Cinryze — Blue Cross Blue Shield of Montana

Preferred products

• icatibant (generic)

Initial criteria

• Diagnosis of hereditary angioedema (HAE) due to C1INH deficiency (Type 1 or Type 2) confirmed by EITHER laboratory measurements (C1-INH protein level, function level, and C4 level) showing: Type 1 HAE – decreased C4, C1-INH protein, and C1-INH function; OR Type 2 HAE – decreased C4 and C1-INH function with normal or elevated C1-INH protein, OR mutation in the C1-INH gene altering protein synthesis/function
• OR diagnosis of hereditary angioedema with normal C1INH (HAE-nl-C1INH) evidenced by normal C1-INH protein level, function level, and C4 level AND either mutation in one of: F12, plasminogen, angiopoietin-1, kininogen-1, heparan sulfate 3-O-sulfotransferase 6, or myoferlin gene OR confirmed HAE-U diagnosis by specialist
• If the patient has an FDA-labeled indication, then EITHER patient’s age within FDA labeling OR support for use at that age for the requested indication
• Medications known to cause angioedema (ACE inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate
• Requested agent used to treat acute HAE attacks
• If requesting a brand agent with an available generic equivalent (Firazyr/icatibant): EITHER member plan type matches BCBS IL Fully Insured, ASO Cost/BBF, HIM, or Non-ERISA ASO/Self-insured Municipalities/Counties OR patient is stable on requested brand OR inadequate response or intolerance/adverse reaction to generic equivalent OR FDA-labeled contraindication to generic equivalent OR generic expected ineffective based on patient characteristics OR generic not in best interest OR prior failure of similar agent OR supported rationale for brand use (chart notes required where indicated)
• Prescriber is or has consulted with a specialist in HAE (e.g., allergist, immunologist)
• Requested agent not used in combination with another agent indicated for acute HAE attacks (Berinert, Ekterly, Firazyr, icatibant, KALBITOR, RUCONEST)
• Patient has no FDA-labeled contraindications
• For BCBS MT Fully Insured or MT HIM members: patient age < 18 years, no FDA-labeled contraindications, indication supported as safe and effective by TWO peer-reviewed journal articles, and age group supported by TWO such articles (not case studies)
• For BCBS NM Fully Insured or NM HIM members: no FDA-labeled contraindications, indication is a rare disease, AND (has another FDA-labeled indication OR supported compendia indication OR TWO peer-reviewed journal articles supporting proposed use)
• For BCBS OH Fully Insured or HIM Shop members: resides in Ohio, no FDA-labeled contraindications, AND (another FDA-labeled indication OR supported compendia indication OR TWO peer-reviewed journal articles supporting proposed use)

Reauthorization criteria

• Patient previously approved for the requested agent through the plan’s Prior Authorization process
• Prescriber is or has consulted with a specialist (e.g., allergist, immunologist)
• Patient has had clinical benefit with the requested agent
• If requesting a brand agent with an available generic equivalent (Firazyr/icatibant): same criteria as initial (plan type, stable use, failed generic, intolerance/adverse events, contraindication, inefficacy, or best interest)
• Prescriber verified frequency/severity of attacks and confirmed patient does not have >1-month supply (sufficient for 2 acute attacks) currently on hand
• Requested agent not used with another acute HAE treatment (Berinert, Ekterly, Firazyr, icatibant, KALBITOR, RUCONEST)
• Patient has no FDA-labeled contraindications

Approval duration

12 months