ILARIS (canakinumab)

ILARIS (canakinumab)

Indication for Prior Authorization:

Periodic Fever Syndromes: Indicated for the treatment of the following autoinflammatory Periodic Fever Syndromes: 

• Cryopyrin-Associated Periodic Syndromes (CAPS), in adults and children 4 years of age and older including, Familial Cold Autoinflammatory Syndrome (FCAS) or Muckle-Wells Syndrome (MWS).
• Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) in adult and pediatric patients.
• Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD) in adult and pediatric patients.
• Familial Mediterranean Fever (FMF) in adult and pediatric patients.

Systemic Juvenile Idiopathic Arthritis (SJIA): Indicated for the treatment of active Systemic Juvenile Idiopathic Arthritis (SJIA) in patients aged 2 years and older. 

Still’s disease (including Adult-Onset Still’s Disease [AOSD]): Indicated for the treatment of active Still’s disease, including Adult-Onset Still’s Disease (AOSD) in patients aged 2 years and older

Coverage Criteria:

For diagnosis of periodic fever syndromes:

• Documented diagnosis of one of the following periodic fever syndromes:
  • Cryopyrin-associated periodic syndromes (CAPS), including:
    • Familial cold autoinflammatory syndrome (FCAS) 
    • Muckle-Wells syndrome (MWS) 
  • Tumor necrosis factor (TNF) receptor associated periodic syndrome (TRAPS) 
  • Hyperimmunoglobulin D (Hyper-IgD) syndrome (HIDS)/Mevalonate kinase deficiency (MKD) 
  • Familial Mediterranean fever (FMF); AND
• Prescribed by or in consultation with a rheumatologist, allergist, immunologist, dermatologist, neurologist, or other medical specialist (e.g., geneticist); AND
• Appropriate dose of medication is requested based on age, weight, and indication; AND
• Both of the following:
  • Patient is not receiving concomitant treatment with Tumor Necrosis Factor (TNF) inhibitors (e.g., Enbrel [etanercept], Humira [adalimumab], Remicade [infliximab]) 
  • Patient is not receiving concomitant treatment with Interleukin-1 inhibitor (e.g., Arcalyst [rilonacept], Kineret [anakinra])

For diagnosis of Systemic Juvenile Idiopathic Arthritis (SJIA):

• Documented diagnosis of active SJIA; AND
• Prescribed by or in consultation with a rheumatologist, AND
• Appropriate dose of medication is requested based on age, weight, and indication; AND
• Trial and failure, contraindication, or intolerance to one of the following conventional therapies at maximally tolerated doses: 
  • Minimum duration of a 3-month trial and failure of methotrexate
  • Minimum duration of a 1-month trial of nonsteroidal anti-inflammatory drug (NSAID) (e.g., ibuprofen, naproxen)
  • Minimum duration of a 2-week trial of systemic glucocorticoid (e.g., prednisone); AND
• Both of the following:
  • Patient is not receiving concomitant treatment with Tumor Necrosis Factor (TNF) inhibitors (e.g., Enbrel [etanercept], Humira [adalimumab], Remicade [infliximab]) 
  • Patient is not receiving concomitant treatment with Interleukin-1 inhibitor (e.g., Arcalyst [rilonacept], Kineret [anakinra])

For diagnosis of Still’s Disease (including Adult-Onset Still’s Disease [AOSD]):

• Documented diagnosis of active Still’s Disease, including AOSD; AND
• Prescribed by or in consultation with a rheumatologist, AND
• Appropriate dose of medication is requested based on age, weight, and indication; AND
• Trial and failure, contraindication, or intolerance to one of the following: 
  • Corticosteroids (e.g., prednisone, methylprednisolone) 
  • Methotrexate 
  • Nonsteroidal anti-inflammatory drugs (NSAIDs) (e.g., ibuprofen, naproxen); AND
• Both of the following:
  • Patient is not receiving concomitant treatment with Tumor Necrosis Factor (TNF) inhibitors (e.g., Enbrel [etanercept], Humira [adalimumab], Remicade [infliximab]) 
  • Patient is not receiving concomitant treatment with Interleukin-1 inhibitor (e.g., Arcalyst [rilonacept], Kineret [anakinra])

Reauthorization Criteria:

For diagnosis of Periodic Fever Syndromes or Still’s Disease:

• Documentation of positive clinical response to therapy; AND
• Both of the following:
  • Patient is not receiving concomitant treatment with Tumor Necrosis Factor (TNF) inhibitors (e.g., Enbrel [etanercept], Humira [adalimumab], Remicade [infliximab]) 
  • Patient is not receiving concomitant treatment with Interleukin-1 inhibitor (e.g., Arcalyst [rilonacept], Kineret [anakinra])

For diagnosis of SJIA:

• Documentation of positive clinical response to therapy as evidenced by at least one of the following: 
  • Reduction in the total active (swollen and tender) joint count from baseline 
  • Improvement in clinical features or symptoms (e.g., pain, fever, inflammation, rash, lymphadenopathy, serositis) from baseline; AND
• Both of the following:
  • Patient is not receiving concomitant treatment with Tumor Necrosis Factor (TNF) inhibitors (e.g., Enbrel [etanercept], Humira [adalimumab], Remicade [infliximab]) 
  • Patient is not receiving concomitant treatment with Interleukin-1 inhibitor (e.g., Arcalyst [rilonacept], Kineret [anakinra])

Dosing:

Periodic Fever Syndromes: CAPS including FCAS, MWS (adults and pediatrics 4 years of age or older):

• 150 mg for CAPS patients with body weight greater than 40 kg and 2 mg/kg for CAPS patients with body weight greater than or equal to 15 kg and less than or equal to 40 kg. For children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg. Administer subcutaneously every 8 weeks.

TRAPS, HIDS/MKD, and FMF (adults and pediatrics):

• Body weight less than or equal to 40 kg: starting dose is 2 mg/kg every 4 weeks. The dose can be increased to 4 mg/kg every 4 weeks if the clinical response is not adequate.
• Body weight greater than 40 kg: starting dose is 150 mg every 4 weeks. The dose can be increased to 300 mg every 4 weeks if the clinical response is not adequate. 

Still’s disease (including AOSC); SJIA (adults and pediatrics 2 years of age or older):

• 4 mg/kg (with a maximum of 300 mg) for patients with a body weight greater than or equal to 7.5 kg. Administer subcutaneously every 4 weeks.

Coverage Duration:

Still’s disease (including AOSC), SJIA:

• Initial: 1 year
• Reauthorization: 1 year

For all other indications (CAPS, FCAS, MWS, TRAPS, HIDS/MKD, FMF):

• Initial: 6 months 
• Reauthorization: 1 year

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information:

• Ilaris use has been associated with serious infections. Contact a healthcare professional immediately if an infection develops after starting Ilaris.
• Healthcare providers should perform administration of ILARIS by the subcutaneous injection route.
• Do not to take Ilaris if you are also taking a drug that blocks TNF, such as Enbrel (etanercept), Remicade (infliximab), or Humira (adalimumab).

Policy Updates:

• 08/18/2020 – Criteria reviewed.
• 11/15/2022 – Revised criteria for periodic fever syndromes, SJIA, and Still’s disease; remove TB testing requirements; created reauthorization criteria; updated initial authorization duration.

References:

1. Ilaris Prescribing Information. Novartis Pharmaceuticals Corporation. East Hanover, NJ. September 2020. 
2. Onel KB, Horton DB, Lovell DJ, et al. 2021 American College of Rheumatology guideline for the treatment of juvenile idiopathic arthritis: therapeutic approaches for oligoarthritis, temporomandibular joint arthritis, and systemic juvenile idiopathic arthritis. Arthritis Rheumatol. 2022;74(4):553-569. 
3. Mimura T, Kondo Y, Ohta A et al. Evidence-based clinical practice guideline for adult Still’s disease. Mod Rheumatol. 2018;28(5):736-757. 
4. Lachmann HJ, Kone-Paut I, Kuemmerle-Deschner JB, et al. Use of canakinumab in the cryopyrin-associated periodic syndrome. N Engl J Med. 2009;360(23):2416-25. 
5. Aksentijevich I, Putnam CD, Remmers EF, et al. Clinical continuum of cryopyrinopathies: novel CIAS1 mutations in North-American patients and a new cryopyrin model. Arthritis Rheum. 2007;56(4):1273-85.