Outcome of immunosuppression in children with IgA vasculitis-related nephritis

Rohner K 1 , Marlais M 2 , Ahn YH 3 , Ali A 4 , Alsharief A 5 , Novak AB 6 Show all authors , Brambilla M 7 , Cakici EK 8 , Candan C 9 , Canpolat N 10 , Chan EY 11 , Decramer S 12 , Didsbury M 13 , Durao F 14 , Durkan AM 15 , Düzova A 16 , Forbes T 13 , Gracchi V 17 , Güngör T 8 , Horinouchi T 18 , Kasap Demir B 19 , Kobayashi Y 20 , Koskela M 21 , Kurt-Sukur ED 16 , La Scola C 22 , Langan D 23 , Li X 24 , Malgieri G 25 , Mastrangelo A 7 , Min J 26 , Mizerska-Wasiak M 27 , Moussaoui N 12 , Noyan A 28 , Nuutinen M 29 , O'Gormon J 30 , Okamoto T 31 , Oni L 32 , Oosterveld M 33 , Pańczyk-Tomaszewska M 27 , Parmaksiz G 28 , Pasini A 22 , Rianthavorn P 34 , Roelofs J 35 , Shen Y 24 , Sinha R 36 , Topaloglu R 16 , Torres DD 37 , Udagawa T 38 , Wennerström M 5 , Yap YC 39 , Tullus K 2

Affiliations 

  • 1 Department of Pediatric Nephrology, University Children's Hospital Zurich, Zurich, Switzerland
  • 2 Paediatric Nephrology Department, Great Ormond Street Hospital for Children, London, UK
  • 3 Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
  • 4 Great North Children's Hospital Newcastle, Newcastle, UK
  • 5 Department of Pediatric Nephrology, The Queen Silvia Children´s Hospital, Sahlgrenska University Hospital, Göteborg, Sweden
  • 6 Pediatric Nephrology Department, Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia
  • 7 Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca' Grande Ospedale Maggiore Policlinico, Milan, Italy
  • 8 Dr Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Department of Pediatric Nephrology, Ankara, Türkiye
  • 9 Istanbul Medeniyet University, Department of Pediatric Nephrology, Istanbul, Türkiye
  • 10 Department of Pediatric Nephrology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Türkiye
  • 11 Paediatric Nephrology Centre, Hong Kong Children's Hospital, Kowloon Bay, Hong Kong SAR
  • 12 Paediatric Nephrology Centre, Hôpital des Enfants, CHU Purpan, Centre de Référence du Sud-Ouest des Maladies Rénales Rares SORARE, Filière ORKiD Toulouse, Toulouse, France
  • 13 Department of Nephrology, Royal Children's Hospital, Melbourne, Australia
  • 14 Pediatric Nephrology and Kidney Transplantation Unit, Department of Pediatrics, Hospital Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal
  • 15 Department of Nephrology, The Children's Hospital at Westmead, Sydney, Australia
  • 16 Division of Pediatric Nephrology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Türkiye
  • 17 Department of pediatric nephrology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
  • 18 Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • 19 İzmir Katip Çelebi University, Department of Pediatrics, Division of Pediatric Nephrology and Rheumatology, Izmir, Türkiye
  • 20 Gunma University Graduate School of Medicine, Department of Pediatric, Gunma, Japan
  • 21 Department of Pediatric Nephrology and Transplantation, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
  • 22 Nephrology and Dialysis Unit, Department of Pediatrics, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
  • 23 UCL Great Ormond Street Institute for Child Health, University College London, London, UK
  • 24 Pediatric Nephrologic and Immunologic Department, Children's Hospital of Soochow University, Soochow, China
  • 25 Division of Nephrology, Dialysis and Transplant, AORN Santobono Pausilipon, Napoli, Italy
  • 26 Department of Pediatrics, Chungnam National University Sejong Hospital, Sejong, Korea
  • 27 Department of Pediatrics and Nephrology, Medical University of Warsaw, Warsaw, Poland
  • 28 Baskent University, Dr Turgut Noyan Training and Research Center, Department of Pediatric Nephrology, Adana, Türkiye
  • 29 Department of Children and Adolescents, Oulu University Hospital, Oulu, Finland
  • 30 Royal Belfast Hospital for Sick Children, Belfast, Northern Ireland
  • 31 Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan
  • 32 Department of Women's and Children's Health, Alder Hey Children's NHS Foundation Trust Hospital and University of Liverpool, Liverpool, UK
  • 33 Emma Children's Hospital, Amsterdam University Medical Center, Amsterdam, The Netherlands
  • 34 Department of Pediatrics, Faculty of Medecine, Chulalongkorn University, Bangkok, Thailand
  • 35 Department of Pathology, Amsterdam UMC, University of Amsterdam, Amsterdam Cardiovascular Sciences, Amsterdam, The Netherlands
  • 36 Division of Pediatric Nephrology, Institute of Child Health, Kolkata, India
  • 37 Pediatric Nephrology and Dialysis Unit, Pediatric Hospital Giovanni XXIII, Bari, Italy
  • 38 Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University, Tokyo, Japan
  • 39 Department of Paediatric, Hospital Tunku Azizah, Women and Children Hospital Kuala Lumpur, Malaysia
Nephrol Dial Transplant, 2024 Jul 31;39(8):1299-1309.
PMID: 38211969 DOI: 10.1093/ndt/gfae009

Abstract

BACKGROUND: Immunoglobulin A vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Due to a lack of evidence, treatment recommendations are based on expert opinion, resulting in variation. The aim of this study was to describe the clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy-proven IgAVN in order to identify prognostic risk factors and signals of treatment efficacy.

METHODS: Retrospective data were collected on 1148 children with biopsy-proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analysed using multivariate analysis. The primary outcome was estimated glomerular filtration rate (eGFR) and persistent proteinuria at last follow-up.

RESULTS: The median follow-up was 3.7 years (interquartile range 2-6.2). At last follow-up, 29% of patients had an eGFR <90 mL/min/1.73 m2, 36% had proteinuria and 3% had chronic kidney disease stage 4-5. Older age, lower eGFR at onset, hypertension and histological features of tubular atrophy and segmental sclerosis were predictors of poor outcome. There was no evidence to support any specific second-line immunosuppressive regimen being superior to others, even when further analysing subgroups of children with reduced kidney function, nephrotic syndrome or hypoalbuminemia at onset. Delayed start of immunosuppressive treatment was associated with a lower eGFR at last follow-up.

CONCLUSION: In this large retrospective cohort, key features associated with disease outcome are highlighted. Importantly, there was no evidence to support that any specific immunosuppressive treatments were superior to others. Further discovery science and well-conducted clinical trials are needed to define accurate treatment and improve outcomes of IgAVN.

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

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