Human C1-esterase inhibitor

Serine Protease Inhibitor (serpin) / Plasma Derived Or Recombinant ProteinRx: PrescriptionCompound: Approved

Also known as: Berinert, C1-INH, Cinryze, Conestat alfa, Haegarda, Ruconest

Educational Only — Not medical advice. Consult a qualified clinician before using any peptide.

Summary

Human C1-esterase inhibitor (C1-INH) is a naturally occurring serpin present in plasma that regulates the complement and contact activation systems. Plasma-derived and recombinant forms are approved for prophylaxis and acute treatment of hereditary angioedema (HAE) due to C1-INH deficiency.

Mechanism of Action

Inhibits complement proteases C1r and C1s, plasma kallikrein, factor XIa, and factor XIIa, thereby suppressing activation of the classical complement pathway and contact activation (kallikrein-kinin) system, reducing bradykinin generation and vascular permeability

Routes of Administration

IntravenousSubcutaneous

Goals & Uses

  • Routine prophylaxis of HAE attacksPreventionHigh
  • Pediatric HAE managementTreatmentModerate
  • Pre-procedural prophylaxis of HAEPreventionHigh
  • Acute treatment of HAE attacksTreatmentHigh
  • Capillary leak syndrome / sepsis-associated inflammationInvestigationalLow

Contraindications

  • Known hypersensitivity to C1-INH preparationsAllergy / HypersensitivityHigh
  • Rabbit allergy (Ruconest/Conestat alfa)AllergyHigh
  • History of life-threatening thrombotic events (high-dose off-label use)Thrombotic RiskHigh

Adverse Effects

  • Hypersensitivity / anaphylaxisImmunologicalRare
  • HeadacheNeurologicCommonPain in the head or upper neck
  • Injection/infusion site reactionsLocalCommon
  • NauseaGastrointestinalUncommonFeeling of sickness or urge to vomit
  • PyrexiaGeneralUncommon
  • ThrombosisHematologicRareFormation of a blood clot

Drug Interactions

  • Estrogen-containing oral contraceptivesModerate
  • Tissue plasminogen activator (tPA)Low

Population Constraints

  • Patients with high thrombosis riskCardiovascularRelative
  • PregnancyReproductive SafetyRelative
  • Neonates / infants (off-label high-dose use)PediatricRelative

Regulatory Status

  • European UnionApprovedApproved: Treatment and prophylaxis of HAE in adults and children (Berinert), Treatment of HAE attacks and short-term prophylaxis (Cinryze), Acute treatment of HAE attacks in adults (Ruconest)EMA approved multiple plasma-derived and recombinant formulations.
  • United StatesApprovedApproved: Acute HAE attacks (Berinert, adults and pediatric patients), Routine prophylaxis of HAE (Cinryze, adults and adolescents), Subcutaneous prophylaxis of HAE (Haegarda, adults and adolescents), Acute HAE attacks (Ruconest/conestat alfa, adults and adolescents ≥13 years)Multiple distinct products approved by FDA under separate BLAs.
  • United KingdomApprovedApproved: Treatment and prophylaxis of HAE (plasma-derived C1-INH products), Acute HAE treatment (Ruconest)Approved via MHRA; products largely mirrored EMA approvals pre-Brexit and independently maintained thereafter.

Multiple products approved in the US, EU, and UK. Plasma-derived C1-INH (Berinert, Cinryze, Haegarda) and recombinant human C1-INH (Ruconest/Conestat alfa) are distinct regulatory entities. Berinert was approved by FDA in 2009; Cinryze in 2008; Haegarda in 2017; Ruconest in 2014.

Evidence & Sources

No sources recorded yet.

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