C1 Inhibitor (Recombinant)

• It is used to treat swelling attacks in people with hereditary angioedema (HAE).

• Store in the original container at room temperature or in a refrigerator.
• Do not freeze.
• Protect from light.
• After mixing, store in a refrigerator. Check with the doctor or pharmacist if you have questions about how long C1 inhibitor may be used after mixing.
• Keep all drugs in a safe place. Keep all drugs out of the reach of children and pets.
• Check with your pharmacist about how to throw out unused drugs.

C1 inhibitor, a serine protease inhibitor (serpin), regulates the activation of the complement and contact system pathways by irreversibly binding target proteases. Suppression of contact system activation by C1 inhibitor through the inactivation of plasma kallikrein and factor XIIa is thought to modulate vascular permeability that leads to clinical manifestations of hereditary angioedema (HAE) attacks by preventing the generation of bradykinin.

Distribution

Vss: ~3 L

Life-threatening immediate hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor preparations or any component of the formulation; allergy to rabbits or rabbit-derived products

There are no dosage adjustments provided in the manufacturer’s labeling (has not been studied).

>10%:

Central nervous system: Headache (more common in adolescents than in adults)

Gastrointestinal: Abdominal pain (adolescents: ≥12%)

Respiratory: Oropharyngeal pain (adolescents: ≥12%)

1% to 10%:

Central nervous system: Vertigo (3%)

Dermatologic: Burning sensation of skin (2%), erythema (2%; marginatum)

Gastrointestinal: Diarrhea (≥2%), nausea (≥2%)

Hematologic & oncologic: C-reactive protein increased (2%), increased fibrin (2%), lipoma (2%)

Hypersensitivity: Angioedema (3%)

Immunologic: Antibody development (after repeat therapy exposure, non-neutralizing)

Neuromuscular & skeletal: Back pain (3%)

Respiratory: Sneezing (2%)

Limited to important or life-threatening: Abdominal pain, arterial thromboembolism, hypersensitivity reaction, venous thromboembolism

Concerns related to adverse effects:

• Hypersensitivity: Severe hypersensitivity reactions (eg, urticaria, hives, tightness of the chest, wheezing, hypotension, anaphylaxis) may occur during or after administration. Signs/symptoms of hypersensitivity reactions may be similar to the attacks associated with hereditary angioedema, therefore, consideration should be given to treatment methods. In the event of acute hypersensitivity reactions to C1 inhibitor therapy, treatment should be discontinued and appropriate treatment should be instituted.

• Thrombotic events: Serious arterial and venous thromboembolic events have been reported at recommended doses in patients with risk factors (eg, presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives or certain androgens, morbid obesity, immobility). Closely monitor patients with preexisting risks for thrombotic events during and after administration.

Monitor for signs/symptoms of hypersensitivity reactions and thrombotic events.

Applies to conestat alfa: intravenous powder for injection

General

The most common adverse reactions were headache, nausea, and diarrhea.[Ref]

Nervous system

Very common (10% or more): Headache (up to 10%)
Common (1% to 10%): Vertigo, procedural headache
Uncommon (0.1% to 1%): Paresthesia[Ref]

Dermatologic

Common (1% to 10%): Angioedema, erythema marginatum, skin burning sensation
Uncommon (0.1% to 1%): Urticaria
Postmarketing reports: Rash[Ref]

Gastrointestinal

Common (1% to 10%): Nausea, diarrhea
Uncommon (0.1% to 1%): Abdominal discomfort, oral paresthesia
Postmarketing reports: Abdominal pain[Ref]

Respiratory

Common (1% to 10%): Sneezing
Uncommon (0.1% to 1%): Throat irritation[Ref]

Hematologic

Common (1% to 10%): Fibrin D-dimer increased[Ref]

Immunologic

Common (1% to 10%): C-reactive protein increased[Ref]

Musculoskeletal

Common (1% to 10%): Back pain[Ref]

Ocular

Common (1% to 10%): Lipoma

Other

Uncommon (0.1% to 1%): Swelling[Ref]

Some side effects of conestat alfa may not be reported. Always consult your doctor or healthcare specialist for medical advice. You may also report side effects to the FDA.

Data not available