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Background Recently, renal risk score on the basis of three clinicopathologic features to predict end-stage renal disease (ESRD) in antineutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis has been proposed. The aim of this multi-centre study was to validate this renal risk score in a large cohort of southern European patients. Methods Data were retrospectively collected from the time of diagnosis by systematic review of medical records from 147 patients with renal vasculitis recruited from three Spanish centres. The renal risk score was calculated in every patient, and renal and global outcomes were analysed according to the risk group assessment. Results ANCA serology was positive in 76.2% of patients: 64.6% showed activity against myeloperoxidase (MPO) and 12.2% against proteinase 3 (PR3). The median (interquartile range) follow-up period was 41 months (9.6–104). Forty-eight patients (32.7%) reached ESRD. Patients were classified into the three groups according to the risk of progression to ESRD: 21.8% of patients were classified into low risk, 52.4% were classified into moderate risk and the remaining 25.9% were classified into high risk. The cumulative proportion of renal survival at 2, 5 and 10 years was 100, 100 and 82% in the low-risk group, 79, 77 and 77% in the medium-risk group and 63, 53 and 40% in the high-risk group (P &amp;lt; 0.001). In regression analysis, the risk score was a good predictor for the development of the ESRD among ANCA positive [hazard ratio (HR) = 2.7, 95% confidence interval (CI) 1.4–4.9; P &amp;lt; 0.001] and ANCA negative (HR = 2.7, 95% CI 1.04–7.1, P = 0.04) patients. Conclusions The renal risk score constitutes an accurate tool to predict renal outcome among patients with renal vasculitis. This study contributes to validate the risk scoring system in a MPO-predominant population, but also among ANCA-negative vasculitis patients.

Long-term validation of the renal risk score for vasculitis in a Southern European population