Clinical Case Discussion
On diagnosis of CON, the patient underwent emergent right orbital decompression with high-dose IV steroids followed by a standard 12-week course of IV steroids combined with ORT for her active TED. Despite these interventions, she developed CON in her left eye requiring left orbital decompression. After 9 months of stable inactive disease, she underwent additional rehabilitative surgery for double vision and eyelid retraction.
This case illustrates a number of points. First, the patient was initially misdiagnosed with allergic conjunctivitis and bacterial conjunctivitis, the most common incorrect diagnoses for mild active TED. Second, the delay in diagnosis could have contributed to her developing more severe TED, with pain, disfigurement, restrictive strabismus, and decreased QoL. Third, her left eye progressed to sight-threatening disease despite IV steroids and ORT, demonstrating that sometimes these treatments cannot prevent vision loss or halt disease progression. Selenium was added as adjuvant therapy, given its relatively benign safety profile in short-term use, even though its efficacy has been proven only in mild active TED, and it has not been studied in moderate/severe active disease. Although the patient recovered well after several rehabilitative surgeries (Figure 1C), her treatment course lasted more than 2 years. This case is an example of the typical delay in diagnosis, sometimes incomplete response to nonspecific medical therapies, and the serious ocular sequelae that can result.
Surgical decompression and biologic therapies were discussed as treatment options for CON of the left eye and steroid-resistant active TED. The patient received teprotumumab, 10 mg/kg followed by 20 mg/kg every 3 weeks × 8 doses. Her extraocular movement improved, proptosis decreased by 3.5 mm, and inflammatory symptoms decreased (Figure 2C). Her vision improved quickly to 20/25 OU and color vision normalized. Side effects included hives and rashes after infusions responsive to diphenhydramine hydrochloride, muscle cramps, diarrhea, autophony, and hyperglycemia responsive to medical therapy.
This case was a therapeutic challenge because of active sight-threatening disease recalcitrant to IV steroids. Teprotumumab was given in the hopes of halting active progressive disease and delaying orbital decompression during the peak of coronavirus disease 2019 quarantine and hospital restrictions in April 2020. The patient had a significant improvement both in CON and active TED symptoms. Following treatment, her rashes, GI symptoms, and muscle cramps resolved. Her ear symptoms, which developed after the sixth infusion and persisted after the final infusion visit, are being monitored and are expected to resolve, as per the data in the clinical trials. She will be monitored for 6 months to ensure durable resolution of active TED prior to undergoing further rehabilitative surgery in the stable phase, if needed.
J Endo Soc. 2021;5(5) © 2021 Endocrine Society