Treatment
Î All patients with HAE should have access to an effective, on-demand
HAE-specific agent. Evidence from double-blind, placebo-controlled
randomized clinical trials demonstrates the efficacy and safety of
treatment of HAE attacks with C1INH concentrates, a plasma kallikrein
inhibitor, or a bradykinin B2 receptor antagonist (A)
Î Management of HAE attacks may involve symptomatic treatment based on
the region of body swelling. (C)
Î Fresh frozen plasma is often effective in abrogating HAE attacks, but
fresh frozen plasma may acutely exacerbate some attacks. For this reason
caution is required. (D)
Î Neither anabolic androgens nor antifibrinolytic drugs provide reliably
effective treatment for acute attacks of angioedema. (D)
Î Epinephrine, corticosteroids and antihistamines are NOT efficacious and
are NOT recommended for treatment of HAE. (C)
Figure 2. HAE Treatment Algorithm
Acute attack?
On-demand
treatment
Predictable
upcoming
stressor?
YES NO
Short-term
prophylaxis
Known HAE
YES
Is AE well
controlled?
Minimize
exacerbating
factors
NO
Start long-term
prophylactic
treatment
NO
YES
Continue on-demand
treatment
Still not well
controlled
Abbreviations: AE, angioedema; HAE, hereditary angioedema
