Because HAE results from bradykinin-mediated swelling, not histaminergic swelling, corticosteroids, H1 antihistamines, and epinephrine lack effectiveness (2).

Androgens are effective in some patients, but are prohibitive in others because of their high burden of adverse effects and should not be used in patients under the age of 16 and in patients who are pregnant, want to become pregnant, or breastfeeding (4-6).

Androgens and antifibrinolytic agents have no role in on-demand therapy (5).

Assess patients for long-term prophylaxis at every office visit and at least yearly (5).

Provide genetic counseling and offer screening for family members and considerations for reproduction (7,9). 

Develop individualized plans that consider long-term prophylaxis, when appropriate (9).

The management plan should address specific treatments, including on-demand therapy and prophylaxis, and provide guidance on travel, sports, school, and work (9).