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Systemic Lupus Erythematosus
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TLDR
Contributions are gathered from physicians and researchers from North America, South America, Europe, and Asia that highlight several important and/or novel aspects of the molecular pathogenesis, clinical organ involvement, and experimental therapies in this prototypical systemic autoimmune disease.Abstract:
In sharp distinction to organ-specific autoimmune diseases such as thyroiditis, diabetes, or myasthenia gravis, systemic lupus erythematosus (SLE) is a constellation of signs and symptoms classified as one nosologic entity. Indeed, it is the diversity of presentation, accumulation of manifestations over time, and undulating disease course that challenge the most astute of clinicians. With rare exception, the unifying laboratory abnormality is the presence of circulating antinuclear antibodies (ANA). Acknowledging the complexity of this disease, its broad differential diagnosis, and the need to develop better and more specific therapies, the American College of Rheumatology (ACR) has designated 11 diagnostic criteria (presented in Table 15A-1) (1,2). These criteria reflect the major clinical features of the disease (mucocutaneous, articular, serosal, renal, neurologic) and incorporate the associated laboratory findings (hematologic and immunologic). The presence of four or more criteria is required for diagnosis. They need not necessarily present simultaneously: a single criterion such as arthritis or thrombocytopenia may recur over months or years before the diagnosis can be confirmed by the appearance of additional features. While there is incomplete agreement among rheumatologists as to whether these criteria need to be strictly applied in a practice setting, or reserved only for formal academic studies, they do facilitate a methodologic approach to evaluate a patient.read more
Citations
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Complement Protein C1q-Mediated Neuroprotection Is Correlated with Regulation of Neuronal Gene and MicroRNA Expression
Marie E. Benoit,Andrea J. Tenner +1 more
TL;DR: Results show that C1q can directly promote neuronal survival, thereby demonstrating new interactions between immune proteins and neuronal cells that may facilitate neuroprotection.
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C5a promotes development of experimental lupus nephritis which can be blocked with a specific receptor antagonist.
TL;DR: Data support an important role for the C5a anaphylatoxin in lupus nephritis, and that blockade of C5 aR represents a potentially viable treatment for human lupUS nephitis, and suggest pharmacological blockade ofC5aR reduces disease manifestations in experimental lupu neph arthritis.
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Seipin: from human disease to molecular mechanism.
TL;DR: The most severe form of congenital generalized lipodystrophy (CGL) is caused by mutations in BSCL2/seipin this article, which is a homo-oligomeric integral membrane protein in the endoplasmic reticulum that concentrates at junctions with cytoplasmic lipid droplets (LDs).
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Foxp3+ Helios+ regulatory T cells are expanded in active systemic lupus erythematosus
Tobias Alexander,Arne Sattler,Lars Templin,Siegfried Kohler,Christian Groß,Andreas Meisel,Birgit Sawitzki,Gerd-Rüdiger Burmester,Renate Arnold,Andreas Radbruch,Andreas Thiel,Falk Hiepe +11 more
TL;DR: The data suggest that Helios-expressing Foxp3+ Treg with functional suppressive capacity and migratory potential into inflamed tissues are expanded in active SLE, presumably through γ-chain signalling cytokines and TCR stimulation, to compensate for autoreactive effector responses.
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B-cell kinase lyn deficiency in patients with systemic lupus erythematosus.
Stamatis-Nick C. Liossis,Elena E. Solomou,Meletios-Athanasios Dimopoulos,Panayiotis Panayiotidis,M. Mavrikakis,Petros P. Sfikakis +5 more
TL;DR: Examination of the expression of the CD22 co-receptor and of kinase Lyn in B cells from patients with SLE revealed that Lyn mRNA was significantly decreased in lupus patients with decreased Lyn protein expression, suggesting that Lyn deficiency may be caused at least in part by defects at the transcription level.
References
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Systemic lupus erythematosus
Jessica J Manson,Anisur Rahman +1 more
TL;DR: Systemic lupus erythematosus is a relapsing and remitting disease, and treatment aims are threefold: managing acute periods of potentially life-threatening ill health, minimizing the risk of flares during periods of relative stability, and controlling the less life- threatening, but often incapacitating day to day symptoms.
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The bimodal mortality pattern of systemic lupus erythematosus
Murray B. Urowitz,Arthur Bookman,Barry E. Koehler,Duncan A. Gordon,Hugh A. Smythe,Metro A. Ogryzlo +5 more
TL;DR: An examination of the deaths in a long-term systematic analysis of 81 patients followed for five years at the University of Toronto Rheumatic Disease Unit found death is associated with inactive lupus, long duration of steroid therapy and a striking incidence of myocardial infarction due to atherosclerotic heart disease.
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Immunological studies concerning the nephritis of systemic lupus erythematosus
TL;DR: The immunochemical evidence for the high specific activity of antinuclear antibodies and the association of DNA antigen with DNA antibody in glomeruli add further support for the antigen-antibody complex hypothesis for renal injury in systemic lupus erythematosus.
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The neurological manifestations of systemic lupus erythematosus.
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Immunologic Factors and Clinical Activity in Systemic Lupus Erythematosus
Peter H. Schur,John I. Sandson +1 more
TL;DR: Serial immunochemical observations may be useful in the management of patients with systemic lupus erythematosus, as they reflect the in vivo formation of immune complexes that cause nephritis.
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