IgA nephropathy: morphologic expression and pathogenesis.
IgA nephropathy: morphologic expression and pathogenesis.
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IgA 肾病：形态表达和发病机制。
DOI：
10.1016/s0272-6386(12)81011-0
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发表时间：
1994
期刊：
影响因子：
--
通讯作者：
Emancipator,SN
中科院分区：
文献类型：
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作者：
Emancipator,SN
免疫球蛋白A肾病（IgAN）定义为肾小球肾炎（GN），其中伊加是沉积在肾小球中的主要免疫球蛋白。1-5除了在没有任何全身性疾病的情况下发生的原发性IgAN之外，还认识到继发于各种疾病的IgAN，特别是肝胆疾病和粘膜上皮或造血细胞的瘤形成。Schonlein和Henoch（SHP）所描述的紫癜综合征的肾炎成分也与原发性IgAN在临床、形态学和免疫学上有密切的相似性，大多数研究者认为IgAN和SHP是相关疾病。除非另有说明，IgAN是指原发性疾病。IgAN在亚洲地区比澳大利亚和欧洲更常见，在北美较少见。男性与女性的比例表明，男性更容易受到这种疾病的影响，特别是在生命的第二和第三个十年。IgAN最初被认为是一种罕见的良性疾病，现在被认为是世界上最常见的GN形式，也是终末期肾病的主要原因。本文简要综述了肾功能和结构的变化，着重介绍了本病的病理特点，并从机理上探讨了这些变化的原因和基础。
IMMUNOGLOBULIN A nephropathy (IgAN) is defined as glomerulonephritis (GN) in which IgA is the predominant immunoglobulin deposited in the glomerulus. 1-5 In addition to primary IgAN, which occurs in the absence of any systemic disease, IgAN secondary to a variety of diseases, especially hepatobiliary disease and neoplasia of mucosal epithelial or hematopoietic cells, is recognized. I, 5 The nephritis component of the purpuric syndrome described by Schonlein and Henoch (SHP) also bears close clinical, morphologic, and immunologic similarity to primary IgAN, and most investigators consider IgAN and SHP to be related diseases, In this review, secondary IgAN and SHP are discussed only in passing. Unless otherwise specified, IgAN refers to primary disease.IgAN is more frequent in the Asian region than in Australia and Europe, and less common in North America. The male to female ratio indicates that males are more likely to be affected by the disease, particularly in the second and third decade oflife. IgAN, initially regarded as an unusual and benign disease, is now considered as the most common form of GN in the world and a major cause of end-stage kidney disease. The purpose here is to review briefly the changes in renal function and structure, with an emphasis on the pathologic features of the disease, and then to consider the reasons for and basis of these changes mechanistically.