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Rituximab treatment for difficult-to-treat nephrotic syndrome in children: a multicenter, retrospective study.

Authors :
TAŞDEMİR, Mehmet
CANPOLAT, Nur
YILDIZ, Nurdan
ÖZÇELİK, Gül
BENZER, Meryem
SAYGILI, Seha Kamil
ÖZKAYIN, Emine Neşe
TÜRKKAN, Özde Nisa
BALAT, Ayşe
CANDAN, Cengiz
ÇELAKIL, Mehtap
YAVUZ, Sevgi
AKINCI, Nurver
GÖKNAR, Nilüfer
AKGÜN, Cihangir
TÜLPAR, Sebahat
ALPAY, Harika
SEVER, Fatma Lale
BİLGE, İlmay
Source :
Turkish Journal of Medical Sciences; 2021, Vol. 51 Issue 4, p1781-1790, 10p
Publication Year :
2021

Abstract

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/m² ) or high (2-4 doses of 375 mg/m² ) 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. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
13000144
Volume :
51
Issue :
4
Database :
Complementary Index
Journal :
Turkish Journal of Medical Sciences
Publication Type :
Academic Journal
Accession number :
152281413
Full Text :
https://doi.org/10.3906/sag-2012-297