Human C1-esterase inhibitor
Also known as: Berinert, C1-INH, Cinryze, Conestat alfa, Haegarda, Ruconest, SERPING1
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Summary
Human C1-esterase inhibitor (C1-INH) is a plasma-derived or recombinant serpin used to prevent and treat attacks of hereditary angioedema by restoring deficient C1-INH activity.
Mechanism of Action
Inhibits plasma serine proteases C1r, C1s, MASP-1, MASP-2, plasma kallikrein, and plasmin, thereby regulating complement activation and bradykinin generation.
Routes of Administration
Goals & Uses
- Routine prophylaxis of HAE attacksPreventionHigh
- Pediatric HAE managementTreatmentModerate
- Pre-procedural prophylaxis of HAEPreventionHigh
- Acute treatment of HAE attacksTreatmentHigh
- Hereditary angioedema prophylaxis and acute treatmentIndicationHigh
- Capillary leak syndrome / sepsis-associated inflammationInvestigationalLow
Contraindications
- Uncontrolled disseminated intravascular coagulationCoagulationModerate
- Severe IgA deficiency with anti‑IgA antibodiesImmunologyModerate
- Known hypersensitivity to C1-INH preparationsAllergy / HypersensitivityHigh
- Hypersensitivity to C1-INH productAllergyHigh
- 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
- Thromboembolic eventsCardiovascularRare
- Hypersensitivity reactionImmunologicRare
- NauseaGastrointestinalUncommonFeeling of sickness or urge to vomit
- PyrexiaGeneralUncommon
- Injection site erythemaLocalCommonRedness at the injection site
- ThrombosisHematologicRareFormation of a blood clot
Drug Interactions
- Estrogen‑containing oral contraceptivesModerate
- Estrogen-containing oral contraceptivesModerate
- ACE inhibitorsModerate
- Tissue plasminogen activator (tPA)Low
- Antifibrinolytics (e.g., tranexamic acid)Low
Population Constraints
- Patients with high thrombosis riskCardiovascularRelative
- PregnancyReproductive SafetyRelative
- Pediatric patients <1 yearAgeRelative
- Pregnant womenReproductiveRelative
- Elderly (>75 years)GeriatricRelative
- Neonates / infants (off-label high-dose use)PediatricRelative
Regulatory Status
- European UnionApprovedApproved: Hereditary angioedema prophylaxis, Acute treatment of hereditary angioedemaApproved under EMA for plasma‑derived C1‑INH products.
- United StatesApprovedApproved: Hereditary angioedema prophylaxis, Acute treatment of hereditary angioedemaCinryze (SC), Berinert (IV), Haegarda (SC) approved.
- United KingdomApprovedApproved: Hereditary angioedema prophylaxis, Acute treatment of hereditary angioedemaApproved by MHRA.
FDA‑approved (Cinryze, Berinert, Haegarda); EMA‑approved; indicated for HAE types I and II.
Evidence & Sources
No sources recorded yet.
Frequently Asked Questions
What is Human C1-esterase inhibitor?
Human C1-esterase inhibitor (C1-INH) is a plasma-derived or recombinant serpin used to prevent and treat attacks of hereditary angioedema by restoring deficient C1-INH activity.
What is Human C1-esterase inhibitor used for?
Human C1-esterase inhibitor is educationally associated with: Routine prophylaxis of HAE attacks, Pediatric HAE management, Pre-procedural prophylaxis of HAE, Acute treatment of HAE attacks, Hereditary angioedema prophylaxis and acute treatment, Capillary leak syndrome / sepsis-associated inflammation. Educational only — not medical advice.
How is Human C1-esterase inhibitor administered?
Recorded routes of administration: Intravenous, Subcutaneous.
What are the potential side effects of Human C1-esterase inhibitor?
Reported adverse effects include: Hypersensitivity / anaphylaxis, Headache, Injection/infusion site reactions, Thromboembolic events, Hypersensitivity reaction, Nausea, Pyrexia, Injection site erythema, Thrombosis. This list is not exhaustive — consult a qualified clinician.
Who should avoid Human C1-esterase inhibitor?
Recorded contraindications: Uncontrolled disseminated intravascular coagulation, Severe IgA deficiency with anti‑IgA antibodies, Known hypersensitivity to C1-INH preparations, Hypersensitivity to C1-INH product, Rabbit allergy (Ruconest/Conestat alfa), History of life-threatening thrombotic events (high-dose off-label use). Consult a qualified clinician before use.