Membranous nephropathy (MN) is characterized histomorphologically by the presence of immune deposits in the subepithelial space of the glomerular filtration barrier; its clinical hallmarks are nephrotic range proteinuria with oedema. In patients with primary MN, autoimmunity is driven by circulating autoantibodies that bind to one or more antigens on the surface of glomerular podocytes. Compared with other autoimmune kidney diseases, the understanding of the pathogenesis of MN has substantially improved in the past decade, thanks to the discovery of pathogenic circulating autoantibodies against phospholipase A₂ receptor 1 (PLA₂R1) and thrombospondin type 1 domain-containing protein 7A (THSD7A). The subsequent identification of more proteins associated with MN, some of which are also endogenous podocyte antigens, might further advance the clinical characterization of MN, including its diagnosis, treatment and prognosis. Insights from studies in patients with MN, combined with the development of novel in vivo and in vitro experimental models, have potential to improve the management of patients with MN. Characterizing the interaction between autoimmunity and local glomerular lesions provides an opportunity to develop more specific, pathogenesis-based treatments.

膜性肾病 (MN) 的组织形态学特征是在肾小球滤过屏障的上皮下间隙中存在免疫沉积物；其临床特征是肾病范围蛋白尿伴水肿。在原发性 MN 患者中，自身免疫是由与肾小球足细胞表面的一种或多种抗原结合的循环自身抗体驱动的。与其他自身免疫性肾脏疾病相比，由于发现了针对磷脂酶 A ₂受体 1 (PLA ₂R1) 和含有 1 型血小板反应蛋白结构域的蛋白 7A (THSD7A)。随后鉴定出更多与 MN 相关的蛋白质，其中一些也是内源性足细胞抗原，可能会进一步推进 MN 的临床表征，包括其诊断、治疗和预后。来自 MN 患者研究的见解，结合新型体内和体外实验模型的开发，有可能改善 MN 患者的管理。表征自身免疫和局部肾小球病变之间的相互作用为开发更具体的、基于发病机制的治疗提供了机会。