Berinert — CareFirst (Caremark)

Initial criteria

• Medication must be prescribed by or in consultation with a prescriber who specializes in the management of hereditary angioedema (HAE).
• For preprocedural prophylaxis: Authorization of 30 days may be granted when both of the following criteria are met at the time of diagnosis:
• • Member has C1 inhibitor deficiency or dysfunction confirmed by laboratory testing AND meets one of the following: (a) C1 inhibitor (C1-INH) antigenic level below the lower limit of normal as defined by the laboratory performing the test, OR (b) Normal C1-INH antigenic level and low C1-INH functional level (functional C1-INH <50% or below the lower limit of normal).
• OR Member has normal C1 inhibitor as confirmed by laboratory testing AND meets one of the following: (a) Pathogenic variant in F12, angiopoietin-1, plasminogen, kininogen-1 (KNG1), heparan sulfate-glucosamine 3-O-sulfotransferase 6 (HS3ST6), or myoferlin (MYOF) confirmed by genetic testing, OR (b) Documented family history of angioedema and refractory to a trial of high-dose antihistamine therapy (e.g., cetirizine 40 mg per day or equivalent) for at least one month.
• • Other causes of angioedema (ACE inhibitor–induced angioedema, estrogen-related angioedema, allergic angioedema) have been ruled out.
• For acute attacks: Authorization of 12 months may be granted for treatment of acute HAE attacks when the requested medication will not be used in combination with any other acute HAE medication AND both of the following are met at diagnosis:
• • Same diagnostic criteria as above for HAE (C1-INH deficiency or pathogenic variant or refractory family history).
• • Other causes of angioedema have been ruled out (ACE inhibitor–induced, estrogen-related, allergic angioedema).

Reauthorization criteria

• For continued preprocedural prophylaxis: Member must meet all requirements from coverage criteria section.
• For continued treatment of acute HAE attacks: Member meets all coverage criteria AND has experienced reduction in severity and/or duration of acute attacks.
• Prophylaxis should be considered based on attack frequency, severity, comorbid conditions, and quality of life.

Approval duration

30 days (preprocedural prophylaxis); 12 months (acute attacks)