Turk Pediatri Ars. 2020 Mar 09;55(1):60-66. doi: 10.14744/TurkPediatriArs.2019.76148. eCollection 2020.
Rituximab experience in children with nephrotic syndrome: what have we observed differently.
Turk pediatri arsivi
İlknur Girişgen, Selçuk Yüksel, Yücel Pekal
Affiliations
Affiliations
- Division of Pediatric Nephrology, Department of Pediatrics, Pamukkale University Faculty of Medicine, Denizli, Turkey.
- Department of Pediatrics, Pamukkale University Faculty of Medicine, Denizli, Turkey.
PMID: 32231451
PMCID: PMC7096562 DOI: 10.14744/TurkPediatriArs.2019.76148
Abstract
AIM: We aimed to evaluate the efficacy of rituximab therapy in children with nephrotic syndromes and to share our experiences.
MATERIAL AND METHODS: Twelve children with nephrotic syndrome (four with steroid-dependent, eight with steroid-resistant nephrotic syndrome) who were treated with rituximab were retrospectively evaluated in terms of clinical and laboratory data and CD19-20 levels. All patients received rituximab (375 mg/m
RESULTS: The overall remission rates in patients with steroid-dependent and steroid-resistant nephrotic syndrome were 100% and 27%. Focal segmental glomerulosclerosis was diagnosed in six patients and the remission rate was 33% in this population. CD19 cell depletion was observed in 10 of the 12 children. Seven of the 10 patients with CD19 depletion achieved remission, whereas the other three had persistent nephrotic proteinuria despite CD19 depletion. Two patients without CD19 depletion never achieved remission. Relapse occurred in three of the seven patients associated with increased CD19.
CONCLUSION: We observed that rituximab could be given without waiting for a proteinuria-free period under steroid therapy. Our result suggest that administering four weekly doses of rituximab increases the likelihood of remission, considering the amount of drug lost in the urine of children with nephrotic proteinuria. However, our findings must be confirmed with dose-comparison studies conducted with larger populations and an evaluation of long-term adverse effects. Some patients did not achieve remission despite B cell depletion, which suggests that B cell depletion is necessary but insufficient for remission in nephrotic syndromes.
Copyright: © 2020 Turkish Archives of Pediatrics.
Keywords: Children; nephrotic syndrome; rituximab
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