Antihistamines and other treatments used for angioedema are of limited benefit in hereditary angioedema. Epinephrine should be used in life-threatening reactions.
The most effective treatment is a C1 inhibitor concentrate, but this may not be available. Fresh frozen plasma contains C1 inhibitor and will help an episode, but rarely, the swelling will become worse.
Attenuated androgens, such as danazol, can reduce the frequency and severity of attacks by increasing the production of C1 inhibitor.
Once an attack occurs, treatment includes pain relief and fluids given through a vein by an intravenous (IV) line.
Helicobacter pylori, a type of bacteria found in the stomach, can trigger abdominal attacks. Getting rid of the bacteria with antibiotic therapy decreases these abdominal attacks.
Hereditary angioedema can be life threatening and treatment options are limited. How well a person does depends on the specific symptoms.
Swelling of the airways may be deadly.
Call or visit your health care provider if you are considering having children and have a family history of this condition, or if you have symptoms of hereditary angioedema.
Swelling of the airway is a life-threatening emergency. If you are having difficulty breathing due to swelling, seek immediate medical attention.
Dreskin Sc. Urticaria and angioedema. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 273.
Levi M, Choi G, Picavet C, Hack CE. Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. J Allergy Clin Immunol. 2006;117(4):904-908.
Visy B, Fust G, Bygum A, et al. Helicobacter pylori infection as a triggering factor of attacks in patients with hereditary angioedema. Helicobacter. 2007;12(3):251-257.
© 2011 University of Maryland Medical Center (UMMC). All rights reserved.
UMMC is a member of the University of Maryland Medical System,
22 S. Greene Street, Baltimore, MD 21201. TDD: 1-800-735-2258 or 1.866.408.6885