Rituximab treatment in children with difficult-to-treat nephrotic syndrome
Öz
Purpose: Rituximab (RTX) has been offered as rescue therapy for patients with difficult-to-treat nephrotic syndrome (frequent relapsing, steroid-dependent and steroid resistant). We aimed to assess the efficacy and long-term outcomes of RTX treatment in children with difficult nephrotic syndrome and shared our experiences
Materials and Methods: Medical records of children with difficult nephrotic syndrome who were treated with RTX were retrospectively evaluated. The relapse-free survival rate at 12 month and monitoring of B-cell depletion were assessed.
Results: In the study included 20 children of which 8 had steroid-dependent (SDNS), 6 had frequent relapsing (FRNS), and 6 had steroid-resistant nephrotic syndrome (SRNS). The median number of relapses at 1 year before and after treatment in FRNS/SDNS patients receiving RTX treatment were compared. The median number of relapses decreased from 2 (1-4) to 0 (0-1) times/year. The mean duration of the follow-up period after RTX treatment was 23 (12-59) months, and 8 patients developed relapse. Repeated doses of RTX were administered to 5 patients who relapsed after RTX treatment. In these patients, CD19+B cells re-emerged during remission, while depletion of memory B-cells remained.
Conclusion: The RTX treatment prolonged the remission time in FRNS/SDNS patients, but it was ineffective in SRNS patients. It was determined that the RTX doses can be repeated to maintain remission in these patients, and the best memory B-cell counts can help in timing the repeat doses.
Anahtar Kelimeler
Rituximab, nephrotic syndrome, memory b-cell count, children
Destekleyen Kurum
Baskent University Research Fund
Proje Numarası
KA20/269
Teşekkür
I would like to thank Professor Dr. Zekiye Aytül Noyan at the Pediatric Nephrology Department for Dr. Turgut Noyan Research and Training Center of Başkent University.
I would like to thank Professor Dr. İlknur Kozanoğlu at the Physiology Department for Dr. Turgut Noyan Research and Training Center Apheresis Unit of Başkent University.
Başkent Üniversitesi Pediatrik Nefroloji Anabilim Dalı'ndan Dr. Turgut Noyan Araştırma ve Eğitim Merkezi'nden Prof. Dr. Zekiye Aytül Noyan'a teşekkür ederim.
Başkent Üniversitesi Dr. Turgut Noyan Araştırma ve Eğitim Merkezi Aferez Ünitesi Fizyoloji Bölümü'nden Prof. Dr. İlknur Kozanoğlu'na teşekkür ederim.
Kaynakça
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