This policy version was replaced on March 28, 2022. To find the newest version, go to https://www.bluecrossmn.com/providers/medical-policy-and-utilization-management, read and accept the Blue Cross Medical Policy Statement, then select “Blue Cross and Blue Shield of Minnesota Medical Policies.” This will bring up the Medical Policy search screen. Enter the policy number without the version number (last 3 digits).

Teprotumumab is a human monoclonal antibody that targets insulin-like growth factor-1 receptor (IGF-1R), a protein that plays a role in immune function. Pathogenic autoantibodies activating a signaling complex result in inflammation and tissue expansion behind the eyes. Teprotumumab blocks the stimulatory effects of these autoantibodies and is administered via intravenous infusion.

The U.S. Food and Drug Administration (FDA) has approved teprotumumab (Tepezza™) for treatment of thyroid eye disease. Thyroid eye disease, also known as Graves’ ophthalmopathy, occurs in a subset of individuals with Graves’ disease, and is often characterized by a forward protrusion of the eyes. Adverse reactions have occurred in patients treated with teprotumumab, including muscle spasm, nausea, alopecia, diarrhea, fatigue, hyperglycemia, hearing impairment, dry skin, dysgeusia, and headache. 

Definitions:

Clinical Activity Score: A score used to assess patients with thyroid eye disease based on seven components: spontaneous retrobulbar pain, pain on attempted eye movements, conjunctival redness, redness of the eyelids, chemosis, swelling of the caruncle or plica, and swelling of the eyelids. Each component is scored as present or absent (score of 1 or 0, respectively). Score is summed, with higher scores indicating greater level of inflammation. 

Diplopia: Double vision.

Proptosis: Abnormal protrusion of the eye.

I.    Review for Teprotumumab (Tepezza™)

Teprotumumab may be considered MEDICALLY NECESSARY AND APPROPRIATE when ALL of the following criteria are met:

• Diagnosis of Graves’ disease; AND
• Moderate-to-severe thyroid eye disease as defined by ONE or more of the following:
  • Lid retraction of ≥ 2 mm;
  • Proptosis of ≥ 3 mm above normal values for race and sex;
  • Recurrent diplopia;
• AND
• Clinical activity score for thyroid eye disease ≥ 4; AND
• ONE of the following:
  • Previously tried and failed glucocorticoids; OR
  • Documented intolerance, FDA labeled contraindication, or hypersensitivity to glucocorticoids; 
• AND
• Not previously treated for thyroid eye disease with orbital irradiation or surgery; AND
• Prescribed by an ophthalmologist; AND
• No FDA labeled contraindications to teprotumumab (see table 1 below); AND
• Dose is within the FDA labeled dose for the indication (see table 2 below); AND
• For commercial health plan members only, step therapy supplement criteria may apply for select conditions (see policy II-242: Step Therapy Supplement).
  

II.   Experimental/Investigative Uses

All other uses of teprotumumab, including but not limited to restarting an incomplete course of therapy, are considered EXPERIMENTAL/ INVESTIGATIVE due to the lack of clinical evidence demonstrating an impact on improved health outcomes. 

Table 1.  FDA Labeled Contraindications

Table 2. Dosing 

NOTE:  See documentation submission requirements below if the requested dose is higher or more frequent than the dosing criteria provided in this table.

Documentation Submission:

Documentation supporting the medical necessity criteria described in the policy must be included in the prior authorization, when prior authorization is required. In addition, the following documentation must also be submitted:

1. Clinical notes describing the diagnosis and clinical features of the diagnosis.
2. Objective measurements of thyroid eye disease. For proptosis, photographs showing degree of proptosis and objective measurements of proptosis using an exophthalmometer or CT scan. For lid retraction, photographs and objective measurements showing retraction.
3. Clinical Activity Score for thyroid eye disease.
4. .Clinical notes describing current and past medications for the diagnosis, including response to the medications.
5. The dose being requested. If the requested dose is higher or more frequent than the dosing guidelines provided in the table above, a clear explanation for the medical necessity of the requested dose MUST be submitted, including prior dosing (strength and frequency) associated with inadequate response.
6. For commercial health plan members only, when step therapy requirements apply for the requested indication, documentation for one or more of the step therapy supplement criteria MUST be provided (see policy II-242).