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Many faces of angioedema: focus on the diagnosis and management of abdominal manifestations of hereditary angioedema

Nzeako, Ugochukwu C.a; Longhurst, Hilary J.b

European Journal of Gastroenterology & Hepatology: April 2012 - Volume 24 - Issue 4 - p 353–361
doi: 10.1097/MEG.0b013e3283517998
Review Article

Angioedema of the intestinal tract is an infrequent but well-described cause of abdominal pain that can occur because of inherited, acquired, allergic, or drug-induced causes. Hereditary angioedema (HAE) is a genetic disorder that causes recurrent attacks of severe edema of various body parts, including the intestinal tract. Moderate to severe abdominal pain occurs in 43–93% of such attacks due to intestinal edema. Laryngeal edema is a potentially life-threatening manifestation. Failure to recognize and diagnose HAE or other causes of intestinal angioedema can lead to years of delay in diagnosis, and in the case of HAE, often to unnecessary abdominal surgeries. Recognizing the typical history of recurrent attacks of abdominal pain, oropharyngeal/laryngeal angioedema or cutaneous angioedema, family history of similar symptoms, association of attacks with stress or menses, and exacerbation of attacks after administration of estrogens or angiotensin-converting enzyme inhibitors will increase diagnostic accuracy. Interdisciplinary treatment is often necessary after the diagnosis of HAE, first with acute management in the emergency room or the intensive care unit, followed by either drug prophylaxis against future attacks using a C1-esterase inhibitor concentrate or attenuated androgens and discontinuation of medications known to trigger attacks. Newer drugs approved for treatment of acute attacks may have future roles in the prevention of attacks if further studies support their efficacy. Gastroenterologists in particular should maintain a high index of suspicion for the possibility of HAE or other causes of intestinal angioedema in patients with a history of recurrent abdominal pain.

aDepartment of Gastroenterology, Hepatology, and Gastrointestinal Endoscopy, Watson Clinic LLP, Lakeland, Florida, USA

bDepartment of Immunology, Barts and the London NHS Trust, London, UK

Correspondence to Ugochukwu C. Nzeako, MD, MPH, Department of Gastroenterology, Hepatology, and Gastrointestinal Endoscopy, Watson Clinic LLP, 1600 Lakeland Hills Boulevard, Lakeland, Florida 33805, USA Tel: +1 863 680 7000; fax: +1 863 616 2457; e-mail:

Received December 22, 2011

Accepted January 16, 2012

© 2012 Lippincott Williams & Wilkins, Inc.