儿童系统性红斑狼疮合并血栓性微血管病临床特点及诱导缓解治疗疗效

Clinical characteristics of systemic lupus erythematosus complicated with thrombotic microangiopaopathy and therapeutic effect of induction-remission in children

目的分析儿童系统性红斑狼疮(SLE)合并血栓性微血管病(TMA)患儿临床特点及诱导缓解疗效。方法回顾性分析本院2019年1月—2023年1月诊治的5例SLE合并TMA患儿的临床资料。结果5例患儿发生TMA时SLE病程(3.2±4.4)个月,年龄(11.2±2.8)岁,诊断TMA时SLE疾病活动度指数评分为重度活动。4例患儿合并溶血性尿毒综合征,其中2例合并恶性高血压,1例合并血栓性血小板减少性紫癜。所有患儿在发生TMA后均给予利妥昔单抗、注射用人免疫球蛋白(IVIG)、甲泼尼龙冲击以及环磷酰胺冲击治疗,4例给予连续性肾脏替代治疗,3例给予血浆置换,1例患儿给予腹膜透析治疗半年后改为血液透析治疗。5例患儿中1例发展为慢性肾功能不全,长期血液透析治疗;2例患儿24 h尿蛋白转阴,肾功能正常,完全缓解。2例患儿随访2月余复查血肌酐及肌酐清除率均正常,部分缓解。结论儿童SLE合并TMA多发生在疾病重度活动期,肾功能衰竭突出。利妥昔单抗联合激素冲击及IVIG的治疗方案能一定程度上改善TMA。

AIM To analyze the clinical characteristics and therapeutic efficacy of systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA)in children.METHODS A retrospective analysis was conducted on the clinical data of 5 children with SLE and TMA in our hospital from January 2019 to January 2023.RESULTS The duration of SLE was(3.2±4.4)months,the age was(11.2±2.8)years,and the SLE disease activity index was severe SLE activity at the time of TMA diagnosis.There were 4 cases with hemolytic uremic syndrome,2 cases with malignant hypertension and 1 case with thrombotic thrombocytopenic purpura.All children were treated with rituximab,intravenous immunoglobulin(IVIG),methylprednisolone shock and cyclophosphamide shock after TMA.Four patients received continuous renal replacement therapy,3 patients received plasma exchange,and 1 child received peritoneal dialysis for six months before hemodialysis.1 of the 5 patients developed chronic renal insufficiency and received long-term hemodialysis treatment.Urine protein turned negative in 24h in 2 patients,renal function was normal,complete remission.The serum creatinine and creatinine clearance of 2 cases were normal after 2 months follow-up and partially relieved.CONCLUSION Children with SLE combined with TMA mostly occur in the severe active stage of the disease,and renal failure is more prominent.Rituximab combined with steriod shock and IVIG therapy can improve TMA to some extent.