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Hereditary angioedema: Epidemiology, clinical manifestations, exacerbating factors, and prognosis

Marco Cicardi, MD
Bruce Zuraw, MD
Section Editor
Sarbjit Saini, MD
Deputy Editor
Anna M Feldweg, MD


Hereditary angioedema (HAE) is a disease characterized by recurrent episodes of angioedema, without urticaria or pruritus, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. Although the swelling is self-limited, laryngeal involvement may cause fatal asphyxiation. Prior to the availability of effective therapy, this disorder was associated with a mortality rate of approximately 30 percent due to asphyxiation from laryngeal swelling.

The epidemiology, clinical manifestations, triggering and exacerbating factors, and prognosis of HAE are discussed in this topic review. The laboratory evaluation, establishing definitive diagnosis, and acute and prophylactic therapies of this disorder are discussed separately:

(See "Hereditary angioedema: Pathogenesis and diagnosis".)

(See "Hereditary angioedema: Treatment of acute attacks".)

(See "Hereditary angioedema: General care and long-term prophylaxis".)

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Literature review current through: Nov 2017. | This topic last updated: Nov 20, 2017.
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