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Published in: Pediatric Nephrology 4/2021

01-04-2021 | Hematuria | Original Article

Clinicopathological significance of glomerular capillary IgA deposition in childhood IgA nephropathy

Authors: Yuko Shima, Koichi Nakanishi, Hironobu Mukaiyama, Yu Tanaka, Takuzo Wada, Ryojiro Tanaka, Hiroshi Kaito, Kandai Nozu, Mayumi Sako, Kazumoto Iijima, Norishige Yoshikawa

Published in: Pediatric Nephrology | Issue 4/2021

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Abstract

Background

IgA nephropathy (IgAN) is characterized by predominant mesangial IgA deposition. Some patients with IgAN demonstrate IgA deposition in glomerular peripheral capillaries (cap-IgA). The clinicopathological significance of cap-IgA remains incompletely investigated in children.

Methods

We retrospectively analyzed 503 consecutive cases of biopsy-proven childhood IgAN between July 1976 and June 2013 to compare clinical and pathological features between IgAN patients with and without cap-IgA.

Results

Among the 503 patients, 30 (6.0%) had cap-IgA. We found significant differences in proteinuria (2.0 vs. 0.5 g/day/m2, p < 0.0001), time from onset to kidney biopsy (2.2 vs. 8.3 months, p < 0.0001), and rate of proteinuria remission after treatment (23.3% vs. 48.0%, p = 0.007) between both groups. Pathological analysis revealed significant differences in M1 (83.3% vs. 56.0%, p = 0.002), ratio of subendothelial electron dense deposits (EDDs, 58.6% vs. 16.5%, p < 0.0001) and subepithelial EDDs (48.3% vs. 16.5%, p = 0.0001), and glomerular basement membrane (GBM) lysis (58.6% vs. 27.1%, p = 0.0006) between both groups. More than half of cap-IgA patients (17/30, 56.7%), whereas only 26.2% of non-cap-IgA patients (124/473), were treated with immunosuppressive treatments. Six of 30 cases (20%) with cap-IgA reached glomerular filtration rate (GFR) categories G3a–G5 (estimated GFR < 60 ml/min/1.73 m2) at most recent observation (mean observation period: 7.0 ± 4.0 years). According to Kaplan-Meier analysis, patients with cap-IgA had significantly lower kidney survival curves than non-cap-IgA patients (72.8% vs. 97.2% at 10 years, p < 0.0001).

Conclusions

Cap-IgA is associated with acute inflammation with GBM changes, resulting in refractory heavier proteinuria. Cap-IgA may represent a poor prognostic factor.
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Metadata
Title
Clinicopathological significance of glomerular capillary IgA deposition in childhood IgA nephropathy
Authors
Yuko Shima
Koichi Nakanishi
Hironobu Mukaiyama
Yu Tanaka
Takuzo Wada
Ryojiro Tanaka
Hiroshi Kaito
Kandai Nozu
Mayumi Sako
Kazumoto Iijima
Norishige Yoshikawa
Publication date
01-04-2021
Publisher
Springer Berlin Heidelberg
Keyword
Hematuria
Published in
Pediatric Nephrology / Issue 4/2021
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-020-04772-4

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