Rituximab treatment for difficult-to-treat nephrotic syndrome in children: A multicenter, retrospective study
Saygılı, Seha Kamil
Özkayın, Emine Neşe
Türkkan, Özde Nisa
Sever, Fatma Lale
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CitationTaşdemir, M., Canpolat, N., Yıldız, N., Özçelik, G., Benzer, M., Saygılı, S. K. ... Bilge, İ. (2021). Rituximab treatment for difficult-to-treat nephrotic syndrome in children: A multicenter, retrospective study. Turkish Journal of Medical Sciences, 51(4), 1781-1790. https://dx.doi.org/10.3906/sag-2012-297
Background/aim: This study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or -resistant) and the dosing regimen. Materials and methods: This multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m(2)) or high (2-4 doses of 375 mg/m(2)) initial dose of rituximab and the steroid response. Clinical outcomes were compared. Results: Data from 42 children [20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9-17.3 years] were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 +/- 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups. Conclusion: The current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined.