摘要IgA肾病（IgA nephropathy，IgAN）是目前全球范围内最常见的原发性肾小球肾炎，20%～40%的患者在诊断后20年内进展为终末期肾病。IgAN的发病机制尚不明确，临床治疗主要以控制病情进展为主，缺乏特异性治疗方案。围绕半乳糖缺乏的IgA1（galactose-deficient IgA1，Gd-IgA1）开展的一系列研究提示IgAN发病涉及多个环节。该文从IgA1的结构特征、IgAN患者体内Gd-IgA1抗体及含Gd-IgA1免疫复合物、Gd-IgA1免疫复合物在肾脏中的沉积、Gd-IgA1免疫复合物沉积对肾脏的损伤、补体在IgAN中的作用、IgA肾病的基因组学及黏膜免疫与IgAN等方面，综述了IgAN发病机制的研究进展，为进一步的研究和临床治疗提供线索和思路。

abstractsIgA nephropathy (IgAN) is currently the most common primary glomerulonephritis worldwide, with 20%-40% of patients progressing to end-stage renal disease within 20 years of diagnosis. At present, the pathogenesis of IgAN is not clear, and clinical treatment is mainly to control the progression, without specific treatment plan. A series of studies on galactose-deficient IgA1 (Gd-IgA1) suggest that the pathogenesis of IgAN involves multiple links. This review summarizes the research progress on the pathogenesis of IgAN, covering the structure characteristics of IgA1, Gd-IgA1 antibodies and Gd-IgA1 immune complexes in IgAN patients, the deposition of Gd-IgA1 immune complexes in the kidneys, kidney damage following the deposition of Gd-IgA1 immune complexes, the role of complement in IgAN, the genomics of IgAN, and mucosal immunity in IgAN, providing clues and insights for further research and clinical treatment.