Epidemiology

The incidence and prevalence of granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis) varies considerably between countries. In the US, one study reported an incidence rate of 1.8 cases per million person-years among children and 12.8 cases per million person-years among adults.[6] The reported incidence and prevalence of GPA in the UK is 11.8 per million person-years and 134.9 per million person-years, respectively.[7] Higher rates have been reported in Scandinavia.[8]

GPA can occur at any age, but the incidence of pediatric-onset GPA was sevenfold lower than the incidence in working-age adults in one large cohort study.[6] The mean age of onset in most series is between 40 and 60 years of age, with males and females equally affected.[9] GPA is most commonly seen in white people, but may occur in any racial and ethnic group.[7][9][10] Incidence appears to increase at northerly and southerly latitudes.[11][12]

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