Hereditary angioedema (HAE) is a genetic disease caused by a deficiency (type I), or dysfunction (type II), of C1-esterase inhibitor (C1-INH). It is characterised by unpredictable, recurring attacks of oedema at various sites of the body, including the hands, feet, face and abdomen; if oedema affects the upper airway it can be life-threatening. The frequency of attacks varies, but many of those affected will experience at least one attack a month. Each attack typically lasts two to five days before spontaneously subsiding. Attacks are also associated with raised levels of bradykinin, which is thought to mediate the formation of oedema by increasing vascular permeability1. Episodes can be unpredictable, or triggered by factors such as trauma, drugs or dental treatment.
This review describes the available evidence for various drug treatment options for the management of HAE. A separate document summarises the primary outcomes of phase III studies together with the licensing status and availability of these drugs.