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Review
. 2019 Sep;60(9):987-995.
doi: 10.1007/s00108-019-0644-1.

[Hereditary angioedema]

[Article in German]
Emel Aygören-Pürsün  1 , Konrad Bork  2
Affiliations
Review

[Hereditary angioedema]

[Article in German]
Emel Aygören-Pürsün et al. Internist (Berl). 2019 Sep.

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.

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