EDUCATIONAL OBJECTIVE: Readers will consider the diagnosis of hereditary angioedema if a patient has recurrent attacks of angioedema that do not respond to usual therapy

Recognizing and managing hereditary angioedema

1. KEVIN TSE, MD

1. Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, La Jolla

1. BRUCE L. ZURAW, MD⇑

+Author Affiliations

1. Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, La Jolla; San Diego Veterans Administration Healthcare, San Diego, CA

1. ADDRESS: Bruce Zuraw, MD, Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, 9500 Gilman Drive, Mailcode 0732, La Jolla, CA 92093; e-mail: bzuraw@ucsd.edu

 

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Abstract

Hereditary angioedema is a rare but life-threatening disease characterized by recurring attacks of swelling of any part of the body, without hives. Prompt recognition is critical so that treatment can be started to minimize morbidity and the risk of death. Drugs have recently become available to prevent and treat acute attacks.

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Key points

Swelling in the airways is life-threatening and requires rapid treatment.

Almost half of attacks involve the abdomen, and abdominal attacks account for many emergency department visits, hospitalizations, and unnecessary surgical procedures for acute abdomen.

Acute attacks can be managed with plasma-derived or recombinant human preparations of C1 inhibitor (which is the deficient factor in this condition), ecallantide (a specific plasma kallikrein inhibitor), or icatibant (a B2 bradykinin receptor antagonist).

Short-term prophylaxis may be used before events that could provoke attacks (eg, dental work or surgery). Long-term prophylaxis may be used in patients who have frequent or severe attacks or require more stringent control of their disease. Plasma-derived C1 inhibitor is both safe and effective when used as prophylaxis. Attenuated androgens are effective but associated with many adverse effects.

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