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Prior Authorization Protocol
ARCALYSTTM (rilonacept), ILARISR (canakinumab)

NATL
Coverage of drugs is first determined by the member’s pharmacy or medical benefit. Please consult with or refer to the Evidence of Coverage document.
  1. FDA Approved Indications:
    • Arcalyst: Treatment of Cryopyrin-Associated Periodic Syndromes (CAPS) including Familial Cold Autoinflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in adults and children 12 years and older
    • Ilaris:
      • Treatment of Cryopyrin-Associated Periodic Syndromes (CAPS) including Familial Cold Autoinflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in adults and children 4 years and older
      • Active Systemic Juvenile Idiopathic Arthritis (SJIA) in patients aged 2 years and older
      • Treatment of tumor necrosis factor receptor associated periodic syndrome (TRAPS) in adults and pediatric patients
      • Treatment of hyperimmunoglobulin D syndrome (HIDS)/mevalonate kinase deficiency (MKD) in adult and pediatric patients
      • Treatment of familial Mediterranean fever (FMF) in adult and pediatric patients
  2. Health Net Approved Indications and Usage Guidelines:
    • Diagnosis of one Cryopyrin-Associated Periodic Syndromes (CAPS) disorder confirmed by a rheumatologist or other physician experienced in the management of CAPS:
      • Familial Cold Autoinflammatory Syndrome
    OR
      • Muckle-Wells Syndrome

    OR

    • For Ilaris only, diagnosis of one of the following indications confirmed by a rheumatologist or other physician experienced in the management of the disease:
      • Systemic Juvenile Idiopathic Arthritis

      OR

      • Tumor necrosis factor receptor associated periodic syndrome (TRAPS)

      OR

      • Hyperimmunoglobulin D syndrome/mevalonate kinase deficiency (HIDS/MKD)

      OR

      • Familial Mediterranean fever (FMF)

      AND

        • Failure of or clinically significant adverse effects to colchicine trialed at maximum indicated doses for ≥ 6 months, unless contraindicated or age < 4 years
  3. Coverage is Not Authorized For:
    • Non-FDA approved indications, which are not listed in the Health Net Approved Indications and Usage Guidelines section, unless there is sufficient documentation of efficacy and safety in the published literature.
  4. General Information:
    • Periodic fever syndromes are a group of rare autoinflammatory diseases that include cryopyrin-associated periodic syndromes (CAPS), tumor necrosis factor receptor associated periodic syndrome (TRAPS), hyperimmunoglobulin D syndrome (HIDS)/mevalonate kinase deficiency (MKD), and familial Mediterranean fever (FMF). Diagnosis of these diseases can be confirmed by genetic testing.
    • Three related conditions make up the broader disease known as CAPS: Familial Cold Auto-inflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS), and Neonatal-Onset Multisystem Inflammatory Disease (NOMID), also known as Chronic Infantile Neurologic Cutaneous Articular Syndrome (CINCA). While Arcalyst and Ilaris are FDA-approved for FCAS and MWS, neither are FDA-approved for use in patients with NOMID/CINCA
    • Arcalyst is not FDA-approved for use in patients with TRAPS, HIDS/MKD, and FMF. Ilaris, on the other hand, is FDA-approved for these patients based on rapid and sustained disease control produced in a 16-week randomized control trial. Additional studies with Ilaris in these diseases are currently ongoing, including a 24-week randomized withdrawal period and a 72-week open-label treatment period. Ilaris is the first therapeutic option for TRAPS and HIDS/MKD and the first biologic option for FMF. In FMF, the current standard of care is colchicine, a relatively safe oral therapy indicated in patients ages 4 and up. Colchicine has well-established benefit in FMF and has been used for decades. Although no US clinical practice guidelines exist for TRAPS, HIDS/MKD, and FMF, the European League Against Rheumatism (EULAR) guidelines for the management of FMF recommend colchicine be initiated at diagnosis for all patients and response to therapy be assessed every 6 months.
    • Pediatric American College of Rheumatology (ACR) responses are defined by achieving levels of percentage improvement (30%, 50%, and 70%) from baseline in at least 3 of the 6 core outcome variables, with worsening of greater than or equal to 30% in no more than one of the remaining variables. Core outcome variables included a physician global assessment of disease activity, parent or patient global assessment of wellbeing, number of joints with active arthritis, number of joints with limited range of motion, C-Reactive Protein (CRP), and functional ability (Childhood Health Assessment Questionnaire - CHAQ).
    • Arcalyst and Ilaris are interleukin-1 blockers and have the potential to increase the risk of infection and reactivate latent, chronic infections. Healthcare providers should follow current CDC guidelines to evaluate and to treat possible latent tuberculosis infections before initiating therapy. Prior to initiation of therapy, it is highly recommended that all patients be immunized due to the increased risk of infection.
    • Concomitant administration of Arcalyst or Ilaris with TNF inhibitors (such as EnbrelR, HumiraR, or RemicadeR) and IL-1 blocking agents (such as KineretR) is not recommended because this may increase the risk of serious infections.
  5. Therapeutic Alternatives:
    Drug Dosing Regimen Dose Limit/ Maximum Dose

    colchicine (Colcrys)

    Familial Mediterranean Fever
    PO in 1-2 divided doses based on age:
    >12 years: 1.2-2.4 mg/day
    6-12 years: 0.9-1.8 mg/day
    4-6 years: 0.3-1.8 mg/day

    2.4 mg/day

    * Requires Prior Authorization
  6. Recommended Dosing Regimen and Authorization Limit:
    Drug Dosing Regimen Authorization Limit

    Arcalyst

    18 yrs and older:
    320 mg SC loading dose followed by 160 mg SC once weekly
    12 to 17 yrs:
    4.4 mg/kg SC loading dose (maximum 320 mg) followed
    by 2.2 mg/kg (maximum of 160 mg) SC once weekly

    Four week initial trial.
    Continued treatment will be approved upon receiving written
    documentation of clinical response by week 4 (normalization of CRP).
    Ongoing treatment will be approved up to one year subject to benefit.

    Ilaris

    Cryopyrin-Associated Periodic Syndromes
    (Not for self-injection)
    Adults > 40 kg:
    150 mg SC q8 weeks
    Children 15 and 40 kg:
    2 mg/kg SC q8 weeks, may increase to 3 mg/kg based on response

    Systemic Juvenile Idiopathic Arthritis
    (Not for self-injection)
    4 mg/kg SC (with a maximum of 300 mg)
    for patients with a body weight greater than or
    equal to 7.5kg. Administer SC q4 weeks.
    Cryopyrin-Associated Periodic Syndromes
    Single dose initial trial over 8 weeks.
    Continued treatment will be approved upon receiving written documentation of clinical response by week 8 (normalization of CRP). 
    Ongoing treatment will be approved up to one year subject to benefit.
    Systemic Juvenile Idiopathic Arthritis
    Four week initial trial.
    Continued treatment will be approved upon receiving documentation of response to therapy using quantitative measures (e.g., physician global assessment of disease activity, parent or patient global assessment of wellbeing, number of joints with active arthritis, number of joints with limited range of motion, CRP, and functional ability [Childhood Health Assessment Questionnaire - CHAQ]) by week
    Ongoing treatment will be approved up to one year subject to benefit.
    Ilaris

    Tumor Necrosis Factor Receptor Associated Periodic Syndrome, Hyperimmunoglobulin D Syndrome /Mevalonate Kinase Deficiency, and Familial Mediterranean Fever
    (Not for self-injection)

    Body weight > 40 kg: 150 mg SC q4 weeks (may be increased to 300 mg if inadequate response)

    Body weight ≤ 40 kg: 2 mg/kg SC q4 weeks (may be increased to 4 mg/kg if inadequate response)

    Four week initial trial.

    Continued treatment will be approved upon receiving documentation of clinical response (e.g., reduction/normalization of CRP or SAA; reduction of flare frequency, symptom severity, or duration). Members who fail to demonstrate positive clinical response may request a dose increase, which will be approved upon receiving documentation of inadequate response to current dose. Ongoing treatment will be approved up to six months subject to benefit.

  7. Product Availability:
    Arcalyst: 220 mg vial as rilonacept lyophilized powder for reconstitution (the reconstituted solution: 80 mg/ml, 2 ml); each vial delivers 160 mg
    Ilaris: 150 mg vial as canakinumab lyophilized powder for reconstitution (the reconstituted solution: 150 mg/ml, 1.2 ml); each vial delivers 150 mg
  8. References:
    1. Arcalyst [package insert]. Tarrytown, NY: Regeneron Pharmaceuticals; September 2014.
    2. Ilaris [package insert]. East Hanover, NJ: Novartis Pharmaceuticals; September 2016.
    3. Hoffman, HM, Throne ML, Amar NJ, et al. Efficacy and safety of rilonacept (Interleuckin-1 Trap) in patients with cryopyrin-associated periodic syndromes. Arthritis and Rheumatism. 2008;58(8): 2443-2452.
    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;360:2416-2425.
    5. DRUGDEXR System [Internet database]. Greenwood Village, Colo: Thomson Healthcare. Updated periodically. http://www.thomsonhc.com. Accessed May 24, 2016.
    6. Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016; 75(4): 644-651.

The material provided to you are guidelines used by this plan to authorize, modify or determine coverage for persons with similar illnesses or conditions. Specific care and treatment may vary depending on individual need and the benefits covered under your contract.