rilonacept — Blue Cross Blue Shield of Texas

Initial criteria

• 1. ONE of the following:
• A. BOTH of the following:
• 1. Diagnosis of Cryopyrin-Associated Periodic Syndrome (CAPS) AND BOTH of the following:
• a. ONE of Familial Cold Autoinflammatory Syndrome (FCAS) OR Muckle-Wells Syndrome (MWS)
• b. BOTH of:
• i. History of elevated pretreatment serum inflammatory markers (CRP/serum amyloid A)
• ii. History of ≥ 2 symptoms typical for CAPS (e.g., urticarialike rash, cold/stress triggered episodes, sensorineural hearing loss, arthralgia/arthritis/myalgia, chronic aseptic meningitis, skeletal abnormalities such as epiphyseal overgrowth/frontal bossing)
• OR
• B. Diagnosis of deficiency of interleukin-1 receptor antagonist (DIRA) AND BOTH of:
• 1. Diagnosis confirmed via genetic testing for mutations in the IL1RN gene
• 2. Requested agent used for maintenance of remission
• OR
• C. Diagnosis of recurrent pericarditis (RP) AND BOTH of:
• 1. Pericarditis that recurs after a symptom-free interval ≥ 4 weeks after an initial episode
• 2. ONE of the following:
• A. All of the following:
• 1. Tried and had inadequate response to colchicine for ≥ 6 months AND
• 2. ONE of:
• a. Colchicine used with NSAID for ≥ 1 week OR
• b. Colchicine used with aspirin for ≥ 1 week OR
• c. Intolerance or hypersensitivity to ≥ 1 NSAID or aspirin OR
• d. FDA labeled contraindication to all NSAIDs and aspirin used in RP AND
• 3. ONE of:
• a. Colchicine used with corticosteroid for ≥ 1 week OR
• b. Intolerance or hypersensitivity to ≥ 1 corticosteroid OR
• c. FDA labeled contraindication to all corticosteroids used in RP
• B. Intolerance or hypersensitivity to colchicine OR
• C. FDA labeled contraindication to colchicine
• OR
• D. Another FDA labeled indication for the requested agent and route of administration
• OR
• E. Another indication supported in compendia for the requested agent and route of administration
• 2. If request is for Arcalyst for DIRA, patient weighs ≥ 10 kg
• 3. Prescriber is a specialist in relevant area (e.g., allergist, cardiologist, immunologist, pediatrician, rheumatologist) or has consulted one
• 4. ONE of the following regarding concomitant immunomodulators:
• A. Will NOT be used with another immunomodulatory agent (e.g., TNF inhibitors, JAK inhibitors, IL-4 inhibitors) OR
• B. Used in combination AND BOTH of:
• 1. Prescribing information does NOT limit combination use
• 2. There is supporting evidence (clinical trials, phase III studies, or guidelines) submitted
• 5. Patient does NOT have any FDA labeled contraindications to the requested agent
• Compendia Allowed: AHFS, or DrugDex 1, 2a, 2b level of evidence
• Length of Approval: 12 months
• Additional approval cases:
• 1. MT members under age 18 years meeting safety and literature-based criteria (no contraindications; supported by ≥ 2 peer-reviewed studies for indication and age bracket)
• OR
• 2. Ohio residents with fully insured or HIM Shop plan meeting one of the following: another FDA labeled indication, another compendia-supported indication, or ≥ 2 peer-reviewed studies supporting proposed use as safe and effective.

Reauthorization criteria

• 1. Patient previously approved for requested agent through plan’s prior authorization process
• 2. Patient has had clinical benefit with requested agent
• 3. Prescriber is specialist in relevant area or has consulted one
• 4. ONE of the following regarding concomitant immunomodulators:
• A. Will NOT be used with another immunomodulatory agent (e.g., TNF inhibitors, JAK inhibitors, IL-4 inhibitors)
• B. Used in combination AND BOTH of:
• 1. Prescribing information does NOT limit combination use
• 2. Supporting evidence (clinical trials, phase III studies, or guidelines) submitted
• 5. Patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

12 months