| 摘要: |
| ［摘要］ IgA肾病（IgAN），又称为Berger病，是以肾小球系膜区IgA或IgA免疫复合物沉积为特征的肾小球疾病，是目前全球最常见的原发性肾小球疾病。IgAN的特征因地域、种族、性别及年龄差异而呈现复杂性，提示遗传与环境因素在IgAN中具有关键作用。近年来关于IgAN发病机制的研究在免疫学与分子病理机制方面取得突破性进展：IgAN发病的核心环节是黏膜免疫紊乱驱动的半乳糖缺乏免疫球蛋白A1（Gd-IgA1）免疫复合物形成与清除失衡，导致系膜区IgA特异性沉积与补体激活，是遗传-环境-免疫因素交互作用的结果。近年来提出的抗系膜细胞自身抗体假说为IgAN的病因研究提供了新的视角。该文系统整合IgAN流行病学与发病机制的前沿证据，为IgAN个体化治疗提供理论依据。 |
| 关键词: IgA肾病 流行病学 半乳糖缺乏免疫球蛋白A1 自身免疫 |
| DOI：10.3969/j.issn.1674-3806.2025.12.02 |
| 分类号:R 692 |
| 基金项目:国家自然科学基金项目（编号：82470741，82270752）；湖南省自然科学基金项目（编号：2025JJ30042）；湖南省卫生健康委科研计划项目（编号：A202303057091） |
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| Precision medicine for IgA nephropathy: frontiers in epidemiology and pathogenesis |
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ZHOU Ruijia, LIU Hong
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Department of Nephrology, the Second Xiangya Hospital of Central South University, Changsha 410011, China
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| Abstract: |
| ［Abstract］ Immunoglobulin A(IgA) nephropathy(IgAN), also known as Berger′s disease, is a glomerular disease characterized by the deposition of IgA or IgA immune complexes in the glomerular mesangial area. At present, IgAN is the most common primary glomerular disease worldwide. The characteristics of IgAN are complex due to differences in region, race, gender and age, suggesting that genetic and environmental factors play key roles in IgAN. In recent years, breakthroughs have been made in the research on the pathogenesis of IgAN in the aspects of immunology and molecular pathological mechanisms. The core link in the development of IgAN is the imbalance in the formation and clearance of the galactose-deficient immunoglobulin A1(Gd-IgA1) immune complex driven by mucosal immune dysregulation, resulting in IgA-specific deposition in the mesangial area and complement activation, which is the result of the interplay of genetic-environmental-immune factors. The hypothesis on anti-mesangial cell autoantibodies proposed in recent years has provided a new perspective for research on the etiology of IgAN. This paper systematically integrates the cutting-edge evidence on the epidemiology and pathogenesis of IgAN, providing a theoretical basis for the individualized treatment of IgAN. |
| Key words: Immunoglobulin A(IgA) nephropathy(IgAN) Epidemiology Galactose-deficient immunoglobulin A1(Gd-IgA1) Autoimmunity |
