Epidemiology

EGPA is the most rare of the three anti-neutrophil cytoplasmic antibody-associated vasculitides, with an estimated annual incidence of between 1 and 3 cases per million people.[6][7][8][9][10] Among asthma patients, the incidence is as high as 67 per million asthma patients.[11][12] EGPA is typically diagnosed in middle age, and there is a slight male predominance.[4][5][13] The outcome in EGPA is dependent on the extent and severity of organ involvement. In most cases, with appropriate immunosuppressive therapy, survival seems comparable to age-matched controls, with published 5-year survival rates of 68% to 100%.[14][15][16][17] Myocardial and central nervous system involvement may confer greatest risk.

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