Unit of Nephrology and Dialysis, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy.
Pediatric Nephrology Unit, AOU Policlinic "G Martino", University of Messina, 98125 Messina, Italy.
Medicina (Kaunas). 2023 Nov 14;59(11):1997. doi: 10.3390/medicina59111997.
Membranous nephropathy is an autoimmune disease affecting the glomeruli and is one of the most common causes of nephrotic syndrome. In the absence of any therapy, 35% of patients develop end-stage renal disease. The discovery of autoantibodies such as phospholipase A2 receptor 1, antithrombospondin and neural epidermal growth factor-like 1 protein has greatly helped us to understand the pathogenesis and enable the diagnosis of this disease and to guide its treatment. Depending on the complications of nephrotic syndrome, patients with this disease receive supportive treatment with diuretics, ACE inhibitors or angiotensin-receptor blockers, lipid-lowering agents and anticoagulants. After assessing the risk of progression of end-stage renal disease, patients receive immunosuppressive therapy with various drugs such as cyclophosphamide, steroids, calcineurin inhibitors or rituximab. Since immunosuppressive drugs can cause life-threatening side effects and up to 30% of patients do not respond to therapy, new therapeutic approaches with drugs such as adrenocorticotropic hormone, belimumab, anti-plasma cell antibodies or complement-guided drugs are currently being tested. However, special attention needs to be paid to the choice of therapy in secondary forms or in specific clinical contexts such as membranous disease in children, pregnant women and patients undergoing kidney transplantation.
膜性肾病是一种影响肾小球的自身免疫性疾病,是肾病综合征最常见的病因之一。在没有任何治疗的情况下,35%的患者会发展为终末期肾病。抗磷脂酶 A2 受体 1、抗凝血酶 3 和神经表皮生长因子样 1 蛋白等自身抗体的发现,极大地帮助我们理解了这种疾病的发病机制,使其得以诊断,并指导其治疗。根据肾病综合征的并发症,患者接受利尿剂、血管紧张素转换酶抑制剂或血管紧张素受体阻滞剂、降脂药和抗凝药等支持治疗。在评估终末期肾病进展风险后,患者接受环磷酰胺、类固醇、钙调神经磷酸酶抑制剂或利妥昔单抗等各种药物的免疫抑制治疗。由于免疫抑制剂可能会导致危及生命的副作用,而且多达 30%的患者对治疗没有反应,目前正在测试新型治疗方法,包括促肾上腺皮质激素、贝利尤单抗、抗浆细胞抗体或补体导向药物。然而,在继发性或特定临床情况下(如儿童、孕妇和肾移植患者的膜性疾病),需要特别注意治疗方案的选择。