Teprotumumab-trbw (Tepezza™)

Restricted Product(s)

• teprotumumab-trbw (Tepezza™) intravenous infusion for administration by a healthcare professional

FDA Approved Use

• For treatment of thyroid eye disease regardless of thyroid eye disease activity or duration

Criteria for Medical Necessity

The restricted product(s) may be considered medically necessary when the following criteria are met:

1. The patient is 18 years of age or older; AND 
2. The patient has a diagnosis of moderate to severe thyroid eye disease (TED) related to Graves’ disease (i.e., Graves’ orbitopathy) [medical record documentation required]; AND 
3. The diagnosis has been confirmed by presence of at least one of the following [medical record documentation required]:
   1. Lid retraction of ≥ 2 mm; OR 
   2. Moderate or severe soft-tissue involvement; OR 
   3. Proptosis of ≥ 3 mm above normal value for race and sex; OR
   4. Periodic or constant diplopia; AND 
4. The patient has a Clinical Activity Score (CAS) of 3 or greater in the more severely affected eye(s) or as scored by a comparable objective scoring system [medical record documentation required]; AND 
5. The patient is euthyroid OR has mild hypo- or hyperthyroidism (i.e., free thyroxine [FT4] and free triiodothyronine [FT3] levels less than 50% above or below normal limits) [medical record documentation required]; AND 
6. The patient has NOT had prior surgical treatment or orbital irradiation for TED [medical record documentation required]; AND
7. The patient is NOT planning on surgical treatment or orbital irradiation for TED during treatment with the requested agent [medical record documentation required]; AND 
8. The patient has tried and had an inadequate response to corticosteroids used in the treatment of TED (e.g., prednisone, methylprednisolone, dexamethasone) [medical record documentation required]; OR
9. The patient has an intolerance, FDA labeled contraindication, or hypersensitivity to ALL corticosteroids used in the treatment of TED [medical record documentation required]; AND 
10. The patient has NOT had a decrease in best corrected visual acuity due to optic neuropathy within the previous 6 months (i.e., a decrease in vision of 2 lines on the Snellen chart, new visual field defect, or color defect secondary to optic nerve involvement) [medical record documentation required]; AND
11. The patient does NOT have corneal decompensation that is unresponsive to medical management [medical record documentation required]; AND 
12. The patient will NOT be using the requested agent beyond one treatment course of 8 infusions; AND 
13. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, ophthalmologist) or has consulted with a specialist in the area of the patient’s diagnosis [medical record documentation required]; AND 
14. For requests for injection or infusion administration of the requested medication in an inpatient or outpatient hospital setting, Site of Care Criteria applies (outlined below)*
    
    Duration of Approval: 180 days (one treatment course of 8 infusions per lifetime)

*Site of Care Medical Necessity Criteria

1. For requests for injection or infusion administration in an inpatient setting, the injection or infusion may be given if the above medical necessity criteria are met AND the inpatient admission is NOT for the sole purpose of administering the injection or infusion; OR
2. For requests for injection or infusion administration in an outpatient hospital setting, the injection or infusion may be given if the above medical necessity criteria are met AND ONE of the following must be met:
   1. History of mild adverse events that have not been successfully managed through mild pre-medication (e.g., diphenhydramine, acetaminophen, steroids, fluids, etc.); OR 
   2. Inability to physically and cognitively adhere to the treatment schedule and regimen complexity; OR 
   3. New to therapy, defined as initial injection or infusion OR less than 3 months since initial injection or infusion; OR 
   4. Re-initiation of therapy, defined as ONE of the following:
      1. First injection or infusion after 6 months of no injections or infusions for drugs with an approved dosing interval less than 6 months duration; OR 
      2. First injection or infusion after at least a 1-month gap in therapy outside of the approved dosing interval for drugs requiring every 6 months dosing duration; OR 
   5. Requirement of a change in the requested restricted product formulation; AND
3. If the Site of Care Medical Necessity Criteria in #1 or #2 above are not met, the injection or infusion will be administered in a home-based infusion or physician office setting with or without supervision by a certified healthcare professional.

References

All information referenced is from FDA package insert unless otherwise noted below.

1. Burch HB, Perros P, Bednarczuk T, et al. Management of thyroid eye disease: a consensus statement by the American Thyroid Association and the European Thyroid Association. Thyroid. 2022;32(12):1439-1470. 
2. Douglas RS, Kahaly GJ, Patel A, et al. Teprotumumab for the treatment of active thyroid eye disease. N Engl J Med. 2020;382:341-52. 
3. Ross DS, Burch HB, Cooper DS, et al. 2016 American Thyroid Association Guidelines for Diagnosis and Management of Hyperthyroidism and Other Causes of Thyrotoxicosis. Thyroid. 2016;26(10):1343-1421. 
4. Smith TJ, Kahaly GJ, Ezra DG, et al. Teprotumumab for thyroid-associated ophthalmopathy. N Engl J Med. 2017;376(18):1748-61. 
5. Wang Y, Smith TJ. Current concepts in the molecular pathogenesis of thyroid-associated ophthalmopathy. Invest Ophthalmol Vis Sci. 2014;55(3):1735-48.

Policy Implementation/Update Information

Criteria and treatment protocols are reviewed annually by the Blue Cross NC P&T Committee, regardless of change. This policy is reviewed in Q4 annually.

July 2023: Criteria change: Added newly updated FDA labeled indication to include thyroid eye disease regardless of thyroid eye disease activity or duration. Removed ‘active’ from diagnostic criteria. Updated Clinical Activity Score (CAS) requirement to 3 or greater. Updated maximum units according to coding unit definition for clarity. Minor adjustments made to formatting and reference added.
October 2021: Criteria change: Added Site of Care medical necessity criteria. Policy notification given 8/2/2021 for effective date 10/1/2021.
June 2021: Criteria change: Added diagnosis confirmation criteria for active, moderate to severe TED related to Graves’ disease; added maximum units; medical policy formatting change. Policy notification given 4/16/2021 for effective date 6/16/2021.
*Further historical criteria changes and updates available upon request from Medical Policy and/or Corporate Pharmacy.