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The complement system in systemic autoimmune disease
Chen, Min1,2; Daha, Mohamed R.3; Kallenberg, Cees G. M.1
关键词Complement Systemic Autoimmune Disease Anca-associated Vasculitis Neutrophils Alternative Pathway Systemic Lupus Erythematosus
刊名JOURNAL OF AUTOIMMUNITY
2010-05-01
DOI10.1016/j.jaut.2009.11.014
34期:3页:J276-J286
收录类别SCI ; ISTP
文章类型Proceedings Paper
WOS标题词Science & Technology
类目[WOS]Immunology
研究领域[WOS]Immunology
关键词[WOS]PRIMARY SJOGRENS-SYNDROME ; HEPATITIS-C VIRUS ; ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES ; ANTIBODY-MEDIATED GLOMERULONEPHRITIS ; MANNAN-BINDING LECTIN ; LUPUS-ERYTHEMATOSUS ; RHEUMATOID-ARTHRITIS ; APOPTOTIC CELLS ; ALTERNATIVE PATHWAY ; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS
英文摘要

Complement is part of the innate immune system. Its major function is recognition and elimination of pathogens via direct killing and/or stimulation of phagocytosis. Activation of the complement system is, however, also involved in the pathogenesis of the systemic autoimmune diseases. Activation via the classical pathway has long been recognized in immune complex-mediated diseases such as cryoglobulinemic vasculitis and systemic lupus erythematosus (SLE). In SLE, the role of complement is somewhat paradoxical. It is involved in autoantibody-initiated tissue damage on the one hand, but, on the other hand, it appears to have protective features as hereditary deficiencies of classical pathway components are associated with an increased risk for SLE. There is increasing evidence that the alternative pathway of complement, even more than the classical pathway, is involved in many systemic autoimmune diseases. This is true for IgA-dominant Henoch Schonlein Purpura, in which additional activation of the lectin pathway contributes to more severe disease. In anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis the complement system was considered not to be involved since immunoglobulin deposition is generally absent in the lesions. However, recent studies, both in human and animal models, demonstrated complement activation via the alternative pathway as a major pathogenic mechanism. Insight into the role of the various pathways of complement in the systemic autoimmune diseases including the vasculitides opens up new ways of treatment by blocking effector pathways of complement. This has been demonstrated for monoclonal antibodies to C5 or C5a in experimental anti-phospholipid antibody syndrome and ANCA-associated vasculitis. (C) 2009 Elsevier Ltd. All rights reserved.

语种英语
WOS记录号WOS:000276818300011
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被引频次:150[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/57113
专题北京大学第一临床医学院_肾脏内科
作者单位1.Univ Groningen, Univ Med Ctr Groningen, Dept Rheumatol & Clin Immunol, NL-9700 AB Groningen, Netherlands
2.Peking Univ, Hosp 1, Div Renal, Beijing 100034, Peoples R China
3.Leiden Univ, Dept Nephrol, Med Ctr, NL-2300 RA Leiden, Netherlands
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GB/T 7714
Chen, Min,Daha, Mohamed R.,Kallenberg, Cees G. M.. The complement system in systemic autoimmune disease[J]. JOURNAL OF AUTOIMMUNITY,2010,34(3):J276-J286.
APA Chen, Min,Daha, Mohamed R.,&Kallenberg, Cees G. M..(2010).The complement system in systemic autoimmune disease.JOURNAL OF AUTOIMMUNITY,34(3),J276-J286.
MLA Chen, Min,et al."The complement system in systemic autoimmune disease".JOURNAL OF AUTOIMMUNITY 34.3(2010):J276-J286.
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