患儿，女，10岁10个月，因乏力、面色苍白3个月，皮疹2周就诊。完善检查发现患儿贫血、血小板减少、血尿蛋白尿合并肾功能不全、低补体血症、多浆膜腔积液、抗核抗体及抗双链DNA抗体阳性，初步诊断为系统性红斑狼疮、狼疮性肾炎。给予糖皮质激素冲击、静脉注射免疫球蛋白冲击及血浆置换治疗，效果欠佳。住院过程中患儿持续贫血、血小板减少、肾功能异常及乳酸脱氢酶升高，补体因子H、I均下降，C3转化酶抗体升高，ADAMTS13活性正常，考虑诊断为系统性红斑狼疮继发补体介导的血栓性微血管病，给予依库珠单抗2次（600 mg/次）治疗后病情缓解。系统性红斑狼疮合并血栓性微血管病患者病情重，预后差，早期识别、积极干预可改善预后。

The patient was a girl aged 10 years and 10 months, with weakness, pale complexion, and rash as the initial presentation. She had the manifestations of anemia, thrombocytopenia, hematuria-proteinuria with renal insufficiency, hypocomplementemia, polyserositis, and positive anti-nuclear antibody and anti-dsDNA antibody. The girl was initially diagnosed with systemic lupus erythematosus and lupus nephritis. She demonstrated a suboptimal response to methylprednisolone pulse therapy, intravenous immunoglobulin administration, and therapeutic plasma exchange. She had persistent anemia, thrombocytopenia, abnormal renal function, elevated lactate dehydrogenase, decreased complement factors H and I, increased antibodies to C3 converting enzyme, and normal ADAMTS13 activity. She was diagnosed with complement-mediated hemolytic thrombotic microangiopathy secondary to systemic lupus erythematosus. The patient's condition improved after treatment with two doses of eculizumab (600 mg per dose). Patients with systemic lupus erythematosus complicated by thrombotic microangiopathy often have a severe disease course and poor prognosis; therefore, early recognition and aggressive intervention are crucial for improving outcomes.