Tedesco Martina, Delbarba Elisa, Izzi Claudia, Scolari Francesco, Alberici Federico
Università degli Studi di Brescia, Dipartimento di Specialità Medico-Chirurgiche, Scienze Radiologiche e Sanità Pubblica (DSMC), Brescia, Italia; ASST Spedali Civili, Brescia, Italia; Dipartimento di scienze biomediche e cliniche 'Luigi Sacco', Milano, Italia.
ASST Spedali Civili, Brescia, Italia.
G Ital Nefrol. 2020 Aug 11;37(4):2020-vol4.
Focal segmental glomerulosclerosis (FSGS) is a pathological spectrum subtended by heterogeneous etiologies. A good knowledge of FSGS and its causes should be included in the nephrologists' clinical background, as it deeply influences the subsequent management of the affected patients. In fact, while immunosuppressive treatment should be considered in idiopathic FSGS, the treatment of secondary forms should primarily aim at curing or containing the underpinning etiologic factors. Furthermore, in contrast to secondary FSGS, idiopathic FSGS tends to relapse after kidney transplantation. Although FSGS has a wide spectrum of etiologies, several pathogenetic "moments" are shared. Furthermore, recent studies have identified a pool of glomerular cells potentially capable of regenerating lost podocytes; these cells might represent a promising therapeutic target. The primary aim of this review is to describe the etiologic factors associated with FSGS, with a focus on the main pathogenetic mechanisms involved in its development.
局灶节段性肾小球硬化（FSGS）是一种由多种不同病因引起的病理类型。肾病学家的临床知识储备中应充分了解FSGS及其病因，因为这对后续受影响患者的治疗管理有着深远影响。事实上，虽然特发性FSGS应考虑采用免疫抑制治疗，但继发性FSGS的治疗主要目标应是治愈或控制潜在病因。此外，与继发性FSGS不同，特发性FSGS在肾移植后容易复发。尽管FSGS病因广泛，但有几个致病“环节”是共有的。此外，最近的研究发现了一组可能能够再生丢失足细胞的肾小球细胞；这些细胞可能是一个有前景的治疗靶点。本综述的主要目的是描述与FSGS相关的病因，重点关注其发生发展过程中的主要致病机制。
