Subject: Arcalyst 1800-A SGM P2022
Policy:
I. INDICATIONS
The indications below including FDA-approved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to the prescribed therapy.
FDA-Approved Indications
Arcalyst is indicated for:
A. Treatment of Cryopyrin Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory
Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in adults and children 12 years of age and older.
B. Maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and pediatric
patients weighing at least 10 kg.
C. Treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in adults and pediatric patients
12 years and older.
All other indications are considered experimental/investigational and not medically necessary.
II. DOCUMENTATION
Submission of the following information is necessary to initiate the prior authorization review:
A. Deficiency of interleukin-1 receptor antagonist (DIRA) initial requests: IL1RN mutation status
B. Recurrent pericarditis:
1. Initial requests: Chart notes, medical record documentation, or claims history supporting previous
medications tried, including response to therapy.
2. Continuation requests: Chart notes or medical record documentation supporting positive clinical response.
III. CRITERIA FOR INITIAL APPROVAL
A. Cryopyrin-associated periodic syndrome (CAPS)
Authorization of 12 months may be granted for treatment of CAPS when all of the following
criteria are met:
1. Member has a diagnosis of familial cold auto-inflammatory syndrome (FCAS) with classic signs
and symptoms (i.e., recurrent, intermittent fever and rash that were often exacerbated by
exposure to generalized cool ambient temperature) or Muckle-Wells syndrome (MWS) with classic
signs and symptoms (i.e., chronic fever and rash of waxing and waning intensity, sometimes
exacerbated by exposure to generalized cool ambient temperature).
2. Member has functional impairment limiting the activities of daily living.
B. Deficiency of interleukin-1 receptor antagonist (DIRA)
Authorization of 12 months may be granted for treatment of DIRA when all of the following
criteria are met:
1. Member has loss-of-function IL1RN mutations.
2. Arcalyst will be used for maintenance of remission following treatment with Kineret (anakinra).
C. Recurrent pericarditis
Authorization of 12 months may be granted for treatment of recurrent pericarditis when both of
the following criteria are met:
1. Member has had at least two episodes of pericarditis.
2. Member has failed at least 2 agents of standard therapy (e.g., colchicine, non-steroidal
anti-inflammatory drugs (NSAIDs), corticosteroids).
IV. CONTINUATION OF THERAPY
A. Recurrent pericarditis
Authorization of 12 months may be granted for all members (including new members) who
are using the requested medication for recurrent pericarditis and who achieve or maintain a positive
clinical response as evidenced by decreased recurrence of pericarditis or improvement in signs and
symptoms of the condition when there is improvement in any of the following:
1. Pericarditic chest pain
2. Pericardial rubs
3. Electrocardiogram (ECG)
4. Pericardial effusion
5. C-reactive protein (CRP)
B. All other indications
Authorization of 12 months may be granted for all members (including new members) who are using
the requested medication for an indication outlined in Section III and who achieve or maintain positive
clinical response as evidenced by low disease activity or improvement in signs and symptoms of the condition.
V. OTHER
For all indications: Member has had a documented negative TB test (which can include a tuberculosis skin test [PPD], an interferon-release assay [IGRA], or a chest x-ray)* within 6 months of initiating therapy for persons who are naïve to biologic DMARDs or targeted synthetic DMARDs associated with an increased risk of TB.
* If the screening testing for TB is positive, there must be further testing to confirm there is no active disease. Do not administer the requested medication to members with active TB infection. If there is latent disease, TB treatment must be started before initiation of the requested medication.
For all indications: Member cannot use the requested medication concomitantly with any other biologic DMARD or targeted synthetic DMARD.
Place of Service:
Outpatient
The above policy is based on the following references:
- Arcalyst [package insert]. London, UK: Kiniksa Pharmaceuticals (UK), Ltd.; May 2021.
- Hoffman HM, Throne ML, Amar NJ, et al. Efficacy and safety of rilonacept (interleukin-1 trap) in patients with cryopyrin-associated periodic syndromes. Results from two sequential placebo-controlled studies. Arthritis Rheum. 2008;58(8):2443-52.
- Centers for Disease Control and Prevention. Tuberculosis (TB). TB risk factors. Available at: https://www.cdc.gov/tb/topic/basics/risk.htm. Accessed: November 15, 2021.
- Adler Y, Charron P, Imazio M, et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC) Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. 2015 Nov 7;36(42):2921-64.
- Chiabrando JG, Bonaventura A, Vecchié, et al. Management of acute and recurrent pericarditis: JACC State-of-the-art review. 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(1):31-41.
Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.
June 26, 2022
