关键词: 补体抑制剂, 儿童风湿病, 系统性红斑狼疮, 狼疮性肾炎, 依库珠单抗

Abstract: Complement inhibitors offer new possibilities for intervention from the beginning in severe pediatric rheumatic diseases；however，in China，no approved indications for such drugs in treating pediatric rheumatic diseases exist yet，and clinicians can only rely on eculizumab for clinical use. This article focuses on practical clinical applications，drawing on existing evidence and expert consensus to systematically elaborate on the target of action of various complement inhibitors and their current approval status in pediatrics. It particularly analyzes the proper identification of indications，optimal initiation timing，and safety management strategies for conditions such as anti-neutrophil cytoplasmic antibody-associated vasculitis，systemic lupus erythematosus（especially lupus nephritis and lupus-associated thrombotic microangiopathy），and catastrophic antiphospholipid syndrome. The article also shares in detail the procedures for confirming vaccination history，antibiotic prophylaxis，alternative approaches for therapeutic drug monitoring，and strategies for discontinuation and dose reduction. Additionally，it emphasizes that lupus nephritis/systemic lupus erythematosus-related thrombotic microangiopathy is a secondary condition；once the underlying disease is under control，active consideration should be given to discontinuing the medication，rather than maintaining it lifelong as is often seen in atypical hemolytic uremic syndrome.

Key words: complement inhibitors, pediatric rheumatic diseases, systemic lupus erythematosus, lupus nephritis, eculizumab