利妥昔单抗治疗难治性激素耐药型肾病综合征的疗效和安全性  

Efficacy and safety of Rituximab in the treatment of refractory steroid-resistant nephrotic syndrome

作　　者：王美秋 王忍 夏正坤 何旭 方香 贾丽丽 张沛 高春林 Wang Meiqiu;Wang Ren;Xia Zhengkun;He Xu;Fang Xiang;Jia Lili;Zhang Pei;Gao Chunlin(Department of Pediatrics,Jinling Hospital,Nanjing School of Clinical Medicine,Southern Medical University,Nanjing 210002,China;Department of Pediatrics,Jinling Hospital,Nanjing Medical University,Nanjing 210002,China)

机构地区：[1]南方医科大学南京临床医学院(东部战区总医院)儿科,南京210002 [2]南京医科大学金陵临床医学院(东部战区总医院)儿科,南京210002

出　　处：《中华实用儿科临床杂志》2021年第5期355-358,共4页Chinese Journal of Applied Clinical Pediatrics

基　　金：江苏省重点研发计划-临床前沿技术项目(BE2017719);江苏省儿科医学创新团队项目(CXTDA2017022)。

摘　　要：目的探讨利妥昔单抗(RTX)治疗儿童难治性激素耐药型肾病综合征(SRNS)的疗效和安全性。方法回顾性分析2013年9月至2018年3月东部战区总医院儿科收治并接受RTX治疗的10例难治性SRNS患儿的临床资料。结果10例患儿发病年龄(4.47±2.75)岁,男女各5例;5例(50%)肾活检为局灶节段性肾小球肾炎,3例(30%)为微小病变肾病,1例(10%)为IgM肾病,1例(10%)为系膜增生性肾小球肾炎。10例患儿于(6.74±2.62)岁时行RTX治疗(1剂或4剂,每次375 mg/m^(2),最大500 mg);治疗1次、3次、8次分别为8例(80%)、1例(10%)、1例(10%)。随访时间11.93(5.17,25.66)个月;随访3个月、6个月以及最后一次随访的缓解率分别为30%(3例)、40%(4例)、40%(4例)。10例患儿RTX治疗前后24 h尿蛋白及白蛋白水平有所改善,但差异均无统计学意义(均P>0.05);治疗前后体液免疫及肾功能无明显变化(均P>0.05)。在治疗及随访过程中,3例(30%)出现输液反应,2例(10%)出现严重肺部感染,1例(10%)严重肺部感染死亡。结论RTX对部分难治性SRNS患儿治疗有效,治疗后需长期随访,避免感染。Objective To investigate the efficacy and safety of Rituximab(RTX)in treating children with refractory steroid-resistant nephrotic syndrome(SRNS).Methods The clinical data of 10 children with refractory SRNS receiving RTX in the Department of Pediatrics,Jinling Hospital from September 2013 to March 2018 were analyzed retrospectively.Results The age of onset of 10 children(including 5 males and 5 females)was(4.47±2.75)years old.The renal biopsy showed focal segmental glomerular sclerosis in 5 cases(50%),minimal change nephropathy in 3 cases(30%),IgM nephropathy in 1 case(10%),and mesangial proliferative glomerulonephritis in 1 case(10%).Ten children received RTX treatment(1 or 4 doses;375 mg/m^(2)once;maximum:500 mg)at the age of(6.74±2.62)years old.There were 8 patients(80%)receiving a single dose of RTX,1 patient(10%)receiving 3 doses,and 1 patient(10%)receiving 8 doses.The follow-up time was 11.93(5.17,25.66)months.The remission rates at the 3-month follow-up,6-month follow-up and last follow-up were 30%(3 patients),40%(4 patients),and 40%(4 patients),respectively.The 24-hour urinary proteinuria and serum albumin levels were improved in 10 children after RTX treatment,but there were no significant statistical difference(all P>0.05).No significant difference was found in humoral immunity and renal function before and after RTX treatment(all P>0.05).During the treatment and follow-up,3 patients(30%)developed infusion reaction,2 patients(20%)showed severe pulmonary infection,and 1 patient(10%)died of severe pulmonary infection.Conclusions RTX is effective in treating some children with refractory SRNS,and a long-term follow-up should be conducted to prevent infection.

关 键 词：难治性激素耐药型肾病综合征 利妥昔单抗 儿童 

分 类 号：R726.9[医药卫生—儿科]