Version May 2024
| Medication | Availability | Dosing | Precautions |
| C1 inhibitor concentrate (plasma derived) (Berinert, Cinryze) | Widely available. In the United States, Cinryze is not approved for acute treatment but is effective. In Europe, Cinryze is approved for children. Despite the differences in product labeling, both products are effective for acute treatment of attacks. | Berinert (all ages) – 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. Cinryze (children) – 500 units for body weight <25 kg and 1000 units ≥25 kg; a second dose should be given if no response. Relapse of symptoms is reported in <1% of episodes. | Do not shake solution, because protein will denature. |
| 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 (2 vials) for those ≥84 kg. Relapse of symptoms is reported in <3% of episodes. | Do not shake solution. |
| Bradykinin B2-receptor antagonist Icatibant (Firazyr) | United States and many other countries. Approved in the European Union for children as young as 2 years of age. | Adults – 30 mg slow subcutaneous infusion (because of volume) in abdominal area. Children – Dosing is weight-based:
Second dose can be given 6 hours after first if symptoms continue to worsen. Relapse of symptoms is reported in up to 10% of episodes. 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 12 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. Relapse is reported in <3% of episodes. May be repeated in 1 hour. | Rare allergy reaction reported usually in <1 hour. Should be administered by a clinician or nurse in a medical setting equipped to treat anaphylaxis. |
| Plasma | Widely available as solvent detergent-treated plasma (preferred) or fresh frozen 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 blood-borne pathogens. Rare reports describe worsening of angioedema after administration of plasma. |
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