When is prophylaxis for hereditary angioedema necessary?

Timothy Craig, Marc Ried, Mark S. Dykewicz, Richard G. Gower, James Baker, Frank J. Edelman, David Hurewitz, Joshua Jacobs, Ira Kalfus

Research output: Contribution to journalReview articlepeer-review

75 Scopus citations


Objective: To determine when newer agents, such as C1 esterase inhibitor protein (C1-INH), should be considered as prophylaxis to decrease hereditary angioedema (HAE) attacks as an alternative to androgens, which have significant adverse events. Data Sources: A literature review (PubMed, Google, and Ovid), guideline review, expert panel meeting, and group discussion were performed to decide when prophylaxis is indicated. Study Selection: Articles addressing HAE therapy published in the peer-reviewed literature were selected. Results: The retrieved studies demonstrate that C1-INH is effective and that the half-life makes it attractive for prophylactic use. The short half-lives of ecallantide, icatibant, and recombinant human C1-INH limit their use as prophylactic agents. Patients with severe anxiety, more than 1 attack per month, rapid progression of attacks, limited access to health care, more than 10 days lost from work or school per year, previous laryngeal swelling, more than 3 emergency department visits per year, more than 1 hospitalization per year, previous intubation, previous intensive care unit care, significant compromise in quality of life, or narcotic dependency should be considered for androgen or C1-INH prophylaxis therapy. Conclusion: Patients with HAE with frequent attacks, severe attacks, past laryngeal attacks, excessive loss of work or school, significant anxiety, and poor quality of life should be considered for C1-INH prophylaxis, especially those who fail, are intolerant of, have adverse reactions to, or are not candidates for androgen therapy. http://lysander.annallergy.org/vl=11267769/cl= 17/nw=1/fm=docpdf/rpsv/cw/acaai/10811206/v102n5/s3/p366.

Original languageEnglish (US)
Pages (from-to)366-372
Number of pages7
JournalAnnals of Allergy, Asthma and Immunology
Issue number5
StatePublished - May 2009

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology
  • Pulmonary and Respiratory Medicine


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