Î Treatment with replacement plasma-derived C1INH provides effective and
safe long-term HAE prophylaxis. (A)
Î The novel agents for treatment of patients with C1INH deficiency
syndromes are more costly than alternative treatment with attenuated
androgens. Formal studies of cost utility and cost-effectiveness are
required to aid providers in the management of patients with C1INH
deficiency syndromes. (D)
Î Treatment with low to moderate doses of anabolic androgens provides
effective and relatively safe long-term HAE prophylaxis for many
patients. (B)
Î Treatment with antifibrinolytic agents provides somewhat effective and
relatively safe long-term HAE prophylaxis but is generally less effective
than androgens. (B)
Î Short-term prophylaxis can be achieved with fresh frozen plasma, C1INH
replacement or short-term high dose anabolic androgen therapy. (B)
Î The need for long-term HAE prophylaxis must be individualized based on
the patient's situation. (D)
Î The dose and effectiveness of long-term prophylaxis should be based on
clinical criteria, not laboratory parameters. (C)
Î Adjunctive strategies such as avoidance of ACE-Is, avoidance of estrogen
therapy as feasible, and stress reduction are important to decrease the
frequency and severity of HAE attacks. (D)
Î Mechanisms of action of 17α-alkylated androgen and antifibrinolytic drugs
for HAE have not been completely elucidated. (D)
Î Pregnancy might be associated with an increase in the frequency and
severity of HAE episodes. For long-term prophylaxis during pregnancy,
treatment with androgens is contraindicated, and plasma-derived C1INH is
preferred (D).
Prevention
