Abstract：
Membranous nephropathy is the most common cause of nephrotic syndrome (NS) in non-diabetic adults; in 80% of patients it is idiopathic (PMN). PMN has an autoimmune pathogenesis, 70%-85% of patients have increased titer of antibodies to the podocyte membrane antigen PLA2R. The etiological, prognostic and predictive role of the Ab anti-PLA2R is demonstrated. Standard therapy consists in anti-CD20 monoclonal antibody rituximab (RTX) combined with steroids or immunosuppressants according to the risk of progressive loss of kidney function. The immunosuppressive therapies are potentially associated to severe adverse events that lead to protocol suspension. Given their pivotal pathogenetic role, serum clearance of anti-PLA2R with plasmapheresis could have a beneficial impact on NS, particularly in patients not requiring or tolerating standard therapies. In this series, we present three cases of PMN anti-PLA2R related treated with a RTX plus plasmapheresis approach and demonstrate its overall effective role on anti-PLA2R titer and clinical outcomes.
摘要：
膜性肾病是非糖尿病成人肾病综合征（NS）的最常见原因；在80％的患者中，它是特发性（PMN）。PMN具有自身免疫性发病机制，70%-85%的患者足细胞膜抗原PLA2R的抗体滴度升高。病因学，证明了Ab抗PLA2R的预后和预测作用。标准治疗包括抗CD20单克隆抗体利妥昔单抗（RTX）与类固醇或免疫抑制剂的联合，根据肾功能进行性丧失的风险。免疫抑制疗法可能与导致方案暂停的严重不良事件有关。鉴于它们的关键致病作用，血浆置换抗PLA2R的血清清除可能对NS产生有益影响，特别是在不需要或不耐受标准疗法的患者中。在这个系列中,我们介绍了3例PMN抗PLA2R相关病例,这些病例采用RTX加血浆置换方法治疗,并证明了其在抗PLA2R滴度和临床结局方面的总体有效作用.
