CurrentCentenePolicy CP PHAR.266

Rilonacept (Arcalyst)

Policy governing medical necessity criteria, dosing limits, prior authorization and continued therapy requirements for rilonacept (Arcalyst) across Commercial, HIM, and Medicaid lines of business for FDA-approved indications (CAPS: FCAS and MWS; DIRA; recurrent pericarditis) including contraindications for combination immunosuppressive therapy.

Policy Summary

PayerCentene

PolicyRilonacept (Arcalyst)

Policy CodePolicy CP PHAR.266

Change TypeCriteria additions and revisions (2021–2025)

Effective DateNov 16, 2016

Next Review Date

Key ActionProvider must submit chart notes, lab results or other clinical information supporting that member has met all approval criteria.

POLICY UPDATE CHANGES

Added criteria for new FDA indication: treatment of recurrent pericarditis and reduction in risk of recurrence in adults and pediatric patients 12 years and older.

Added criteria for DIRA indication and requirements to confirm diagnosis/severity for CAPS.

Reiterated requirement against combination use with bDMARDs or JAKi across initial and continued therapy sections.

Updated Section III.B to include additional biologics and biosimilar verbiage (Spevigo and others) and updated references.

3FDA-approved Indications Covered

320 mgMax Loading Dose

160 mgStandard Weekly Dose

Coverage Summary & Scope

Scope: This policy governs medical necessity criteria, dosing limits, prior authorization and continued therapy requirements for rilonacept (Arcalyst) across Commercial, HIM, and Medicaid lines of business for FDA-approved indications: CAPS (FCAS and MWS), DIRA, and recurrent pericarditis. Coverage stance: covered_with_criteria. FDA-approved indications summarized: FCAS and MWS (CAPS) for patients ≥ 12 years; maintenance of remission for DIRA in patients ≥ 10 kg; and treatment/reduction in recurrence of recurrent pericarditis for patients ≥ 12 years. Avoid initiation of rilonacept in patients with active or chronic infections.

Key Thresholds

Age — CAPS (FCAS, MWS)≥ 12 years

Age — Recurrent Pericarditis (RP)≥ 12 years

Weight — DIRA≥ 10 kg

Dose limit — Loading320 mg (two injections)

Dose limit — Maintenance160 mg once weekly (single injection)

DIRA dosing limit4.4 mg/kg once weekly (up to 320 mg)

RP recurrence intervalSymptom-free interval of ≥ 4 weeks after an acute episode

Initial Therapy Criteria

Initial Approval Criteria - CAPS (FCAS or MWS)

Arcalyst is medically necessary when ALL of the following are met:

ALL of the following

Diagnosis of FCAS or MWS
Prescribed by or in consultation with a rheumatologist
Age ≥ 12 years
Documentation of one of the following
- For FCAS: classic signs and symptoms (e.g., recurrent, intermittent fever and rash often exacerbated by exposure to generalized cool ambient temperature) AND functional impairment limiting activities of daily living

Continuation Therapy Criteria

Provider Actions & Billing

Prior Authorization

Prior Authorization Required

Requests for Arcalyst (rilonacept) require prior authorization. Prior authorization requests must demonstrate that the member meets the applicable initial or continuation criteria in this policy and that dosing, age, diagnosis, prescriber specialty, and weight-based requirements (when applicable) are satisfied before coverage will be approved.

Affected code: J2793 (rilonacept)
Prescriber requirement: prescribed by or in consultation with the appropriate specialist (rheumatologist for CAPS/DIRA; cardiologist or rheumatologist for recurrent pericarditis)
Diagnosis requirement: request must correspond to an FDA‑approved indication listed in the policy (CAPS [FCAS or MWS], DIRA in remission, or recurrent pericarditis)
Age/weight/dosing requirements must be met: e.g., CAPS/RP — loading 320 mg (two injections) then 160 mg once weekly (single injection); DIRA — 4.4 mg/kg (up to 320 mg) loading and 2.2 mg/kg (up to 320 mg) once weekly; minimum weight ≥10 kg for DIRA; age limits per indication

Applicable Codes

Drug HCPCSHCPCS

J2793

Injection, rilonacept, mg

Clinical Evidence & References

Evidence: FDA Prescribing Information last accessed: 2021 label and subsequent updates referenced (accessed 2025-02-27). The FDA-approved indications noted in the label include treatment of CAPS (FCAS and MWS), maintenance of remission for DIRA in patients weighing at least 10 kg, and treatment/reduction in risk of recurrence of recurrent pericarditis in patients ≥ 12 years. The label and policy materials advise to not initiate Arcalyst in patients with active or chronic infections.

Background & Definitions

Background: Rilonacept (Arcalyst) is an interleukin-1 (IL-1) blocker. It is FDA-indicated for CAPS (including FCAS and MWS) in patients ≥ 12 years, for maintenance of remission in DIRA in patients weighing ≥ 10 kg, and for treatment and reduction of recurrence in recurrent pericarditis in patients ≥ 12 years. Dosage guidance in the product information includes a 320 mg loading dose (two injections) and 160 mg once weekly maintenance for most indications, with DIRA dosing per weight (up to 4.4 mg/kg, max 320 mg). The labeling and policy explicitly warn: do not initiate Arcalyst in patients with active or chronic infections.

Definitions: CAPS — cryopyrin-associated periodic syndromes (FCAS, MWS, NOMID/CINCA).

DIRA — deficiency of interleukin-1 receptor antagonist; confirmed by loss-of-function IL1RN mutations.

bDMARD — biological disease-modifying antirheumatic drugs (policy enumerates specific agents).

JAKi — Janus kinase inhibitors.

Revision History

2021added (material)

Criteria added for new FDA indication: treatment of recurrent pericarditis and reduction in risk of recurrence in adults and pediatric patients 12 years and older; references reviewed and updated; P&T approval noted.

2Q2021revised (material)

Added criteria for DIRA indication and requirements to confirm diagnosis/severity for CAPS; added combination of bDMARDs under Section III; references updated; P&T approval recorded.

2Q2022revised (material)

Reiterated requirement against combination use with bDMARDs or JAKi by moving prohibition from Section III into Sections I and II; references reviewed and updated; P&T approval recorded.

Policy Summary

PayerCentene

PolicyRilonacept (Arcalyst)

Policy CodePolicy CP PHAR.266

Change TypeCriteria additions and revisions (2021–2025)

Effective DateNov 16, 2016

Next Review Date

Key ActionProvider must submit chart notes, lab results or other clinical information supporting that member has met all approval criteria.

On This Page

For MWS: classic signs and symptoms (e.g., chronic fever and rash of waxing and waning intensity, sometimes exacerbated with exposure to generalized cool ambient temperature) AND functional impairment limiting activities of daily living

Member does not have combination use with biological disease-modifying antirheumatic drugs (bDMARDs) or Janus kinase inhibitors (JAKi)

See Section III for specific agents not authorized

Dose limits

Loading dose: 320 mg (two injections)
Maintenance dose: 160 mg once weekly (single injection)
Dose does not exceed both 320 mg loading and 160 mg weekly maintenance

Documentation Required

Submit supporting documentation

Submit clinical records that support the medical necessity criteria being requested. Include documentation that verifies diagnosis, prior therapies/trials, prescriber specialty, dosing and weight calculations, and objective evidence of response or stability for continuation requests.

Office chart notes/consultation notes (including specialist documentation)
Relevant laboratory results (e.g., inflammatory markers such as CRP, serum amyloid A)
Genetic testing confirming IL1RN loss‑of‑function mutation for DIRA requests
Medication history showing prior therapies tried, intolerances, or contraindications (e.g., NSAID/colchicine trials for recurrent pericarditis)
Treatment plan showing dosing calculations and weight for pediatric patients

Denial Risk

Denial risk for combination therapy

Coverage is NOT authorized for combination use of Arcalyst with biological disease‑modifying antirheumatic drugs (bDMARDs), Janus kinase inhibitors (JAKi), anti‑CD20 agents, potent immunosuppressants, or other agents listed in Section III due to additive immunosuppression and increased risk of neutropenia and serious infections. Combination use across initial and continued therapy is prohibited and may result in claim denial.

Combination use with bDMARDs (e.g., TNF antagonists, IL‑6/IL‑1/IL‑23/IL‑17 inhibitors, co‑stimulation modulators, integrin antagonists) is not allowed
Combination use with JAK inhibitors (e.g., tofacitinib/Xeljanz, baricitinib/Olumiant, upadacitinib/Rinvoq) is not allowed
Combination use with potent immunosuppressants (e.g., rituximab, TYK2 inhibitors, S1P modulators) is not allowed
Combination prohibition applies to concurrent therapy and to combination across initial and continued therapy periods

Billing Rule

Dose billing

Bill rilonacept using HCPCS code J2793 and follow per‑mg billing guidance. Ensure the billed dose reflects the actual administered dose and does not exceed policy maximums or the approved regimen.

HCPCS code: J2793 — rilonacept
Dose caps per policy: loading dose maximum = 320 mg (administered as two injections); maintenance dose maximum = 160 mg once weekly for CAPS and recurrent pericarditis
DIRA dosing cap: 4.4 mg/kg loading (up to 320 mg) and 2.2 mg/kg once weekly (up to 320 mg) — ensure mg billed corresponds to weight‑based calculation and does not exceed 320 mg

2Q2024revised

Updated Section III.B by adding multiple biologic agents (Bimzelx, Zymfentra, Omvoh, Wezlana, Sotyktu, Tofidence, Velsipity) and removed supplemental concomitant-use information in Appendix D; references reviewed and updated; P&T approval recorded.

2Q2025revisedLatest

No significant policy changes; updated Section III.B with Spevigo and biosimilar verbiage and reviewed/updated references; P&T approval recorded.