UMEM Educational Pearls


31 year-old male with recently diagnosed hypertension presents with rapid lip swelling. He started taking an unknown medication for his hypertension last week. Further history reveals that he has had prior, although milder, episodes previously. Name two medications that may help treat him.


Answer: The patient has hereditary angioedema with an episode triggered by an ACE inhibitor. Treatment includes the usual cocktail of histamine blockers and steroids plus:

  • Fresh frozen plasma
  • C1 inhibitor concentrate
  • Danazol (modified testosterone)
  • Ecallantide (kallikrien inhibitor)

The precipitant cause is unknown in most cases, but common etiologies include drugs (e.g. ACE inhibitors), infections, dental work, or stress.

Serum C4 level may assist in the diagnosis, but are rarely helpful acutely.

Fifty percent of patients will have laryngeal swelling at one point in their lives and asphyxiation is the leading cause of death; mortality is ~14-33%

The airway must be emergently evaluated to determine the need for intubation. If required, the most skilled person should take the first look.

If access to the oropharynx is limited (secondary to lip and tongue swelling) fiberoptic nasolaryngoscopy may be considered however, always be prepared to perform an emergent surgical airway.

Bonus Pearl: Check out this months V-Cast hosted by Dr. Amal Mattu. Dr. Jim Roberts (of Roberts and Hedges fame) reviews angioedema. Check out this great review here:



Joseph J. Moellman, and Jonathan A. Bernstein. Diagnosis and Management of Hereditary Angioedema: An Emergency Medicine Perspective. Journal of Emergency Medicine 2012

Follow me on Twitter (@criticalcarenow) or on Google+ (+criticalcarenow)