局灶节段性肾小球硬化的发病机制及其病理亚型的形态发生学基础

Pathogenesis of focal segmental glomerulosclerosis and morphogenesis underlying its pathological variants

局灶节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)是一组病因和发病机制各异的临床病理综合征,临床主要表现为大量蛋白尿甚至肾病综合征,病理特征为部分肾小球发生节段性硬化和足突融合。哥伦比亚病理分型标准按照光镜下改变将FSGS分为5种亚型:塌陷型、顶端型、细胞型、门周型和非特异型。哥伦比亚病理分型标准重在强调临床病理联系,并未完全阐明FSGS的发病机制以及5种亚型的病理生理学基础。近年来关于FSGS的发病机制及其不同病理亚型的形态学发生基础的研究取得了较大的进展,本文就相关内容作一综述。

Focal segmental glomerulosclerosis （FSGS） is defined as a clinicopathological entity of different etiologies and pathogeneses. The clinical manifestations include proteinuria,usually of nephritic range,and are associated with lesions of focal segmental glomerular sclerosis and foot process effacement. The Columbia classification of FSGS based on light microscopic assessment includes five subtypes：collapsing variant,tip variant,cellular variant,perihilar variant,and not otherwise specified. Columbia classification emphasizes the association of clinical with pathologic characteristics. However,both the physiopathology of FSGS and morphogenesis underlying the five morphologic variants are not fully described in Columbia classification. Over the past few years,significant progress has been made in the pathogenesis of FSGS and morphogenesis of diverse variants of FSGS. This review recapitulates recent important advances in the pathogenesis of FSGS and morphogenesis basis underlying the pathological variants of FSGS.