Abstract：
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.
摘要：
免疫球蛋白A肾病（IgAN）是全球最常见的原发性肾小球肾炎，个体之间具有不同的临床和组织病理学特征，尤其是在种族中。作为一种自身免疫性疾病，IgAN起因于半乳糖缺陷型IgA1的循环水平增加和含有IgA的免疫复合物的系膜沉积的后果。它们被认为是IgAN广泛接受的“多命中”发病机制中的关键事件。新出现的证据进一步提供了对基因作用的见解，环境,粘膜免疫和补体系统。这些发展伴随着诊断工具的日益普及，潜在的生物标志物和治疗剂。在这次审查中,我们总结了目前的证据并概述了预后的新发现，从IgAN发病机制的最新视角进行临床试验和转化研究。
