Rituximab Treatment for Difficult-to-treat Nephrotic Syndrome in Children: a Multicenter, Retrospective Study

Overview

Journal Turk J Med Sci

Specialty General Medicine

Date 2021 Feb 13

PMID 33581711

Citations 1

Authors

Mehmet Tasdemir

Nur Canpolat

Nurdan Yildiz

Gul Ozcelik

Meryem Benzer

Seha Kamil Saygili

Emine Nese Ozkayin

Ozde Nisa Turkkan

Ayse Balat

Cengiz Candan

Mehtap Celakil

Sevgi Yavuz

Nurver Akinci

Nilufer Goknar

Cihangir Akgun

Sebahat Tulpar

Harika Alpay

Fatma Lale Sever

Ilmay Bilge

Affiliations

Soon will be listed here.

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.

Citing Articles

Successful treatment of peritoneal dialysis for two patients with refractory nephrotic syndrome and acute kidney injury: a case report.

Yu L, Cheng S, Zhang M, Zhou T, Chen Y, Zhang Z Front Med (Lausanne). 2023; 10:1263780.

PMID: 37920598 PMC: 10618994. DOI: 10.3389/fmed.2023.1263780.

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