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

Chan EY 1 , Sinha A 2 , Yu ELM 3 , Akhtar N 4 , Angeletti A 5 , Bagga A 2 Show all authors , Banerjee S 6 , Boyer O 7 , Chan CY 8 , Francis A 9 , Ghiggeri GM 5 , Hamada R 10 , Hari P 2 , Hooman N 11 , Hopf LS 12 , I MI 13 , Ijaz I 14 , Ivanov DD 15 , Kalra S 16 , Kang HG 17 , Lucchetti L 18 , Lugani F 5 , Ma AL 19 , Morello W 20 , Camargo Muñiz MD 21 , Pradhan SK 22 , Prikhodina L 23 , Raafat RH 24 , Sinha R 25 , Teo S 8 , Tomari K 26 , Vivarelli M 27 , Webb H 28 , Yap HK 8 , Yap DY 29 , Tullus K 30

Affiliations 

  • 1 Department of Paediatrics, The Chinese University of Hong Kong, Shatin, Hong Kong SAR; Paediatric Nephrology Centre, Department of Paediatric and Adolescent Medicine, Hong Kong Children's Hospital, Kowloon Bay, Hong Kong SAR. Electronic address: eugene.chan@cuhk.edu.hk
  • 2 Division of Nephrology, Department of Pediatrics, Indian Council of Medical Research Advanced Center for Research in Nephrology, India Institute of Medical Sciences, New Delhi, India
  • 3 Clinical Research Center, Princess Margaret Hospital, Lai Chi Kok, Hong Kong SAR
  • 4 Department of Pediatric Nephrology, University of Child Health Sciences, The Children's Hospital Lahore, Pakistan
  • 5 Division of Nephrology, Dialysis, and Transplantation, IRCCS (Scientific Institute for Research and Health Care) Istituto Giannina Gaslini, Genoa, Italy
  • 6 Department of Pediatrics, Calcutta Medical Research Institute, Kolkata, India
  • 7 Néphrologie Pédiatrique, Centre de Référence du Syndrome Néphrotique de l'Enfant et de l'Adulte, Hôpital Necker Enfants Malades, Assistance publique-hôpitaux de Paris (APHP), Institut Imagine, Institut national de la santé et de la recherche médicale (INSERM) U1163, Université de Paris, Paris, France
  • 8 Department of Paediatrics, Yong Loo Lin School of Medicine, National University of Singapore, Singapore; Department of Paediatrics, Khoo Teck Puat-National University Children's Medical Institute, National University Health System, Singapore
  • 9 Department of Nephrology, Queensland Children's Hospital, Brisbane, Australia
  • 10 Department of Nephrology and Rheumatology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan
  • 11 Aliasghar Clinical Research Development Center, Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
  • 12 Department of Pediatrics, University Medical Center Hamburg-Eppendorf, University Children's Hospital, Hamburg, Germany
  • 13 Department of Pediatrics, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, Kota Bharu, Kelantan, Malaysia
  • 14 Children Kidney Center, Department of Pediatrics, King Edward Medical University, Lahore, Pakistan
  • 15 Department of Nephrology and Renal Replacement Therapy, Shupyk National Healthcare University of Ukraine, Kyiv, Ukraine; Department of Nephrology and Extracorporeal Treatment, Bogomolets National Medical University Kyiv, Kyiv, Ukraine
  • 16 Department of Pediatrics, Command Hospital, New Delhi, India
  • 17 Department of Pediatrics, Kidney Disease Center for Children and Adolescents, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea
  • 18 Division of Nephrology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
  • 19 Paediatric Nephrology Centre, Department of Paediatric and Adolescent Medicine, Hong Kong Children's Hospital, Kowloon Bay, Hong Kong SAR
  • 20 Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy
  • 21 Department of Pediatrics, Northeast National Medical Center, High Specialty Medical Unit No. 25, Instituto Mexicano del Seguro Social, Monterrey, N.L., México
  • 22 Division of Pediatric Nephrology, Sardar Vallabhbhai Patel Post Graduate Institute of Paediatrics (SVPPGIP) and Srirama Chandra Bhanja (SCB) Medical College, Cuttack, Odisha, India
  • 23 Division of Inherited & Acquired Kidney Diseases, Veltishev Research Clinical Institute for Pediatrics & Children Surgery, Pirogov Russian National Research Medical University, Moscow, Russia; Russian Medical Academy of Continuous Postgraduate Education, Moscow, Russia
  • 24 Division of Pediatric Nephrology and Pediatric Kidney Transplant, Joe DiMaggio Children's Hospital, Memorial Health System, Hollywood, Florida, USA
  • 25 Division of Paediatric Nephrology, Institute of Child Health, Kolkata, India
  • 26 Department of General Pediatrics, Okinawa Prefectural Nanbu Medical Center and Children's Medical Center, Okinawa, Japan
  • 27 Laboratory of Nephrology and Clinical Trial Center, Bambino Gesù Children's Hospital, IRCCS (Scientific Institute for Research and Health Care), Rome, Italy
  • 28 Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK
  • 29 Division of Nephrology, Department of Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong. Electronic address: desmondy@hku.hk
  • 30 Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK. Electronic address: kjell.tullus@gosh.nhs.uk
Kidney Int, 2024 Dec;106(6):1146-1157.
PMID: 39395629 DOI: 10.1016/j.kint.2024.09.011

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.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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