Hereditary angioedema due to a deficiency of C1 inhibitor (HAE-C1INH) is clinically characterised by recurrent, generally unpredictable attacks of angioedema.1 Untreated episodes are often severe and prolonged, lasting up to 3–5 days and engendering considerable morbidity and even mortality.2 Unlike angioedema associated with mast cell mediators, the swelling in HAE-C1INH does not respond to antihistamines, corticosteroids, or epinephrine.
Kaynak: The Lancet
