Teprotumumab (Tepezza) Form

Teprotumumab (Tepezza™) is an insulin-like growth factor 1 receptor (IGF-1R) inhibitor. FDA Approved Indication(s) Tepezza is indicated for the treatment of thyroid eye disease (TED) regardless of TED activity or duration. Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.
It is the policy of health plans affiliated with Centene Corporation® that Tepezza is medically necessary when the following criteria are met:
I. Initial Approval Criteria
A. Thyroid Eye Disease (must meet all):

1. Diagnosis of Graves’ disease with associated TED (i.e., Graves’ ophthalmopathy, Graves’ orbitopathy);
2. Prescribed by or in consultation with an ophthalmologist;
   
   3. Age ≥ 18 years;
   4. One of the following (a or b): a. Member is euthyroid with documentation of a recent (within the last 30 days) free thyroxine (FT4) and total triiodothyronine (T3) or free T3 (FT3) levels within the laboratory defined reference range; b. Member has a recent (within the last 30 days) free thyroxine (FT4) and total triiodothyronine (T3) or free T3 (FT3) levels less than 50% above or below the laboratory defined reference range and is undergoing treatment to correct the mild hypo- or hyperthyroidism to maintain a euthyroid state;

3. Member has not had previous surgical intervention for TED;

   6. Member does not require surgical ophthalmological intervention;
   7. Failure of a 4-week trial of a systemic corticosteroid (at up to maximally indicated doses), unless one of the following (a, b, or c): a. Clinically significant adverse effects are experienced or all are contraindicated;
      b. Member has significant proptosis (examples may include but are not limited to proptosis ≥ 3 mm above the upper limit for race and sex, or proptosis that impacts activities of daily life [e.g., reading, driving, computer work, and watching television]); Page 1 of 8

   CLINICAL POLICY Teprotumumab
   c. Member has diplopia;

4. Member has not received ≥ 8 Tepezza infusions (including the initial 10 mg/kg first infusion);
5. 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):
6. 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, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or
7. 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 health insurance marketplace, and CP.PMN.53 for Medicaid. II. Continued Therapy A. Thyroid Eye Disease (must meet all):
8. Member meets one of the following (a or b): a. Currently receiving medication via Centene benefit or member has previously met initial approval criteria; b. Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums for CC.PHARM.03A and CC.PHARM.03B);
9. Member has not had previous surgical intervention for TED;
   3. Member does not require surgical ophthalmological intervention;
   4. Member has not received ≥ 8 Tepezza infusions (including the initial 10 mg/kg first infusion);

10. 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. Page 2 of 8

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    Approval duration: 1 month (up to 8 total lifetime infusions) B. Other diagnoses/indications (must meet 1 or 2):

11. 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, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

12. 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 health insurance marketplace, 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 health insurance marketplace, 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 prednisone methylprednisolone (SOLU-Medrol®) 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. 30 mg/day PO 500 mg IV once weekly for weeks 1 to 6, then 250 mg IV once weekly for weeks 7-12 Dose Limit/ Maximum Dose 30 mg/day 500 mg/week Page 3 of 8

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    Appendix C: Contraindications/Boxed Warnings None reported Appendix D: General Information • Use of systemic corticosteroids in TED is supported by the following treatment guidelines: o 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 are pending. o 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) Weight Range (kg) 40 – 52.5
    52.6 – 55
    55.1 – 77.5 77.6 – 102.5
    102.6 – 105
    105.1 – 127.5 127.6 – 152.5
    152.6 – 155 155.1 – 170
    Initial Dose (1 dose) Vial Quantity Recommendation 1 1 2 2 2 3 3 3 4 Maintenance (7 doses total) Vial Quantity Recommendation 14 (2 vials per dose) 21 (3 vials per dose) 21 (3 vials per dose) 28 (4 vials per dose) 35 (5 vials per dose) 35 (5 vials per dose) 42 (6 vials per dose) 49 (7 vials per dose) 49 (7 vials per dose) V. Dosage and Administration Indication TED Dosing Regimen Initial: 10 mg/kg IV one time dose Maintenance: 20 mg/kg IV every 3 weeks for seven infusions Maximum Dose See dosing regimen VI. Product Availability
    Single-dose vial: 500 mg Page 4 of 8

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    VII.