Newly approved biologic agent teprotumumab may be an effective therapy for the treatment of compressive optic neuropathy resulting from thyroid eye disease, according to a letter published in JAMA Ophthalmology.1

Researchers from the W.K. Kellogg Eye Center at the University of Michigan, Ann Arbor, detailed the case of a 62-year-old man with moderate to severe thyroid eye disease. The patient had been experiencing symptoms for 6 months, with worsening in the 1 week prior to presentation. The patient had hyperthyroidism with elevated thyroid-stimulating immunoglobulin levels and was being treated with methimazole. 

Upon evaluation, researchers found a clinical activity score of 5, bilateral proptosis (25 mm), conjunctival injection, and severe right-sided bullous chemosis. Visual acuity was 20/25 OD and 20/20 OS; color vision was normal and no relative afferent pupillary defect was noted. However, the researchers did find both new right-sided optic nerve hemorrhages and edema, in addition to visual field defects. 


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The patient received teprotumumab within 48 hours following the recommended protocol. In the 2 weeks after the initial infusion, the patient’s clinical activity score was 0 and visual acuity was 20/20 OU, with “markedly reduced” congestion, resolution of right optic nerve edema, and improved proptosis (5 mm). These therapeutic responses continued at 25 weeks, and all 8 infusions were completed with no adverse effects. 

“The clinical trials assessing [the utility of teprotumumab] in active, moderate to severe TED excluded vision threatening disease,” the researchers wrote, including compressive optic neuropathy. Based on the results of this case, as well as an additional case published in Ophthalmic Plastic and Reconstructive Surgery, investigators recommend that the effectiveness of teprotumumab for compressive optic neuropathy should be evaluated and compared with both high-dosage steroids and urgent surgical decompression in randomized clinical trials.2

“We observed rapid resolution of early compressive optic neuropathy with teprotumumab,” the study shows. “This newly approved biologic agent may represent an effective therapy.” 

References

  1. Slentz DH, Smith TJ, Kim DS, Joseph SS. Teprotumumab for optic neuropathy in thyroid eye disease. Published online December 3, 2020. JAMA Ophthalmol. doi: 10.1001/jamaophthalmol.2020.5296
  2. Sears CM, Azad AD, Dosiu C, Kossler AL. Teprotumumab for dysthyroid optic neuropathy: early response to therapy. Published online September 22, 2020. Ophthalmic Plast Reconstr Surg. doi: 10.1097/IOP0000000000001831