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Purpose: To report the case of a patient with reactivated, refractory thyroid eye disease (TED) treated with teprotumumab., Observations: A 51-year-old female with a 16-year history of thyroid eye disease previously treated with orbital decompression and multiple eyelid surgeries presented in a recurrent flare of the disease. The disease recurrence was refractory to intravenous steroid therapy and only partially responsive to oral steroid therapy, and the patient developed dysthyroid optic neuropathy in the right eye with decreased visual acuity and color vision. Clinical activity score was 8/10 and proptosis measurements were 27 mm OD and 26 mm OS. The patient underwent treatment with eight infusions of teprotumumab coinciding with a low taper of oral prednisone and experienced resolution of dysthyroid optic neuropathy, decrease of clinical activity score to 1, and dramatic improvement in proptosis (17 mm OD, 17 mm OS) and extraocular muscle size on imaging. Thirty weeks after completion of teprotumumab and 2 weeks after the second dose of the COVID vaccine, she experienced another flare and subsequently underwent bilateral orbital decompressions., Conclusion: This case report suggests teprotumumab may be used in patients with reactivation of longstanding thyroid eye disease. Reduction of extraocular muscle size and improvement in proptosis suggest teprotumumab may be disease-modifying even in advanced cases., Competing Interests: DMS is a consultant and speaker for Horizon Therapeutics. OTC has no financial disclosures., (© 2022 The Authors.)

Purpose: To report a case series of subjective and objective hearing function changes associated with teprotumumab treatment for thyroid eye disease., Observations: A 74-year-old female with a history of Graves' disease with thyroid eye disease was treated with teprotumumab. She had a history of bilateral tinnitus and noticed a subjective improvement in her tinnitus after the second infusion. Audiology testing obtained before, during, and after completion of infusions showed symmetric and rapidly progressive worsening of the patient's sensorineural hearing loss. In contrast, a 42-year-old male with a history of Grave's disease endorsed worsening intermittent tinnitus and low-pitched hearing loss after initiation of teprotumumab. Audiology testing before, during, and after completion of infusions showed stable and normal hearing function bilaterally., Conclusion and Importance: This case series highlights the importance of objective testing in patients prior to and after teprotumumab initiation as subjective hearing changes may not accurately reflect objective hearing function. In addition, this report suggests that teprotumumab may play a role in potentiating sensorineural hearing loss., Competing Interests: The following authors have financial disclosures: EMS is a consultant/advisor for Horizon Therapeutics. The following authors have no financial disclosures: CYY, TC, BAS, MRH., (© 2021 Published by Elsevier Inc.)

Purpose: To study post-interventional findings in patients with dysthyroid optic neuropathy (DON) treated with teprotumumab., Observations: In this multicenter observational Case series , patients with DON were treated with teprotumumab, an insulin-like growth factor I receptor inhibitor (10 mg/kg for the first infusion then 20 mg/kg for subsequent infusions, every three weeks for a total 8 infusions). This study included patients with acute and chronic thyroid eye disease (TED) with DON who had failed conventional therapies and were not candidates for surgical decompression. Data collected included best corrected visual acuity (BCVA), color vision, RAPD when present, and orbital CT or MRI. Proptosis, clinical activity score (CAS), Gorman diplopia score (GDS), and Humphrey visual fields (HVF) were also evaluated.Ten patients (6 women, 4 men) with an average age 64 years old were included in this study. Mean follow up after completion of infusions was 15 weeks. Baseline visual acuity (VA) impairment ranged from hand motion (HM) to 20/25 in affected eyes. All patients had pre-treatment orbital CT or MRI that confirmed orbital apex compression. Seventy percent of patients had objective improvement in DON after 2 infusions of teprotumumab measured as significant improvement in visual acuity, resolution of RAPD, or both. After completion of treatment, affected eyes had a mean BCVA improvement of 0.87 logMAR (p=0.0207), proptosis reduction of 4.7 mm (p<0.00001), CAS improvement of 5.25 points (p<0.00001), and GDS improvement of 0.75 points (p=0.160). All 6 patients who presented with an RAPD had resolution or improvement of RAPD. All 7 patients who presented with color vision deficits had normalization or improvement of color vision., Conclusions and Importance: Teprotumumab infusions resulted in medical decompression and objective resolution or improvement of dysthyroid optic neuropathy. Most patients had rapid improvement of visual acuity and reversal of RAPD. Post-infusion imaging demonstrated reduction in extraocular muscle size that correlated with improvement in visual dysfunction. However, patients who presented with longstanding severe visual loss had limited improvement. There was no recurrence of DON after completion of teprotumumab in our cohort., Competing Interests: Andrea L Kossler, Kimberly Cockerham and Raymond Douglas are consultants for Horizon Therapeutics. The following authors have no financial disclosure: CMS, YW, LAB, RT, PSS., (© 2021 Published by Elsevier Inc.)

Purpose: To report the first case of a patient with chronic thyroid eye disease (TED) treated with teprotumumab., Observations: A 50-year-old female with a 3-year history of Graves' disease presented with bilateral exophthalmos greatest on the left side. She was followed for 2 years with stable proptosis measurements (23mm OD, 28mm OS). Her clinical activity score (CAS) was 1 and there were no examination findings reflective of active inflammation. The patient underwent systemic treatment with teprotumumab and despite chronic TED and low CAS, she had notable improvement in proptosis (18mm OD, 22mm OS) and decrease in extraocular muscle volume as noted on orbital imaging., Conclusion and Importance: This case report suggests that teprotumumab may be used in patients with chronic TED and low CAS. Improvement in the proptosis and reduction in extraocular muscle volume suggest that teprotumumab may alter disease course even in patients with inactive or quiescent TED., Competing Interests: BSK and DOK are consultants for Horizon Therapeutics. DJO has no financial disclosures., (© 2020 The Authors.)