Berinert for treating acute hereditary angioedema attacks

Berinert is a human plasma-derived C1 esterase inhibitor concentrate used to treat acute hereditary angioedema (HAE) attacks involving the abdomen, face, or larynx in adult and pediatric patients. It is administered intravenously as a reconstituted powder to manage symptoms during acute episodes but is not approved for preventive use.

• Treatment of acute hereditary angioedema attacks: Indicated for adults and children experiencing acute abdominal, facial, or laryngeal attacks of HAE.

C1 esterase inhibitor (human)

Berinert works by replacing the deficient or dysfunctional C1 esterase inhibitor protein in patients with hereditary angioedema. This helps control excessive activation of the complement and contact systems that cause swelling, thereby reducing the severity of acute HAE attacks.

Berinert is for intravenous use only.

• Dose: 20 International Units (IU) per kg of body weight.
• Doses lower than 20 IU/kg should not be given.
• Reconstitute the 500 IU lyophilized powder with 10 mL of provided Sterile Water for Injection.
• Use the provided silicone-free syringe and Mix2Vial transfer set or suitable sterile equipment for reconstitution and administration.
• Administer the reconstituted solution by slow intravenous injection at approximately 4 mL per minute.
• Use the solution within 8 hours of reconstitution; store at room temperature (2-30°C, 36-86°F) and do not freeze.
• Do not mix Berinert with other medicinal products; administer by a separate infusion line.

Appropriately trained patients may self-administer upon recognizing an HAE attack, but should seek immediate medical care after treating laryngeal attacks.

Use is contraindicated in individuals with a history of life-threatening immediate hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor products.

Hypersensitivity and allergic reactions

Severe hypersensitivity reactions, including anaphylaxis, may occur. Epinephrine should be immediately available to treat acute reactions. Signs may include hives, chest tightness, wheezing, hypotension, and anaphylaxis during or after administration. Discontinue Berinert immediately if hypersensitivity is suspected.

Thromboembolic events

Serious arterial and venous thromboembolism (TE) events have been reported at recommended doses in patients with HAE. Risk factors include indwelling venous catheters, prior thrombosis, atherosclerosis, use of oral contraceptives or androgens, morbid obesity, and immobility. Patients with these risk factors should be monitored carefully during and after treatment. Higher than recommended doses or unapproved uses also carry TE risk.

Risk of infectious disease transmission

Berinert is made from human plasma and carries a theoretical risk of transmitting infectious agents, including viruses and the agents of variant Creutzfeldt-Jakob disease (vCJD). Extensive screening and viral inactivation processes minimize this risk, and no confirmed transmissions have been documented. Any suspected infection should be reported to the manufacturer.

Laryngeal attacks

Because of potential airway obstruction during laryngeal HAE attacks, patients self-administering Berinert for these attacks should be advised to seek emergency medical care immediately after treatment.

• Serious: Increase in severity of pain associated with HAE has been reported.
• Common (>4% incidence): Dysgeusia (altered taste).

Thromboembolic events have been reported following administration of C1 esterase inhibitor products at higher than recommended doses for unapproved indications.

• Store unopened vials protected from light at 2-30°C (36-86°F).
• Do not freeze.
• After reconstitution, store solution at room temperature and use within 8 hours.
• Do not refrigerate or freeze the reconstituted solution.