Xu Zhifeng, Chen Lu, Xiang Huiling, Zhang Chun, Xiong Jing
Department of Nephrology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Geriatrics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Kidney Dis (Basel). 2020 Sep;6(5):330-345. doi: 10.1159/000507704. Epub 2020 Jun 2.
Membranous nephropathy (MN), a major cause of nephrotic syndrome, has attracted people's attention in recent years for its growing prevalence. It is the second or third leading cause of ESRD in patients with primary glomerulonephritis and is the leading glomerulopathy that recurs after kidney transplantation.
MN can be classified as idiopathic membranous nephropathy (IMN) and secondary MN. The discovery of the M-type phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) provides the new diagnostic methods and treatment strategies for IMN on the molecular level. The study on single nucleotide polymorphism of IMN genes, such as the single M-type phospholipase A2 receptor 1 () gene and human leukocyte antigen () gene, explains the pathogenesis of the disease from the perspective of genetics and conforms to the trend of the era of precision medicine.
This review focuses on advances in the pathogenesis of IMN, including molecular and genetic pathogenesis, as well as discussing the diagnostic and treatment guiding value brought by these new discoveries.
膜性肾病(MN)是肾病综合征的主要病因,近年来其患病率不断上升,备受关注。它是原发性肾小球肾炎患者终末期肾病的第二或第三大主要病因,也是肾移植后复发的主要肾小球病。
MN可分为特发性膜性肾病(IMN)和继发性MN。M型磷脂酶A2受体(PLA2R)和含血小板反应蛋白1型结构域7A(THSD7A)的发现为IMN在分子水平上提供了新的诊断方法和治疗策略。对IMN基因单核苷酸多态性的研究,如单M型磷脂酶A2受体1()基因和人类白细胞抗原()基因,从遗传学角度解释了该疾病的发病机制,符合精准医学时代的趋势。
本综述重点关注IMN发病机制的进展,包括分子和遗传发病机制,同时讨论这些新发现带来的诊断和治疗指导价值。