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Date Printed: June 23, 2017: 06:33 PM

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This medical policy (medical coverage guideline) is Copyright 2017, Blue Cross and Blue Shield of Florida (BCBSF). All Rights Reserved. You may not copy or use this document or disclose its contents without the express written permission of BCBSF. The medical codes referenced in this document may be proprietary and owned by others. BCBSF makes no claim of ownership of such codes. Our use of such codes in this document is for explanation and guidance and should not be construed as a license for their use by you. Before utilizing the codes, please be sure that to the extent required, you have secured any appropriate licenses for such use. Current Procedural Terminology (CPT) is copyright 2017 American Medical Association. All Rights Reserved. No fee schedules, basic units, relative values, or related listings are included in CPT. The AMA assumes no liability for the data contained herein. Applicable FARS/DFARS restrictions apply to government use. CPT® is a trademark of the American Medical Association. The use of specific product names is illustrative only. It is not intended to be a recommendation of one product over another, and is not intended to represent a complete listing of all products available.

09-J2000-03

Original Effective Date: 10/15/13

Reviewed: 09/14/16

Revised: 11/15/16

Next Review: No Longer Scheduled for Routine Review (NLR)

Subject: Canakinumab (Ilaris®) Injection

THIS MEDICAL COVERAGE GUIDELINE IS NOT AN AUTHORIZATION, CERTIFICATION, EXPLANATION OF BENEFITS, OR A GUARANTEE OF PAYMENT, NOR DOES IT SUBSTITUTE FOR OR CONSTITUTE MEDICAL ADVICE. ALL MEDICAL DECISIONS ARE SOLELY THE RESPONSIBILITY OF THE PATIENT AND PHYSICIAN. BENEFITS ARE DETERMINED BY THE GROUP CONTRACT, MEMBER BENEFIT BOOKLET, AND/OR INDIVIDUAL SUBSCRIBER CERTIFICATE IN EFFECT AT THE TIME SERVICES WERE RENDERED. THIS MEDICAL COVERAGE GUIDELINE APPLIES TO ALL LINES OF BUSINESS UNLESS OTHERWISE NOTED IN THE PROGRAM EXCEPTIONS SECTION.

           
Dosage/ Administration Position Statement Billing/Coding Reimbursement Program Exceptions Definitions
           
Related Guidelines Other References Updates  
           

DESCRIPTION:

Canakinumab is a recombinant, human anti-human interleukin-1 beta (IL-1B) monoclonal antibody of the IgG1/kappa isotype. By binding to human IL-1B, canakinumab blocks the IL-1 receptor interaction and neutralizes overactive IL-1B activity which is present in disorders such as Cryopyrin-Associated Periodic Syndromes (CAPS) and systemic juvenile idiopathic arthritis (SJIA). Canakinumab does not bind IL-1 alpha or IL-1 receptor antagonist (IL-ra).

Cryopyrin-Associated Periodic Syndromes (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.

The NLRP-3 gene encodes the protein cryopyrin, an important component of the inflammasome. Cryopyrin regulates the protease caspase-1 and controls the activation of interleukin-1 beta (IL-1β). Mutations in NLRP-3 result in an overactive inflammasome resulting in excessive release of activated IL-1β that drives inflammation. Systemic juvenile idiopathic arthritis (SJIA) is a severe auto-inflammatory disease, driven by innate immunity by means of pro-inflammatory cytokines such as interleukin 1β (IL-1β).

Canakinumab is FDA-approved for the treatment of CAPS including Familial Cold Autoinflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in adults and children 4 years of age and older. Canakinumab is also FDA-approved for the treatment of active systemic juvenile idiopathic arthritis (SJIA) in patients aged 2 years and older.

Canakinumab was FDA-approved in September 2016 for the treatment of Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS), Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD), and Familial Mediterranean Fever (FMF).

POSITION STATEMENT:

I. Initiation of canakinumab (Ilaris) meets the definition of medical necessity for members diagnosed with ANY of the following conditions when ALL associated criteria are met:

1. Cryopyrin-Associated Periodic Syndrome (CAPS) or Cold Induced Auto-inflammatory Syndrome (CAIS) including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS)

a. There is clinical documentation of functional impairment resulting in limitations of activities of daily living

b. Canakinumab is NOT being administered with a tumor necrosis factor (TNF) inhibitor or an interleukin (IL) blocker, including (but not limited to) ANY of the following:

Abatacept (Orencia®)

Adalimumab (Humira®)

Anakinra (Kineret®)

Certolizumab (Cimzia®)

Etanercept (Enbrel®)

Golimumab (Simponi®)

Infliximab (Remicade®)

Ixekizumab (Taltz®)

• Secukinumab (Cosentyx®)

Tocilizumab (Actemra®)

Tofacitinib (Xeljanz®)

Ustekinumab (Stelara®)

Rilonacept (Arcalyst®)

c. The dose does not exceed 150 mg every 8 weeks

d. The member is 4 years of age and older.

2. Active Systemic Juvenile Idiopathic Arthritis (SJIA) (Pediatric Still’s Disease)

a. Member’s disease is moderately to severely active

b. Member has tried/failed, disease activity progressed following treatment, or has a contraindication to one or more DMARDs (e.g., methotrexate, cyclosporine, leflunomide)

c. Canakinumab is NOT being administered with a tumor necrosis factor (TNF) inhibitor or an interleukin (IL) blocker, including (but not limited to) ANY of the following:

Abatacept (Orencia®)

Adalimumab (Humira®)

Anakinra (Kineret®)

Certolizumab (Cimzia®)

Etanercept (Enbrel®)

Golimumab (Simponi®)

Infliximab (Remicade®)

Ixekizumab (Taltz®)

• Secukinumab (Cosentyx®)

Tocilizumab (Actemra®)

Tofacitinib (Xeljanz®)

Ustekinumab (Stelara®)

Rilonacept (Arcalyst®)

d. The dose does not exceed 300 mg every 4 weeks

e. Member is 2 years of age or older

3. Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS)

a. Member has chronic or recurrent disease activity

b. Canakinumab is NOT being administered with a tumor necrosis factor (TNF) inhibitor or an interleukin (IL) blocker, including (but not limited to) ANY of the following:

Abatacept (Orencia®)

Adalimumab (Humira®)

Anakinra (Kineret®)

Certolizumab (Cimzia®)

Etanercept (Enbrel®)

Golimumab (Simponi®)

Infliximab (Remicade®)

Ixekizumab (Taltz®)

Secukinumab (Cosentyx®)

Tocilizumab (Actemra®)

Tofacitinib (Xeljanz®)

Ustekinumab (Stelara®)

Rilonacept (Arcalyst®)

c. The dose does not exceed 300 mg every 4 weeks

d. Member is 2 years of age or older

4. Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD)

a. Member has a diagnosis of HIDS with mevalonate kinase deficiency and a history of flares

b. Canakinumab is NOT being administered with a tumor necrosis factor (TNF) inhibitor or an interleukin (IL) blocker, including (but not limited to) ANY of the following:

Abatacept (Orencia®)

Adalimumab (Humira®)

Anakinra (Kineret®)

Certolizumab (Cimzia®)

Etanercept (Enbrel®)

Golimumab (Simponi®)

Infliximab (Remicade®)

Ixekizumab (Taltz®)

Secukinumab (Cosentyx®)

Tocilizumab (Actemra®)

Tofacitinib (Xeljanz®)

Ustekinumab (Stelara®)

Rilonacept (Arcalyst®)

c. The dose does not exceed 300 mg every 4 weeks

d. Member is 2 years of age or older

5. Familial Mediterranean Fever (FMF)

a. Member continues to have flares on colchicine therapy or has a contraindication/intolerance to colchicine

b. Canakinumab is NOT being administered with a tumor necrosis factor (TNF) inhibitor or an interleukin (IL) blocker, including (but not limited to) ANY of the following:

Abatacept (Orencia®)

Adalimumab (Humira®)

Anakinra (Kineret®)

Certolizumab (Cimzia®)

Etanercept (Enbrel®)

Golimumab (Simponi®)

Infliximab (Remicade®)

Ixekizumab (Taltz®)

Secukinumab (Cosentyx®)

Tocilizumab (Actemra®)

Tofacitinib (Xeljanz®)

Ustekinumab (Stelara®)

Rilonacept (Arcalyst®)

c. The dose does not exceed 300 mg every 4 weeks

d. Member is 2 years of age or older

Duration of approval: 12 months

II. Continuation of canakinumab (Ilaris) injection meets the definition of medical necessity for members meeting ALL of the following criteria:

1. Member has a history of beneficial clinical response with canakinumab therapy for the treatment of ONE of the following indications:

a. Cryopyrin-Associated Periodic Syndrome (CAPS) or Cold Induced Auto-inflammatory Syndrome (CAIS) including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS)

b. Active Systemic Juvenile Idiopathic Arthritis (SJIA) (Pediatric Still’s Disease)

c. Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS)

d. Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD)

e. Familial Mediterranean fever (FMF)

2. The member has been previously approved by Florida Blue or another healthplan in the past 2 years, OR the member has previously met all indication-specific criteria.

3. Canakinumab is NOT being administered with a tumor necrosis factor (TNF) inhibitor or an interleukin (IL) blocker, including (but not limited to) ANY of the following:

• Abatacept (Orencia®)

• Adalimumab (Humira®)

• Anakinra (Kineret®)

• Certolizumab (Cimzia®)

• Etanercept (Enbrel®)

• Golimumab (Simponi®)

• Infliximab (Remicade®)

Ixekizumab (Taltz®)

• Secukinumab (Cosentyx®)

• Tocilizumab (Actemra®)

Tofacitinib (Xeljanz®)

Ustekinumab (Stelara®)

Rilonacept (Arcalyst®)

4. The dose does not exceed the following based on indication:

a. Cryopyrin-Associated Periodic Syndrome (CAPS) or Cold Induced Auto-inflammatory Syndrome (CAIS) including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS): 150 mg subcutaneous every 8 weeks

b. Active Systemic Juvenile Idiopathic Arthritis (SJIA) (Pediatric Still’s Disease): 300 mg subcutaneous every 4 weeks

c. Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS), Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD), or Familial Mediterranean Fever (FMF): 300 mg subcutaneous every 4 weeks

Duration of approval: 12 months

Canakinumab (Ilaris) injection is considered experimental or investigational for treatment of Adult Onset Still’s Disease.

DOSAGE/ADMINISTRATION:

THIS INFORMATION IS PROVIDED FOR INFORMATIONAL PURPOSES ONLY AND SHOULD NOT BE USED AS A SOURCE FOR MAKING PRESCRIBING OR OTHER MEDICAL DETERMINATIONS. PROVIDERS SHOULD REFER TO THE MANUFACTURER’S FULL PRESCRIBING INFORMATION FOR DOSAGE GUIDELINES AND OTHER INFORMATION RELATED TO THIS MEDICATION BEFORE MAKING ANY CLINICAL DECISIONS REGARDING ITS USAGE.

FDA-approved

Cryopyrin-Associated Periodic Syndromes

Body weight greater than 40 kg: 150 mg subcutaneously every 8 weeks

Body weight greater than or equal to 15 kg and less than or equal to 40 kg: 2 mg/kg (may increase to 3 mg/kg if inadequate response) subcutaneously every 8 weeks

Systemic Juvenile Idiopathic Arthritis (SJIA)

4 mg/kg (maximum of 300 mg; body weight greater than or equal to 7.5 kg) subcutaneously every 4 weeks

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS)

Body weight greater than 40 kg: 150 mg subcutaneously every 4 weeks (may increase to 300 mg every 4 weeks if the clinical response is not adequate)

Body weight less than or equal to 40 kg: 2 mg/kg administered every 4 weeks (may increase to 4 mg/kg every 4 weeks if the clinical response is not adequate)

Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD)

Body weight greater than 40 kg: 150 mg subcutaneously every 4 weeks (may increase to 300 mg every 4 weeks if the clinical response is not adequate)

Body weight less than or equal to 40 kg: 2 mg/kg administered every 4 weeks (may increase to 4 mg/kg every 4 weeks if the clinical response is not adequate)

Familial Mediterranean Fever (FMF)

Body weight greater than 40 kg: 150 mg subcutaneously every 4 weeks (may increase to 300 mg every 4 weeks if the clinical response is not adequate)

Body weight less than or equal to 40 kg: 2 mg/kg administered every 4 weeks (may increase to 4 mg/kg every 4 weeks if the clinical response is not adequate)

Dose Adjustments

None

Drug Availability

180 mg single-use, glass vial

PRECAUTIONS:

Boxed Warning

None

Contraindications

Confirmed hypersensitivity to the active substance or to any of the excipients

Precautions/Warnings

Interleukin-1 blockade may interfere with immune response to infections; discontinue treatment if serious or active infection

Live vaccines should not be given concurrently

It is unknown if treatment with immunosuppressants such as anti-interleukin-1 therapy result in an increased risk of malignancy.

Hypersensitivity reactions have occurred.

Macrophage activation syndrome (MAS) is a known, life-threatening disorder that may develop in patients with rheumatic conditions and should be aggressively treated. Prescribers should be attentive to symptoms of infection or worsening SJIA.

BILLING/CODING INFORMATION:

The following codes may be used to describe:

HCPCS Coding:

J0638

Injection, canakinumab, 1 mg

ICD-10 Diagnoses Codes That Support Medical Necessity: (Effective 10/01/15)

D89.89

Other specified disorders involving the immune mechanism, not elsewhere classified

E85.0

Non-neuropathic heredofamilial amyloidosis

L50.2

Urticaria due to cold and heat

M04.1

Periodic fever syndrome

M04.2

Cryopyrin-associated periodic syndromes

M04.8

Other autoinflammatory syndromes

M04.9

Autoinflammatory syndrome, unspecified

M08.00 – M.08.09

Unspecified juvenile rheumatoid arthritis of unspecified sites

M08.1

Juvenile ankylosing spondylitis

M08.20 – M08.29

Juvenile rheumatoid arthritis with systemic onset, unspecified sites

M08.3

Juvenile rheumatoid polyarthritis (seronegative)

M08.40 – M08.48

Pauciarticular juvenile rheumatoid arthritis, unspecified sites

M08.80 – M08.89

Other juvenile arthritis, unspecified sites

M08.90 – M08.99

Juvenile arthritis, unspecified, unspecified sites

M35.9

Systemic involvement of connective tissue, unspecified

REIMBURSEMENT INFORMATION:

Refer to section entitled POSITION STATEMENT.

PROGRAM EXCEPTIONS:

Federal Employee Program (FEP): Follow FEP guidelines.

State Account Organization (SAO): Follow SAO guidelines.

Medicare Advantage: No National Coverage Determination (NCD) and/or Local Coverage Determination (LCD) were found at the time this guideline was drafted.

DEFINITIONS:

Cryopyrin-Associated Periodic Syndrome (CAPS) aka Cold Induced Auto-inflammatory Syndrome (CAIS1): is a spectrum of auto-inflammatory syndromes including familial cold auto-inflammatory syndrome (FCAS, formerly termed familial cold-induced urticaria), the Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID, also called chronic infantile neurologic cutaneous and articular syndrome or CINCA).

Familial cold auto-inflammatory syndrome (FCAS): is an autosomal dominant condition characterized by rash, conjunctivitis, fever/chills and arthralgias elicited by exposure to cold.

Familial Mediterranean fever (FMF): is an inherited autosomal recessive condition characterized by recurrent episodes of painful inflammation in the abdomen, chest, joints, muscles, scrotum or skin accompanied by fever.

Hyperimmunoglobulin D syndrome (HIDS): is a rare inherited autosomal recessive condition characterized by recurrent episodes of fever associated with painful inflammation in the abdomen or joints and skin rash. The condition is associated with decreased activity of mevalonate kinase (MVK).

Macrophage Activation Syndrome (MAS): a life-threatening condition characterized by fever, organomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, coagulopathy, and other symptoms.

Muckle-Wells syndrome (MWS): is a rare autosomal dominant disease which causes sensorineural deafness, recurrent hives, and can lead to amyloidosis.

Neonatal-onset multisystem inflammatory disease (NOMID) or chronic infantile neurologic cutaneous and articular syndrome or CINCA: is a rare genetic periodic fever syndrome which causes uncontrolled inflammation in multiple parts of the body starting in the newborn period.

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS): a rare genetic disease with episodes of recurrent fever, abdominal, chest and muscle pain and a typical rash lasting for more than one week.

RELATED GUIDELINES:

Rilonacept (Arcalyst®) Injection, 09-J2000-04

OTHER:

None

REFERENCES:

  1. AHFS Drug Information. Bethesda (MD): American Society of Health-System Pharmacists, Inc; 2016 [cited 2016 Aug 17]. In: STAT!Ref Online Electronic Medical Library [Internet]. Available from: http://online.statref.com/.
  2. Clinical Pharmacology [Internet]. Tampa (FL): Gold Standard, Inc.; 2016 [cited 2016 Oct 11]. Available from: http://www.clinicalpharmacology.com/.
  3. DRUGDEX® System [Internet]. Greenwood Village (CO): Thomson Micromedex; Updated periodically [cited 2016 Oct 11]. Available from: http://www.thomsonhc.com/.
  4. FDA Orphan Drug Designations and Approvals [Internet]. Washington, D.C. [cited 2016 Aug 17]. Available from: http://www.accessdata.fda.gov/scripts/opdlisting/oopd/
  5. Ilaris® [package insert]. East Hanover, New Jersey: Novartis Pharmaceuticals Corporation; September 2016.
  6. Lachmann HJ, Kone-Paut I, Kuemmerle-Deschner JB et al. Use of canakinumab in the cryopyrin-associated periodic syndrome. N Engl J Med. 2009; 360: 2416-2425.
  7. Ringold S, Weiss PF, Beukelman T et al. 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 Rheum. 2013; 65 : 2499-2512.
  8. Ruperto N, Brunner HI, Quartier P et al. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012; 367: 2396-2406.

COMMITTEE APPROVAL:

This Medical Coverage Guideline (MCG) was approved by the BCBSF Pharmacy Coverage Committee on 09/14/16.

GUIDELINE UPDATE INFORMATION:

10/15/13

New Medical Coverage Guideline.

10/15/14

Review and revision to guideline; consisting of revising position statement, dosage/administration, precautions/warnings, coding, references.

10/15/15

Review and revision to guideline; consisting of revising position statement, precautions/warnings, definitions, coding, references.

11/01/15

Revision: ICD-9 Codes deleted.

10/01/16

Update to ICD-10 codes.

10/15/16

Review and revision to guideline; consisting of revising position statement, definitions, coding, references.

11/15/16

Revision to guideline; consisting of updating position statement and references.

Date Printed: June 23, 2017: 06:33 PM