Hereditary Angioedema (HAE) – Cinryze Prior Authorization (with Quantity Limit) Criteria – Medicare Part D

Initial Evaluation

Cinryze will be approved when ALL of the following are met:

1. The patient has a diagnosis of hereditary angioedema (HAE) which has been confirmed via two confirmatory tests of C1-INH antigenic level, C1-INH functional level, and C4 level as follows:
   1. Hereditary angioedema (HAE) due to C1INH deficiency [HAE-C1INH (Type I)]: decreased quantities of C4 and C1-INH (antigenic and functional level)
      OR
   2. Hereditary angioedema (HAE) due to C1INH deficiency [HAE-C1INH (Type II)]: decreased quantities of C4 and C1-INH function (C1-INH protein level may be normal) 
      OR
   3. Hereditary angioedema (HAE) with normal C1INH [HAE-nI-C1INH (Type III)]: Normal levels of C4 and C1-INH [antigenic and functional level (at baseline and during an attack)] AND ONE of the following:
      1. BOTH of the following:
         1. Family history of angioedema
            AND
         2. ALL other causes of angioedema have been ruled out
            OR
      2. Patient demonstrates a Factor XII mutation, angiopoietin-1 (ANGPT1) mutation, plasminogen (PLG) mutation, kininogen1 mutation, heparan sulfate 3-Osulfotransferase 6 gene mutation, or myoferlin gene mutation that is associated with the disease
         AND
2. Medications known to cause angioedema (e.g., ACE-Inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate 
   AND
3. The patient is within the FDA labeled age for the requested medication
   AND
4. ONE of the following:
   1. A. The requested medication will be used to treat acute HAE attacks AND the patient will NOT be using the requested medication in combination with another HAE medication indicated for treatment of acute HAE attacks 
      OR
   2. B. The requested medication will be used for prophylaxis against HAE attacks AND the patient will NOT be using the requested medication in combination with another HAE medication indicated for prophylaxis against HAE attacks
      AND
5. The patient does NOT have any FDA labeled contraindications to the requested medication
   AND
6. ONE of the following:
   1. The requested quantity (dose) does NOT exceed the program quantity limit 
      OR
   2. The requested quantity (dose) is greater than the program quantity limit and the prescriber has provided information (e.g., If used for acute attacks, frequency of attacks within the past 3 months has been greater than 2 attacks per month) in support of therapy with a higher dose or quantity for the requested indication 

Length of Approval: 12 months

Renewal Evaluation

Cinryze will be approved when ALL of the following are met:

1. The patient has been previously approved for the requested medication through the plan’s Prior Authorization criteria 
   AND
2. The patient has a diagnosis of hereditary angioedema (HAE) AND ONE of the following:
   1. The requested medication will be used to treat acute HAE attacks AND the patient will NOT be using the requested medication in combination with another HAE medication indicated for treatment of acute HAE attacks 
      OR
   2. The requested medication will be used for prophylaxis against HAE attacks AND the patient will NOT be using the requested medication in combination with another HAE medication indicated for prophylaxis against HAE attacks
      AND
3. The patient is within the FDA labeled age for the requested medication
   AND
4. The patient has had a decrease in the frequency or severity of acute attacks or has had stabilization of disease from use of the requested medication 
   AND
5. The patient does NOT have any FDA labeled contraindications to the requested medication
   AND
6. ONE of the following:
   1. The requested quantity (dose) does NOT exceed the program quantity limit 
      OR
   2. The requested quantity (dose) is greater than the program quantity limit and the prescriber has provided information (e.g., If used for acute attacks, frequency of attacks within the past 3 months has been greater than 2 attacks per month) in support of therapy with a higher dose or quantity for the requested indication

Length of Approval: 12 months