Epidemiology

• Autosomal dominant – look for a positive family history
• Usually diagnosed early in life

Mediators

• Caused by elevations in bradykinin
• No effect by histamine or mast cells

Clinical Presentation

• Recurrent angioedema without hives or pruritis
• Skin and GI tract most commonly affected
• Colicky abdominal pain of unexplained etiology
• Hypokalemia due to bradykinin elevation leading to high levels of ACE which activate the RAA system

Treatment

• Usually self-limited within 2-5 days
• Can use FFP as FFP contained C1 inhibitor and ACE
• Can use C1 inhibitor analogues, kallikrein antagonists, or bradykinin receptor antagonists for treatment if concern for rapidly progressive angioedema