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自身免疫性肾病。

Autoimmune kidney diseases.

机构信息

Section of Nephrology, Department of Clinical Sciences, Lund University, Sweden.

出版信息

Autoimmun Rev. 2010 Mar;9(5):A366-71. doi: 10.1016/j.autrev.2009.11.007. Epub 2009 Nov 10.

DOI:10.1016/j.autrev.2009.11.007
PMID:19906361
Abstract

The second most common cause of chronic renal failure is glomerulonephritis, which is a collective term used for numerous diseases with the common denominator of histological renal inflammation emanating from the glomerular tuft. Whether all forms of glomerulonephritis should be considered as autoimmune disease is debatable, but immune mechanisms are important in all of them. This review focuses on four relatively well delineated forms of primary glomerulonephritis: Goodpastures or anti-GBM disease, IgA nephritis, membranous nephropathy and membranoproliferative glomerulonephritis. The autoantibodies are directed either to molecules within the glomeruli, such as the glomerular basement membrane in anti-GBM disease and to the podocytes in membranous glomerulonephritis, or to components of the immune system such as C3 convertase in membranoproliferative glomerulonephritis and IgA in IgA nephritis. Differences in diagnostic practices and classification controversies obscure comparative epidemiological studies, but there seem to be huge differences between incidence rates between countries and over time, both genetic factors and infections seem to matter but strong indications for a role of other environmental factors are still lacking.

摘要

慢性肾衰竭的第二个常见原因是肾小球肾炎,这是一个用于描述许多疾病的术语,这些疾病的共同特征是从肾小球簇发出的组织学肾炎症。所有形式的肾小球肾炎是否都应被视为自身免疫性疾病尚有争议,但免疫机制在所有这些疾病中都很重要。本篇综述重点介绍四种相对明确的原发性肾小球肾炎:Goodpasture 病或抗肾小球基底膜病、IgA 肾病、膜性肾病和膜增生性肾小球肾炎。自身抗体针对的是肾小球内的分子,如抗肾小球基底膜病中的肾小球基底膜和膜性肾小球肾炎中的足细胞,或者针对免疫系统的成分,如膜增生性肾小球肾炎中的 C3 转化酶和 IgA 肾病中的 IgA。诊断实践和分类争议的差异使得比较流行病学研究变得复杂,但不同国家和不同时间的发病率似乎存在巨大差异,遗传因素和感染似乎很重要,但其他环境因素的作用仍缺乏有力证据。

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