Podcast 651: Hereditary Angioedema

<p><strong>Contributor:  Katie Sprinkel, MD</strong></p> <p><strong>Educational Pearls:</strong></p> <ul> <li style="font-weight: 400;" aria-level="1"><span style= "font-weight: 400;">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</span></li> <li style="font-weight: 400;" aria-level="1"><span style= "font-weight: 400;">HA episodes last 3-5 days, typically occur first in childhood or adolescence, and decrease in frequency with aging</span></li> <li style="font-weight: 400;" aria-level="1"><span style= "font-weight: 400;">Airway angioedema is the most dangerous but least common</span></li> <li style="font-weight: 400;" aria-level="1"><span style= "font-weight: 400;">Skin angioedema is usually uncomfortable and should be monitored if involving the face</span></li> <li style="font-weight: 400;" aria-level="1"><span style= "font-weight: 400;">GI angioedema involves nausea, vomiting, and severe abdominal pain</span></li> <li style="font-weight: 400;" aria-level="1"><span style= "font-weight: 400;">No definitive triggers, but episodes may be preceded by a variable prodrome but can include headache, GI symptoms, and even a rash</span></li> <li style="font-weight: 400;" aria-level="1"><span style= "font-weight: 400;">Treatment can include infusion of a C-1 inhibitor, such as Berinert but it is very expensive</span></li> </ul> <p> </p> <p><strong>References</strong></p> <p><span style="font-weight: 400;">Busse PJ, Christiansen SC. Hereditary Angioedema. N Engl J Med. 2020 Mar 19;382(12):1136-1148. doi: 10.1056/NEJMra1808012. PMID: 32187470.</span></p> <p><span style="font-weight: 400;">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.</span></p> <p> </p> <p><em><span style="font-weight: 400;">Summarized by John Spartz, MS3 | Edited by