• Home

Home > Medical Reference > Encyclopedia (English)

Toggle: English / Spanish

Hereditary angioedema

• Overview
• Symptoms
• Treatment
• Prevention

Definition:

Hereditary angioedema is a rare but serious problem with the immune system that is passed down through families. It causes swelling, particularly of the face and airways, and abdominal cramping.

See also: Angioedema

Alternative Names:

Quincke's disease

Causes, incidence, and risk factors:

Hereditary angioedema (HAE) is caused by low levels or improper function of a protein called C1 inhibitor . This problem affects the blood vessels. People with hereditary angioedema can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, or airway (larynx or trachea).

Unlike an allergic reaction , there is usually no itching or hives . Swelling of the gut can cause intestinal cramping. If swelling closes the airway, it can be deadly. Attacks of swelling can become more severe in late childhood and adolescence.

There is usually a family history of the condition. However, relatives may be unaware of previous cases, which may have just been reported as an unexpected, sudden, and premature death of a parent, aunt, uncle, or grandparent.

Many attacks occur without reason. However, anxiety, stress, sickness (including colds and the flu), and surgery have been shown to trigger certain attacks of hereditary angioedema. Dental procedures increase the risk of attacks in patients with HAE.

References:

Weiler CR, van Dellen RG. Genetic test indications and interpretations in patients with hereditary angioedema. Mayo Clin Proc . 2006 Jul;81(7):958-72. Review.

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 Apr;117(4):904-8. Epub 2006 Feb 14.

Bork K, Barnstedt SE. Treatment of 193 episodes of laryngeal edema with C1 inhibitor concentrate in patients with hereditary angioedema. Arch Intern Med . 2001; 161(5):714-8.

• Review Date: 10/30/2006
• Reviewed By: Donald Accetta, MD, MPH, President, Allergy & Asthma Care, PC, Taunton, MA. Review provided by VeriMed Healthcare Network.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process . A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 1997-2007 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.