Ruconest – NC Standard

Restricted Product(s)

• Ruconest® (C1 esterase inhibitor (recombinant))

FDA Approved Use

• For the treatment of acute attacks of hereditary angioedema (HAE) in adults and adolescent patients 13 years of age and older.

Criteria for Approval of Restricted Product(s)

Initial Coverage

1. The patient has a diagnosis of Hereditary Angioedema (HAE) as confirmed through complement testing (Please submit medical record documentation to demonstrate C4 level, C1-inhibtor level, and C1-INH function where applicable); AND 
   1. If HAE-I, please demonstrate low C4 and C1-inhibitor level; AND 
   2. If HAE-II, please demonstrate low C4 with a normal C1-inhibitor level and a decreased C1-INH function; AND 
   3. If HAE-III, please demonstrate normal C4 and C1-inhibitor and family history of angioedema; AND 
2. The patient is 13 years of age or older; AND 
3. This medication will be used to treat acute HAE attacks; AND 
4. The patient must be experiencing at least one symptom of moderate or severe HAE attack (e.g., airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion); AND 
5. The patient will NOT be using the requested agent in combination with another agent indicated for the treatment of acute HAE attacks (i.e., Firazyr, icatibant, Kalbitor, Berinert); AND 
6. The prescribed dose and quantity are appropriate based on intended use and FDA labeling; AND 
7. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., allergist, immunologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis; AND 
8. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.
   
   Duration of Approval: 365 days (1 year)

Continuation Criteria

1. The patient has been previously approved with Blue Cross and Blue Shield of North Carolina (Blue Cross NC) or would have met initial criteria for approval upon the start of therapy; AND 
2. There is documentation that the patient has shown quick symptomatic relief with the requested medication (i.e. decrease in median time to near-complete symptom relief with the patient’s HAE attacks) (medical record documentation required); AND 
3. The patient will NOT be using the requested agent in combination with another agent indicated for the treatment of acute HAE attacks (i.e., Firazyr, Icatibant, Kalbitor, Berinert); AND 
4. The prescribed dose and quantity is appropriate based on intended use and FDA labeling; AND 
5. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., allergist, immunologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis.
   
   Duration of Approval: 365 days (1 year)

Quantity Limit Exception Criteria

1. The quantity (dose) requested is for documented titration purposes at the initiation of therapy (authorization for a 90 day titration period); AND 
2. The prescribed dose cannot be achieved using a lesser quantity of a higher strength; AND 
3. The quantity (dose) requested does not exceed the maximum FDA labeled dose, when specified, or to the safest studied dose per the manufacturer’s product insert; OR 
4. If the quantity (dose) requested exceeds the maximum FDA labeled dose, when specified, or to the safest studied dose per the manufacturer’s product insert, then the prescriber must submit documentation in support of therapy with a higher dose for the intended diagnosis (submitted documentation may include medical records OR fax form which reflects medical record documentation that shows the length of time the requested dose has been used, and what other medications and doses have been tried and failed). 

References

All information referenced is from FDA package insert unless otherwise noted below.

Policy Implementation/Update Information

April 2022: Criteria change: Added complement testing with results for HAE I, II, and III. Added patient will NOT be using the requested agent in combination with another anti-HAE agent. Added prescriber is a specialist or consulted a specialist. Added dose and quantity are appropriate based on FDA labeling. Added continuation criteria.
February 2021: Original utilization management criteria issued