C1 esterase inhibitor recombinant (Rx)

Brand and Other Names:Ruconest
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

lyophilized powder for reconstitution

  • 2100 IU/vial

Hereditary Angioedema

Indicated for treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE)

<84 kg: 50 IU/kg infused over 5 minutes; not to exceed 4200 IU/dose

≥84 kg: 4200 IU IV infused over 5 min

If the attack symptoms persist, may administer a second dose at the recommended dose level (not to exceed 4200 IU/dose)

Not to exceed 2 doses/24 hr

Dosing Considerations

Effectiveness is not established in HAE patients with laryngeal attacks

Dosage Forms & Strengths

lyophilized powder for reconstitution

  • 2100 IU/vial

Hereditary Angioedema

Indicated for treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE)

Infants and children: Safety and efficacy not established

Adolescents

  • <84 kg: 50 IU/kg IV infused over 5 minutes; not to exceed 4200 IU/dose
  • ≥84 kg: 4200 IU IV infused over 5 min
  • If the attack symptoms persist, may administer a 2nd dose at the recommended dose level (not to exceed 4200 IU/dose)
  • Not to exceed 2 doses/24 hr

Dosing Considerations

Effectiveness is not established in HAE patients with laryngeal attacks

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Adverse Effects

1-10%

Headache (9%)

Nausea (2%)

Diarrhea (2%)

Postmarketing Reports

Abdominal pain

Rash

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Warnings

Contraindications

History of allergy to rabbits or rabbit-derived products

History of life-threatening immediate hypersensitivity reactions to C1 esterase inhibitor preparations, including anaphylaxis

Cautions

Severe hypersensitivity reactions may occur during or after injection; signs and symptoms include hives, generalized urticaria, tightness of the chest, wheezing, hypotension, and/or anaphylaxis

Serious arterial and venous thromboembolic (TE) events reported at recommended doses of plasma-derived C1-esterase inhibitor products in patients with risk factors; risk factors include indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives or certain androgens, morbid obesity, and immobility

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Pregnancy & Lactation

Pregnancy Category: B; may use during pregnancy if clearly needed

Studies performed in rats and rabbits at doses up to 12.5 times the human could not exclude an effect on embryofetal development; no adequate and well-controlled studies in pregnant women

Lactation: Unknown if distributed in human breast milk

Pregnancy Categories

A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA: Information not available.

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Pharmacology

Mechanism of Action

C1 esterase inhibitor (C1INH) is a normal constituent of human blood and is one of the serine protease inhibitors (serpins)

Human recombinant C1 esterase inhibitor purified from the milk of genetically modified (transgenic) rabbits; restores level of functional C1 esterase inhibitor in a patient’s plasma, thereby treating the acute attack of swelling

Patients with HAE have low levels of endogenous or functional C1 inhibitor; C1 inhibitor suppresses contact system activation through inactivation of plasma kallikrein and factor XIIa, modulating vascular permeability by preventing bradykinin generation

Absorption

Peak plasma concentration: 1.2-2.3 IU/mL

Peak plasma time: 0.31 hr

AUC: 3.3-10.6 IU•hr/mL

Distribution

Vd: 2.4-3 L

Elimination

Half-life: 2.4-2.7 hr

Clearance: 781-1207 mL/hr

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Administration

IV Preparation

Ensure C1 esterase inhibitor recombinant and sterile water for injection (SWI) are at room temperature

Reconstitute each vial (2100 IU) by adding 14 mL SWI; add the diluent slowly to avoid forceful impact on the powder

Swirl the vial slowly to mix and avoid foaming

Visually inspect for particulate matter and discoloration after reconstitution and prior to administration; reconstituted solution should be colorless, clear, and free from visible particles

Do not use if the solution is cloudy, colored, or contains particulates

Resulting solution concentration is 150 IU/mL

Use the reconstituted product immediately, or within 8 hr if stored refrigerated

Discard partially used vials

IV Administration

Do not mix with other medicinal products; administer by a separate infusion line

Administer by slow IV injection over 5 minutes

For self-administration, provide the patient with instructions and training for IV injection outside of a clinic setting so patients may self-administer upon recognition of symptoms of an HAE attack

Discard any unused product

Storage

Unreconstituted vials

  • Store in the original package in order to protect from light
  • Shelf life: 48 months when stored at 2-25°C (36-77°F)
  • Do not freeze

Reconstituted vials

  • Contains no preservatives
  • May store for 8 hr at 2-8°C (36-46°F)
  • Do not freeze
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Images

No images available for this drug.
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Patient Handout

A Patient Handout is not currently available for this monograph.
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Formulary

FormularyPatient Discounts

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The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

Tier Description
1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
NC NOT COVERED – Drugs that are not covered by the plan.
Code Definition
PA Prior Authorization
Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
QL Quantity Limits
Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
ST Step Therapy
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
OR Other Restrictions
Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.