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学科主题: 临床医学
题名:
Advances in human antiglomerular basement membrane disease
作者: Cui, Zhao; Zhao, Ming-Hui
刊名: NATURE REVIEWS NEPHROLOGY
发表日期: 2011-12-01
DOI: 10.1038/nrneph.2011.89
卷: 7, 期:12, 页:697-705
收录类别: SCI
文章类型: Review
WOS标题词: Science & Technology
类目[WOS]: Urology & Nephrology
研究领域[WOS]: Urology & Nephrology
关键词[WOS]: ANTI-GBM ANTIBODIES ; EXPERIMENTAL AUTOIMMUNE GLOMERULONEPHRITIS ; NORMAL RENAL-FUNCTION ; T-CELL EPITOPE ; RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS ; NORMAL HUMAN SERA ; IV COLLAGEN ; GOODPASTURE AUTOANTIGEN ; CRESCENTIC GLOMERULONEPHRITIS ; NATURAL AUTOANTIBODIES
英文摘要:

Antiglomerular basement membrane (anti-GBM) disease is an autoimmune disorder that mostly presents as raised titers of antibodies against the GBM, rapidly progressive glomerulonephritis and pulmonary hemorrhage. The disease is caused by antibodies against noncollagenous domain of alpha 3 chain of type IV collagen, which contains the epitopes E(A) and E(B). The humoral and cellular immunity contributing to the initiation of anti-GBM disease has been extensively studied as a model for autoimmune diseases, although most of the data come from animal studies. The disease is rare, but diagnoses have been made in hundreds of patients. Substantial advances have been made in the understanding of human anti-GBM disease, and it can be treated successfully. In this Review we summarize the current knowledge on the prevalence, clinical manifestations, treatment and outcomes of human anti-GBM disease. We discuss findings on pathogenesis from human studies, with close attention to disease initiation and the immunological features of progression from quiescent autoimmune homeostasis in healthy individuals to fulminant anti-GBM disease. Further studies on autoreactive T cells are expected to clarify specific features of human anti-GBM disease and could lead to the development of new therapies.

语种: 英语
所属项目编号: 81021004
项目资助者: Natural Science Fund of China
WOS记录号: WOS:000297926800005
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/56172
Appears in Collections:北京大学第一临床医学院_肾脏内科_期刊论文

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作者单位: Peking Univ, Hosp 1, Key Lab Renal Dis, Renal Div,Dept Med,Inst Nephrol,Minist Hlth China, Beijing 100034, Peoples R China

Recommended Citation:
Cui, Zhao,Zhao, Ming-Hui. Advances in human antiglomerular basement membrane disease[J]. NATURE REVIEWS NEPHROLOGY,2011,7(12):697-705.
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