Rituximab treatment for idiopathic steroid-dependent nephrotic syndrome in children

Background: In the last decade, the results of many studies have shown the successful use of rituximab (RTX) in the treatment of children with steroid-dependent nephrotic syndrome (SDNS), which relapses despite of the use of conventional immunosupressive agents. The aim of our study was to evaluate the efficacy and safety of rituximab therapy in children with refractory SDNS. Materials and methods: The group of patients included 9 children (median age 11.5 years). All patients were dependent on high doses of prednisolone: the median 0.46 (range 0.32-0.78) mg/kg/day, revealed a high incidence of relapses NS 3.0 (1.8-6.0) times/years, and had clinical signs of severe steroid toxicity. The children received one to four intravenous infusions weekly of 375 mg/m2 RTX during the first course. Results: Within 6 months and 12 months after an initial treatment with RTX: none of the children developed relapses of NS; steroids have been discontinued in 33.3% and 66.6% of patients, respectively; in other children the dose of prednisone was significantly reduced to 0.095 (0.04-0.25) mg/kg/day (p < 0,01) and 0.04 (0.02-0.04) mg/kg/day (p < 0,01). All five patients with long-term follow-up required repeated courses of RTX. The maintenance therapy of low-dose prednisone (median 0.04 mg/kg/day) was reintroduced in 80% of patients showing a significant reduction in the frequency of relapses of NS (median 0.4 times/year). Cyclosporine A have been discontinued in all patients. Conclusion: The use of Rituximab leads to a significant reduction in the frequency of relapses, as well as a significant steroid-sparing and cyclosporine-sparing effect in patients with refractory SDNS.

Ритуксимаб, идиопатический стероид-зависимый нефротический синдром, иммуносупрессивные агенты, дети, Rituximab, Idiopatic steroid-dependent nephrotic syndrome, Immunosuppresive agents, Children

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