Medication | Availability | Dosing | Precautions |
C1 inhibitor concentrate (plasma derived) (Berinert, Berinert P, Cinryze) | In the United States:
Other countries:
| 20 units per kg body weight given intravenously over 10 minutes. Symptoms usually stabilize in 30 minutes. Second dose uncommonly needed but may be given 30 minutes to 2 hours after first dose. | Do not shake solution, because protein will denature. Cinryze is not US FDA approved for acute attacks but has efficacy. |
Recombinant C1 inhibitor Conestat alfa (Ruconest, Rhucin) | Europe, United States, some other countries. | 50 units per kg body weight for patients <84 kg. 4200 units (two vials) for those ≥84 kg. Second dose rarely needed. | Patients should be screened for rabbit allergy prior to receiving with rabbit-specific IgE immunoassay and should not receive drug if positive. |
Bradykinin B2-receptor antagonist Icatibant (Firazyr) | United States and many other countries. Approved in the United States for individuals over the age of 18 years. | 30 mg slow subcutaneous infusion (because of volume) in abdominal area. Second dose needed in approximately 10% of patients and can be given 6 hours after first dose. Maximum of 3 doses in 24 hours. | Caution in patients with unstable angina. Mild injection-site reactions are common. |
Kallikrein inhibitor Ecallantide (Kalbitor) | United States only. Approved in the United States for individuals over the age of 16 years. | 30 mg (3 doses of 10 mg each) given at 3 separate sites subcutaneously in abdomen, upper arm, or thigh and away from site of angioedema. | Rare allergy reaction reported, usually in <1 hour. Should be administered by a clinician or nurse in a medical facility equipped to treat anaphylaxis. |
Plasma |
| 2 units initially. Can be repeated every 2 to 4 hours, if needed. | Monitor for volume overload in patients with underlying conditions predisposing to volume overload. Theoretical risk of transmission of bloodborne pathogens. |
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