The successful use of rituximab in children with IgA nephropathy and minimal change-like lesions: a case series.

IgA nephropathy with minimal change disease (IgAN-MCD) is a rare glomerulopathy combining features of both entities, typically presenting as steroid-sensitive nephrotic syndrome (NS) with frequent relapses. While rituximab (RTX) is commonly used in pediatric glomerular diseases, its efficacy in IgAN-MCD remains unclear. Our retrospective study evaluated the efficacy and safety of RTX in eight children with IgAN-MCD who remained steroid-dependent or frequently relapsed despite treatment with glucocorticoids and second-line immunosuppressants before RTX. Over a follow-up period of 12-41 months, RTX significantly reduced the annual relapse rate by 1.1 episodes per year, prolonged remission by 3.5 months, and extended the steroid-free interval by 7.4 months. Overall, 87.5% of patients successfully discontinued glucocorticoids, and 50% stopped immunosuppressants. Three patients relapsed at 9, 16, and 19 months, likely due to insufficient CD19+ B-cell monitoring and lack of consolidation dosing, yet all achieved sustained remission by the final follow-up. No serious RTX-related adverse events were observed. These findings suggest that RTX effectively reduces relapse, prolongs remission, and decreases dependence on steroids and immunosuppressants in pediatric IgAN-MCD, with a favorable safety profile for refractory cases.