Clinical Policy: Teprotumumab (Tepezza) Form

CENTENE Corporation

Clinical Policy: Teprotumumab (Tepezza) Reference Number: CP.PHAR.465 Effective Date: 01.21.20 Last Review Date: 02.26 Line of Business: Commercial, HIM-Medical Benefit*, Medicaid

Coding Implications Revision Log

See Important Reminder at the end of this policy for important regulatory and legal information.

Description Teprotumumab (Tepezza™) is an insulin-like growth factor 1 receptor (IGF-1R) inhibitor.

*For Health Insurance Marketplace (HIM), coverage of Tepezza is excluded for the pharmacy benefit and should not be approved using these criteria; these criteria may be used for medical benefit review.

FDA Approved Indication(s) Tepezza is indicated for the treatment of thyroid eye disease (TED) regardless of TED activity or duration.

Policy/Criteria

9. Dose does not exceed both of the following (a and b):
    a. A single 10 mg/kg dose followed by seven 20 mg/kg infusions given every 3
       weeks (see Appendix E for vial rounding recommendations);
    b. Vial quantity as identified by the online dose calculator using the member’s
       weight (see Appendix D) or as recommended in Appendix E for vial rounding.

Approval duration: 6 months (up to 8 total lifetime infusions)

B. Other diagnoses/indications (must meet 1 or 2):

1. If this drug has recently (within the last 6 months) undergone a label change (e.g.,
   newly approved indication, age expansion, new dosing regimen) that is not yet
   reflected in this policy, refer to one of the following policies (a or b):
    a. For drugs on the formulary (commercial, health insurance marketplace) or PDL
       (Medicaid), the no coverage criteria policy for the relevant line of business:
       CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and
       CP.PMN.255 for Medicaid; or
    b. For drugs NOT on the formulary (commercial, HIM-Medical Benefit) or PDL
       (Medicaid), the non-formulary policy for the relevant line of business:
       CP.CPA.190 for commercial, HIM.PA.33 for HIM-Medical Benefit, and
       CP.PMN.16 for Medicaid;
2. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed
   under section III (Diagnoses/Indications for which coverage is NOT authorized) AND
   criterion 1 above does not apply, refer to the off-label use policy for the relevant line
   of business: CP.CPA.09 for commercial, HIM.PA.154 for HIM-Medical Benefit, and
   CP.PMN.53 for Medicaid.

II. Continued Therapy A. Thyroid Eye Disease (must meet all):

1. 
5. If request is for a dose increase, new dose does not exceed both of the following (a
   and b):
    a. A total of seven 20 mg/kg infusions given every 3 weeks (see Appendix E for vial
       rounding recommendations);
    b. Vial quantity as identified by the online dose calculator using the member’s
       weight (see Appendix D) or as recommended in Appendix E for vial rounding.

Approval duration: 1 month (up to 8 total lifetime infusions)

B. Other diagnoses/indications (must meet 1 or 2):

1. If this drug has recently (within the last 6 months) undergone a label change (e.g.,
   newly approved indication, age expansion, new dosing regimen) that is not yet
   reflected in this policy, refer to one of the following policies (a or b):
    a. For drugs on the formulary (commercial, health insurance marketplace) or PDL
       (Medicaid), the no coverage criteria policy for the relevant line of business:
       CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and
       CP.PMN.255 for Medicaid; or
    b. For drugs NOT on the formulary (commercial, HIM-Medical Benefit) or PDL
       (Medicaid), the non-formulary policy for the relevant line of business:
       CP.CPA.190 for commercial, HIM.PA.33 for HIM-Medical Benefit, and
       CP.PMN.16 for Medicaid;
2. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed
   under section III (Diagnoses/Indications for which coverage is NOT authorized) AND
   criterion 1 above does not apply, refer to the off-label use policy for the relevant line
   of business: CP.CPA.09 for commercial, HIM.PA.154 for HIM-Medical Benefit, and
   CP.PMN.53 for Medicaid.

III. Diagnoses/Indications for which coverage is NOT authorized: A. Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off label use policies – CP.CPA.09 for commercial, HIM.PA.154 for HIM-Medical Benefit, and CP.PMN.53 for Medicaid, or evidence of coverage documents.

IV. Appendices/General Information Appendix A: Abbreviation/Acronym Key CAS: clinical activity score FDA: Food and Drug Administration GO: Graves’ ophthalmopathy TED: thyroid eye disease

Appendix B: Therapeutic Alternatives This table provides a listing of preferred alternative therapy recommended in the approval criteria. The drugs listed here may not be a formulary agent for all relevant lines of business and may require prior authorization.

| Drug Name | Dosing Regimen | Dose Limit/

Maximum Dose |

|-----------|---------------|----------------|

| prednisone | 30 mg/day PO | 30 mg/day |

| Drug Name | Dosing Regimen | Dose Limit/

Maximum Dose |

|-----------|---------------|----------------|

| methylprednisolone (SOLU-Medrol®) | 500 mg IV once weekly for weeks 1 to 6, then 250 mg IV once weekly for weeks 7-12 | 500 mg/week |

Therapeutic alternatives are listed as Brand name® (generic) when the drug is available by brand name only and generic (Brand name®) when the drug is available by both brand and generic.

Appendix C: Contraindications/Boxed Warnings None reported

Appendix D: General Information • Use of systemic corticosteroids in TED is supported by the following treatment guidelines:

○ 2021 European Group on Graves’ orbitopathy (EUGOGO) clinical practice
   guidelines for the medical management of Graves’ orbitopathy: A combination of IV
   methylprednisolone and mycophenolate sodium is recommended as first-line
   treatment. If response to primary treatment is poor and Graves’ ophthalmopathy (GO)
   is still moderate-to-severe and active, teprotumumab is considered a second-line
   option as longer-term data, availability, affordability, costs, and need for subsequent
   rehabilitative surgery is pending.
○ 2016 American Thyroid Association Guidelines for Diagnosis and Management of
   Hyperthyroidism and Other Causes of Thyrotoxicosis: In the absence of any strong
   contraindication to GC, consider for coverage of mild active GO who are treated with
   RAI, even in the absence of risk factors for GO deterioration (weak recommendation,
   low-quality evidence). Additionally in mild GO patients who are treated with RAI,
   steroid coverage is recommended if there are concomitant risk factors for GO
   deterioration (strong recommendation, moderate-quality evidence).

• The following link will provide the dose and appropriate vial quantity based on the member’s weight (note this does not account for dose rounding recommendations found in Appendix E below): https://www.tepezzahcp.com/starting-patients/dosing-and- administration

Appendix E: Vial Rounding Recommendations (40 to 170 kg)

V. Dosage and Administration

VI. Product Availability Single-dose vial: 500 mg

Coding Implications Codes referenced in this clinical policy are for informational purposes only. Inclusion or exclusion of any codes does not guarantee coverage. Providers should reference the most up-to- date sources of professional coding guidance prior to the submission of claims for reimbursement of covered services.

HCPCS

Reviews, Revisions, and Approvals