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Hereditary angioedema: General care and long-term prophylaxis

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

INTRODUCTION

Hereditary angioedema (HAE or inherited C1 inhibitor deficiency) is a rare genetic disorder resulting from deficiency (type I) or dysfunction (type II) of C1 inhibitor (C1INH). It is characterized by recurrent episodes of angioedema without pruritus or urticaria, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. Although swelling resolves spontaneously in two to five days in the absence of treatment, angioedema is often temporarily debilitating and laryngeal angioedema may cause fatal asphyxiation.

In patients with sufficiently frequent or severe episodes of angioedema, regularly-administered prophylaxis is required to maintain an acceptable quality of life. This is referred to as long-term prophylaxis, in contrast to short-term prophylaxis, which is premedication given briefly before a specific medical or dental procedure. Short-term prophylaxis is discussed separately. (See "Hereditary angioedema: Prophylaxis prior to procedures (short-term prophylaxis)".)

The general management of adults and children with HAE and the various long-term prophylactic therapies that are used to prevent attacks will be reviewed here. The management of acute attacks as well as the clinical manifestations, pathogenesis, and diagnosis of this condition are discussed elsewhere:

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

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

                                     

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Literature review current through: Nov 2016. | This topic last updated: Thu Oct 20 00:00:00 GMT 2016.
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