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Hereditary Angioedema Update

Patients with hereditary angioedema (HAE) experience acute, intermittent, and potentially life-threatening attacks of edema involving the skin and mucosa. Only a few percent of the angioedema cases seen by physicians are HAE. The best screening test for HAE is testing for C4 and “functional” C1 esterase inhibitor levels. HAE is not responsive to epinephrine or anti-histamines. In recent years, four competing treatments have been developed. In August 2010, the New England Journal of medicine published one study of each of these agents. As yet, there has been no published head-to-head comparative trial. There are distinct differences in mechanism and administration. Each agent was effective against acute attacks to some degree. Used Prophylactically, the C1 inhibitor concentrates reduced the frequency of acute attacks.

Agents for HAE:

Plasma derived C1 esterase inhibitor: “Cinryze/Berinert”
Recombinant derived C1 esterase inhibitor: “Rhucin”
Bradykinin B2 receptor antagonist: Icantibant brand name “Firazyr”
Inhibitor of Kallikrein activation: Ecallantide brand name “Kalbitor”

Best to know the spelling before the swelling gets out of control!