Berinert — Medical Mutual

Preferred products

• Ruconest

Initial criteria

• Must be prescribed by, or in consultation with, a specialist in allergy, immunology, hematology, pulmonology, or medical genetics; AND
• Confirmation the patient is avoiding the following possible triggers for HAE attacks: Estrogen-containing oral contraceptive agents AND hormone replacement therapy; AND Antihypertensive agents containing ACE inhibitors or angiotensin II receptor blockers (ARBs); AND Dipeptidyl peptidase IV (DPP-IV) inhibitors (e.g., sitagliptin); AND Neprilysin inhibitors (e.g., sacubitril); AND
• Patient has a history of moderate to severe cutaneous attacks (without concomitant urticaria) OR abdominal attacks OR mild to severe airway swelling attacks of HAE (i.e. debilitating cutaneous/gastrointestinal symptoms OR laryngeal/pharyngeal/tongue swelling); AND
• Patient meets one of the following: Patient has tried the preferred product, Ruconest [documentation required]; OR Patient has had a history of at least one laryngeal attack that had been treated with Berinert, as per the prescriber; OR Patient has an allergy to rabbits or rabbit-derived products; OR Patient is less than 13 years of age; AND
• Patient has one of the following clinical presentations consistent with a HAE subtype, confirmed by repeat blood testing:
• HAE I (C1-Inhibitor deficiency): Low C1 inhibitor antigenic level below lab normal AND Low C4 level below lab normal AND Low C1-INH functional level below lab normal; AND Patient has a family history of HAE OR Acquired angioedema ruled out (symptom onset < age 30, normal C1q, no underlying disease such as lymphoma or MGUS)
• HAE II (C1-Inhibitor dysfunction): Normal or elevated C1-INH antigenic level; AND Low C4 level below lab normal; AND Low C1-INH functional level below lab normal
• HAE with normal C1INH (HAE III): Normal/near normal C1-INH antigenic, C4, and functional levels; AND Repeat blood testing during attack confirms not HAE I/II; AND either patient has known HAE-causing mutation OR patient has family history of HAE AND documented lack of efficacy of chronic high-dose antihistamine therapy ± corticosteroids/omalizumab

Reauthorization criteria

• Patient continues to meet the universal and other indication-specific relevant criteria identified in section III; AND
• Significant improvement in severity and duration of attacks have been achieved and sustained; AND
• Absence of unacceptable toxicity from the drug (e.g., severe hypersensitivity reaction, serious thromboembolic events); AND
• Patient meets one of the following: Patient has tried the preferred product, Ruconest [documentation required]; OR Patient has had a history of at least one laryngeal attack that had been treated with Berinert, as per the prescriber; OR Patient has an allergy to rabbits or rabbit-derived products; OR Patient is less than 13 years of age; AND
• The cumulative amount of HAE medication on-hand is taken into account; authorization provides sufficient supply to treat up to 4 acute attacks per 4 weeks

Approval duration

12 weeks