ModifiedCignaPolicy IP0235

Ilaris Prior Authorization Policy

Cigna's coverage and prior authorization criteria for Ilaris (canakinumab) for specified inflammatory and autoinflammatory indications, including required documentation, prescriber specialties, dosing, and approval durations. Applies to members whose benefits are administered by Cigna Companies.

Policy Summary

PayerCigna

PolicyIlaris Prior Authorization Policy

Policy CodePolicy IP0235

Change TypeMaterial revisions to criteria, dosing, and review pathways

Effective DateMay 1, 2026

Next Review DateN/A

Key ActionSubmit prior authorization with specialist prescriber documentation; initial approvals typically 6 months and continuation approvals 1 year with required clinical documentation supporting response.

SourceLink

POLICY UPDATE CHANGES

Added clarification throughout the policy that for a patient currently receiving Ilaris, the patient has been established on the medication for at least 6 months and patients with <6 months or restarting are reviewed under Initial Therapy criteria.

Updated dosing and age criteria for Cryopyrin-Associated Periodic Syndromes (CAPS) including age ≥ 4 years and altered dosing allowances in prior revisions.

Added requirement for Still's disease (AOSD/SJIA) that initial therapy approvals require prescription by or consultation with a rheumatologist and defined durations for initial and continuation therapy.

7+Indications listed

6 moInitial approval

1 yrContinuation approval

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

4 mg/kg (≤300 mg)Max dose

Indication-specific Coverage Criteria

CAPS (Cryopyrin-Associated Periodic Syndromes)

Approve for CAPS when ONE of the following is met

CAPS - Initial or Continuing: A) Initial Therapy — Approve for 6 months if ALL of the following are met: i. Patient is ≥ 4 years of age; AND ii. Confirmed diagnosis of Cryopyrin-Associated Periodic Syndromes [documentation required]; AND iii. Medication is prescribed by or in consultation with a rheumatologist, geneticist, allergist/immunologist, or dermatologist. OR B) Patient is Currently Receiving Ilaris — Approve for 1 year if BOTH: i. Patient has been established on this medication for at least 6 months (patients with <6 months or restarting are reviewed under Initial Therapy); AND ii. Patient meets at least ONE of: a) When assessed by at least one objective measure, patient experienced a beneficial clinical response from baseline; OR b) Compared with baseline, patient experienced an improvement in at least one symptom.

Dosing options: for patients 15–40 kg approve up to 8 mg/kg per dose q4w; for patients >40 kg approve up to 600 mg per dose q4w.

Familial Mediterranean Fever (FMF)

Approve for Familial Mediterranean Fever when ONE of the following is met

FMF - Initial or Continuing: A) Initial Therapy — Approve for 6 months if ALL of the following are met: i. Patient is ≥ 2 years of age; AND ii. ONE of: a) Patient has tried colchicine at the maximum tolerated dose AND will take Ilaris in combination with colchicine; OR b) Colchicine is contraindicated or not tolerated according to the prescriber; AND iii. Prior to starting medication both: a) C-reactive protein (CRP) ≥ 10 mg/L OR ≥2× ULN for the reporting laboratory; AND b) History of at least one flare per month despite colchicine or hospitalization for a severe flare; AND iv. Medication is prescribed by or in consultation with a rheumatologist, nephrologist, geneticist, gastroenterologist, oncologist, or hematologist. OR B) Patient is Currently Receiving Ilaris — Approve for 1 year if BOTH: i. Patient has been established on this medication for at least 6 months; AND ii. Patient meets at least ONE of: a) When assessed by at least one objective measure, patient experienced a beneficial clinical response from baseline; OR b) Compared with baseline, patient experienced an improvement in at least one symptom.

Dosing: ≤40 kg up to 4 mg/kg per dose q4w; >40 kg up to 300 mg per dose q4w.

Gout, Acute Flare

Approve for gout acute flare when ALL of the following are met

Gout, Acute Flare: A) Patient is ≥ 18 years of age; AND B) ONE of: i) Patient has both an intolerance, contraindication, or lack of response to NSAIDs for acute gout flares AND an intolerance, contraindication, or lack of response to colchicine for acute gout flares; OR ii) Patient was previously treated with corticosteroids for an acute gout flare AND, according to the prescriber, is unable to be retreated with a repeat course of corticosteroids; AND C) According to the prescriber, patient is receiving or will be taking concomitant urate-lowering therapy for prevention of gout unless contraindicated; AND D) Medication is prescribed by or in consultation with a rheumatologist.

Dosing: approve up to 150 mg subcutaneously no more frequently than once every 12 weeks.

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

Approve for HIDS/MKD when ONE of the following is met

HIDS/MKD - Initial or Continuing: A) Initial Therapy — Approve for 6 months if ALL of the following are met: i. Patient is ≥ 2 years of age; AND ii. Prior to starting Ilaris, BOTH: a) C-reactive protein (CRP) ≥ 10 mg/L OR ≥2× ULN for the reporting laboratory; AND b) History of at least three febrile acute flares within the previous 6 months or hospitalization for a severe flare; AND iii. Medication is prescribed by or in consultation with a rheumatologist, nephrologist, geneticist, oncologist, or hematologist. OR B) Patient is Currently Receiving Ilaris — Approve for 1 year if BOTH: i. Patient has been established on this medication for at least 6 months; AND ii. Patient meets at least ONE of: a) When assessed by at least one objective measure, patient experienced a beneficial clinical response from baseline; OR b) Compared with baseline, patient experienced an improvement in at least one symptom.

Dosing: ≤40 kg up to 4 mg/kg per dose q4w; >40 kg up to 300 mg per dose q4w.

Adult-Onset Still's Disease (AOSD)

Approve for Adult-Onset Still's Disease when ONE of the following is met

Adult-Onset Still's Disease (AOSD): A) Initial Therapy — Approve for 6 months if BOTH: i. Patient is ≥ 18 years of age; AND ii. Medication is prescribed by or in consultation with a rheumatologist. OR B) Patient is Currently Receiving Ilaris — Approve for 1 year if BOTH: i. Patient has been established on this medication for at least 6 months (patients with <6 months or restarting are reviewed under Initial Therapy); AND ii. Patient meets at least ONE of: a) When assessed by at least one objective measure, patient experienced a beneficial clinical response from baseline; OR b) Compared with baseline, patient experienced an improvement in at least one symptom.

Dosing: up to 4 mg/kg to a maximum of 300 mg per dose q4w.

TRAPS

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) — covered when ONE of the following is met

A or B: A) Initial Therapy — Approve for 6 months if ALL of the following are met: i. Patient is ≥ 2 years of age; AND ii. Prior to starting medication BOTH: a) C-reactive protein (CRP) ≥ 10 mg/L OR ≥2× ULN for the reporting laboratory; AND b) History of at least six flares per year or hospitalization for a severe flare; AND iii. Medication is prescribed by or in consultation with a rheumatologist, geneticist, nephrologist, oncologist, or hematologist. OR B) Patient is Currently Receiving Ilaris — Approve for 1 year if BOTH: i. Patient has been established on this medication for at least 6 months; AND ii. Patient meets at least ONE of: a) When assessed by at least one objective measure, patient experienced a beneficial clinical response from baseline (examples include decreased frequency of attacks, normalization of CRP/amyloid A, reduction in proteinuria, stabilization of creatinine); OR b) Compared with baseline, patient experienced an improvement in at least one symptom.

Dosing: ≤40 kg up to 4 mg/kg per dose q4w; >40 kg up to 300 mg per dose q4w.

Reimbursement is not allowed for services billed for conditions or diagnoses that are not covered under this Coverage Policy. Claims submitted without diagnosis or procedure codes that correspond to the covered indications in this policy will be denied as not covered. When billing, providers must use the most appropriate codes as of the effective date of submission; failure to submit covered code(s) under the applicable Coverage Policy may result in denial.

Use of Ilaris (canakinumab) for any indication not specifically listed in this policy is considered not medically necessary and may be denied. This includes concurrent biologic therapy; Ilaris has not been evaluated and should not be administered in combination with another biologic agent for an inflammatory condition, and concomitant administration with other IL‑1 blockers or biologics is not recommended. Rheumatoid arthritis is specifically listed as not covered due to lack of established efficacy.

Use of Ilaris in indications not listed in this policy (for example, rheumatoid arthritis) or for concurrent administration with other biologic agents is considered not medically necessary. Concomitant administration with other agents that block IL‑1 or its receptors is not recommended because combination use has not been evaluated and safety concerns have been reported with similar agents.

Billing and Coding

Coding guidancemixed

no specific CPT/HCPCS listed

Policy states providers must use the most appropriate codes as of the effective date; specific covered billing codes are not enumerated in this portion of the document.

Considered Medically Necessary (HCPCS)HCPCSCovered

J0638

Injection, canakinumab, 1 mg

C-reactive protein (CRP) — laboratory thresholds

CRP thresholdC-reactive protein (CRP) ≥ 10 mg/L or ≥ 2× the upper limit of normal for the reporting laboratory

Indication use in pivotal trialPivotal periodic fever trial required CRP ≥ 10 mg/L at time of disease flare

Documentation roleCRP may be used as a baseline and follow‑up objective measure to demonstrate clinical response (e.g., normalization or clinically significant improvement)

Age thresholds — indication-specific

CAPS age thresholdPatient must be ≥ 4 years of age for CAPS indications

FMF age threshold

Prior Authorization, Documentation, and Prescriber Requirements

Prior Authorization

Prior Authorization and Specialist Prescriber

Prior Authorization is required for benefit coverage of Ilaris. Initial approval requires prescription by or consultation with a specialist who manages the condition being treated (e.g., rheumatologist, geneticist, allergist/immunologist, dermatologist, nephrologist, gastroenterologist, oncologist, hematologist). Requests for doses outside the dosing ranges in this policy will be reviewed case‑by‑case by a clinician. All approvals are time‑limited and provided for the duration noted in the applicable indication criteria.

Specialist prescriber or consultation required for initial approval
Case‑by‑case review for doses outside policy

Documentation Required

Time‑Limited Approvals and Criteria

Approvals are time‑limited. Initial therapy approvals are typically for 6 months (often adequate for three doses); continuation (patient currently receiving Ilaris) approvals are typically for 1 year when continuation criteria are met. For continuation, the patient must have been established on therapy for at least 6 months and show benefit by objective measures or symptom improvement compared with baseline.

Drug Background and Context

Ilaris (canakinumab) is an interleukin‑1β (IL‑1β) blocker indicated for multiple periodic fever syndromes and autoinflammatory conditions, including Cryopyrin‑Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF), HIDS/MKD, TRAPS, Still's disease (SJIA/AOSD), and for certain gout flares. Guideline committees and the policy note IL‑1 blockade is a recognized therapy for many of these conditions, and the policy operationalizes indication‑specific age limits, dosing, and review pathways for initial versus continuation therapy.

Definitions and Objective Measures

CAPS — definition and spectrum

CAPS definitionCryopyrin‑Associated Periodic Syndromes (CAPS) are a spectrum of related rare genetic syndromes caused by NLRP3 mutations including familial cold autoinflammatory syndrome (FCAS), Muckle‑Wells syndrome (MWS), and neonatal onset multisystem inflammatory disease (NOMID/CINCA)

Spectrum conceptCAPS syndromes are considered one condition along a spectrum of disease severity with IL‑1 blockade recommended across the spectrum

Clinical management noteDose and/or frequency of IL‑1 blockade should be adjusted to control disease activity, normalize inflammation markers, and support appropriate growth in pediatric patients

Still's disease — SJIA and AOSD (collective term and age notes)

DefinitionStill's disease is a collective term encompassing systemic juvenile idiopathic arthritis (SJIA) and adult‑onset Still's disease (AOSD); they are considered the same disease differing by age of onset

Policy Summary

PayerCigna

PolicyIlaris Prior Authorization Policy

Policy CodePolicy IP0235

Change TypeMaterial revisions to criteria, dosing, and review pathways

Effective DateMay 1, 2026

Next Review DateN/A

SourceLink

On This Page

Familial Mediterranean Fever (FMF): pediatric and adult patients; minimum age for FMF initiation noted as ≥ 2 years in criteria where specified

HIDS/MKD age thresholdHyperimmunoglobulin D syndrome (HIDS)/Mevalonate kinase deficiency (MKD): patient must be ≥ 2 years of age for initial therapy criteria

Still's disease / SJIA and AOSD groupingStill's disease is a collective term for SJIA and AOSD; SJIA and AOSD considered the same disease differing by age of onset (SJIA ≥ 2 years; AOSD ≥ 18 years)

AOSD and gout age thresholdsAdult‑onset Still's disease (AOSD) and gout indications require patients ≥ 18 years of age

Dosing / frequency thresholds

Weight‑based dosing (≤ 40 kg)Approve up to 4 mg/kg per dose for patients ≤ 40 kg; administered subcutaneously no more frequently than once every 4 weeks

Maximum per‑dose cap (> 40 kg)For patients > 40 kg, approve up to 300 mg per dose administered subcutaneously no more frequently than once every 4 weeks

Overall frequency limitDosing frequency: no more often than once every 4 weeks (q4w) for all approved regimens

Initial approvals: generally 6 months
Continuation approvals: generally 1 year (patient established ≥ 6 months)
Continuation requires objective response or symptom improvement versus baseline

Billing Rule

Prior Authorization and Dosing Requirements

Prior authorization is required for all Ilaris dosing. Weight‑based and fixed dosing limits in this policy must be followed: for patients ≤ 40 kg approve up to 4 mg/kg per dose subcutaneously no more frequently than once every 4 weeks (up to a maximum of 300 mg per dose where noted); for patients > 40 kg approve up to 300 mg per dose subcutaneously no more frequently than once every 4 weeks. For gout flares the approved regimen is up to 150 mg subcutaneously no more frequently than once every 12 weeks. Requests that exceed these dosing or frequency limits must be justified and will be reviewed individually.

≤ 40 kg: up to 4 mg/kg per dose, SC, ≥ 4‑week interval (max per policy where specified)
> 40 kg: up to 300 mg per dose, SC, no more frequently than every 4 weeks
Gout, acute flare: up to 150 mg SC no more frequently than every 12 weeks
Prior authorization required for all dosing; deviations reviewed case‑by‑case

Prior Authorization

Colchicine Requirement for FMF

For Familial Mediterranean Fever (FMF) indications, the patient must have tried colchicine at the maximum tolerated dose prior to initiating Ilaris unless colchicine is contraindicated or not tolerated. Documentation of colchicine trial and dosing is required.

Colchicine trial at maximum tolerated dose required unless contraindicated or not tolerated
Documentation of colchicine use/dose required

Prior Authorization

Conventional Therapy Requirement for Gout

For gout (acute flare) indication, the patient must have trialed and had intolerance or contraindication or lack of response to both NSAIDs and colchicine for acute gout flares, unless the alternative pathway is met (prior corticosteroid use with inability to retreat). Patients should be receiving or will receive concomitant urate‑lowering therapy for prevention unless contraindicated. Rheumatology consult/prescription is required.

Trial/intolerance/contraindication to NSAIDs and colchicine required OR prior corticosteroid treatment with inability to retreat
Concomitant urate‑lowering therapy required unless contraindicated (e.g., allopurinol, febuxostat, probenecid)
Prescribed by or in consultation with a rheumatologist

Step Therapy

Step Therapy / Prior Biologic Requirements (Still's Disease)

For Still's disease indications the policy operationalizes prior biologic considerations: historically a trial of one other biologic or starting Ilaris in the hospital were included in initial criteria; the current policy may require documentation of prior biologic therapy or follow the specific initial vs maintenance pathways noted in the Still's disease sections. Check the specific indication criteria for whether a prior biologic is required or if the patient qualifies under the initial therapy pathway.

Operationalizes maintenance vs initial therapy review pathways for Still's disease (AOSD and SJIA)
Prior biologic trial may be required per indication history—refer to the specific indication criteria and recent revision history

Billing Rule

Coding Mismatch / Not Covered

Claims submitted for services that are not accompanied by covered diagnosis or procedure codes listed in this policy will be denied as not covered. When billing, providers must use the most appropriate codes as of the date of service.

Claims without covered diagnosis/procedure codes will be denied
Use most appropriate, current codes when submitting claims

Denial Risk

Conditions Not Covered — Denial Risks

Ilaris is considered not medically necessary (not covered) for uses not specifically listed as covered in this policy. Notable denial risks include concurrent use with another biologic agent for an inflammatory condition (concomitant IL‑1 blockade or other biologics), rheumatoid arthritis (efficacy not established), and other indications listed in the 'Conditions Not Covered' section. Review the Conditions Not Covered list before authorizing therapy.

Concurrent biologic therapy (co‑administration with other biologics/IL‑1 blockers) is not recommended and is a denial risk
Rheumatoid arthritis is not covered (efficacy not established)
Other non‑listed uses are considered not medically necessary per the policy

Age‑based noteFor AOSD the initial therapy criteria apply to patients ≥ 18 years; for SJIA criteria apply to patients ≥ 2 years

Guideline recommendationEULAR/PReS guidelines treat SJIA and AOSD as the same disease and recommend initiating IL‑1 or IL‑6 inhibition early after diagnosis

Objective measures — examples of response

Examples of objective measuresResolution of fever, improvement in rash or skin manifestations, and clinically significant improvement or normalization of serum markers (e.g., CRP, ESR, amyloid A)

Additional objective examplesDecreased frequency of attacks, reduction in proteinuria, or stabilization of serum creatinine can be used as objective measures of response

Symptom improvement examplesFewer attacks, less joint pain/tenderness/stiffness/swelling, decreased fatigue, and improved function or activities of daily living are acceptable measures of clinical improvement