The Improved Kidney Risk Score in ANCA-Associated Vasculitis for Clinical Practice and Trials

Sebastian Bate, Dominic McGovern, Francesca Costigliolo, Pek Ghe Tan, Vojtech Kratky, Jennifer Scott, Gavin B. Chapman, Nina Brown, Lauren Floyd, Benoit Brilland, Eduardo Martín-Nares, Mehmet Fethullah Aydın, Duha Ilyas, Arslan Butt, Eithne Nic an Riogh, Marek Kollar, Jennifer S. Lees, Abdülmecit Yildiz, Andrea Hinojosa-Azaola, Ajay DhaygudeStephen A. Roberts, Avi Rosenberg, Thorsten Wiech, Charles D. Pusey, Rachel B. Jones, David R.W. Jayne, Ingeborg Bajema, J. Charles Jennette, Kate I. Stevens, Jean Francois Augusto, Juan Manuel Mejía-Vilet, Neeraj Dhaun, Stephen P. McAdoo, Vladimir Tesar, Mark A. Little, Duruvu Geetha, Silke R. Brix

Research output: Contribution to journalArticlepeer-review


Background Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. A retrospective international longitudinal cohort was collated to revise the ANCA renal risk score. Methods The primary end point was ESKD with patients censored at last follow-up. Cox proportional hazards were used to reweight risk factors. Kaplan–Meier curves, Harrell’s C statistic, receiver operating characteristics, and calibration plots were used to assess model performance. Results Of 1591 patients, 1439 were included in the final analyses, 2:1 randomly allocated per center to development and validation cohorts (52% male, median age 64 years). In the development cohort (n5959), the ANCA renal risk score was validated and calibrated, and parameters were reinvestigated modifying interstitial fibrosis and tubular atrophy allowing semiquantitative reporting. An additional cutoff for kidney function (K) was identified, and serum creatinine replaced GFR (K0:,250 mmol/L50, K1: 250–450 mmol/L54, K2: .450 mmol/L511 points). The risk points for the percentage of normal glomeruli (N) and interstitial fibrosis and tubular atrophy (T) were reweighted (N0: .25%50, N1: 10%–25%54, N2:,10%57, T0: none/mild or,25%50, T1: $ mild-moderate or $25%53 points), and four risk groups created: low (0–4 points), moderate (5–11), high (12–18), and very high (21). Discrimination was C50.831, and the 3-year kidney survival was 96%, 79%, 54%, and 19%, respectively. The revised score performed similarly well in the validation cohort with excellent calibration and discrimination (n5480, C50.821). Conclusions The updated score optimizes clinicopathologic prognostication for clinical practice and trials.

Original languageEnglish (US)
Pages (from-to)335-346
Number of pages12
JournalJournal of the American Society of Nephrology
Issue number3
StatePublished - Mar 1 2024

ASJC Scopus subject areas

  • General Medicine


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