Last Review Date: 08/01/2024

Date of Origin:  11/07/2013

Dates Reviewed:  08/2014, 07/2015, 07/2016, 10/2016, 10/2017, 08/2018, 08/2019, 08/2020, 08/2021, 08/2022, 08/2023, 10/2023, 08/2024

1. Length of Authorization

Coverage will be provided for 12 months and may be renewed, unless otherwise specified.

• Gout Flare: Coverage will be provided for 1 dose (12 weeks). Additional doses for retreatment of a new flare will be covered, provided that the criteria for re-treatment is met. (Refer to Section III for specific re-treatment criteria)

2. Dosing Limits

A. Quantity Limit (max daily dose) [NDC Unit]:

• Ilaris 150 mg single-dose vial: 2 vials every 28 days

B. Max Units (per dose and over time) [HCPCS Unit]:

   Cryopyrin-Associated Periodic Syndromes:

• 150 billable units every 8 weeks (56 days)

     Gout Flare:

• 150 billable units every 12 weeks (84 days)

   All Other Indications:

• 300 billable units every 4 weeks (28 days)

3. Initial Approval Criteria ¹

Coverage is provided in the following conditions:

• Patient is up to date with all vaccinations, in accordance with current vaccination guidelines, prior to initiating therapy; AND

Universal Criteria ¹

• Patient has been evaluated and screened for the presence of latent tuberculosis (TB) infection prior to initiating treatment and will receive ongoing monitoring for the presence of TB during treatment; AND
• Patient does not have an active infection, including clinically important localized infections; AND
• Will not be administered concurrently with live vaccines; AND
• Patient is not on concurrent therapy with other IL-1 blocking agents (e.g., anakinra, rilonacept, etc.): AND
• Patient is not on concurrent treatment with another TNF inhibitor, biologic response modifier or other non-biologic immunomodulating agent (e.g., abrocitinib, apremilast, tofacitinib, baricitinib, upadacitinib, deucravacitinib, etc.); AND

Cryopyrin-Associated Periodic Syndromes (CAPS) † Ф ^1,2,6,8,9

• Patient is at least 4 years of age; AND
• Used as a single agent; AND
• Patient has documented baseline serum levels of inflammatory proteins (C-Reactive Protein [CRP] and/or Serum Amyloid A [SAA]; AND
• Patient has documented laboratory evidence of a genetic mutation in the Cold-Induced Auto-inflammatory Syndrome 1 (CIAS1), also known as NLRP3; AND
  • Diagnosis of Familial Cold Autoinflammatory Syndrome (FCAS); OR
  • Diagnosis of Muckle-Wells Syndrome (MWS); AND
• Patient has two or more of any of the CAPS-typical symptoms:

• urticaria-like rash
• cold-triggered episodes
• sensorineural hearing loss
• musculoskeletal symptoms
• chronic aseptic meningitis
• skeletal abnormalities

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) † Ф ^1,10

• Patient is at least 2 years of age; AND
• Used as a single agent; AND
• Patient has the presence of a pathogenic mutation in the tumor necrosis factor receptor-1 (TNFR1) gene (TNFRSF1A); AND

• Patient has chronic or recurrent disease (defined as > 6 flares per year); AND
• Patient has documented baseline serum levels of C-Reactive Protein (CRP)

 Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD) † Ф ^{1,10, 12}

• Patient is at least 2 years of age; AND
• Used as a single agent; AND
• Patient has a confirmed diagnosis of HIDS/MKD by one of the following:
  • Patient has a pathogenic mutation in the MVK gene; OR
  • Patient has significantly elevated serum IgD levels; AND

• Patient has a documented history of at least three (3) febrile episodes within a 6 month period; AND
• Patient has documented baseline serum levels of C-Reactive Protein (CRP)

Familial Mediterranean Fever (FMF) † Ф ^1,10

• Patient is at least 2 years of age; AND
• Used as a single agent; AND
• Patient has a confirmed diagnosis based on at least one known MEFV exon 10 mutation; AND

• Patient has failed on colchicine therapy or has a documented allergy or intolerance; AND
• Patient has active disease defined as at least one febrile episode per month; AND
• Patient has documented baseline serum levels of C-Reactive Protein (CRP)

Still’s Disease (Adult-Onset Still’s Disease [AOSD] and Systemic Juvenile Idiopathic Arthritis [SJIA]) † Ф ^1,3,5,11

• Patient has active disease; AND
• Physician has assessed baseline disease severity utilizing an objective measure/tool; AND
• Patient has had at least a 1-month trial and failure (unless contraindicated or intolerant) of previous therapy with either oral non-steroidal anti-inflammatory drugs (NSAIDs) OR a systemic glucocorticoid (prednisone, methylprednisolone, etc.); AND
  • Patient is at least 18 years of age and has active Adult-Onset Still’s Disease; OR
  • Patient is at least 2 years of age and has active Systemic Juvenile Idiopathic Arthritis

Gout Flare † ^1,13

• Patient is at least 18 years of age; AND
  • Patient has NOT received previous treatment with canakinumab for gout flare(s); AND
    • Patient has had ≥ 3 gout flares within the previous 12 months; AND
    • Patient has failed on non-steroidal anti-inflammatory drugs (NSAIDs) therapy, unless contraindicated or intolerant; AND
    • Patient has failed on colchicine therapy, unless contraindicated or intolerant; AND
    • Patient is not a candidate for repeated courses of corticosteroids; OR
  • Patient has received previous treatment with canakinumab for gout flare(s) resulting in a decrease or resolution of joint pain in the affected joints; AND
    • Patient requires re-treatment for a new gout flare; AND
    • Patient has not received treatment with canakinumab in the previous 12 weeks

† FDA Approved Indication(s); ‡ Compendia Recommended Indication(s); Ф Orphan Drug

4. Renewal Criteria ^1,3,8-11

Coverage can be renewed based upon the following criteria:

• Patient continues to meet universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in Section III; AND
• Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: severe hypersensitivity reactions, serious infections (including but not limited to tuberculosis), and macrophage activation syndrome (MAS); AND

Cryopyrin-Associated Periodic Syndromes

• Disease response as indicated by improvement in patient’s symptoms from baseline AND improvement in serum levels of inflammatory proteins (e.g. CRP and/or SAA, etc.) from baseline

Adult-Onset Still’s Disease/Systemic Juvenile Idiopathic Arthritis

• Disease response as indicated by improvement in signs and symptoms compared to baseline such as the number of tender and swollen joint counts and/or an improvement on a disease activity scoring tool [e.g. an improvement on a composite scoring index such as Juvenile Arthritis Disease Activity Score (JADAS) or the American College of Rheumatology (ACR) Pediatric (ACR-Pedi 30) of at least 30% improvement from baseline in three of six variables]

Tumor Necrosis Factor Receptor Associated Periodic Syndrome; Hyperimmunoglobulin D Syndrome/Mevalonate Kinase Deficiency; Familial Mediterranean Fever

• Disease response as indicated by improvement in patient’s symptoms from baseline AND improvement of serum levels of CRP

Gout Flare † ^1,13

• Refer to Section III for re-treatment criteria  

5. Dosage/Administration ¹

6. Billing Code/Availability Information

HCPCS Code:

• J0638 – Injection, canakinumab, 1 mg: 1 billable unit = 1 mg

NDC:

• Ilaris 150 mg single-dose solution vial: 00078-0734-xx

7. References

1. Ilaris [package insert]. East Hanover, NJ; Novartis Pharmaceuticals Corporation; August 2023. Accessed July 2024.
2. Lachmann, HJ, Kone-Paut, I, Kuemmerle-Deschner, JB, et al. Use of canakinumab in the cryopyrin-associated periodic syndrome. N Engl J Med. 2009 Jun 4; 360(23):2416-25.
3. Ruperto N, Brunner H, Quartier P, et al. Two Randomized Trials of Canakinumab in Systemic Juvenile Idiopathic Arthritis. N Engl J Med 2012; 367:2396-2406.
4. Ringold, S., Weiss, P. F., Beukelman, T., DeWitt, E. M., Ilowite, N. T., Kimura, Y., Laxer, R. M., Lovell, D. J., Nigrovic, P. A., Robinson, A. B. and Vehe, R. K. (2013), 2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for the Medical Therapy of Children With Systemic Juvenile Idiopathic Arthritis and Tuberculosis Screening Among Children Receiving Biologic Medications. Arthritis & Rheumatism, 65: 2499–2512. Doi: 10.1002/art.38092
5. DeWitt EM, Kimura Y, Beukelman T, et al.  Consensus treatment plans for new-onset systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2012 Jul;64(7):1001-10.
6. Kuemmerle-Deschner JB, Ozen S, Tyrrell PN, et al. Diagnostic criteria for cryopyrin-associated periodic syndrome (CAPS). Ann Rheum Dis. 2017 Jun;76(6):942-947. Doi: 10.1136/annrheumdis-2016-209686.
7. Terreri MT, Bernardo WM, Len CA, et al. Guidelines for the management and treatment of periodic fever syndromes: Cryopyrin-associated periodic syndromes (cryopyrinopathies – CAPS). Rev Bras Reumatol Engl Ed. 2016 Jan-Feb;56(1):44-51. Doi: 10.1016/j.rbre.2015.08.020.
8. Koné-Paut I, Lachmann HJ, Kuemmerle-Deschner JB, et al. Sustained remission of symptoms and improved health-related quality of life in patients with cryopyrin-associated periodic syndrome treated with canakinumab: results of a double-blind placebo-controlled randomized withdrawal study. Arthritis Res Ther. 2011;13(6):R202. Doi:10.1186/ar3535.
9. Kuemmerle-Deschner JB, Hachulla E, Cartwright R, et al. Two-year results from an open-label, multicentre, phase III study evaluating the safety and efficacy of canakinumab in patients with cryopyrin-associated periodic syndrome across different severity phenotypes. Ann Rheum Dis. 2011;70(12):2095-2102. Doi:10.1136/ard.2011.152728.
10. De Benedetti F, Gattorno M, Anton J, et al. Canakinumab for the Treatment of Autoinflammatory Recurrent Fever Syndromes. N Engl J Med. 2018;378(20):1908-1919. Doi:10.1056/NEJMoa1706314.
11. Nirmala N, Brachat A, Feist E, et al. Gene-expression analysis of adult-onset Still’s disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity. Pediatr Rheumatol Online J. 2015;13:50. Published 2015 Nov 20. Doi:10.1186/s12969-015-0047-3.
12. Drenth JP, van der Meer JW. Hereditary periodic fever. N Engl J Med. 2001;345(24):1748.
13. Schlesinger N, Alten RE, Bardin T, et al. Canakinumab for acute gouty arthritis in patients with limited treatment options: results from two randomised, multicentre, active-controlled, double-blind trials and their initial extensions. Ann Rheum Dis. 2012 Nov;71(11):1839-48. doi: 10.1136/annrheumdis-2011-200908. Epub 2012 May 14.

Appendix 1 – Covered Diagnosis Codes

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

The preceding information is intended for non-Medicare coverage determinations. Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determinations (NCDs) and/or Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. Local Coverage Articles (LCAs) may also exist for claims payment purposes or to clarify benefit eligibility under Part B for drugs which may be self-administered. The following link may be used to search for NCD, LCD, or LCA documents: https://www.cms.gov/medicare-coverage-database/search.aspx. Additional indications, including any preceding information, may be applied at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA): N/A