Division of Nephrology and Dialysis, Ospedale Pediatrico Bambino Gesù-IRCCS, Piazza S. Onofrio 4, 00165, Rome, Italy.
Pediatr Nephrol. 2018 Apr;33(4):573-584. doi: 10.1007/s00467-017-3677-5. Epub 2017 Apr 27.
The pathogenesis of idiopathic nephrotic syndrome (INS) is as yet unknown, but several lines of evidence indicate that the immune system may play a crucial pathogenic role in non-genetic INS. The most important of these are, first, the effectiveness of therapy based on immunosuppression and, second, a vast body of data derived both from experimental models and from patient studies that implicate T cells and more recently B cells as major players in INS pathogenesis. However, recent findings also suggest a direct role of podocytes as drivers of the disease process, and the interplay between the glomerulus and the immune system is still being elucidated. In this review we provide an overview of current knowledge on the role of different components of the immune system in determining disease. Advances in our understanding of the pathogenesis of INS may help drive new, more tailored therapeutic approaches.
特发性肾病综合征（INS）的发病机制尚不清楚，但有几方面的证据表明免疫系统可能在非遗传性 INS 中发挥关键的致病作用。其中最重要的是，首先，基于免疫抑制的治疗的有效性，其次，大量来自实验模型和患者研究的数据表明 T 细胞和最近的 B 细胞是 INS 发病机制中的主要参与者。然而，最近的发现也表明足细胞在疾病过程中具有直接作用，肾小球和免疫系统之间的相互作用仍在阐明之中。在这篇综述中，我们概述了免疫系统不同成分在决定疾病中的作用的现有知识。对 INS 发病机制的理解的进展可能有助于推动新的、更有针对性的治疗方法。
