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Outcome of immunosuppression in children with IgA vasculitis-related nephritis.

Authors :
Rohner K
Marlais M
Ahn YH
Ali A
Alsharief A
Novak AB
Brambilla M
Cakici EK
Candan C
Canpolat N
Chan EY
Decramer S
Didsbury M
Durao F
Durkan AM
Düzova A
Forbes T
Gracchi V
Güngör T
Horinouchi T
Kasap Demir B
Kobayashi Y
Koskela M
Kurt-Sukur ED
La Scola C
Langan D
Li X
Malgieri G
Mastrangelo A
Min J
Mizerska-Wasiak M
Moussaoui N
Noyan A
Nuutinen M
O'Gormon J
Okamoto T
Oni L
Oosterveld M
Pańczyk-Tomaszewska M
Parmaksiz G
Pasini A
Rianthavorn P
Roelofs J
Shen Y
Sinha R
Topaloglu R
Torres DD
Udagawa T
Wennerström M
Yap YC
Tullus K
Source :
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association [Nephrol Dial Transplant] 2024 Jul 31; Vol. 39 (8), pp. 1299-1309.
Publication Year :
2024

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.<br />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.<br />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.<br />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.<br /> (© The Author(s) 2024. Published by Oxford University Press on behalf of the ERA.)

Details

Language :
English
ISSN :
1460-2385
Volume :
39
Issue :
8
Database :
MEDLINE
Journal :
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
Publication Type :
Academic Journal
Accession number :
38211969
Full Text :
https://doi.org/10.1093/ndt/gfae009