[Systemic lupus erythematosus of an anasarcous type appearing in a patient with chronic discoid lupus erythematosus].
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Edema↗
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The role of Epstein-Barr virus in systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is a devastating autoimmune disease with no known cure. Lupus patients suffer from a myriad of clinical symptoms which variably include arthritis, pleuritis, pericarditis, vasculitis, and nephritis. The underlying mechanisms behind these clinical findings and the etiologic events preceding and causing disease onset, however, remain largely unknown. For many years, investigators have suspected that Epstein-Barr virus might somehow be involved in the etiology and/or pathogenesis of systemic lupus. Numerous studies have examined this possibility from various angles and have arrived at different conclusions. This work reviews these historical papers in the context of new results and presents a hypothetical role for this virus as an etiological environmental trigger for SLE.
Antibodies, Viral↗
Hemolytic anemia and thrombocytopenic purpura: two related subsets of systemic lupus erythematosus.
Systemic lupus erythematosus patients who develop hemolytic anemia or thrombocytopenic purpura differ from other lupus patients and are similar enough to be considered two related subsets with a more benign course. Thirty-one lupus patients with either or both these hemocytopenias were found to be significantly younger, more often males, and had less frequent fever, polyarthritis, serositis, cutaneous vasculitis, nephropathy, neurologic manifestations, and persistent hypocomplementemia than 62 lupus patients without any of these hemocytopenias. They also had lower index scores of overall disease severity and required less treatment. It seems important to subdivide lupus patients in subsets for therapeutic and prognostic purposes.
Adolescent↗
The contribution of inherited and acquired thrombophilic defects, alone or combined with antiphospholipid antibodies, to venous and arterial thromboembolism in patients with systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is associated with an increased risk of venous (VTE) and arterial thromboembolism (ATE). Lupus anticoagulant (LA) and anticardiolipin antibodies (ACAs) are established risk factors. We assessed the contribution of deficiencies of antithrombin, protein C, total protein S, factor V Leiden, the prothrombin G20210A mutation and APC resistance, either alone or in various combinations with LA and/or ACAs, to the thrombotic risk in a cohort of 144 consecutive patients with SLE. Median follow-up was 12.7 years. VTE had occurred in 10% and ATE in 11% of patients. LA,ACAs, factor V Leiden, and the prothrombin mutation were identified as risk factors for VTE. Annual incidences of VTE were 2.01 (0.74-4.37) in patients with one of these disorders and 3.05 (0.63-8.93) in patients with 2 disorders. The risk of VTE was 20- and 30-fold higher, respectively, compared with the normal population. In contrast with LA and ACAs, thrombophilic disorders did not influence the risk of ATE. In conclusion, factor V Leiden and the prothrombin mutation contribute to the risk of VTE in patients with SLE, and potentiate this risk when one of these thrombophilic defects are combined with LA and/or ACAs.
Activated Protein C Resistance↗
B cell loss leading to remission in severe systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) pathogenesis is mediated in part by autoantibodies. We describe a patient with central nervous system lupus who developed a loss of B cells with associated hypogammaglobulinemia and sinopulmonary infections requiring intravenous immunoglobulin. The SLE went into complete remission. Of 18 reported patients with SLE developing persistent hypogammaglobulinemia, only 5 patients including ours had a nearly complete loss of circulating B cells. Of those whose SLE and B cell status was reported, 5/5 with B cell loss and 1/10 without B cell loss experienced a durable response of SLE (p = 0.002). These cases illustrate that B cell ablative therapies may have efficacy for SLE.
Adult↗
Lupus anticoagulant and anticardiolipin antibodies in systemic lupus erythematosus.
BACKGROUND: Lupus anticoagulant and anticardiolipin antibodies are antiphospholipid antibodies which have been independently associated with a high incidence of thrombotic diseases. However, the importance of their combined occurrence has not yet been examined. METHODS: We investigated 70 patients with systemic lupus erythematosus for the presence of anticardiolipin antibodies paying particular attention to a history of thrombosis and abortion. Lupus anticoagulant was detected using the kaolin clotting time, its mixing tests with normal plasma and the inosithin neutralization test. Anticardiolipin antibodies were detected using the ELISA technique. RESULTS: Lupus anticoagulant was detected in 11 patients (16%) and anticardiolipin antibodies in 13 (19%). Six patients were positive for both lupus anticoagulant and anticardiolipin antibodies. These patients had a higher incidence of thrombosis or recurrent abortion (5 of 6) compared to those with lupus anticoagulant (2 of 5) or anticardiolipin antibodies alone (1 of 7). The amount of inosithin required to neutralize lupus anticoagulant was greater [mean (SD) 27.5 (20.5) micrograms] in patients with both lupus anticoagulant and anticardiolipin antibodies than in patients with lupus anticoagulant alone [mean (SD) 4.0 (5.4) micrograms]. CONCLUSION: The presence of lupus anticoagulant is associated with thrombosis and recurrent abortion which are more frequent when both lupus anticoagulant and anticardiolipin antibodies are present and these patients probably have more severe disease as the amount of inosithin required to neutralize the lupus anticoagulant was greater.
Adolescent↗
[Childhood-onset systemic lupus erythematosus].
Systemic Lupus Erythematosus (SLE) remains a challenging autoimmune disease in term of etiology, pathogenesis and treatment. It is estimated that 10-17% of lupus patients present before the age of 16. SLE in children appears to have more severe organ involvement than in adults. The outcome of childhood SLE has improved during the last decade, but the morbidity remains high.
Adolescent↗
Molecular aspects in the pathogenesis of human systemic lupus erythematosus.
Systemic lupus erythematosus is a common and often devastating systemic autoimmune disease of unknown etiology. In this communication we review the latest developments of the molecular pathogenesis of human lupus. Novel genetic studies of multiplex lupus families have revealed potential disease-associated genome intervals, put special emphasis on genetic loci mapping in the long arm of chromosome 1 and have underscored the complexity of the underlying genetic background. New data have emerged on the role of estrogens in the function of lymphocytes and a number of studies have recently emphasized the relative Th-1/Th-2 cytokine imbalance in favor of a Th-2 type cytokine immune response. Finally, novel experiments have revealed an abnormal antigen receptor-mediated signaling process in lupus T and B cells, which may influence the aberrant expression and function of costimulatory molecules as well as of other aspects of immune cell function. It is important to decipher the underlying molecular mechanisms that govern the expression of human lupus, because we may design novel, rational approaches in the treatment of a human lupus, a disease that has high morbidity and mortality.
Antibodies, Antinuclear↗
[Morbidity and mortality in systemic lupus erythematosus].
Systemic lupus erythematosus is an autoimmune disease that can affect any organ system, mostly central and peripheral nervous system, lungs, heart, kidneys, skin, serous membranes, and components of blood. Lupus is more prevalent in females, has no etiology, and has a distinct association with several immune response genes. Understanding and treatment of lupus has progressed in the last decade, contributing to the improvement in survival and quality of life. Improvement in prognosis is associated with many factors including the socioeconomic state, advanced medicine, and access to medical sources. Three aspects of the disease are necessary to establish and describe the prognosis in SLE: disease activity, accumulated damage, and health status/quality of life.
Humans↗
Genetics and systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is a complex, multifactorial autoimmune disease. Genetic factors are believed to contribute to its pathogenesis. There have been numerous recent advances in the study of murine and human lupus genetics. In well-defined, experimental, transgenic or gene-knockout mouse models, the development of lupus-like disease has implicated specific genes and pathways in the disease pathogenesis. Linkage analyses have mapped multiple susceptibility loci and disease suppressive loci using inbred strains of mice that spontaneously develop lupus-like disease. Elegant genetic dissection has demonstrated that a component phenotype of SLE is displayed by each congenic strain carrying a single susceptibility locus on a resistant genetic background, whereas polycongenic strains exhibit fatal lupus nephritis. These studies suggest that genes in separate pathways can interact to augment or suppress the initiation and progression of systemic autoimmunity. In association studies of human lupus, the contributions of the MHC loci, Fcg receptors, various cytokines, components of the complement cascade, and proteins involved in apoptosis have been explored. Most recently, linkage analyses have been performed and provide many chromosomal regions for further exploration for susceptibility genes. Studies to identify the genes in the susceptibility regions are underway. An understanding of the genes involved in the development of lupus should provide targets for more focused therapy in lupus.
Animals↗
An update on genetic studies of systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is a complex, multifactorial autoimmune disease. Genetic factors are thought to contribute to its pathogenesis. There have been numerous recent advances in the study of murine and human lupus genetics. In well-defined experimental transgenic or gene-knockout mouse models, the development of lupus-like disease has implicated specific genes and pathways in the disease pathogenesis. Linkage analyses have mapped multiple susceptibility loci and disease suppressive loci using inbred strains of mice that spontaneously develop lupus-like disease. Elegant genetic dissection and function studies have led to the recent identification of two murine candidate susceptibility genes, Ifi202 (encoding an interferon-inducible protein) and Cr2 (encoding complement receptors 1 and 2). In human lupus, case- control studies have established associations of SLE with certain major histocompatibility class II alleles, complement deficiencies, and polymorphisms of Fc gamma receptor genes, a complement-related gene, and cytokine genes. During the past several years, linkage analyses using SLE multiplex families have provided many chromosomal regions for further exploration of susceptibility genes. Six regions exhibiting significant linkage to SLE are promising. Studies are underway to fine map these linked regions and to identify the genes in the susceptibility regions. An understanding of the genes involved in the development of lupus should provide targets for more focused therapy in lupus.
Alleles↗
Genetics and systemic lupus erythematosus.
Systemic lupus erythematosus is a complex, multifactorial, autoimmune disease. Genetic factors are believed to contribute to its pathogenesis. There have been numerous recent advances in the study of both murine and human lupus genetics. In murine lupus, congenic strains of three susceptibility loci have been developed. Transgenic and knock-out mice models of candidate genes now exist. In association studies of human lupus, the contributions of the MHC loci, Fcgamma receptors, various cytokines, components of the complement cascade, and proteins involved in apoptosis have been explored. Most recently, linkage analyses have been performed and provide numerous regions for further exploration for susceptibility genes. Studies to identify the genes in the susceptibility regions are underway. An understanding of the genes involved in the development of lupus should provide targets for more focused therapy.
Animals↗
Genetics of systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is a complex autoimmune disease in which multiple genes appear to play important roles in pathogenesis. Hereditary deficiencies, both complete and partial, of several components of the complement system clearly predispose to lupus. HLA class II alleles appear to mediate specific autoantibodies, many of which are pathogenic. Modern molecular techniques are now providing insight into the specific major histocompatibility complex class II polymorphisms that are associated with different autoantibodies in SLE. Other genetic systems also may be important.
Complement System Proteins↗
Persistent scarring, atrophy, and dyspigmentation in a preteen girl with neonatal lupus erythematosus.
Neonatal lupus erythematosus is an uncommon autoimmune disease with distinctive cutaneous findings. Descriptions of chronic cutaneous sequelae are rare. We describe a 12-year-old girl with persistent dyspigmentation, scarring, and atrophy as a result of neonatal lupus occurring during infancy.
Atrophy↗
[B-cell anomalies in systemic lupus erythematosus].
Systemic lupus erythematosus is a non-organ-specific autoimmune disease characterized biologically by B lymphocyte hyperactivity and the production of autoantibodies directed against various cellular components, in particular nuclear antigens. Different strains of mice spontaneously develop a lupus-like disease and constitute a guidelight for human SLE. Both polyclonal B cell stimulation and clonal expansion induced by self-antigens participate in B cell hyperactivity observed in human and mouse SLE. B cells are hyperactive to various stimuli, in particular those delivered by T cells through surface molecules or cytokines. The consequences are an increased production of immunoglobulins and the development of autoantibodies thought to induce the major part of tissue lesions. B cells also participate in the pathological process as antigen-presenting and cytokine-secreting cells. An intrinsic defect of B cells is suspected to be responsible for B cell anomalies as illustrated by certain spontaneous murine models of SLE (motheaten mice) and by lupus-like syndromes observed in mice rendered deficient for genes controlling the B-cell receptor (BCR) signaling pathway. Genome wide scan analysis of various lupus strains allowed to identify several loci predisposing to lupus among which certain are associated with B cell hyperactivity suggesting that the intrinsic defect is inherited.
Animals↗
Acute visual loss in systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) often presents as a multisystem disease that can be difficult to diagnose. Although ocular symptoms are infrequent, actual acute visual loss has been reported. A review of four cases of acute visual loss from a lupus clinic revealed that two patients had visual loss as a presenting sign of SLE. One had bilateral occipital lobe infarctions, the other multiple cotton wool spots and an attenuated retinal vascular system. Of the two patients with documented SLE prior to the onset of visual problems, one presented with a coincidental retinal tear and the other with retinal phlebitis.
Adult↗
B cell abnormalities in systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease characterized by the differentiation of short- and long-lived immunoglobulin secreting plasma cells that secrete pathogenic autoantibodies. Ectopic germinal centers and plasma cells secreting autoantibodies have been observed in lupus nephritis kidneys. Candidate genetic susceptibility loci for SLE include genes that affect differentiation and survival of plasma cells, such as those that influence activation, proliferation, cytokine and chemokine secretion/responsiveness, and apoptosis of the T and B cells that are involved in humoral immunity generated in germinal centers, as well as genes that are involved in presentation and clearance of apoptotic material and autoantigens by antigen presenting cells and other phagocytes. Emerging data have demonstrated that B lymphocytes are active participants in humoral immune responses that lead to T-dependent and T-independent differentiation of immunoglobulin-secreting plasma cells by homotypic CD154-CD40 interactions as well as continued stimulation by B cell activating factor through B cell maturation antigen, B cell activating factor receptor and transmembrane activater.
B-Cell Activating Factor↗
The role of nitric oxide in abnormal T cell signal transduction in systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by production of antinuclear autoantibodies and diverse array of clinical manifestations. T cells from patients with SLE have been shown to be activated in vivo and provide help to autoreactive B cells. Lupus T cells exhibit enhanced spontaneous and diminished activation-induced apoptosis and predisposition to necrosis. Persistent mitochondrial hyperpolarization and ATP depletion - associated with significantly increased mitochondrial mass - characterize T lymphocyte dysfunction in SLE. In addition to cell death abnormalities, mitochondrial dysfunction is associated with altered signal transduction through the T cell receptor and Ca2+ fluxing. Exposure of normal T cell to nitric oxide induces mitochondrial hyperpolarization and biogenesis and regenerates the Ca2+ signaling profile of lupus T cells. This article reviews a novel understanding of the role of nitric oxide in signal transduction and cell death abnormalities in SLE.
Animals↗