[Hereditary angioedema]

Abstract

Hereditary angioedema (HAE) encompasses a heterogeneous group of diseases with similar phenotypes but different underlying genotypes. Specific clinical signs may point to HAE as opposed to histaminergic angioedema: the typical prolonged development of angioedema over time, positive family history, a lack of response to antihistamines and steroids and response to bradykinin antagonists are typical signs of HAE. The different types of HAE may be associated with a severe clinical course. They are life-long conditions and are still potentially life-threatening. The quality of life of patients with HAE may be considerably impaired. Management plans should be individualized, which is facilitated by the variety of specific medicastions available.

Keywords: Bradykinin; Complement C1 inhibitor protein; Factor XII; Hereditary angioedema; Prophylaxis, angioedema.