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29-06-2023 | IgA Vasculitis | Original Article

Long-term outcome of combination therapy with corticosteroids, mizoribine and RAS inhibitors as initial therapy for severe childhood IgA vasculitis with nephritis

Authors: Sadayuki Nagai, Tomoko Horinouchi, Takeshi Ninchoji, Yuta Ichikawa, Yu Tanaka, Hideaki Kitakado, Chika Ueda, Atsushi Kondo, Yuya Aoto, Nana Sakakibara, Hiroshi Kaito, Ryojiro Tanaka, Yuko Shima, Junya Fujimura, Naohiro Kamiyoshi, Shingo Ishimori, Koichi Nakanishi, Norishige Yoshikawa, Kazumoto Iijima, Kandai Nozu

Published in: Pediatric Nephrology | Issue 12/2023

Abstract

Background

Patients with severe IgA vasculitis with nephritis (IgAVN) typically receive aggressive therapy as an initial approach. We have consistently performed combination therapy including corticosteroids and immunosuppressants as initial therapy for severe IgAVN over a 20-year-plus period, with only minor changes to the treatment protocol. This study seeks to reveal the efficacy of combination therapy for severe IgAVN.

Methods

We retrospectively studied 50 Japanese children diagnosed between 1996 and 2019 with clinicopathologically severe IgAVN who were defined as ISKDC classification grade IIIb-V and/or serum albumin < 2.5 g/dL.

Results

The median age at the onset of IgAVN was 8.0 years (IQR: 6.0–10.0). At biopsy, 44% of patients had nephrotic syndrome and 14% had kidney dysfunction. All patients were treated with combination therapy after biopsy. Abnormal proteinuria resolved after initial therapy in all 50 patients. However, eight patients (16%) had recurrence of proteinuria. Abnormal proteinuria was again resolved in three of these patients with additional treatment. At the last follow-up (median 59.5 months; IQR, 26.2–84.2), the median urine protein-to-creatine ratio was 0.08 g/gCr (IQR, 0.05–0.15), and only one patient had kidney dysfunction.

Conclusions

Combination therapy provided good kidney outcomes for Japanese children with severe IgAVN. Even including recurrent cases, the degree of proteinuria was slight, and kidney function was good at the last follow-up.

Graphical abstract

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Title: Long-term outcome of combination therapy with corticosteroids, mizoribine and RAS inhibitors as initial therapy for severe childhood IgA vasculitis with nephritis
Authors: Sadayuki Nagai
Tomoko Horinouchi
Takeshi Ninchoji
Yuta Ichikawa
Yu Tanaka
Hideaki Kitakado
Chika Ueda
Atsushi Kondo
Yuya Aoto
Nana Sakakibara
Hiroshi Kaito
Ryojiro Tanaka
Yuko Shima
Junya Fujimura
Naohiro Kamiyoshi
Shingo Ishimori
Koichi Nakanishi
Norishige Yoshikawa
Kazumoto Iijima
Kandai Nozu
Publication date: 29-06-2023
Publisher: Springer Berlin Heidelberg
Keywords: IgA Vasculitis
IgA Vasculitis
Nephrotic Syndrome
Hematuria
Glomerulonephritis
Published in: Pediatric Nephrology / Issue 12/2023
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-023-06052-3

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Long-term outcome of combination therapy with corticosteroids, mizoribine and RAS inhibitors as initial therapy for severe childhood IgA vasculitis with nephritis

Abstract

Background

Methods

Results

Conclusions

Graphical abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

Graphical abstract

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