Diagnosis: Thyroid Eye Disease.
Treatment: Tepezza 500 milligrams (mg) injection (inj.).

The insurer denied coverage for the Tepezza 500 mg inj.
The health plan's determination is overturned.

The patient is a female with a medical history of hyperthyroidism and thyroid eye disease of the left eye. The patient had taken oral prednisone and reported no improvement. She noted that a chin up posture was required to reduce her double vision.

The patient's examination was notable for visual acuities of, the ability to see an object clearly from 20 feet away (20/20) in the right eye (OD) and the ability to see an object clearly from 25 feet away (20/25) in the left eye (OS). The patient had intraocular pressures of 22 millimeters of mercury (mm Hg) in both eyes (OU). Diplopia was present in her primary and upgaze. The left eye was restricted to an upgaze. Edema of the patient's left periorbital tissues was also noted. Conjunctival chemosis was noted inferotemporally in the OS. The hertels measured 19 millimeters (mm) in her OD and 23 mm in her OS on a base of 101 mm. The clinical activity score was 5 out of 7 and then 3 out of 3 on follow up for a total of 8 out of 10. Coverage of Tepezza was previously denied since the patient did not try a 4-week course of systemic corticosteroids. The patient's thyroid levels were performed are reported as stable and within the reference range.

The health plan, in its determination of medical necessity, did not act reasonably, with sound medical judgement and in the best interest of the patient. Coverage was originally denied as the plan required a trial of systemic steroids which was reasonable. However, subsequently, coverage was denied because thyroid studies which had previously been performed and met criteria were now considered out of date. Additional thyroid studies were obtained confirming the patient's levels were stable.

The requested health service/treatment of Tepezza 500 mg inj. is medically necessary for the patient. Thyroid eye disease (TED) is an autoimmune inflammatory disorder involving the soft tissues of the orbit. The classic clinical findings include eyelid retraction, exophthalmos, extraocular muscle restriction, and optic nerve dysfunction (all of which the patient has). Additional examination features are eyelid lag (von Graefe sign), lagophthalmos, conjunctival hyperemia over the horizontal rectus muscle insertions, resistance to globe retropulsion, and superior limbic keratoconjunctivitis. During the course of TED, eyelid retraction occurs in 90 percent (%) of patients, with exophthalmos present in 60%. The combination of these features can lead to scleral show and exposure keratitis. Commonly associated symptoms include ocular pain, dry eyes, tearing, photophobia, and blurred vision. Diplopia from a restrictive myopathy presents in 40% of these patients. Approximately 20% of TED patients undergo surgical treatment, including orbital decompression (7%), strabismus surgery (9%), and eyelid surgery (13%).

Current medical therapies for thyroid-associated ophthalmopathy primarily consist of glucocorticoids which have limited efficacy and present safety concerns with continued use. Rituximab, a monoclonal antibody that targets the cluster of differentiate 20 (CD-20) on B lymphocytes (B-cells) which has been used experimentally with some promising results. Orbital radiation can be used alone or in conjunction with corticosteroids but has radiation retinopathy as a side effect. Ultimately, orbital decompression and/or strabismus surgery may be needed. Inhibition of the Insulin-like growth factor I receptor (IGF-IR) is a new therapeutic strategy to attenuate the underlying autoimmune pathogenesis of ophthalmopathy.

Tepezza was Food and Drug Administration (FDA) approved for the treatment of Thyroid Eye Disease on 1/21/2020.