C1 Human Esterase Inhibitor

Aka: C1 Human Esterase Inhibitor, C1 Antagonist, C1 Inhibitor, Berinert, Plasma-Derived C1 Esterase Inhibitor

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II. Indications

  1. Severe, airway compromising Angioedema
    1. Hereditary Angioedema (prophylaxis and treatment of choice)
    2. Not found effective in ACE InhibitorAngioedema

III. Background

  1. Human plasma-derived C1-INH concentrate
  2. Pasteurized and nanofiltered plasma sample
  3. In Hereditary Angioedema, C1 esterase inhibitor is deficient or non-functional
    1. C1 Esterase Inhibitor suppresses the Classical Complement Pathway (C1 to C3 activation)
    2. C1 Esterase inhibitor also suppresses blood Coagulation Factors (Factor XII and plasma Kallikrein)
      1. Deficiency results in increased bradykinin (vasoactivity) and increased tissue edema

IV. Dose: Hereditary Angioedema Prophylaxis

  1. Adults and Teens: 60 IU/kg SQ twice weekly (every 3-4 days)
  2. Reduces episodes of Angioedema by 95%

V. Dose: Acute Management

  1. Berinert 20 IU/kg IV for 1 dose
  2. Cost: $3000 per dose

VI. Adverse Effects

  1. Headache (common)
  2. Nausea (common)
  3. Dizziness
  4. Dysgeusia
  5. Fever
  6. Local skin reaction and Rash
  7. Flu-like symptoms
  8. Hypersensitivity Reaction (uncommon)
  9. Thrombotic event, especially at doses higher than recommended (uncommon)

VII. References

  1. (2015) Lexicomp, accessed online 4/9/2015
  2. Lovecchio (2021) Crit Dec Emerg Med 31(10): 24
  3. Farkas (2007) J Allergy Clin Immunol 120(4):941-7 +PMID:17761272 [PubMed]

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