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Plasma-derived complement inhibitor

C1-esterase inhibitor

Brand names: Berinert, Cinryze

Adult dose

Dose: HAE acute: 20 units/kg IV; Prophylaxis: 1000 units IV q3–4 days
Route: IV
Frequency: PRN attack / scheduled prophylaxis

Clinical pearls

  • Hereditary angioedema (HAE) — acute attacks (laryngeal/abdominal) and short-term prophylaxis
  • UK PIN / WAOI / HAEi: specialist immunology centres
  • Patient self-administration training; alternative: lanadelumab, icatibant

Contraindications

  • Hypersensitivity

Side effects

  • Hypersensitivity / anaphylaxis
  • Thrombosis (high-dose, prolonged)
  • Headache
  • Nausea
  • Injection-site reactions
  • Theoretical viral transmission (plasma-derived)

Interactions

  • No clinically significant interactions

Monitoring

  • Attack frequency / severity
  • Hypersensitivity
  • Thrombotic complications

Reference: BNF; UK PIN HAE guidance; WAO HAE guideline; SmPC; https://bnf.nice.org.uk/drugs/c1-esterase-inhibitor/. Verify against your local formulary and the latest BNF before prescribing.

Related

Curated clinical cross-links plus same-class fallbacks.