利妥昔单抗治疗儿童激素耐药型肾病综合征的应用进展

Current understanding in treating children with steroid-resistant nephrotic syndrome using rituximab

摘要:

激素耐药型肾病综合征（SRNS）的病因异质性高，可由单基因突变、免疫因素等因素引起。由于SRNS仅占原发性肾病综合征的15%左右，如何对SRNS进行有效治疗与管理仍是临床上的难题。目前指南和学会均推荐将钙调神经磷酸酶抑制剂作为SRNS患者的一线诱导治疗药物，但仍有20%～30%的SRNS患儿在一线治疗后不能缓解。利妥昔单抗（RTX）对频繁复发或类固醇依赖性肾病综合征儿童的疗效已得到证实，但其对SRNS的疗效仍存在争议。该综述旨在总结近几年RTX在SRNS患儿中的应用，以期为RTX治疗SRNS的临床实践与研究提供参考。

Abstract:

Steroid-resistant nephrotic syndrome (SRNS) exhibits high etiological heterogeneity and can be caused by factors such as single-gene mutations and immune-related mechanisms. Since only approximately 15% of patients with idiopathic nephrotic syndrome were SRNS, treatment and management of SRNS remain a major clinical challenge. Current guidelines and professional societies recommend calcineurin inhibitors (CNIs) as the first-line induction therapy for patients with SRNS. Unfortunately, 20% to 30% of children with SRNS still fail to achieve remission with the CNIs treatment. The efficacy of rituximab (RTX) in children with frequent relapses or steroid-dependent nephrotic syndrome has been well established. However, the efficacy of RTX for SRNS remains controversial. This review aims to summarize advancements in the application of RTX in children with SRNS in recent years, with a view to providing a reference for clinical practice and research on RTX for SRNS.