Association of the ANCA Kidney Risk Score With Kidney Failure in Anti-Glomerular Basement Membrane Disease
Year: 2026
Type: article
Study design: Observational Study
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.
Source: Kidney International Reports
Authors: Pauline Caillard, Cécile Vigneau, Jean-Michel Halimi, Marc Hazzan, Eric Thervet, Morgane Heitz, Laurent Juillard, Vincent Audard, Marion Rabant, Alexandre Hertig, Benoit Brilland, Vincent Vuiblet, Dominique Guerrot, Mathilde Tamain, Marie Essig, Thierry Lobbedez, Thomas Quemeneur, Mathieu Legendre, Alexandre Ganea, Marie-Noëlle Peraldi, François Vrtovsnik, Maïté Daroux, Gabriel Choukroun, Dimitri Titeca-Beauport
Institutions: Centre National de la Recherche Scientifique, Inserm, Centre Hospitalier Universitaire de Limoges, Contrôle de la Réponse Immune B et Lymphoproliférations, Université de Limoges, École des Hautes Études en Santé Publique, Institut de Recherche en Santé, Environnement et Travail, Centre Hospitalier Universitaire de Rennes, Université de Rennes, Université de Tours, Centre Hospitalier Universitaire de Tours, Université de Lille, Centre Hospitalier Universitaire de Lille, Assistance Publique – Hôpitaux de Paris, Hôpital Européen Georges-Pompidou, Paris Cardiovascular Research Center, Centre Hospitalier Annecy Genevois, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Université Paris-Est Créteil, Centre Hospitalier Universitaire Henri-Mondor, Hôpitaux Universitaires Henri-Mondor, Hôpital Necker-Enfants Malades, Université Paris Cité, Université de Versailles Saint-Quentin-en-Yvelines, Hôpital Foch, Centre de Recherche en Cancérologie et Immunologie Intégrée Nantes Angers, Centre Hospitalier Universitaire d'Angers, Université d'Angers, Nantes Université, Centre Hospitalier Universitaire de Reims, Université Paris-Saclay, Hôpital Ambroise-Paré, Centre Hospitalier Universitaire de Caen Normandie, Centre Hospitalier de Valenciennes, CHU Dijon Bourgogne, Centre hospitalier universitaire d'Orléans, Sorbonne Paris Cité, Hôpital Bichat-Claude-Bernard, Université Paris Diderot, Centre Hospitalier de Boulogne sur Mer, Mécanismes physiopathologiques et conséquences des calcifications cardiovasculaires, Université de Picardie Jules Verne
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,800
OpenAlex ID: W7220733677