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, North-western 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
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