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Kidney involvement is one of the most severe manifestations of ANCA-associated vasculitis (AAV) characterized by unfavorable outcomes. The aim of the study was to identify factors associated with kidney survival in patients with biopsy proven ANCA-associated glomerulonephritis (ANCA-GN).
In our multicenter retrospective cohort study, we enrolled adult (≥18 years) patients with biopsy-proven ANCA-GN who were followed between 2014 and 2025. All patients were stratified according to the histopathologic class (Berden et al), ANCA Renal Risk Score (ARRS), and ANCA Kidney Risk Score (AKRiS).
The primary end-point was time to CKD G5 as defined by KDIGO (2024). Time-to-event was measured from the date of kidney biopsy. Kidney function was measured in serum creatinine and GFR was estimated with the 2021 CKD-EPI (creatinine) equation.
Cox proportional hazards, Kaplan–Meier curves, Harrell’s C-statistic, and receiver operating characteristics were used to compare the characteristics of the prognostication tools.
The study was approved by the Sechenov University local ethics committee (#10-22, 19.05.2022).
We enrolled 195 patients, 85 (44%) males and 110 (56%) females. Median age at AAV onset was 54 (37; 60) years. The majority of patients (88%) were ANCA-positive. Thirty-nine (20%) patients were diagnosed with renal-limited AAV. Median Birmingham vasculitis activity score (BVAS v.3) at biopsy was 14 (12; 18). Median follow-up was 40 (15; 75) months.
Fifty-five (28%) patients developed CKD G5 (fig. 1). Histopathologic classification demonstrated a significantly lower predictive ability (Harrel’s C=0.669, 95% CI 0.599-0.739) compared to ARRS (C=0.790, 95% CI 0.741-0.839, p<0.001) and AKRiS (C=0.798, 95% CI 0.745-0.851, p=0.003).
In the first multivariate Cox regression model, sclerotic class (HR 3.86 [1.01–14.72]), baseline eGFR <15 mL/min/1.73 m2 (HR 11.30 (3.14-40.65]), IFTA >25% (HR 2.71 [1.21–6.09]), dialysis dependance at the time of biopsy (HR 2.77 (1.26–6.08]), and acute tubular necrosis (ATN, HR 2.65 [1.12–6.32]) showed significant association with CKD G5. In the second multivariate Cox regression model, ARRS high risk group (HR 4.47 [1.16–17.20]), hemoglobin <100 g/L (HR 2.73 [1.27–5.90]), global glomerulosclerosis ≥50% (HR 3.39 (1.53–7.49]), dialysis dependance at the time of biopsy (HR 3.57 [1.71–7.45]), and ATN (HR 2.33 [1.02–5.31]) were significantly associated with the outcome. In the multivariate Cox regression model 3, AKRiS very high risk group (HR 10.24 [3.08–34.07]), hemoglobin <100 g/L (HR 2.47 [1.02–5.95]), global glomerulosclerosis ≥50% (HR 4.79 [2.25 – 10.21]), dialysis dependance at the time of biopsy (HR 3.94 [1.80 – 8.62]), and ATN (HR 2.65 [1.13–6.21]) were associated with the outcome. Female sex was associated with a significantly lower risk of kidney outcome in all three models.
Figure 1. Kaplan-Meier curves showing kidney survival according to the histopathologic classification (A) and different risk groups according to ARRS (B) and AKRiS (C).
In our multicenter cohort, sclerotic class, high risk and very high risk groups stratified according to ARRS and AKRiS, respectively, global glomerulosclerosis ≥50%, ATN, baseline hemoglobin <100 g/L, and dialysis dependance at the time of kidney biopsy were significantly associated with worse renal survival in patients with ANCA-GN.
