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This medical policy (medical coverage guideline) is Copyright 2017, Blue Cross and Blue Shield of Florida (BCBSF). All Rights Reserved. You may not copy or use this document or disclose its contents without the express written permission of BCBSF. The medical codes referenced in this document may be proprietary and owned by others. BCBSF makes no claim of ownership of such codes. Our use of such codes in this document is for explanation and guidance and should not be construed as a license for their use by you. Before utilizing the codes, please be sure that to the extent required, you have secured any appropriate licenses for such use. Current Procedural Terminology (CPT) is copyright 2017 American Medical Association. All Rights Reserved. No fee schedules, basic units, relative values, or related listings are included in CPT. The AMA assumes no liability for the data contained herein. Applicable FARS/DFARS restrictions apply to government use. CPT® is a trademark of the American Medical Association. The use of specific product names is illustrative only. It is not intended to be a recommendation of one product over another, and is not intended to represent a complete listing of all products available.

09-J2000-04

Original Effective Date: 10/15/13

Reviewed: 09/14/16

Revised: 11/15/16

Next Review: No Longer Scheduled for Routine Review (NLR)

Subject: Rilonacept (Arcalyst®) Injection

THIS MEDICAL COVERAGE GUIDELINE IS NOT AN AUTHORIZATION, CERTIFICATION, EXPLANATION OF BENEFITS, OR A GUARANTEE OF PAYMENT, NOR DOES IT SUBSTITUTE FOR OR CONSTITUTE MEDICAL ADVICE. ALL MEDICAL DECISIONS ARE SOLELY THE RESPONSIBILITY OF THE PATIENT AND PHYSICIAN. BENEFITS ARE DETERMINED BY THE GROUP CONTRACT, MEMBER BENEFIT BOOKLET, AND/OR INDIVIDUAL SUBSCRIBER CERTIFICATE IN EFFECT AT THE TIME SERVICES WERE RENDERED. THIS MEDICAL COVERAGE GUIDELINE APPLIES TO ALL LINES OF BUSINESS UNLESS OTHERWISE NOTED IN THE PROGRAM EXCEPTIONS SECTION.

           
Dosage/ Administration Position Statement Billing/Coding Reimbursement Program Exceptions Definitions
           
Related Guidelines Other References Updates  
           

DESCRIPTION:

Rilonacept is a dimeric fusion protein consisting of the ligand-binding domains of the extracellular portions of the human interleukin-1 type I receptor (IL-1RI) and IL-1 receptor accessory protein (IL-1RAcP) linked in-line to the Fc portion of human immunoglobulin G1. The drug is an interleukin-1 (IL-1) receptor antagonist (IL-1Ra) FDA-approved for the management of cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome (FCAS) and Muckle-Wells syndrome (MWS) in adults and children 12 years of and older.

Cryopyrin-associated periodic syndromes are rare genetic syndromes generally caused by mutations in the NLRP-3 gene. Inflammation in CAPS is usually associated with mutations in the NLRP-3 gene that encodes the protein cryopyrin, which is an important component of the inflammasome. Cryopyrin regulates the protease caspase-1 and controls the activation of interleukin-1 beta (IL-1b). Mutations in the NLRP-3 gene result in an overactive inflammasome, which causes excessive release of activated IL-1b. Rilonacept blocks IL-1b signaling by acting as a soluble decoy receptor that binds IL-1b and prevents its interaction with cell surface receptors. The drug also binds interleukin-1 alpha (IL-1a) and interleukin-1 receptor antagonist (IL-1Ra) to a lesser extent.

Rilonacept received orphan designation in 2013 for the treatment of familial Mediterranean fever.

POSITION STATEMENT:

Comparative Effectiveness

The Food and Drug Administration has deemed the drug(s) or biological product(s) in this coverage policy to be appropriate for self-administration or administration by a caregiver (i.e., not a healthcare professional). Therefore, coverage (i.e., administration) in a provider-administered setting such as an outpatient hospital, ambulatory surgical suite, physician office, or emergency facility is not considered medically necessary.

I. Initiation of rilonacept (Arcalyst) meets the definition of medical necessity for members meeting the following criteria:

1. Member is diagnosed with Cryopyrin-Associated Periodic Syndrome (CAPS) or Cold Induced Auto-inflammatory Syndrome (CAIS) including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS)

2. There is clinical documentation of functional impairment resulting in limitations of activities of daily living

3. Rilonacept is NOT being administered with a tumor necrosis factor (TNF) inhibitor or an interleukin (IL)-1 blocker, including (but not limited to) ANY of the following:

a. Abatacept (Orencia®)

b. Adalimumab (Humira®)

c. Anakinra (Kineret®)

d. Canakinumab (Ilaris®)

e. Certolizumab (Cimzia®)

f. Etanercept (Enbrel®)

g. Golimumab (Simponi®)

h. Infliximab (Remicade®)

i. Ixekizumab (Taltz®)

j. Secukinumab (Cosentyx®)

k. Tocilizumab (Actemra®)

l. Tofacitinib (Xeljanz®)

m. Ustekinumab (Stelara®)

4. The loading dose does not exceed 320 mg, and subsequent dosing does not exceed 160 mg weekly

5. The member is 12 years of age and older.

Duration of approval: 12 months

II. Rilonacept meets the definition of medical necessity when used as a single agent for the following designated Orphan Drug indication (http://www.fda.gov/orphan/designat/list.htm) when the dose does not exceed the maximum FDA-approved dose:

1. Treatment of familial Mediterranean fever (FMF)

Duration of approval: 12 months

III. Continuation of rilonacept (Arcalyst) injection meets the definition of medical necessity for members meeting ALL of the following criteria:

1. Member has a history of beneficial clinical response with rilonacept therapy for the treatment of ONE of the following indications:

a. Cryopyrin-Associated Periodic Syndrome (CAPS) or Cold Induced Auto-inflammatory Syndrome (CAIS) including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS)

b. Familial Mediterranean fever

2. The member has been previously approved by Florida Blue or another healthplan in the past 2 years, OR the member has previously met all indication-specific criteria.

3. Rilonacept NOT being administered with a tumor necrosis factor (TNF) inhibitor or an interleukin (IL)-1 blocker, including (but not limited to) ANY of the following:

a. Abatacept (Orencia®)

b. Adalimumab (Humira®)

c. Anakinra (Kineret®)

d. Canakinumab (Ilaris®)

e. Certolizumab (Cimzia®)

f. Etanercept (Enbrel®)

g. Golimumab (Simponi®)

h. Infliximab (Remicade®)

i. Ixekizumab (Taltz®)

j. Secukinumab (Cosentyx®)

k. Tocilizumab (Actemra®)

l. Tofacitinib (Xeljanz®)

m. Ustekinumab (Stelara®)

4. The loading dose does not exceed 320 mg, and subsequent dosing does not exceed 160 mg weekly

Duration of approval: 12 months

DOSAGE/ADMINISTRATION:

THIS INFORMATION IS PROVIDED FOR INFORMATIONAL PURPOSES ONLY AND SHOULD NOT BE USED AS A SOURCE FOR MAKING PRESCRIBING OR OTHER MEDICAL DETERMINATIONS. PROVIDERS SHOULD REFER TO THE MANUFACTURER’S FULL PRESCRIBING INFORMATION FOR DOSAGE GUIDELINES AND OTHER INFORMATION RELATED TO THIS MEDICATION BEFORE MAKING ANY CLINICAL DECISIONS REGARDING ITS USAGE.

FDA-approved

Age 18 years or older

Age 12 to 17 years

Dose Adjustments

None

Drug Availability

PRECAUTIONS:

Boxed Warning

None

Contraindications

None

Precautions/Warnings

BILLING/CODING INFORMATION:

The following codes may be used to describe:

HCPCS Coding:

J2793

Injection, rilonacept, 1 mg

ICD-10 Diagnoses Codes That Support Medical Necessity: (Effective 10/01/15)

E85.0

Non-neuropathic heredofamilial amyloidosis

L50.2

Urticaria due to cold and heat

M04.1

Periodic fever syndromes

M04.2

Cryopyrin-associated periodic syndromes

M04.8

Other autoinflammatory syndromes

M04.9

Autoinflammatory syndrome, unspecified

REIMBURSEMENT INFORMATION:

Refer to section entitled POSITION STATEMENT.

PROGRAM EXCEPTIONS:

Federal Employee Program (FEP): Follow FEP guidelines.

State Account Organization (SAO): Follow SAO guidelines.

Medicare Advantage: No National Coverage Determination (NCD) and/or Local Coverage Determination (LCD) were found at the time this guideline was drafted.

DEFINITIONS:

Cryopyrin-Associated Periodic Syndrome (CAPS) aka Cold Induced Auto-inflammatory Syndrome (CAIS1): is a spectrum of autoinflammatory syndromes including familial cold autoinflammatory syndrome (FCAS, formerly termed familial cold-induced urticaria), the Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID, also called chronic infantile neurologic cutaneous and articular syndrome or CINCA).

Familial cold autoinflammatory syndrome (FCAS): is an autosomal dominant condition characterized by rash, conjunctivitis, fever/chills and arthralgias elicited by exposure to cold.

Familial Mediterranean fever (FMF): is an inherited autosomal recessive disease characterized by recurrent episodes of painful inflammation in the abdomen, chest, or joints. These episodes are often accompanied by fever and sometimes a rash.

Muckle-Wells syndrome (MWS): is a rare autosomal dominant disease which causes sensorineural deafness, recurrent hives, and can lead to amyloidosis.

RELATED GUIDELINES:

Canakinumab (Ilaris®) Injection, 09-J2000-03

OTHER:

None

REFERENCES:

  1. AHFS Drug Information. Bethesda (MD): American Society of Health-System Pharmacists, Inc; 2016[cited2016 Aug 17]. In: STAT!Ref Online Electronic Medical Library [Internet]. Available from: http://online.statref.com/.
  2. Clinical Pharmacology [Internet]. Tampa (FL): Gold Standard, Inc.; 2016 [cited 2016 Aug 17]. Available from: http://www.clinicalpharmacology.com/.
  3. DRUGDEX® System [Internet]. Greenwood Village (CO): Thomson Micromedex; Updated periodically [cited 2016 Aug 17]. Available from: http://www.thomsonhc.com/.
  4. Arcalyst® [package insert]. Tarrytown, NY: Regeneron Pharmaceuticals, Inc.; February 2015.
  5. FDA Orphan Drug Designations and Approvals [Internet]. Washington, D.C. [cited 2016 Aug 19]. Available from: http://www.accessdata.fda.gov/scripts/opdlisting/oopd/.

COMMITTEE APPROVAL:

This Medical Coverage Guideline (MCG) was approved by the BCBSF Pharmacy Coverage Committee on 09/14/16.

GUIDELINE UPDATE INFORMATION:

10/15/13

New Medical Coverage Guideline.

10/15/14

Review and revision to guideline; consisting of updating position statement, dosage/administration, precautions, references.

10/15/15

Review and revision to guideline; consisting of updating position statement, precautions, coding, references.

11/01/15

Revision: ICD-9 Codes deleted.

10/01/16

Update to ICD-10 codes.

10/15/16

Review and revision to guideline; consisting of updating position statement and references.

11/15/16

Revision to guideline consisting of updating position statement and coding.

Date Printed: October 20, 2017: 02:04 PM