The Pathogenesis of Idiopathic Membranous Nephropathy: A 50-Year Odyssey
The Pathogenesis of Idiopathic Membranous Nephropathy: A 50-Year Odyssey
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DOI：
10.1053/j.ajkd.2010.01.008
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发表时间：
2010-07-01
影响因子：
13.2
通讯作者：
Glassock, Richard J.
中科院分区：
文献类型：
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作者：
Glassock, Richard J.
自从1957年特发性膜性肾病（MN）首次被描述为一种独特的临床病理实体以来，MN一直是实验室和临床研究的主题。这种疾病的实验室模型（特别是主动和被动海曼肾炎）的可用性对研究人员来说是一个布恩。关于免疫存款形成的基本机制，一个必要条件的特发性MN的概念，已经演变，现在已经牢固确立。循环自身抗体（免疫球蛋白G4和免疫球蛋白G1亚类）与足细胞膜-基底膜界面处的肾小球毛细血管壁中的天然抗原或植入抗原相互作用通常被认为是基本的病理生物学机制。因此，MN现在被认为是足细胞病。免疫沉积物引起肾小球毛细血管通透性的改变，可能是通过补体介导的足细胞及其裂孔膜损伤;然而，细胞介导的免疫也可能起作用，并且免疫沉积物的物理存在和基底膜改变也可能参与。在人类特发性MN中起作用的自身抗体系统的确切性质正在迅速被发现。人们希望这50年的漫长旅程将在人类疾病的诊断、预后和治疗方面取得真实的进展。美国肾脏病杂志56：157-167. (C)2010年，美国国家肾脏基金会（National Kidney Foundation，Inc.）
Ever since its first delineation as a distinct clinicopathologic entity in 1957, idiopathic membranous nephropathy (MN) has been the subject of intense laboratory and clinical investigation. The availability of laboratory models (particularly active and passive Heymann nephritis) of this disorder has been a boon to investigators. Concepts regarding the fundamental mechanisms of immune deposit formation, a sine qua non of idiopathic MN, have evolved and now are firmly established. Circulating autoantibodies (immunoglobulin G4 and immunoglobulin G1 subclasses) interacting with antigens native to or planted in the glomerular capillary wall at the podocyte cell membrane-basement membrane interface generally are regarded as the fundamental pathobiological mechanism. Thus, MN now is regarded as a podocytopathy. The immune deposits evoke an alteration in glomerular capillary permeability, probably through complement-mediated injury of the podocyte and its slit-pore membrane; however, cell-mediated immunity also may have a role, and the physical presence of immune deposits and basement membrane alterations also may participate. The exact nature of the autoantibody systems operative in human idiopathic MN is being uncovered rapidly. It is hoped that this 50-year odyssey will culminate in real progress in the diagnosis, prognosis, and therapy for the human disease. Am J Kidney Dis 56:157-167. (C) 2010 by the National Kidney Foundation, Inc.