Giant Cell Arteritis

POLICY NUMBER

L.5.01.516

Actemra (tocilizumab)

Rinvoq (upadacitinib)

Tofidence (tocilizumab-bavi)

Tyenne (tocilizumab-aazg)

Please perform a formulary drug search on your patient’s member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific member’s benefit plan.

DESCRIPTION

Giant cell arteritis (GCA), also known as temporal arteritis, is categorized as a vasculitis affecting primarily large- and medium-sized vessels. Many clinical features result from vascular inflammation of the branches of the carotid arteries. Cranial manifestations include headaches, scalp tenderness, vision loss, and jaw claudication; however, patients with large vessel involvement may lack cranial manifestations. GCA most commonly occurs in patients over 50 years of age. Diagnosis is based on clinical presentation, temporal artery biopsy, and/or vascular imaging. Systemic glucocorticoid therapy is recommended for patients with newly diagnosed GCA and may be used with or without glucocorticoid-sparing agents. Tocilizumab, an interleukin-6 inhibitor, and upadacitinib, a Janus kinase inhibitor, are immunomodulators indicated for the treatment of adults with GCA.

Tocilizumab is indicated for the following:

• Treatment of giant cell arteritis (GCA) in adult patients;

• Treatment of adult patients with moderately to severely active rheumatoid arthritis who have had an inadequate response to one or more DMARD;

• Slowing the rate of decline in pulmonary function in adult patients with systemic sclerosis-associated interstitial lung disease;

• Treatment of active polyarticular juvenile idiopathic arthritis in patients 2 years of age and older;

• Treatment of active systemic juvenile idiopathic arthritis in patients 2 years of age and older;

• Treatment of chimeric antigen receptor (CAR) T-cell induced severe or life-threatening cytokine release syndrome in adults and pediatric patients 2 years of age and older;

• Treatment of coronavirus disease 2019 (COVID-19) in hospitalized adult patients who are receiving systemic corticosteroids and require supplemental oxygen, non-invasive or invasive mechanical ventilation, or extracorporeal membrane oxygenation (ECMO).

Upadacitinib is indicated for the following:

• Treatment of adults with moderately to severely active rheumatoid arthritis who have had an inadequate response or intolerance to one or more TNF blockers;

• Treatment of adults and pediatric patients 2 years of age and older with active psoriatic arthritis who have had an inadequate response or intolerance to one or more TNF blockers;

• Treatment of adults and pediatric patients 12 years of age and older with refractory, moderate to severe atopic dermatitis whose disease is not adequately controlled with other systemic drug products, including biologics, or when use of those therapies are inadvisable;

• Treatment of adult patients with moderately to severely active ulcerative colitis who have had an inadequate response or intolerance to one or more TNF blockers;

• Treatment of adult patients with moderately to severely active Crohn’s disease who have had an inadequate response or intolerance to one or more TNF blockers;

• Treatment of adults with active ankylosing spondylitis who have had an inadequate response or intolerance to one or more TNF blockers;

• Treatment of adults with active non-radiographic axial spondyloarthritis with objective signs of inflammation who have had an inadequate response or intolerance to TNF blocker therapy;

• Treatment of patients 2 years of age and older with active polyarticular juvenile idiopathic arthritis (pJIA) who have had an inadequate response or intolerance to one or more TNF blockers;

• Treatment of adults with giant cell arteritis.

 Related policies:

• Anti-Rheumatic Biologicals

• Biosimilar Medications

• Treatment of Coronavirus Disease 2019 (COVID-19)

• Interstitial Lung Disease

POLICY

Prior authorization is required.

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements.

The following medications are not covered on any BCBSMS Formulary:

• Actemra (tocilizumab)

• Tofidence (tocilizumab-bavi)

Initial Criteria

Rinvoq (upadacitinib) and Tyenne (tocilizumab-aazg) may be considered medically necessary when ALL of the following criteria are met:

1. The individual is ≥18 years of age;

2. The individual has a documented diagnosis of giant cell arteritis (GCA) and ALL of the following:

   1. Presence of cranial symptoms (i.e., new-onset localized headache, scalp or temporal artery tenderness, ischemia-related vision loss, or otherwise unexplained mouth/jaw pain upon mastication), constitutional signs/symptoms (i.e., unexplained fever), OR signs/symptoms of vascular abnormalities (i.e., limb claudication, asymmetric blood pressures, vascular bruits);

   2. ONE of the following:

      1. ESR ≥30 mm/hr; OR

      2. CRP ≥1 mg/dL;

   3. Features of GCA and evidence of large-vessel vasculitis supported by temporal artery biopsy and/or imaging [e.g., color Doppler ultrasound (CDUS), high-resolution magnetic resonance imaging (MRI) with MR angiography (MRA)]; AND

   4. The individual is currently receiving systemic glucocorticoids;

3. The individual has been tested for latent TB prior to initiation of the requested agent;

4. The prescriber is a specialist or has consulted with a specialist in an area of the individual's diagnosis (i.e., rheumatologist, radiologist, etc.);

5. The individual is not currently being treated with another immunomodulatory agent (i.e., TNF-inhibitor, IL-inhibitor, anti-IgE antibody, Otezla etc.); 

6. The individual does not have any FDA-labeled contraindication(s) to therapy with the requested agent; AND

7. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval:12 months

Renewal Criteria

Rinvoq (upadacitinib) and Tyenne (tocilizumab-aazg) may be approved for RENEWAL when ALL of the following criteria are met:

1. The individual has been previously approved for therapy with the requested agent through the BCBSMS PA process;

2. The individual has had a clinical response or stabilization from baseline in at least ONE of the following:

   1. Reduction of corticosteroid dose;

   2. Improvement in clinical symptoms; OR

   3. Reduction of ESR or CRP;

3. The prescriber is a specialist or has consulted with a specialist in an area of the individual's diagnosis (i.e., rheumatologist, radiologist, etc.);

4. The individual is not currently being treated with another immunomodulatory agent (i.e., TNF-inhibitor, IL-inhibitor, anti IgE antibody, Otezla, etc.);

5. The individual does not have any FDA-labeled contraindication(s) to therapy with the requested agent; AND

6. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage;

Length of Approval: 12 months

Services related to delivery and/or administration of a medication which have not been approved through the BCBSMS PA review process will be considered not medically necessary.

POLICY EXCEPTIONS

State Health Plan (State and School Employees) Participants

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A.  consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

B.  appropriate with regard to standards of good medical practice; and

C.  not solely for the convenience of the Member, his or her Provider; and

D.  the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

BCBSMS may request medical records for determination of medical necessity. When medical records are requested, letters of support and/or explanation are often useful, but are not sufficient documentation unless all specific information needed to make a medical necessity determination is included.

POLICY HISTORY

01/25/2018: New policy added.

03/27/2018: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee.

07/30/2020: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee. Added drug name to the top of the policy. Added statement to perform a formulary drug search on the patient's member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific member's benefit plan. Policy statements updated to add "tocilizumab" and to list examples of biologic immunomodulatory agents. Policy criteria for initial approval updated to change the age requirement from "50 years" to "18 years." Policy Guidelines updated to define medication failure. Sources updated.

10/29/2020: Code Reference section updated to add HCPCS code J3262.

07/13/2021: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee. Policy description updated to include "tocilizumab." Policy section updated to add that the use of samples by a Member will not be considered current or stable therapy for purposes of Medical Policy review. Initial and renewal criteria updated regarding prescriber information and FDA-labeled contraindications. Added statement that services related to delivery and/or administration of a medication which have not been approved through the BCBSMS PA review process will be considered not medically necessary. Policy Exceptions updated regarding State Health Plan members. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity." Added information regarding BCBSMS request for medical records. Sources updated.

03/11/2022: Policy title changed from "Giant Cell Arteritis" to "Actemra (tocilizumab)." Policy description updated regarding indications for Actemra (tocilizumab). Medically necessary statement updated to add criteria for diagnosis of systemic sclerosis-associated interstitial lung disease. Renewal criteria updated to state that the member has been adherent to medication and has documented clinical improvement for the conditions specified. Sources updated.

06/14/2022: Policy description updated to remove quantity limits table for Actemra. Medically necessary policy statement criteria revised to state the following: The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage for the formulation being used. Criteria regarding request for the Actemra (tocilizumab) syringe moved under section B regarding documented diagnosis of systemic sclerosis-associated interstitial lung disease.

02/01/2023: Policy Exceptions updated to add the following: State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

07/01/2023: Links to related policies updated in Policy Description. Policy Exceptions updated regarding State Health Plan (State and School Employees) Participants.

10/01/2024: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy title changed from "Actemra (tocilizumab)" to "Giant Cell Arteritis." Policy description updated regarding giant cell arteritis and indications for Actemra (tocilizumab). Policy section updated to change "member" to "individual." Updated initial and renewal criteria for Actemra (tocilizumab). Policy Guidelines updated to remove language regarding medication failure. Sources updated.

11/01/2024: Effective 01/01/2025 - Policy description updated to remove Actemra. Policy statement revised to state that the use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Policy statements updated to remove "Actemra (tocilizumab)" and add "Tyenne (tocilizumab-aazg)" as medically necessary when the listed criteria are met. Added statement that Actemra (tocilizumab) and Tofidence (tocilizumab-bavi) are not covered on any BCBSMS Formulary. Sources updated. Code Reference section updated to add HCPCS code Q5135 to the Medically Necessary Codes table. Added HCPCS codes J3262 and Q5133 to the Not Covered/Non-Formulary Codes table. Policy update effective 01/01/2025.

07/22/2025: Policy updated to add Rinvoq (upadacitinib) to the list of drugs. Policy description updated regarding systemic glucocorticoid therapy and indications for upadacitinib. Policy criteria updated to add Rinvoq (upadacitinib) as medically necessary when all of the listed criteria are met. Initial length of approval changed from "6" to "12" months. Sources updated.

SOURCE(S)

1. Actemra prescribing information. Genentech, Inc. November 2024. Last accessed May 2025.

2. Maz, M., et al (2021). 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis Rheumatol, 73: 1349-1365.  https://doi.org/10.1002/art.41774 .

3. Rinvoq prescribing information. AbbVie Inc. April 2025. Last accessed May 2025.

4. Salvarani C, Muratore F. Treatment of giant cell arteritis. In: UpToDate, Connor RF (Ed), Wolters Kluwer. (Accessed on May 14, 2025.)

5. Salvarani C, Muratore F. Diagnosis of giant cell arteritis. In: UpToDate, Connor RF (Ed), Wolters Kluwer. Updated April 2024. (Accessed on August 29, 2024.)

6. Tofidence prescribing information. Biogen MA Inc. March 2025. Last accessed May 2025.

7. Tyenne prescribing information. Fresenius Kabi USA, LLC. March 2024. Last accessed October 2024.

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Medically Necessary Codes

Not Covered/Non-Formulary Codes

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