塌陷性肾小球病

Collapsing glomerulopathy

塌陷性肾小球病(collapsing glomerulopathy,CG)在病理上属于塌陷型局灶节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)的一种,病理特征表现为局灶或球性毛细血管袢塌陷伴足细胞肥大、增生,同时肾小管间质损伤显著。1979年报道了第一例CG,因其发病原因不清,传统免疫抑制治疗效果差,肾脏存活率低,目前已成为终末期肾病的重要原发病之一。过去20多年,CG发病机制的研究取得了长足进展,近年研究的重点在于CG易感基因筛选,足细胞受损、增生的机制,包括细胞周期调控紊乱、异常信号通路激活等。本文就CG发病机制的最新进展作一简述。

According to the Columbia classification,collapsing glomerulopathy （CG） is an morphologic variant of focal segmental glomerulosclerosis （FSGS）, characterized by segmental or global glomerular capillary loop collapse with marked proliferation and hypertrophy of overlying podocytes. Since the first case of CG was published in 1979, the CG is now becoming an important cause of ESRD because of the poorly understood of underlying mechanism and poor responders to standard therapies. Gratifyingly,the exciting progress has been made in research into the pathogenesis of CG during the past two decades. Recently, the researches have focused on the origin of proliferative podocytes and the underlying mechanism behind the phenomenon. In addtion to discovering susceptibility genes for CG and the growing awareness of the signaling pathways participate in podocytes injure provide important insights into the pathogenesis of CG. This review highlights the recent advances in research into the pathogenesis of CG.