematosus. In: Carey WD, ed. Cleveland Clinic: Current Clinical ...

  2. Chronic hydroxychloroquine improves endothelial dysfunction and protects kidney in a mouse model of systemic lupus erythematosus.

    PubMed

    Gómez-Guzmán, Manuel; Jiménez, Rosario; Romero, Miguel; Sánchez, Manuel; Zarzuelo, María José; Gómez-Morales, Mercedes; O'Valle, Francisco; López-Farré, Antonio José; Algieri, Francesca; Gálvez, Julio; Pérez-Vizcaino, Francisco; Sabio, José Mario; Duarte, Juan

    2014-08-01

    Hydroxychloroquine has been shown to be efficacious in the treatment of autoimmune diseases, including systemic lupus erythematosus. Hydroxychloroquine-treated lupus patients showed a lower incidence of thromboembolic disease. Endothelial dysfunction, the earliest indicator of the development of cardiovascular disease, is present in lupus. Whether hydroxychloroquine improves endothelial function in lupus is not clear. The aim of this study was to analyze the effects of hydroxychloroquine on hypertension, endothelial dysfunction, and renal injury in a female mouse model of lupus. NZBWF1 (lupus) and NZW/LacJ (control) mice were treated with hydroxychloroquine 10 mg/kg per day by oral gavage, or with tempol and apocynin in the drinking water, for 5 weeks. Hydroxychloroquine treatment did not alter lupus disease activity (assessed by plasma double-stranded DNA autoantibodies) but prevented hypertension, cardiac and renal hypertrophy, proteinuria, and renal injury in lupus mice. Aortae from lupus mice showed reduced endothelium-dependent vasodilator responses to acetylcholine and enhanced contraction to phenylephrine, which were normalized by hydroxychloroquine or antioxidant treatments. No differences among all experimental groups were found in both the relaxant responses to acetylcholine and the contractile responses to phenylephrine in rings incubated with the nitric oxide synthase inhibitor N(G)-nitro-l-arginine methyl ester. Vascular reactive oxygen species content and mRNA levels of nicotinamide adenine dinucleotide phosphate oxidase subunits NOX-1 and p47(phox) were increased in lupus mice and reduced by hydroxychloroquine or antioxidants. Chronic hydroxychloroquine treatment reduced hypertension, endothelial dysfunction, and organ damage in severe lupus mice, despite the persistent elevation of anti-double-stranded DNA, suggesting the involvement of new additional mechanisms to improve cardiovascular complications.

  3. [Retinal vasculopathy in systemic lupus erythematosus: a case of lupus vasculitis and a case of non-vasculitis venous occlusion].

    PubMed

    Figueras-Roca, M; Rey, A; Mesquida, M; Pelegrín, L; Llorens, V; Fontenla, J R; Adán, A

    2014-02-01

    Two patients with systemic lupus erythematosus presented with vision loss, and were diagnosed with retinal vasculopathy. Patient 1 had occlusive vasculitis with macular oedema and retinal ischaemia in the right eye. Corticosteroid therapy was increased and intravenous rituximab added. Intravitreal therapy and panretinal photocoagulation were performed. Patient 2 presented with a left central retinal vein occlusion without vasculitis but was on anticoagulation therapy due to having an antiphospholipid syndrome. Both patients maintained a stable visual acuity. Occlusive lupus retinal vasculitis has severe visual and systemic consequences (central nervous system vasculitis). It is crucial to differentiate it from standard vascular occlusion syndromes. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

  4. An update on childhood-onset systemic lupus erythematosus.

    PubMed

    Barsalou, Julie; Levy, Deborah M; Silverman, Earl D

    2013-09-01

    This manuscript will provide a review of studies published in the last year examining the major disease manifestations, comorbidities, biomarkers and therapeutic trials involving childhood-onset systemic lupus erythematosus (cSLE) patients. Recent multicenter prospective cohort studies supported previous findings that cSLE patients accrue damage early on their disease. Four studies showed that ethnicity altered disease severity and incidence of both cSLE and lupus nephritis. Description of clinical features and response to therapy in a large group of cSLE patients with neuropsychiatric involvement provided useful information on this feature. Advancement in the field of biomarkers was seen but the new biomarkers are not yet ready for clinical use. A randomized placebo-controlled trial of statins to prevent atherosclerosis progression did not meet its primary endpoint but did show a trend in improvement of carotid intima-media thickness, a surrogate marker of atherosclerosis. No other prospective interventional treatment trials designed specifically for cSLE patients were reported in the past year. There is an urgent need to better characterize the long-term outcome of cSLE patients and identify early on those at risk of a worse outcome. Advances in the field of biologics and small molecules will hopefully allow better targeted therapies of the cSLE population.

  5. Pediatric systemic lupus erythematosus in a single nephrology unit.

    PubMed

    Youssef, Doaa Mohammed; Tawfek, Doaa Mostafa; Mohammed, Abdelsalam Mohammed; Mohammed, Rania; Khalifa, Naglaa Ahmed

    2015-03-01

    Clinical manifestations of systemic lupus erythematosus (SLE) are widely variable, and its course is unpredictable. SLE that begins in childhood has been considered more severe than SLE with onset during adulthood. Our aim was to determine the presentation and the outcome of SLE of 26 children (20 females and 6 males, with a female to male ratio of 3.8:1) with SLE in our center, their ages ranging from 5 - 18 years and followed from 2005 till October 2011. They were diagnosed according to the American Rheumatism Association's revised criteria. Complete blood count, erythrocyte sedimentation rate, C3, urine analysis, 24-h urinary protein, antinuclear antibodies, anti-ds DNA and renal biopsy were obtained for the patients. We found that the most extra-renal manifestation of SLE was fever (57.7%), while lupus nephritis (LN) was the most commonly affected organ (50%). Hemolytic anemia was the most common hematological abnormality (80.8%), while immunological characteristics were positive in all the patients. Remission in patients without LN was more than 5.3-times the remission in LN patients. The outcome of the patients without LN was better than the patients with LN.

  6. Immunodeficiency and autoimmunity: lessons from systemic lupus erythematosus

    PubMed Central

    Grammatikos, Alexandros P.; Tsokos, George C.

    2011-01-01

    Recent evidence suggests that systemic autoimmunity and immunodeficiency are not separate entities, but rather interconnected processes. Immunodeficiency results from distinct defects of the immune response and primarily presents as infections, but also frequently with autoimmune features. Systemic autoimmunity is the combined effect of multiple genetic variations, infectious and immunoregulatory factors that result in dominant autoimmune manifestations in addition to frequent and opportunistic infections. The overlap in disease manifestations and symptoms suggests that immunodeficiency should be considered in the presence of autoimmunity, and vice versa. In this review, we present the shared or similar aspects of immunodeficiency and autoimmunity using systemic lupus erythematosus as a paradigm and discuss the implications for clinical care. PMID:22177735

  7. Pathogenesis of human systemic lupus erythematosus: recent advances

    PubMed Central

    Crispín, José C.; Liossis, Stamatis-Nick C.; Kis-Toth, Katalin; Lieberman, Linda A.; Kyttaris, Vasileios C.; Juang, Yuang-Taung; Tsokos, George C.

    2010-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that predominantly affects women and presents with manifestations derived from the involvement of multiple organs including the kidneys, joints, nervous system, and hematopoietic organs. Immune system aberrations, as well as heritable, hormonal, and environmental factors interplay in the expression of organ damage. Recent contributions from different fields have developed our understanding of SLE and reshaped current pathogenic models. Here, we review novel information that deals with 1) genes associated with disease expression, 2) immune cell molecular abnormalities that lead to autoimmune pathology, 3) the role of hormones and sex chromosomes in the development of disease, 4) environmental and epigenetic factors thought to contribute to the expression of SLE. Finally, we emphasize molecular defects intimately associated with the disease process of SLE that represent ideal therapeutic targets and disease biomarkers. PMID:20138006

  8. Systemic lupus erythematosus with intestinal perforation: A case report

    PubMed Central

    GU, YUQING; ZHU, TAO; WANG, YIQING; XU, HONGXING

    2015-01-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune inflammatory disease, which can affect almost all systems and organs. Gastrointestinal disorder is one of the most noteworthy complications of patients with SLE. However, gastrointestinal disorder with intestinal perforation is rare, but potentially life-threatening if not treated promptly. The present study reported a case of SLE with intestinal perforation, where surgical intervention was performed and a crevasse (~3 cm in diameter) was detected in the ileum, ~60 cm from the ileocecal valve. Following surgery, the patient suffered from difficult ventilator weaning, septic shock and intestinal obstruction. The patient was successfully treated and discharged from the hospital after ~4 months of treatment. Intestinal perforation in SLE patients is potentially life-threatening; early diagnosis and prompt treatment are crucial to the management of this rare complication of SLE. PMID:26622471

  9. Central serous retinopathy complicating systemic lupus erythematosus: a case series.

    PubMed

    Khng, C G; Yap, E Y; Au-Eong, K G; Lim, T H; Leong, K H

    2000-08-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with widespread manifestations including the eye. Central serous retinopathy (CSR) has been associated as a complicating event in SLE, although it is uncommon. We present a case series of four female Chinese SLE patients who developed CSR during the course of their systemic disease. All four presented clinically with typical CSR. Angiographic findings did not show evidence of choroidal ischaemia or delayed choroidal filling. Resolution of the serous retinal detachment occurred in all four patients. Recovery of vision was seen in three patients. The clinical outcome was similar to that occurring in the usual male population. Central serous retinopathy as a manifestation of SLE may be caused by various factors. These include SLE-associated choroidopathy, systemic hypertension, renal disease, retinal pigment epithelial dysfunction and glucocorticoid therapy.

  10. Aplastic anemia as a feature of systemic lupus erythematosus: a case report and literature review.

    PubMed

    Chalayer, Émilie; Ffrench, Martine; Cathébras, Pascal

    2015-06-01

    Peripheral cytopenias are common in systemic lupus erythematosus, but bone marrow involvement is rarely reported. Aplastic anemia is the result of immune-mediated destruction of hematopoietic stem cells causing pancytopenia and characterized by an empty bone marrow. This rare but serious disease has been described as an unusual manifestation of systemic lupus erythematosus. We reviewed the 25 cases published in the English language literature and discuss the clinical presentation, outcome, treatment, and pathophysiology of aplastic anemia as a complication of systemic lupus erythematosus. We report here the first case of aplastic anemia associated with systemic lupus erythematosus treated with an allogeneic hematopoietic stem cell transplant. Over one half of patients received concomitantly the diagnoses of systemic lupus erythematosus and aplastic anemia. No clinical or histological features can distinguish primary aplastic anemia from aplastic anemia occurring in systemic lupus erythematosus patients. The overall mortality is about 15% and corticosteroid-based therapy alone or in combination with other immunomodulatory drugs can restore bone marrow function. Systemic lupus erythematosus may be complicated by bone marrow involvement. The diagnosis of peripheral cytopenias should be confirmed by bone marrow aspiration. All these patients should receive cortisone as a first treatment. Plasma exchanges seem to have some efficacy. Other different immunomodulatory therapies were used with variable results.

  11. A polymorphism within interleukin-21 receptor (IL21R) confers risk for systemic lupus erythematosus

    PubMed Central

    Webb, Ryan; Merrill, Joan T.; Kelly, Jennifer A.; Sestak, Andrea; Kaufman, Kenneth M.; Langefeld, Carl D.; Ziegler, Julie; Kimberly, Robert P.; Edberg, Jeffrey C.; Ramsey-Goldman, Rosalind; Petri, Michelle; Reveille, John D.; Alarcón, Graciela S.; Vilá, Luis M.; Alarcón-Riquelme, Marta E.; James, Judith A.; Gilkeson, Gary S.; Jacob, Chaim O.; Moser, Kathy L.; Gaffney, Patrick M.; Vyse, Timothy J.; Nath, Swapan K.; Lipsky, Peter; Harley, John B.; Sawalha, Amr H.

    2009-01-01

    Objective Interleukin (IL) 21 is a member of the type I cytokine superfamily that exerts a variety of effects on the immune system including B cell activation, plasma cell differentiation, and immunoglobulin production. The expression of IL21R is reduced in B cells from lupus patients, while IL21 serum levels are increased in both lupus patients and some lupus-murine models. We recently reported that polymorphisms within the IL21 gene are associated with increased susceptibility to lupus. Herein, we examined the genetic association between SNPs within IL21R and lupus. Methods We genotyped 17 SNPs in the IL21R gene in two large cohorts of lupus patients and ethnically-matched healthy controls. Genotyping was performed with the Illumina BeadStation 500GX instrument using Illumina Infinum II genotyping assays. Results We identified and confirmed the association between rs3093301 within the IL21R gene and lupus in two independent European-derived and Hispanic cohorts (meta analysis odds ratio= 1.16, 95% CI= 1.08-1.25, meta analysis p=1.0×10-4). Conclusion We identified IL21R as a novel susceptibility gene for lupus. PMID:19644854

  12. Anti-apolipoprotein A-I antibodies and paraoxonase 1 activity in Systemic Lupus Erythematosus

    PubMed Central

    Ahmed, Mohammed Mahmoud; Elserougy, Eman Mahmoud; Al-Gazzar, Iman Ibrahim; Fikry, Iman Mohamed; Habib, Dawoud Fakhry; Younes, Khaled Mohamed; Salem, Neveen Abd El-hameed

    2013-01-01

    Systemic lupus erythematosus (SLE) patients have an increased risk of atherosclerosis. Identification of at-risk patients and the pathogenesis of atherosclerosis in SLE remain elusive. Paraoxonase 1 (PON1) and anti-apolipoprotein A-I antibody (anti-Apo A-I) appear to have a potential role in premature atherosclerosis in SLE. The aim of this work was to study PON1 activity and anti-Apo A-I antibody in SLE female patients and to demonstrate their relations to disease activity as well as disease related damage. Forty SLE female patients and 40 apparently healthy volunteers were included. Anti-Apo A-I antibodies levels and PON1 activity levels were assessed. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and systemic Lupus International Collaboration Clinics (SLICC)/American College of Rheumatology (ACR) damage index were preformed in all patients. Compared with controls, SLE patients showed significantly lower PON1 activity and significantly higher titers of anti-Apo A-I. Anti-Apo A-I antibody titers correlated inversely with PON1 activity. Elevated titers of anti-Apo A-I antibody and reduced PON activity were related to increased SLEDAI and (SLICC/ACR) damage index scores. We concluded that there is decreased PON1 activity and formation of anti-Apo A-I antibodies in female patients with SLE. SLE-disease activity assessed by SLEDAI and SLE disease related organ damage assessed by SLICC/ACR damage index are negatively correlated with PON1 activity and positively correlated with anti-Apo A-I antibodies. PON1 activity and anti-Apo A-I antibodies might be involved in the pathogenesis of atherosclerosis in SLE patients. PMID:26622215

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

    PubMed Central

    Reihl, Alec M.

    2016-01-01

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

  14. Systemic Lupus Erythematosus: From Genes to Organ Damage

    PubMed Central

    Kyttaris, Vasileios C.

    2011-01-01

    Systemic lupus erythematosus (SLE) is a disease characterized by inappropriate response to self-antigens. Genetic, environmental and hormonal factors are believed to contribute to the development of the disease. We think of SLE pathogenesis as occurring in three phases of variable duration. A series of regulatory failures during the ontogeny of the immune system lead to the emergence of auto-reactive clones and the production of auto-antibodies (phase I). As the immune response to self-antigens broadens, the auto-antibody repertoire is enriched (phase II) and clinical manifestations eventually ensue (phase III). The final result is tissue damage that if not treated will lead to the functional failure of such important organs as the kidney and brain. PMID:20824476

  15. Interleukin-17 and systemic lupus erythematosus: current concepts.

    PubMed

    Nalbandian, A; Crispín, J C; Tsokos, G C

    2009-08-01

    The emerging role of interleukin (IL)-17 as a hallmark proinflammatory cytokine of the adaptive immune system, produced primarily by a new T helper cell subset termed 'Th17', has received considerable attention. Differentiation of Th17 cells is driven by the simultaneous presence of transforming growth factor-beta and certain inflammatory cytokines (e.g. IL-6, IL-21), and recent studies have shown that inflammation instigated by IL-17-producing cells is central to the development and pathogenesis of several human autoimmune diseases and animal models of autoimmunity. In this review, we focus on the information regarding IL-17 and systemic lupus erythematosus (SLE), a chronic autoimmune disease. The work that has explored the development and behaviour of IL-17-producing cells in SLE is discussed, and different mechanisms by which IL-17 could potentially augment inflammation and autoantibody production in the context of SLE are proposed.

  16. Eosinophilic enteritis in association with systemic lupus erythematosus.

    PubMed

    Jaimes-Hernandez, J; Aranda-Peirera, P; Melendez-Mercado, C I

    2009-04-01

    Eosinophilic gastroenteritis (EGE) is an uncommon disease and has rarely been reported in association with connective tissue diseases as systemic lupus erythematosus. We report a 36-year-old woman who developed recurrent episodes of abdominal pain, nausea, vomiting and melena. Complete blood counts showed elevated eosinophil counts. Ultrasound and CT-scan images studies were significant for bowel wall thickening and ascites. The patient underwent an exploratory laparotomy with a mesenteric biopsy and appendectomy that showed eosinophil infiltration in the muscularis propria, establishing the diagnosis of EGE. The patient developed pleural effusions, with laboratory studies showing haemolytic anaemia, thrombocytopenia, positive antinuclear antibody and anticardiolipin antibodies. The patient was treated with high-dose systemic corticosteroid therapy, with successful resolution of symptoms. Three months later, she developed a new episode of abdominal pain defined as intestinal pseudo-obstruction that was resolved without complications.

  17. Systemic lupus erythaematosus presenting as spontaneous splenic rupture.

    PubMed

    Cruz, António José; Castro, Alexandra

    2015-11-27

    Systemic lupus erythaematosus (SLE) is known to involve the reticuloendothelial system, but spontaneous splenic rupture (SSR) in the context of the disease is a very rare complication. We observed a 61-year-old woman with an unremarkable previous medical history who presented with SSR and underwent an emergency splenectomy. The histopathological analysis of the specimen revealed signs of vasculitis. On review of symptoms with the patient, a history of oligoarthralgia, photosensitivity, xerostomia and Raynaud phenomenon was elicited. Laboratory investigations revealed lymphopaenia, mild proteinuria and positive antinuclear and anti-dsDNA antibodies. The patient was started on hydroxychloroquine and the disease has since remained silent. This article addresses the rare association between SLE and SSR. 2015 BMJ Publishing Group Ltd.

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

    PubMed

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

    2013-06-01

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

  19. A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis

    PubMed Central

    Basnyat, Shristi; Eid, Hala; Kuzyshyn, Halyna; Feinstein, David

    2016-01-01

    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. PMID:28127489

  20. [Chronic intestinal pseudo-obstruction in systemic lupus erythematosus].

    PubMed

    Ghannouchi Jaafoura, N; Khalifa, M; Atig, A; Ben Jazia, E; Alaoua, A; Braham Krifa, A; Letaief, A; Bahri, F

    2011-01-01

    Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.

  1. Prevalence and expression of photosensitivity in systemic lupus erythematosus.

    PubMed Central

    Wysenbeek, A J; Block, D A; Fries, J F

    1989-01-01

    Photosensitivity was assessed in 125 patients with systemic lupus erythematosus (SLE) and in 281 patients with rheumatoid arthritis (RA) as controls. Photosensitivity was reported by 87/119 (73%) patients with SLE and in 62/269 (23%) patients with RA; involving the face in 72/122 (59%) patients with SLE, then arms, chest, and neck. Patients with SLE reported that sun exposure could exacerbate various systemic symptoms, 51/121 (42%) reported medical treatment for photosensitivity and 41/118 (35%) reported that photosensitivity had a significant impact on their lifestyle. There was no significant difference in disease severity, as judged by physician or laboratory results, between patients scoring high or low on the photosensitivity scale. PMID:2742401

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

    PubMed

    Vijay, S; Imthiaz, V K; Hitesh, S

    2017-03-01

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

  3. Sharing experiences and social support requests in an Internet forum for patients with systemic lupus erythematosus.

    PubMed

    Mazzoni, Davide; Cicognani, Elvira

    2014-05-01

    Internet forums represent a useful but understudied resource to understand psychosocial aspects of living with systemic lupus erythematosus. This study was aimed to describe the demand/supply of social support through the Internet in relation with the description of personal illness experiences. All the posts (118) from an Italian forum for systemic lupus erythematosus patients were collected and analyzed combining qualitative content analysis with statistical textual analysis. The results showed different purposes for posts: starting new relationships, seeking information, receiving emotional support, and giving a contribution. Lexical analysis identified three ways of describing patients' experiences. Discussion focuses on the relationship between the requested/offered support and systemic lupus erythematosus experiences.

  4. Systemic lupus erythematosus in three ethnic groups. XII. Risk factors for lupus nephritis after diagnosis.

    PubMed

    Bastian, H M; Roseman, J M; McGwin, G; Alarcón, G S; Friedman, A W; Fessler, B J; Baethge, B A; Reveille, J D

    2002-01-01

    The purpose of this study was to determine the cumulative incidence of lupus nephritis (LN) and the factors predictive of its occurrence in a multiethnic systemic lupus erythematosus (SLE) cohort. We studied 353 SLE patients as defined by the American College of Rheumatology (ACR) criteria (65 Hispanics, 93 African-Americans and 91 Caucasians). First, we determined the cumulative incidence of LN in all patients. Next, we determined the predictors for LN in those with nephritis occurring after diagnosis. The dependent variable, LN, was defined by: (1) A renal biopsy demonstrating World Health Organization (WHO), class II-V histopathology; and/or (2) proteinuria > or = 0.5 g/24 h or 3+ proteinuria attributable to SLE; and/or (3) one of the following features also attributable to SLE and present on two or more visits, which were performed at least 6 months apart--proteinuria > or = 2+, serum creatinine > or = 1.4 mg/dl, creatinine clearance < or = 79 ml/min, > or = 10 RBCs or WBCs per high power field (hpf), or > or = 3 granular or cellular casts per hpf. Independent variables assessed at diagnosis, and if absent, at baseline, were from four domains: sociodemographic, clinical, immunologic and immunogenetic (including the complete antibody profile and MHC class II alleles), and health habits. Variables with P < 0.05 by chi square analyses were entered into domain-specific stepwise logistic regression analyses controlling for disease duration, with LN as the dependent variable. Significant domain-specific regression variables (P < or = 0.1) were then entered into an overall model. The cumulative incidence of LN was 54.3% in all patients, and 35.3% for those developing LN after diagnosis. LN after diagnosis occurred in 43.1% of 65 Hispanics, 50.5% of 93 African-Americans, and 14.3% of 91 Caucasians, P < 0.0001. The duration of follow-up for those with LN after diagnosis was 5.5+/-2.4 vs 4.0+/-2.9 years for those without LN. Hispanic (odds ratio (OR) = 2.71, 95

  5. The progressive systemic sclerosis/systemic lupus overlap: an unusual clinical progression.

    PubMed Central

    Asherson, R A; Angus, H; Mathews, J A; Meyers, O; Hughes, G R

    1991-01-01

    Three patients with the unusual combinations of discoid lupus, systemic lupus erythematosus (SLE), and progressive systemic sclerosis (PSS) are reported. The first patient developed PSS eight years after a diagnosis of discoid lupus had been made and this was complicated by myositis six years later. The second patient developed PSS more than 20 years after being diagnosed as having SLE. The third patient developed SLE with predominant features of urticarial vasculitis six years after PSS. Mild myositis also ensued. There were no antibodies to U1RNP demonstrable in any of these patients. The clinical progression of SLE to PSS or vice versa in the absence of features of mixed connective tissue disease is distinctly uncommon. Images PMID:2042989

  6. Early discoid lupus erythematosus protects against renal disease in patients with systemic lupus erythematosus: longitudinal data from a large Latin American cohort.

    PubMed

    Pons-Estel, G J; Aspey, L D; Bao, G; Pons-Estel, B A; Wojdyla, D; Saurit, V; Alvarellos, A; Caeiro, F; Haye Salinas, M J; Sato, E I; Soriano, E R; Costallat, L T L; Neira, O; Iglesias-Gamarra, A; Reyes-Llerena, G; Cardiel, M H; Acevedo-Vásquez, E M; Chacón-Díaz, R; Drenkard, C

    2017-01-01

    The objective of this study was to examine whether early discoid lupus erythematosus (DLE) would be a protective factor for further lupus nephritis in patients with systemic lupus erythematosus (SLE). We studied SLE patients from GLADEL, an inception longitudinal cohort from nine Latin American countries. The main predictor was DLE onset, which was defined as physician-documented DLE at SLE diagnosis. The outcome was time from the diagnosis of SLE to new lupus nephritis. Univariate and multivariate survival analyses were conducted to examine the association of DLE onset with time to lupus nephritis. Among 845 GLADEL patients, 204 (24.1%) developed lupus nephritis after SLE diagnosis. Of them, 10 (4.9%) had DLE onset, compared to 83 (12.9%) in the group of 641 patients that remained free of lupus nephritis (hazard ratio 0.39; P = 0.0033). The cumulative proportion of lupus nephritis at 1 and 5 years since SLE diagnosis was 6% and 14%, respectively, in the DLE onset group, compared to 14% and 29% in those without DLE (P = 0.0023). DLE onset was independently associated with a lower risk of lupus nephritis, after controlling for sociodemographic factors and disease severity at diagnosis (hazard ratio 0.38; 95% confidence interval 0.20-0.71). Our data indicate that DLE onset reduces the risk of further lupus nephritis in patients with SLE, independently of other factors such as age, ethnicity, disease activity, and organ damage. These findings have relevant prognosis implications for SLE patients and their clinicians. Further studies are warranted to unravel the biological and environmental pathways associated with the protective role of DLE against renal disease in patients with SLE. © The Author(s) 2016.

  7. The need to define treatment goals for systemic lupus erythematosus.

    PubMed

    Franklyn, Kate; Hoi, Alberta; Nikpour, Mandana; Morand, Eric F

    2014-09-01

    In the current therapeutic climate, mortality rates from systemic lupus erythematosus (SLE) remain unacceptably high. Although new therapies are on the horizon, pending their emergence and availability, optimization of the currently available therapies is potentially achievable. A 'treat-to-target' approach is now considered routine for many diseases, including rheumatoid arthritis, for which it has substantially improved patient outcomes. The heterogeneity of SLE, as well as lack of universal agreement over methods to measure disease activity and treatment responses, has impeded the development of such an approach for this disease. In this article, the potential benefits of a treatment-target definition are explored, obstacles to the development of a treatment target in SLE are identified, and possible strategies to achieve this goal are discussed.

  8. Understanding mechanisms of hypertension in systemic lupus erythematosus

    PubMed Central

    Taylor, Erin B.; Ryan, Michael J.

    2016-01-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that predominately affects women of reproductive age. Hypertension is an important cardiovascular risk factor that is prevalent in this patient population. Despite the high incidence of hypertension in women with SLE, the pathophysiological mechanisms underlying the development of hypertension remain poorly understood. This review will focus on disease-related factors, including inflammation, autoantibodies, and sex hormones that may contribute to hypertension in patients with SLE. In addition, we will highlight studies performed by our laboratory using the female NZBWF1 (F1 hybrid of New Zealand Black and New Zealand White strains) mouse model, a spontaneous model of SLE that mimics human disease and develops hypertension and renal injury. Specifically, using female NZBWF1 mice, we have demonstrated that multiple factors contribute to the pathogenesis of hypertension, including the inflammatory cytokine, tumor necrosis factor (TNF)-α, oxidative stress, as well as B-cell hyperactivity and autoantibody production. PMID:26985016

  9. Novel therapeutic agents in clinical development for systemic lupus erythematosus

    PubMed Central

    2013-01-01

    Conventional immunosuppressive therapies have radically transformed patient survival in systemic lupus erythematosus (SLE), but their use is associated with considerable toxicity and a substantial proportion of patients remain refractory to treatment. A more comprehensive understanding of the complexity of SLE immunopathogenesis has evolved over the past decade and has led to the testing of several biologic agents in clinical trials. There is a clear need for new therapeutic agents that overcome these issues, and biologic agents offer exciting prospects as future SLE therapies. An array of promising new therapies are currently emerging or are under development including B-cell depletion therapies, agents targeting B-cell survival factors, blockade of T-cell co-stimulation and anti-cytokine therapies, such as monoclonal antibodies against interleukin-6 and interferon-α. PMID:23642011

  10. [Salmonella infections in patients with systemic lupus erythematosus].

    PubMed

    Priemé, H B; Kriegbaum, J N; Lester, A; Halberg, P

    1991-09-23

    A retrospective review was undertaken of zoonotic Salmonella infections among 173 patients with systemic lupus erythematosus (SLE) who were followed by two departments of rheumatology in Copenhagen during an average period of 16 years. A total of six Salmonella infections were registered in five patients as one patient had two episodes of infection with Salmonella typhimurium with an interval of three years. All six infections were diagnosed during the years 1986-1990. During the period 1984 to 1988, the number of registered Salmonella infections increased from 900 to 3,500 in the Danish background population. All six infections were accompanied by Salmonella bacteraemia. the present investigation and studies of the literature demonstrate a considerably increased risk of Salmonella bacteraemia in SLE patients as compared with the population as a whole. This should be borne in mind when febrile SLE patients are investigated.

  11. Therapy of systemic lupus erythematosus: a look into the future.

    PubMed

    Smolen, Josef S

    2002-01-01

    The prognosis for patients with systemic lupus erythematosus has greatly improved over the past two decades. However, therapies that are more effective and that have fewer sequelae are needed to rescue patients from organ failure and further reduce mortality. Research under way, including that into induction of tolerance to self-antigens, prevention of the consequences of pathogenic autoantibody production, interference with the cytokine network and signal transduction, the identification and treatment of any infectious triggers, and stem cell therapy, offers hope of improved remedies or even of cure. Given the fact that a number of biological therapies for rheumatologic disease are already in use or are in the development stage, such progress may come soon.

  12. Why Targeted Therapies are Necessary for Systemic Lupus Erythematosus

    PubMed Central

    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

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

    PubMed Central

    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

  14. Systemic lupus erythematosus following total body irradiation for malignant lymphoma.

    PubMed

    Spinozzi, F; Capodicasa, E; Gerli, R; Bertotto, A; Rambotti, P; Grignani, F

    1986-01-01

    A case of a 63-year old man, who developed systemic lupus erythematosus three years after an initial diagnosis of small-cleaved centrofollicular lymphoma is described. The diagnosis of SLE was made on the basis of the accepted "1982 revised criteria for the classification of SLE". The autoimmune disease arose after a cycle of total body irradiation, despite the treatment with combination chemotherapeutic doses such a CVP or COAP or Cyclophosphamide, Vincristine, VM-26 and Prednisone. Genetic, immunological and exogenous environmental factors may co-exist and might equally be implicated in the pathogenesis of SLE and malignant lymphoma. However, the onset of SLE after total body irradiation could have been caused by the inactivation of suppressor T lymphocytes, which are known to be sensitive to radiations in vitro.

  15. Venous syndromes and pulmonary embolism in systemic lupus erythematosus.

    PubMed

    Gladman, D D; Urowitz, M B

    1980-08-01

    Deep vein thrombophlebitis (DVT) and pulmonary emboli (PE) have been uncommonly reported manifestations of systemic lupus erythematosus (SLE). This may be partly due to their being masked by other more familiar lesions of the lungs and extremities in SLE. We have identified 17 patients with SLE from a population of 180 being followed up prospectively who had 21 attacks of DVT and/or PE. Of the total of 21 episodes the SLE was considered to be active in 14, inactive in 6, and variable in a patient with recurring phlebitis. The incidence of hyperlipidaemia, smoking history, and use of birth control medication or corticosteroids was not higher in these patients. These clinical findings thus constitute additional features of SLE occurring in about 9% of patients and may be significance for morbidity and mortality.

  16. Systemic lupus erythematosus in children with sickle cell disease.

    PubMed

    Saxena, Vinay Raj; Mina, Rina; Moallem, Hamid J; Rao, Sreedhar P; Miller, Scott T

    2003-08-01

    Coexistence of sickle cell disease (SCD) and systemic lupus erythematosus (SLE) has been reported in 11 patients. The authors describe five additional patients with SCD and symptoms initially attributable to SCD who were later found to have SLE. Patients were identified over a 10-year period (1991-2001) in a pediatric sickle cell population numbering approximately 350. All patients are African-American. Age at diagnosis of SLE was 9 to 17 years (median 11 years), and follow-up after diagnosis ranged from 6 months to 10 years (median 3 years). SLE cerebritis (n = 3), serositis (n = 4), and nephritis (n = 2) were common findings. Physicians should be alerted to the possible development of SLE in patients with SCD.

  17. Second-to-fourth Digit Ratio in Systemic Lupus Erythematosus.

    PubMed

    Doe, Kentaro; Nozawa, Kazuhisa; Hirai, Takuya; Tsushima, Hiroshi; Hayashi, Eri; Hiruma, Kaori; Ando, Seiichiro; Nakano, Soichiro; Kon, Takayuki; Amano, Hirofumi; Yamaji, Ken; Tamura, Naoto; Takasaki, Yoshinari

    2015-05-01

    Systemic lupus erythematosus (SLE) occurs predominantly in women, and sex hormones play an important role in SLE. Variation in the second-to-fourth digit ratio (2D4D ratio) is attributed to sex hormone exposure. Therefore, we evaluated the relationship between sex hormones and SLE by measuring 2D4D ratios. We measured 2D4D ratios in 100 patients with SLE and 200 normal healthy controls (NHC). Patients with SLE had a lower 2D4D ratio than NHC. Our study suggests that patients with SLE have experienced high prenatal testosterone and low prenatal estrogen. To our knowledge, this is the first study evaluating the association between 2D4D ratio and SLE.

  18. The incidence of systemic lupus erythematosus in North American Indians.

    PubMed

    Morton, R O; Gershwin, M E; Brady, C; Steinberg, A D

    1976-06-01

    The annual incidence (AI) of systemic lupus erythematosus (SLE) was determined in 75 highly inbred North American Indian tribes, a total of approximately 800,000 people, during the fiscal years 1971-1975. Seventy-two of the Indian tribes had an AI of SLE which was of similar magnitude to previously published studies from Sweden, Rochester (Minn.), Alabama, New York City, and San Francisco. However, Three tribes, the Crow, Arapahoe, and Sioux Indians, had a markedly elevated AI of SLE. These three tribes share common historical, geographic, and cultural characteristics. Further, they all reside in the northern half of the United States, in states that do not receive intense sun exposure, thereby eliminating photosensitivity as a major determinant of this increased prevalence. Finally, the AI of SLE in the Sioux Indians was highest for "full-blooded" members and lowest for genetic admixtures.

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

    PubMed

    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.

  20. Systemic lupus erythematosus: strategies to improve pregnancy outcomes

    PubMed Central

    Yamamoto, Yuriko; Aoki, Shigeru

    2016-01-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with a high prevalence in females of childbearing age. Thus, reproduction in SLE patients is a major concern for clinicians. In the past, SLE patients were advised to defer pregnancy because of poor pregnancy outcomes and fear of SLE flares during pregnancy. Investigations to date show that maternal and fetal risks are higher in females with SLE than in the general population. However, with appropriate management of the disease, sufferers may have a relatively uncomplicated pregnancy course. Factors such as appropriate preconception counseling and medication adjustment, strict disease control prior to pregnancy, intensive surveillance during and after pregnancy by both the obstetrician and rheumatologist, and appropriate interventions when necessary play a key role. This review describes the strategies to improve pregnancy outcomes in SLE patients at different time points in the reproduction cycle (preconception, during pregnancy, and postpartum period) and also details the neonatal concerns. PMID:27468250

  1. Contraception in patients with systemic lupus erythematosus and antiphospholipid syndrome.

    PubMed

    Sammaritano, L R

    2014-10-01

    Contraceptive choice in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) is challenging but important. Long-acting forms of contraception such as the progesterone intrauterine device (IUD) or subdermal implant are preferable for most patients. Estrogen-containing hormonal contraceptives may be used in stable, inactive SLE patients but are contraindicated in patients with positive antiphospholipid antibodies (aPL). The levonorgestrel IUD is a good alternative for many APS patients and often decreases menstrual blood loss. It is prudent to avoid depot medroxyprogesterone acetate (DMPA) in corticosteroid-treated or other patients at risk for osteoporosis because of the inhibition of ovulation. Effective and safe contraception in patients with SLE and APS permits planning for pregnancy during inactive disease and while on pregnancy-compatible medications, preventing a poorly timed pregnancy that may jeopardize maternal and/or fetal health.

  2. Systemic lupus erythematosus, pregnancy and carcinoma of the tongue.

    PubMed

    Unsworth, Jeffrey David; Baldwin, Andrew; Byrd, Louise

    2013-05-31

    We present a case which describes a 29-year-old woman with systemic lupus erythematosus who was treated aggressively with cytotoxic immunosuppression. Five years later and approximately 12 weeks pregnant, she is confirmed as having carcinoma of the tongue. Not wishing to consider termination of her pregnancy, she underwent surgical resection, which included partial glossectomy with microvascular reconstruction. Good oral function (speech and swallowing) was restored within 2 weeks. The pregnancy proceeded relatively uneventfully to 37 weeks gestation when proteinuric hypertension necessitated induction of labour. She remains well with no evidence of recurrence. This case highlights the options available in the treatment of carcinoma of the tongue during pregnancy together with the ethical considerations required, balanced against optimising maternal outcomes.

  3. Systemic lupus erythematosus: strategies to improve pregnancy outcomes.

    PubMed

    Yamamoto, Yuriko; Aoki, Shigeru

    2016-01-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with a high prevalence in females of childbearing age. Thus, reproduction in SLE patients is a major concern for clinicians. In the past, SLE patients were advised to defer pregnancy because of poor pregnancy outcomes and fear of SLE flares during pregnancy. Investigations to date show that maternal and fetal risks are higher in females with SLE than in the general population. However, with appropriate management of the disease, sufferers may have a relatively uncomplicated pregnancy course. Factors such as appropriate preconception counseling and medication adjustment, strict disease control prior to pregnancy, intensive surveillance during and after pregnancy by both the obstetrician and rheumatologist, and appropriate interventions when necessary play a key role. This review describes the strategies to improve pregnancy outcomes in SLE patients at different time points in the reproduction cycle (preconception, during pregnancy, and postpartum period) and also details the neonatal concerns.

  4. Successful treatment of systemic lupus erythematosus with subcutaneous immunoglobulin.

    PubMed

    Brasileiro, A; Fonseca Oliveira, J; Pinheiro, S; Paiva-Lopes, M J

    2016-05-01

    The therapeutic efficacy of high-dose intravenous immunoglobulin in systemic lupus erythematosus (SLE) patients is well established. However, side effects might limit its use and lead to the consideration of therapeutic alternatives, such as the subcutaneous formulation of immunoglobulin, which has been used in some patients with other autoimmune diseases. We report a case of SLE refractory to classical therapies. High-dose intravenous immunoglobulin was effective, but gave rise to significant side effects. The patient was successfully treated with subcutaneous human immunoglobulin, achieving and maintaining clinical and laboratory remission. A lower immunoglobulin dose was needed and no side effects were observed, compared to the intravenous administration. Subcutaneous immunoglobulin could be a better-tolerated and cost-saving therapeutic option for select SLE patients.

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

    PubMed

    Fortuna, Giulio; Brennan, Michael T

    2013-10-01

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

  6. Psoriatic Alopecia in a Patient with Systemic Lupus Erythematosus

    PubMed Central

    Iamsumang, Wimolsiri; Sriphojanart, Tueboon; Suchonwanit, Poonkiat

    2017-01-01

    Psoriasis is a chronic, recurrent, and relatively common inflammatory dermatologic condition, which demonstrates various clinical manifestations including hair loss. It was once believed that alopecia was not a presentation of scalp psoriasis, but it is now widely accepted that psoriatic alopecia exists. Although the majority of patients get hair regrowth, it can potentially lead to permanent hair loss. Herein, we report a case of 26-year-old female patient with systemic lupus erythematosus who presented with scalp hair loss and nonpruritic scaly plaques on the scalp. Her clinical presentation, dermoscopic, and histopathologic findings were consistent with psoriatic alopecia. Additionally, we also described a novel scalp dermoscopic pattern of “patchy dotted vessels” which we detected in the lesion of scalp psoriasis. PMID:28611622

  7. Shrinking lung syndrome in systemic lupus erythematosus-scleroderma overlap.

    PubMed

    Guleria, Vivek S; Singh, Pradeep K; Saxena, Puneet; Subramanian, Shankar

    2014-10-01

    Shrinking lung syndrome (SLS) is a infrequently reported manifestation of systemic lupus erythematosus (SLE). Reported prevalence of SLS is about 0.5% in SLE patients. Pathogenesis is not fully understood and different therapeutic modalities have been employed with variable results, as only 77 cases of SLS have been documented in literature. SLS in SLE-Scleroderma overlap has not been reported yet. We report a patient of SLE - scleroderma overlap presenting with dyspnea, intermittent orthopnea and pleuritic chest pain. Evaluation revealed elevated hemidiaphragms and severe restrictive defect. She was eventually diagnosed as a case of SLS. This case report is a reminder to the medical fraternity that SLS although a rare complication must be thought of in the special subset of patients of SLE having respiratory symptoms.

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

    PubMed

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

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

  9. Toward new criteria for systemic lupus erythematosus-a standpoint.

    PubMed

    Aringer, M; Dörner, T; Leuchten, N; Johnson, S R

    2016-07-01

    While clearly different in their aims and means, classification and diagnosis both try to accurately label the disease patients are suffering from. For systemic lupus erythematosus (SLE), this is complicated by the multi-organ nature of the disease and by our incomplete understanding of its pathophysiology. Hallmarks of SLE are the presence of antinuclear antibodies (ANA), and multiple immune-mediated organ symptoms that are largely independent. In an attempt to overcome limitations of the current sets of SLE classification criteria, a new four-phase approach is being developed, which is jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). This review attempts to delineate the performance of the current sets of criteria, the reasons for the decision for classification, and not diagnostic, criteria, and to provide a background of the current approach taken.

  10. Immunoregulation of NKT Cells in Systemic Lupus Erythematosus

    PubMed Central

    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. PMID:26819956

  11. Vascular lesions in systemic lupus erythematosus (SLE) with pulmonary hypertension.

    PubMed

    Wakaki, K; Koizumi, F; Fukase, M

    1984-05-01

    An autopsy case with SLE suffering from Raynaud's phenomenon and pulmonary hypertension was reported. Histological examinations revealed systemically marked fibrous intimal thickening of arteries and arterioles with or without thrombus throughout the whole body, especially of the pulmonary arteries and arterioles. Pulmonary arterial changes in the present case were compared with those in 52 autopsied cases with SLE without pulmonary hypertension, but there were no cases with such marked arterial changes as the present case. In addition, the incidence of pulmonary thrombosis was significantly higher in the cases with Raynaud's phenomenon than the cases without this phenomenon. However, the relation between pulmonary hypertension and Raynaud's phenomenon, pulmonary thrombosis, fibrous pericarditis, or type of lupus nephritis in SLE could not be clarified with a significant difference.

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

    PubMed

    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 < 0.0001); they had higher disease activity and reduced renal function. Linear regression analysis revealed that low 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.

  13. Quality of care in systemic lupus erythematosus: the association between process and outcome measures in the Lupus Outcomes Study.

    PubMed

    Yazdany, Jinoos; Trupin, Laura; Schmajuk, Gabriela; Katz, Patricia P; Yelin, Edward H

    2014-08-01

    Although process measures to assess quality of care in systemic lupus erythematosus (SLE) are available, their relationship to long-term outcomes has not been studied. Using a prospective, longitudinal cohort study, we examined the associations between high-quality care and two important SLE outcomes, disease activity and damage. Data were derived from the University of California, San Francisco Lupus Outcomes Study. Participants were followed from 2009 through 2013, responding to yearly surveys. Primary outcomes in this study were clinically meaningful increases in disease activity and damage, assessed by the Systemic Lupus Activity Questionnaire (SLAQ) and the Brief Index of Lupus Damage (BILD), respectively. Using multivariable regression, we examined the relationship between high performance on 13 validated quality measures (receipt of ≥85% of quality measures), and disease outcomes, adjusting for disease status, sociodemographic characteristics, healthcare services and follow-up time. The 737 participants were eligible for a mean of five quality measures (SD 2, range 2-12). There were 155 and 162 participants who had clinically meaningful increases in SLAQ and BILD, respectively. In our models, we found no statistically significant relationship between performance on quality measures and changes in SLAQ. However, receiving higher-quality SLE care was significantly protective against increased disease damage (adjusted OR 0.4, 95% CI 0.4 to 0.7), even after adjusting for covariates. In this community-based cohort, we illustrate for the first time a strong link between processes of care, defined by SLE quality measures, and the subsequent accumulation of disease damage, an important outcome. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  14. External Validation of the Lupus Impact Tracker in a Southeastern US Longitudinal Cohort With Systemic Lupus Erythematosus.

    PubMed

    Brandt, Jennifer E; Drenkard, Cristina; Kan, Hong; Bao, Gaobin; Dunlop-Thomas, Charmayne; Pobiner, Bonnie; Chang, David J; Jolly, Meenakshi; Lim, S Sam

    2017-06-01

    To examine the external validity of the Lupus Impact Tracker (LIT), a systemic lupus erythematosus (SLE)-specific, health-related quality of life (HRQoL) tool in a population-based cohort of patients with SLE in Atlanta, Georgia. We modeled the association of LIT scores with patient-reported measures of SLE activity (Systemic Lupus Activity Questionnaire [SLAQ]) and organ damage (self-administered Brief Index of Lupus Damage [SA-BILD]). We investigated the association of LIT scores with general HRQoL using the Short Form 12 (SF-12). Correlation, multivariable regression, and longitudinal analyses using general linear modeling with fixed effects were performed to investigate the association between the LIT and patient-reported disease activity (SLAQ); patient-reported disease damage (SA-BILD); mental health (mental component summary [MCS] of the SF-12); and physical health (physical component summary [PCS] of the SF-12). Demographic trends related to the LIT were also assessed using cross-sectional analysis. The LIT was significantly associated with disease activity (SLAQ), organ damage (SA-BILD), MCS scores, and PCS scores in both adjusted and unadjusted regression analysis (P < 0.0001). Longitudinal analysis demonstrated a significant association between the LIT and disease activity (SLAQ), MCS scores, and PCS scores (P < 0.0001), but not organ damage (SA-BILD). The LIT is a simple, patient-centered tool that can be used to assess HRQoL in patients with SLE. This study provides external validity of the LIT in a population-based cohort with a large number of African American patients with a relatively high disease burden. © 2016, American College of Rheumatology.

  15. Quality of Care in Systemic Lupus Erythematosus: The Association between Process and Outcome Measures in the Lupus Outcomes Study

    PubMed Central

    Yazdany, Jinoos; Trupin, Laura; Schmajuk, Gabriela; Katz, Patricia P.; Yelin, Edward H.

    2014-01-01

    Objectives Although process measures to assess quality of care in systemic lupus erythematosus (SLE) are available, their relationship to long-term outcomes has not been studied. Using a prospective, longitudinal cohort study, we examined the associations between high quality care and two important SLE outcomes, disease activity and damage. Methods Data derive from the University of California, San Francisco Lupus Outcomes Study. Participants were followed from 2009 through 2013, responding to yearly surveys. Primary outcomes in this study were clinically meaningful increases in disease activity and damage, assessed by the Systemic Lupus Activity Questionnaire (SLAQ) and the Brief Index of Lupus Damage (BILD), respectively. Using multivariable regression, we examined the relationship between high performance on 13 validated quality measures (receipt of ≥85% of quality measures), and disease outcomes, adjusting for disease status, sociodemographic characteristics, health care services, and follow-up time. Results The 737 participants were eligible for a mean of 5 quality measures (SD 2, range 2-12). There were 155 and 162 participants who had clinically meaningful increases in SLAQ and BILD, respectively. In our models, we found no statistically significant relationship between performance on quality measures and changes in SLAQ. However, receiving higher quality SLE care was significantly protective against increased disease damage (adjusted OR 0.4, 95% CI 0.4-0.7), even after adjusting for covariates. Discussion In this community-based cohort, we illustrate for the first time a strong link between processes of care, defined by SLE quality measures, and the subsequent accumulation of disease damage, an important outcome. PMID:24614054

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

    PubMed

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

    2017-04-01

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

  17. Anti-C1q antibodies in systemic lupus erythematosus.

    PubMed

    Orbai, A-M; Truedsson, L; Sturfelt, G; Nived, O; Fang, H; Alarcón, G S; Gordon, C; Merrill, Jt; Fortin, P R; Bruce, I N; Isenberg, D A; Wallace, D J; Ramsey-Goldman, R; Bae, S-C; Hanly, J G; Sanchez-Guerrero, J; Clarke, A E; Aranow, C B; Manzi, S; Urowitz, M B; Gladman, D D; Kalunian, K C; Costner, M I; Werth, V P; Zoma, A; Bernatsky, S; Ruiz-Irastorza, G; Khamashta, M A; Jacobsen, S; Buyon, J P; Maddison, P; Dooley, M A; Van Vollenhoven, R F; Ginzler, E; Stoll, T; Peschken, C; Jorizzo, J L; Callen, J P; Lim, S S; Fessler, B J; Inanc, M; Kamen, D L; Rahman, A; Steinsson, K; Franks, A G; Sigler, L; Hameed, S; Pham, N; Brey, R; Weisman, M H; McGwin, G; Magder, L S; Petri, M

    2015-01-01

    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. Information and blood samples were obtained in a cross-sectional study from patients with SLE (n = 308) and other rheumatologic diseases (n = 389) from 25 clinical sites (84% female, 68% Caucasian, 17% African descent, 8% Asian, 7% other). IgG anti-C1q against the collagen-like region was measured by ELISA. Prevalence of anti-C1q was 28% (86/308) in patients with SLE and 13% (49/389) in controls (OR = 2.7, 95% CI: 1.8-4, p < 0.001). Anti-C1q was associated with proteinuria (OR = 3.0, 95% CI: 1.7-5.1, p < 0.001), red cell casts (OR = 2.6, 95% CI: 1.2-5.4, p = 0.015), anti-dsDNA (OR = 3.4, 95% CI: 1.9-6.1, p < 0.001) and anti-Smith (OR = 2.8, 95% CI: 1.5-5.0, p = 0.01). Anti-C1q was independently associated with renal involvement after adjustment for demographics, ANA, anti-dsDNA and low complement (OR = 2.3, 95% CI: 1.3-4.2, p < 0.01). Simultaneously positive anti-C1q, anti-dsDNA and low complement was strongly associated with renal involvement (OR = 14.9, 95% CI: 5.8-38.4, p < 0.01). Anti-C1q was more common in patients with SLE and those of Asian race/ethnicity. We confirmed a significant association of anti-C1q with renal involvement, independent of demographics and other serologies. Anti-C1q 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. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  18. Anti-C1q Antibodies in Systemic Lupus Erythematosus

    PubMed Central

    ORBAI, ANA-MARIA; TRUEDSSON, LENNART; STURFELT, GUNNAR; NIVED, OLA; FANG, HONG; ALARCÓN, GRACIELA S.; GORDON, CAROLINE; MERRILL, JOAN T.; FORTIN, PAUL R.; BRUCE, IAN N.; ISENBERG, DAVID A.; WALLACE, DANIEL J.; RAMSEY-GOLDMAN, ROSALIND; BAE, SANG-CHEOL; HANLY, JOHN G.; SANCHEZ-GUERRERO, JORGE; CLARKE, ANN E.; ARANOW, CYNTHIA B.; MANZI, SUSAN; UROWITZ, MURRAY B.; GLADMAN, DAFNA D.; KALUNIAN, KENNETH C.; COSTNER, MELISSA I.; WERTH, VICTORIA P.; ZOMA, ASAD; BERNATSKY, SASHA; RUIZ-IRASTORZA, GUILLERMO; KHAMASHTA, MUNTHER A.; JACOBSEN, SOREN; BUYON, JILL P.; MADDISON, PETER; DOOLEY, MARY ANNE; VAN VOLLENHOVEN, RONALD F.; GINZLER, ELLEN; STOLL, THOMAS; PESCHKEN, CHRISTINE; JORIZZO, JOSEPH L.; CALLEN, JEFFREY P.; LIM, S. SAM; FESSLER, BARRI J.; INANC, MURAT; KAMEN, DIANE L.; RAHMAN, ANISUR; STEINSSON, KRISTJAN; FRANKS, ANDREW G.; SIGLER, LISA; HAMEED, SUHAIL; PHAM, NEENA; BREY, ROBIN; WEISMAN, MICHAEL H.; MCGWIN, GERALD; MAGDER, LAURENCE S.; PETRI, MICHELLE

    2014-01-01

    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 multi-center study. Methods Information and blood samples were obtained in a cross-sectional study from patients with SLE (n=308) and other rheumatologic diseases (n=389) from 25 clinical sites (84% female, 68% Caucasian, 17% African descent, 8% Asian, 7% other). IgG anti-C1q against the collagen-like region was measured by ELISA. Results Prevalence of anti-C1q was 28% (86/308) in patients with SLE and 13% (49/389) in controls (OR=2.7, 95% CI: 1.8-4, p<0.001). Anti-C1q was associated with proteinuria (OR=3.0, 95% CI: 1.7-5.1, p<0.001), red cell casts (OR=2.6, 95% CI: 1.2-5.4, p=0.015), anti-dsDNA (OR=3.4, 95% CI: 1.9-6.1, p<0.001) and anti-Smith (OR=2.8, 95% CI: 1.5-5.0, p=0.01). Anti-C1q was independently associated with renal involvement after adjustment for demographics, ANA, anti-dsDNA and low complement (OR=2.3, 95% CI: 1.3-4.2, p<0.01). Simultaneously positive anti-C1q, anti-dsDNA and low complement was strongly associated with renal involvement (OR=14.9, 95% CI: 5.8-38.4, p<0.01). Conclusions Anti-C1q was more common in patients with SLE and those of Asian race/ethnicity. We confirmed a significant association of anti-C1q with renal involvement, independent of demographics and other serologies. Anti-C1q 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. PMID:25124676

  19. Borderline tuberculoid leprosy in childhood onset systemic lupus erythematosus patient.

    PubMed

    Lopes, V A P; Lourenço, D M R; Guariento, A; Trindade, M A; Avancini, J; Silva, C A

    2015-11-01

    Leprosy is a contagious and chronic systemic granulomatous disease caused by the bacillus Mycobacterium leprae. To our knowledge, no case of leprosy in a childhood-onset systemic lupus erythematosus (c-SLE) patient has been reported. For a period of 31 years, 312 c-SLE patients were followed at the Pediatric Rheumatology Unit of our University Hospital. One of them (0.3%) had tuberculoid leprosy skin lesions during the disease course and is here reported. A 10-year-old boy from Northwest of Brazil was diagnosed with c-SLE based on malar rash, photosensitivity, oral ulcers, lymphopenia, proteinuria, positive antinuclear antibodies, anti-double-stranded DNA, anti-Sm and anti-Ro/SSA autoantibodies. He was treated with prednisone, hydroxychloroquine and intravenous cyclophosphamide, followed by mycophenolate mofetil. At 12-years-old, he presented asymmetric skin lesions characterized by erythematous plaques with elevated external borders and hypochromic center with sensory loss. Peripheral nerve involvement was not evidenced. No history of familial cases of leprosy was reported, although the region where the patient resides is considered to be endemic for leprosy. Skin biopsy revealed a well-defined tuberculoid form. A marked thickening of nerves was observed, often destroyed by granulomas, without evidence of Mycobacterium leprae bacilli. At that time, the SLEDAI-2K score was 4 and he had been receiving prednisone 15 mg/day, hydroxychloroquine 200 mg/day and mycophenolate mofetil 3 g/day. Paucibacillary treatment for leprosy with dapsone and rifampicine was also introduced. In conclusion, we have reported a rare case of leprosy in the course of c-SLE. Leprosy should always be considered in children and adolescents with lupus who present skin abnormalities, particularly with hypoesthesic or anesthesic cutaneous lesions.

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

    PubMed

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

    2016-10-01

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

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

    PubMed Central

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

    2015-01-01

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

  2. [Tuberculosis in a cohort of patients with systemic lupus erythematosus].

    PubMed

    González León, Rocío; Garrido Rasco, Rocío; Chinchilla Palomares, Eduardo; García Hernández, Francisco José; Castillo Palma, M Jesús; Sánchez Román, Julio

    2010-01-01

    1) To study tuberculosis (TB) infection in a cohort of patients with systemic lupus erythematosus (SLE) and to compare its frequency and characteristics with that of others series. 2) To look for differential characteristic among SLE patients with and without TB. 3) To investigate if there was any relationship between TB's most severe forms and higher doses of glucocorticoids (GC) or other immunosuppressants. Retrospective review of medical records of 789 SLE patients and description of the clinical characteristics of 13 cases of active TB infection among them. Bibliographical search in MEDLINE-PubMed of the SLE/TB series published, using the terms: infection, tuberculosis, systemic lupus erythematosus. Comparative study of clinical, biological and therapeutic differences between cases (SLE/TB+) and controls (SLE/TB) using χ(2) and Fisher exact test. Thirteen patients with active tuberculosis were detected (10 women, average age 36 years/SD 11,2/prevalence 1,6%). Nine (69,2%) of them were primary infections and 4 (30,8%) reactivations. Microbiological diagnosis (smear examination for acid-fast bacilli and/or culture on Lowestein-Jensen medium) was established in 11 patients (84,6%). TB Pulmonary manifestations was present in 9 patients (69,2%) and extra-pulmonary manifestations were found in 8 [(61,5%); 6 of them (46%) were disseminated forms]. Nine (69,2%) patients were on GC therapy at the moment TB was diagnosed. Four of the TB patients died (30,8%). Myositis was more frequent in TB cases (p < 0,05). This data is similar to that reported in the literature. In our series, TB mortality was high (30,8%) in a patients with SLE. Frequency of extrapulmonary forms was double than that described in the Spanish population. Patients with higher GC dose had more severe forms of TB. Copyright © 2009 Elsevier España, S.L. All rights reserved.

  3. Immunogenetics of systemic lupus erythematosus: A comprehensive review.

    PubMed

    Ghodke-Puranik, Yogita; Niewold, Timothy B

    2015-11-01

    Our understanding of the genetic basis of systemic lupus erythematosus has progressed rapidly in recent years. While many genetic polymorphisms have been associated with disease susceptibility, the next major step involves integrating these genetic polymorphisms into the molecular mechanisms and cellular immunology of the human disease. In this review, we summarize some recent work in this area, including the genetics of the type I IFN response in SLE, including polygenic and monogenic factors, as well as epigenetic influences. Contributions of both HLA and non-HLA polymorphisms to the complex genetics of SLE are reviewed. We also review recent reports of specific gene deficits leading to monogenic SLE-like syndromes. The molecular functions of common SLE-risk variants are reviewed in depth, including regulatory variations in promoter and enhancer elements and coding-change polymorphisms, and studies which are beginning to define the molecular and cellular functions of these polymorphisms in the immune system. We discuss epigenetic influences on lupus, with an emphasis on micro-RNA expression and binding, as well as epigenetic modifications that regulate the expression levels of various genes involved in SLE pathogenesis and the ways epigenetic marks modify SLE susceptibility genes. The work summarized in this review provides a fascinating window into the biology and molecular mechanisms of human SLE. Understanding the functional mechanisms of causal genetic variants underlying the human disease greatly facilitates our ability to translate genetic associations toward personalized care, and may identify new therapeutic targets relevant to human SLE disease mechanisms. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. Possible risk factors associated with greater damage in systemic lupus erythematosus patients: an Egyptian multicenter study.

    PubMed

    Hammad, M; Eissa, M; Fathi, S

    2016-08-01

    Systemic lupus erythematosus (SLE) is a prototypic multisystem autoimmune disorder. The total damage in a patient with SLE may result from SLE itself or from any other pathologic process. The aim of this study was to assess risk factors of greater damage in a sample of Egyptian SLE patients. This Egyptian multicenter retrospective study included 100 SLE patients: 64 patients from Cairo University Hospitals and 36 patients from Zagazig University Hospitals. The Systemic Lupus International Collaborative Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (ACR-DI) was used to document the damage in each patient. The total SLICC/ACR-DI score ranged from 0 to 8. A higher DI score was found in hypertensive patients, compared to normotensive patients; and among those with positive anti-phospholipid antibodies, compared to those with negative anti-phospholipid antibodies. This difference was statistically significant (p < 0.01). Also, a higher DI score was found in cyclophosphamide users, compared to non-users; and in those with proteinuria and seizures, compared to those without; and the difference was statistically significant (p < 0.05). There was a significant positive correlation between the DI and patient age (p < 0.05). Damage in SLE cannot be prevented completely, as SLE disease is considered an aggressive disease treated by aggressive medications, but rheumatologists should try to minimize damage as much as possible to maintain the patients' health, functioning and general wellbeing. © The Author(s) 2016.

  5. Ethnic differences in DNA methyltransferases expression in patients with systemic lupus erythematosus.

    PubMed

    Wiley, Kenneth L; Treadwell, Edward; Manigaba, Kayihura; Word, Beverly; Lyn-Cook, Beverly D

    2013-02-01

    Systemic lupus erythematous (SLE) is a systemic autoimmune inflammatory disease with both genetic and epigenetic etiologies. Evidence suggests that deregulation of specific genes through epigenetic mechanisms may be a contributing factor to SLE pathology. There is increasing evidence that DNA methyltransferase activity may be involved. This study demonstrated modulation in expression of DNA methyltransferases (DNMTs) according to ethnicity in patients diagnosed with SLE. Furthermore, differential expression in one of the DNMTs was found in a subset of lupus patients on dehydroepiandrosterone (DHEA) therapy. Real-time PCR analyses of DNMT1, DNMT3A and DNMT3B in peripheral blood mononuclear cells from a cohort of African American and European American lupus and non-lupus women were conducted. Also, global DNA methylation was assessed using the MethylFlash(TM) methylated quantification colorimetric assay. Significant increase in DNMT3A (p < 0.001) was shown in lupus patients when compared to age-matched healthy controls. This increase was associated with a higher SLEDI index. More striking was that expression levels for African American (AA) women were higher than European American women in the lupus populations. A subset of AA women on DHEA therapy showed a significant decrease (p < 0.05) in DNMT3A expression in comparison to lupus patients not on the therapy. DHEA is an androgenic steroid found in low levels in the serum of lupus patients. Supplementation of this hormone has been shown to be beneficial to some lupus patients. DHEA was not shown to effect DNMT1 or DNMT3B expression. Increased expression was also noted in DNMT3B (p < 0.05) in lupus patients compared to age-matched healthy controls. However, no significant difference was noted in DNMT1 (p = 0.2148) expression between lupus patients and healthy controls. Although increases were detected in de novo methyltransferases, a global decrease (p < 0.001) in 5-methycytosine was observed in

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

    PubMed

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

    2017-01-01

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

  7. Carbon monoxide inhibits T cell activation in target organs during systemic lupus erythematosus

    PubMed Central

    Mackern-Oberti, J P; Obreque, J; Méndez, G P; Llanos, C; Kalergis, A M

    2015-01-01

    Systemic lupus erythematosus is characterized by the presence of circulating anti-nuclear antibodies (ANA) and systemic damage that includes nephritis, haematological manifestations and pulmonary compromise, among others. Although major progress has been made in elucidating the molecular mechanisms responsible for autoimmunity, current therapies for lupus have not improved considerably. Because the exposure of carbon monoxide (CO) has been shown to display beneficial immunoregulatory properties in different immune-mediated diseases, we investigated whether CO therapy improves lupus-related kidney injury in lupus mice. MRL-Faslpr lupus mice were exposed to CO and disease progression was evaluated. ANA, leucocyte-infiltrating populations in spleen, kidney and lung and kidney lesions, were measured. CO therapy significantly decreased the frequency of activated B220+ CD4− CD8− T cells in kidneys and lungs, as well as serum levels of ANA. Furthermore, we observed that CO therapy reduced kidney injury by decreasing proliferative glomerular damage and immune complexes deposition, decreased proinflammatory cytokine production and finally delayed the impairment of kidney function. CO exposure ameliorates kidney and lung leucocyte infiltration and delays kidney disease in MRL-Faslpr lupus mice. Our data support the notion that CO could be explored as a potential new therapy for lupus nephritis. PMID:26095291

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

    PubMed Central

    Urowitz, M B; Gladman, D D; Anderson, N M; Su, J; Romero-Diaz, J; Bae, S C; Fortin, P R; Sanchez-Guerrero, J; Clarke, A; Bernatsky, S; Gordon, C; Hanly, J G; Wallace, D J; Isenberg, D; Rahman, A; Merrill, J; Ginzler, E; Alarcón, G S; Fessler, B F; Petri, M; Bruce, I N; Khamashta, M; Aranow, C; Dooley, M; Manzi, S; Ramsey-Goldman, R; Sturfelt, G; Nived, O; Steinsson, K; Zoma, A; Ruiz-Irastorza, G; Lim, S; Kalunian, K C; Ỉnanç, M; van Vollenhoven, R; Ramos-Casals, M; Kamen, D L; Jacobsen, S; Peschken, C; Askanase, A; Stoll, T

    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 15 months of SLE diagnosis. MIs were reported and attributed on a specialised vascular event form. MIs were confirmed by one or more of the following: abnormal ECG, typical or atypical symptoms with ECG abnormalities and elevated enzymes (≥2 times upper limit of normal), or abnormal stress test, echocardiogram, nuclear scan or angiogram. Descriptive statistics were used. Results 31 of 1848 patients who entered the cohort had an MI. Of those, 23 patients had an MI prior to SLE diagnosis or within the first 2 years of disease. Of the 23 patients studied, 60.9% were female, 78.3% were Caucasian, 8.7% black, 8.7% Hispanic and 4.3% other. The mean age at SLE diagnosis was 52.5±15.0 years. Of the 23 MIs that occurred, 16 MIs occurred at a mean of 6.1±7.0 years prior to diagnosis and 7 occurred within the first 2 years of follow-up. Risk factors associated with early MI in univariate analysis are male sex, Caucasian, older age at diagnosis, hypertension, hypercholesterolaemia, family history of MI and smoking. In multivariate analysis only age (OR=1.06 95% CI 1.03 to 1.09), hypertension (OR=5.01, 95% CI 1.38 to 18.23), hypercholesterolaemia (OR=4.43, 95% CI 1.51 to 12.99) and smoking (OR=7.50, 95% 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. PMID:27099765

  9. Pulmonary hypertension, systemic lupus erythematosus, and the contraceptive pill: another report.

    PubMed

    Miller, M H

    1987-02-01

    I report the case of a woman with systemic lupus erythematosus who had pulmonary hypertension unassociated with chronic interstitial lung disease or pulmonary emboli. She had started taking the contraceptive pill seven months previously.

  10. [Intravenous immunoglobulin administration to a patient with systemic lupus erythematosus and pneumococcal septicemia].

    PubMed

    Maltbaek, N; Harreby, M S; Thøgersen, B

    1994-07-04

    A case history is presented of a woman with systemic lupus erythematosus, sepsis and pneumonia caused by Streptococcus pneumoniae. Conventional treatment was supplemented with intravenous human immunoglobulin with remarkable effect. The treatment is discussed.

  11. Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review

    PubMed Central

    2012-01-01

    Introduction The occurrence of systemic lupus erythematosus has been only rarely reported in patients with sickle-cell disease. Case presentation We describe the case of a 23-year-old North-African woman with sickle-cell disease and systemic lupus erythematosus, and discuss the pointers to the diagnosis of this combination of conditions and also present a review of literature. The diagnosis of systemic lupus erythematosus was delayed because our patient’s symptoms were initially attributed to sickle-cell disease. Conclusions Physicians should be alerted to the possible association of sickle-cell disease and systemic lupus erythematosus so as not to delay correct diagnosis and initiation of appropriate treatment. PMID:23101910

  12. Successful Pregnancy Following Assisted Reproduction in Woman With Systemic Lupus Erythematosus and Hypertension: A Case Report.

    PubMed

    de Macedo, José Fernando; de Macedo, Gustavo Capinzaiki; Campos, Luciana Aparecida; Baltatu, Ovidiu Constantin

    2015-09-01

    Patients with systemic lupus erythematosus have a poor prognosis of pregnancy, since it is associated with significant maternal and fetal morbidity, including spontaneous miscarriage, pre-eclampsia, intrauterine growth restriction, fetal death and pre-term delivery. We report a case with successful pregnancy in a patient with systemic lupus erythematosus and hypertension. A 39-year-old nulliparous woman presented with systemic lupus erythematosus with antinuclear and antiphospholipid antibodies, hypertension and recurrent pregnancy loss presented for assisted reproduction. The patient responded well to enoxaparin and prednisone during both assisted reproduction and prenatal treatment. This case report indicates that prescription of immunosuppressant and blood thinners can be safely recommended throughout the whole prenatal period in patients with systemic lupus erythematosus. Enoxaparin and prednisone may be prescribed concurrently during pregnancy.

  13. Acute acalculous cholecystitis in systemic lupus erythematosus: a rare initial manifestation.

    PubMed

    Manuel, Valdano; Pedro, Gertrudes Maria; Cordeiro, Lemuel Bornelli; de Miranda, Sandra Maria da Rocha Neto

    2016-01-01

    Acute acalculous cholecystitis is a very rare gastrointestinal manifestation in systemic lupus erythematosus and becomes rarer as an initial manifestation. There are only two cases reported. The authors report a 20-year-old black woman that presented acute acalculous cholecystitis revealed by abdominal computed tomography. During hospitalization, she was diagnosed systemic lupus erythematosus. Conservative treatment with antibiotics was performed with complete remission of the symptoms. Corticosteroid was started in ambulatory. Cholecystectomy has been the treatment of choice in acute acalculous cholecystitis as a complication of systemic lupus erythematosus. The patient responded well to conservative treatment, and surgery was not required. This case is unique in the way that corticosteroid was started in ambulatory care. We should not forget that the acute acalculous cholecystitis can be the initial presentation of systemic lupus erythematosus although its occurrence is very rare. Conservative treatment should be considered. Abdominal computed tomography was a determinant exam for better assessment of acute acalculous cholecystitis.

  14. A spontaneous intercostal artery hemorrhage in systemic lupus erythematosus.

    PubMed

    Lu, Chun-Chi; Chen, Chen-Hung; Yeh, Song-Feng; Lai, Jenn-Haung; Chang, Deh-Ming

    2012-03-01

    Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that can lead to damage to several vital organs. Antiphospholipid syndrome (APS), manifesting as vascular thromboembolic events and morbidities of pregnancy in the presence of antiphospholipid antibodies (aPL), has been described in patients with SLE. Catastrophic antiphospholipid syndrome (CAPS), in contradistinction to APS, is defined as three or more organs affected by thrombotic microangiopathy in patients demonstrating aPL and can result in mortality up to 50%. We describe a unique SLE patient who was diagnosed with recurrent APS presented with axillary venous thrombosis and subsequent superficial edema and compartment syndrome. The CAPS followed and revealed thromboses over liver, spleen, and acute pancreatitis. The spontaneous hemorrhage of left fourth intercostal artery (ICA) and left axillary artery occured at the same time without vasculitis or severe trauma. Though emergency transcatheter arterial embolization (TAE) of the left fourth ICA was successfully accomplished by the radiologist. The repeated computed tomography angiogram of chest demonstrated remission of ruptured ICA. Nevertheless, the patient died of diffuse alveolar hemorrhage and respiratory failure and shock. Both disseminated intravascular coagulation (DIC) and CAPS share similar characteristics encompassing thrombotic microangiopathy, bleeding, thromboembolism, and multiple organ dysfunction. It is difficult to distinguish between them, especially in cases such as our uremic SLE patient with a calamitous disease progression. The emphasis of treatment for DIC is on platelet and fresh plasma transfusion, in contrast with anti-coagulant for CAPS. To the best of our knowledge, this is the first report describing ICA hemorrhage in an SLE patient without vasculitis or aneurysm. The lupus flare initiated a pathological immunological cascade and resulted in the CAPS and the vascular damage.

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

    PubMed

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

    2016-03-01

    The incidence of thrombosis in patients with systemic lupus erythematosus (SLE) is 25 to 50-fold higher than in the general population; we aimed to define the characteristics of venous thrombotic events (VTE) and arterial thrombotic events (ATE) to identify the patients at highest risk. The study included 219 patients with recent-onset SLE. At baseline, standardized medical history and laboratory tests were done. Followup visits occurred quarterly, and information about damage accrual, comorbidities, and cardiovascular risk factors was updated annually. Main outcome was development of TE after SLE diagnosis. Thirty-five patients (16%) developed TE (27 VTE, 8 ATE) during 5.21 years of followup; incidence rate 31/1000 patient-years. Most events (57%) developed within the first year of diagnosis, and 69% were not associated with lupus anticoagulant (LAC), determined with 1 method. VTE developed earlier than ATE (2.0 vs 57.5 mos, p = 0.02). In the multivariate analysis, variables preceding VTE included cutaneous vasculitis, nephrotic syndrome, dose of prednisone, and LAC in combination with anti-RNP/Sm antibodies (p < 0.03). Patients with ATE were older (median age 44 vs 29 yrs, p = 0.04), smokers, and had hypertension, diabetes mellitus, dyslipidemia, at least 2 traditional risk factors, nephrotic syndrome, chronic damage, and a higher cumulative dose of prednisone (p < 0.05). LAC in combination with anti-RNP/Sm antibodies was associated with VTE and improved the accuracy for predicting it. Our study suggests that in SLE, VTE and ATE have different risk factors. Understanding these differences is helpful for identifying patients at highest risk. The use of LAC plus anti-RNP/Sm for predicting VTE deserves further study.

  16. Genetic Risk Factors for Thrombosis in Systemic Lupus Erythematosus

    PubMed Central

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

    2013-01-01

    Objectives Thrombosis is a serious complication of systemic lupus erythematosus (SLE). We investigated whether genetic variants implicated in thrombosis pathways are associated with thrombosis among two ethnically diverse SLE cohorts. Methods Our discovery cohort consisted of 1698 SLE patients enrolled in the UCSF Lupus Genetics Project and our replication cohort included 1361 SLE patients enrolled in the PROFILE cohort. Patients fulfilled American College of Rheumatology SLE criteria and data relevant to thrombosis were available. Thirty-three single nucleotide polymorphisms (SNPs) previously shown to be associated with risk of deep venous thrombosis in the general population or implicated in thrombosis pathways were genotyped and tested for association with thrombosis in bivariate allelic analyses. SNPs with p<0.1 in the bivariate analyses were further tested in multivariable logistic regression models adjusted for age, sex, disease duration, anti-phospholipid antibody status, smoking, nephritis, and medications. Results In the discovery cohort, 23% of SLE patients experienced a thrombotic event. SNPs in the following genes demonstrated association with thrombosis risk overall in the discovery or replication cohorts and were assessed using meta-analytic methods: factor V Leiden (FVL) rs6025 (OR 1.85, p 0.02) and methylenetetrahydrofolate reductase (MTHFR) rs1801133 (OR 0.75, p 0.04) in European Americans and fibrinogen gamma (FGG) rs2066865 (OR 1.91, p 0.01) in Hispanic Americans. SNPs associated with venous thrombosis risk included: MTHFR rs1801131 (OR 1.51, p=0.01), MTHFR rs1801133 (OR 0.70, p=0.04), FVL rs6025 (OR 2.69, p=0.002) and FGG rs2066865 (OR 1.49, p=0.02) in European Americans. SNPs associated with arterial thrombosis risk included FGG rs2066865 (OR 2.19, p=0.003) in Hispanics. Conclusion Our results implicate specific genetic risk factors for thrombosis in patients with SLE and suggest that genetic risk for thrombosis differs across ethnic groups

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

    PubMed Central

    Sinicato, Nailú Angélica; Postal, Mariana; Peres, Fernando Augusto; Peliçari, Karina de Oliveira; Marini, Roberto; dos Santos, Allan de Oliveira; Ramos, Celso Dario; Appenzeller, Simone

    2014-01-01

    Background. In systemic lupus erythematosus (SLE), atherosclerosis is attributed to traditional and lupus related risk factors, including metabolic syndrome (MetS), obesity, and inflammation. Objective. To evaluate the association between obesity, measures of body fat content, serum tumor necrosis factor alpha (TNF-α), and interleukin (IL)-6 and -10 levels in childhood-onset SLE (cSLE). Methods. We screened consecutive cSLE patients followed up in the Pediatric Rheumatology Outpatient Clinic of the State University of Campinas. cSLE patients were assessed for disease and damage. Obesity was definite as body mass index (BMI) ≥30 kg/m2. Serum TNF-α, IL-6, and IL-10 levels were measured by ELISA. Dual-energy X-ray absorptiometry was used to determine total fat mass, lean mass, and percent of body fat. Results. We included 52 cSLE patients and 52 controls. cSLE patients had higher serum TNF-α  (P = 0.004), IL-6 (P = 0.002), and IL-10 (P < 0.001) levels compared to controls. We observed higher serum TNF-α  (P = 0.036) levels in cSLE patients with obesity. An association between serum TNF-α levels and body fat percent (P = 0.046) and total fat mass on trunk region (P = 0.035) was observed. Conclusion. Serum TNF-α levels were associated with obesity and body fat content in cSLE. Our finding suggests that obesity may contribute to the increase of serum TNF-α levels in cSLE. PMID:24741576

  18. Understanding the Epidemiology and Progression of Systemic Lupus Erythematosus

    PubMed Central

    Pons-Estel, Guillermo J.; Alarcón, Graciela S.; Scofield, Lacie; Reinlib, Leslie; Cooper, Glinda S.

    2009-01-01

    Objectives This review examines the burden and patterns of disease in systemic lupus erythematosus (SLE) and the influence and interactions of gender, ethnicity, age, and psychosocial attributes with respect to disease progression, focusing on issues relevant to clinical practice and research. Methods PubMed literature search complemented by review of bibliographies listed in identified articles. Results An increased risk among reproductive age women is clearly seen in African Americans in the United States. However, in other populations, a different pattern is generally seen, with the highest age-specific incidence rates occurring in women after age 40 years. The disease is 2 to 4 times more frequent, and more severe, among nonwhite populations around the world and tends to be more severe in men and in pediatric and late-onset lupus. SLE patients now experience a higher than 90% survival rate at 5 years. The less favorable survival experience of ethnic minorities is possibly related to socioeconomic status rather than to ethnicity per se, and adequate social support has been shown to be a protective factor, in general, in SLE patients. Discordance between physician and patient ratings of disease activity may affect quality of care. Conclusions Our understanding of ways to improve outcomes in SLE patients could benefit from patient-oriented research focusing on many dimensions of disease burden. Promising research initiatives include the inclusion of community-based patients in longitudinal studies, use of self-assessment tools for rating disease damage and activity, and a focus on self-perceived disease activity and treatment compliance. PMID:19136143

  19. Variables associated to fetal microchimerism in systemic lupus erythematosus patients.

    PubMed

    da Silva Florim, Greiciane Maria; Caldas, Heloisa Cristina; Pavarino, Erika Cristina; Bertollo, Eny Maria Goloni; Fernandes, Ida Maria Maximina; Abbud-Filho, Mario

    2016-01-01

    In the present study, we sought to identify the factors during the pregnancy of systemic lupus erythematosus (SLE) patients that could be linked to the presence and proliferation of male fetal cells (MFC) and the possible relation between these factors and development of lupus nephritis (LN). We evaluated 18 healthy women (control group) and 28 women affected by SLE. Genomic DNA was extracted from peripheral blood and quantified using the technique of quantitative real-time polymerase chain reaction (qPCR) for specific Y chromosome sequences. The amount of MFC was significantly higher in the SLE group compared with the controls (SLE 252 ± 654 vs control 2.13 ± 3.7; P = 0.029). A higher amount of MFC was detected among multiparous SLE patients when compared with the control group (SLE 382 ± 924 vs control 0.073 ± 0.045; P = 0.019). LN was associated with reduced amount of MFC (LN 95.5 ± 338 vs control 388 ± 827; P = 0.019) especially when they have delivered their child before age 18 (LN 0.23 ± 0.22 vs control 355 ± 623; P = 0.028). SLE patients present a higher amount of MFC, which may increase with the time since birth of the first male child. LN patients showed an inverse correlation with MFC, suggesting that the role of the cells may be ambiguous during the various stages of development of the disease.

  20. Modified Framingham Risk Factor Score for Systemic Lupus Erythematosus.

    PubMed

    Urowitz, Murray B; Ibañez, Dominique; Su, Jiandong; Gladman, Dafna D

    2016-05-01

    The traditional Framingham Risk Factor Score (FRS) underestimates the risk for coronary artery disease (CAD) in patients with systemic lupus erythematosus (SLE). We aimed to determine whether an adjustment to the FRS would more accurately reflect the higher prevalence of CAD among patients with SLE. Patients with SLE without a previous history of CAD or diabetes followed regularly at the University of Toronto Lupus Clinic were included. A modified FRS (mFRS) was calculated by multiplying the items by 1.5, 2, 3, or 4. In the first part of the study, using one-third of all eligible patients, we evaluated the sensitivity and specificity of the FRS and the different multipliers for the mFRS. In the second part of the study, using the remaining 2/3 of the eligible patients, we compared the predictive ability of the FRS to the mFRS. In the third part of the study, we assessed the prediction for CAD in a time-dependent analysis of the FRS and mFRS. There were 905 women (89.3%) with a total of 95 CAD events included. In part 1, we determined that a multiplier of 2 provided the best combination of sensitivity and specificity. In part 2, 2.4% of the patients were classified as moderate/high risk based on the classic FRS and 17.3% using the 2FRS (the FRS with a multiplier of 2). In part 3, a time-dependent covariate analysis for the prediction of the first CAD event revealed an HR of 3.22 (p = 0.07) for the classic FRS and 4.37 (p < 0.0001) for the 2FRS. An mFRS in which each item is multiplied by 2 more accurately predicts CAD in patients with SLE.

  1. Hearing and vestibular disorders in patients with systemic lupus erythematosus.

    PubMed

    Batuecas-Caletrío, A; del Pino-Montes, J; Cordero-Civantos, C; Calle-Cabanillas, M I; Lopez-Escamez, J A

    2013-04-01

    Systemic lupus erythematosus (SLE) is associated with several comorbidities, including hearing and vestibular disorders. We recently described an increase of SLE prevalence in patients with Menierés disease (MD). The aim of this study is to explore if a subset of SLE patients may have a common inner ear disorder and determine the potential relationship with migraine. METHODS; Eighty-nine patients with SLE (according to the American College of Rheumatology criteria for the diagnosis of SLE) were evaluated for audiovestibular symptoms by a telephone interview carried out by two experienced otoneurologists. Twenty-one patients with SLE were referred to the otoneurology clinic for a detailed clinical history for criteria assessment for MD and a complete audiovestibular examination (audiogram, oculomotor, and caloric testing with video-oculography). Recurrent headache was found in 50/89 patients, and 26 of them had diagnostic criteria for migraine. Twenty-four percent of patients reported sensorineural hearing loss (SNHL) or episodic vertigo. Among the eight patients (9%) with episodic vertigo, one had criteria for definite MD and another two patients had criteria for possible MD. SNHL was found to be associated with a history of episodic vertigo (Fisher's test, p=0.02), but not with headache or migraine. SLE and episodic vertigo were not associated with tinnitus, migraine, lupus nephritis, antinuclear antibodies or antiphospholipid syndrome. Migraine, SNHL and episodic vertigo are comorbid conditions in patients with SLE, but migraine is not associated with SNHL or vertigo in these patients. However, SNHL and vertigo are associated conditions in SLE, suggesting a common audiovestibular dysfunction.

  2. Indices to assess patients with systemic lupus erythematosus in clinical trials, long-term observational studies, and clinical care.

    PubMed

    Castrejón, I; Tani, C; Jolly, M; Huang, A; Mosca, M

    2014-01-01

    This review summarises most currently used indices to assess and monitor patients with systemic lupus erythematosus (SLE) in clinical trials, long-term observational studies, and clinical care. Six SLE disease activity indices include the British Isles Lupus Assessment Group Index (BILAG), European Consensus Lupus Activity Measurement (ECLAM), Systemic Lupus Activity Measure (SLAM), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), Lupus Activity Index (LAI), and Systemic Lupus Erythematosus Activity Questionnaire (SLAQ). Three SLE responder indices include Responder Index for Lupus Erythematosus (RIFLE), SLE Responder Index (SRI), and BILAG Based Combined Lupus Assessment (BICLA). Three SLE damage indices include the Systemic Lupus International Collaborating Clinics/American College of Rheumatology-Damage Index (SLICC/ACE-DI), Lupus Damage Index Questionnaire (LDIQ), and Brief Index of Lupus Damage (BILD). The SLAQ, LDIQ and the BILD are patient self-report questionnaires, which appear to give similar information to physician-completed indices, but are pragmatically more easily completed as patients do almost all the work. Additional self-report indices which have been used to assess and monitor patients with in SLE include a generic general health short form 36 (SF36), a SLE-specific Lupus Patient Reported Outcome (LupusPRO), and a generic rheumatology index, Routine Assessment of Patient Index Data 3 (RAPID3). These activity, response, damage and patient self-report indices have been validated at different levels with no consensus about what it is the most appropriate for every setting. Sensitive and feasible assessment of SLE in clinical trials, observational studies, and busy clinical settings remains a challenge to the rheumatology community.

  3. The interplay of inflammation and cardiovascular disease in systemic lupus erythematosus

    PubMed Central

    2011-01-01

    Patients with systemic lupus erythematosus have up to a 50-fold increased risk of developing atherosclerotic cardiovascular disease. Recent advances in the etiology of vascular damage in this disease stress the interplay of lupus-specific inflammatory factors with traditional cardiac risk factors, leading to increased endothelial damage. This review analyzes the putative role that immune dysregulation and lupus-specific factors may play in the pathogenesis of premature vascular damage in this disease. The potential role of various cytokines, in particular type I interferons, in the development of accelerated atherosclerosis is examined. Potential therapeutic targets are discussed. PMID:21371346

  4. Rapid onset of massive ascites as the initial presentation of systemic lupus erythematosus.

    PubMed

    Weinstein, P J; Noyer, C M

    2000-01-01

    Ascites in systemic lupus erythematosus (SLE) is rarely massive, and either accompanies the typical manifestations of active disease or results from nephrotic syndrome, protein-losing enteropathy, constrictive pericarditis, and conditions unrelated to lupus. Marked ascites has been attributed to chronic lupus peritonitis, characterized by the insidious onset of massive, painless ascites and unrelated to disease activity. Regardless of the etiology, ascites typically has a gradual onset and occurs after a diagnosis of SLE has been made. We describe a young woman presenting with the rapid development of massive ascites as the initial manifestation of SLE.

  5. Molecular therapies for systemic lupus erythematosus: clinical trials and future prospects

    PubMed Central

    Monneaux, Fanny; Muller, Sylviane

    2009-01-01

    The prognosis of patients with systemic lupus erythematosus has greatly improved since treatment regimens combining corticosteroids and immunosuppressive medications have been widely adopted in therapeutic strategies given to these patients. Immune suppression is evidently efficient but also leads to higher susceptibility to infectious and malignant diseases. Toxic effects and sometimes unexpectedly dramatic complications of current therapies have been progressively reported. Identifying novel molecular targets therefore remains an important issue in the treatment of lupus. The aim of this review article is to highlight emerging pharmacological options and new therapeutic avenues for lupus with a particular focus on non-antibody molecular strategies. PMID:19591653

  6. [Spontaneous infrapatellar tendon rupture in a patient with systemic lupus erythematosus].

    PubMed

    Jakobsen, L P; Knudsen, T B; Bloch, T

    2000-09-18

    A case is described of a 33-year old woman with systemic lupus erythematosus (SLE) in longterm treatment with corticosteroids who experienced spontaneous rupture of the left patellar tendon. A comparative study of 28 previously reported cases of SLE patients with spontaneous tendon rupture in weight bearing joints is performed. It is suggested that renal disease may be an etiological factor for spontaneous tendon rupture in patients with systemic lupus erythematosus.

  7. Cognitive and White Matter Tract Differences in MS and Diffuse Neuropsychiatric Systemic Lupus Erythematosus.

    PubMed

    Cesar, B; Dwyer, M G; Shucard, J L; Polak, P; Bergsland, N; Benedict, R H B; Weinstock-Guttman, B; Shucard, D W; Zivadinov, R

    2015-10-01

    Multiple sclerosis and neuropsychiatric systemic lupus erythematosus are autoimmune diseases with similar CNS inflammatory and neurodegenerative characteristics. Our aim was to investigate white matter tract changes and their association with cognitive function in patients with MS and those with neuropsychiatric systemic lupus erythematosus compared with healthy controls by using diffusion tensor imaging. Thirty patients with relapsing-remitting MS and 23 patients with neuropsychiatric systemic lupus erythematosus matched for disease severity and duration and 43 healthy controls were scanned with 3T MR imaging. The DTI was postprocessed, corrected for lesions, and analyzed with tract-based spatial statistics. Cognitive assessment included examination of processing speed; visual, auditory/verbal, and visual-spatial memory; and sustained attention and executive function. Differences were considered significant at P < .05. Tract-based spatial statistics analysis revealed significantly decreased fractional anisotropy and increased mean diffusivity in patients with MS compared with healthy controls, decreased fractional anisotropy in patients with MS compared with those with neuropsychiatric systemic lupus erythematosus, and an increased mean diffusivity in patients with neuropsychiatric systemic lupus erythematosus compared with healthy controls. Patients with MS showed decreased fractional anisotropy throughout central WM pathways, including the corpus callosum and the inferior longitudinal and fronto-occipital fasciculi compared with those with neuropsychiatric systemic lupus erythematosus. Altered cognitive scores in patients with MS were significantly associated with decreased fractional anisotropy and increased mean diffusivity in all examined domains, while in patients with diffuse neuropsychiatric systemic lupus erythematosus, only decreased fractional anisotropy in the superior WM pathways showed significant association with executive function. Patients with MS

  8. Neurologic complications of systemic lupus erythematosus, sjögren syndrome, and rheumatoid arthritis.

    PubMed

    Bhattacharyya, Shamik; Helfgott, Simon M

    2014-09-01

    Neurologic complications are frequent and often morbid in systemic lupus erythematosus, Sjögren syndrome, and rheumatoid arthritis. Although all are systemic inflammatory syndromes, each disease affects the nervous system distinctly, such as peripheral neuropathy in Sjögren syndrome, cerebrovascular disease in lupus, and cervical spine subluxation in rheumatoid arthritis. Some neurologic complications share convergent pathophysiology across diseases, such as neuromyelitis optica spectrum disorders in both Sjögren syndrome and lupus. Ill-defined cognitive complaints are especially common in lupus and Sjögren syndrome. For the majority of the complications, evidence for treatment efficacy is limited and requires further investigation. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  9. Imbalance between Endothelial Damage and Repair: A Gateway to Cardiovascular Disease in Systemic Lupus Erythematosus

    PubMed Central

    2014-01-01

    Atherosclerosis is accelerated in patients with systemic lupus erythematosus (SLE) and it leads to excessive cardiovascular complications in these patients. Despite the improved awareness of cardiovascular disease and advent of clinical diagnostics, the process of atherogenesis in most patients remains clinically silent until symptoms and signs of cardiovascular complications develop. As evidence has demonstrated that vascular damage is already occurring before clinically overt cardiovascular disease develops in lupus patients, intervention at the preclinical stage of atherogenesis would be plausible. Indeed, endothelial dysfunction, one of the earliest steps of atherogenesis, has been demonstrated to occur in lupus patients even when they are naïve for cardiovascular disease. Currently known “endothelium-toxic” factors including type 1 interferon, proinflammatory cytokines, inflammatory cells, immune complexes, costimulatory molecules, neutrophils extracellular traps, lupus-related autoantibodies, oxidative stress, and dyslipidemia, coupled with the aberrant functions of the endothelial progenitor cells (EPC) which are crucial to vascular repair, likely tip the balance towards endothelial dysfunction and propensity to develop cardiovascular disease in lupus patients. In this review, altered physiology of the endothelium, factors leading to perturbed vascular repair contributed by lupus EPC and the impact of proatherogenic factors on the endothelium which potentially lead to atherosclerosis in lupus patients will be discussed. PMID:24790989

  10. Healthcare costs of pregnancy in systemic lupus erythematosus: retrospective observational analysis from a US health claims database.

    PubMed

    Petri, M; Daly, R P; Pushparajah, D S

    2015-01-01

    Systemic lupus erythematosus is a complex autoimmune disease, most frequently affecting women of childbearing age. Women with lupus are at increased risk of pregnancy complications that are exacerbated by active disease. Despite this, their use of medications and hospital resources has not been extensively studied. Retrospective analyses of the Truven Health MarketScan database (2006-2012) aimed to quantify drug and resource utilization in pregnant women with lupus, as well as the incidence of pregnancy complications in these patients. Records of women aged 12-54 were reviewed and both lupus patients and pregnancies identified. Pregnant women with lupus were matched 1:5 with either pregnant women without lupus, or non-pregnant women with lupus. Pregnancies with lupus were associated with increased complications when compared to pregnancies without lupus. During pregnancy, the use of immunosuppressants decreased in pregnant women with lupus, as did rheumatologist visits, while the number of women not treated with any immunosuppressant increased. Pregnant women with lupus showed higher overall treatment costs than controls. However, compared to non-pregnant women with lupus, medication costs actually dropped, possibly due to the withdrawal of medications from these patients or women becoming pregnant while disease activity was low. The large database analyses reported here revealed that pregnancies in women with lupus were associated with a higher risk of complications, higher healthcare costs, and fewer prescribed medications, including immunosuppressants, than the control groups. The increased risk of complications and decreased immunosuppressant use suggest that patients require additional guidance from physicians to give them the best chance of experiencing a safe pregnancy. Indeed, despite the recognized role active lupus plays in increasing pregnancy complications, women with lupus had fewer rheumatology visits during pregnancy, although their visits to their

  11. Different end-points to assess effects in systemic lupus erythematosus patients exposed to pesticide mixtures.

    PubMed

    Simoniello, M F; Contini, L; Benavente, E; Mastandrea, C; Roverano, S; Paira, S

    2017-02-01

    Systemic Lupus Erythematosus (SLE) is an autoimmune disease with high female predominance in reproductive years. It is characterized by a pronounced inflammation and production of a variety of autoantibodies. SLE pathogenesis is influenced by genes, hormones and environmental agents. The aim of this study was assess the possible effect of environmental pesticide mixtures in SLE patients. Oxidative DNA damage was measured using the comet assay modified by enzyme Endo III for detection of oxidized bases (Endo Sites), and oxidative stress by the measurement of the activity of catalase (CAT), superoxide dismutase (SOD) and lipid peroxidation (TBARS). Eighty-nine patients with diagnosis of SLE were included, 46% of them came from areas highly sprayed with pesticides and were compared with patients from urban areas with the same clinical and socio-demographic characteristics (p≥0.155). In order to identify factors that could predict DNA damage and oxidative stress, a binary logistic regression model with independent variables was developed: place of residence (p=0.007) have 75% of positive predictive value while smoking habit (p=0.186) have a 56% negative predictive value. The Odd Ratio (OR) obtained indicate that lupus patients living in rural areas presented 3.52 times more oxidative DNA damage compared to those living in the city. The prospects of applying biomarkers to assess exposure and biological effects, such as DNA damage and oxidative stress in autoimmune diseases, allow improving the characterization of individual risk.

  12. Discrimination and Cumulative Disease Damage Among African American Women With Systemic Lupus Erythematosus.

    PubMed

    Chae, David H; Drenkard, Cristina M; Lewis, Tené T; Lim, S Sam

    2015-10-01

    We examined associations between unfair treatment, attributions of unfair treatment to racial discrimination, and cumulative disease damage among African American women with systemic lupus erythematosus (SLE). We used multivariable regression models to examine SLE damage among 578 African American women in metropolitan Atlanta, Georgia, recruited to the Georgians Organized Against Lupus cohort. When we controlled for demographic, socioeconomic, and health-related covariates, reporting any unfair treatment was associated with greater SLE damage compared with reporting no unfair treatment (b = 0.55; 95% confidence interval = 0.14, 0.97). In general, unfair treatment attributed to nonracial factors was more strongly associated with SLE damage than was unfair treatment attributed to racial discrimination, although the difference was not statistically significant. Unfair treatment may contribute to worse disease outcomes among African American women with SLE. Unfair treatment attributed to nonracial causes may have a more pronounced negative effect on SLE damage. Future research may further examine possible differences in the effect of unfair treatment by attribution.

  13. Sjögren’s syndrome associated with systemic lupus erythematosus

    PubMed Central

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

    2016-01-01

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

  14. Current Perspectives on Arthroplasty in Systemic Lupus Erythematosus: Rates, Outcomes, and Adverse Events.

    PubMed

    Kasturi, Shanthini; Goodman, Susan

    2016-09-01

    Systemic lupus erythematosus (SLE) is a chronic debilitating condition with significant impact on the musculoskeletal system. Arthroplasty may be indicated for damage related to active lupus or its treatment. As therapies for SLE have advanced, morbidity and mortality have declined, while the rate of joint replacement has increased. The age of SLE patients undergoing arthroplasty is increasing, and the indication for surgery is evolving-while avascular necrosis was previously the predominant indication for arthroplasty, osteoarthritis now accounts for a larger proportion of surgeries. Pain and functional outcomes of arthroplasty in SLE patients are comparable to those of the general population with osteoarthritis, but lupus remains an independent risk factor for post-hip arthroplasty complications and mortality. Further research is needed to characterize the impact of lupus disease activity and severity on arthroplasty outcomes.

  15. Plasmapheresis as rescue therapy for systemic lupus erthyematosus-associated diffuse alveolar haemorrhage

    PubMed Central

    Claridge, Simon; Das, Partha; Dorling, Anthony; Robson, Michael

    2011-01-01

    Diffuse alveolar haemorrhage (DAH) is a rare complication of systemic lupus erythematosus (SLE), which can have a potentially lethal outcome. Conventional treatments of the condition include cyclophosphamide and intravenous immunoglobulins. Successful treatment with rituximab and plasmapheresis has also been described. The authors report the case of a woman recently diagnosed with SLE who developed DAH refractory to conventional treatment while on immunosuppressive treatment for class III lupus nephritis whose DAH responded to plasmapheresis. PMID:22698899

  16. Tuberculosis infection causing intestinal perforations in 2 patients with systemic lupus erythematosus.

    PubMed

    González, Luis A; Muñoz, Carolina; Restrepo, Mauricio; Vanegas, Adriana Lucía; Vásquez, Gloria

    2014-08-01

    Patients with systemic lupus erythematosus (SLE) have a higher incidence rate of tuberculosis and a more frequent extrapulmonary involvement than the general population. We present 2 SLE patients who developed gastrointestinal tuberculosis complicated with intestinal perforation, a rare but serious complication that could be confused with lupus-associated intestinal vasculitis. Opportunistic infections such as tuberculosis must be suspected in SLE patients with abdominal symptoms on immunosuppressive therapy because its early recognition could prevent catastrophic complications such as intestinal perforation and subsequent peritonitis.

  17. Paediatric systemic lupus erythematosus: insights from translational research.

    PubMed

    Wright, Tracey B; Punaro, Marilynn

    2017-04-01

    Investigations in paediatric SLE contributed significantly to the discovery of the association of type I IFNs with lupus and underscored the potential application of this knowledge by informing the use of glucocorticoid therapy. Recent, promising research reveals biomarkers that may yield more focused clinical monitoring and assessment of response to treatment. This article reviews unique features of paediatric SLE and details important developments in paediatric lupus research.

  18. Pregnancy Associated with Systemic Lupus Erythematosus: Immune Tolerance in Pregnancy and Its Deficiency in Systemic Lupus Erythematosus--An Immunological Dilemma.

    PubMed

    Gluhovschi, Cristina; Gluhovschi, Gheorghe; Petrica, Ligia; Velciov, Silvia; Gluhovschi, Adrian

    2015-01-01

    Pregnancy is a physiological condition that requires immune tolerance to the product of conception. Systemic lupus erythematosus (SLE) is a disease with well-represented immune mechanisms that disturb immune tolerance. The association of pregnancy with systemic lupus erythematosus creates a particular immune environment in which the immune tolerance specific of pregnancy is required to coexist with alterations of the immune system caused by SLE. The main role is played by T regulatory (Treg) cells, which attempt to regulate and adapt the immune system of the mother to the new conditions of pregnancy. Other components of the immune system also participate to maintain maternal-fetal immune tolerance. If the immune system of pregnant women with SLE is not able to maintain maternal immune tolerance to the fetus, pregnancy complications (miscarriage, fetal hypotrophy, and preterm birth) or maternal complications (preeclampsia or activation of SLE, especially in conditions of lupus nephritis) may occur. In certain situations this can be responsible for neonatal lupus. At the same time, it must be noted that during pregnancy, the immune system is able to achieve immune tolerance while maintaining the anti-infectious immune capacity of the mother. Immunological monitoring of pregnancy during SLE, as well as of the mother's disease, is required. It is important to understand immune tolerance to grafts in transplant pathology.

  19. Employment and disability issues in systemic lupus erythematosus: a review.

    PubMed

    Scofield, Lacie; Reinlib, Leslie; Alarcón, Graciela S; Cooper, Glinda S

    2008-10-15

    To summarize research pertaining to work disability in lupus patients, discuss challenges patients face applying for federal disability assistance in the US, and make recommendations for clinical and health policy research. We searched Medline for articles on work or disability in lupus patients and gathered information from the Social Security Administration and the National Organization of Social Security Claimants' Representatives. We found 12 publications with employment-related data; 6 included analysis of predictors of work status. The prevalence of inability to work or cessation of work was 15-51% in these studies (3-15 years after diagnosis); 20-32% of patients received disability benefits. Lower education level, higher disease activity, higher disease damage, older age, and higher physical job strain were independent predictors of work disability or work cessation in at least 2 studies. Lupus patients may be less successful than patients with other diseases when applying for federal disability assistance, possibly because medical records may not accurately reflect functional limitations. In addition, symptoms contributing to work disability (e.g., fatigue, pain, neurocognitive dysfunction) may be difficult to assess and quantify. Work disability in lupus patients is common. Additional research on risk factors for work disability in lupus patients and on strategies for reducing the impact of these factors on work-related activities is needed. The development of better measures and rating scales for the symptoms responsible for work disability in lupus patients and studies of factors influencing the success of obtaining federal disability benefits would also be useful.

  20. Retinal vasculopathy in patients with systemic lupus erythematosus.

    PubMed

    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

  1. Immunoglobulin light chain allelic inclusion in systemic lupus erythematosus

    PubMed Central

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

    2015-01-01

    The principles of allelic exclusion state that each B cell expresses a single light and heavy chain pair. Here, we show that B cells with both kappa and lambda light chains (Igκ and Igλ) are enriched in some patients with the systemic autoimmune disease systemic lupus erythematosus (SLE), but not in the systemic autoimmune disease control granulomatosis with polyangiitis. Detection of dual Igκ and Igλ expression by flow cytometry could not be abolished by acid washing or by DNAse treatment to remove any bound polyclonal antibody or complexes, and was retained after two days in culture. Both surface and intracytoplasmic dual light chain expression was evident by flow cytometry and confocal microscopy. We observed reduced frequency of rearrangements of the kappa‐deleting element (KDE) in SLE and an inverse correlation between the frequency of KDE rearrangement and the frequency of dual light chain expressing B cells. We propose that dual expression of Igκ and Igλ by a single B cell may occur in some patients with SLE when this may be a consequence of reduced activity of the KDE. PMID:26036683

  2. The pathophysiology of hypertension in systemic lupus erythematosus.

    PubMed

    Ryan, Michael J

    2009-04-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disorder that predominantly affects women during their reproductive years. Although SLE can affect any organ system, the kidneys are prominently involved in the form of immune complex glomerulonephritis. In addition, in women with SLE, risk for the development of cardiovascular disease is dramatically increased. Hypertension is a major risk factor for cardiovascular disease and is highly prevalent in women with SLE. Nevertheless, there has been little exploration of the pathophysiological mechanisms that promote SLE hypertension. This review discusses the role of several mechanisms, with an emphasis on the kidney, in SLE hypertension. These mechanisms include the renin-angiotensin system, endothelin, oxidative stress, sex steroids, metabolic changes, peroxisome proliferator-activated receptor-gamma, and, perhaps most importantly, chronic inflammation and cytokines. Growing evidence suggests a link between chronic inflammation and hypertension. Therefore, elucidation of mechanisms that promote SLE hypertension may be of significant value not only for patients with SLE, but also for a better understanding of the basis for essential hypertension.

  3. Blood lymphocyte ultrastructure and deoxyribonucleic acid content in children with systemic lupus erythematosis.

    PubMed

    Ptasekas, R; Matulis, A; Urmonas, V; Graziene, V; Zukiene, G

    1980-01-01

    Two varieties of peripheral blood lymphocytes have been disclosed in systemic lupus erythematosus (SLE) cases: one showing signs of degradation and nuclear chromatine elimination and the other one manifesting a state of biological activation, possibly of an immunologic nature. This karyostructural lymphocyte heterogeneity in SLE may cause a great scattering of these cells on histograms in respect to their nuclear deoxyribonucleic acid content determined by cytophotometry. On the other hand, the expressiveness of the scattering and the degree of predominance of negative tendency towards proliferation (with a shift to the left from 2 n) may thereby serve as a very objective quantitative indication of nuclear structure degradation and of loss by lymphocytes of chromatine with deoxyribonucleic acid during SLE.

  4. Acute respiratory distress syndrome in a pregnant woman with systemic lupus erythematosus: a case report.

    PubMed

    Chen, Y-J A; Tseng, J-J; Yang, M-J; Tsao, Y-P; Lin, H-Y

    2014-12-01

    When the disease activity of systemic lupus erythematosus (SLE) is controlled appropriately, a pregnant woman who has lupus is able to carry safely to term and deliver a healthy infant. While the physiology of a healthy pregnancy itself influences ventilatory function, acute pulmonary distress may decrease oxygenation and influence both mother and fetus. Though respiratory failure in pregnancy is relatively rare, it remains one of the leading conditions requiring intensive care unit admission in pregnancy and carries a high risk of maternal and fetal morbidity and mortality, not to mention the complexity caused by lupus flare. We report a case of SLE complicated with lupus pneumonitis and followed by acute respiratory distress during pregnancy. Though there is a high risk of maternal and fetal morbidity and mortality, maternal respiratory function improved after cesarean section and treatment of the underlying causes. The newborn had an extremely low birth weight but was well at discharge.

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

    PubMed

    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.

  6. Acute necrotizing retinal vasculitis as onset of systemic lupus erythematosus

    PubMed Central

    Monov, Simeon; Hristova, Ruska; Dacheva, Rositza; Toncheva, Reni; Shumnalieva, Russka; Shoumnalieva-Ivanova, Viara; Monova, Daniela

    2017-01-01

    Abstract Rationale: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by autoantibody production, complement activation, and deposition of immune complexes in tissues and organs. SLE can involve any region of the visual system. Although ocular manifestations are not part of the classification criteria for SLE, they can be observed in up to one-third of the patients with SLE. They are rarely reported at the time of disease onset. Retinal vasculitis is usually associated with active generalized disease. Due to its low frequency, we report a case of acute necrotizing retinal vasculitis as onset of SLE. Patient concerns and diagnosis: A 25-year-old white female was referred to the rheumatology clinic with gradually and rapid deterioration of the vision due to abnormal vessel permeability in the right fundus with edema along the vessels, occlusion of arterial branches in the middle periphery with leakage of the dye in these areas and indentical but less prominent changes with cotton wool spots in the papillomacular area and extensive hemorrhages in the left eye. The onset of malar rash, arthralgias and positive antinuclear, anti-double stranded DNA, anti-ribosomal P and anti-β2 glycoprotein I antibodies with decreased C4 complement levels, as well as the positive lupus-band test confirmed the diagnosis of SLE. Interventions: Aggressive immunomodulating therapy with high-dose methylprednisolone, intravenous immunoglobulin, and cyclophosphamide was used for suppression of the disease activity followed by azathioprine as maintaince therapy. Outcomes: Substantial improvement and partial resorption of the vasculitic changes, including central retinal artery and vein, was achieved prominently in the left eye. The study was conducted in accordance with the Declaration of Helsinki and written informed consent was obtained from the patient. Because of this, there is no need to conduct special ethic review and the ethical approval is not necessary

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

    PubMed

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

    2006-09-01

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

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

    PubMed

    Moori, M; Ghafoori, H; Sariri, R

    2016-03-01

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

  9. The sense of smell in systemic lupus erythematosus.

    PubMed

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

    2009-05-01

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

  10. Genetics and novel aspects of therapies in systemic lupus erythematosus.

    PubMed

    Relle, Manfred; Weinmann-Menke, Julia; Scorletti, Eva; Cavagna, Lorenzo; Schwarting, Andreas

    2015-11-01

    Autoimmune diseases, such as rheumatoid arthritis, multiple sclerosis, autoimmune hepatitis and inflammatory bowel disease, have complex pathogeneses and the factors which cause these disorders are not well understood. But all have in common that they arise from a dysfunction of the immune system, interpreting self components as foreign antigens. Systemic lupus erythematosus (SLE) is one of these complex inflammatory disorders that mainly affects women and can lead to inflammation and severe damage of virtually any tissue and organ. Recently, the application of advanced techniques of genome-wide scanning revealed more genetic information about SLE than previously possible. These case-control or family-based studies have provided evidence that SLE susceptibility is based (with a few exceptions) on an individual accumulation of various risk alleles triggered by environmental factors and also help to explain the discrepancies in SLE susceptibility between different populations or ethnicities. Moreover, during the past years new therapies (autologous stem cell transplantation, B cell depletion) and improved conventional treatment options (corticosteroids, traditional and new immune-suppressants like mycophenolate mofetile) changed the perspective in SLE therapeutic approaches. Thus, this article reviews genetic aspects of this autoimmune disease, summarizes clinical aspects of SLE and provides a general overview of conventional and new therapeutic approaches in SLE. Copyright © 2015. Published by Elsevier B.V.

  11. Outcomes in hospitalized pediatric patients with systemic lupus erythematosus.

    PubMed

    Son, Mary Beth F; Johnson, Victor M; Hersh, Aimee O; Lo, Mindy S; Costenbader, Karen H

    2014-01-01

    Disparities in outcomes among adults with systemic lupus erythematosus (SLE) have been documented. We investigated associations between sociodemographic factors and volume of annual inpatient hospital admissions with hospitalization characteristics and poor outcomes among patients with childhood-onset SLE. By using the Pediatric Health Information System, we analyzed admissions for patients aged 3 to <18 years at index admission with ≥ 1 International Classification of Diseases, Ninth Revision code for SLE from January 2006 to September 2011. Summary statistics and univariable analyses were used to examine demographic characteristics of hospital admissions, readmissions, and lengths of stay. We used multivariable logistic regression analyses, controlling for patient gender, age, race, ethnicity, insurance type, hospital volume, US census region, and severity of illness, to examine risk factors for poor outcomes. A total of 10,724 admissions occurred among 2775 patients over the study period. Hispanic patients had longer lengths of stay, more readmissions, and higher in-hospital mortality. In multivariable analysis, African American race was significantly associated with ICU admission. African American race and Hispanic ethnicity were associated with end-stage renal disease and death. Volume of patients with SLE per hospital and hospital location were not significantly associated with outcomes. In this cohort of hospitalized children with SLE, race and ethnicity were associated with outcomes. Further studies are needed to elucidate the relationship between sociodemographic factors and poor outcomes in patients with childhood-onset SLE.

  12. Competitive endogenous RNA network: potential implication for systemic lupus erythematosus.

    PubMed

    Li, Lian-Ju; Zhao, Wei; Tao, Sha-Sha; Leng, Rui-Xue; Fan, Yin-Guang; Pan, Hai-Feng; Ye, Dong-Qing

    2017-06-01

    Competitive endogenous RNA (ceRNA) hypothesis proposes that RNA transcripts, both coding and non-coding, crosstalk with and coregulate each other using microRNA response elements (MREs). CeRNA analysis tremendously expands functional information of coding and non-coding RNAs. Mounting evidence have shown that various types of RNAs, including pseudogenes, long non-coding RNAs, circular RNAs, and messenger RNAs, can function as ceRNAs in distinct physiological and pathophysiological states. Many validated ceRNA pairs participate in the initiation and progression of cancers, and systemic ceRNA network analyses revealing potential of ceRNAs in diagnosis, therapy, and prognosis of cancers have also been performed. Areas covered: This review concisely introduces ceRNA hypothesis and characteristics of ceRNA regulations. The major sections focus on representative examples of both protein coding and non-coding RNA transcripts acting as ceRNAs. CeRNA prediction programs and databases and implications of ceRNA network in cancers are then discussed. In the end, we surmise potential implications of ceRNA network for SLE. Expert opinion: The role of ceRNA network in systemic lupus erythematosus (SLE) remains undefined. We speculate that dissecting ceRNA network in SLE may help expand our comprehension of roles of transcriptome, particularly non-coding transcripts, and richen our knowledge of pathogenesis, diagnosis, and therapy of SLE.

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

    PubMed

    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%.

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

    PubMed

    Li, William; Frohwein, Thomas; Ong, Kenneth

    2017-08-01

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

  15. Degos-Like Lesions Associated with Systemic Lupus Erythematosus

    PubMed Central

    Jang, Min Soo; Park, Jong Bin; Yang, Myeong Hyeon; Jang, Ji Yun; Kim, Joon Hee; Lee, Kang Hoon; Kim, Geun Tae; Hwangbo, Hyun

    2017-01-01

    Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions. PMID:28392651

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

    PubMed

    Maloney, K C; Ferguson, T S; Stewart, H D; Myers, A A; De Ceulaer, K

    2017-01-01

    Background Epidemiological studies in systemic lupus erythematosus have been reported in the literature in many countries and ethnic groups. Although systemic lupus erythematosus in Jamaica has been described in the past, there has not been a detailed evaluation of systemic lupus erythematosus patients in urban Jamaica, a largely Afro-Caribbean population. The goal of this study was to describe the clinical features, particularly disease activity, damage index and immunological features, of 150 systemic lupus erythematosus subjects. Methods 150 adult patients (≥18 years) followed in rheumatology clinic at a tertiary rheumatology hospital centre (one of two of the major public referral centres in Jamaica) and the private rheumatology offices in urban Jamaica who fulfilled Systemic Lupus International Collaborating Clinics (SLICC) criteria were included. Data were collected by detailed clinical interview and examination and laboratory investigations. Hence demographics, SLICC criteria, immunological profile, systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) and SLICC/American College of Rheumatology (ACR) damage index (SDI) were documented. Results Of the 150 patients, 145 (96.7%) were female and five (3.3%) were male. The mean age at systemic lupus erythematosus onset was 33.2 ± 10.9. Mean disease duration was 11.3 ± 8.6 years. The most prevalent clinical SLICC criteria were musculoskeletal, with 141 (94%) of subjects experiencing arthralgia/arthritis, followed by mucocutaneous manifestations of alopecia 103 (68.7%) and malar rash 46 (30.7%), discoid rash 45 (30%) and photosensitivity 40 (26.7%). Lupus nephritis (biopsy proven) occurred in 42 (28%) subjects and 25 (16.7%) met SLICC diagnostic criteria with only positive antinuclear antibodies/dsDNA antibodies and lupus nephritis on renal biopsy. The most common laboratory SLICC criteria were positive antinuclear antibodies 136 (90.7%) followed by anti-dsDNA antibodies 95 (63.3%) and

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

    PubMed

    van Weelden, Marlon; Queiroz, Lígia B; Lourenço, Daniela M R; Kozu, Katia; Lourenço, Benito; Silva, Clovis A

    2016-01-01

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

  18. TNF-α and TGF-β Counter-Regulate PD-L1 Expression on Monocytes in Systemic Lupus Erythematosus

    PubMed Central

    Ou, Jing-Ni; Wiedeman, Alice E.; Stevens, Anne M.

    2012-01-01

    Monocytes in patients with systemic lupus erythematosus (SLE) are hyperstimulatory for T lymphocytes. We previously found that the normal program for expression of a negative costimulatory molecule programmed death ligand-1 (PD-L1) is defective in SLE patients with active disease. Here, we investigated the mechanism for PD-L1 dysregulation on lupus monocytes. We found that PD-L1 expression on cultured SLE monocytes correlated with TNF-α expression. Exogenous TNF-α restored PD-L1 expression on lupus monocytes. Conversely, TGF-β inversely correlated with PD-L1 in SLE and suppressed expression of PD-L1 on healthy monocytes. Therefore, PD-L1 expression in monocytes is regulated by opposing actions of TNF-α and TGF-β. As PD-L1 functions to fine tune lymphocyte activation, dysregulation of cytokines resulting in reduced expression could lead to loss of peripheral T cell tolerance. PMID:22389764

  19. [Clinical features and adverse pregnancy outcomes of new onset systemic lupus erythematosus during pregnancy].

    PubMed

    Zhan, Z P; Yang, Y; Zhan, Y F; Chen, D Y; Liang, L Q; Yang, X Y

    2016-11-08

    Objective: To investigate the clinical characteristics and adverse pregnancy outcomes in pregnant women with new onset systemic lupus erythematosus (SLE) during pregnancy. Methods: The clinical data of 263 pregnancies with SLE in the First Affiliated Hospital of Zhongshan University from 2001 to 2015 were collected and analyzed retrospectively. Results: Of all the 263 pregnancies, 188 were diagnosed before pregnancy and 75 were newly diagnosed during pregnancy. Among the 75 new onset SLE, 27, 31, 14 and 3 cases were diagnosed during first trimester, second trimester, third trimester and puerperium, respectively. Active lupus was noted in 81.3% of the patients with new onset SLE. The main clinical manifestations of new onset SLE were lupus nephritis (57.3%) and thrombocytopenia (38.7%). SLEPDAI scores as well as the prevalence of lupus nephritis, and thrombocytopenia in patients with new onset SLE was higher than those in the previously diagnosed ones (P<0.05). Among the 75 new onset SLE pregnancies, adverse pregnancy outcomesoccurred in 53 patients, including 34 with pregnancy loss, 15with premature, 8with intrauterine growth restriction, 5with fetal distress and5 with neonatal lupus. Compared with patients withnon-newonset SLE, patients with newonset SLEhad a higher prevalence of adverse pregnancy outcomes (56.4% vs 70.7%, P<0.05), and pregnancy loss (21.8% vs 45.3%, P<0.01) but less live birth (78.2% vs 54.7%, P<0.05). Conclusion: Most of the patients with new onset SLE occurred during the first and second trimester. The most common clinical features of new onset SLE were lupus nephritis and thrombocytopenia. Patients with new onset SLE were more prone to active lupus, lupus nephritis and thrombocytopenia, as well as more adverse pregnancy outcomes and pregnancy loss.

  20. Disease-specific patient reported outcome tools for systemic lupus erythematosus.

    PubMed

    Jolly, Meenakshi; Pickard, A Simon; Block, Joel A; Kumar, Rajan B; Mikolaitis, Rachel A; Wilke, Caitlyn T; Rodby, Roger A; Fogg, Louis; Sequeira, Winston; Utset, Tammy O; Cash, Thomas F; Moldovan, Iona; Katsaros, Emmanuel; Nicassio, Perry; Ishimori, Mariko L; Kosinsky, Mark; Merrill, Joan T; Weisman, Michael H; Wallace, Daniel J

    2012-08-01

    Systemic lupus erythematosus (SLE) can significantly affect both health and non-health-related quality of life (HRQOL and non-HRQOL). However, of the existent published patient-reported outcome (PRO) tools, none were developed from US patients, an ethnically diverse population. Furthermore, these tools do not address men with SLE or assess non-HRQOL issues. Herein, we present the development and validation of the Lupus Patient-Reported Outcome tool (LupusPRO) and discuss its clinical utility and research value compared with other PRO tools currently available for SLE. Beginning with a conceptual framework, items for LupusPRO were generated using feedback from women and men with SLE. The tool underwent iterations based on patient feedback and clinimetric and psychometric analyses. Validity (content, construct, and criterion) and reliability (internal consistency and test-retest) for the 44-item LupusPRO tool are presented. Consistent with the conceptual framework, items were identified that were related to HRQOL and non-HRQOL constructs. HRQOL domains included (1) lupus symptoms; (2) physical health (physical function, role physical); (3) pain-vitality; (4) emotional health (emotional function and role emotional); (5) body image; (6) cognition; (7) procreation; and (8) lupus medications. Non-HRQOL domains were (1) available social support and coping; (2) desires-goals; and (3) satisfaction with medical care. Internal consistency reliability (0.68-0.94), test-retest reliability (0.55-0.92), content, construct (r > 0.50 with SF-36), and criterion (r > -0.35 with disease activity) validity were fair to good. LupusPRO is a valid and reliable disease-targeted patient-reported health outcome tool that is generalizable to SLE patients in the United States of varied ethnic backgrounds and either gender. Copyright © 2012. Published by Elsevier Inc.

  1. Epidemiology of Systemic Lupus Erythematosus and Cutaneous Lupus in a Predominantly White Population in the United States

    PubMed Central

    Jarukitsopa, Sudumpai; Hoganson, Deana D; Crowson, Cynthia S; Sokumbi, Olayemi; Davis, Mark D; Michet, Clement J; Matteson, Eric L; Maradit –Kremers, Hilal; Chowdhary, Vaidehi R

    2014-01-01

    Objective Epidemiologic studies comparing the incidence and prevalence of systemic lupus erythematosus (SLE) and isolated cutaneous lupus erythematosus (CLE) are few. Olmsted County, Minnesota provides a unique setting for such a study owing to resources of the Rochester Epidemiology Project. We sought to describe and compare the incidence and prevalence of SLE and CLE from 1993 to 2005. Methods SLE cases were identified from review of medical records and fulfilled the 1982 ACR classification criteria. CLE cases included patients with classic discoid LE (CDLE), subacute cutaneous LE (SCLE), lupus panniculitis and bullous LE. Age-and sex-adjusted incidence and prevalence were standardized to 2000 US white population. Results The age- and sex-adjusted incidence of SLE (2.9 per 100,000; 95% CI 2.0, 3.7) was similar to that of CLE (4.2 per 100,000; 95% CI 3.1, 5.2, p= 0.10). However, incidence of CLE was three times higher than SLE in males (2.4 versus 0.8 per 100,000, p=0.009). The age- and sex-adjusted prevalence of CLE on January 1, 2006 was higher than that of SLE (70.4 versus 30.5 per 100,000; p<0.001). The prevalence of CLE and SLE in women were similar but the CLE prevalence was higher in men than in women (56.9 versus 1.6 per 100,000, p<0.001). The incidence of CLE rose steadily with age and peaked at 60-69 years. Conclusion The incidences of CLE and SLE are similar but CLE is more common than SLE in males and in older adults. These findings may reflect differences in genetic or environmental etiology of CLE. PMID:25369985

  2. MIF: Implications in the Pathoetiology of Systemic Lupus Erythematosus.

    PubMed

    Lang, Tali; Foote, Andrew; Lee, Jacinta P W; Morand, Eric F; Harris, James

    2015-01-01

    Macrophage migration Inhibitory factor (MIF) was one of the earliest pro-inflammatory cytokines to be identified. Increasing interest in this cytokine in recent decades has followed the cloning of human MIF and the generation of Mif(-/-) mice. Deepening understanding of signaling pathways utilized by MIF and putative receptor mechanisms have followed. MIF is distinct from all other cytokines by virtue of its unique induction by and counter regulation of glucocorticoids (GCs). MIF is further differentiated from other cytokines by its structural homology to specific tautomerase and isomerase enzymes and correlative in vitro enzymatic functions. The role of MIF in immune and inflammatory states, including a range of human autoimmune diseases, is now well established, as are the relationships between MIF polymorphisms and a number of inflammatory diseases. Here, we review the known pleiotropic activities of MIF, in addition to novel functions of MIF in processes including autophagy and autophagic cell death. In addition, recent developments in the understanding of the role of MIF in systemic lupus erythematosus (SLE) are reviewed. Finally, we discuss the potential application of anti-MIF strategies to treat human diseases such as SLE, which will require a comprehensive understanding of the unique and complex activities of this ubiquitously expressed cytokine.

  3. Systemic lupus-erythematosus: deoxyribonuclease 1 in necrotic chromatin disposal.

    PubMed

    Napirei, Markus; Gültekin, Aykut; Kloeckl, Thomas; Möröy, Tarik; Frostegård, Johan; Mannherz, Hans Georg

    2006-03-01

    Systemic lupus-erythematosus is an auto-immune-disease characterized by pathogenic anti-nuclear auto-antibodies. These form immune-complexes that after deposition at basal membranes at various locations initiate inflammatory reactions. There is a clear genetic and gender predisposition (females are affected 10 times more frequently), but also infectious agents and further environmental factors have been shown to be causative for the initiation of the disease. It has been suggested that the auto-antibodies arise after release and/or inefficient removal of nuclear components during cell death (defective cellular "waste disposal" theory). So far, increased apoptotic cell death has been made responsible, but recent data suggest that defective cellular waste disposal during/after necrosis may also lead to the release and prolonged exposure of nuclear components. Here, we concentrate on chromatin disposal during necrosis and the involvement of Deoxyribonuclease 1 in this process with respect to its possible role in the prevention of anti-nuclear auto-immunity.

  4. Patient-Reported Outcomes in Systemic Lupus Erythematosus

    PubMed Central

    Ramsey-Goldman, Rosalind

    2016-01-01

    Successful management of complex conditions such as systemic lupus erythematosus, SLE, and comorbid conditions benefit significantly from patient-reported outcomes (PRO) instruments that validly and precisely measure relevant aspects of health status (e.g., symptoms) and health related quality of life. Measuring health related quality of life and other aspects of health status (e.g., symptoms, functioning) with PROs provides SLE patients with an opportunity to participate in their treatment and to facilitate better communication with the multi-disciplinary team of health professionals involved in their care. Health outcomes research has produced a number of well-validated instruments that can be used across diseases whereas some have been specifically developed for SLE. The use of either a generic and SLE-specific PRO depends on the particular needs of a variety of clinical applications including population monitoring, treatment decision-making in clinical practice, clinical trials research, and for evaluating and comparing the effect of therapies across conditions, therapies, trials and patients in clinical research studies. PMID:27133488

  5. Thyroid nodules in Hispanic patients with systemic lupus erythematosus.

    PubMed

    Quintanilla-Flores, D L; Hernández-Coria, M I; Elizondo-Riojas, G; Galarza-Delgado, D A; González-González, J; Tamez-Pérez, H E

    2013-12-01

    A thyroid nodule (TN) is a discrete lesion in the thyroid gland radiologically distinct from the adjacent parenchyma, with a prevalence variable depending on the diagnostic method used and the study population. Thyroid disorders have been identified in more than 50% of patients with systemic lupus erythematosus (SLE); however, the prevalence of TN has not been frequently studied. We identified a prevalence of 27% TN in 55 SLE patients > 16 years of age. One-third of TN were >1 cm with radiological features of malignancy. The mean age of patients with TN was 39 ± 11 years, 93% women, and SLE duration 10 ± 6 years. Among patients, we reported family history of cancer in three cases (20%), thyroid disease in one (7%), and autoimmune disease in six (40%). Regarding treatment, 50% of patients with TN were treated with azathioprine vs. 23% of patients without TN (p = 0.02), with an OR of 3.94 (95% CI 1.12-13.84, p = 0.03). As a conclusion a high prevalence of TN in SLE patients was found. Prevalence of TN correlated only with history of azathioprine use. We don't know the long-term implications of our findings; however, a functional and morphological evaluation of the thyroid gland is warranted in all patients with SLE.

  6. Transancestral mapping and genetic load in systemic lupus erythematosus

    PubMed Central

    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.; Almlöf, Jonas Carlsson; Acevedo-Vásquez, Eduardo M.; Alarcón, 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 Rantapää; D’Alfonso, Sandra; Da Silva, Berta Martins; de la Rúa Figueroa, Iñigo; Doria, Andrea; Edberg, Jeffrey C.; Endreffy, Emőke; Esquivel-Valerio, Jorge A.; Fortin, Paul R.; Freedman, Barry I.; Frostegård, Johan; García, Mercedes A.; de la Torre, Ignacio García; 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.; Kovács, László; Laustrup, Helle; Lauwerys, Bernard R.; Li, Quan-Zhen; Maradiaga-Ceceña, Marco A.; Martín, Javier; McCune, Joseph M.; McWilliams, David R.; Merrill, Joan T.; Miranda, Pedro; Moctezuma, José 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, José M.; Aguilar-Salinas, Carlos A.; Scherbarth, Hugo R.; Scorza, Raffaella; Seldin, Michael F.; Sjöwall, Christopher; Svenungsson, Elisabet; Thompson, Susan D.; Toloza, Sergio M. A.; Truedsson, Lennart; Tusié-Luna, Teresa; Vasconcelos, Carlos; Vilá, Luis M.; Wallace, Daniel J.; Weisman, Michael H.; Wither, Joan E.; Bhangale, Tushar; Oksenberg, Jorge R.; Rioux, John D.; Gregersen, Peter K.; Syvänen, Ann-Christine; Rönnblom, Lars; Criswell, Lindsey A.; Jacob, Chaim O.; Sivils, Kathy L.; Tsao, Betty P.; Schanberg, Laura E.; Behrens, Timothy W.; Silverman, Earl D.; Alarcón-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 distinct non-HLA regions in EA, 9 in AA and 16 in HA (∼50% of these regions have multiple independent associations); these include 24 novel SLE regions (P<5 × 10−8), refined association signals in established regions, extended associations to additional ancestries, and a disentangled complex HLA multigenic effect. The risk allele count (genetic load) exhibits an accelerating pattern of SLE risk, leading us to posit a cumulative hit hypothesis for autoimmune disease. Comparing results across the three ancestries identifies both ancestry-dependent and ancestry-independent contributions to SLE risk. Our results are consistent with the unique and complex histories of the populations sampled, and collectively help clarify the genetic architecture and ethnic disparities in SLE. PMID:28714469

  7. Regional brain metabolism in a murine systemic lupus erythematosus model.

    PubMed

    Vo, An; Volpe, Bruce T; Tang, Chris C; Schiffer, Wynne K; Kowal, Czeslawa; Huerta, Patricio T; Uluğ, Aziz M; Dewey, Stephen L; Eidelberg, David; Diamond, Betty

    2014-08-01

    Systemic lupus erythematosus (SLE) is characterized by multiorgan inflammation, neuropsychiatric disorders (NPSLE), and anti-nuclear antibodies. We previously identified a subset of anti-DNA antibodies (DNRAb) cross-reactive with the N-methyl-D-aspartate receptor, present in 30% to 40% of patients, able to enhance excitatory post-synaptic potentials and trigger neuronal apoptosis. DNRAb+ mice exhibit memory impairment or altered fear response, depending on whether the antibody penetrates the hippocampus or amygdala. Here, we used 18F-fluorodeoxyglucose (FDG) microPET to plot changes in brain metabolism after regional blood-brain barrier (BBB) breach. In DNRAb+ mice, metabolism declined at the site of BBB breach in the first 2 weeks and increased over the next 2 weeks. In contrast, DNRAb- mice exhibited metabolic increases in these regions over the 4 weeks after the insult. Memory impairment was present in DNRAb+ animals with hippocampal BBB breach and altered fear conditioning in DNRAb+ mice with amygdala BBB breach. In DNRAb+ mice, we observed an inverse relationship between neuron number and regional metabolism, while a positive correlation was observed in DNRAb- mice. These findings suggest that local metabolic alterations in this model take place through different mechanisms with distinct time courses, with important implications for the interpretation of imaging data in SLE subjects.

  8. Tolerogenic probiotics: potential immunoregulators in Systemic Lupus Erythematosus.

    PubMed

    Esmaeili, Seyed-Alireza; Mahmoudi, Mahmoud; Momtazi, Amir Abbas; Sahebkar, Amirhossein; Doulabi, Hassan; Rastin, Maryam

    2017-08-01

    Probiotics are commensal or nonpathogenic microbes that colonize the gastrointestinal tract and confer beneficial effects on the host through several mechanisms such as competitive exclusion, anti-bacterial effects, and modulation of immune responses. There is growing evidence supporting the immunomodulatory ability of some probiotics. Several experimental and clinical studies have been shown beneficial effect of some probiotic bacteria, particularly Lactobacillus and Bifidobacteria strains, on inflammatory and autoimmune diseases. Systemic lupus erythematosus (SLE) is an autoimmune disease that is mainly characterized by immune intolerance towards self-antigens. Some immunomodulatory probiotics have been found to regulate immune responses via tolerogenic mechanisms. Dendritic and T regulatory (Treg) cells, IL-6, IFN-γ, IL-17, and IL-23 can be considered as the most determinant dysregulated mediators in tolerogenic status. As demonstrated by documented experimental and clinical trials on inflammatory and autoimmune diseases, a number of probiotic bacterial strains can restore tolerance in host through modification of such dysregulated mediators. Since there are limited reports regarding to impact of probiotic supplementation in SLE patients, the preset review was aimed to suggest a number of probiotics bacteria, mainly from Bifidobacteria and Lactobacillus strains that are able to ameliorate immune responses. The aim was followed through literature survey on immunoregulatory probiotics that can restore tolerance and also modulate the important dysregulated pro/anti-inflammatory cytokines contributing to the pathogenesis of SLE. © 2016 Wiley Periodicals, Inc.

  9. Management of cardiovascular complications in systemic lupus erythematosus

    PubMed Central

    Skamra, Carly; Ramsey-Goldman, Rosalind

    2010-01-01

    Cardiovascular disease (CVD) is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Patients with SLE have an excess risk compared with the general population; this is particularly pronounced in younger women with SLE who have an excess risk of over 50-fold compared with population controls. There is a higher prevalence of subclinical atherosclerosis in patients with SLE compared with controls, as demonstrated by a variety of imaging modalities discussed in this review. The causality of the excess risk of CVD and subclinical atherosclerosis is multifactorial in patients with SLE. While traditional risk factors play a role, after controlling for the traditional Framingham risk factors, the excess risk is still 7.5-fold greater than the general population. This review will also cover novel cardiovascular risk factors and some SLE-specific variables that contribute to CVD risk. This review discusses the risk factor modification and the evidence available for treatment of these risk factors in SLE. There have not yet been any published randomized, controlled trials in patients with SLE with respect to CVD risk factor modifications. Thus, the treatment and management recommendations are based largely on published guidelines for other populations at high risk for CVD. PMID:20305727

  10. [Assessing the cardiovascular risk in patients with systemic lupus erythematosus].

    PubMed

    Arnaud, L; Mathian, A; Bruckert, E; Amoura, Z

    2014-11-01

    Multiple factors contribute to the increased cardiovascular risk observed in patients with systemic lupus erythematosus (SLE). Among these are the so-called classical cardiovascular risk factors, the disease itself through its activity, treatments, and complications, and the thrombotic risk due to antiphospholipid antibodies (aPL). Observational studies suggest that most classical cardiovascular risk factors are observed more frequently in SLE patients than in the general population, and that these are insufficient to explain the increased cardiovascular risk observed in most studies. Given this high risk, adequate management of cardiovascular risk factors should be recommended in SLE patients. Paradoxically, the benefit due to the anti-inflammatory properties of treatments such as corticosteroids may exceed, in certain cases, their pro-atherogenic effect. Importantly, the tools that were developed for the estimation of cardiovascular risk at the individual level among the general population cannot be used reliably in SLE patients, as these tools appear to underestimate the true cardiovascular risk. The adequate indications and targets of cardiovascular treatments are therefore not fully known in SLE. A better understanding of the determinants of the cardiovascular risk in SLE will allow the identification and more tailored management of these high-risk patients.

  11. Periodontal findings in systemic lupus erythematosus patients and healthy controls

    PubMed Central

    Al-Mutairi, Khalid D.; Al-Zahrani, Mohammad S.; Bahlas, Sami M.; Kayal, Rayyan A.; Zawawi, Khalid H.

    2015-01-01

    Objectives: To compare periodontal findings in systemic lupus erythematosus (SLE) patients and healthy controls, and to determine, whether there is a correlation between periodontal parameters and SLE biomarkers. Methods: This cross-sectional study was conducted in the Faculty of Dentistry, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia between November 2012 and February 2014. Twenty-five participants diagnosed with SLE and 50 healthy controls were selected. Periodontal assessment consisted of clinical attachment level (CAL), probing depth (PD), bleeding on probing, and plaque scores. For the SLE group, several laboratory tests were obtained, such as, white blood cell count, hemoglobin level, platelet count, anti-nuclear antibody, anti-double-stranded DNA antibody, calcium level, and vitamin D. Results: Periodontal findings in SLE patients and controls were not significantly different. The SLE patients who had no flare-ups for more than a year showed significant bleeding on probing and deeper PD compared with those who had flare-ups less than a year before starting the study. The SLE patients with arthritis symptoms showed more CAL than those without arthritis. In the SLE patients, no significant correlation was found between their periodontal findings and SLE biomarkers. Conclusion: Periodontal health was not different between SLE patients and healthy controls. In SLE patients however, flare-ups and presence of arthritis had a significant relation with periodontal health. PMID:25828284

  12. Systemic lupus erythematosus (SLE) at the Kenyatta National Hospital.

    PubMed

    Ekwom, Paul Etau

    2013-08-01

    Systemic lupus erythematosus (SLE) is a complex disease with varied clinical presentation and autoantibody production. It has previously been reported as rare in Black Africans. We established a Rheumatology clinic at the Kenyatta National Hospital in April 2010, and a 1 year audit of this clinic was carried out in September 2011. This is a report of this audit of patients who met the American College of Rheumatology (ACR) criteria for SLE. Thirteen patients met the ACR criteria; their mean age was 34 years, and they were all female. The commonest manifestations were malar rash and arthritis in 69.2 %. Antinuclear antibody was present in 79.6 %, and anti-dsDNA was present in 38.5 %. None of them had human immunodeficiency virus infection; 30 % had other comorbidities (hypertension, diabetes, and renal failure). Thirty percent also had an infection during this period. All these 13 were on prednisolone and 92 % of them were on hydroxychloroquine. There was no reported death during the study period. This confirms the presence of SLE in patients in Kenya who meet the ACR criteria.

  13. [Crohn's disease with the onset resembling systemic lupus erythematosus].

    PubMed

    Shimizu, T; Nishinarita, S; Son, K; Tomita, Y; Yoshihiro; Matsukawa; Kitamura, N; Horie, T; Baba, M; Hiranuma, M

    1999-06-01

    We described a 37-year-old man with Crohn's disease (CD) resembling systemic lupus erythematosus (SLE) at his disease onset. He was admitted to the municiple Akiru Hospital in October 1986 by fever, aphtous oral ulcerations, sore throat and polyarthralgia. Hematologic examination showed leukocytopenia, lymphocytopenia, positive tests for antinuclear antibody, anti-DNA antibody and LE cell phenomenon. He has had episodes of convulsion and conciousness loss of unknown etiology when he was 17 years old. The diagnosis of SLE was made, and oral medication of prednisolone was started. Several weeks later, most of symptoms and autoantibodies disappeared, although the oral aphtous ulcerations and leukocytopenia remained. In May 1987, he admitted to the other hospital because of bloody vomiting. Endoscopic examination showed the esophagial ulceration, and histology of biopsied-specimen was nonspecific esophagitis. The combination of prednisolone and oral cyclophosphamide or methotrexate was employed thereafter. However, the leukocytopenia, oral aphtous ulceration and esophagial ulceration continued in spite of these treatments. All the immunosuppressive treatment was stopped at March 1992. In October 1995, he admitted to our hospital because of body weight loss and continuous diarrhea with occasional bloody stool. Barium enema and endoscopic examination of the colon revealed the findings compatible with CD. The patient responded favorably to methylprednisolone pulse therapy followed by oral sulphasalazine. This case indicated that cases with inflammatory bowel diseases like CD could show similar clinical signs and symptoms to SLE, and in some cases of CD might satisfied the classification of criteria for SLE.

  14. Cranial and lumbosacral hypertrophic pachymeningitis associated with systemic lupus erythematosus

    PubMed Central

    Han, Fei; Zhong, Ding-Rong; Hao, Hong-Lin; Kong, Wei-Ze; Zhu, Yi-Cheng; Guan, Hong-Zhi; Cui, Li-Ying

    2016-01-01

    Abstract Background: Hypertrophic pachymeningitis (HP) is a chronic disease characterized by inflammatory hypertrophy and fibrosis of dura mater. It can be divided into cranial and spinal forms depending on the location of the lesion. HP involving 2 separate sites simultaneously is quite uncommon. Case summary: This study presents a case of a 49-year-old woman with pathologically confirmed cranial and lumbosacral hypertrophic pachymeningitis associated with systemic lupus erythematosus (SLE), which is a rare etiology of HP. She experienced persistent numbness and pain of the left lower limb, followed by headache and seizures. In laboratory tests, levels of erythrocyte sedimentation rate and C-reactive protein were elevated, and antinuclear antibodies and anti–double-strand deoxyribonucleic acid (DNA) antibodies were detected. Magnetic resonance imaging revealed dural thickening with homogenous gadolinium enhancement both at lumbosacral level and over cerebral convexities. Histology suggested chronic inflammation in spinal dura mater with extensive fibrosis, dense lymphoplasmacytic infiltrate, and focal vasculitis. Treatment with corticosteroids and cyclophosphamide was started with significant clinical and radiological improvement. Conclusion: HP is etiologically heterogeneous. Despite its rarity, SLE should be considered in the differential diagnosis of HP. Early recognition and therapy may provide an optimal outcome. PMID:27684799

  15. Systemic lupus erythematosus and pemphigus vulgaris: association or coincidence.

    PubMed

    Calebotta, A; Cirocco, A; Giansante, E; Reyes, O

    2004-01-01

    Few cases have been published relating systemic lupus erythematosus (SLE) and pemphigus vulgaris (PV). We describe a patient with this association. A 35-year old woman who started to develop persistent pain and morning stiffness of proximal inter and metacarpo-phalangeal joints. During the following year, the patient recalled the onset of blisters on both legs, face, arms and thorax, as well as erosions appearing on oral mucous membranes. We observed generalized multiple erosions on her trunk and legs, flaccid bullae located on her right thigh and multiple erosions on oral mucous membranes. A skin biopsy reported PV Direct immunofluorescence on the perilesional skin specimen, showed beehive intercellular IgG deposits in the epidermis (+++), suggesting PV; granular discontinuous IgM and C3 deposits in the dermal-epidermal union (+++), suggesting SLE. Direct immunofluorescence of the healthy unexposed skin specimen, reported granular discontinuous IgG deposits in the dermal-epidermal union and beehive intercellular IgG deposits in the lower levels of the epidermis (+++); granular continuous IgM deposits in the dermal-epidermal union (+++). The results of rheumatic studies were obtained as follows: ANA :3 +, Anti-DNA, Anti-Sm, Anti-Ro and Anti-La :4 + . The definite diagnosis was PVand SLE. Treatment with 50 mg of prednisone daily with good evolution.

  16. Complement activation by antibodies to Sm in systemic lupus erythematosus.

    PubMed

    Sabharwal, U K; Fong, S; Hoch, S; Cook, R D; Vaughan, J H; Curd, J G

    1983-02-01

    An enzyme linked immunosorbent assay was developed to quantitate antibodies to Sm (anti-Sm) and to measure complement activation by anti-Sm in vitro. Anti-Sm in plasma of patients with systemic lupus erythematosus (SLE) were bound to purified Sm bound to polyvinyl chloride microtitre plates and assayed for bound IgG or IgM using enzyme linked anti-gamma or anti-mu. The activation of C4 by anti-Sm was measured by adding diluted normal human serum (complement) to the wells and quantitating the amount of C4 bound to the well surface using (Fab')2 goat anti-C4 followed by enzyme linked rabbit anti-goat IgG. The plasmas of 12 of 36 patients with SLE contained anti-Sm and all 12 activated complement (complement activating anti-Sm). Twenty-eight plasmas containing anti-Sm from 12 patients with SLE were studied. Ten of the 12 patients had anti-Sm of the IgG class whereas two had anti-Sm of both IgG and IgM classes. The amount of C4 activating anti-Sm correlated significantly with the in vivo activation of C4 measured by rocket immunoelectrophoresis for C4d and C4, suggesting that complement activation by anti-Sm is important in vivo.

  17. The Importance of an Early Diagnosis in Systemic Lupus Erythematosus.

    PubMed

    Sebastiani, Gian D; Prevete, Immacolata; Iuliano, Annamaria; Minisola, Giovanni

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a high degree of variability at onset, making it difficult to reach a correct and prompt diagnosis. To present the difficulties faced by the clinician in making a SLE diagnosis, based on the characteristics at study entry of an Italian cohort of SLE patients with recent onset as compared to two similar cohorts. Beginning on 1 January 2012 all patients with a diagnosis of SLE (1997 ACR criteria) and disease duration of less than 12 months were consecutively enrolled in a multicenter prospective study. Information on clinical and serological characteristics was collected at study entry and every 6 months thereafter. Our cohort consisted of 122 patients, of whom 103 were females. Among the manifestations included in the 1997 American College of Rheumatology (ACR) criteria, cutaneous, articular and hematologic symptoms were the most prevalent symptoms at study entry. Data from the literature confirm that the diagnosis of SLE is challenging, and that SLE is a severe disease even at onset when a prompt diagnosis is necessary for initiating the appropriate therapy.

  18. MEFV gene variations in patients with systemic lupus erythematosus.

    PubMed

    Erer, Burak; Cosan, Fulya; Oku, Basar; Ustek, Duran; Inanc, Murat; Aral, Orhan; Gul, Ahmet

    2014-01-01

    The aim of this study was to investigate the frequency of familial Mediterranean fever (FMF)-associated MEFV gene variations in patients with systemic lupus erythematosus (SLE). The study group comprised 190 SLE patients and 101 healthy controls of Turkish origin with no clinical features of FMF. All individuals were genotyped for the four most common MEFV gene variations (M694V, M680I, V726A and E148Q) by PCR-restriction fragment length polymorphism analysis. The frequency of carrying any of the four MEFV gene variations under study was 15 % in patients with SLE and 10 % in the healthy controls (p = 0.23). After the exclusion of the less penetrant E148Q variation, re-analysis for the three penetrant mutations revealed a significant association between exon 10 variations and pericarditis [p = 0.038, odds ratio (OR) 3.5, 95 % confidence interval (CI) 1.0-12.1], and pleural effusion (p = 0.043, OR 5.2, 95 % CI 0.8-30.9). No significant association was detected between the MEFV gene variations and a higher acute phase response. The MEFV gene variations analyzed in our study do not seem to increase the overall susceptibility to SLE and do not have any strong association with its clinical manifestations. The possibility of a modest effect of penetrant exon 10 MEFV variants on the development of serosal effusions needs to be explored in a larger series of patients.

  19. Deforming arthropathy of the hands in systemic lupus erythematosus.

    PubMed

    Alarcón-Segovia, D; Abud-Mendoza, C; Diaz-Jouanen, E; Iglesias, A; De los Reyes, V; Hernández-Ortiz, J

    1988-01-01

    Forty-one of 858 patients with systemic lupus erythematosus (SLE) developed clinical deformity of their hands. This deformity was clinically and radiologically different from that found in 40 patients with classical or definite rheumatoid arthritis (RA), and tended to appear early in the course of disease. Characteristics of this arthropathy included nonerosive carpal collapse; exceptional erosion of the styloid processes; Z deformity of the thumb; nonerosive ulnar deviation and subluxation of MCP joints; parametacarpophalangeal joint hook formation; scant and asymmetric joint erosions; and swan neck deformity of the fingers. Most of these changes seemed to be due to involvement of the ligaments rather than to the destructive effect of synovitis. Patients with SLE with deforming arthropathy had a higher frequency of rheumatoid factor positivity, sicca symptoms and antibodies to native DNA, whereas they had lower incidence of facial rash and photosensitivity than did those without. Other manifestations did not differ. We propose that most patients with SLE with deforming arthropathy belong to a subset of SLE rather than representing the coexistence of SLE and RA.

  20. Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Prevalence and Predictors.

    PubMed

    Pérez-Peñate, Gregorio Miguel; Rúa-Figueroa, Iñigo; Juliá-Serdá, Gabriel; León-Marrero, Fernándo; García-Quintana, Antonio; Ortega-Trujillo, José Ramón; Erausquin-Arruabarrena, Celia; Rodríguez-Lozano, Carlos; Cabrera-Navarro, Pedro; Ojeda-Betancor, Nazario; Gómez-Sánchez, Miguel Ángel

    2016-02-01

    Pulmonary arterial hypertension (PAH) prevalence has been reported to be between 0.5% and 17% in systemic lupus erythematosus (SLE). This study assessed PAH prevalence and predictors in an SLE cohort. The Borg dyspnea scale, DLCO, N-terminal pro-brain natriuretic peptide (NT-proBNP), and Doppler echocardiographic (DE) were performed. An echocardiographic Doppler exercise test was conducted in selected patients. When DE systolic pulmonary arterial pressure was ≥ 45 mmHg or increased during exercise > 20 mmHg, a right heart catheterization was performed. Hemodynamic during exercise was measured if rest mean pulmonary arterial pressure was < 25 mmHg. Of the 203 patients with SLE, 152 were included. The mean age was 44.9 ± 12.3 years, and 94% were women. Three patients had known PAH. The algorithm diagnosed 1 patient with chronic thromboembolic pulmonary hypertension and 5 with exercise-induced pulmonary artery pressure increase (4 with occult left diastolic dysfunction). These patients had significantly more dyspnea, higher NT-proBNP, and lower DLCO. These data confirm the low prevalence of PAH in SLE. In our cohort, occult left ventricular diastolic dysfunction was a frequent diagnosis of unexplained dyspnea. Dyspnea, DLCO, and NT-proBNP could be predictors of pulmonary hypertension in patients with SLE.

  1. Cardiopulmonary correlates of cognition in systemic lupus erythematosus.

    PubMed

    Kozora, E; Zell, J; Swigris, J; Strand, M; Duggan, E C; Burleson, A; Make, B

    2015-02-01

    We aimed to evaluate the relationship between cognitive dysfunction and lung function, exercise endurance, and self-reported activity levels in patients with systemic lupus erythematosus (SLE). Cognitive dysfunction is present in 20%-60% of SLE patients. No studies to date have investigated the inter-relationships between cardiopulmonary factors and cognition in this population. Thirty-seven SLE patients without overt neuropsychiatric histories and 16 healthy controls completed neuropsychological testing, measures of lung function, exercise capacity (distance walked during a timed walk test,(1) maximal oxygen uptake(2)), and exercise questionnaires. Thirty-two percent of SLE patients demonstrated cognitive impairment. Cognitive impairment was correlated with Six-Minute Walk Distance (6MWD) (r = 0.37, p = 0.02) and certain measures of lung function. Also, in SLE patients, self-reported physical activity was correlated with 6MWD (p = 0.012), but none of the more complex measures of physical activity (VO2max). Patients with mild SLE disease activity have cognitive dysfunction associated with certain objective markers of exercise capacity and activity levels. The lack of associations between self-report activity and VO2max suggests the possibility that multiple factors mediate the relationships between perceived and actual physical ability. Additional studies are needed to better understand the relationship between cognition and physical activity in patients with SLE. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  2. The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus

    PubMed Central

    Ünlü, Ozan; Zuily, Stephane; Erkan, Doruk

    2016-01-01

    Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage. The use of low-dose aspirin (LDA) is controversial for primary thrombosis and pregnancy morbidity prevention because of the lack of strong prospective controlled data. Similarly, the use of anticoagulation is controversial for patients with an aPL-related nephropathy. Until further studies are available, physicians should discuss the risk/benefits of LDA or anticoagulation as well as the available literature with patients. PMID:27708976

  3. A Quality Indicator Set for Systemic Lupus Erythematosus

    PubMed Central

    Yazdany, Jinoos; Panopalis, Pantelis; Gillis, Joann Zell; Schmajuk, Gabriela; MacLean, Catherine; Wofsy, David; Yelin, Edward

    2009-01-01

    Objective To systematically develop a quality indicator (QI) set for systemic lupus erythematosus (SLE). Methods We used a validated process that combined available scientific evidence and expert consensus to develop a QI set for SLE. First, we extracted 20 candidate indicators from a systematic literature review of clinical practice guidelines pertaining to SLE. An advisory panel revised and augmented these candidate indicators, and through two rounds of voting, arrived at 25 QIs. These QIs advanced to the next phase of the project, in which we employed a modification of the RAND/UCLA Appropriateness Method. A systematic review of the literature was performed for each QI, linking the proposed process of care to potential improved health outcomes. After reviewing this scientific evidence, a second interdisciplinary expert panel convened to discuss the evidence and provide final ratings on the validity and feasibility of each QI. Results The final expert panel rated 20 QIs as both valid and feasible. Areas covered include diagnosis, general preventive strategies (e.g. vaccinations, sun avoidance counseling, screening for cardiovascular disease), osteoporosis prevention and treatment, drug toxicity monitoring, renal disease, and reproductive health. Conclusions We employed a rigorous multi-step approach with systematic literature reviews and two expert panels to develop QIs for SLE. This new set of indicators provides an opportunity to assess health care quality in SLE, and represents an initial step toward the important goal of improving care in this patient population. PMID:19248127

  4. Transancestral mapping and genetic load in systemic lupus erythematosus.

    PubMed

    Langefeld, Carl D; Ainsworth, Hannah C; Cunninghame Graham, Deborah S; Kelly, Jennifer A; Comeau, Mary E; Marion, Miranda C; Howard, Timothy D; Ramos, Paula S; Croker, Jennifer A; Morris, David L; Sandling, Johanna K; Almlöf, Jonas Carlsson; Acevedo-Vásquez, Eduardo M; Alarcón, 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 Rantapää; D'Alfonso, Sandra; Da Silva, Berta Martins; de la Rúa Figueroa, Iñigo; Doria, Andrea; Edberg, Jeffrey C; Endreffy, Emőke; Esquivel-Valerio, Jorge A; Fortin, Paul R; Freedman, Barry I; Frostegård, Johan; García, Mercedes A; de la Torre, Ignacio García; 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; Kovács, László; Laustrup, Helle; Lauwerys, Bernard R; Li, Quan-Zhen; Maradiaga-Ceceña, Marco A; Martín, Javier; McCune, Joseph M; McWilliams, David R; Merrill, Joan T; Miranda, Pedro; Moctezuma, José 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, José M; Aguilar-Salinas, Carlos A; Scherbarth, Hugo R; Scorza, Raffaella; Seldin, Michael F; Sjöwall, Christopher; Svenungsson, Elisabet; Thompson, Susan D; Toloza, Sergio M A; Truedsson, Lennart; Tusié-Luna, Teresa; Vasconcelos, Carlos; Vilá, Luis M; Wallace, Daniel J; Weisman, Michael H; Wither, Joan E; Bhangale, Tushar; Oksenberg, Jorge R; Rioux, John D; Gregersen, Peter K; Syvänen, Ann-Christine; Rönnblom, Lars; Criswell, Lindsey A; Jacob, Chaim O; Sivils, Kathy L; Tsao, Betty P; Schanberg, Laura E; Behrens, Timothy W; Silverman, Earl D; Alarcón-Riquelme, Marta E; Kimberly, Robert P; Harley, John B; Wakeland, Edward K; Graham, Robert R; Gaffney, Patrick M; Vyse, Timothy J

    2017-07-17

    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 distinct non-HLA regions in EA, 9 in AA and 16 in HA (∼50% of these regions have multiple independent associations); these include 24 novel SLE regions (P<5 × 10(-8)), refined association signals in established regions, extended associations to additional ancestries, and a disentangled complex HLA multigenic effect. The risk allele count (genetic load) exhibits an accelerating pattern of SLE risk, leading us to posit a cumulative hit hypothesis for autoimmune disease. Comparing results across the three ancestries identifies both ancestry-dependent and ancestry-independent contributions to SLE risk. Our results are consistent with the unique and complex histories of the populations sampled, and collectively help clarify the genetic architecture and ethnic disparities in SLE.

  5. Dehydroepiandrosterone for the treatment of systemic lupus erythematosus.

    PubMed

    van Vollenhoven, Ronald F

    2002-01-01

    The adrenal steroidal hormone dehydroepiandrosterone (DHEA) has been studied as a potential pharmacological agent in the treatment of the autoimmune disease systemic lupus erythematosus (SLE). Both the endocrine effects (the ability to be converted peripherally to androgenic and oestrogenic sex steroids) and the immunomodulatory effects of DHEA (the production of the Th(1) cytokines, such as IL-2) suggest that this hormone could be of benefit for patients with SLE. During the past decade, five controlled clinical trials and a number of additional observational studies have been performed investigating these possibilities. The results from these studies suggest that 200 mg/day of DHEA for 7 - 12 months decreases corticosteroid requirement for the patients, the frequency of disease flares, has an anti-osteoporotic effect and has an overall beneficial effect on SLE disease activity in female patients. A small study suggested benefits for cognitive function in such patients. The side effects acne and hirsutism were seen relatively frequently (30 - 40% and 10 - 12% of patients, respectively) but in most instances were deemed mild. DHEA treatment resulted in changes in lipid profile and may have endocrine effects, the consequences of which will need to be ascertained through longer-term follow-up studies.

  6. Group psychotherapy reduces illness intrusiveness in systemic lupus erythematosus.

    PubMed

    Edworthy, Steven M; Dobkin, Patricia L; Clarke, Ann E; Da Costa, Deborah; Dritsa, Maria; Fortin, Paul R; Barr, Susan; Ensworth, Stephanie; Esdaile, John M; Beaulieu, André; Zummer, Michael; Senécal, Jean-Luc; Goulet, Jean-Richard; Choquette, Denis; Rich, Eric; Smith, Doug; Cividino, Alfred; Gladman, Dafna; Devins, Gerald M

    2003-05-01

    We investigated whether brief supportive-expressive group psychotherapy might reduce illness-induced interference with valued activities and interests (i.e., illness intrusiveness) among women with systemic lupus erythematosus (SLE) in relation to 3 life domains: (1) relationships and personal development (family relationships, other social relationships, self-expression), (2) intimacy (relationship with spouse, sex life), and/or (3) instrumental life (work, finances, active recreation). Women with SLE recruited from 9 rheumatology centers were randomly assigned to receive either usual care (n = 66) or a 12 week brief supportive-expressive group psychotherapy followed by 3 monthly booster sessions (n = 58). Standard instruments assessed disease activity and damage, illness intrusiveness, and psychological distress at 4 measurement occasions: (1) pretreatment, (2) posttreatment, (3) 6 month followup, and (4) 12 month followup. Analysis of covariance, controlling for disease activity and household income, indicated that women who received brief supportive-expressive group psychotherapy experienced significant reductions in illness intrusiveness for 2 of 3 domains: (1) relationships and personal development and (2) intimacy. Benefits were evident at 6 and 12 month followups. Brief supportive-expressive group psychotherapy facilitates adaptation to SLE by assisting women in reducing illness-induced disruptions into important domains of life experience.

  7. Regional brain metabolism in a murine systemic lupus erythematosus model

    PubMed Central

    Vo, An; Volpe, Bruce T; Tang, Chris C; Schiffer, Wynne K; Kowal, Czeslawa; Huerta, Patricio T; Uluğ, Aziz M; Dewey, Stephen L; Eidelberg, David; Diamond, Betty

    2014-01-01

    Systemic lupus erythematosus (SLE) is characterized by multiorgan inflammation, neuropsychiatric disorders (NPSLE), and anti-nuclear antibodies. We previously identified a subset of anti-DNA antibodies (DNRAb) cross-reactive with the N-methyl-D-aspartate receptor, present in 30% to 40% of patients, able to enhance excitatory post-synaptic potentials and trigger neuronal apoptosis. DNRAb+ mice exhibit memory impairment or altered fear response, depending on whether the antibody penetrates the hippocampus or amygdala. Here, we used 18F-fluorodeoxyglucose (FDG) microPET to plot changes in brain metabolism after regional blood–brain barrier (BBB) breach. In DNRAb+ mice, metabolism declined at the site of BBB breach in the first 2 weeks and increased over the next 2 weeks. In contrast, DNRAb− mice exhibited metabolic increases in these regions over the 4 weeks after the insult. Memory impairment was present in DNRAb+ animals with hippocampal BBB breach and altered fear conditioning in DNRAb+ mice with amygdala BBB breach. In DNRAb+ mice, we observed an inverse relationship between neuron number and regional metabolism, while a positive correlation was observed in DNRAb− mice. These findings suggest that local metabolic alterations in this model take place through different mechanisms with distinct time courses, with important implications for the interpretation of imaging data in SLE subjects. PMID:24824914

  8. Bilateral Cytomegalovirus Retinitis in a Patient with Systemic Lupus Erythematosus

    PubMed Central

    Haze, Masaya; Kobayashi, Takatoshi; Kakurai, Keigo; Shoda, Hiromi; Takai, Nanae; Takeda, Sayako; Tada, Rei; Maruyama, Kouichi; Kida, Teruyo; Ikeda, Tsunehiko

    2016-01-01

    Purpose The purpose of this study was to report the case of a patient who underwent vitrectomy for bilateral rhegmatogenous retinal detachment caused by cytomegalovirus (CMV) retinitis while undergoing steroid and immunosuppressant therapy for systemic lupus erythematosus (SLE). Case Report We report on a 29-year-old female who was undergoing steroids and immunosuppressants treatment for SLE at Osaka Medical College Hospital, Takatsuki City, Japan. Examination of the patient due to prolonged and worsening diarrhea revealed positive test results for C7-HRP, and she was diagnosed with CMV colitis. She was subsequently admitted to the hospital and started on intravenous ganciclovir for treatment. Approximately 1.5 months later, her primary complaint was deterioration of the upper visual field in her left eye, and she was then referred to the Department of Ophthalmology. Numerous granular exudative spots were found around the lower retinal area of her left eye with retinal breaks that had developed in an area of retinal necrosis that resulted in retinal detachment. After time was allowed for the patient's general condition to improve, a vitrectomy was performed on that eye. The patient subsequently developed a similar retinal detachment in her right eye, for which she underwent a vitrectomy. Although the patient required multiple surgeries on both eyes, her retinas currently remain reattached and the inflammation has subsided. Conclusion The findings of this study show that strict attention must be paid to SLE patients on immunosuppressive therapy due to the possible association of CMV retinitis. PMID:27462259

  9. Drugs in early clinical development for Systemic Lupus Erythematosus

    PubMed Central

    Postal, Mariana; Sinicato, Nailú Angélica; Appenzeller, Simone; Niewold, Timothy B

    2016-01-01

    Introduction While immunosuppressive therapy has positively impacted the prognosis of systemic lupus erythematosus (SLE), many patients still do not respond to traditional therapy. Thus, active SLE disease remains a significant problem. Furthermore, conventional immunosuppressive treatments for SLE are associated a high risk of side effects. These issues call for improvement in our current therapeutic armamentarium. Areas covered In this review, the authors highlight the recent developments in therapies for SLE, and present an overview of drugs which are in early clinical development for SLE. There are many new therapeutic approaches being developed, including those focused on B-cell targets, T-cell downregulation, co-stimulatory blockade, anti-cytokine agents, and kinase inhibition, and Toll-like receptor inhibition. They also discuss peptide therapy as a potential method to re-establish immune tolerance, and some of the challenges ahead in developing and testing novel agents for SLE. Expert opinion Many novel agents are currently in development for SLE, but this encouraging news is tempered by several disappointments in clinical trials and provides a timely moment to reflect on the future of therapeutic development in SLE. It seems likely that biological heterogeneity between patients is a major contributor to difficulty in drug design in SLE. PMID:26950689

  10. Genital Mycoplasma infection among Mexican women with systemic lupus erythematosus.

    PubMed

    Méndez-Martínez, Socorro; García-Carrasco, Mario; Cedillo-Ramírez, María L; Mendoza-Pinto, Claudia; Etchegaray-Morales, Ivet; Gil-Juárez, Constantino; Montiel-Jarquín, Álvaro J; Taboada-Cole, Alejandro; Jiménez-Herrera, Erick A; Muñóz-Guarneros, Margarita; Cervera, Ricard

    2017-07-01

    To assess the prevalence of genital Mycoplasma spp. among women with systemic lupus erythematosus (SLE) and to identify factors associated with such infection. A cross-sectional study was conducted among patients with SLE and healthy women who attended a hospital in Puebla, Mexico, between July 29, 2014, and January 4, 2015. All participants were aged 18 years or older and sexually active. A structured interview assessed sociodemographic, obstetric, gynecologic, and clinical characteristics. Disease activity was evaluated using the Mexican SLE Disease Activity Index. Polymerase chain reaction was used to detect the presence of Mycoplasma spp. in genital samples. Ureaplasma urealyticum was the only genital mycoplasma detected; it was present in 32 (24.6%) of 130 patients with SLE and 12 (12.8%) of 94 healthy women. Patients with SLE had increased odds of infection (odds ratio 2.120, 95% confidence interval 1.046-4.296). Among patients with SLE, multiparity was more common in those with U. urealyticum infection (P=0.043). One-quarter of women with SLE had genital infection with U. urealyticum. An association was found between infection and multiparity among women with SLE. © 2017 International Federation of Gynecology and Obstetrics.