Icatibant subcutaneous injection (Firazyr) can be self-administered by patients upon recognition of HAE attack

Approximately 30,000 individuals in the U.S. have Hereditary Angioedema (HAE).

Icatibant, a bradykinin B2 receptor antagonist, is supplied in a prefilled syringe. Itc can be stored at room temperature (up to 77°F) for immediate use.


New therapies for hereditary angioedema (HAE) (click to enlarge the image).

Two other agents were approved by the FDA in 2009 for treatment of HAE attacks:

- plasma-derived C1-esterase inhibitor concentrate for injection (Berinert) for HAE-related acute abdominal attacks and facial swelling. HAE patients can self-administer Berinert C1-INH by IV infusion - FDA, 2012.

- ecallantide subcutaneous injection (Kalbitor) for treatment of HAE attacks

Cinryze (C1-esterase inhibitor concentrate) is approved for prophylaxis of HAE, not for treatment of acute attacks.

This is a good summary from Medscape's Atopic Topics blog:

Unlike Ecallantide which is only approved for administration in a supervised setting (due to risk of hypersensitivity reaction), Firazyr is approved for use by the patient.

Screening for HAE is a simple process - C4 is always low in HAE (unless the patient is already treated for the disease). A normal C4 therefore rules it out. If C4 is low, a quantitative AND qualitative C1 inhibitor levels should be checked

References:

FDA Approves New Drug for Hereditary Angioedema. Medscape, 2011.

HAE patients can self-administer Berinert C1-INH by IV infusion - FDA, 2012.