Rituximab in treatment of children with frequently relapsed nephrotic syndrome / 中华实用儿科临床杂志

ABSTRACT

Objective To explore the effect and safety of rituximab (RTX) in children with frequently relapsed nephrotic syndrome.Methods Twelve children (8 male and 4 female) with frequently relapse nephrotic syndrome were treated with RTX.They all showed resistance to various immunosuppressive agents and relapsed when steroid was reduced at 1.5-2.0 mg/kg.All immunosuppressive agents were stopped.Steroid was prescribed at doses of 2 mg/(kg · d),followed by alternate-day dosing (2 mg/kg) after proteinuria was negative for 5 days,and then tapered by 5 mg every 2 weeks until to low dose (＜ 0.5 mg/kg on alternate day) or discontinued.RTX was administered at a dose of 375 mg/m2 once every week for 3 weeks.Results The patients were followed up for 4-19 months [median (7.79 ± 3.00) months].Proteinuria turned negative on 1-14 d after first RTX treatment.Mean steroid dosages were significantly reduced than before treatment [alternate-day dosing (1.83 ± 1.02) mg/kg vs alternate-day dosing (0.34 ± 0.16) mg/kg,t =3.78,P =0.002].Gingival hyperplasia and Cushing appearance were significantly improved and steroid-induced glaucoma disappeared.The height was significantly increased compared with before treatment [(2.21 ± 1.40) cm/year vs (8.27 ±2.10) cm/year].Six cases had no relapse during follow-up.Another 6 cases relapsed in 5.5-19.0 months.All patients showed CD19 + B lymphocytes depletion after RTX treatment.However,CD19 + recovery could be seen in 5.5-12.0 months.Serum immunoglobulin did not significantly change.None of the patients was found with adverse events.Conclusions RTX can significantly reduce the dosage of steroid or even stop medication in children free of immunosuppressive agents,maintain remission for (7.79 ± 3.00) months,significantly improve the adverse reaction induced by steroid and immune-suppression.Thus,for children with frequently relapsed nephritic syndrome/steroid-dependent nephrotic syndrome who show resistance to immunosuppressive therapies,RTX treatment may be considered.