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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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Journal ArticleDOI
IL-6/STAT3 pathway induced deficiency of RFX1 contributes to Th17-dependent autoimmune diseases via epigenetic regulation
Ming Zhao,Yixin Tan,Qiao Peng,Cancan Huang,Yu Guo,Gongping Liang,Bochen Zhu,Yi Huang,Aiyun Liu,Zijun Wang,Mengying Li,Xiaofei Gao,Ruifang Wu,Haijing Wu,Hai Long,Qianjin Lu +15 more
TL;DR: It is shown that the transcription factor RFX1 limits Th17 differentiation and is protective against the pathogenesis of Th17-driven autoimmune diseases, highlighting a non-canonical pathway that regulates differentiation of Th 17 cells.
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Mortality in lupus nephritis.
TL;DR: A bimodal pattern of early deaths due to active lupus and sepsis and late deaths from vascular events was found superimposed on a constant rate of death from renal failure.
Journal Article
Neurometabolism of active neuropsychiatric lupus determined with proton MR spectroscopy.
TL;DR: Major NPSLE, past or present, is associated with decreased levels of NAA, and neurochemical markers determined by MR spectroscopy may be useful for determining activity and degree of brain injury in NPSle.
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Significantly increased levels of mannose-binding lectin (MBL) in rheumatic heart disease: a beneficial role for MBL deficiency.
TL;DR: The significantly elevated circulating MBL levels in patients with RHD together with the greater prevalence of MBL deficiency in controls suggest that MBL may cause undesirable complement activation contributing to the pathogenesis of RHD.
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Concomitant new diagnosis of systemic lupus erythematosus and COVID-19 with possible antiphospholipid syndrome. Just a coincidence? A case report and review of intertwining pathophysiology.
TL;DR: In the midst of the COVID-19 pandemic, further understanding of its complications points towards dysregulated immune response as a major component, and it is possible that SARS-CoV-2, as it happens with other viruses, might lead to the formation of antiphospholipid antibodies, potentially contributing to the increased rates of thrombosis seen in CO VID-19.
References
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Journal ArticleDOI
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.
Journal ArticleDOI
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.
Journal ArticleDOI
The neurological manifestations of systemic lupus erythematosus.
Journal ArticleDOI
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.
Related Papers (5)
Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus.
Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.
Michelle Petri,Ana Maria Orbai,Graciela S. Alarcón,Caroline Gordon,Joan T. Merrill,Paul R. Fortin,Ian N. Bruce,David A. Isenberg,Daniel J. Wallace,Ola Nived,Gunnar Sturfelt,Rosalind Ramsey-Goldman,Sang Cheol Bae,John G. Hanly,Jorge Sanchez-Guerrero,Ann E. Clarke,Cynthia Aranow,Susan Manzi,Murray B. Urowitz,Dafna D. Gladman,Kenneth C. Kalunian,Melissa Costner,Victoria P. Werth,Asad Zoma,Sasha Bernatsky,Guillermo Ruiz-Irastorza,Munther A. Khamashta,Søren Jacobsen,Jill P. Buyon,Peter J. Maddison,Mary Anne Dooley,Ronald F van Vollenhoven,Ellen M. Ginzler,Thomas Stoll,Christine A. Peschken,Joseph L. Jorizzo,Jeffrey P. Callen,S. Sam Lim,Barri J. Fessler,Murat Inanc,Diane L. Kamen,Anisur Rahman,Kristjan Steinsson,Andrew G. Franks,Lisa Sigler,Suhail Hameed,Hong Fang,Ngoc Minh Pham,Robin L. Brey,Michael H. Weisman,Gerald McGwin,Laurence S. Magder +51 more