Rituximab and intravenous immunoglobulin in the treatment of steroid-dependant nephrotic syndrome

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Introduction Recent studies demonstrated the ability of rituximab (RTX) to decrease the number of relapse in children with steroid-dependant nephrotic syndrome (SDNS). However, the remission rate after two years is only 30 to 40% and strategies using repeated RTX injections increase the risk of infection and of persistent hypogammaglobulinemia. Polyvalent intravenous Immunoglobulin (IvIg) demonstrated the ability to modulate B cells immune response both in vitro and in vivo. We aim to evaluate the efficacy of the association of Rituximab and IvIg to induce long-term remission in children with SDNS. Material and methods Twenty-eight patients with SDNS were included in a pilot study and treated with 1 injection of RTX 375 mg/m 2 followed by IvIg 2 g/kg once a month for 6 months and were compared with a historic cohort of 43 patients treated with a single injection of RTX. The primary outcome was the relapse-free survival 24 months after RTX injection. Cox regression was used to adjust for potential confounders. Results Compared to the control group, our patients were significantly younger (median age in years: at first flair 2.4 vs. 4.4 and at RTX 12.0 vs. 13.8, P = 0.04). They also presented a shorter duration of B-cell depletion (4.0 vs. 5.6 months, P = 0.03. Two-years relapse-free survivals were 45% and 30% in the experimental and control group respectively with a statistically significant difference in favor of IvIg [HR 0.32 (0.15–0.69)]. Conclusion The association of IvIg with RTX improved relapse-free survival in children with SDNS. This finding gives new insight in the pathophysiology of SDNS and offers new hypothesis for basic research. A prospective randomized trial is currently ongoing to further demonstrate the efficacy of this strategy.