Clinicians generally recommend long term therapy for patients who experience more than one attack per month, or who believe that the disease significantly interferes with their life style. These agents produce an increase in C1-inhibitor levels, but the exact mechanism of how they do so has not been precisely defined. Some patients report success with a class of drugs called antifibrinolytics, but their use has largely been abandoned because androgens have proven more effective. However, a recent study noted success using inhaled epinephrine to prevent complete airway closure. Short-term therapy is necessary for patients who do not require ongoing treatment but are facing dental procedures or elective surgeries.
Current practice calls for daily androgen therapy, during the week prior to surgery.
There is no currently approved treatment for acute attacks available in the United States. Fresh frozen plasma is used effectively by some clinicians, but this therapy is considered controversial because of a theoretical chance for attack exacerbation. C1-inhibitor concentrate is the treatment of choice for acute attacks of HAE and has been available to patients in Europe for over a decade. C1 inhibitor concentrate treatment resolves the angioedema in thirty minutes to two hours with complete remission in twenty-four hours. A C1-inhibitor concentrate phase III clinical trial sponsored by Baxter Hyland Immuno is now underway in various centers throughout the United States.