Current options for the acute treatment and prophylaxis of hereditary angioedema

Over the past 3 years, the availability of targeted therapies, including C1-esterase inhibitors, bradykinin receptor antagonists and kallikrein inhibitors, have dramatically altered the treatment paradigm of hereditary angioedema. For acute attacks of hereditary angioedema, fresh frozen plasma have been replaced by first-line agents such as C1-esterase inhibitors (Berinert®, Cetor®, Cinryze® and Rhucin®), ecallantide (Kalbitor®) and icatibant (Firazyr®). Short-term prophylaxis is now generally limited to having C1-esterase inhibitors available for minor manipulations and administering a dose of either 20 U/kg or 1000 U prior to the procedures for longer and more traumatic procedures or androgens such as danazol 200 mg three-times daily 5-7 days before and 2 days post procedure or if nothing else is available FFP. Which ever drug is used on demand therapy should be available since no prophylaxis is 100% protective. For patients with recurrent attacks, those poorly controlled with androgens, or those who fail on-demand therapy for acute attacks, long-term prophylaxis should be considered with C1-esterase inhibitors while giving consideration to the high associated cost.