Idiopathic membranous nephropathy (IMN) is a pathological pattern of glomerular damage caused by an autoimmune response. Immune complex deposition, thickness of glomerular basement membrane, and changes in the podocyte morphology are responsible for the development of proteinuria, which is caused by the targeted binding of auto-antibodies to podocytes. Several auto-antigens have recently been identified in IMN, including M-type receptor for secretory phospholipase A2 (PLA2R1), thrombospondin type-1 domain-containing 7A (THSD7A), and neural epidermal growth factor-like 1 protein (NELL-1). The measurement of peripheral circulating antibodies has become an important clinical reference index. However, some clinical features of IMN remain elusive and need to be further investigated, such as the autoimmunity initiation, IgG4 predominance, spontaneous remission, and the unique glomerular lesion. As these unresolved issues are closely related to clinical practice, we have proposed a hypothetical pathogenesis model of IMN. Induced by environmental stimuli or other causes, the PLA2R1 antigen and/or THSD7A antigen exposed to extrarenal tissues, such as lungs, then produce the auto-antibodies that target and cause damage to the podocytes in circulation. In this review, we highlighted the potential association between environmental stimuli, immune activity, and glomerular lesions, the underlying basis for spontaneous immune and proteinuria remission.

特发性膜性肾病（IMN）是由自身免疫反应引起的肾小球损害的病理模式。免疫复合物的沉积，肾小球基底膜的厚度以及足细胞形态的变化与蛋白尿的发展有关，这是由自身抗体与足细胞的靶向结合引起的。最近在IMN中发现了几种自身抗原，包括分泌型磷脂酶A2的M型受体（PLA2R1），含血小板反应蛋白1型域的7A（THSD7A）和神经表皮生长因子样1蛋白（NELL-1）。 。外周循环抗体的测量已成为重要的临床参考指标。但是，IMN的某些临床特征仍然难以捉摸，需要进一步研究，例如自身免疫起始，IgG4优势，自发缓解，并有独特的肾小球病变。由于这些未解决的问题与临床实践密切相关，因此我们提出了IMN的假设发病机制模型。由环境刺激或其他原因诱导，暴露于肾外组织（如肺）的PLA2R1抗原和/或THSD7A抗原随后产生靶向并造成循环足细胞损害的自身抗体。在这篇综述中，我们强调了环境刺激，免疫活性和肾小球病变之间的潜在联系，这是自发免疫和蛋白尿缓解的基础。暴露于肾外组织（例如肺）的PLA2R1抗原和/或THSD7A抗原，然后产生靶向循环并破坏足细胞的自身抗体。在这篇综述中，我们强调了环境刺激，免疫活性和肾小球病变之间的潜在联系，这是自发免疫和蛋白尿缓解的基础。暴露于肾外组织（例如肺）的PLA2R1抗原和/或THSD7A抗原，然后产生靶向循环并破坏足细胞的自身抗体。在这篇综述中，我们强调了环境刺激，免疫活性和肾小球病变之间的潜在联系，这是自发免疫和蛋白尿缓解的基础。