Kidney Survival in Children With Steroid-Resistant Nephrotic Syndrome Treated by Rituximab.

Authors

Eugene Yu-Hin Chan, Irene Yuk-Ying Ho, Chun-Ting Au, Ellen L M Yu, Andrea Angeletti, Sushmita Banerjee, Olivia Boyer, Chang-Yien Chan, Anna Francis, Gian Marco Ghiggeri, Riku Hamada, Nakysa Hooman, Luke Sydney Hopf, Mohamad Ikram I, Iftikhar Ijaz, Dmytro D Ivanov, Hee Gyung Kang, Laura Lucchetti, Francesca Lugani, Alison Lap-Tak Ma, William Morello, Subal Kumar Pradhan, Larisa Prikhodina, Reem H Raafat, Rajiv Sinha, Sharon Teo, Kouki Tomari, Marina Vivarelli, Hazel Webb, Hui Kim Yap, Desmond Yat-Hin Yap, Kjell Tullus

Published in

Kidney international reports. Volume 11. Issue 9. Pages 106638. Epub Jun 03, 2026.

Abstract

Kidney outcomes in children with steroid-resistant nephrotic syndrome (SRNS) following rituximab remain unclear.
We conducted an international retrospective cohort study across 23 centers in 17 countries, including children with SRNS who did not respond to calcineurin inhibitors (CNIs) and subsequently, received rituximab. Patients with genetic variants were excluded. The primary outcome was kidney survival.
We analyzed data on 151 children (53% males; median age at onset: 6.8 years; primary vs. secondary SRNS: 54% vs. 46%; focal segmental glomerulosclerosis [FSGS]: 62%). All subjects received CNIs before rituximab (0-3 months: 21%; 3-6 months: 26%; 6-12 months: 26%; > 12 months: 26%). Upon rituximab, 73 subjects (48%) had normal kidney function, 47 (31%) had chronic kidney disease (CKD)2, and 31 (21%) had CKD3. Twenty-eight (19%) developed kidney failure. Overall kidney survival was 82.7%, 75.8%, and 72.3% at 3, 5, and 7 years post-rituximab. Baseline CKD staging before rituximab was associated with kidney survival at 5 years (log-rank P < 0.001); CKD stage 1, 91.7%; CKD stage 2, 70.4%; and CKD stage 3, 35.5%). The predictive factors for inferior kidney survival were CKD2 (adjusted hazard ratio [HR_adj]: 2.7, 95% confidence interval [CI]: 1.5-4.9, P < 0.001), lower baseline serum albumin (log-rank P = 0.01; HR_adj: 0.88, 95% CI: 0.81-0.95; P < 0.001) and FSGS (log-rank P = 0.03; HR_adj: 3.4, 95% CI: 1.1-10.0; P < 0.001). Nonremission at 6 months was associated with inferior kidney survival (61.2% at 5 years), compared with complete remission (CR) or partial remission (PR) (100% at 5 years) (log-rank P = 0.01; HR_adj: 14.1, 95% CI: 2.2-93.5, P = 0.01). Duration of prior calcineurin inhibition and SRNS type were not significant predictors.
Kidney survival of SRNS following add-on rituximab is 70% to 80% over 3 to 7 years. Nonresponse, preexisting CKD, lower albumin, and FSGS predict inferior kidney survivals.

PMID:
42437162
Bibliographic data and abstract were imported from PubMed on 12 Jul 2026.

Read full publication at:
Please sign in to see all details.

Stats

1-terrible, 9-excellent. How would you rate this publication? Sign in in to submit your rating.

Post a comment

You need to be signed in to post comments. You can sign in here.

Comments

There are no comments yet.