C1-Esterase Inhibitor (Recombinant)
Class: Complement Inhibitors
CAS Number: CAS-80295-38-1
Brands: Ruconest
Introduction
Biosynthetic (recombinant DNA-origin) preparation of complement 1 (C1)-esterase inhibitor.
C1-esterase inhibitor is a naturally occurring inhibitor of certain serine proteases (e.g., C1 complement, kallikrein, coagulation factor XIIa, plasmin) involved in the complement, coagulation (contact), and fibrinolytic systems.
Uses for C1-Esterase Inhibitor (Recombinant)
Hereditary Angioedema
Treatment of acute angioedema attacks in patients with hereditary angioedema (HAE). Designated an orphan drug by FDA for this use.
Clinical studies were conducted principally in patients with abdominal, facial, or peripheral angioedema; efficacy in patients with laryngeal HAE attacks not established.
Recommended by US and international consensus guidelines as one of several options for treatment of acute HAE attacks; other drugs include ecallantide (plasma kallikrein inhibitor) and icatibant (bradykinin B2-receptor antagonist).
C1-Esterase Inhibitor (Recombinant) Dosage and Administration
Administration
IV Administration
Administer by slow IV injection.
Initiate therapy under supervision of a qualified healthcare professional experienced in the treatment of HAE. May be self-administered after appropriate training is provided.
Do not mix or administer with any other drug or solution.
Vials are for single use only; discard any unused portions.
Reconstitution
Allow vials of drug and diluent (sterile water for injection) to reach room temperature prior to reconstitution.
Reconstitute vial containing 2100 units of C1-esterase inhibitor (recombinant) with 14 mL of sterile water for injection. Add diluent slowly to avoid forceful impact on the powder. Gently swirl vial to ensure complete dissolution. Resultant solution contains 150 units/mL. If more than 1 vial is required to obtain a dose, may pool reconstituted contents of multiple vials into a single administration device (i.e., syringe).
Administer reconstituted solutions immediately or within 8 hours if stored at 2–8°C.
Rate of Administration
Administer by IV injection over approximately 5 minutes.
Dosage
Dosage of C1-esterase inhibitor (recombinant) is expressed in international units (IU, units). One unit of C1-esterase inhibitor (recombinant) is equivalent to the C1-esterase-inhibiting activity present in 1 mL of pooled normal plasma.
Pediatric Patients
Hereditary Angioedema
Treatment of Acute Angioedema Attacks
IVAdolescents 13–17 years of age: 50 units/kg (up to 4200 units) for patients weighing <84 kg; 4200 units in patients weighing ≥84 kg.
May administer a second dose if attack symptoms persist.
Adults
Hereditary Angioedema
Treatment of Acute Angioedema Attacks
IV50 units/kg (up to 4200 units) for patients weighing <84 kg; 4200 units in patients weighing ≥84 kg.
May administer a second dose if attack
