Outcomes in ANCA-associated vasculitis in Scotland: validation of the renal risk score in a complete national cohort

Dominic P. McGovern (Lead / Corresponding author), Jennifer S. Lees, Jamie P. Traynor, Bruce Mackinnon, Samira Bell, Robert W. Hunter, Neeraj Dhaun, Wendy Metcalfe, Dana Kidder, Michelle Lim, Nicola Joss, Michael Kelly, Alison Taylor, Zoe Causland, Vishal Dey, Kate Buck, Silke Brix, Colin C. Geddes, Emily P. McQuarrie, Kathryn I. Stevens

Research output: Contribution to journalArticlepeer-review

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Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) causes autoimmune-mediated inflammation of small blood vessels in multiple organs, including the kidneys. The ability to accurately predict kidney outcomes would enable a more personalized therapeutic approach.

Methods: We used our national renal biopsy registry to validate the ability of ANCA Renal Risk Score (ARRS) to predict end-stage kidney disease (ESKD) for individual patients. This score uses histopathological and biochemical data to stratify patients as high, medium, or low risk for developing ESKD.

Results: A total of 288 patients were eligible for inclusion in the study (low risk n = 144, medium risk n = 122, high risk n = 12). Using adjusted Cox proportional hazard models with the low-risk group as reference, we show that outcome differs between the categories: high-risk hazard ratio (HR) 16.69 (2.91–95.81, P = 0.002); medium risk HR 4.14 (1.07–16.01, P = 0.039). Incremental multivariable-adjusted Cox proportional hazards models demonstrated that adding ARRS to a model adjusted for multiple clinical parameters enhanced predictive discrimination (basic model C-statistic 0.864 [95% CI 0.813–0.914], basic model plus ARRS C-statistic 0.877 [95% CI 0.823–0.931]; P <0.01).

Conclusion: The ARRS better discriminates risk of ESKD in AAV and offers clinicians more prognostic information than the use of standard biochemical and clinical measures alone. This is the first time the ARRS has been validated in a national cohort. The proportion of patients with high-risk scores is lower in our cohort compared to others and should be noted as a limitation of this study.

Original languageEnglish
Pages (from-to)1648-1656
Number of pages9
JournalKidney International Reports
Issue number8
Early online date1 Jun 2023
Publication statusPublished - Aug 2023


  • ANCA Renal Risk Score (ARRS)
  • ANCA-associated vasculitis (AAV)
  • biopsy registry
  • end-stage kidney disease (ESKD)

ASJC Scopus subject areas

  • Nephrology


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