Podcast 651: Hereditary Angioedema

Hereditary angioedema (HA) is an autosomal dominant bradykinin-mediated disease affecting the airway, skin or gastrointestinal tract and typically presents with swelling in the absence of pruritus

HA episodes last 3-5 days, typically occur first in childhood or adolescence, and decrease in frequency with aging

Airway angioedema is the most dangerous but least common

Skin angioedema is usually uncomfortable and should be monitored if involving the face

GI angioedema involves nausea, vomiting, and severe abdominal pain

No definitive triggers, but episodes may be preceded by a variable prodrome but can include headache, GI symptoms, and even a rash

Treatment can include infusion of a C-1 inhibitor, such as Berinert but it is very expensive