Du Yating, Cheng Tingzhu, Liu Chenxuan, Zhu Tingting, Guo Chuan, Li Shen, Rao Xiangrong, Li Jinpu
Department of Nephrology, Guang'anmen Hospital, China Academy of Chinese Medical Sciences, Beijing 100053, China.
Diagnostics (Basel). 2023 Jan 13;13(2):303. doi: 10.3390/diagnostics13020303.
Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide, with varied clinical and histopathological features between individuals, particularly across races. As an autoimmune disease, IgAN arises from consequences of increased circulating levels of galactose-deficient IgA1 and mesangial deposition of IgA-containing immune complexes, which are recognized as key events in the widely accepted "multi-hit" pathogenesis of IgAN. The emerging evidence further provides insights into the role of genes, environment, mucosal immunity and complement system. These developments are paralleled by the increasing availability of diagnostic tools, potential biomarkers and therapeutic agents. In this review, we summarize current evidence and outline novel findings in the prognosis, clinical trials and translational research from the updated perspectives of IgAN pathogenesis.
免疫球蛋白A肾病（IgAN）是全球最常见的原发性肾小球肾炎，个体之间，尤其是不同种族之间，其临床和组织病理学特征各异。作为一种自身免疫性疾病，IgAN是由循环中缺乏半乳糖的IgA1水平升高以及含IgA免疫复合物在系膜区沉积所导致的，这些被认为是IgAN广泛接受的“多打击”发病机制中的关键事件。新出现的证据进一步揭示了基因、环境、黏膜免疫和补体系统的作用。这些进展伴随着诊断工具、潜在生物标志物和治疗药物的日益增多。在本综述中，我们从IgAN发病机制的最新视角总结了当前证据，并概述了在预后、临床试验和转化研究方面的新发现。
