Association of the ANCA Kidney Risk Score with kidney failure in anti-GBM disease
Abstract
Introduction Anti-glomerular basement membrane (anti-GBM) disease is a rare vasculitis that is characterized by a poor renal prognosis. Despite the high predictive accuracy of the improved ANCA Kidney Risk Score (AKRiS) in ANCA-associated vasculitis, its prognostic value in anti-GBM disease remains to be established. Methods In this multicenter retrospective study, 163 biopsy-proven anti-GBM patients were included. AKRiS was calculated at diagnosis, stratifying patients into low-, moderate-, high-, and very high-risk groups. The Fine-Gray model was used for the analysis of 36-month kidney failure, with the predictive performance assessed via Harrell's C-index. Results The median AKRiS was 18 [11–21], with 69% of patients classified as high or very high-risk. The 36-month cumulative incidence of kidney failure was significantly higher in the high- (92% [95%CI: 82–97]) and very high-risk (98% [95%CI: 86–100]) groups, when compared to the low-risk (40% [95%CI: 19–61]; p<0.0001). In the multivariable analysis, AKRiS was independently associated with a poor renal prognosis (sHR 2.51 and 2.82 for the high- and very high-risk groups vs. low-risk, respectively). The model combining oligoanuria and AKRiS improved the predictive performance of AKRiS alone (C-index 0.90 [95%CI: 0.85–0.94] vs 0.85 [95%CI: 0.80–0.91]; p=0.02). Despite higher AKRiS scores, double-positive patients had similar 36-month kidney failure compared to single-positive patients, reflecting their divergent pathophysiological and therapeutic responsiveness. Conclusion AKRiS is associated with renal risk in anti-GBM disease, irrespective of ANCA status. Its predictive performance further improves when combined with oligoanuria, supporting the development of pragmatic prognostic models for anti-GBM disease.
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BibTeX
@article{caillard2026association,
title = {{Association of the ANCA Kidney Risk Score with kidney failure in anti-GBM disease}},
author = {Pauline Caillard and Cécile Vigneau and Jean-Michel Halimi and Marc Hazzan and Eric Thervet and Morgane Heitz and Laurent Juillard and Vincent Audard and Marion Rabant and Alexandre Hertig and Benoit Brilland and Vincent Vuiblet and Dominique Guerrot and Mathilde Tamain and Marie Essig and Thierry Lobbedez and Thomas Quemeneur and Mathieu Legendre and Alexandre Ganea and Marie-Noëlle Peraldi and François Vrtovsnik and Maïté Daroux and Gabriel Choukroun and Dimitri Titeca-Beauport},
journal = {Kidney International Reports},
year = {2026},
month = oct,
doi = {10.1016/j.ekir.2026.107130},
url = {https://u-picardie.hal.science/hal-05795068v1},
note = {CC BY},
}
RIS
TY - JOUR
TI - Association of the ANCA Kidney Risk Score with kidney failure in anti-GBM disease
AU - Pauline Caillard
AU - Cécile Vigneau
AU - Jean-Michel Halimi
AU - Marc Hazzan
AU - Eric Thervet
AU - Morgane Heitz
AU - Laurent Juillard
AU - Vincent Audard
AU - Marion Rabant
AU - Alexandre Hertig
AU - Benoit Brilland
AU - Vincent Vuiblet
AU - Dominique Guerrot
AU - Mathilde Tamain
AU - Marie Essig
AU - Thierry Lobbedez
AU - Thomas Quemeneur
AU - Mathieu Legendre
AU - Alexandre Ganea
AU - Marie-Noëlle Peraldi
AU - François Vrtovsnik
AU - Maïté Daroux
AU - Gabriel Choukroun
AU - Dimitri Titeca-Beauport
PY - 2026
DA - 2026/10/12
JO - Kidney International Reports
DO - 10.1016/j.ekir.2026.107130
UR - https://u-picardie.hal.science/hal-05795068v1
AB - Introduction Anti-glomerular basement membrane (anti-GBM) disease is a rare vasculitis that is characterized by a poor renal prognosis. Despite the high predictive accuracy of the improved ANCA Kidney Risk Score (AKRiS) in ANCA-associated vasculitis, its prognostic value in anti-GBM disease remains to be established. Methods In this multicenter retrospective study, 163 biopsy-proven anti-GBM patients were included. AKRiS was calculated at diagnosis, stratifying patients into low-, moderate-, high-, and very high-risk groups. The Fine-Gray model was used for the analysis of 36-month kidney failure, with the predictive performance assessed via Harrell's C-index. Results The median AKRiS was 18 [11–21], with 69% of patients classified as high or very high-risk. The 36-month cumulative incidence of kidney failure was significantly higher in the high- (92% [95%CI: 82–97]) and very high-risk (98% [95%CI: 86–100]) groups, when compared to the low-risk (40% [95%CI: 19–61]; p<0.0001). In the multivariable analysis, AKRiS was independently associated with a poor renal prognosis (sHR 2.51 and 2.82 for the high- and very high-risk groups vs. low-risk, respectively). The model combining oligoanuria and AKRiS improved the predictive performance of AKRiS alone (C-index 0.90 [95%CI: 0.85–0.94] vs 0.85 [95%CI: 0.80–0.91]; p=0.02). Despite higher AKRiS scores, double-positive patients had similar 36-month kidney failure compared to single-positive patients, reflecting their divergent pathophysiological and therapeutic responsiveness. Conclusion AKRiS is associated with renal risk in anti-GBM disease, irrespective of ANCA status. Its predictive performance further improves when combined with oligoanuria, supporting the development of pragmatic prognostic models for anti-GBM disease.
SN - 2468-0249
ER -