Mechanisms of proteinuria in noninflammatory glomerular diseases.
Mechanisms of proteinuria in noninflammatory glomerular diseases.
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DOI：
10.1016/s0272-6386(12)80260-5
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发表时间：
1993-04
期刊：
影响因子：
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通讯作者：
V. J. Savin
中科院分区：
文献类型：
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作者：
V. J. Savin
在肾病综合征中，引起蛋白尿的起始因素和损伤的近因都没有明确的定义。免疫机制已被假定在微小病变肾病综合征（MCNS），局灶节段性肾小球硬化（FSGS），肾小球硬化与人类免疫缺陷病毒（HIV）感染。在MCNS和FSGS中已经提出了循环因子，尽管尚未确定特定的介质。类固醇治疗后蛋白尿的迅速缓解和MCNS患者免疫反应性的改变已被用于支持免疫机制参与MCNS的发病机制。FSGS和HIV相关肾病通常都是类固醇耐药的。FSGS和HIV相关性肾病的免疫机制分别是移植后早期复发和大量相关免疫异常。基于肾小球电荷中和、足细胞毒性损伤、肾小球成分抗体注射或转基因小鼠异常的肾病综合征实验模型已被用于确定肾小球损伤的机制。本文综述了MCNS，FSGS和HIV相关肾病和肾病综合征的几个实验模型中的生理和免疫异常，并概述了可能导致这些实体肾小球功能障碍的免疫机制和细胞反应。
Neither the initiating factors nor the proximate causes of injury that produce proteinuria in nephrotic syndrome have been clearly defined. Immune mechanisms have been postulated in minimal-change nephrotic syndrome (MCNS), focal segmental glomerular sclerosis (FSGS), and glomerular sclerosis associated with human immunodeficiency virus (HIV) infection. Circulating factors have been proposed in MCNS and FSGS, although no specific mediator has been identified. Prompt remission of proteinuria following steroid treatment and the presence of altered immune responsiveness in patients with MCNS have been used to support the participation of an immune mechanism in the pathogenesis of MCNS. Both FSGS and HIV-related nephropathy are usually steroid-resistant. Immune mechanisms are postulated in FSGS because of early recurrence after transplantation, and in HIV-related nephropathy because of the numerous associated immune abnormalities. Experimental models of nephrotic syndrome based on neutralization of glomerular charge, toxic injury to podocytes, injection of antibodies to glomerular components, or abnormalities in transgenic mice have been used to define mechanisms of glomerular injury. This review summarizes physiologic and immunologic abnormalities in MCNS, FSGS, and HIV-associated nephropathy and in several experimental models of nephrotic syndrome, and outlines the immunologic mechanisms and cellular reactions that may be responsible for glomerular dysfunction in these entities.