抗 PLA2R 相关特发性膜性肾病的治疗性浆血疗法:病例系列。

IF 1.4 4区 医学 Q4 HEMATOLOGY Journal of Clinical Apheresis Pub Date : 2024-06-27 DOI:10.1002/jca.22134
Dario Musone, Valentina Nicosia, Riccardo D'Alessandro, Marco Andrietti, Paolo Francesco Steri, Carolina Ruosi, Sofia Giuliana, Claudia Elefante, Pina Cuccurullo, Antonio Treglia
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引用次数: 0

摘要

膜性肾病是非糖尿病成人肾病综合征(NS)最常见的病因;80%的患者是特发性肾病(PMN)。膜性肾病有自身免疫发病机制,70%-85%的患者荚膜抗原 PLA2R 的抗体滴度增高。抗 PLA2R 抗体在病因学、预后和预测方面的作用已得到证实。标准疗法包括抗 CD20 单克隆抗体利妥昔单抗(RTX)与类固醇或免疫抑制剂(根据肾功能进行性丧失的风险而定)。免疫抑制剂疗法可能会导致严重的不良反应,从而导致方案中止。鉴于抗PLA2R的关键致病作用,通过血浆置换清除血清中的抗PLA2R可对NS产生有益影响,尤其是对不需要或不能耐受标准疗法的患者。在本系列报道中,我们介绍了采用 RTX 加血浆置换疗法治疗的三例 PMN 抗 PLA2R 相关病例,并展示了 RTX 对抗 PLA2R 滴度和临床结果的总体有效作用。
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Therapeutic plasmapheresis in idiopathic membranous nephropathy anti-PLA2R-related: A case series

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.

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来源期刊
CiteScore
2.80
自引率
13.30%
发文量
70
审稿时长
>12 weeks
期刊介绍: The Journal of Clinical Apheresis publishes articles dealing with all aspects of hemapheresis. Articles welcomed for review include those reporting basic research and clinical applications of therapeutic plasma exchange, therapeutic cytapheresis, therapeutic absorption, blood component collection and transfusion, donor recruitment and safety, administration of hemapheresis centers, and innovative applications of hemapheresis technology. Experimental studies, clinical trials, case reports, and concise reviews will be welcomed.
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