CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Ilaris (canakinumab) — Coverage Criteria

Medical policy governing coverage criteria, dosing, authorization length, and renewal requirements for canakinumab (Ilaris) for Medicaid, Commercial, and Medicare members of Neighborhood Health Plan of Rhode Island.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyIlaris (canakinumab) — Coverage Criteria

Policy CodePolicy N/A

Change TypeNo material change

Effective Date01/01/2020

Next Review DateN/A

Key ActionObtain prior authorization; initial approvals are for 6 months (renewable) and gout flares are single 150 mg doses with ≥12 weeks required before retreatment.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsstandard authorization period

12 weeksgout retreatment interval (min)

150common dosing unit

J0638HCPCS code

>= dozensICD-10 diagnoses listed

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Initial Approval Criteria

Coverage is provided when ALL applicable universal criteria AND the indication-specific criteria are met.

Universal Criteria

Age and single-agent use: Many indications require minimum ages and single-agent use; see the indication-specific nodes below for exact age and single-agent requirements.
Refer to indication nodes

Cryopyrin-Associated Periodic Syndromes (CAPS): Patient is ≥4 years of age; will use canakinumab as a single agent; has documented baseline CRP and/or SAA; has laboratory evidence of a pathogenic NLRP3 (CIAS1) mutation; has a diagnosis of Familial Cold Autoinflammatory Syndrome (FCAS) OR Muckle-Wells Syndrome (MWS); AND has two or more CAPS-typical symptoms (urticaria-like rash, triggered episodes, sensorineural hearing loss, musculoskeletal symptoms, chronic aseptic meningitis, skeletal abnormalities).

Chunk 8

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS): Patient is ≥2 years of age; will use canakinumab as a single agent; has a pathogenic TNFRSF1A mutation; has chronic or recurrent disease (flares per year); and has documented baseline CRP.

Chunk 9

Hyperimmunoglobulin D Syndrome (HIDS) / Mevalonate Kinase Deficiency (MKD): Patient is ≥2 years of age; will use canakinumab as a single agent; has confirmed diagnosis by either a pathogenic MVK mutation OR significantly elevated serum IgD; has a documented history of ≥3 febrile episodes within a 6-month period; and has documented baseline CRP.

Chunk 10

Familial Mediterranean Fever (FMF): Patient is ≥2 years of age; will use canakinumab as a single agent; has confirmed diagnosis with at least one known MEFV exon 10 mutation; has failed or is intolerant to colchicine; has active disease defined as ≥1 febrile episode per month; and has documented baseline CRP.

Chunk 11

Still's Disease (Adult-Onset Still's Disease [AOSD] or Systemic Juvenile Idiopathic Arthritis [SJIA]): For AOSD: age ≥18 with active disease; OR for SJIA: age ≥2 with active disease; physician-assessed baseline disease severity using an objective tool; and at least a 1-month trial and failure of NSAIDs OR systemic glucocorticoid unless contraindicated/intolerant, OR patient already established on biologic/targeted synthetic therapy for AOSD or SJIA.

Chunk 12

Gout Flare: Patient is ≥18 years of age; has not received canakinumab for gout in the prior 12 weeks; has had ≥3 gout flares in the previous 12 months; has failed NSAIDs (unless contraindicated/intolerant) AND failed colchicine (unless contraindicated/intolerant); is not a candidate for repeated corticosteroid courses OR has prior response to canakinumab resulting in decreased/resolved joint pain for retreatment of a new flare.

Chunk 13

Renewal Criteria

Coverage may be renewed when renewal criteria are satisfied.

General Renewal: Patient continues to meet universal and indication-specific criteria; there is absence of unacceptable toxicity such as severe hypersensitivity, serious infections (including TB), or macrophage activation syndrome (MAS).

Chunk 14

CAPS Renewal: Demonstrated clinical improvement in symptoms from baseline AND improvement in inflammatory markers (CRP and/or SAA) from baseline.

Chunk 15

AOSD/SJIA Renewal: Improvement in signs and symptoms compared to baseline (e.g., tender/swollen joint counts), reduction in CRP, and/or improvement on a disease activity scoring tool (for example JADAS or ACR-Pedi with ≥30% improvement in 3 of 6 variables).

Chunk 16

TRAPS/HIDS/MKD/FM F Renewal:

Dosing and re-treatment

Dosing and escalation rules as specified by indication

CAPS dosing: Weight-based regimen: for patients ≈40 kg, 150 mg subcutaneously every 8 weeks; alternative weight bands and escalation to 3 mg/kg may be used if inadequate response.

Chunk 19

AOSD and SJIA dosing: Weight-based dosing given as mg/kg subcutaneously every 4 weeks with a maximum single dose of 300 mg; escalation described for inadequate response.300 mg maximum

Chunk 19

TRAPS, HIDS/MKD, and FMF dosing: Standard dose 150 mg subcutaneously every 4 weeks; may increase to 300 mg if inadequate response; pediatric weight-based increments (e.g., 2 mg/kg then increase to 4 mg/kg if needed) are referenced.

Chunk 19

Clinical coverage criteria

Covered when ALL of the following are met

Primary coverage requirements: Use is consistent with FDA-approved labeling and/or relevant clinical literature; documentation supports the medically accepted indication and consideration of cost-effective alternatives when applicable.

Policy rationale and P&T Committee intent

The policy's universal criteria explicitly prohibit concurrent administration with live vaccines and require that the patient is not on concurrent treatment with another biologic therapy (examples listed include IL‑inhibitors and TNF‑inhibitors). These conditions are part of the baseline eligibility requirements and represent denial triggers if present at the time of request.

The portion of the document containing dosing and administration information does not include a separate, explicit exclusion section; dosing text focuses on weight‑based regimens, single‑dose gout flare use, and re‑treatment intervals rather than listing formal exclusions.

For Medicare members the plan's criteria apply only in the absence of an applicable NCD or LCD. Where a Medicare National Coverage Determination, Local Coverage Determination, or Local Coverage Article exists, compliance with that CMS guidance is required; the plan will give individual consideration when reviewing requests and will defer to NCD/LCD/LCA where applicable.

The universal criteria require latent TB screening and ongoing TB monitoring and state that patients must not have an active infection. These infection‑related safeguards also underscore the warning that live vaccines must not be given concurrently with therapy, consistent with standard biologic safety precautions.

Within the cited portions of the policy there is no explicit statement labeling any particular use as categorically “not medically necessary.” The document instead addresses appropriateness through universal criteria, dosing, re‑treatment intervals, and requirements to align use with FDA labeling or relevant literature.

The policy states that use outside FDA‑approved labeling must be supported by relevant clinical literature and documentation; requests for off‑label use should demonstrate that the indication is medically accepted in the literature and that consideration was given to more cost‑effective alternatives when applicable. Lack of such supporting evidence may lead to non‑coverage after plan review.

Billing, Codes, and Diagnosis Linkage

Referenced billing units and dosingmixed

150 billable units	Dose unit referenced for multiple indications (e.g., 150 units every 8 or 12 weeks or every 4 weeks depending on indication)
300 billable units	Dose unit referenced for 'all other indications' every 4 weeks and maximum single-dose for some indications

HCPCS CodeHCPCSCovered

J0638

Injection, canakinumab, 1 mg: 1 billable unit = 1 mg

NDCNDCCovered

00078-0734-XX

Ilaris 150 mg single-dose solution vial

Covered ICD-10 Diagnosis Codes (selected examples shown; full list included in policy appendix)ICD-10Covered

M04.1	Periodic fever syndromes
M04.2	Cryopyrin-associated periodic syndromes
M04.9	Autoinflammatory syndrome, unspecified
M06.1	Adult-onset Still's disease
M08.00	Unspecified juvenile rheumatoid arthritis of unspecified site
M08.011	Unspecified juvenile rheumatoid arthritis, right shoulder
M08.012	Unspecified juvenile rheumatoid arthritis, left shoulder
M08.019	Unspecified juvenile rheumatoid arthritis, unspecified shoulder
M10.9	Gout, unspecified

Covered ICD-10 Diagnosis CodesICD-10Covered

M10.332	Gout due to renal impairment; left wrist
M10.339	Gout due to renal impairment unspecified wrist
M10.341	Gout due to renal impairment_right hand
M10.342	Gout due to renal impairment_left hand
M10.349	Gout due to renal impairment unspecified hand
M10.351	Gout due to renal impairment_right hip
M10.352	Gout due to renal impairment left hip
M10.359	Gout due to renal impairment unspecified hip
M10.361	Gout due to renal impairment;_right knee
M10.362	Gout due to renal impairmentleft knee

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CMS coverage referencesmixed

NCD/LCD/LCA

References to applicable Medicare National and Local Coverage Determinations (NCDs/LCDs/LCAs)

Medicare Part B MACsmixed

MAC jurisdictions

List of Medicare Part B Administrative Contractor jurisdictions and contractors (e.g., Noridian, WPS, NGS, Novitas, First Coast, Palmetto GBA, CGS)

Gout retreatment interval

Gout flare retreatment intervalAt least 12 weeks between canakinumab doses for retreatment of a new gout flare (single 150 mg subcutaneous dose per flare).

Single-dose regimenGout flare dosing specified as a single 150 mg subcutaneous dose; re-treatment only after meeting the minimum interval.

Age requirementPatient must be at least 18 years of age for gout flare indication.

Re-treatment interval

>= 12 weeksRe-treatment interval requirement: subsequent canakinumab doses for gout flare must be given no sooner than 12 weeks after the prior dose.

Documentation noteRequests for re-treatment should document prior response and that at least 12 weeks have elapsed since last Ilaris dose.

Denial riskFailure to meet the ≥12-week interval may trigger denial per universal/re-treatment criteria.

Prior Authorization, Documentation, and Billing Guidance

Prior Authorization

Prior Authorization Required and Length of Authorization

Prior authorization is required for initial and renewal requests. Initial approvals are provided for up to 6 months and may be renewed for additional 6-month periods. For gout flares, coverage is limited to a single 150 mg dose; additional doses for retreatment of a new flare will be considered only if re-treatment criteria are met and at least 12 weeks have passed since the prior dose.

Initial and renewal duration: 6 months
Gout flare single-dose coverage: 150 mg x 1; re-treatment requires meeting re-treatment criteria and ≥12 weeks since prior dose

Billing Rule

Prior Authorization and Billing for Canakinumab (J0638)

Requests for canakinumab must be prior authorized. Billing should use HCPCS J0638 (canakinumab) reported per mg (1 unit = 1 mg). When applicable, the NDC for Ilaris 150 mg single-dose vial (00078-0734-XX) should support the dispensed product. Documentation must support the dose administered and the corresponding J0638 units billed.

Bill as J0638 (Injection, canakinumab, 1 mg) — 1 unit = 1 mg
Document NDC when available: Ilaris 150 mg vial (00078-0734-XX)
Ensure clinical documentation supports billed mg and diagnosis linkage

Denial Risk

Denial Triggers from Universal Safety Criteria

Requests may be denied if universal safety or administrative criteria are not met. Examples that may trigger denial include active infection, failure to screen for latent tuberculosis, administration with live vaccines, or concurrent use with another biologic agent. Medicare requests are subject to any applicable NCD/LCD; where an NCD/LCD exists it supersedes these criteria.

Active infection present — denial risk
No documented latent TB screening or monitoring — denial risk
Concurrent live vaccine administration — denial risk
Concurrent biologic therapy (e.g., IL inhibitors, TNF inhibitors, JAK inhibitors) — denial risk
Medicare: follow NCD/LCD when applicable

Denial Risk

Additional Denial Triggers

Requests may also be denied if the submitted information does not demonstrate a medically accepted indication, lack objective documentation linking the billed units to the diagnosis, or fails step/ prerequisite therapy requirements (when applicable). The prescriber must submit clinical information consistent with FDA labeling or peer-reviewed literature to support medical necessity.

Insufficient clinical documentation linking billed J0638 units to covered diagnosis codes (see covered diagnoses) — denial possible
Request does not meet indication-specific criteria or lacks objective baseline labs/genetic testing where required — denial possible
Failure to demonstrate cost-effective consideration or step therapy when required — denial possible

Billing Rule

Billing and Diagnosis Linkage; Required Documentation

Clinical documentation must link billed J0638 units to a covered diagnosis and the dispensed product NDC where available. Include baseline inflammatory markers (CRP and/or SAA) when indicated, genetic testing results for hereditary autoinflammatory syndromes, patient age, and confirmation that canakinumab is being used as a single agent where required.

Include patient age and diagnosis code consistent with covered indications
Document baseline CRP and/or SAA when required by indication
Provide genetic/mutation testing results for CAPS, TRAPS, HIDS/MKD, FMF as applicable
Ensure billed J0638 units correspond to documented mg administered and match NDC when applicable

Step Therapy

Prerequisite Therapy and Step Requirements

Many indications require prior failure, intolerance, or contraindication to specific therapies before canakinumab will be approved. For example, Familial Mediterranean Fever (FMF) requires failure or intolerance to colchicine. Gout flare requires prior failure of NSAIDs and colchicine (unless contraindicated or intolerant) and ≥3 flares in the past 12 months when initiating therapy.

FMF: documented failure or intolerance to colchicine required
Gout flare: ≥3 flares in past 12 months and failure/intolerance to NSAIDs and colchicine required for initial therapy
Still's disease (AOSD/SJIA): trial and failure of NSAIDs or systemic glucocorticoid for ≥1 month unless contraindicated or intolerant; or established on biologic/targeted synthetic therapy

Clinical Background and Policy Rationale

Canakinumab (Ilaris) is an interleukin‑1β (IL‑1β) inhibitor indicated in the policy for several autoinflammatory syndromes and for treatment of acute gout flares. The dosing section summarizes weight‑based regimens for indications such as CAPS, AOSD/SJIA, TRAPS, HIDS/MKD, and FMF, and specifies a single 150 mg subcutaneous dose for gout flare with a required re‑treatment interval of at least 12 weeks. The policy rationale references P&T Committee review and notes clinical trial evidence supporting reductions in disease activity and inflammatory markers, with infections among the most common adverse events.

Inflammatory markers (definitions)

Defined markersC-Reactive Protein (CRP) and Serum Amyloid A (SAA) are specified as baseline inflammatory markers to document disease activity and response to canakinumab therapy.

Use in renewalImprovement in CRP and/or SAA is required evidence of response for renewal in indications such as CAPS and other periodic fever syndromes.

Required documentationPrescribers must submit baseline CRP and/or SAA values when requesting initial authorization or renewal where applicable.

Genetic confirmation (definitions)

CAPS (NLRP3/CIAS1)Pathogenic mutation in NLRP3 (also known as CIAS1) required for CAPS diagnosis confirmation.

TRAPS (TNFRSF1A)Presence of a pathogenic TNFRSF1A mutation required for TRAPS confirmation.

HIDS/MKD (MVK) and FMF (MEFV exon 10)HIDS/MKD: pathogenic MVK mutation OR significantly elevated serum IgD; FMF: confirmed diagnosis with at least one known MEFV exon 10 mutation.

Gout Flare (definition)

Gout flare definitionAn acute gouty arthritis episode for which a single 150 mg subcutaneous dose of canakinumab is prescribed.

Treatment courseSingle 150 mg dose specified per gout flare; re-treatment permitted only after at least 12 weeks have passed since prior dose.

Eligibility criteriaPatient must be ≥18 years, have ≥3 gout flares in prior 12 months, and have failed NSAIDs and colchicine unless contraindicated/intolerant.

CAPS (Cryopyrin-associated periodic syndromes)

Inclusion of CAPSCryopyrin-associated periodic syndromes (CAPS) are listed as an approved indication for canakinumab with specified dosing and diagnostic requirements.

CAPS dosing overviewWeight-based CAPS dosing: example given as 150 mg subcutaneously every 8 weeks for a 40 kg patient, with escalation to 3 mg/kg if inadequate response.

CAPS diagnostic requirementsPatient ≥4 years, single-agent use, documented baseline CRP and/or SAA, documented NLRP3 (CIAS1) mutation, and CAPS-typical symptoms required for coverage.

Neighborhood Health Plan P&T Committee

P&T Committee reviewIlaris was reviewed and the clinical coverage criteria were adopted by the Neighborhood Health Plan of Rhode Island Pharmacy & Therapeutics (P&T) Committee to ensure use consistent with FDA labeling and relevant clinical literature.

Individual considerationNeighborhood will give individual consideration to requests based on prescriber-submitted information and available plan data.

Medicare applicabilityFor Medicare members, these coverage criteria apply only in the absence of applicable NCD or LCD criteria.

OpenPayer

Ilaris (canakinumab) — Coverage Criteria

Coverage Criteria and Clinical Requirements

Billing, Codes, and Diagnosis Linkage

Prior Authorization, Documentation, and Billing Guidance

Clinical Background and Policy Rationale