Tepezza (teprotumumab-trbw)

POLICY NUMBER

L.5.01.571

Tepezza (teprotumumab-trbw)

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

Tepezza (teprotumumab-trbw), an insulin-like growth factor-1 receptor inhibitor, is a fully human IgG1 monoclonal antibody that is indicated for the treatment of adults with Thyroid Eye Disease (TED) regardless of TED activity or duration. TED is a rare autoimmune condition where the muscles and fatty tissues behind the eye become inflamed, causing the eyes to be pushed forward and bulge outwards (proptosis). This can lead to a variety of symptoms such as eye pain, diplopia (double vision), light sensitivity, or difficulty closing the eye.

Tepezza (teprotumumab-trbw) is administered once every three weeks for a total of eight infusions.

POLICY

Tepezza infusions which do not meet ALL of the criteria outlined below are considered cosmetic and not eligible for coverage. Benefits will not be provided for Prescription Drugs utilized for cosmetic purposes or any services related to the delivery or administration of the drug. Benefits will not be provided for any complications resulting from Prescription Drugs utilized for cosmetic purposes. 

Prior authorization is required.

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

Initial Review

Tepezza (teprotumumab-trbw) may be considered medically necessary when ALL of the following are met:

1. Individual is ≥18 years old;

2. Individual does not require immediate surgical ophthalmological intervention AND has not had prior orbital irradiation or surgery for Thyroid Eye Disease (TED);

3. Individual has a clinical diagnosis of non-sight threatening moderate to severe active TED that is related to Graves’ Disease (i.e., Graves’ orbitopathy, Graves’ ophthalmopathy) defined as one or more of the following with documented onset in previous 9 months and cannot be attributed to previous ophthalmologic procedure:

   1. Lid retraction ≥ 2mm;

   2. Moderate or severe soft tissue involvement with documentation of CT must be provided (medial rectus, inferior rectus);

   3. Exophthalmos (forward protrusion, proptosis) is measured using Hertel Exophthalmometer and is >3 mm above the upper limit of normal (see Policy Guidelines); OR

   4. Diplopia;

4. Individual has a recent, within previous 3 months, clinical activity score (CAS) ≥ 4 (on the 7-item scale) in the most severely affected eye (see Policy Guidelines);

5. Prescribed by or in consultation with an endocrinologist AND ophthalmologist and ALL of the following:

   1. Records provided from endocrinologist must adequately document that the individual is euthyroid with the baseline thyroid disease under control (radioactive iodine, ablation, supplementation, etc.) prior to starting therapy with Tepezza (teprotumumab-trbw) OR have mild hyper- or hypothyroidism (free thryroxine [FT4] and free triiodothyronine [FT3] levels are <50% above or below normal limits). Every attempt should be made to correct hyper- or hypothyroidism promptly and remain in the euthyroid state throughout treatment with Tepezza (teprotumumab-trbw);

   2. Records provided from ophthalmologist must include all applicable ophthalmologic surgical records; AND

   3. Records provided to support that both endocrinologist and ophthalmologist have reviewed the individual's case and agree with diagnosis and treatment plan;

6. Individual has failed the accepted standard of care for moderate to severe TED defined as ONE of the following unless contraindicated or clinically significant adverse effects are experienced:

   1. For moderate symptoms (inflamed eyes and increasing diplopia or proptosis, ≥3 mm above upper limit of normal for race, mild corneal irritation), a trial of oral prednisone, 30mg/day for 4 weeks or IV methylprednisolone 500mg once weekly for weeks 1 to 6, then 250mg once weekly for weeks 7 to 12 with cumulative dose 4.5 to 5 grams over 12 weeks); OR

   2. For severe symptoms (constant diplopia), cycles of IV methylprednisolone 1 gram three times a week every 3-6 weeks PRN based on results not to exceed a cumulative dose of 8 grams.

7. Individual has not been treated with rituximab product, tocilizumab or other immunosuppressive agent within 3 months prior to Screening;

8. Individual does not have uncontrolled diabetes;

9. Individual does not have biopsy-proven or clinically suspected inflammatory bowel disease;

10. Individual does not utilize tobacco in any form;

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

12. Medication must be obtained using a BCBSMS Network DSP-Pharmacy and administered via home infusion or outpatient infusion.

Length of Approval: 6 months (max of 8 infusions)

Renewal Criteria

Coverage cannot be renewed.

Services related to delivery and/or administration of a medication determined to be not medically necessary will also 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.

Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria. Pharmacy printouts may be requested if medication fills cannot be verified by BCBSMS.

Hertel Exophthalmometer measurements: Below are the values for upper limit of normal for forward protrusion of the eye in adult patients:

• Men:

  • Asian: 18.6mm

  • Black: 24.7mm

  • Indian: 19mm

  • White: 21.7mm

• Women:

  • Asian: 18.6mm

  • Black: 23.0mm

  • Indian: 21mm

  • White: 20.1mm

• For any other ethnicity and gender combination which is not listed, an increase from baseline measurements of 3mm or more within 6 months must be documented to support clinically significant protrusion.

Clinical Activity Score (CAS) Assessment:

The Graves’ orbitopathy CAS elements below are each assigned a score of 1. Graves’ orbitopathy is considered active in patients with a CAS of ≥ 3. The Phase 3 clinical trial evaluating teprotumumab-trbw enrolled patients with a CAS of ≥ 4.

Initial Visit: One point for each item evaluated (7-item scale):

• Spontaneous orbital pain over last four weeks

• Pain with eye movement (gaze) during last four weeks

• Redness of the eyelids (erythema)

• Redness of the conjunctiva (erythema) that is considered to be due to active (inflammatory phase) TED/GO

• Swelling of the eyelids that is considered to be due to active (inflammatory phase) TED/GO

• Chemosis (edema of the conjunctiva)

• Inflammation of caruncle (flesh body at medial angle of eye) or plica

Medication failure is defined as disease progression despite maximally tolerated dose as appropriate for disease state being treated. Experience of common side effects of medication will not be considered medication failure for the purpose of this review.

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 Nervous/Mental Conditions, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

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

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

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

4. 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 Medically Necessary, “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.

Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of 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. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.

POLICY HISTORY

09/22/2020: New policy added. Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee. Effective 10/31/2020.

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: Policy Exceptions updated regarding State Health Plan (State and School Employees) Participants.

06/03/2025: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy description updated regarding indications for Tepezza (teprotumumab-trbw). Policy statement revised to state that the use of samples/voucher by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Medically necessary criteria updated to change "member" to "individual" and revised statement regarding FDA labeled dosing. Added statement that services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Sources updated.

SOURCE(S)

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. Eur Thyroid J. 2022 Dec 8;11(6):e220189. doi: 10.1530/ETJ-22-0189. PMID: 36479875; PMCID: PMC9727317.

2. Clinical trials.gov. Treatment of graves’ orbitopathy (thyroid eye disease) to reduct proptosis with teprotumumab infusions in a randomized, placebo-controlled, clinical study (OPTIC). (NCD 03298867). Available at: https://www.clinicaltrials.gov/ct2/show/NCT03298867 . Accessed on March 15, 2020.

3. 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. DOI: 10.1056/NEJMoa1910434.

4. Migliori ME, Gladstone GJ. Determination of the normal range of exophthalmometric values for black and white adults. Am J Ophthalmol. 1984;98(4):438-442. doi:10.1016/0002-9394(84)90127-2

5. Mourits MP, Prummel MF, Wiersinga WM, Koornneef L. Clinical activity score as a guide in the management of patients with Graves’ ophthalmopathy. Clin Endocrinol (Oxf) 1997; 47:9.

6. Punita Kumari Sodhi, V.P. Gupta & Ravindra M. Pandey (2001) Exophthalmometric values in a normal Indian population, Orbit, 20:1, 1-9, DOI: 10.1076/orbi.20.1.1.2647

7. Tepezza prescribing information. Horizon Therapeutics USA, Inc. February 2025. Last accessed April 2025.

8. Tsai, C., Kau, H., Kao, S. et al. Exophthalmos of patients with Graves' disease in Chinese of Taiwan. Eye 20, 569–573 (2006).

   https://doi.org/10.1038/sj.eye.6701925

9. U.S. Food and Drug Administration (FDA). FDA approves first eye treatment for thyroid eye disease. January 2020.

   https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/761143s000lbl.pdf

   .

   Accessed March 2020.

10. Xu N, Cui Y, Xie T, Zheng M. Comparative Efficacy of Medical Treatments for Thyroid Eye Disease: A Network Meta-Analysis. J Ophthalmol 2018; 2018:7184163.

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

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