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

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，...

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

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/m²once;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.