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The objective was to evaluate the clinical efficacy and safety of telitacicept in the treatment of pediatric IgA vasculitis nephritis (IgAVN). A retrospective analysis was conducted on clinical data from patients aged 3-18 years with IgAVN treated with telitacicept at the Department of Pediatrics, The First Affiliated Hospital of Henan University of Chinese Medicine, between August 2023 and August 2024. Laboratory parameters were assessed at baseline and at 1, 3, 6, and 12 months posttreatment to determine therapeutic efficacy and safety. A total of 16 patients were enrolled (7 males [43.75%]; mean age 14.3 years). The median disease duration from diagnosis to telitacicept initiation was 4.25 (2.06-33.00) months, and the mean treatment duration was 8.63 ± 1.89 months. Early clinical improvement was observed as early as 1 month compared with baseline, characterized by substantial reductions in 24-h urinary protein (24 h-UP) and urinary red blood cell counts compared to baseline. At 12 months, mean 24 h-UP decreased further to 0.16 ± 0.15 g (P < 0.001), with 68.75% (11/16) of patients achieving complete remission. Serum IgG, IgM, and IgA levels were significantly reduced (P < 0.05), while serum albumin significantly increased (P < 0.001). No serious adverse events occurred; three patients experienced mild upper respiratory tract infections that resolved with symptomatic treatment.

 Telitacicept effectively reduces proteinuria, improves renal function, and enhances clinical remission rates in pediatric IgAVN, demonstrating a favorable safety profile.

• Telitacicept, a dual BAFF/APRIL inhibitor, has shown potential effi cacy in IgA nephropathy (IgAN) and IgA vasculitisnephritis (IgAVN), particularly in reducing proteinuria and maintaining stable renal function. • Evidence regarding the use of telitacicept in pediatric IgAVN remains limited and is mainly derived from small case series and retrospective studies.

• This study provides real-world data on the use of telitacicept in pediatric IgAVN, showing improvement in proteinuria with stable renal function during follow-up. • Most patients were able to discontinue glucocorticoids and/or immunosuppressive agents within 3-6 months after telitacicept initiation, suggesting a potential steroid- and immunosuppressant-sparing effect.