IgA nephropathy (IgAN) is a common form of primary glomerulonephritis and represents an important cause of chronic kidney disease globally, with observational studies indicating that most patients are at risk of developing kidney failure within their lifetime. Several research advances have provided insights into the underlying disease pathogenesis, framed by a multi-hit model whereby an increase in circulating IgA1 that lacks galactose from its hinge region — probably derived from the mucosal immune system — is followed by binding of specific IgG and IgA antibodies, generating immune complexes that deposit within the glomeruli, which triggers inflammation, complement activation and kidney damage. Although treatment options are currently limited, new therapies are rapidly emerging that target different pathways, cells and mediators involved in the disease pathogenesis, including B cell priming in the gut mucosa, the cytokines APRIL and BAFF, plasma cells, complement activation and endothelin pathway activation. As more treatments become available, there is a realistic possibility of transforming the long-term outlook for many individuals with IgAN.

IgA 肾病 （IgAN） 是原发性肾小球肾炎的一种常见形式，是全球慢性肾病的重要原因，观察性研究表明，大多数患者在其一生中都有发生肾衰竭的风险。几项研究进展提供了对潜在疾病发病机制的见解，该模型由多击模型构成，其中循环 IgA1 的增加缺乏其铰链区的半乳糖——可能来自粘膜免疫系统——随后特异性 IgG 和 IgA 抗体结合，产生沉积在肾小球内的免疫复合物，从而引发炎症， 补体激活和肾损伤。尽管目前的治疗选择有限，但针对参与疾病发病机制的不同途径、细胞和介质的新疗法正在迅速出现，包括肠粘膜中的 B 细胞启动、细胞因子 APRIL 和 BAFF、浆细胞、补体激活和内皮素途径激活。随着更多治疗方法的出现，许多 IgAN 患者的长期前景有可能发生改变。