Unità di Nefrologia e Dialisi, Fondazione Ca' Granda IRCCS Ospedale Maggiore Policlinico di Milano, Milan, Italy.
Divisione di Nefrologia e Dialisi - Padiglione Croff, Fondazione Ca' Granda, IRCCS Ospedale Maggiore Policlinico, Via della Commenda 15, 20122, Milan, Italy.
J Nephrol. 2018 Feb;31(1):37-45. doi: 10.1007/s40620-017-0402-1. Epub 2017 May 3.
Focal and segmental glomerulosclerosis (FSGS) is a histological pattern clinically characterized by nephrotic proteinuria, hypoalbuminemia, edema and dyslipidemia. Approximately 50% of patients progress to end-stage renal disease within 5-10 years, particularly those not responding to the therapies. FSGS pathogenesis is largely unknown and therapy is symptomatic and unspecific. The podocyte is considered as the pathogenetic main target and FSGS is now categorized as a podocytopathy together with minimal change disease, diffuse mesangial proliferation and collapsing glomerulonephritis. This paper provides an overview on the treatment of idiopathic FSGS in adults, citing the latest published trials and the most reliable pathogenetic hypotheses of the disease. A large part of the review then focuses on emerging therapies, specifying for each new drug the assumed mechanism of action and the data available in the literature on the drug's use in experimental animals and humans.
局灶节段性肾小球硬化症（FSGS）是一种组织学表现，临床上以肾病性蛋白尿、低白蛋白血症、水肿和血脂异常为特征。大约 50%的患者在 5-10 年内会进展为终末期肾病，特别是那些对治疗无反应的患者。FSGS 的发病机制在很大程度上尚不清楚，治疗主要是对症治疗且缺乏特异性。足细胞被认为是主要的致病靶点，FSGS 现在与微小病变性肾病、弥漫性系膜增殖和塌陷性肾小球肾炎一起被归类为足细胞病。本文概述了成人特发性 FSGS 的治疗方法，引用了最新发表的临床试验和该疾病最可靠的发病机制假说。然后，大部分内容集中在新兴治疗方法上，为每种新药具体说明其作用机制以及该药物在实验动物和人类中的应用的文献中的数据。
