Abstract
<title>Abstract</title> <p>Background: In 25-35% children with non-genetic calcineurin inhibitor (CNI) -resistant- steroid resistant nephrotic syndrome (SRNS), rituximab has been effective as rescue therapy. Use of add-on immunosuppression to maintain remission has not been studied well. Methods and materials: Recorded demographic details, response and relapse rate, infections, hospitalizations, progression to chronic kidney disease and death were compared between two groups: mycophenolate mofetil (MMF) with rituximab, CNI with rituximab. Results: Of 95 children with SRNS, 24 children received 32 rituximab treatment-events, including 18 in the MMF group and 14 in the CNI group. Baseline demographic, clinical, and histopathological characteristics were comparable. Minimal change disease was the commonest histopathological diagnosis (57.6%). Following the first rituximab cycle, complete or partial remission was achieved in 33.3% of treatment events in the MMF group and 28.6% in the CNI group. Relapse rates were similar between groups (38.9% vs. 35.7%; p=0.854). Median relapse-free survival was longer in CNI with rituximab group (mean 18.2 months +/-6.5 months) as compared to MMF with rituximab group (mean 12.17 +/- 11.56 months)( p,0.07). Hospitalizations for edema and infections decreased following rituximab therapy in both groups. Among first-cycle non-responders, 23.5% progressed to chronic kidney disease (CKD) stage ≥3. CKD progression (46.2% vs. 18.2%) and mortality (23.1% vs. 9.1%) were more frequent in the MMF group. At 36 months, overall survival was 70.5% in the MMF group and 90.0% in the CNI group. Conclusion: In CNI-resistant-SRNS, proportionately higher remission, fewer hospitalizations and fewer progression to CKD is seen in CNI with rituximab compared to MMF with rituximab. Small sample size requires longitudinal prospective studies for validation.</p>