Table 1.
Acute management of HAE attacks | |
Preferred options | Intravenous C1-esterase inhibitor (C1-INH) concentrate (Berinert, Ruconest) Ecallantide (kallikrein inhibitor) Icatibant (bradykinin-receptor antagonist) |
Alternatives | Fresh frozen plasma |
P re procedural prophylaxis | |
No specific C1-INH concentrate is currently indicated in the USA (options are dependent on insurance formulary and coverage). | |
L ong-term prophylaxis | |
First-Iine options | Intravenous C1-INH concentrate (Cinryze) Subcutaneous C1-INH concentrate (Haegarda) Lanadelumab (antikallkrien monoclonal antibody) |
Alternatives | Androgens Tranexamic acid* |
*Tranexamic acid should be used when no other alternatives are available.