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Comparative performance of renal histological scores in predicting kidney outcomes in ANCA-associated vasculitis.

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Title: Comparative performance of renal histological scores in predicting kidney outcomes in ANCA-associated vasculitis.
Alternate Title: Comparacioń de los scores histologicos ́ renales en la prediccioń de la evolucioń renal en la vasculitis asociada a ANCA.
Authors: Oliveira-Silva, Catarina1 catarina.ors@gmail.com, Coelho, Cláudia1, Viana, Johanna1, Rocha, Joana1, Falcão, Luís1, Teixeira, Catarina1, Costa, Rui1, Vaz, Raquel1
Source: Nefrologia. Jun/Jul2026, Vol. 46 Issue 6, p1-11. 11p.
Subjects: PREDICTION models, ANTINEUTROPHIL cytoplasmic antibodies, CHRONIC kidney failure, DISEASE risk factors
Abstract (English): Background: Kidney involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains a major determinant of adverse patient outcomes. Several histological and clinicopathological scoring systems have been proposed to predict kidney prognosis and guide treatment decisions. Methods: This retrospective cohort study evaluated patients with kidney biopsy-proven AAV, diagnosed between January 2013 and December 2023. The primary endpoint was progression to end-stage kidney disease (ESKD). Cox regression analysis was used to evaluate clinical and histological predictors of ESKD. Kaplan–Meier analysis, Harrell’s C-statistics, and Crameŕ ’s V were used to assess the performance and concordance of Berden classification, Mayo Clinic Chronicity Score (MCCS), Renal Risk Score (RRS), and Improved ANCA Kidney Risk Score (AKRiS). Results: Of 53 included patients, 22.6% progressed to ESKD. Interstitial fibrosis and tubular atrophy ≥25% wasthe strongest histological predictor of ESKD, and MCCS (HR 1.574 [95% CI 1.123–2.206]), RRS (HR 1.405 [95% CI 1.110–1.777]) and AKRiS (HR 1.254 [95% CI 1.090–1.442]) proved to be independent predictors of ESKD. AKRiS showed the highest discriminative performance (C-statistic 0.871 [95% CI 0.781–0.989]) and differentiated kidney survival across all risk categories. Cross-comparison revealed significant concordance among low and high-risk groups of MCCS, RRS, and AKRiS. Conclusions: In a contemporary cohort of AAV, MCCS and AKRiS demonstrated the strongest predictive performance for ESKD. The combined use of histological and clinicopathological scores may improve risk stratification and support more personalized treatment decisions, particularly in patients with intermediate risk. [ABSTRACT FROM AUTHOR]
Abstract (Spanish): Introducción: La afectación renal en la vasculitis asociada a anticuerpos anticitoplasma de neutrófilo (AAV) es un determinante mayor de resultados adversos en los pacientes. Se han propuesto varios sistemas de puntuación histológicos y clinicopatológicos para predecir el pronóstico renal y guiar las decisiones terapéuticas. Métodos: Este estudio de cohorte retrospectivo evaluó a pacientes con AAV confirmada por biopsia renal, diagnosticados entre enero de 2013 y diciembre de 2023. El objetivo principal fue la progresión a enfermedad renal terminal (ERT). Se utilizó análisis de regresión de Cox para evaluar los predictores clínicos e histológicos de ERT. El análisis de Kaplan-Meier, las estadísticas C de Harrell y el V de Cramér se emplearon para evaluar el rendimiento y la concordancia de la clasificación de Berden, la Puntuación de Cronicidad de la Mayo Clinic (MCCS), el Renal Risk Score (RRS) y el Improved ANCA Kidney Risk Score (AKRIS). Resultados: De los 53 pacientes incluidos, el 22,6% progresó a ERT. La fibrosis intersticial y atrofia tubular ≥25% fue el principal predictor histológico de ERT, y MCCS (HR 1,574 [IC95% 1,123-2,206]), RRS (HR 1,405 [IC95% 1,110-1,777]) y AKRIS (HR 1,254 [IC95% 1,090-1,442]) demostraron ser predictores independientes de ERT. AKRIS mostró el mejor rendimiento discriminativo (estadístico C 0.871 [95% CI 0.781-0.989]) у diferenció la supervivencia renal en todas las categorías de riesgo. La comparación cruzada reveló una concordancia significativa entre los grupos de bajo y alto riesgo de MCCS, RRS y AKRIS. Conclusiones: En una cohorte contemporánea de AAV, MCCS y AKRIS demostraron el mejor poder discriminativo para ERT. El uso combinado de puntuaciones histológicas y clinicopatológicas puede mejorar la estratificación del riesgo y apoyar decisiones terapéuticas más personalizadas en pacientes con riesgo intermedio. [ABSTRACT FROM AUTHOR]
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Abstract:Background: Kidney involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains a major determinant of adverse patient outcomes. Several histological and clinicopathological scoring systems have been proposed to predict kidney prognosis and guide treatment decisions. Methods: This retrospective cohort study evaluated patients with kidney biopsy-proven AAV, diagnosed between January 2013 and December 2023. The primary endpoint was progression to end-stage kidney disease (ESKD). Cox regression analysis was used to evaluate clinical and histological predictors of ESKD. Kaplan–Meier analysis, Harrell’s C-statistics, and Crameŕ ’s V were used to assess the performance and concordance of Berden classification, Mayo Clinic Chronicity Score (MCCS), Renal Risk Score (RRS), and Improved ANCA Kidney Risk Score (AKRiS). Results: Of 53 included patients, 22.6% progressed to ESKD. Interstitial fibrosis and tubular atrophy ≥25% wasthe strongest histological predictor of ESKD, and MCCS (HR 1.574 [95% CI 1.123–2.206]), RRS (HR 1.405 [95% CI 1.110–1.777]) and AKRiS (HR 1.254 [95% CI 1.090–1.442]) proved to be independent predictors of ESKD. AKRiS showed the highest discriminative performance (C-statistic 0.871 [95% CI 0.781–0.989]) and differentiated kidney survival across all risk categories. Cross-comparison revealed significant concordance among low and high-risk groups of MCCS, RRS, and AKRiS. Conclusions: In a contemporary cohort of AAV, MCCS and AKRiS demonstrated the strongest predictive performance for ESKD. The combined use of histological and clinicopathological scores may improve risk stratification and support more personalized treatment decisions, particularly in patients with intermediate risk. [ABSTRACT FROM AUTHOR]
ISSN:02116995
DOI:10.1016/j.nefro.2026.501520