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Interleukin (IL)-1 Inhibitors Prior Authorization with Quantity Limit Program Summary
Policy Number: PH-1051
This program applies to Blue Partner, Commercial, GenPlus, NetResults A series, SourceRx and Health Insurance Marketplace formularies.
POLICY REVIEW CYCLE
Effective Date |
Date of Origin |
07-01-2024 |
|
FDA LABELED INDICATIONS AND DOSAGE
Agent(s) |
FDA Indication(s) |
Notes |
Ref# |
Arcalyst® (rilonacept) Subcutaneous injection |
Treatment of Cryopyrin Associated Periodic Syndrome (CAPS), including Familial Cold Auto-Inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in adults and children 12 years and older Maintenance of remission of deficiency of interleukin-1 receptor antagonist (DIRA) in adults and pediatric patients weighing at least 10 kg Treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in adults and pediatric patients 12 years and older |
|
1 |
See package insert for FDA prescribing information: https://dailymed.nlm.nih.gov/dailymed/index.cfm
CLINICAL RATIONALE
Cryopyrin-Associated Periodic Syndromes (CAPS) |
Cryopyrin-associated periodic syndrome (CAPS) is a rare autosomal dominant hereditary autoimmune disorder associated with a defect in the cryopyrin protein. CAPS syndrome is caused by a gain of function mutation in the NLRP3 gene leading to over secretion of fever causing cytokine IL-1B. The CAPS spectrum includes mild, moderate, and severe phenotypes. The mild phenotype is called familial cold autoinflammatory syndrome (FCAS), the moderate phenotype is also known as Muckle-Wells syndrome (MWS), the neonatal-onset multisystem inflammatory disease (NOMID)/chronic infantile neurologic cutaneous articular syndrome (CINCA) describes the severe phenotype. CAPS is diagnosed clinically and genetically. There are more than 240 sequence variants of the NLRP3 gene and mutations in this gene are not inclusive of a CAPS diagnosis. The diagnostic criteria of CAPS recognize that all but a few patients with CAPS have detectable systemic inflammation and use unique CPS-specific clinical features along the whole disease spectrum to achieve reasonable specificity and sensitivity to aid clinicians in making the CAPS diagnosis. These diagnostic criteria do not include genetic confirmation, and therefore can be applied in places where genetic testing is not available. The diagnostic criteria for CAPS are as follows:(6)
FCAS is characterized by episodes of rash, fever. and joint pain following generalized exposure to cold. Attacks usually occur 1-2 hours after exposure and last less than 24.(2) Patients experience urticaria, arthralgia, fever with chills, severe thirst, red-eyes, and headache after a general cold exposure, including air conditioning. In MWS, inflammation can occur spontaneously as well as from triggers, such as stress, cold, or exercise, with episodes lasting from one to three days. MWS shares the same characteristics as FCAS, but is also characterized by renal amyloidosis, sensorineural hearing loss, and conjunctivitis. Hearing loss, partial or complete, often develop by teenage years.(3) NOMID is a rare chronic inflammatory disease. NOMID is characterized by fever, urticarial rash, aseptic meningitis, deforming arthropathy, hearing loss, and intellectual disability. An urticaria-like rash develops within the first six weeks of life, and a characteristic bony overgrowth predominantly involving the knees develops in most affected children. Therapies are aimed at suppressing inflammation and have included high-dose corticosteroids, disease-modifying antirheumatic drugs, and biologic agent targeting tumor necrosis factor (TNF). Selective blockade of interleukin-1B is effective in the pathophysiology and organ-specific manifestations of NMOSD, in particular the CNS manifestations of the disease.(5) Treatment aims are to suppress systemic inflammation, to improve functionality, to prevent organ damage, and to increase patients' quality of life. To achieve these aims, cytokine targeting drugs are important and evidence-based treatment. Since IL-1 plays a central role in CAPS pathogenesis, the anti-IL1 treatments (anakinra, canakinumab, and rilonacept) are recommended for the whole CAPS spectrum.(6) |
Deficiency of the IL-1 Receptor Antagonist (DIRA) |
Systemic autoinflammatory diseases (SAIDs) are a group of multisystem immunodysregulatory disorders caused primarily by the dysfunction of the innate immune system. Currently, SAIDs are comprised of a wide range of disorders with systemic and organ-specific inflammation in the absence of infections or autoimmunity. In a subset of genetically defined SAIDs, the pathogenesis is driven by increased release or signaling of the pro-inflammatory cytokine IL-1.(7) Patients with DIRA present with early-onset pustular rashes that can be triggered by mechanical stress (pathergy), with sterile osteomyelitis, and nail changes (onychomadesis). Although inflammatory markers are typically highly elevated, fever may be absent. Vertebral involvement can include odontoid osteomyelitis resulting in destruction and neck instability, vertebral block formation and gibbus-like spinal changes that need to be screened for by MRI or CT. The differential diagnosis for DIRA includes chronic recurrent multifocal osteomyelitis (CRMO), synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome and pustular psoriasis. Genetic testing for monogenic defects with overlapping clinical features should include LPIN2, FGR, FBLIM1 for CRMO, CARD14 for CARD14-Mediated Psoriasis (CAMPS), IL36RN for Deficiency of IL-36 Receptor Antagonist (DITRA), AP1S3 for other pustular psoriasis and MEFV for Pyrin-Associated Autoinflammation with Neutrophilic Dermatosis (PAAND).(7) Aims of therapy are early control of disease activity, prevention of disease and treatment related damage, and optimal health-related quality of life. The ultimate goal of a treat-to-target approach is complete remission. In absence of a consensus definition of remission or minimal disease activity for these diseases, remission has been defined for clinical studies and clinical monitoring as an absence of clinical symptoms and normal inflammatory markers. Anakinra and rilonacept both block IL-1α and IL-1β and should be used for DIRA patients.(7) |
Recurrent Pericarditis |
Pericarditis is inflammation of the pericardial layers around the heart and is the most common form of pericardial disease. Pericarditis may be caused infections, post-cardiac injury syndrome, or pericarditis may be idiopathic. Pericarditis is categorized into four types, acute, incessant, recurrent, and chronic. These categories are based on the length of time of the attack and the presentation. Acute pericarditis is an event lasting 4 weeks or less, incessant is an event lasting more than 4 weeks without a remission, recurrent pericarditis is new signs and symptoms of pericarditis after a symptom-free interval of 4 to 6 weeks, and chronic is an event lasting more than 3 months. Roughly 20% to 30% of patients that develop acute pericarditis will have recurrences, and 50% of patients that have a recurrence will experience more recurrences.(9) The treatment algorithm for therapeutic management of patients with recurrent pericarditis is as follows:(10)
The American College of Cardiology note that mycophenolate mofetil and methotrexate have also been shown to be effective in the treatment of recurrent pericarditis that are not responsive to corticosteroids, corticosteroid dependent, or intolerant to corticosteroids.(9) |
Safety |
Arcalyst does not have any FDA labeled contraindications.(1) |
REFERENCES
Number |
Reference |
1 |
Arcalyst prescribing information. Kiniksa Pharmaceuticals (UK), Ltd. May 2021. |
2 |
Hoffman HM, Wanderer AA, Broide DH. Familial cold autoinflammatory syndrome: phenotype and genotype of an autosomal dominant periodic fever. J Allergy Clin Immunol. 2001 Oct;108(4):615-20. doi: 10.1067/mai.2001.118790. PMID: 11590390; PMCID: PMC4321996. |
3 |
Yu, J. R., & Leslie, K. S. (2010). Cryopyrin-associated periodic syndrome: an update on diagnosis and treatment response. Current allergy and asthma reports, 11(1), 12-20. |
4 |
Reference no longer used. |
5 |
Goldbach-Mansky R, Dailey NJ, Canna SW, Gelabert A, Jones J, Rubin BI, Kim HJ, Brewer C, Zalewski C, Wiggs E, Hill S, Turner ML, Karp BI, Aksentijevich I, Pucino F, Penzak SR, Haverkamp MH, Stein L, Adams BS, Moore TL, Fuhlbrigge RC, Shaham B, Jarvis JN, O'Neil K, Vehe RK, Beitz LO, Gardner G, Hannan WP, Warren RW, Horn W, Cole JL, Paul SM, Hawkins PN, Pham TH, Snyder C, Wesley RA, Hoffmann SC, Holland SM, Butman JA, Kastner DL. Neonatal-onset multisystem inflammatory disease responsive to interleukin-1beta inhibition. N Engl J Med. 2006 Aug 10;355(6):581-92. doi: 10.1056/NEJMoa055137. PMID: 16899778; PMCID: PMC4178954. |
6 |
Welzel T, Kuemmerle-Deschner JB. Diagnosis and Management of the Cryopyrin-Associated Periodic Syndromes (CAPS): What Do We Know Today? J Clin Med. 2021 Jan 1;10(1):128. doi: 10.3390/jcm10010128. PMID: 33401496; PMCID: PMC7794776. |
7 |
Romano M, Arici ZS, Piskin D, Alehashemi S, Aletaha D, Barron K, Benseler S, Berard RA, Broderick L, Dedeoglu F, Diebold M, Durrant K, Ferguson P, Foell D, Hausmann JS, Jones OY, Kastner D, Lachmann HJ, Laxer RM, Rivera D, Ruperto N, Simon A, Twilt M, Frenkel J, Hoffman HM, de Jesus AA, Kuemmerle-Deschner JB, Ozen S, Gattorno M, Goldbach-Mansky R, Demirkaya E. The 2021 EULAR/American College of Rheumatology Points to Consider for Diagnosis, Management and Monitoring of the Interleukin-1 Mediated Autoinflammatory Diseases: Cryopyrin-Associated Periodic Syndromes, Tumour Necrosis Factor Receptor-Associated Periodic Syndrome, Mevalonate Kinase Deficiency, and Deficiency of the Interleukin-1 Receptor Antagonist. Arthritis Rheumatol. 2022 Jul;74(7):1102-1121. doi: 10.1002/art.42139. Epub 2022 May 27. PMID: 35621220; PMCID: PMC9531906. |
8 |
Reference no longer used |
9 |
Chiabrando JG, Bonaventura A, Vecchié A, et al. Management of Acute and Recurrent Pericarditis: JACC State-of-the-Art Review. J Am Coll Cardiol 2020; 75:76. |
10 |
Andreis A, Imazio M, Casula M, Avondo S, Brucato A. Recurrent pericarditis: an update on diagnosis and management. Intern Emerg Med. 2021 Apr;16(3):551-558. doi: 10.1007/s11739-021-02639-6. Epub 2021 Feb 28. PMID: 33641044; PMCID: PMC7914388. |
POLICY AGENT SUMMARY PRIOR AUTHORIZATION
Target Brand Agent(s) |
Target Generic Agent(s) |
Strength |
Targeted MSC |
Available MSC |
Final Age Limit |
Preferred Status |
|
||||||
Arcalyst |
Rilonacept For Inj 220 MG |
220 MG |
M ; N ; O ; Y |
N |
|
|
POLICY AGENT SUMMARY QUANTITY LIMIT
Target Brand Agent Name(s) |
Target Generic Agent Name(s) |
Strength |
QL Amount |
Dose Form |
Day Supply |
Duration |
Addtl QL Info |
Allowed Exceptions |
Targeted NDCs When Exclusions Exist |
|
|||||||||
Arcalyst |
Rilonacept For Inj 220 MG |
220 MG |
8 |
Vials |
28 |
DAYS |
|
|
|
CLIENT SUMMARY – PRIOR AUTHORIZATION
Target Brand Agent Name(s) |
Target Generic Agent Name(s) |
Strength |
Client Formulary |
Arcalyst |
Rilonacept For Inj 220 MG |
220 MG |
Blue Partner ; Commercial ; GenPlus ; Health Insurance Marketplace ; NetResults A Series ; SourceRx |
CLIENT SUMMARY – QUANTITY LIMITS
Target Brand Agent Name(s) |
Target Generic Agent Name(s) |
Strength |
Client Formulary |
Arcalyst |
Rilonacept For Inj 220 MG |
220 MG |
Blue Partner ; Commercial ; GenPlus ; Health Insurance Marketplace ; NetResults A Series ; SourceRx |
PRIOR AUTHORIZATION CLINICAL CRITERIA FOR APPROVAL
Module |
Clinical Criteria for Approval |
||
|
Initial Evaluation Target Agent(s) will be approved when ALL of the following are met:
Compendia Allowed: AHFS or DrugDex 1 or 2a level of evidence Length of Approval: 12 months NOTE: If Quantity Limit applies, please refer to Quantity Limit Criteria.
Renewal Evaluation Target Agent(s) will be approved when ALL of the following are met:
Length of Approval: 12 months NOTE: If Quantity Limit applies, please refer to Quantity Limit Criteria. |
QUANTITY LIMIT CLINICAL CRITERIA FOR APPROVAL
Module |
Clinical Criteria for Approval |
|
Quantity Limit for the Target Agent(s) will be approved when ONE of the following is met:
Length of Approval: up to 12 months |
CONTRAINDICATION AGENTS
Contraindicated as Concomitant Therapy |
Agents NOT to be used Concomitantly Abrilada (adalimumab-afzb) Actemra (tocilizumab) Adalimumab Adbry (tralokinumab-ldrm) Amjevita (adalimumab-atto) Arcalyst (rilonacept) Avsola (infliximab-axxq) Benlysta (belimumab) Bimzelx (bimekizumab-bkzx) Cibinqo (abrocitinib) Cimzia (certolizumab) Cinqair (reslizumab) Cosentyx (secukinumab) Cyltezo (adalimumab-adbm) Dupixent (dupilumab) Enbrel (etanercept) Entyvio (vedolizumab) Fasenra (benralizumab) Hadlima (adalimumab-bwwd) Hulio (adalimumab-fkjp) Humira (adalimumab) Hyrimoz (adalimumab-adaz) Idacio (adalimumab-aacf) Ilaris (canakinumab) Ilumya (tildrakizumab-asmn) Inflectra (infliximab-dyyb) Infliximab Kevzara (sarilumab) Kineret (anakinra) Litfulo (ritlecitinib) Nucala (mepolizumab) Olumiant (baricitinib) Omvoh (mirikizumab-mrkz) Opzelura (ruxolitinib) Orencia (abatacept) Otezla (apremilast) Remicade (infliximab) Renflexis (infliximab-abda) Riabni (rituximab-arrx) Rinvoq (upadacitinib) Rituxan (rituximab) Rituxan Hycela (rituximab/hyaluronidase human) Ruxience (rituximab-pvvr) Siliq (brodalumab) Simponi (golimumab) Simponi ARIA (golimumab) Skyrizi (risankizumab-rzaa) Sotyktu (deucravacitinib) Stelara (ustekinumab) Taltz (ixekizumab) Tezspire (tezepelumab-ekko) Tremfya (guselkumab) Truxima (rituximab-abbs) Tysabri (natalizumab) Velsipity (etrasimod) Wezlana (ustekinumab-auub) Xeljanz (tofacitinib) Xeljanz XR (tofacitinib extended release) Xolair (omalizumab) Yuflyma (adalimumab-aaty) Yusimry (adalimumab-aqvh) Zeposia (ozanimod) Zymfentra (infliximab-dyyb) |
This pharmacy policy is not an authorization, certification, explanation of benefits or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All pharmacy policies are based on (i) information in FDA approved package inserts (and black box warning, alerts, or other information disseminated by the FDA as applicable); (ii) research of current medical and pharmacy literature; and/or (iii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.
The purpose of Blue Cross and Blue Shield of Alabama’s pharmacy policies are to provide a guide to coverage. Pharmacy policies are not intended to dictate to physicians how to practice medicine. Physicians should exercise their medical judgment in providing the care they feel is most appropriate for their patients.
Neither this policy, nor the successful adjudication of a pharmacy claim, is guarantee of payment.
Commercial _ PS _ Interleukin_(IL)-1_Inhibitors_PAQL _ProgSum_ 07-01-2024
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