epidemiology of ANCA associated vasculitis

Last edited 10/2020

  • annual incidence of ANCA-associated vasculitis (AAV) in Norfolk during 1988-2010 was estimated to be 19.5/million (GPA (Wegener) 11.3/million, MPA (microscopic polyangiitis) 5.9/million) with a prevalence of 255/million (1)
    • AAV entities are rare diseases with combined prevalence estimated at between 90 and 144 per million, and with annual incidence of around 20 per million (2)
  • peak age at diagnosis was 65-74 years, with a male preponderance
    • high age at onset has been confirmed from other centres in Europe (Spain and Scandinavia)
    • a cyclical pattern of occurrence has been noted for GPA but not MPA
  • approximately 85% to 90% of patients with GPA, 70% of those with MPA and approximately 30% to 40% of patients with EGPA are ANCA-positive (2)
  • there are geographical differences in the incidence of AAV
    • for example a comparative study in Europe using the same classification criteria in three populations (Lugo, Northwestern Spain; Norwich, UK; and Tromsø, Northern Norway) reported that GPA was more common in Norway than in Spain, while MPA had the reverse distribution
    • in South-East Asian populations (Japan, China) myeloperoxidase (MPO)-AAV is relatively more common than vasculitis associated with antibodies to PR3 (PR3-AAV), although the overall incidence of AAV is similar


  • 1) ARC Autumn 2012. Topical Reviews - ANCA-associated vasculitis; 1:1-12.
  • 2) Bala MM et al. Anti-cytokine targeted therapies for ANCA-associated vasculitis. Cochrane Database of Systematic Reviews 2020, Issue 9. Art. No.: CD008333.
    DOI: 10.1002/14651858.CD008333.pub2.