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Table 3 Therapies for HAE supported by high level evidence

From: The International/Canadian Hereditary Angioedema Guideline

HAE-specific treatmentProduct name and companyMechanism of actionApproved indicationsDose and route of administrationCounty licensed and age indications
pdC1-INHBerinert®a (CSL)Replaces C1-INHAcute treatment20 U/kg intravenousAustralia, Canada, EU, USA (adult and pediatric)
Pre-proceduralAdults: 1000 U
Pediatrics: 15 to 30 U/kg body weight
EU (adult and pediatric)
Cinryze® (Shire—now part of Takeda)Replaces C1-INHAcute treatment≥ 12 years: 1000 U intravenous
2–11 years: 1000 U (> 25 kg body weight)
500 U (< 25 kg body weight)
Australia (≥ 12 years)
EU (≥ 2 years)
Pre-procedural≥ 12 years: 1000 U intravenous
2–11 years: 1000 U (> 25 kg body weight)
500 U (< 25 kg body weight)
Australia (≥ 12 years)
EU (≥ 2 years)
Long-term prophylaxis1000 U intravenous
q 3–4 days (6–11 years 500 U q 3–4 days)b
Australia, Canada (≥ 12 years)
EU, USA (≥ 6 years)
Haegarda® (CSL)Replaces C1-INHLong-term prophylaxis60 U/kg body weight twice weekly (every 3–4 days)Australiac, Canada, EUd, USA (≥ 12 years)
rhC1-INHRuconest® (Ruconest)Replaces C1-INHAcute treatment50 U/kg intravenous (< 84 kg);
4200 U intravenous (≥ 84 kg)
EU (adults), USA (adults and adolescents)
EcallantideKalbitor® (Shire—now part of Takeda)Selective, reversible inhibitor of plasma kallikreinAcute treatment30 mg (3 × 10 mg/1 ml) subcutaneous injectionsUSA (≥ 12 years)
IcatibantFirazyr® (Shire—now part of Takeda)Synthetic selective and specific antagonist of bradykinin 2 receptorAcute treatment30 mg subcutaneous injection; dose-adjusted for adolescents < 65 kg and children ≥ 2 yearseUSA (≥ 18 years) Australia, Canada, EU (≥ 2 years)
LanadelumabTakhzyro® (Shire—now part of Takeda)Fully human monoclonal antibody that binds plasma kallikrein and inhibits its proteolytic activityLong-term prophylaxis300 mg subcutaneous injection every 2 weeks
a dosing interval of 300 mg every 4 weeks may be considered if the patient is well-controlled (e.g., attack free) for more than 6 months
Australia, Canada, EU, USA (≥ 12 years)
  1. Please refer to current country-specific monographs for further details regarding specific indications and listings of adverse events
  2. aBerinert 1500 in EU
  3. bDose-adjustment up to 2500 U q3–4 days for ages 12 and above, and up to 1000 U q3–4 days for ages 6-11, based on patient response
  4. cBerinert SC in Australia
  5. dBerinert 2000/3000 in EU
  6. e12 kg to 25 kg: 10 mg (1.0 ml); 26 kg to 40 kg: 15 mg (1.5 ml); 41 kg to 50 kg: 20 mg (2.0 ml); 51 kg to 65 kg: 25 mg (2.5 ml); > 65 kg: 30 mg (3.0 ml)