Abstract：
:Antineutrophil cytoplasmic autoantibodies (ANCA) are the likely cause for necrotizing small-vessel vasculitis and crescentic glomerulonephritis. Unlike other forms of crescentic glomerulonephritis induced by immune complexes or anti-glomerular basement membrane antibodies that have conspicuous vessel wall immunoglobulin and complement, there is a paucity, although usually not an absence, of vessel wall immunoglobulin and complement in ANCA-associated glomerulonephritis. Despite this comparatively lower level and more localized distribution of vessel wall complement, experimental and clinical observations strongly incriminate alternative complement pathway activation as critically important in the pathogenesis of ANCA disease. Experimental data in animal models and in vitro experiments has shown that primed neutrophils are activated by ANCA, which generates C5a, which engages C5a receptors on neutrophils. This attracts and in turn primes more neutrophils for activation by ANCA. In patients with ANCA disease, plasma levels of C3a, C5a, soluble C5b-9, and Bb have been reported to be higher in active disease than in remission, whereas no difference was reported in plasma C4d in active versus ANCA disease remission. Thus, experimental and clinical data support the hypothesis that ANCA-induced neutrophil activation activates the alternative complement pathway and generates C5a. C5a not only recruits additional neutrophils through chemotaxis but also primes neutrophils for activation by ANCA. This creates a self-fueling inflammatory amplification loop that results in the extremely destructive necrotizing vascular injury.
journal_name
Semin Nephroljournal_title
Seminars in nephrologyauthors
Charles Jennette J,Xiao H,Hu Pdoi
10.1016/j.semnephrol.2013.08.006subject
Has Abstractpub_date
2013-11-01 00:00:00pages
557-64issue
6eissn
0270-9295issn
1558-4488pii
S0270-9295(13)00134-4journal_volume
33pub_type
杂志文章,评审abstract：:Besides loss of life and property, natural disasters result in a number of renal challenges, either by the creation of rhabdomyolysis and crush-induced acute kidney injury or by the destruction of existing dialysis facilities, leaving chronic dialysis patients and kidney transplanted patients without access to their r...
journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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更新日期：1998-03-01 00:00:00
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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更新日期：1997-05-01 00:00:00
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1995-07-01 00:00:00
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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doi：
更新日期：2000-07-01 00:00:00
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
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journal_title：Seminars in nephrology
pub_type： 杂志文章
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
doi：
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
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journal_title：Seminars in nephrology
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doi：
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
doi：
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
doi：
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
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journal_title：Seminars in nephrology
pub_type： 杂志文章,评审
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更新日期：2004-09-01 00:00:00
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journal_title：Seminars in nephrology
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