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Hereditary angioedema: Treatment of acute attacks

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


Hereditary angioedema (abbreviated HAE throughout this review) is a rare autosomal dominant disorder characterized by recurrent episodes of well-demarcated angioedema without urticaria, which most often affects the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. Although swelling resolves spontaneously in two to four days in the absence of treatment, laryngeal edema may cause fatal asphyxiation, and the pain of gastrointestinal attacks may be incapacitating or lead to unnecessary abdominal surgery. The most common forms of HAE (types I and II) are caused by deficiency or dysfunction in C1 inhibitor (C1INH-HAE) [1]. There are other forms of the disorder in which C1INH is normal called hereditary angioedema with normal C1 inhibitor (HAE with normal C1INH), which are discussed separately. (See "Hereditary angioedema with normal C1 inhibitor".)

The evaluation and treatment of acute attacks of HAE in adults and children will be reviewed here. Other aspects of these conditions are discussed elsewhere:

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

(See "Hereditary angioedema: Epidemiology, clinical manifestations, exacerbating factors, and prognosis".)

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

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Literature review current through: Nov 2017. | This topic last updated: Aug 29, 2016.
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