Sierra Health and Life Ilaris Coverage Update

Medical Necessity and Indication Criteria

Cryopyrin-Associated Periodic Syndromes (CAPS) including FCAS and MWS

Covered when ALL of the following are met

CAPS initial therapy

diagnosis: Diagnosis of FCAS or MWS by, or in consultation with, a rheumatologist or immunologist with expertise
dosing and duration: Ilaris dosing for FCAS/MWS is in accordance with the United States Food and Drug Administration approved labeling; initial authorization will be for no more than 12 months

CAPS continuation

current therapy and response: Patient is currently on Ilaris therapy for FCAS or MWS and documentation of positive clinical response to Ilaris therapy
dosing and duration: Ilaris dosing for FCAS/MWS is in accordance with the United States Food and Drug Administration approved labeling; reauthorization will be for no more than 12 months

_note: References: Kuemmerle-Deschner et al (2011) and Lachmann et al (2009) support efficacy and CRP/SAA normalization in CAPS

TRAPS (Tumor Necrosis Factor Receptor-Associated Periodic Syndrome)

Covered when ALL of the following are met

TRAPS initial therapy: Diagnosis of TRAPS by, or in consultation with, a rheumatologist or immunologist with expertise in the diagnosis of TRAPS; Ilaris dosing for TRAPS is in accordance with the United States Food and Drug Administration approved labeling; initial authorization will be for no more than 12 months

TRAPS continuation: Patient is currently receiving Ilaris therapy for TRAPS with documentation of a positive clinical response; Ilaris dosing for TRAPS is in accordance with the United States Food and Drug Administration approved labeling; reauthorization will be for no more than 12 months

HIDS/MKD (Hyper-IgD Syndrome / Mevalonate Kinase Deficiency)

Covered when ALL of the following are met

HIDS/MKD initial therapy: Diagnosis of HIDS or MKD by, or in consultation with, a rheumatologist or immunologist with expertise in the diagnosis of HIDS or MKD; Ilaris dosing for HIDS/MKD is in accordance with the United States Food and Drug Administration approved labeling; initial authorization will be for no more than 12 months

HIDS/MKD continuation: Patient is currently receiving Ilaris for HIDS or MKD with documentation of a positive clinical response to therapy; Ilaris dosing for HIDS/MKD is in accordance with the United States Food and Drug Administration approved labeling; reauthorization will be for no more than 12 months

Familial Mediterranean Fever (FMF)

Covered when ALL of the following are met

FMF initial therapy: Diagnosis of FMF by, or in consultation with, a rheumatologist or immunologist with expertise in the diagnosis of FMF; history of failure, contraindication, or intolerance to colchicine; Ilaris dosing for FMF is in accordance with the United States Food and Drug Administration approved labeling; initial authorization will be for no more than 12 months

FMF continuation: Patient is currently receiving Ilaris for FMF with documentation of a positive clinical response to therapy; Ilaris dosing for FMF is in accordance with the United States Food and Drug Administration approved labeling; reauthorization will be for no more than 12 months

Active Still's Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA)

Covered when ALL of the following are met

AOSD/SJIA initial therapy: Diagnosis of AOSD or SJIA by, or in consultation with, a rheumatologist or immunologist with expertise in the diagnosis of AOSD or SJIA; Ilaris dosing for AOSD/SJIA is in accordance with the United States Food and Drug Administration approved labeling; patient is not receiving Ilaris in combination with a targeted immunomodulator; initial authorization will be for no more than 12 months

AOSD/SJIA continuation: Patient is currently receiving Ilaris for AOSD/SJIA with documentation of a positive clinical response to therapy; Ilaris dosing for AOSD/SJIA is in accordance with the United States Food and Drug Administration approved labeling; patient is not receiving Ilaris in combination with a targeted immunomodulator; reauthorization will be for no more than 12 months

Gout Flares

Covered when ALL of the following are met

gout initial: Diagnosis of a gout flare; history of contraindication, intolerance, or treatment failure with both colchicine and an NSAID; provider attests that the patient is not an appropriate candidate for systemic corticosteroids; Ilaris dosing is in accordance with the United States Food and Drug Administration labeled dosing for gout flares; authorization will be issued for one dose for 12 weeks

gout prescriber: For gout flares, prescribed by a rheumatologist or nephrologist

Coverage criteria (FDA-aligned and gout therapy sequencing)

Covered when clinical indication matches FDA labeling or demonstrated evidence in trials and member benefit permits:

FDA-indicated conditions: Diagnosis is one of the FDA-indicated conditions (CAPS, TRAPS, HIDS/MKD, FMF, SJIA, AOSD) or symptomatic gout flares when NSAIDs and colchicine are contraindicated, not tolerated, or ineffective and repeated corticosteroids are not appropriate.

Supports: FDA indications and trial evidence

Gout flare prior therapy: For gout flares, patient has documented contraindication, intolerance, or inadequate response to NSAIDs and colchicine, and repeated corticosteroids are not appropriate.

Per FDA label and ACR gout guideline conditional recommendation

FDA Indications

Covered when consistent with the FDA-labeled indications and member-specific benefit plan:

FDA labeled indications: Treatment of CAPS (including FCAS and MWS) in adults and children 4 years of age and older; TRAPS, HIDS/MKD, FMF in adult and pediatric patients; active Still's disease (AOSD and SJIA) in patients aged 2 years and older; symptomatic treatment of gout flares in adults when NSAIDs and colchicine are contraindicated, not tolerated, or ineffective and repeated courses of corticosteroids are not appropriate.

Informational — actual plan criteria and prior authorization requirements may further restrict coverage.

Requests for Ilaris (canakinumab) to treat cardiovascular disease are outside the scope of coverage in this policy. The policy states that Ilaris is not proven or medically necessary for the management or treatment of cardiovascular disease, and such requests may be denied when submitted for that indication.

Coverage for off-label or experimental uses of Ilaris depends on the member's specific Certificate of Coverage and any applicable state mandates. Some Certificates permit coverage of investigational uses under defined circumstances, and certain state off‑label mandates can supersede plan language; therefore, confirm the member-specific benefit document and applicable mandates before authorizing off-label use.

FDA approval alone does not guarantee coverage. This policy notes that FDA approval alone is not a basis for coverage; authorization decisions must reference the member-specific benefit plan and any federal or state mandates, which govern when their terms conflict with this policy.

Reiterating exclusions: the policy explicitly indicates that Ilaris is not proven or medically necessary for cardiovascular disease management. Requests for canakinumab for cardiovascular indications should be reviewed against this policy and the member's benefit plan and are subject to denial.

For gout flares, IL‑1 inhibitors including canakinumab are positioned after first‑line therapies. The policy requires a documented history of contraindication, intolerance, or treatment failure with both colchicine and an NSAID, and provider attestation that systemic corticosteroids are not appropriate. This aligns with guideline recommendations that prioritize oral colchicine, NSAIDs, or glucocorticoids as first‑line therapy; IL‑1 inhibition is a conditional option when those agents are not suitable.

This policy is intended as an interpretive guide for UnitedHealthcare standard benefit plans and does not substitute for the member-specific benefit document or constitute medical advice. Prior authorization decisions should reference the member's plan terms and any applicable federal or state requirements.

Applicable Billing and Diagnosis Codes

Applicable HCPCS CodesHCPCSCovered

J0638

Injection, canakinumab, 1 mg

Applicable ICD-10 Codes (partial list provided)ICD-10

M04.1	Periodic fever syndromes.
M04.2	Cryopyrin-associated periodic syndromes.
M06.1	Adult-onset Still's disease.
M08.20	Juvenile rheumatoid arthritis with systemic onset, unspecified site.
M10.00	Idiopathic gout, unspecified site.

Applicable ICD-10-CM CodesICD-10

M10.222	Drug-induced gout, left elbow
M10.229	Drug-induced gout, unspecified elbow
M10.231	Drug-induced gout, right wrist
M10.232	Drug-induced gout, left wrist
M10.239	Drug-induced gout, unspecified wrist
M10.241	Drug-induced gout, right hand
M10.242	Drug-induced gout, left hand
M10.249	Drug-induced gout, unspecified hand
M10.251	Drug-induced gout, right hip
M10.252	Drug-induced gout, left hip

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FDA Indications (informational — no billing codes provided in this excerpt)mixed

No codes listed

inv-19: CRP / SAA normalization value — lab threshold referenced

CRP / SAA normalization threshold< 10 mg/L

ContextReferenced as a laboratory normalization target for autoinflammatory disease monitoring (CRP and SAA).

Source trial supportUsed in SJIA/AOSD trials and long-term follow-up assessments as a marker of systemic inflammation response to canakinumab.

inv-20: SJIA inclusion CRP threshold

SJIA inclusion CRP threshold> 30 mg/L

Study contextTrial 1 enrolled SJIA patients with CRP > 30 mg per liter as part of inclusion criteria.

Age range in trialPatients aged 2-19 years in the referenced SJIA trial.

inv-21: Pediatric age threshold for SJIA and Still's disease

Pediatric threshold for SJIA/Still's diseasePatients aged 2 years and older

Clinical trial supportSJIA trials enrolled patients aged 2-19 years (supports pediatric threshold).

FDA alignmentFDA indicates Ilaris for active Still's disease (AOSD/SJIA) in patients aged 2 years and older.

inv-22: Pediatric age threshold for CAPS and other autoinflammatory indications

Pediatric threshold for CAPS and other autoinflammatory indicationsPatients 4 years of age and older (for CAPS)

FDA context for CAPSIlaris is indicated for CAPS in adults and children 4 years of age and older, including FCAS and MWS.

Background—genetic basisCAPS disorders are caused by NLRP3 mutations leading to inflammasome overactivation and excess IL-1β.

Authorization, Documentation, and Step Therapy Requirements

Prior Authorization

Prior Authorization Required

Prior authorization required. Verify the requested indication aligns with the FDA-labeled uses or the specific policy-listed conditions and confirm member benefits before submitting a request. Reference the member's Certificate of Coverage when benefit language conflicts with this policy.

Check member-specific benefit plan for coverage limits or state-mandated exceptions.
If plan language conflicts with this policy, the member-specific Certificate of Coverage governs.
Document that the indication matches an FDA-approved or policy-specified indication (e.g., CAPS, TRAPS, HIDS/MKD, FMF, AOSD/SJIA, or gout flare per FDA criteria).

Documentation Required

Diagnosis Coding and Documentation

Document the clinical diagnosis using the appropriate ICD-10 code from the policy's applicable codes list and include supporting clinical information in the authorization request.

Include the ICD-10 diagnosis code that corresponds to the patients condition (see policy applicable codes for gout and other included diagnoses).
Attach relevant clinical notes, consultation reports, or specialist documentation that support the diagnosis and treatment plan.

Step Therapy

Step Therapy Requirements

Step therapy expectations must be met prior to approval where specified. For Familial Mediterranean Fever (FMF), document history of failure, intolerance, or contraindication to colchicine. For gout flares, document trials of first-line therapies per guideline recommendations.

FMF: History of failure, intolerance, or contraindication to colchicine is required for initial therapy.
Gout flares: Document trial and inadequate response or contraindication/intolerance to both colchicine and an NSAID before IL-1 inhibition is considered.
When colchicine is used for gout, low-dose colchicine is preferred per guideline recommendations.

Step Therapy

Gout Flare Prior Therapy Expectations

For gout flares, prior therapy expectations align with guideline and FDA-directed positioning of IL-1 inhibitors as later-line therapy. IL-1 inhibition is appropriate only after failure, intolerance, or contraindication to first-line agents and when corticosteroids are not appropriate.

Patient must have documented contraindication, intolerance, or treatment failure with both colchicine and an NSAID.
Provider must attest the patient is not an appropriate candidate for systemic corticosteroids or repeated corticosteroid courses are not appropriate.
Ilaris dosing must follow FDA-labeled dosing for gout flares; authorization is for one dose covering 12 weeks per policy.
Prescriber for gout flares should be a rheumatologist or nephrologist as required by the policy.

Documentation Required

Provider Attestation and Required Clinical Documentation

Include specialist involvement and required clinical elements in the request to reduce risk of denial. Many indications require diagnosis by or consultation with a rheumatologist or immunologist and documentation of prior therapy trials and response.

For CAPS, TRAPS, HIDS/MKD, FMF: diagnosis by or in consultation with a rheumatologist or immunologist with relevant expertise is required for initial authorization.
For continuation, document current treatment with Ilaris and objective evidence of clinical benefit.
Note: Ilaris is not considered proven or medically necessary for cardiovascular disease; requests for this indication may be denied.

Condition Definitions and Abbreviations

inv-37: Named autoinflammatory and rheumatologic conditions listed in the policy (CAPS, TRAPS, HIDS/MKD, FMF, AOSD/SJIA, gout flare)

Named conditions listed for Ilaris coverageCryopyrin-associated periodic syndromes (CAPS, including FCAS and MWS); TRAPS; HIDS/MKD; FMF; Active Still's disease (AOSD and SJIA); Gout flares

Indication groupingPolicy lists these as FDA-indicated or covered-with-criteria uses when diagnosis-specific requirements are met.

Provider requirementDiagnosis should be by or in consultation with a rheumatologist or immunologist for many indications.

inv-38: CAPS definition — Cryopyrin-Associated Periodic Syndromes (contextual definition)

CAPS definition (contextual)Cryopyrin-Associated Periodic Syndromes (CAPS) are rare genetic syndromes generally caused by NLRP3 mutations leading to inflammasome overactivation and excessive IL-1β; includes FCAS and MWS.

Common featuresFever, urticaria-like rash, arthralgia, myalgia, fatigue, and conjunctivitis are common across CAPS disorders.

Inheritance and mechanismCAPS disorders are autosomal dominant; NLRP3 (cryopyrin) mutations cause overactive inflammasome and excess IL-1β-driven inflammation.

inv-39: SJIA — systemic juvenile idiopathic arthritis definition

SJIA definitionSystemic juvenile idiopathic arthritis (SJIA) is a severe autoinflammatory disease driven by innate immunity and proinflammatory cytokines such as IL-1β; characterized by systemic features including fever and active arthritis.

Clinical trial criteriaTrials enrolled patients with active systemic features (fever; ≥2 active joints; CRP > 30 mg/L) demonstrating canakinumab efficacy in SJIA.

Age considerationsSJIA indication and trials include pediatric patients aged 2 years and older.

inv-40: CAPS short definition (alternate chunk)

CAPS (short definition)Cryopyrin-associated periodic syndromes (CAPS): NLRP3-related autoinflammatory disorders including FCAS and MWS characterized by IL-1β-driven inflammation.

Regulatory noteIlaris is indicated for CAPS in adults and children 4 years and older.

Typical symptomsRecurrent fever, rash, arthralgia/myalgia, and systemic inflammation.

inv-41: HIDS/MKD short definition

HIDS/MKD short definitionHyperimmunoglobulin D syndrome (HIDS)/Mevalonate kinase deficiency (MKD): autoinflammatory metabolic disorders characterized by recurrent febrile episodes and inflammation; included among FDA-indicated uses for canakinumab.

Diagnosis noteDiagnosis should be made by or in consultation with a rheumatologist or immunologist with expertise.

Regulatory statusIlaris is indicated for HIDS/MKD in adult and pediatric patients per FDA labeling.

inv-42: Still's disease definition (AOSD and SJIA)

Still's disease definitionStill's disease encompasses adult-onset Still's disease (AOSD) and systemic juvenile idiopathic arthritis (SJIA); both are autoinflammatory disorders driven in part by IL-1β.

Clinical featuresCharacterized by systemic features such as fever and active arthritis; SJIA trials used CRP and systemic signs as inclusion criteria.

Age thresholdAOSD/SJIA indication includes patients aged 2 years and older (per FDA labeling and trials).

OpenPayer

Ilaris (canakinumab) — medical benefit drug policy

Medical Necessity and Indication Criteria

Applicable Billing and Diagnosis Codes

Authorization, Documentation, and Step Therapy Requirements

Drug and Clinical Background

Condition Definitions and Abbreviations