Table 3 Therapies for HAE supported by high level evidence

pdC1-INH

Berinert^®a (CSL)

Replaces C1-INH

Acute treatment

20 U/kg intravenous

Australia, Canada, EU, USA (adult and pediatric)

Pre-procedural

Adults: 1000 U

Pediatrics: 15 to 30 U/kg body weight

EU (adult and pediatric)

Cinryze^® (Shire—now part of Takeda)

Replaces C1-INH

Acute 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 prophylaxis

1000 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-INH

Long-term prophylaxis

60 U/kg body weight twice weekly (every 3–4 days)

Australia^c, Canada, EU^d, USA (≥ 12 years)

rhC1-INH

Ruconest^® (Ruconest)

Replaces C1-INH

Acute treatment

50 U/kg intravenous (< 84 kg);

4200 U intravenous (≥ 84 kg)

EU (adults), USA (adults and adolescents)

Ecallantide

Kalbitor^® (Shire—now part of Takeda)

Selective, reversible inhibitor of plasma kallikrein

Acute treatment

30 mg (3 × 10 mg/1 ml) subcutaneous injections

USA (≥ 12 years)

Icatibant

Firazyr^® (Shire—now part of Takeda)

Synthetic selective and specific antagonist of bradykinin 2 receptor

Acute treatment

30 mg subcutaneous injection; dose-adjusted for adolescents < 65 kg and children ≥ 2 years^e

USA (≥ 18 years) Australia, Canada, EU (≥ 2 years)

Lanadelumab

Takhzyro^® (Shire—now part of Takeda)

Fully human monoclonal antibody that binds plasma kallikrein and inhibits its proteolytic activity

Long-term prophylaxis

300 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)