贝利尤单抗治疗儿童活动性狼疮性肾炎12例临床观察  

Clinical observation of belimumab in the treatment of 12 children with active lupus nephritis

作　　者：陈元 孔林笑语[2] 孙书珍 王莉 李倩[1,2] 王京 余丽春[1,2] 杨振乐 Chen Yuan;Kong Linxiaoyu;Sun Shuzhen;Wang Li;Li Qian;Wang Jing;Yu Lichun;Yang Zhenle(Department of Pediatric Nephrology and Rheumatism and Immunology,Shandong Provincial Hospital Affilicated to Shangdong First Medical University,Jinan 250021,China;Department of Pediatric Nephrology and Rheumatism and Immunology,Shandong Provincial Hospital Affiliated to Shandong University,Jinan 250021,China)

机构地区：[1]山东第一医科大学附属省立医院小儿肾脏风湿免疫科,济南250021 [2]山东大学附属山东省立医院小儿肾脏风湿免疫科,济南250021

出　　处：《中国小儿急救医学》2022年第12期981-984,共4页Chinese Pediatric Emergency Medicine

基　　金：山东省自然科学基金项目(ZR2022QH076)。

摘　　要：目的分析一线治疗效果不佳的儿童活动性狼疮性肾炎(LN)患儿应用贝利尤单抗治疗的临床病例资料,探讨贝利尤单抗治疗儿童LN的疗效与安全性,为临床治疗提供一定的经验指导。方法收集2020年8月至2021年9月就诊于我院的疾病活动指数(SLEDAI)评分≥8分的一线治疗效果不佳的LN患儿12例,回顾分析其临床表现、治疗过程及SLEDAI评分、补体C3、补体C4、抗dsDNA抗体滴度、蛋白尿缓解情况。结果应用贝利尤单抗治疗前SLEDAI评分8分3例,10分5例,12分2例,16分2例;尿蛋白阳性6例;抗dsDNA抗体滴度高于正常值8例;补体C3降低8例,补体C4降低6例。贝利尤单抗治疗后,12例患儿SLEDAI评分及抗dsDNA抗体、6例尿蛋白阳性患儿24 h尿蛋白定量于4周内开始下降;抗dsDNA抗体在第12周,24 h尿蛋白定量在第16周均降至正常;12例患儿的补体C3、补体C4于4周内开始上升,补体C3于24周内恢复正常,补体C4于28周内恢复正常。结论对于一线治疗方案效果不佳或疾病持续活动的LN患儿,加用贝利尤单抗治疗后,其补体上升,SLEDAI、抗dsDNA抗体滴度下降,蛋白尿有效缓解。贝利尤单抗的应用对一线治疗方案效果不佳的儿童活动性LN有一定疗效,值得临床推广。Objective To analyze the clinical data of children with active lupus nephritis(LN)with poor first-line treatment and further treatment with belimumab,and explore the efficacy and safety of belimumab in the treatment of children with LN,so as to provide experience and guidance for clinical treatment of children with LN.Methods From August 2020 to September 2021,12 children with LN whose systemic lupus erythematosus disease activity index(SLEDAI)score was≥8 and with poor first-line treatment were collected,and their clinical manifestations,treatment process,SLEDAI score,complement C3,complement C4,anti-dsDNA antibody titer,and proteinuria relief were analyzed retrospectively.Results Before treatment with belimumab,the SLEDAI score was 8 in 3 cases,10 in 5 cases,12 in 2 cases and 16 in 2 cases.Theurine protein was positive in 6 cases.The anti-dsDNA antibody titer was higher than normal value in 8 cases.The complement C3 decreased in 8 cases and the complement C4 decreased in 6 cases.The SLEDAI scores and the anti-dsDNA antibody of 12 children and 24-hour urine protein quantification of 6 children with positive urine protein began to decrease within 4 weeks after treatment with belimumab.Anti-dsDNA antibody decreased to normal in 12th week and 24 h urine protein decreased to normal in 16th week.The levels of complement C3 and C4 began to rise within 4 weeks,complement C3 returned to normal within 24 weeks,and complement C4 returned to normal within 28 weeks.Conclusion For LN children with poor response to first-line therapy or persistent disease activity,the addition of belimumab resulted in increased complement,decreased disease activity index and anti-dsDNA antibody titer,and effective relief of proteinuria.The application of belimumab has a certain effect on active LN children with poor response to first-line therapy,which is worthy of clinical promotion.

关 键 词：贝利尤单抗 狼疮性肾炎 活动性 儿童 

分 类 号：R725.9[医药卫生—儿科]