Nephrology Section, Department of Medicine, Boston Medical Center, Boston, MA.
Division of Renal Diseases and Hypertension, Department of Internal Medicine, McGovern Medical School, UTHealth Houston, Houston, TX.
Adv Kidney Dis Health. 2024 Jul;31(4):299-308. doi: 10.1053/j.akdh.2024.04.004.
Membranous nephropathy is a major etiology of nephrotic syndrome in adults and less frequently in children. Circulating antibodies to intrinsic podocyte antigens, such as M-type phospholipase A2 receptor, or to extrinsic proteins accumulate beneath the podocyte to cause damage via complement activation and/or other mechanisms. The availability of clinical testing for autoantibodies to M-type phospholipase A2 receptor has allowed noninvasive diagnosis of this form of membranous nephropathy and a means to monitor immunologic activity to guide immunosuppressive therapy. Treatment of membranous nephropathy includes optimal supportive care with renin-angiotensin-system blockers, lipid-lowering agents, diuretics, lifestyle changes, and additional immunosuppressive therapy in patients with an increased risk of progression to kidney failure. Rituximab has been recognized as a first-line immunosuppressive therapy for most membranous nephropathy patients with an increased risk of progressive disease, except those with life-threatening nephrotic syndrome or rapidly deteriorating kidney function from membranous nephropathy. This article discusses the major and minor antigens described in membranous nephropathy, the natural history of the disease, and guidelines for clinical management and immunosuppressive treatment.
膜性肾病是成人肾病综合征的主要病因，在儿童中则较少见。循环中的抗固有足细胞抗原（如 M 型磷脂酶 A2 受体）或外在蛋白的抗体在足细胞下蓄积，通过补体激活和/或其他机制引起损伤。M 型磷脂酶 A2 受体自身抗体的临床检测手段的出现，使得这种形式的膜性肾病可以进行无创诊断，并可以监测免疫活性以指导免疫抑制治疗。膜性肾病的治疗包括最佳支持治疗，如肾素-血管紧张素系统阻滞剂、降脂药物、利尿剂、生活方式改变，以及对有进展为肾衰竭风险的患者进行额外的免疫抑制治疗。除了有生命危险的肾病综合征或因膜性肾病导致肾功能迅速恶化的患者外，利妥昔单抗已被确认为大多数有进展性疾病风险的膜性肾病患者的一线免疫抑制治疗药物。本文讨论了膜性肾病中描述的主要和次要抗原、疾病的自然史，以及临床管理和免疫抑制治疗的指南。
