An international, multi-center study evaluated rituximab therapy in childhood steroid-resistant nephrotic syndrome

The efficacy and safety of rituximab in childhood steroid-resistant nephrotic syndrome (SRNS) remains unclear. Therefore, we conducted a retrospective cohort study at 28 pediatric nephrology centers from 19 countries in Asia, Europe, North America and Oceania to evaluate this. Children with SRNS treated with rituximab were analyzed according to the duration of calcineurin inhibitors (CNIs) treatment before rituximab [6 months or more (CNI-resistant) and under 6 months]. Primary outcome was complete/partial remission (CR/PR) as defined by IPNA/KDIGO guidelines. Secondary outcomes included kidney failure and adverse events. Two-hundred-forty-six children (mean age, 6.9 years; 136 boys; 57% focal segmental glomerulosclerosis, FSGS) were followed a median of 32.4 months after rituximab. All patients were in non-remission before rituximab. (146 and 100 children received CNIs for 6 month or more or under 6 months before rituximab, respectively). In patients with CNI-resistant SRNS, the remission rates (CR/PR) at 3-, 6-, 12- and 24-months were 26% (95% confidence interval 19.3-34.1), 35.6% (28.0-44.0), 35.1% (27.2-43.8) and 39.1% (29.2-49.9), respectively. Twenty-five patients were in PR at 12-months, of which 22 had over 50% reduction in proteinuria from baseline. The remission rates among children treated with CNIs under 6 months before rituximab were 42% (32.3-52.3), 52% (41.8-62.0), 54% (44.3-64.5) and 60% (47.6-71.3) at 3-, 6-, 12-, and 24-months. Upon Kaplan-Meier analysis, non-remission and PR at 12-months after rituximab, compared to CR, were associated with significantly worse kidney survival. Adverse events occurred in 30.5% and most were mild. Thus, rituximab enhances remission in a subset of children with SRNS, is generally safe and CR following rituximab is associated with favorable kidney outcome.

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Publication Details

Journal
Kidney International
Published
2024-10-10
DOI
https://doi.org/10.1016/j.kint.2024.09.011
Citations
27
Primary Topic
Renal Diseases and Glomerulopathies
Type
article
Field-Weighted Citation Impact
9.16
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article

An international, multi-center study evaluated rituximab therapy in childhood steroid-resistant nephrotic syndrome

E. Yu, Andrea Angeletti, Nakysa Hooman, Mohamad Ikram Ilias et al.
27 citations
Kidney International
Renal Diseases and Glomerulopathies
9.16
article

An international, multi-center study evaluated rituximab therapy in childhood steroid-resistant nephrotic syndrome

E. Yu, Andrea Angeletti, Nakysa Hooman, Mohamad Ikram Ilias, Riku Hamada, Hee Gyung Kang, Reem Raafat, Kjell Tullus, Larisa Prikhodina, Gian Marco Ghiggeri, Hui‐Kim Yap, Francesca Lugani, Hazel Webb, Kouki Tomari, Chang‐Yien Chan, William Morello, Rajiv Sinha, Sushmita Banerjee, Marina Vivarelli, Suprita Kalra, Iftikhar Ijaz, Arvind Bagga, Luke Sydney Hopf, Anna Francis, Eugene Yu-hin Chan, Alison Lap‐tak, Aditi Sinha, Olivia Boyer, Д.Д. Іванов, Naureen Akhtar, Laura Lucchetti, Pankaj Hari, M Muniz, Subal Kumar Pradhan, Sharon Teo, Desmond Yat‐Hin Yap
article en
27 citations

Abstract

The efficacy and safety of rituximab in childhood steroid-resistant nephrotic syndrome (SRNS) remains unclear. Therefore, we conducted a retrospective cohort study at 28 pediatric nephrology centers from 19 countries in Asia, Europe, North America and Oceania to evaluate this. Children with SRNS treated with rituximab were analyzed according to the duration of calcineurin inhibitors (CNIs) treatment before rituximab [6 months or more (CNI-resistant) and under 6 months]. Primary outcome was complete/partial remission (CR/PR) as defined by IPNA/KDIGO guidelines. Secondary outcomes included kidney failure and adverse events. Two-hundred-forty-six children (mean age, 6.9 years; 136 boys; 57% focal segmental glomerulosclerosis, FSGS) were followed a median of 32.4 months after rituximab. All patients were in non-remission before rituximab. (146 and 100 children received CNIs for 6 month or more or under 6 months before rituximab, respectively). In patients with CNI-resistant SRNS, the remission rates (CR/PR) at 3-, 6-, 12- and 24-months were 26% (95% confidence interval 19.3-34.1), 35.6% (28.0-44.0), 35.1% (27.2-43.8) and 39.1% (29.2-49.9), respectively. Twenty-five patients were in PR at 12-months, of which 22 had over 50% reduction in proteinuria from baseline. The remission rates among children treated with CNIs under 6 months before rituximab were 42% (32.3-52.3), 52% (41.8-62.0), 54% (44.3-64.5) and 60% (47.6-71.3) at 3-, 6-, 12-, and 24-months. Upon Kaplan-Meier analysis, non-remission and PR at 12-months after rituximab, compared to CR, were associated with significantly worse kidney survival. Adverse events occurred in 30.5% and most were mild. Thus, rituximab enhances remission in a subset of children with SRNS, is generally safe and CR following rituximab is associated with favorable kidney outcome.

Kidney InternationalVol. 106(6)
Hôpital Necker-Enfants Malades (FR), King Edward Medical University (PK), Inserm (FR), Universität Hamburg (DE), Iran University of Medical Sciences (IR), Mexican Social Security Institute (MX), National University of Singapore (SG), Chinese University of Hong Kong (HK), Université Paris Cité (FR), Shupyk National Healthcare University of Ukraine (UA), Great Ormond Street Hospital (GB), Great Ormond Street Hospital for Children NHS Foundation Trust (GB), Queen Mary Hospital (CN), Istituto Giannina Gaslini (IT), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (IT), University of Health Sciences Lahore (PK), Sriram Chandra Bhanja Medical College Hospital (IN), Pirogov Russian National Research Medical University (RU), Indian Council of Medical Research (IN), Institut Necker Enfants Malades (FR), Hospital Universiti Sains Malaysia (MY), Duchess of Kent Children's Hospital (CN), Assistance Publique – Hôpitaux de Paris (FR), Seoul National University Children's Hospital (KR), Russian Medical Academy of Continuous Professional Education (RU), University Medical Center Hamburg-Eppendorf (DE), Princess Margaret Hospital (NZ), Bambino Gesù Children's Hospital (IT), Memorial Health System (US), Institute of Child Health (IN), Tokyo Metropolitan Children's Medical Center (JP), National University Health System (SG), Istituti di Ricovero e Cura a Carattere Scientifico (IT), Joe DiMaggio Children's Hospital (US), Command Hospital (IN), Children's Health Queensland Hospital and Health Service (AU), University College London (GB), Institute of Medical Sciences (IN), Medical College and Hospital, Kolkata (IN), Bogomolets National Medical University (UA), University of Hong Kong (HK)
Openalex Percentile: Top 1%
Renal Diseases and Glomerulopathies
9.16
DOIOpen Access PDFOpenAlex
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