Contributor: Katie Sprinkel, MD
Educational Pearls:
1. 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
2. HA episodes last 3-5 days, typically occur first in childhood or adolescence, and decrease in frequency with aging
3. Airway angioedema is the most dangerous but least common
4. Skin angioedema is usually uncomfortable and should be monitored if involving the face
5. GI angioedema involves nausea, vomiting, and severe abdominal pain
6. No definitive triggers, but episodes may be preceded by a variable prodrome but can include headache, GI symptoms, and even a rash
7. Treatment can include infusion of a C-1 inhibitor, such as Berinert but it is very expensive
References:
Busse PJ, Christiansen SC. Hereditary Angioedema. N Engl J Med. 2020 Mar 19;382(12):1136-1148. doi: 10.1056/NEJMra1808012. PMID: 32187470.
Longhurst HJ, Bork K. Hereditary angioedema: an update on causes, manifestations and treatment. Br J Hosp Med (Lond). 2019 Jul 2;80(7):391-398. doi: 10.12968/hmed.2019.80.7.391. PMID: 31283393.
Summarized by John Spartz, MS3 | Edited by Erik Verzemnieks, MD
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