依库珠单抗在儿童系统性红斑狼疮治疗中的应用及思考

doi:10.12372/jcp.2025.25e0192

摘要/Abstract

摘要：

系统性红斑狼疮（SLE）是儿童最常见的系统性自身免疫性疾病之一，血栓性微血管病（TMA）是SLE的严重并发症之一。并发TMA的SLE往往预后不佳、病死率较高。C5抑制剂依库株单抗是成人和儿童不典型溶血尿毒综合征（aHUS）的首选治疗药物。目前在临床儿童SLE治疗中，依库株单抗被用于SLE合并TMA的治疗，取得了积极的效果。本文对目前发表的关于应用依库珠单抗治疗儿童SLE的研究进行了梳理和回顾，并从儿科肾脏专科医师的角度，就依库株单抗治疗儿童SLE的适应证选择、治疗时机、疗程和不良反应等方面，阐述了个人的一些见解。依库珠单抗治疗儿童SLE中的一些问题，尚有待未来多中心、大样本、以及多层次治疗方案的随机对照研究进一步解答。

关键词: 系统性红斑狼疮, 血栓性微血管病, 不典型溶血尿毒综合征, 依库珠单抗, 儿童

Abstract:

Systemic lupus erythematosus (SLE) is one of the most common systemic autoimmune diseases in children. Thrombotic microangiopathy (TMA) is one of the serious complications of SLE. SLE patients with concurrent TMA often have a poor prognosis and a higher mortality rate. Eculizumab is a humanized monoclonal anti-C5 antibody, which is the preferred treatment for atypical hemolytic uremic syndrome (aHUS) in both adults and children. Currently, Eculizumab is used for the clinical treatment of pediatric SLE combined with TMA, and has achieved positive outcomes. This article reviews and summarizes current published studies on the application of eculizumab in treating pediatric SLE. From the perspective of pediatric nephrologists, it elaborates on some personal insights regarding the selection of indications, treatment timing, treatment course, and adverse reactions of eculizumab in the treatment of pediatric SLE. Some issues in the treatment of pediatric SLE with eculizumab still need to be further addressed by future multi-center, large-sample, and multi-level treatment regimen randomized controlled studies.

Key words: systemic lupus erythematosus, thrombotic microangiopathy, atypical hemolytic-uremic syndrome, eculizumab, child

中图分类号:

张宏文. 依库珠单抗在儿童系统性红斑狼疮治疗中的应用及思考[J]. 临床儿科杂志, 2025, 43(6): 403-407.

ZHANG Hongwen. Application and consideration of eculizumab in the treatment of childhood systemic lupus erythematosus[J]. Journal of Clinical Pediatrics, 2025, 43(6): 403-407.

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