Coverage Policy Manual
Policy #: 2020026
Category: Pharmacy
Initiated: February 2021
Last Review: August 2025
  Canakinumab (e.g., Ilaris)
Description:
Canakinumab (e.g., Ilaris) is a recombinant, human anti-human-IL-1β monoclonal antibody that belongs to the IgG1/κ isotype subclass. Canakinumab binds to human IL-1β and neutralizes its activity by blocking its interaction with IL-1 receptors, but it does not bind IL-1α or IL-1 receptor antagonist (IL-1ra). (Ilaris, 2020)
 
Regulatory Status
 
Canakinumab (e.g., Ilaris) is approved by the U.S. Food and Drug Administration (FDA) for the following indications:
1. Periodic Fever Syndrome
a. Cryopyrin-Associated Periodic Syndromes (CAPS)
b. Familial Mediterranean Fever (FMF)
c. Hyperimmunoglobulin D Syndrome/ Mevalonate Kinase Deficiency (HIDS/MKD)
d. Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS)
2. Still’s Disease
a. Adult-Onset Still’s Disease (AOSD)
b. Systemic Juvenile Idiopathic Arthritis (SJIA)  
 
On August 25, 2023, the U.S. Food and Drug Administration approved Canakinumab (e.g., Ilaris) approved the addition of a new indication: gout flares in adults in whom non-steroidal anti-inflammatory drugs (NSAIDS) and colchicine are contraindicated, are not tolerated, or do not provide an adequate response, and in whom repeated courses of corticosteroids are not appropriate.
 
Coding
 
See CPT/HCPCS Code section below.
Policy/
Coverage:
Effective February 25, 2021 Prior Approval is required for Canakinumab (e.g., Ilaris).
 
The initial use of this drug requires documentation of direct physician involvement and signature in the ordering and evaluation as documented in the medical records submitted for prior approval. Concurrent review will require continued evidence of appropriate physician involvement.  
 
INITIAL AND CONTINUATION APPROVAL will be for duration of treatment course or 12 months (whichever comes first). Approval timeframes may differ for members/participants of Self-Insured plans.
 
Effective August 6, 2025
 
Meets Primary Coverage Criteria or Is Covered for Contracts Without Primary Coverage Criteria
 
Canakinumab (e.g., Ilaris) meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes when ALL the following criteria are met:
 
CRYOPYRIN-ASSOCIATED PERIODIC SYNDROMES (CAPS)
 
INITIAL APPROVAL
 
1. Individual 4 years of age or older has diagnosis of either of the following CAPS:
a. Familial cold auto inflammatory syndrome (Ilaris, 2024); OR
b. Muckle-Wells syndrome (Ilaris, 2024); AND
2. Documentation submitted confirming NLRP3 mutation (Lachmann, 2009); AND
3. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat CAPS (e.g., rilonacept, anakinra); AND
4. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab.
 
CONTINUATION OF THERAPY
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: fewer cold-induced attacks resolution of fever, improvement in rash or skin manifestations, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat CAPS (e.g., rilonacept, anakinra).
 
FAMILIAL MEDITERRANEAN FEVER (FMF)
 
INITIAL APPROVAL
 
1. Individual 2 years of age or older has a diagnosis of familial mediterranean fever (FMF); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND
3. Individual has had an inadequate response to, is intolerance of, or has a contraindication to colchicine (Benedetti, 2018); AND
4. Documentation submitted confirming at least one known MEFV gene exon 10 mutation (Benedetti, 2018); AND
5. Individual will not be using in combination with TNF antagonists or another IL-1 antagonist.
 
CONTINUATION OF THERAPY
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: resolution of fever, improvement in rash or skin manifestations, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonist or another IL-1 antagonist.
 
HYPERIMMUNOGLOBULIN D SYNDROME/ MEVALONATE KINASE DEFICIENCY
 
INITIAL APPROVAL
 
1. Individual 2 years of age or older has a diagnosis of hyperimmunoglobulin D syndrome/ mevalonate kinase deficiency (HIDS/MKD); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND
3. Documentation submitted confirming genetic or enzymatic diagnosis of mevalonate kinase deficiency (Benedetti, 2018); AND
4. Individual will not be using in combination with TNF antagonists or another IL-1 antagonist.
 
CONTINUATION OF THERAPY  
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living).
 
TUMOR NECROSIS FACTOR RECEPTOR ASSOCIATED PERIODIC SYNDROME
 
INITIAL APPROVAL
 
1. Individual 2 years of age or older has a diagnosis of tumor necrosis factor receptor associated periodic syndrome (TRAPS); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND
3. Documentation submitted confirming TNFRSF1A mutation (Benedetti, 2018); AND
4. Individual will not be using in combination with TNF antagonists or other IL-1 antagonist.
 
CONTINUATION OF THERAPY  
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonist or another IL-1 antagonist.
 
ADULT-ONSET STILL’S DISEASE & SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS
 
INITIAL APPROVAL:
 
1. Individual 2 years of age or older has a diagnosis of SJIA or AOSD; AND
2. Individual has had an inadequate response to at least a two-week trial, is intolerant of, or has a contraindication to oral or intravenous corticosteroids (ACR, 2013); OR
3. Individual has had an inadequate response to at least a one-month trial, is intolerant of, or has a contraindication to NSAIDs (ACR, 2013); OR
4. Individual has had an inadequate response to at least a three-month trial, is intolerant of, or has a contraindication to methotrexate or leflunomide (ACR, 2013); AND
5. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat SJIA or Still’s disease (e.g., tocilizumab, anakinra).
 
CONTINUATION OF THERAPY  
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat SJIA or Still’s disease (e.g., tocilizumab, anakinra).
 
GOUT FLARES
 
INITIAL APPROVAL
 
1. Individual is 18 years of age or older; AND
2. Individual is using for the treatment of acute gout flares; AND
3. Individual is NOT using as a prophylactic therapy for chronic gout, the use of canakinumab for prophylaxis of gout flares is not covered; AND
4. Individual has experienced at least three gout flares in the previous year (Ilaris, 2023); AND
5. Individual meets either of the following:
a. Individual has had an inadequate response to two nonsteroidal anti-inflammatory drugs (NSAIDS) and colchicine; OR
b. Individual has a contraindication to or is intolerant of both NSAIDS and colchicine; AND
6. Documentation that repeated courses of corticosteroids have been ineffective or are contraindicated for the individual; AND
7. Canakinumab is prescribed by or in consultation with a rheumatologist; AND
8. Individual is currently receiving or will be taking concomitant urate lowering medication for the prevention of gout (e.g., allopurinol, febuxostat, probenecid) if indicated; AND
9. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other specialty drug intended to treat gout (e.g., anakinra); AND
10. If individual requires re-treatment with canakinumab for a gout flare, there is at least a 12-week interval between treatments (Ilaris, 2023.
 
CONTINUATION OF THERAPY  
 
1. Individual has met initial criteria and has previously received canakinumab for a gout flare; AND
2. Individual is using for the treatment of acute gout flares; AND
3. Individual is NOT using as a prophylactic therapy for chronic gout, the use of canakinumab for prophylaxis of gout flares is not covered; AND
4. Canakinumab is prescribed by or in consultation with a rheumatologist; AND
5. Individual is currently receiving or will be taking concomitant urate lowering medication for the prevention of gout (e.g., allopurinol, febuxostat, probenecid) if indicated; AND
6. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other specialty drug intended to treat gout (e.g., anakinra); AND
7. If individual requires re-treatment with canakinumab for a gout flare, there is at least a 12-week interval between treatments (Ilaris, 2023).
 
Does Not Meet Primary Coverage Criteria Or Is Not Covered For Contracts Without Primary Coverage Criteria
 
Canakinumab (e.g., Ilaris), for any indication or circumstance not described above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For individuals with contracts without primary coverage criteria, Canakinumab (e.g., Ilaris), for any indication or circumstance not described above, is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
POLICY GUIDELINES
 
There should be an absence of unacceptable toxicity from the drug, including serious infections and hypersensitivity reactions and drug reaction with eosinophilia and systemic symptoms (DRESS).
 
Canakinumab (e.g., Ilaris) will not be used in combination with TNF antagonist or another IL-1 antagonist.
 
Individual does not have any of the following (Schlesinger, 2012):
1. Rheumatoid arthritis or evidence or suspicion of infectious or septic arthritis or other acute inflammatory arthritis; OR
2. Severe renal function impairment; OR
3. Refractory heart failure; OR
4. Unstable cardiac arrhythmias or unstable symptomatic coronary ischemia.
 
Prescriber is responsible for verification that Individual does not have latent tuberculosis or serious active infection before starting the treatment.
 
Physician Global Assessment of disease activity is taking into account fever and clinical signs and symptoms associated with each disease (as listed below) with the use of a 5-point scale with scores of 0 (none), 1 (minimal), 2 (mild), 3 (moderate) and 4 (severe).
 
FMF                           HIDS/MKD                    TRAPS__________
Chest pain                  Lymphadenopathy          Skin rash
Abdominal pain           Aphthous ulcers             Musculoskeletal pain
Arthralgia/arthritis       Abdominal pain               Abdominal pain
Skin rash                                                        Eye manifestations
 
DOSAGE AND ADMINISTRATION
 
For FDA labeled indications, Canakinumab (e.g., Ilaris) must be dosed in accordance with the indication specific recommended dose per FDA label unless otherwise specified below.
 
The recommended dose of Canakinumab (e.g., Ilaris) is based on indication:
 
CAPS- 150 mg for individuals with body weight greater than 40 kg and 2 mg/kg for individuals with body weight greater than or equal to 15 kg and less than or equal to 40 kg. For children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg. Administer subcutaneously every 8 weeks.
 
FMF, HIDS/MKD, and TRAPS- 150mg for individuals with body weight greater than 40 kg and can be increased to 300mg if the clinical response is not adequate. 2mg/kg for individuals with body weight less than or equal to 40 kg and can be increased to 4mg/kg if the clinical response is not adequate. Administer subcutaneously every 4 weeks.
 
AOSD and SJIA- 4 mg/kg (with a maximum of 300 mg) for individuals with a body weight greater than or equal to 7.5 kg. Administer subcutaneously every 4 weeks.
 
Gout Flares – 150 mg subcutaneously. For individuals who require re-treatment, there should be an interval of at least 12 weeks before a new dose of canakinumab may be administered.
 
Canakinumab (e.g., Ilaris) is administered by subcutaneous injection by a healthcare provider.
 
Canakinumab (e.g., Ilaris)  is available as 150mg lyophilized powder in single dose vials and 150mg solution in single dose vials.
 
Please refer to a separate policy on Site of Care or Site of Service Review policy #2018030 for pharmacologic/biologic medications.
 
Effective January 29, 2025 to August 5, 2025
 
Meets Primary Coverage Criteria or Is Covered for Contracts Without Primary Coverage Criteria
 
Canakinumab (e.g., Ilaris) meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes when ALL the following criteria are met:
 
CRYOPYRIN-ASSOCIATED PERIODIC SYNDROMES
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
1. Individual 4 years of age or older has diagnosis of either of the following CAPS:
a. Familial cold auto inflammatory syndrome (FDA, 2020); OR
b. Muckle-Wells syndrome (FDA, 2020); AND
2. Documentation submitted confirming NLRP3 mutation (Lachmann, 2009); AND
3. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat CAPS (e.g., rilonacept, anakinra); AND
4. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab; AND
5. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: fewer cold-induced attacks resolution of fever, improvement in rash or skin manifestations, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND  
3. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat CAPS (e.g., rilonacept, anakinra); AND
4. Must be dosed in accordance with the FDA label.
 
FAMILIAL MEDITERRANEAN FEVER
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
1. Individual 2 years of age or older has a diagnosis of familial mediterranean fever (FMF); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND
3. Individual has had an inadequate response to, is intolerance of, or has a contraindication to colchicine (Benedetti, 2018); AND
4. Documentation submitted confirming at least one known MEFV gene exon 10 mutation (Benedetti, 2018); AND
5. Individual will not be using in combination with TNF antagonists or another IL-1 antagonist; AND
6. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: resolution of fever, improvement in rash or skin manifestations, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND  
3. Individual is not using the medication in combination with TNF antagonist or another IL-1 antagonist; AND
4. Must be dosed in accordance with the FDA label.
 
HYPERIMMUNOGLOBULIN D SYNDROME/ MEVALONATE KINASE DEFICIENCY
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
1. Individual 2 years of age or older has a diagnosis of hyperimmunoglobulin D syndrome/ mevalonate kinase deficiency (HIDS/MKD); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND  
3. Documentation submitted confirming genetic or enzymatic diagnosis of mevalonate kinase deficiency (Benedetti, 2018); AND
4. Individual will not be using in combination with TNF antagonists or another IL-1 antagonist; AND
5. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonist or another IL-1 antagonist; AND
4. Must be dosed in accordance with the FDA label.
 
TUMOR NECROSIS FACTOR RECEPTOR ASSOCIATED PERIODIC SYNDROME
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
1. Individual 2 years of age or older has a diagnosis of tumor necrosis factor receptor associated periodic syndrome (TRAPS); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND
3. Documentation submitted confirming TNFRSF1A mutation (Benedetti, 2018); AND
4. Individual will not be using in combination with TNF antagonists or other IL-1 antagonist; AND
5. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonist or another IL-1 antagonist; AND
4. Must be dosed in accordance with the FDA label.
 
ADULT-ONSET STILL’S DISEASE & SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS
 
1. Individual 2 years of age or older has a diagnosis of SJIA or AOSD; AND
2. Individual has had an inadequate response to at least a two-week trial, is intolerant of, or has a contraindication to oral or intravenous corticosteroids (ACR, 2013); OR
3. Individual has had an inadequate response to at least a one-month trial, is intolerant of, or has a contraindication to NSAIDs (ACR, 2013); OR
4. Individual has had an inadequate response to at least a three-month trial, is intolerant of, or has a contraindication to methotrexate or leflunomide (ACR, 2013); AND
5. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat SJIA or Still’s disease (e.g., tocilizumab, anakinra); AND
6. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat SJIA or Still’s disease (e.g., tocilizumab, anakinra); AND
4. Must be dosed in accordance with the FDA label.
 
GOUT FLARES
 
STANDARD REVIEW for up to 6 months:
 
1. Individual is 18 years of age or older; AND
2. Individual is using for the treatment of acute gout flares; AND
3. Individual is NOT using as a prophylactic therapy for chronic gout, the use of canakinumab for prophylaxis of gout flares is not covered; AND
4. Individual has experienced at least three gout flares in the previous year (Ilaris, 2023); AND
5. Individual meets either of the following:
a. Individual has had an inadequate response to both nonsteroidal anti-inflammatory drugs (NSAIDS) and colchicine; OR
b. Individual has a contraindication to or is intolerant of both NSAIDS and colchicine; AND
6. Documentation that repeated courses of corticosteroids have been ineffective or are contraindicated for the individual; AND
7. Canakinumab is prescribed by or in consultation with a rheumatologist; AND
8. Individual is currently receiving or will be taking concomitant urate lowering medication for the prevention of gout (e.g., allopurinol, febuxostat, probenecid) unless contraindicated; AND
9. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other specialty drug intended to treat gout (e.g., pegloticase, anakinra); AND
10. Individual does not have ANY of the following (Schlesinger, 2012):
a. Rheumatoid arthritis or evidence or suspicion of infectious or septic arthritis or other acute inflammatory arthritis; OR
b. Severe renal function impairment; OR
c. Refractory heart failure; OR
d. Unstable cardiac arrhythmias or unstable symptomatic coronary ischemia; AND
11. If individual requires re-treatment with canakinumab for a gout flare, there is at least a 12-week interval between treatments (Ilaris, 2023); AND
12. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial criteria and has previously received canakinumab for a gout flare; AND
2. Individual is using for the treatment of acute gout flares; AND
3. Individual is NOT using as a prophylactic therapy for chronic gout, the use of canakinumab for prophylaxis of gout flares is not covered; AND
4. Canakinumab is prescribed by or in consultation with a rheumatologist; AND
5. Individual is currently receiving or will be taking concomitant urate lowering medication for the prevention of gout (e.g., allopurinol, febuxostat, probenecid) unless contraindicated; AND
6. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other specialty drug intended to treat gout (e.g., pegloticase, anakinra); AND
7. Individual does not have ANY of the following (Schlesinger, 2012):
a. Rheumatoid arthritis or evidence or suspicion of infectious or septic arthritis or other acute inflammatory arthritis; OR
b. Severe renal function impairment; OR
c. Refractory heart failure; OR
d. Unstable cardiac arrhythmias or unstable symptomatic coronary ischemia; AND
8. If individual requires re-treatment with canakinumab for a gout flare, there is at least a 12-week interval between treatments (Ilaris, 2023); AND
9. Must be dosed in accordance with the FDA label.
 
Policy Guidelines
 
Prescriber is responsible for verification that Individual does not have latent tuberculosis or serious active infection before starting the treatment.
 
Physician Global Assessment of disease activity is taking into account fever and clinical signs and symptoms associated with each disease (as listed below) with the use of a 5-point scale with scores of 0 (none), 1 (minimal), 2 (mild), 3 (moderate) and 4 (severe).
 
FMF                                      HIDS/MKD                               TRAPS
Chest pain                             Lymphadenopathy                     Skin rash
Abdominal pain                      Aphthous ulcers                        Musculoskeletal pain
Arthralgia/arthritis                   Abdominal pain                        Abdominal pain
Skin rash                                                                             Eye manifestations
 
Dosage and Administration
Dosing per FDA Guidelines
 
The recommended dose of Canakinumab is based on indication:
 
CAPS- 150 mg for individuals with body weight greater than 40 kg and 2 mg/kg for individuals with body weight greater than or equal to 15 kg and less than or equal to 40 kg. For children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg. Administer subcutaneously every 8 weeks.
 
FMF, HIDS/MKD, and TRAPS- 150mg for individuals with body weight greater than 40 kg and can be increased to 300mg if the clinical response is not adequate. 2mg/kg for individuals with body weight less than or equal to 40 kg and can be increased to 4mg/kg if the clinical response is not adequate. Administer subcutaneously every 4 weeks.
 
AOSD and SJIA- 4 mg/kg (with a maximum of 300 mg) for individuals with a body weight greater than or equal to 7.5 kg. Administer subcutaneously every 4 weeks.
 
Gout Flares – 150 mg subcutaneously. For individuals who require re-treatment, there should be an interval of at least 12 weeks before a new dose of canakinumab may be administered.
 
Canakinumab is administered by subcutaneous injection by a healthcare provider.
 
Canakinumab is available as 150mg lyophilized powder in single dose vials and 150mg solution in single dose vials.
 
Please refer to a separate policy on Site of Care or Site of Service Review policy #2018030 for pharmacologic/biologic medications.
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
Canakinumab, for any indication or circumstance not described above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For individuals with contracts without primary coverage criteria, Canakinumab, for any indication or circumstance not described above, is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Effective August 7, 2024 to January 28, 2025
 
Meets Primary Coverage Criteria or Is Covered for Contracts Without Primary Coverage Criteria
 
Canakinumab (e.g., Ilaris) meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes when ALL the following criteria are met:
 
CRYOPYRIN-ASSOCIATED PERIODIC SYNDROMES
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
1. Individual 4 years of age or older has diagnosis of either of the following CAPS:
a. Familial cold auto inflammatory syndrome (FDA, 2020); OR
b. Muckle-Wells syndrome (FDA, 2020); AND
2. Documentation submitted confirming NLRP3 mutation (Lachmann, 2009); AND
3. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat CAPS (e.g., rilonacept, anakinra); AND
4. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections; AND
5. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab; AND
6. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: fewer cold-induced attacks resolution of fever, improvement in rash or skin manifestations, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND  
3. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat CAPS (e.g., rilonacept, anakinra); AND
4. Must be dosed in accordance with the FDA label.
 
FAMILIAL MEDITERRANEAN FEVER
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
1. Individual 2 years of age or older has a diagnosis of familial mediterranean fever (FMF); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND
3. Individual has had an inadequate response to, is intolerance of, or has a contraindication to colchicine (Benedetti, 2018); AND
4. Documentation submitted confirming at least one known MEFV gene exon 10 mutation (Benedetti, 2018); AND
5. Individual will not be using in combination with TNF antagonists or another IL-1 antagonist; AND
6. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections; AND
7. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: resolution of fever, improvement in rash or skin manifestations, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND  
3. Individual is not using the medication in combination with TNF antagonist or another IL-1 antagonist; AND
4. Must be dosed in accordance with the FDA label.
 
HYPERIMMUNOGLOBULIN D SYNDROME/ MEVALONATE KINASE DEFICIENCY
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
1. Individual 2 years of age or older has a diagnosis of hyperimmunoglobulin D syndrome/ mevalonate kinase deficiency (HIDS/MKD); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND  
3. Documentation submitted confirming genetic or enzymatic diagnosis of mevalonate kinase deficiency (Benedetti, 2018); AND
4. Individual will not be using in combination with TNF antagonists or another IL-1 antagonist; AND
5. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections; AND
6. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonist or another IL-1 antagonist; AND
4. Must be dosed in accordance with the FDA label.
 
TUMOR NECROSIS FACTOR RECEPTOR ASSOCIATED PERIODIC SYNDROME
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
1. Individual 2 years of age or older has a diagnosis of tumor necrosis factor receptor associated periodic syndrome (TRAPS); AND
2. Individual has had active flares within the last 6 months defined as:
a. Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018); OR
b. C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018); AND
3. Documentation submitted confirming TNFRSF1A mutation (Benedetti, 2018); AND
4. Individual will not be using in combination with TNF antagonists or other IL-1 antagonist; AND
5. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections; AND
6. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonist or another IL-1 antagonist; AND
4. Must be dosed in accordance with the FDA label.
 
ADULT-ONSET STILL’S DISEASE & SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS
 
1. Individual 2 years of age or older has a diagnosis of SJIA or AOSD; AND
2. Individual has had an inadequate response to at least a two-week trial, is intolerant of, or has a contraindication to oral or intravenous corticosteroids (ACR, 2013); OR
3. Individual has had an inadequate response to at least a one-month trial, is intolerant of, or has a contraindication to NSAIDs (ACR, 2013); OR
4. Individual has had an inadequate response to at least a three-month trial, is intolerant of, or has a contraindication to methotrexate or leflunomide (ACR, 2013); AND
5. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat SJIA or Still’s disease (e.g., tocilizumab, anakinra); AND
6. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections; AND
7. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial approval criteria; AND
2. Individual has experienced a documented positive clinical response (for example: improvement in rash or skin manifestations, resolution of fever, clinically significant improvement or normalization of serum markers such as C-reactive protein or amyloid A, reduction of proteinuria, stabilization of serum creatinine, less joint pain, less joint stiffness, less swelling, decreased fatigue, improved activities of daily living); AND
3. Individual is not using the medication in combination with TNF antagonists, other IL-1 antagonists or any other biologic intended to treat SJIA or Still’s disease (e.g., tocilizumab, anakinra); AND
4. Must be dosed in accordance with the FDA label.
 
GOUT FLARES
 
STANDARD REVIEW for up to 6 months:
 
1. Individual is 18 years of age or older; AND
2. Individual is using for the treatment of acute gout flares; AND
3. Individual is NOT using as a prophylactic therapy for chronic gout, the use of canakinumab for prophylaxis of gout flares is not covered; AND
4. Individual has experienced at least three gout flares in the previous year (Ilaris, 2023); AND
5. Individual meets either of the following:
a. Individual has had an inadequate response to both nonsteroidal anti-inflammatory drugs (NSAIDS) and colchicine; OR
b. Individual has a contraindication to or is intolerant of both NSAIDS and colchicine; AND
6. Documentation that repeated courses of corticosteroids have been ineffective or are contraindicated for the individual; AND
7. Canakinumab is prescribed by or in consultation with a rheumatologist; AND
8. Individual is currently receiving or will be taking concomitant urate lowering medication for the prevention of gout (e.g., allopurinol, febuxostat, probenecid) unless contraindicated; AND
9. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other specialty drug intended to treat gout (e.g., pegloticase, anakinra); AND
10. Individual does not have tuberculosis, other active serious infections, or a history of recurrent infections (Ilaris, 2023); AND
11. Individual does not have ANY of the following (Schlesinger, 2012):
a. Rheumatoid arthritis or evidence or suspicion of infectious or septic arthritis or other acute inflammatory arthritis; OR
b. Severe renal function impairment; OR
c. Refractory heart failure; OR
d. Unstable cardiac arrhythmias or unstable symptomatic coronary ischemia; AND
12. If individual requires re-treatment with canakinumab for a gout flare, there is at least a 12-week interval between treatments (Ilaris, 2023); AND
13. Must be dosed in accordance with the FDA label.
 
CONTINUED APPROVAL for up to 12 months:
 
1. Individual has met initial criteria and has previously received canakinumab for a gout flare; AND
2. Individual is using for the treatment of acute gout flares; AND
3. Individual is NOT using as a prophylactic therapy for chronic gout, the use of canakinumab for prophylaxis of gout flares is not covered; AND
4. Canakinumab is prescribed by or in consultation with a rheumatologist; AND
5. Individual is currently receiving or will be taking concomitant urate lowering medication for the prevention of gout (e.g., allopurinol, febuxostat, probenecid) unless contraindicated; AND
6. Individual will not be using in combination with TNF antagonists, other IL-1 antagonists or any other specialty drug intended to treat gout (e.g., pegloticase, anakinra); AND
7. Individual does not have ANY of the following (Schlesinger, 2012):
a. Rheumatoid arthritis or evidence or suspicion of infectious or septic arthritis or other acute inflammatory arthritis; OR
b. Severe renal function impairment; OR
c. Refractory heart failure; OR
d. Unstable cardiac arrhythmias or unstable symptomatic coronary ischemia; AND
8. If individual requires re-treatment with canakinumab for a gout flare, there is at least a 12-week interval between treatments (Ilaris, 2023); AND
9. Must be dosed in accordance with the FDA label.
 
Policy guidelines
 
Physician Global Assessment of disease activity is taking into account fever and clinical signs and symptoms associated with each disease (as listed below) with the use of a 5-point scale with scores of 0 (none), 1 (minimal), 2 (mild), 3 (moderate) and 4 (severe).
 
FMF                                      HIDS/MKD                               TRAPS
Chest pain                             Lymphadenopathy                     Skin rash
Abdominal pain                      Aphthous ulcers                        Musculoskeletal pain
Arthralgia/arthritis                   Abdominal pain                        Abdominal pain
Skin rash                                                                             Eye manifestations
 
Dosage and Administration
Dosing per FDA Guidelines
 
The recommended dose of Canakinumab is based on indication:
 
CAPS- 150 mg for individuals with body weight greater than 40 kg and 2 mg/kg for individuals with body weight greater than or equal to 15 kg and less than or equal to 40 kg. For children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg. Administer subcutaneously every 8 weeks.
 
FMF, HIDS/MKD, and TRAPS- 150mg for patients with body weight greater than 40 kg and can be increased to 300mg if the clinical response is not adequate. 2mg/kg for patients with body weight less than or equal to 40 kg and can be increased to 4mg/kg if the clinical response is not adequate. Administer subcutaneously every 4 weeks.
 
AOSD and SJIA- 4 mg/kg (with a maximum of 300 mg) for individuals with a body weight greater than or equal to 7.5 kg. Administer subcutaneously every 4 weeks.
 
Gout Flares – 150 mg subcutaneously. For individuals who require re-treatment, there should be an interval of at least 12 weeks before a new dose of canakinumab may be administered.
 
Canakinumab is administered by subcutaneous injection by a healthcare provider.
 
Canakinumab is available as 150mg lyophilized powder in single dose vials and 150mg solution in single dose vials.
 
Please refer to a separate policy on Site of Care or Site of Service Review policy #2018030 for pharmacologic/biologic medications.
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
Canakinumab, for any indication or circumstance not described above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For individuals with contracts without primary coverage criteria, Canakinumab, for any indication or circumstance not described above, is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Effective August 23, 2023 to August 6, 2024
 
Meets Primary Coverage Criteria or Is Covered for Contracts Without Primary Coverage Criteria
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
The use of Canakinumab meets primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes when the following criteria are met based on diagnosis:
 
Cryopyrin-Associated Periodic Syndromes
Individual 4 years of age or older has diagnosis of either of the following CAPS:
    1. Familial cold auto inflammatory syndrome (FDA, 2020) OR
    2. Muckle-Wells syndrome (FDA, 2020) AND
    3. Documentation submitted confirming NLRP3 mutation (Lachmann, 2009) AND
    4. Individual will not be using in combination with TNF antagonists or other IL-1 antagonist AND
    5. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections, AND
    6. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention -recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab AND
    7. Must be dosed in accordance with the FDA label.
 
Familial Mediterranean Fever
Individual 2 years of age or older has a diagnosis of FMF AND
    1. Individual has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND
2.  Individual has had an inadequate response to, is intolerance of, or has a contraindication to colchicine (Benedetti, 2018) AND
3.  Documentation submitted confirming at least one known MEFV gene exon 10 mutation (Benedetti, 2018) AND
4. Individual will not be using in combination with TNF antagonists or other IL-1 antagonist AND
5. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections, AND
6. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention -recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab AND
7. Must be dosed in accordance with the FDA label.
 
Hyperimmunoglobulin D Syndrome/ Mevalonate Kinase Deficiency
Individual 2 years of age or older has a diagnosis of HIDS/MKD AND
    1. Individual has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND  
2.  Documentation submitted confirming genetic or enzymatic diagnosis of mevalonate kinase deficiency (Benedetti, 2018) AND
3. Individual will not be using in combination with TNF antagonists or other IL-1 antagonist AND
4. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections, AND
5. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention -recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab AND  
6. Must be dosed in accordance with the FDA label.
 
Tumor Necrosis Factor Receptor Associated Periodic Syndrome
Individual 2 years of age or older has a diagnosis of TRAPS AND
    1. Individual has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND
2.  Documentation submitted confirming TNFRSF1A mutation (Benedetti, 2018) AND
3. Individual will not be using in combination with TNF antagonists or other IL-1 antagonist AND
4. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections, AND
5. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention -recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab AND
6. Must be dosed in accordance with the FDA label.
 
Adult-Onset Still’s Disease & Systemic Juvenile Idiopathic Arthritis
Individual 2 years of age or older has a diagnosis of SJIA or AOSD AND
    1. Individual has had an inadequate response to at least a two-week trial, is intolerant of, or has a contraindication to oral or intravenous corticosteroids (ACR, 2013) OR
    2. Individual has had an inadequate response to at least a one-month trial, is intolerant of, or has a contraindication to NSAIDs (ACR, 2013) OR
    3. Individual has had an inadequate response to at least a three-month trial, is intolerant of, or has a contraindication to methotrexate or leflunomide (ACR, 2013) AND
    4. Individual will not be using in combination with TNF antagonists or other IL-1 antagonist AND
    5. Individual does not have Tuberculosis, other active serious infections, or a history of recurrent infections, AND
    6. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention -recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab AND
    7. Must be dosed in accordance with the FDA label.
 
Continuation for 12 months:
 
Authorization for continuation may be granted for all individuals who achieve or maintain positive clinical response as evidence by low disease activity or improvement in signs and symptoms.
 
Physician Global Assessment of disease activity, taking into account fever and clinical signs and symptoms associated with each disease (as listed below) with the use of a 5-point scale with scores of 0 (none), 1 (minimal), 2 (mild), 3 (moderate) and 4 (severe).
 
FMF                                     HIDS/MKD                              TRAPS
Chest pain                            Lymphadenopathy                    Skin rash
Abdominal pain                     Aphthous ulcers                         Musculoskeletal pain
Arthralgia/arthritis                  Abdominal pain                        Abdominal pain
Skin rash                                                                            Eye manifestations
 
Dosage and Administration
Dosing per FDA Guidelines
 
The recommended dose of Canakinumab is based on indication:
 
CAPS- 150 mg for individuals with body weight greater than 40 kg and 2 mg/kg for individuals with body weight greater than or equal to 15 kg and less than or equal to 40 kg. For children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg. Administer subcutaneously every 8 weeks.
 
FMF, HIDS/MKD, and TRAPS- 150mg for patients with body weight greater than 40 kg and can be increased to 300mg if the clinical response is not adequate. 2mg/kg for patients with body weight less than or equal to 40 kg and can be increased to 4mg/kg if the clinical response is not adequate. Administer subcutaneously every 4 weeks.
 
AOSD and SJIA- 4 mg/kg (with a maximum of 300 mg) for individuals with a body weight greater than or equal to 7.5 kg. Administer subcutaneously every 4 weeks.
 
Canakinumab is administered by subcutaneous injection by a healthcare provider.
 
Canakinumab is available as 150mg lyophilized powder in single dose vials and 150mg solution in single dose vials.
 
Please refer to a separate policy on Site of Care or Site of Service Review policy #2018030 for pharmacologic/biologic medications.
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
The use of canakinumab for any indication or circumstance other than those described above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For individuals with contracts without primary coverage criteria, the use of Canakinumab for any indication or circumstance not described above is considered investigational.
 
Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Effective December 1, 2021 to August 22, 2023
 
Meets Primary Coverage Criteria or Is Covered for Contracts Without Primary Coverage Criteria
 
The use of Canakinumab meets primary coverage criteria when dosed in accordance with FDA approved labeling AND when the following criteria are met based on diagnosis:
 
INITIAL APPROVAL STANDARD REVIEW for up to 12 months:
 
Cryopyrin-Associated Periodic Syndromes
Individual 4 years of age or older has diagnosis of either of the following CAPS:
    1. Familial cold auto inflammatory syndrome (FDA, 2020) OR
    2. Muckle-Wells syndrome (FDA, 2020) AND
    3. Documentation submitted confirming NLRP3 mutation (Lachmann, 2009)
    4. Must be dosed in accordance with the FDA label unless otherwise specified.
 
Familial Mediterranean Fever
Individual 2 years of age or older has a diagnosis of FMF AND
    1. Member has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND
2.  Individual has had an inadequate response to, is intolerance of, or has a contraindication to colchicine (Benedetti, 2018) AND
3.  Documentation submitted confirming at least one known MEFV gene exon 10 mutation (Benedetti, 2018)
4.  Must be dosed in accordance with the FDA label unless otherwise specified.
 
Hyperimmunoglobulin D Syndrome/ Mevalonate Kinase Deficiency
Individual 2 years of age or older has a diagnosis of HIDS/MKD AND
    1. Individual has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND  
2.  Documentation submitted confirming genetic or enzymatic diagnosis of mevalonate kinase deficiency (Benedetti, 2018)
3.  Must be dosed in accordance with the FDA label unless otherwise specified.
 
Tumor Necrosis Factor Receptor Associated Periodic Syndrome
Individual 2 years of age or older has a diagnosis of TRAPS AND
    1. Individual has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND
2.  Documentation submitted confirming TNFRSF1A mutation (Benedetti, 2018)
3.  Must be dosed in accordance with the FDA label unless otherwise specified.
 
Adult-Onset Still’s Disease & Systemic Juvenile Idiopathic Arthritis
Individual 2 years of age or older has a diagnosis of SJIA or AOSD AND
    1. Individual has had an inadequate response to at least a two-week trial, is intolerant of, or has a contraindication to oral or intravenous corticosteroids (ACR, 2013) OR
    2. Individual has had an inadequate response to at least a one-month trial, is intolerant of, or has a contraindication to NSAIDs (ACR, 2013) OR
    3. Individual has had an inadequate response to at least a three-month trial, is intolerant of, or has a contraindication to methotrexate or leflunomide (ACR, 2013)
    4. Must be dosed in accordance with the FDA label unless otherwise specified.
 
Continuation for 12 months:
Authorization for continuation may be granted for all members who achieve or maintain positive clinical response as evidence by low disease activity or improvement in signs and symptoms.
 
Physician Global Assessment of disease activity, taking into account fever and clinical signs and symptoms associated with each disease (as listed below) with the use of a 5-point scale with scores of 0 (none), 1 (minimal), 2 (mild), 3 (moderate) and 4 (severe).
 
FMF                                     HIDS/MKD                              TRAPS
Chest pain                            Lymphadenopathy                    Skin rash
Abdominal pain                     Aphtous ulcers                         Musculoskeletal pain
Arthralgia/arthritis                  Abdominal pain                        Abdominal pain
Skin rash                                                                            Eye manifestations
 
Dosage and Administration
 
The recommended dose of Canakinumab is based on indication:
    1. CAPS- 150 mg for patients with body weight greater than 40 kg and 2 mg/kg for patients with body weight greater than or equal to 15 kg and less than or equal to 40 kg. For children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg. Administer subcutaneously every 8 weeks.
    2. FMF, HIDS/MKD, and TRAPS- 150mg for patients with body weight greater than 40 kg and can be increased to 300mg if the clinical response is not adequate. 2mg/kg for patients with body weight less than or equal to 40 kg and can be increased to 4mg/kg if the clinical response is not adequate. Administer subcutaneously every 4 weeks.
    3. AOSD and SJIA- 4 mg/kg (with a maximum of 300 mg) for patients with a body weight greater than or equal to 7.5 kg. Administer subcutaneously every 4 weeks.
 
Canakinumab is administered by subcutaneous injection by a healthcare provider. Canakinumab is available as 150mg lyophilized powder in single dose vials and 150mg solution in single dose vials.
 
Please refer to a separate policy on Site of Care or Site of Service Review policy #2018030 for pharmacologic/biologic medications.
 
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
The use of canakinumab does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes including the following:
    1. In combination with TNF antagonists or other IL-1 antagonist OR  
    2. Tuberculosis, other active serious infections, or a history of recurrent infections; OR  
    3. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention -recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab
 
For individuals with contracts without primary coverage criteria, the use of Canakinumab for any indication or circumstance not described above is considered investigational including the following:
    1. In combination with TNF antagonists or other IL-1 antagonist OR  
    2. Tuberculosis, other active serious infections, or a history of recurrent infections; OR  
    3. Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention -recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab
 
Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Effective 2/18/2021 to11/30/2021
 
Meets Primary Coverage Criteria or Is Covered for Contracts Without Primary Coverage Criteria
The use of Canakinumab meets primary coverage criteria when dosed in accordance with FDA approved labeling AND when the following criteria are met based on diagnosis:
 
Initial approval for 12 months:
 
Cryopyrin-Associated Periodic Syndromes
Member 4 years of age or older has diagnosis of either of the following CAPS:
  1. Familial cold auto inflammatory syndrome OR
  2. Muckle-Wells syndrome AND
  3. Documentation submitted confirming NLRP3 mutation
 
Familial Mediterranean Fever
Member 2 years of age or older has a diagnosis of FMF AND
  1. Member has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND
2.  Member has had an inadequate response to, is intolerance of, or has a contraindication to colchicine AND
3.  Documentation submitted confirming at least one known MEFV gene exon 10 mutation (Benedetti, 2018)
 
Hyperimmunoglobulin D Syndrome/ Mevalonate Kinase Deficiency
Member 2 years of age or older has a diagnosis of HIDS/MKD AND
  1. Member has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND
2.  Documentation submitted confirming elevated serum IgD based on member age OR
3.  Documentation submitted confirming genetic mutation in the MVK gene (Benedetti, 2018)
 
Tumor Necrosis Factor Receptor Associated Periodic Syndrome
Member 2 years of age or older has a diagnosis of TRAPS AND
  1. Member has had active flares within the last 6 months defined as:
a.  Physician’s Global Assessment score greater than or equal to 2 (Benedetti, 2018) OR
b.  C-reactive protein (CRP) greater than 10 mg/L (Benedetti, 2018) AND
2.  Documentation submitted confirming TNFRSF1A mutation (Benedetti, 2018)
 
Adult-Onset Still’s Disease & Systemic Juvenile Idiopathic Arthritis
Member 2 years of age or older has a diagnosis of SJIA or AOSD AND
  1. Member has had an inadequate response to at least a two-week trial, is intolerant of, or has a contraindication to oral or intravenous corticosteroids OR
  2. Member has had an inadequate response to at least a one-month trial, is intolerant of, or has a contraindication to NSAIDs OR
  3. Member has had an inadequate response to at least a three-month trial, is intolerant of, or has a contraindication to methotrexate or leflunomide
 
Continuation for 12 months:
Authorization for continuation may be granted for all members who achieve or maintain positive clinical response as evidence by low disease activity or improvement in signs and symptoms.
 
Physician Global Assessment of disease activity, taking into account fever and clinical signs and symptoms associated with each disease (as listed below) with the use of a 5-point scale with scores of 0 (none), 1 (minimal), 2 (mild), 3 (moderate) and 4 (severe).
 
FMF                                     HIDS/MKD                              TRAPS
Chest pain                            Lymphadenopathy                    Skin rash
Abdominal pain                     Aphtous ulcers                          Musculoskeletal pain
Arthralgia/arthritis                  Abdominal pain                         Abdominal pain
Skin rash                                                                             Eye manifestations
 
Requests for Canakinumab do not meet primary coverage criteria for the following:
  • In combination with TNF antagonists or other IL-1 antagonist OR  
  • Tuberculosis, other active serious infections, or a history of recurrent infections; OR  
  • Individual has not had a tuberculin skin test (TST) or a Centers for Disease Control (CDC-) and Prevention -recommended equivalent to evaluate for latent tuberculosis prior to initiating Canakinumab.
 
Dosage and Administration
The recommended dose of Canakinumab is based on indication:
  1. CAPS- 150 mg for patients with body weight greater than 40 kg and 2 mg/kg for patients with body weight greater than or equal to 15 kg and less than or equal to 40 kg. For children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg. Administer subcutaneously every 8 weeks.
  2. FMF, HIDS/MKD, and TRAPS- 150mg for patients with body weight greater than 40 kg and can be increased to 300mg if the clinical response is not adequate. 2mg/kg for patients with body weight less than or equal to 40 kg and can be increased to 4mg/kg if the clinical response is not adequate. Administer subcutaneously every 4 weeks.
  3. AOSD and SJIA- 4 mg/kg (with a maximum of 300 mg) for patients with a body weight greater than or equal to 7.5 kg. Administer subcutaneously every 4 weeks.
 
Canakinumab is administered by subcutaneous injection by a healthcare provider. Canakinumab is available as 150mg lyophilized powder in single dose vials and 150mg solution in single dose vials.
 
Please refer to a separate policy on Site of Care or Site of Service Review policy #2018030 for pharmacologic/biologic medications.
 
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
The use of canakinumab does not meet member benefit certificate primary coverage criteria for any other indication.
 
For members with contracts without primary coverage criteria, the use of Canakinumab in any other condition than listed above is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
Rationale:
The efficacy and safety of Canakinumab for the treatment of CAPS was demonstrated in a three-part, 48-week, double-blind, placebo-controlled, randomized, withdrawal study. Patients who had CAPS associated with a NLRP3 mutation and who required treatment were eligible for enrollment. 35 patients received 150mg of Canakinumab (2mk/kg for patients weighing 15 to 40kg) subcutaneously in Part 1. At Week 8, 34 of the 35 patients (97%) had complete response to Canakinumab and entered into Part 2. These patients were randomly assigned to receive either 150mg of Canakinumab or placebo every 8 weeks for up to 24 weeks. After 24 weeks or at the time of relapse, whichever occurred first, patients entered into Part 3. These patients received at least two more doses of Canakinumab once every 8 weeks in an open-label active treatment phase.   81% of the patients randomized to placebo in Part 2 flared as compared to non (0%) of the patients randomized to Canakinumab. 28/30 (90%) of patients who completed part 3 were in remission.
 
The efficacy and safety of Canakinumab for the treatment of TRAPS, HIDS/MKD, and FMF was demonstrated in a 4-part study consisting of three separate disease cohorts. Eligible patients was 2 years of age or older. Inclusion criteria for patients with FMF were fulfillment of Tel-Hasomer diagnostic criteria, at least one known MEFV exon 10 mutation, and historical data documenting at least one fever episode per month despite standard dose of colchicine. Patients were allowed to continue colchicine dose. Inclusion criteria for patients with HIDS/MKD was a genetic or enzymatic diagnosis of mevalonate kinase deficiency and historical data documenting at least three fever episodes in a 6-month period. Inclusion criteria for patients with TRAPS was TNFRSF1A mutation and chronic or recurrent disease (recurrent disease was defined as >6 fever episodes per year).
 
The primary outcome was the proportion of patients who had a complete response, defined as resolution of baseline flare at Day 15 and no new flare until Week 16. Resolution of the disease flare was defined as a Physician’s Global Assessment (PGA) Disease Activity score less than 2 and CRP within normal range or reduction greater than or equal to 70% from baseline. A new flare was defined as a PGA score greater than or equal to 2 and CRP greater to or equal than 30mg/L.
 
185 patients were enrolled in the study. Patients in each cohort entered a 12-week screening period during which they were evaluated for the onset of disease flare. Patients were randomized at flare onset into a 12-week double-blind, placebo-controlled treatment period where they received either 150 Canakinumab (2mk/kg for patients weighing less than or equal to 40kg) or placebo once every four weeks. Patients treated with Canakinumab were given an additional dose of 150mg Canakinumab (2mg/kg for patients weighing less than or equal to 40kg) if their disease flare did not resolve, or they had persistent disease activity from Day 8 to Day 14. Patients treated with Canakinumab also received an additional dose of 150mg Canakinumab or 2mg/kg for patients weighing less than or equal to 40kg if their disease flare did not resolve, or who has persistent disease activity from Day 15 to Day 28.
 
46 patients were randomized in the TRAPS cohort. 22 patients were randomized to receive 150mg Canakinumab every 4 weeks and 11/22 (50%) of these patients received up-titration to 300mg every 4 weeks during the 16-week treatment period. 21/24 (87.5%) patients randomized to placebo crossed over to Canakinumab. At Week 16 10/22 (45.5%) of the Canakinumab patients and 2/24 (8.3%) of the placebo patients achieved complete response. 72 patients were randomized in the NID/MKD cohort. 37 patients were randomized to receive 150mg Canakinumab every 4 weeks and 19/37 (51.4%) of these patients received up-titration to 300mg every 4 weeks during the 16-week treatment period. 31/35 (88.6%) patients randomized to placebo crossed over to Canakinumab. At Week 16, 13/37 (35.1%) of the Canakinumab patients and 2/35 (5.7%) of the placebo patients achieved complete response. 63 patients were randomized in the FMF cohort. 31 patients were randomized to receive 150mg Canakinumab every 4 weeks and 10/31 (32.3%) of these patients received up-titration every 4 weeks during the 16-week treatment period. 27/32 (84.4%) patients randomized to placebo crossed over to Canakinumab. At Week 16, 19/31 (61.3%) of the Canakinumab patients and 2/32 (6.3%) of the placebo patients achieved complete response.
 
The efficacy of Canakinumab for the treatment of active SJIA was evaluated in two phase 3, randomized, double-blind, placebo-controlled studies. Patients aged 2 to 19 years of age with SJIA were eligible. Inclusion criteria was active disease, defined as at least 2 joints with active arthritis, documented spiking, intermittent fever, and CRP > 30mg/L. Patients were allowed to continue methotrexate, corticosteroids, and/or NSAIDs without change, except for tapering of the corticosteroid dose as per study design in Study 2.  
 
Study 1 was a single-dose 4-week study assessing the short-term efficacy of Canakinumab in 84 patients randomized to receive a single subcutaneous dose of 4mg/kg Canakinumab or placebo. The primary objective was the portion of patients who achieved at least 30% improvement in an adapted pediatric American College of Rheumatology (ACR) response criterion which included both the pediatric ACR core set and absence of fever at Day 15.
 
At Day 15, 36 patients (84%) in the Canakinumab group had an adapted ACR 30 response compared to 4 patients (10%) in the placebo group. At Day 29, 81% of patients in the Canakinumab group had an adapted ACR 30 response compared to 10% in the placebo group.
 
Study 2 was a withdrawal study of flare prevention by Canakinumab in patients with active SJIA. Flare was defined by worsening of greater than or equal to 30% in at least 3 of the 6 core pediatric ACR response variables combined with improvement of greater than or equal to 30% in no more than 1 of the 6 variables, or reappearance of fever not due to infection for at least 2 consecutive days. Part 1 enrolled 177 patients who received 4mg/kg Canakinumab subcutaneously once every 4 weeks. 100 of these patients continued into Part 2 to receive either 4mg/kg Canakinumab or placebo subcutaneously every 4 weeks. Of the total 128 patients taking corticosteroids who entered the open-label portion of Study 2, 92 attempted corticosteroid tapering. 62% of patients who attempted tapering were able to successfully taper their corticosteroid dose and 46% discontinued corticosteroids altogether.  
 
Part 2, randomized withdrawal design, demonstrated that the time to flare was longer with Canakinumab than with placebo. There was a 64% relative reduction in the risk of flare for patients in the Canakinumab group compared to those in the placebo group.  
 
The efficacy of ILARIS in adults with AOSD is based on the pharmacokinetic exposure and extrapolation of the established efficacy of ILARIS in SJIA patients. Efficacy of ILARIS was also assessed in a randomized, double-blind, placebo-controlled study that enrolled 36 patients (22 to 70 years old) diagnosed with AOSD. The efficacy data were generally consistent with the results of a pooled efficacy analysis of SJIA patients. (Ilaris, 2020)
 
2022 Update
The CLUSTER trial evaluated the effect of canakinumab on health-related quality of life (HRQoL), work/school and social life of patients with autoinflammatory recurrent fever syndromes, including colchicine-resistant familial Mediterranean fever, mevalonate kinase deficiency, and tumor necrosis factor receptor-associated periodic syndrome.
 
HRQoL of patients who received canakinumab 150 mg or 300 mg every four weeks in the CLUSTER trial (n=173) was assessed at baseline and Weeks 17 and 41. For children we used the Child Health Questionnaire - Parent Form 50 (CHQ-PF50), including psychosocial (PsS) and physical (PhS) component summary scores. For adults, the Short-Form-12 (SF-12) Health Survey was used, including physical (PFS) and mental (PCS) component summary scores. The Sheehan Disability Scale (SDS) was used to determine the impact of treatment on work/school, social and family life.
 
The results obtained were remarkably consistent in both pediatric and adult patients across the three disease cohorts. At baseline, median scores for physical components were relatively low (26-29 for PhS and 34-38 for PFS); they improved to values similar to those expected in the general population by Week 17, and this improvement was sustained at Week 41, when median PhS scores were 47-50 and PFS 44-54. Psychosocial and mental scores also improved from baseline to Week 17 and 41, with scores comparable to the general population. Notable improvements were also observed in the SDS scale.
 
Patients with three inherited autoinflammatory syndromes experienced sustained improvements on their HRQoL, work/school, and social life on treatment with canakinumab. (Lachmann HJ, Lauwerys B, Miettunen P, 2021)
 
2023 Update
Annual policy review completed with a literature search using the MEDLINE database through August 2023. No new literature was identified that would prompt a change in the coverage statement.
 
2024 Update
The efficacy of ILARIS was demonstrated in two 12-week, randomized, double-blind, active-controlled studies in patients with gout flares for whom NSAIDs and/or colchicine were contraindicated, not tolerated or ineffective, and who had experienced at least three gout flares in the previous year (Studies 1 and 2). The studies continued in 1) two 12-week, double-blind, active-controlled extensions, followed by 2) two open-label extensions and continued 3) in a third open label extension (combined for both studies) up to a maximum of 36 months where all patients were treated with ILARIS upon a new flare.
 
In Study 1 (NCT01029652), patients were randomized to receive ILARIS 150 mg subcutaneous (N = 115) or triamcinolone acetonide 40 mg intramuscular (N = 115) at baseline and thereafter treated upon a new flare. Two patients randomized to canakinumab were not included in the analysis as they did not receive any study medication. In Study 2 (NCT01080131), patients were randomized to receive ILARIS 150 mg subcutaneous (N =112) or triamcinolone acetonide 40 mg intramuscular (N =114) at baseline and thereafter treated upon a new flare.
 
In Studies 1 and 2, over 85% of patients had at least one co-morbidity, including hypertension (60%), obesity (53%), diabetes (15%), and ischemic heart disease (12%). Twenty-five percent of patients had chronic kidney disease (stage ≥3), based on eGFR. Concomitant treatment with allopurinol or other uric acid lowering therapies was reported by 42% of patients at entry.
 
The majority of patients (73%) reported between 3-6 flares in the year prior to study entry and the remainder reported seven or more flares. Approximately one-third of the patients enrolled [76 in the ILARIS group (33.5%) and 84 in the triamcinolone acetonide (36.7%) group] had documented inability (intolerance, contraindication or lack of response) to use both, NSAIDs and colchicine. The remainder had intolerance, contraindication or lack of response to either NSAIDs or colchicine.
 
In both studies, the co-primary endpoints were: (i) patient’s assessment of gout flare pain intensity at the most affected joint at 72 hours post-dose measured on a 0-100 mm visual analogue scale (VAS) and (ii) the time to first new gout flare. The studies aimed to determine whether ILARIS 150 mg would be superior to triamcinolone acetonide 40 mg. Study 3 (NCT01356602), an additional 12-week, randomized, double-blind, active-controlled study, enrolled 397 patients with ILARIS 150 mg subcutaneous (Pre-Filled Syringe (PFS), N=133, Lyophilizate (LYO), N=132) or triamcinolone acetonide 40 mg intramuscular (N=132). Eight patients (2 ILARIS PFS, 3 ILARIS LYO, 3 triamcinolone) were not included for efficacy assessment as they did not receive study medication. Pain intensity at the most affected joint, assessed on a 0-100 mm VAS at 72-hours post-dose was the primary endpoint, and time to first new gout flare was a secondary endpoint. Approximately 44% of patients (45.9% ILARIS PFS group, 47.4%, ILARIS LYO group and 40.6% in the triamcinolone acetonide group) were unable to use NSAIDs and colchicine (due to contraindications, intolerance, or inadequate response) in this study. Analyses of both endpoints were conducted for Studies 1, 2, and 3 for the subpopulation of patients unable to use NSAIDs and colchicine (due to contraindications, intolerance, or inadequate response) and overall population of patients unable to use NSAIDs and/or colchicine.
 
In both studies, the co-primary endpoints were: (i) patient’s assessment of gout flare pain intensity at the most affected joint at 72 hours post-dose measured on a 0-100 mm visual analogue scale (VAS) and (ii) the time to first new gout flare. The studies aimed to determine whether ILARIS 150 mg would be superior to triamcinolone acetonide 40 mg.
 
Study 3 (NCT01356602), an additional 12-week, randomized, double-blind, active-controlled study, enrolled 397 patients with ILARIS 150 mg subcutaneous (Pre-Filled Syringe (PFS), N=133, Lyophilizate (LYO), N=132) or triamcinolone acetonide 40 mg intramuscular (N=132). Eight patients (2 ILARIS PFS, 3 ILARIS LYO, 3 triamcinolone) were not included for efficacy assessment as they did not receive study medication. Pain intensity at the most affected joint, assessed on a 0-100 mm VAS at 72-hours post-dose was the primary endpoint, and time to first new gout flare was a secondary endpoint. Approximately 44% of patients (45.9% ILARIS PFS group, 47.4%, ILARIS LYO group and 40.6% in the triamcinolone acetonide group) were unable to use NSAIDs and colchicine (due to contraindications, intolerance, or inadequate response) in this study.
 
Analyses of both endpoints were conducted for Studies 1, 2, and 3 for the subpopulation of patients unable to use NSAIDs and colchicine (due to contraindications, intolerance, or inadequate response) and overall population of patients unable to use NSAIDs and/or colchicine.
 
Efficacy on Pain
In all studies (Study 1, 2, and 3), pain intensity of the most affected joint (0-100 mm VAS) at 72 hours post-dose was consistently lower for patients treated with ILARIS compared with triamcinolone acetonide in the subpopulation of patients unable to use NSAIDs and colchicine as shown in Table 9, and Figure 3 (Study 3). This benefit of ILARIS on pain intensity was comparable to the overall patient populations i.e., patients unable to use NSAIDs and/or colchicine in all three studies.
 
Time to New Flare
In the subpopulation of patients in Studies 1, 2 and 3 unable to use NSAIDs and colchicine, time to new flare over 12 weeks from randomization showed a reduction in the risk of a new flare when treated with ILARIS compared with triamcinolone acetonide 40 mg (see Table 10). This risk reduction for a new flare after ILARIS treatment versus triamcinolone acetonide was comparable to the overall patient population over 12 weeks in all 3 studies.(FDA, 2023)
 
2025 Update
Annual policy review completed with a literature search using the MEDLINE database through August 2025. No new literature was identified that would prompt a change in the coverage statement.
CPT/HCPCS:
J0638Injection, canakinumab, 1 mg
References: ACIP(2025) Advisory Committee on Immunization Practices (ACIP). Centers for Disease Control and Prevention www.cdc.gov/acip/index.html

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