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Hereditary Angioedema
A Broad Review for Clinicians
Ugochukwu C. Nzeako, MD, MPH;
Evangelo Frigas, MD;
William J. Tremaine, MD
Arch Intern Med. 2001;161:2417-2429.
Hereditary angioedema (HAE) is an autosomal dominant disease that afflicts
1 in 10 000 to 1 in 150 000 persons; HAE has been reported in all
races, and no sex predominance has been found. It manifests as recurrent attacks
of intense, massive, localized edema without concomitant pruritus, often resulting
from one of several known triggers. However, attacks can occur in the absence
of any identifiable initiating event. Historically, 2 types of HAE have been
described. However, a variant, possibly X-linked, inherited angioedema has
recently been described, and tentatively it has been named "type 3" HAE. Signs
and symptoms are identical in all types of HAE. Skin and visceral organs may
be involved by the typically massive local edema. The most commonly involved
viscera are the respiratory and gastrointestinal systems. Involvement of the
upper airways can result in severe life-threatening symptoms, including the
risk of asphyxiation, unless appropriate interventions are taken. Quantitative
and functional analyses of C1 esterase inhibitor and complement components
C4 and C1q should be performed when HAE is suspected. Acute exacerbations
of the disease should be treated with intravenous purified C1 esterase inhibitor
concentrate, where available. Intravenous administration of fresh frozen plasma
is also useful in acute HAE; however, it occasionally exacerbates symptoms.
Corticosteroids, antihistamines, and epinephrine can be useful adjuncts but
typically are not efficacious in aborting acute attacks. Prophylactic management
involves long-term use of attenuated androgens or antifibrinolytic agents.
Clinicians should keep this disorder in their differential diagnosis of unexplained,
episodic cutaneous angioedema or abdominal pain.
From the Divisions of Gastroenterology (Drs Nzeako and Tremaine), Hepatology
(Drs Nzeako and Tremaine), and Allergy (Dr Frigas), Mayo Clinic and Foundation,
Rochester, Minn.
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