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Ilaris® (canakinumab)

Policy Number: PH-0177

Subcutaneous

Last Review Date: 08/08/2023

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

FOR PEEHIP Members Only -Coverage excludes the provider-administered medication(s) outlined in this drug policy from being accessed through a specialty pharmacy. It must be obtained through buy and bill.

  1. Length of Authorization

Coverage will be provided for 12 months and may be renewed.

  1. Dosing Limits

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

  • Ilaris 150 mg: 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)

    All other indications:

  • 300 billable units every 4 weeks (28 days)
  1. 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]) 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

FDA Approved Indication(s); Ф Orphan Drug

  1. 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.
  1. Dosage/Administration 1

Indication

Dose

CAPS

Weight: > 40 kg

  • 150 mg subcutaneously every 8 weeks

Weight: 15 to 40 kg

  • 2 mg/kg subcutaneously every 8 weeks. May increase dose to 3 mg/kg if inadequate response.

AOSD and SJIA

Weight: ≥ 7.5 kg

  • 4 mg/kg (with a maximum of 300mg) subcutaneously every 4 weeks.

TRAPS, HIDS/MKD, and FMF

Weight: > 40 kg

  • 150 mg subcutaneously every 4 weeks. May increase dose to 300mg if inadequate response.

Weight: ≤ 40 kg

  • 2 mg/kg subcutaneously every 4 weeks. May increase dose to 4 mg/kg if inadequate response.

Administration is by healthcare provider

  1. 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
  1. References
  1. Ilaris [package insert]. East Hanover, NJ; Novartis Pharmaceuticals Corporation; September 2020. Accessed July 2023.
  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.

Appendix 1 – Covered Diagnosis Codes

ICD-10

ICD-10 Description

M04.1

Periodic fever syndromes

M04.2

Cryopyrin-associated periodic syndromes

M04.9

Autoinflammatory syndrome, unspecified

M06.1

Adult-onset Still’s disease

M08.0A

Unspecified juvenile rheumatoid arthritis,  other specified site

M08.011

Unspecified juvenile rheumatoid arthritis, right shoulder

M08.012

Unspecified juvenile rheumatoid arthritis, left shoulder

M08.019

Unspecified juvenile rheumatoid arthritis, unspecified shoulder

M08.021

Unspecified juvenile rheumatoid arthritis, right elbow

M08.022

Unspecified juvenile rheumatoid arthritis, left elbow

M08.029

Unspecified juvenile rheumatoid arthritis, unspecified elbow

M08.031

Unspecified juvenile rheumatoid arthritis, right wrist

M08.032

Unspecified juvenile rheumatoid arthritis, left wrist

M08.039

Unspecified juvenile rheumatoid arthritis, unspecified wrist

M08.041

Unspecified juvenile rheumatoid arthritis, right hand

M08.042

Unspecified juvenile rheumatoid arthritis, left hand

M08.049

Unspecified juvenile rheumatoid arthritis, unspecified hand

M08.051

Unspecified juvenile rheumatoid arthritis, right hip

M08.052

Unspecified juvenile rheumatoid arthritis, left hip

M08.059

Unspecified juvenile rheumatoid arthritis, unspecified hip

M08.061

Unspecified juvenile rheumatoid arthritis, right knee

M08.062

Unspecified juvenile rheumatoid arthritis, left knee

M08.069

Unspecified juvenile rheumatoid arthritis, unspecified knee

M08.071

Unspecified juvenile rheumatoid arthritis, right ankle and foot

M08.072

Unspecified juvenile rheumatoid arthritis, left ankle and foot

M08.079

Unspecified juvenile rheumatoid arthritis, unspecified ankle and foot

M08.08

Unspecified juvenile rheumatoid arthritis, vertebrae

M08.09

Unspecified juvenile rheumatoid arthritis, multiple sites

M08.2A

Juvenile rheumatoid arthritis with systemic onset,  other specified site

M08.211

Juvenile rheumatoid arthritis with systemic onset, right shoulder

M08.212

Juvenile rheumatoid arthritis with systemic onset, left shoulder

M08.219

Juvenile rheumatoid arthritis with systemic onset, unspecified shoulder

M08.221

Juvenile rheumatoid arthritis with systemic onset, right elbow

M08.222

Juvenile rheumatoid arthritis with systemic onset, left elbow

M08.229

Juvenile rheumatoid arthritis with systemic onset, unspecified elbow

M08.231

Juvenile rheumatoid arthritis with systemic onset, right wrist

M08.232

Juvenile rheumatoid arthritis with systemic onset, left wrist

M08.239

Juvenile rheumatoid arthritis with systemic onset, unspecified wrist

M08.241

Juvenile rheumatoid arthritis with systemic onset, right hand

M08.242

Juvenile rheumatoid arthritis with systemic onset, left hand

M08.249

Juvenile rheumatoid arthritis with systemic onset, unspecified hand

M08.251

Juvenile rheumatoid arthritis with systemic onset, right hip

M08.252

Juvenile rheumatoid arthritis with systemic onset, left hip

M08.259

Juvenile rheumatoid arthritis with systemic onset, unspecified hip

M08.261

Juvenile rheumatoid arthritis with systemic onset, right knee

M08.262

Juvenile rheumatoid arthritis with systemic onset, left knee

M08.269

Juvenile rheumatoid arthritis with systemic onset, unspecified knee

M08.271

Juvenile rheumatoid arthritis with systemic onset, right ankle and foot

M08.272

Juvenile rheumatoid arthritis with systemic onset, left ankle and foot

M08.279

Juvenile rheumatoid arthritis with systemic onset, unspecified ankle and foot

M08.28

Juvenile rheumatoid arthritis with systemic onset, vertebrae

M08.29

Juvenile rheumatoid arthritis with systemic onset, multiple sites

M08.3

Juvenile rheumatoid polyarthritis (seronegative)

M08.4A

Pauciarticular juvenile rheumatoid arthritis,  other specified site

M08.411

Pauciarticular juvenile rheumatoid arthritis, right shoulder

M08.412

Pauciarticular juvenile rheumatoid arthritis, left shoulder

M08.419

Pauciarticular juvenile rheumatoid arthritis, unspecified shoulder

M08.421

Pauciarticular juvenile rheumatoid arthritis, right elbow

M08.422

Pauciarticular juvenile rheumatoid arthritis, left elbow

M08.429

Pauciarticular juvenile rheumatoid arthritis, unspecified elbow

M08.431

Pauciarticular juvenile rheumatoid arthritis, right wrist

M08.432

Pauciarticular juvenile rheumatoid arthritis, left wrist

M08.439

Pauciarticular juvenile rheumatoid arthritis, unspecified wrist

M08.441

Pauciarticular juvenile rheumatoid arthritis, right hand

M08.442

Pauciarticular juvenile rheumatoid arthritis, left hand

M08.449

Pauciarticular juvenile rheumatoid arthritis, unspecified hand

M08.451

Pauciarticular juvenile rheumatoid arthritis, right hip

M08.452

Pauciarticular juvenile rheumatoid arthritis, left hip

M08.459

Pauciarticular juvenile rheumatoid arthritis, unspecified hip

M08.461

Pauciarticular juvenile rheumatoid arthritis, right knee

M08.462

Pauciarticular juvenile rheumatoid arthritis, left knee

M08.469

Pauciarticular juvenile rheumatoid arthritis, unspecified knee

M08.471

Pauciarticular juvenile rheumatoid arthritis, right ankle and foot

M08.472

Pauciarticular juvenile rheumatoid arthritis, left ankle and foot

M08.479

Pauciarticular juvenile rheumatoid arthritis, unspecified ankle and foot

M08.48

Pauciarticular juvenile rheumatoid arthritis, vertebrae

M08.80

Other juvenile arthritis, unspecified site

M08.811

Other juvenile arthritis, right shoulder

M08.812

Other juvenile arthritis, left shoulder

M08.819

Other juvenile arthritis, unspecified shoulder

M08.821

Other juvenile arthritis, right elbow

M08.822

Other juvenile arthritis, left elbow

M08.829

Other juvenile arthritis, unspecified elbow

M08.831

Other juvenile arthritis, right wrist

M08.832

Other juvenile arthritis, left wrist

M08.839

Other juvenile arthritis, unspecified wrist

M08.841

Other juvenile arthritis, right hand

M08.842

Other juvenile arthritis, left hand

M08.849

Other juvenile arthritis, unspecified hand

M08.851

Other juvenile arthritis, right hip

M08.852

Other juvenile arthritis, left hip

M08.859

Other juvenile arthritis, unspecified hip

M08.861

Other juvenile arthritis, right knee

M08.862

Other juvenile arthritis, left knee

M08.869

Other juvenile arthritis, unspecified knee

M08.871

Other juvenile arthritis, right ankle and foot

M08.872

Other juvenile arthritis, left ankle and foot

M08.879

Other juvenile arthritis, unspecified ankle and foot

M08.88

Other juvenile arthritis, other specified site

M08.89

Other juvenile arthritis, multiple sites

M08.9A

Juvenile arthritis, unspecified,  other specified site

M08.911

Juvenile arthritis, unspecified, right shoulder

M08.912

Juvenile arthritis, unspecified, left shoulder

M08.919

Juvenile arthritis, unspecified, unspecified shoulder

M08.921

Juvenile arthritis, unspecified, right elbow

M08.922

Juvenile arthritis, unspecified, left elbow

M08.929

Juvenile arthritis, unspecified, unspecified elbow

M08.931

Juvenile arthritis, unspecified, right wrist

M08.932

Juvenile arthritis, unspecified, left wrist

M08.939

Juvenile arthritis, unspecified, unspecified wrist

M08.941

Juvenile arthritis, unspecified, right hand

M08.942

Juvenile arthritis, unspecified, left hand

M08.949

Juvenile arthritis, unspecified, unspecified hand

M08.951

Juvenile arthritis, unspecified, right hip

M08.952

Juvenile arthritis, unspecified, left hip

M08.959

Juvenile arthritis, unspecified, unspecified hip

M08.961

Juvenile arthritis, unspecified, right knee

M08.962

Juvenile arthritis, unspecified, left knee

M08.969

Juvenile arthritis, unspecified, unspecified knee

M08.971

Juvenile arthritis, unspecified, right ankle and foot

M08.972

Juvenile arthritis, unspecified, left ankle and foot

M08.979

Juvenile arthritis, unspecified, unspecified ankle and foot

M08.98

Juvenile arthritis, unspecified, vertebrae

M08.99

Juvenile arthritis, unspecified, multiple sites

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

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 Determination (NCD), Local Coverage Determinations (LCDs), and Local Coverage Articles (LCAs) may exist and compliance with these policies is required where applicable. They can be found at: http://www.cms.gov/medicare-coverage-database/search/advanced-search.aspx. Additional indications may be covered at the discretion of the health plan.

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

Medicare Part B Administrative Contractor (MAC) Jurisdictions

Jurisdiction

Applicable State/US Territory

Contractor

E (1)

CA, HI, NV, AS, GU, CNMI

Noridian Healthcare Solutions, LLC

F (2 & 3)

AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ

Noridian Healthcare Solutions, LLC

5

KS, NE, IA, MO

Wisconsin Physicians Service Insurance Corp (WPS)

6

MN, WI, IL

National Government Services, Inc. (NGS)

H (4 & 7)

LA, AR, MS, TX, OK, CO, NM

Novitas Solutions, Inc.

8

MI, IN

Wisconsin Physicians Service Insurance Corp (WPS)

N (9)

FL, PR, VI

First Coast Service Options, Inc.

J (10)

TN, GA, AL

Palmetto GBA, LLC

M (11)

NC, SC, WV, VA (excluding below)

Palmetto GBA, LLC

L (12)

DE, MD, PA, NJ, DC (includes Arlington & Fairfax counties and the city of Alexandria in VA)

Novitas Solutions, Inc.

K (13 & 14)

NY, CT, MA, RI, VT, ME, NH

National Government Services, Inc. (NGS)

15

KY, OH

CGS Administrators, LLC

 

 

 

ILARIS® (canakinumab) Prior Auth Criteria
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