ARCALYST (rilonacept)

ARCALYST (rilonacept)

Self-Administration – subcutaneous injection

Diagnosis considered for coverage:

• Cryopyrin-Associated Periodic Syndromes (CAPS): Indicated for the treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in adults and pediatric patients 12 years and older.
• Deficiency of Interleukin-1 Receptor Antagonist (DIRA): Indicated for the maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and pediatric patients weighing at least 10 kg.
• Recurrent Pericarditis: Indicated for the treatment of recurrent pericarditis and reduction in risk of recurrence in adults and pediatric patients 12 years and older.

Coverage Criteria:

For diagnosis of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory Syndrome (FCAS) and/or Muckle-Wells Syndrome (MWS):

• Documented diagnosis of CAPS including FCAS or MWS; AND
• Prescribed by or in consultation with one of the following:
  • Immunologist
  • Allergist
  • Dermatologist
  • Rheumatologist
  • Neurologist
  • Specialist with expertise in the management of CAPS; AND
• The medication will not be used in combination with another biologic agent.

For diagnosis of Deficiency of Interleukin-1 Receptor Antagonist (DIRA):

• Documented diagnosis of DIRA; AND
• Patient weighs at least 10 kg; AND
• Patient is currently in remission (e.g., no fever, skin rash, and bone pain; no radiological evidence of active bone lesions; C-reactive protein [CRP] less than 5 mg/L).

For diagnosis of Recurrent Pericarditis:

• Diagnosis of recurrent pericarditis as evidenced by at least 2 episodes that occur a minimum of 4 to 6 weeks apart; AND
• Prescribed by or in consultation with a cardiologist; AND
• Trial and failure, contraindication, or intolerance to at least one of the following:
  • nonsteroidal anti-inflammatory drugs (e.g., ibuprofen, naproxen)
  • colchicine
  • corticosteroids (e.g., prednisone).

Reauthorization Criteria:

For diagnosis of CAPS including FCAS or MWS:

• Patient has experienced disease stability or improvement in clinical symptoms while on therapy as evidenced by one of the following:
  • Improvement in rash, fever, joint pain, headache, or conjunctivitis
  • Decreased number of disease flare days
  • Normalization of inflammatory markers (C-reactive protein [CRP], erythrocyte sedimentation rate [ESR], serum amyloid A [SAA])
  • Corticosteroid dose reduction
  • Improvement in MD global score or active joint count

For diagnosis of Recurrent Pericarditis:

• Documentation of positive clinical response to therapy

Dosing:

CAPS/FCAS/MWS; Recurrent Pericarditis:

• Adults: Initiate treatment with a loading dose of 320 mg delivered as two, 2-mL subcutaneous injections of 160 mg each, administered on the same day at two different injection sites. Continue dosing with a once-weekly injection of 160 mg administered as a single, 2-mL subcutaneous injection.
• Pediatrics (12-17yr old): Initiate treatment with a loading dose of 4.4 mg/kg, up to a maximum dose of 320 mg, administered as one or two subcutaneous injections, not to exceed single-injection volume of 2 mL per injection site. If the initial dose is given as two injections, administer on the same day at two different sites. Continue dosing with a once-weekly injection of 2.2 mg/kg, up to a maximum of 160 mg, administered as a single subcutaneous injection, up to 2 mL.
• If a once-weekly dose is missed, instruct the patient to administer the injection within 7 days from the missed dose and then resume the patient’s original schedule. If the missed dose is not administered within 7 days, instruct the patient to administer the dose, starting a new schedule based on this date.

DIRA:

• Adults: The recommended dose of ARCALYST is 320 mg, once weekly, administered as two subcutaneous injections on the same day at two different sites with a maximum single-injection volume of 2 mL. ARCALYST should not be given more often than once weekly.
• Pediatrics (weighing 10kg or more): The recommended dose of ARCALYST is 4.4 mg/kg (up to a maximum of 320 mg), once weekly, administered as one or two subcutaneous injections with a maximum single-injection volume of 2 mL. If the dose is given as two injections, administer both on the same day, each one at a different site.
• When switching from another IL-1 blocker (e.g., Kineret, Ilaris), discontinue the IL-1 blocker and begin ARCALYST treatment at the time of the next dose.

Coverage Duration:

• 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:

• CAPS refer to rare genetic syndromes generally caused by mutations in the NLRP-3 [Nucleotide-binding domain, leucine rich family (NLR), pyrin domain containing 3] gene (also known as Cold-Induced Auto-inflammatory Syndrome-1 [CIAS1]). CAPS disorders are inherited in an autosomal dominant pattern with male and female offspring equally affected. Features common to all disorders include fever, urticaria-like rash, arthralgia, myalgia, fatigue, and conjunctivitis. In most cases, inflammation in CAPS is associated with mutations in the NLRP-3 gene which encodes the protein cryopyrin, an important component of the inflammasome. Mutations in NLRP-3 result in an overactive inflammasome resulting in excessive release of activated IL-1β that drives inflammation.

Policy Review History:

• 06/01/2023 – New utilization management program for established drug approved by P&T (05/16/2023).

References:

1. Arcalyst Prescribing Information. Regeneron Pharmaceuticals. Zug, Switzerland. May 2021.
2. Aksentijevich I, Putnam CD, Remmers EF, et al. The clinical continuum of cryopyrinopathies: novel CIAS1 mutations in North American Patients and a new cryopyrin model. Arthritis Rheum. 2007; 56(4):1273-1285.
3. McDermott M, Aksentijevich I. The auto-inflammatory syndromes. Curr Opin Allergy Clin Immunol. 2002; 2:511-516.
4. Chiabrando JG, Bonaventura A, Vecchie A, et al. Management of acute and recurrent pericarditis. J Am Coll Cardiol. 2020;75(1):76–92.
5. Klein AL, Imazio M, Cremer P, et al. Phase 3 trial of interleukin-1 trap rilonacept in recurrent pericarditis. N Engl J Med 2021;384:31-41.