Abstract Introduction The ANCA Renal Risk Score was revised in 2023 to improve clinico-pathological prognostication in patients with ANCA-associated vasculitis and kidney involvement. Our study aimed to assess whether incorporating recently identified predictors of kidney survival in ANCA-associated vasculitis could further refine the prognostic accuracy of the updated ANCA Renal Risk Score (AKRiS) in our multicentric cohort. Methods We retrospectively reviewed all incident ANCA-associated vasculitis with kidney biopsy from 2005 to 2020. Cox regression analysis examined factors (AKRiS, dialysis within 4 weeks, UPCR and hematuria at baseline, C3 deposits, renal arteritis on biopsy, eGFR, UPCR and hematuria after induction) associated with kidney failure. These factors in combination with AKRis were analyzed using the area under the receiver operating characteristic curve (AUROC). Results The cohort included 115 patients (age 64, 55% male, 57% myeloperoxidase-ANCA, baseline creatinine 3.6 mg/dl, eGFR 16 ml/min/1.73m2), with 34 (30%) dialysed within 4 weeks. During a median 6.4 year follow-up, 39 (34%) patients progressed to kidney failure, and 13 (11%) died. Cox analysis identified AKRiS, dialysis within 4 weeks, C3 deposits, renal arteritis on biopsy, lower eGFR after induction and higher UPCR after induction as unadjusted risk factors for kidney failure. After adjusting for AKRiS, dialysis within 4 weeks (HR 6.20 (2.76 to 13.95), P≤0.001), eGFR after induction (HR 0.94 (0.89 to 0.99, P=0.03)) and UPCR after induction (HR 1.62 (1.02 to 2.58), P=0.04) remained significantly associated with kidney outcome. The AUROC was 0.77 for AKRiS, increasing to 0.82, 0.80 and 0.79 when adding dialysis within 4 weeks, eGFR and UPCR after induction, respectively. Conclusion Dialysis within 4 weeks, eGFR after induction and UPCR after induction are able to predict long-term kidney outcome in AAV patients. Adjusting AKRiS for these variables slightly enhances its predictive power. We propose using them as placeholder endpoints for kidney failure in future studies.
Maalouli et al. (Tue,) studied this question.