Rilonacept (Arcalyst™) is a dimeric fusion protein consisting of the ligand-binding domains of the extracellular portions of the human interleukin-1 receptor component (IL-1RI) and IL-1 receptor accessory protein (IL-1RAcP) linked in-line to the Fc portion of human IgG1. Rilonacept (Arcalyst) blocks IL-1b signaling by acting as a soluble decoy receptor that binds IL-1b and prevents its interaction with cell surface receptors. Rilonacept (Arcalyst) also binds IL-1α and IL-1 receptor antagonist (IL-1ra) with reduced affinity.

Canakinumab (Ilaris®) is a recombinant, human anti-human-IL-1b monoclonal antibody that belongs to the IgG1/K isotype subclass. Canakinumab (Ilaris) binds to human IL-1b and neutralizes its activity by blocking its interaction with IL-1 receptors, but it does not bind IL-1a or IL-1 receptor antagonist (IL-1ra).

The use of rilonacept (Arcalyst) for an initial authorization period of 12 months may be considered medically necessary when the following criteria are met:

Cryopyrin-Associated Periodic Syndromes (CAPS)

• As treatment of ANY of the following CAPS diagnoses in individuals 12 years of age or older:
  • Familial cold autoinflammatory syndrome (FCAS); or
  • Muckle-Wells Syndrome (MWS); or

Deficiency of Ineterleukin-1 Receptor Antagonist (DIRA)

• Individual diagnosed with DIRA requiring maintenance of remission in individuals weighing ten (10) kg or more; and
• Individual has previously experienced clinical benefit from anakinra for the induction treatment of DIRA; or

Recurrent Pericarditis (RP)

• Individual diagnosed with RP; and
• Individual aged 12 years or older; and
• The individual has had at least one (1) episode of acute pericarditis in the past 365 days; and
• The individual has had therapeutic failure, intolerance, or contraindication to colchicine; and
• The individual has had therapeutic failure or intolerance to one (1) of the following or contraindication to all:
  • Oral nonsteroidal anti-inflammatory drug (NSAID); or
  • Systemic corticosteroid.

Reauthorization Criteria

Continuation of therapy with rilonacept (Arcalyst) may be considered medically necessary when the following criteria are met:

·       The individual has one of the above diagnoses; and

·       Provider attestation that individual has demonstrated a disease stability or beneficial response to therapy; and

·       Reauthorization valid for 12 months.

The use of rilonacept (Arcalyst) for any other indication is considered not medically necesary.

The use of canakinumab (Ilaris) for an initial authorization period of 12 months may be considered medically necessary when the following criteria are met:

CAPS

• As treatment of ANY of the following CAPS diagnoses in individuals four (4) years of age or older:
  • FCAS; or
  • MWS; or

Juvenile Idiopathic Arthritis (SJIA)

• As treatment of SJIA in individuals two (2) years of age or older; and
• Individual has had an inadequate response, intolerance or contraindication to one (1) or more DMARD therapies; or

Still’s Disease

• Individual diagnosed with active Still’s disease, including Adult-Onset Still’s Disease (AOSD) in individuals two (2) years or older; and
• The individual has experienced therapeutic failure, intolerance or contraindication to at least one (1) nonsteroidal anti-inflammatory drug (NSAID); and
• The individual has experienced therapeutic failure, intolerance or contraindication to at least one (1) corticosteroid; and
• The individual has experienced therapeutic failure, intolerance or contraindication to at least one (1) disease-modifying antirheumatic drug (DMARD); or

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS), Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD), and Familial Mediterranean Fever (FMF)

• As treatment of individuals with ANY of the following:
  • FMF; or
  • HIDS; or
  • MKD; or
  • TRAPS.

 Reauthorization Criteria

Continuation of therapy with canakinumab (Ilaris) may be considered medically necessary when the following criteria are met:

·       The individual has one of the above diagnoses; and

·       Provider attestation that individual has demonstrated a disease stability or beneficial response to therapy; and

·       Reauthorization valid for 12 months.

The use of canakinumab (Ilaris) for any other indication is considered not medically necessary. 

NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Highmark may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.