Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy.
Unit of Nephrology and Dialysis, Department of Medicine, Azienda Socio-Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy.
Nephron. 2020;144(1):30-35. doi: 10.1159/000501858. Epub 2019 Jul 23.
Patients with membranous nephropathy (MN) and persistent nephrotic syndrome (NS) are at increased risk of -progression to end-stage renal disease. The discovery of -autoantibodies against the podocyte-expressed M-type phospholipase A2 receptor (PLA2R) provided a clear pathophysiological rationale for interventions targeting the B-cell lineage to prevent antibody production and subepithelial immune-complex deposition. The anti-CD20 monoclonal antibodies, rituximab and ofatumumab, are safe and achieve remission of NS in approximately two-thirds of patients with MN. In patients with PLA2R-related MN, remission can be predicted by anti-PLA2R antibody depletion, and faster depletion is associated with earlier reduction of proteinuria and improved nephroprotection. Selective apheresis methods, such as double-filtration plasmapheresis (DFPP), may accelerate the clearance of autoreactive antibodies and at the same time avoid the side effects of plasma-exchange. In this preliminary, explorative, proof-of-concept study, we observed that in patients with PLA2R-related MN, NS and high antibody levels, ofatumumab-induced B-cell depletion followed by DFPP accelerated anti-PLA2R depletion compared to anti-CD20 monotherapy. This therapeutic regimen was safe and well tolerated. These observations may provide the background for controlled trials aimed at formally testing whether the addition of DFPP to anti-CD20 therapy could offer a novel therapeutic option, especially for patients with more severe MN.
膜性肾病(MN)和持续性肾病综合征(NS)患者进展为终末期肾病的风险增加。抗足细胞表达的 M 型磷脂酶 A2 受体(PLA2R)自身抗体的发现为针对 B 细胞系的干预提供了明确的病理生理学依据,以防止抗体产生和上皮下免疫复合物沉积。抗 CD20 单克隆抗体利妥昔单抗和奥法妥珠单抗安全,可使大约三分之二的 MN 患者 NS 缓解。在 PLA2R 相关 MN 患者中,抗 PLA2R 抗体耗竭可预测缓解,更快的耗竭与蛋白尿更早减少和更好的肾脏保护相关。选择性血浆清除方法,如双重滤过血浆置换(DFPP),可能会加速自身反应性抗体的清除,同时避免血浆置换的副作用。在这项初步的、探索性的、概念验证研究中,我们观察到在 PLA2R 相关 MN、NS 和高抗体水平的患者中,与抗 CD20 单药治疗相比,奥法妥珠单抗诱导的 B 细胞耗竭后进行 DFPP 可加速抗 PLA2R 耗竭。该治疗方案安全且耐受良好。这些观察结果可能为旨在正式测试 DFPP 联合抗 CD20 治疗是否可为更严重的 MN 患者提供新的治疗选择的对照试验提供背景。