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:
- Arcalyst Prescribing Information. Regeneron Pharmaceuticals. Zug, Switzerland. May 2021.
- 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.
- McDermott M, Aksentijevich I. The auto-inflammatory syndromes. Curr Opin Allergy Clin Immunol. 2002; 2:511-516.
- Chiabrando JG, Bonaventura A, Vecchie A, et al. Management of acute and recurrent pericarditis. J Am Coll Cardiol. 2020;75(1):76–92.
- 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.
Last review date: June 1, 2023