儿童系统性红斑狼疮合并血栓性微血管病的诊治与思考  

Diagnosis,treatment,and reflection on pediatric systemic lupus erythematosus complicated by thrombotic microangiopathy

作　　者：倪佳佳 朱亚菊[1] 金晶[1] 李皎宇 郭桂梅 NI Jiajia;ZHU Yaju;JIN Jing;LI Jiaoyu;GUO Guimei(Department of Pediatric Nephrology,Rheumatology and Immunology,Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine,Shanghai 200092,China)

机构地区：[1]上海交通大学医学院附属新华医院儿肾脏风湿免疫科,上海200092

出　　处：《临床儿科杂志》2025年第2期150-156,共7页Journal of Clinical Pediatrics

摘　　要：患儿,女,12岁,因“双下肢浮肿伴咳嗽40余天,确诊系统性红斑狼疮1个月”就诊。患儿外院肾穿刺结果提示狼疮性肾炎型(Ⅳ型)合并血栓性微血管病(TMA),经甲基泼尼松龙、环磷酰胺、贝利尤单抗治疗后,患儿仍存在重度高血压、肾功能损伤,血小板、血红蛋白、结合珠蛋白进行性下降,乳酸脱氢酶升高,外周血见破碎红细胞,入院后完善血管性血友病因子裂解酶活性正常,抗磷脂抗体阴性,可溶性c5-9明显升高。给予依库珠单抗治疗后,患儿病情好转,血红蛋白、血小板逐步回升,肾功能好转,随访病情稳定。本病例提示临床医师在系统性红斑狼疮(SLE)合并TMA治疗中,需尽早识别补体参与证据,及时启动依库珠单抗治疗,可极大改善患者远期预后。A 12-year-old female SLE patient with lupus nephritis(classⅣ)and TMA was admitted to our hospital.After treatments including blood purification,methylprednisolone and CTX pulse therapy,even belimumab infusion,she still exhibited renal dysfunction and abnormal blood tests including decreased levels in platelet count,hemoglobin and fragmented red blood cells.Further tests showed normal activity of the von Willebrand factor-cleaving protease,negative antiphospholipid antibodies,and significantly elevated soluble c5-9 levels.After initiating treatment with eculizumab,the patient's condition improved.The patient remained stable during a three-month follow-up.This case provides clinicians with insights into the treatment of SLE complicated by TMA.Early identification of complement involvement and prompt initiation of eculizumab treatment can significantly improve the patient's long-term prognosis.

关 键 词：系统性红斑狼疮 血栓性微血管病 非典型尿毒综合征 依库珠单抗 

分 类 号：R725.9[医药卫生—儿科]