When is prophylaxis for hereditary angioedema necessary?

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.