Hereditary angioedema – 1/10/18

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

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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

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