Kalbitor (ecallantide) Indication: Treatment of acute attacks of hereditary agioedema Dosing: 30mg (3, 1mL injections) SQ; may

repeat 30mg within 24hrs Administration: SQ in abdomen, upper arm, or thigh. Do not admin @ site of attack. Separate injections by 2 inches or can inject at different sites. No need to rotate sites. AEs: Headache (8-16%), fatigue (12%), N(513%)V(6%)D(4-11%), URTI (8%), Many others <10% Warnings: BBW Hypersensitivity rxns (may resemble symptoms of HAE) (up to 4%) (usually occur w/in 1hr of injection) MOA: Recombinant protein prevents formation of bradykinin from kininogen by reversibly inhibiting plasma kallkrein.

Cinryze (C1 Inhibitor [Human]) Indication: Routine prophylaxis against HAE attacks Dosing: 1000U (2, 5mL vials) q3-4d Administration: IV @ 1mL/min (over 10mins). Allow to warm to room temp.

AEs: Headache ( 12%), 1-10%: Pruritis, back/extremity pain, URTI, Abdominal pain.

Warnings: (1) Thrombotic events at higher than recc. doses (2) Hypersensitivity rxns (may resemble symptoms of HAE) MOA: C1 inhibitor is a serine protease & when administered to deficient patients, it is thought to prevent bradykinin production by inactivating plasma kallikrein and factor XIIa. (Unregulated bradykinin production is thought to vascular permeability and angioedema in HAE) y Berinert is another C1 inhibitor used for the treatment of HAE attacks y Firazyr (icatibant), a bradykinin antagonist, was approved 8/11 for the treatment of HAE attacks y Androgens (danazol or stanozolol) OR antifibrinolytic agents (tranexamic acid) are also used for prophylaxis of HAE attacks. [Androgens have been shown to be more effective than antifibrinolytics] y There are guidelines for HAE. [2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema]. The guidelines state that C1 inhibitors (Berinert) have typically been 1st line therapy for HAE attacks, however they do not specify in their report if Firazyrr (icatibant) or Kalbitor (ecallantide) are more or less effective than C1 inhibitors and all are given the same level of recommendation in the treatment algorithm. For long-term prophylaxis, C1 inhibitors OR androgens are 1st line therapy.