ISSUE 1541
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The FDA has approved Haegarda (CSL Bering), a human plasma-derived C1 esterase inhibitor (C1INH), for prophylaxis against hereditary angioedema (HAE) attacks in adolescents and adults. Haegarda is the fourth C1INH to be approved by the FDA and the second to be approved for HAE prophylaxis. Unlike the other C1INHs, Haegarda is self-administered subcutaneously rather than intravenously.
THE DISEASE — HAE, an autosomal dominant disorder with an estimated prevalence of 1:10,000-50,000, is usually caused by a mutation of the C1INH gene (types 1 and 2). A deficiency in endogenous or functional C1INH leads to an overproduction of bradykinin, a vasoactive substance that increases vascular permeability and is believed to be the mediator of acute angioedema attacks. Patients experience recurrent, often unpredictable attacks of angioedema lasting 2-5 days, typically involving the extremities, GI
... more- A recombinant C1 esterase inhibitor (Ruconest) for hereditary angioedema. Med Lett Drugs Ther 2016; 58:e44.
- T Craig et al. WAO guideline for the management of hereditary angioedema. World Allergy Organ J 2012; 5:182.
- BL Zuraw et al. Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema. N Engl J Med 2010; 363:513.
- H Longhurst et al. Prevention of hereditary angioedema attacks with a subcutaneous C1 inhibitor. N Engl J Med 2017; 376:1131.
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