Epidemiology of ANCA-associated vasculitis

Rheum Dis Clin North Am. 2010 Aug;36(3):447-61. doi: 10.1016/j.rdc.2010.04.002. Epub 2010 Jun 15.


The epidemiology of the antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), comprising Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome, poses considerable challenges to epidemiologists. These challenges include the difficulty of defining a case with a lack of clear distinction between the different disorders, case capture, and case ascertainment. The AAV are rare and therefore a large population is required to determine the incidence and prevalence, and this poses questions of feasibility. Despite these difficulties a considerable body of data on the epidemiology of the AAV has been built in the past 20 years with an interesting age, geographic, and ethnic tropism gradually being revealed. Most of the data come from White populations of European descent, and the overall annual incidence is estimated at approximately 10-20/million with a peak age of onset in those aged 65 to 74 years.

MeSH terms

  • Age of Onset
  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / epidemiology*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / ethnology
  • Australia / epidemiology
  • Europe / epidemiology
  • European Continental Ancestry Group*
  • Female
  • Humans
  • Incidence
  • Japan / epidemiology
  • Male
  • Middle Aged
  • Prevalence
  • United States / epidemiology