Outcome of immunosuppression in children with IgA vasculitis–related nephritis

Katharina Rohner; Matko Marlais; Yo Han Ahn; Alaa Ali; Abrar Alsharief; Anja Blejc Novak; Marta Brambilla; Evrim Kargin Cakici; Cengiz Candan; Nur Canpolat; Eugene Yu Hin Chan; Stéphane Decramer; Madeleine Didsbury; Filipa Durao; Anne M. Durkan; Ali Düzova; Thomas Forbes; Valentina Gracchi; Tulin Güngör; Tomoko Horinouchi; Belde Kasap Demir; Yasuko Kobayashi; Mikael Koskela; Eda Didem Kurt Sukur Dean Langan; Xiaozhong Li; Gabriele Malgieri; Antonio Mastrangelo; Claudio La Scola; Jeesu Min; Malgorzata Mizerska-Wasiak; Nabila Moussaoui; Aytul Noyan; Matti Nuutinen; Jennifer O’Gormon; Takayuki Okamoto; Michiel Oosterveld; Malgorzata Pańczyk-Tomaszewska; Gonul Parmaksiz; Andrea Pasini; Pornpimol Rianthavorn; Louise Oni; Joris Roelofs; Yunyan Shen; Rajiv Sinha; Rezan Topaloglu; Diletta Domenica Torres; Tomohiro Udagawa; Martin Wennerström; Yok Chin Yap; Kjell Tullus

doi:10.1093/ndt/gfae009

Outcome of immunosuppression in children with IgA vasculitis–related nephritis

Katharina Rohner^*, Matko Marlais, Yo Han Ahn, Alaa Ali, Abrar Alsharief, Anja Blejc Novak, Marta Brambilla, Evrim Kargin Cakici, Cengiz Candan, Nur Canpolat, Eugene Yu Hin Chan, Stéphane Decramer, Madeleine Didsbury, Filipa Durao, Anne M. Durkan, Ali Düzova, Thomas Forbes, Valentina Gracchi, Tulin Güngör, Tomoko HorinouchiBelde Kasap Demir, Yasuko Kobayashi, Mikael Koskela, Eda Didem Kurt Sukur Dean Langan, Xiaozhong Li, Gabriele Malgieri, Antonio Mastrangelo, Claudio La Scola, Jeesu Min, Malgorzata Mizerska-Wasiak, Nabila Moussaoui, Aytul Noyan, Matti Nuutinen, Jennifer O’Gormon, Takayuki Okamoto, Michiel Oosterveld, Malgorzata Pańczyk-Tomaszewska, Gonul Parmaksiz, Andrea Pasini, Pornpimol Rianthavorn, Louise Oni, Joris Roelofs, Yunyan Shen, Rajiv Sinha, Rezan Topaloglu, Diletta Domenica Torres, Tomohiro Udagawa, Martin Wennerström, Yok Chin Yap, Kjell Tullus

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

5 Citations (Scopus)

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.

Original language	English
Pages (from-to)	1299-1309
Number of pages	11
Journal	Nephrology Dialysis Transplantation
Volume	39
Issue number	8
DOIs	https://doi.org/10.1093/ndt/gfae009
Publication status	Published - 1-Aug-2024

Keywords

children
Henoch-Schönlein purpura nephritis
IgA vasculitis nephritis
immunosuppression

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Rohner, K., Marlais, M., Ahn, Y. H., Ali, A., Alsharief, A., Novak, A. B., Brambilla, M., Cakici, E. K., Candan, C., Canpolat, N., Chan, E. Y. H., Decramer, S., Didsbury, M., Durao, F., Durkan, A. M., Düzova, A., Forbes, T., Gracchi, V., Güngör, T., ... Tullus, K. (2024). Outcome of immunosuppression in children with IgA vasculitis–related nephritis. Nephrology Dialysis Transplantation, 39(8), 1299-1309. https://doi.org/10.1093/ndt/gfae009

@article{782b2711ff30437c93c03c41cf52cbe7,

title = "Outcome of immunosuppression in children with IgA vasculitis–related nephritis",

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.",

keywords = "children, Henoch-Sch{\"o}nlein purpura nephritis, IgA vasculitis nephritis, immunosuppression",

author = "Katharina Rohner and Matko Marlais and Ahn, {Yo Han} and Alaa Ali and Abrar Alsharief and Novak, {Anja Blejc} and Marta Brambilla and Cakici, {Evrim Kargin} and Cengiz Candan and Nur Canpolat and Chan, {Eugene Yu Hin} and St{\'e}phane Decramer and Madeleine Didsbury and Filipa Durao and Durkan, {Anne M.} and Ali D{\"u}zova and Thomas Forbes and Valentina Gracchi and Tulin G{\"u}ng{\"o}r and Tomoko Horinouchi and Demir, {Belde Kasap} and Yasuko Kobayashi and Mikael Koskela and Langan, {Eda Didem Kurt Sukur Dean} and Xiaozhong Li and Gabriele Malgieri and Antonio Mastrangelo and Scola, {Claudio La} and Jeesu Min and Malgorzata Mizerska-Wasiak and Nabila Moussaoui and Aytul Noyan and Matti Nuutinen and Jennifer O{\textquoteright}Gormon and Takayuki Okamoto and Michiel Oosterveld and Malgorzata Pa{\'n}czyk-Tomaszewska and Gonul Parmaksiz and Andrea Pasini and Pornpimol Rianthavorn and Louise Oni and Joris Roelofs and Yunyan Shen and Rajiv Sinha and Rezan Topaloglu and Torres, {Diletta Domenica} and Tomohiro Udagawa and Martin Wennerstr{\"o}m and Yap, {Yok Chin} and Kjell Tullus",

year = "2024",

month = aug,

day = "1",

doi = "10.1093/ndt/gfae009",

language = "English",

volume = "39",

pages = "1299--1309",

journal = "Nephrology Dialysis Transplantation",

issn = "0931-0509",

publisher = "Oxford University Press",

number = "8",

}

Rohner, K, Marlais, M, Ahn, YH, Ali, A, Alsharief, A, Novak, AB, Brambilla, M, Cakici, EK, Candan, C, Canpolat, N, Chan, EYH, Decramer, S, Didsbury, M, Durao, F, Durkan, AM, Düzova, A, Forbes, T, Gracchi, V, Güngör, T, Horinouchi, T, Demir, BK, Kobayashi, Y, Koskela, M, Langan, EDKSD, Li, X, Malgieri, G, Mastrangelo, A, Scola, CL, Min, J, Mizerska-Wasiak, M, Moussaoui, N, Noyan, A, Nuutinen, M, O’Gormon, J, Okamoto, T, Oosterveld, M, Pańczyk-Tomaszewska, M, Parmaksiz, G, Pasini, A, Rianthavorn, P, Oni, L, Roelofs, J, Shen, Y, Sinha, R, Topaloglu, R, Torres, DD, Udagawa, T, Wennerström, M, Yap, YC & Tullus, K 2024, 'Outcome of immunosuppression in children with IgA vasculitis–related nephritis', Nephrology Dialysis Transplantation, vol. 39, no. 8, pp. 1299-1309. https://doi.org/10.1093/ndt/gfae009

TY - JOUR

T1 - Outcome of immunosuppression in children with IgA vasculitis–related nephritis

AU - Rohner, Katharina

AU - Marlais, Matko

AU - Ahn, Yo Han

AU - Ali, Alaa

AU - Alsharief, Abrar

AU - Novak, Anja Blejc

AU - Brambilla, Marta

AU - Cakici, Evrim Kargin

AU - Candan, Cengiz

AU - Canpolat, Nur

AU - Chan, Eugene Yu Hin

AU - Decramer, Stéphane

AU - Didsbury, Madeleine

AU - Durao, Filipa

AU - Durkan, Anne M.

AU - Düzova, Ali

AU - Forbes, Thomas

AU - Gracchi, Valentina

AU - Güngör, Tulin

AU - Horinouchi, Tomoko

AU - Demir, Belde Kasap

AU - Kobayashi, Yasuko

AU - Koskela, Mikael

AU - Langan, Eda Didem Kurt Sukur Dean

AU - Li, Xiaozhong

AU - Malgieri, Gabriele

AU - Mastrangelo, Antonio

AU - Scola, Claudio La

AU - Min, Jeesu

AU - Mizerska-Wasiak, Malgorzata

AU - Moussaoui, Nabila

AU - Noyan, Aytul

AU - Nuutinen, Matti

AU - O’Gormon, Jennifer

AU - Okamoto, Takayuki

AU - Oosterveld, Michiel

AU - Pańczyk-Tomaszewska, Malgorzata

AU - Parmaksiz, Gonul

AU - Pasini, Andrea

AU - Rianthavorn, Pornpimol

AU - Oni, Louise

AU - Roelofs, Joris

AU - Shen, Yunyan

AU - Sinha, Rajiv

AU - Topaloglu, Rezan

AU - Torres, Diletta Domenica

AU - Udagawa, Tomohiro

AU - Wennerström, Martin

AU - Yap, Yok Chin

AU - Tullus, Kjell

PY - 2024/8/1

Y1 - 2024/8/1

N2 - 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.

AB - 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.

KW - children

KW - Henoch-Schönlein purpura nephritis

KW - IgA vasculitis nephritis

KW - immunosuppression

UR - http://www.scopus.com/inward/record.url?scp=85196437780&partnerID=8YFLogxK

U2 - 10.1093/ndt/gfae009

DO - 10.1093/ndt/gfae009

M3 - Article

C2 - 38211969

AN - SCOPUS:85196437780

SN - 0931-0509

VL - 39

SP - 1299

EP - 1309

JO - Nephrology Dialysis Transplantation

JF - Nephrology Dialysis Transplantation

IS - 8

ER -

Outcome of immunosuppression in children with IgA vasculitis–related nephritis

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