The efficacy and tolerability of rituximab in children with steroid and cyclosporine-resistant and steroid and cyclosporine-dependent pediatric nephrotic syndrome

Background: A few clinical trial-based evidences could show the effectiveness of rituximab in relieving manifestations of nephrotic syndrome especially in affected children. The present study aimed to assess the therapeutic effectiveness of Rituximab in steroid and cyclosporine-resistant and steroid and cyclosporine-dependent pediatric nephrotic syndrome.Methods: This prospective interventional study was performed on 42 children aged lower than 18 years with steroid and cyclosporine-resistant or steroid and cyclosporine-dependent pediatric nephrotic syndrome who were candidate for administrating rituximab. All patients received intravenous rituximab (375mg/m2) per week for 4 weeks.For assessment of renal functional status, dipstick urinalysis was scheduledweekly for up to four weeks and then once a month to three months. Results: Regarding underlying pathological pattern, 73.8% had FSGS, 21.4% had MCNS and 4.8% suffered MGN. Overall, 57.1% of children suffered steroid and cyclosporine-resistant nephrotic syndrome, 16.7% had drug late- resistant nephrotic syndrome and 26.2% had steroid and cyclosporine-dependent pattern of disease. Regarding response to treatment, 61.9% responded completely to treatment with Rituximab, 7.1% had partial response, 4.8% had clinical response and others remained with no significant response to this drug. Drug-related side effects were found only in 4 patients as leukopenia in two children, alopecia in one children and eosinophilia in another one. In those children who suffered steroid and cyclosporine-resistant disease, complete, partial, and clinical response was revealed in 37.5%, 12.5%, and 4.2% respectively, while in the group with late resistant pattern was 87.5%, 0%, and 14.3%, and in those with steroid and cyclosporine-dependent pediatric nephrotic syndrome was 100%, 0% and 0% respectively indicating significantly higher complete response in those with drug-dependent pattern (p = 0.006). The number of relapses was reduced after receiving rituximab when compared to before treatment. The response rate to Rituximab did not dependent to gender, age, or disease pathology. Conclusion: Rituximab is effective for children with nephrotic syndrome with high efficacy and well tolerabilityespecially in those with steroid and cyclosporine-dependent pediatric nephrotic syndrome