Bilateral dysthyroid compressive optic neuropathy responsive to teprotumumab

Thyroid eye disease is an auto-immune mediated orbitopathy which can cause dysthyroid compressive optic neuropathy. Traditional management of active thyroid eye disease includes temporizing high-dose steroids, orbital radiation and surgical decompression, which each possess significant limitations and/or side effects. Teprotumumab is an IGF-IR inhibitor recently FDA-approved for active thyroid eye disease. The authors report reversal of bilateral dysthyroid compressive optic neuropathy managed medically utilizing teprotumumab.

Trends in Treatment of Active, Moderate-to-Severe Thyroid Eye Disease in the United States

Introduction Limited data exist on US referral/management patterns for moderate-to-severe thyroid eye disease (TED), a disabling condition. Methods US ophthalmologists and endocrinologists experienced in treating TED provided medical record data of moderate-to-severe TED patients and information on referral/treatment practices. Data on signs/symptoms, medical/surgical treatments, treatment response, and referral history were collected. Moderate and severe cases were stratified to interrogate treatment/practice differences. Results A total of 181 physicians provided data on 714 patients (49.4 ± 13.6 years old, 65% women, 14% severe disease). Reporting physicians diagnosed 55% of patients themselves and solely managed 37% of cases, with similar referral/comanagement patterns between moderate and severe cases. Topical therapies included lubricating (79%) and glucocorticoid (39%) eye drops. Systemic therapies included oral glucocorticoids (36%), IV glucocorticoids (15%), and rituximab and/or tocilizumab (12%). Few patients underwent orbital radiation (4%) or surgical intervention (4%). IV glucocorticoids (33% vs. 12%), biologics (26% vs. 10%), orbital radiation (11% vs. 3%), and ocular surgery (12% vs. 3%) were used more often in severe versus moderate cases (all P < 0.001). However, severe disease was less responsive to therapy (very responsive to therapy: 28% vs. 49%, P < 0.001). Conclusions Participating physicians were primarily responsible for just over one-half of TED diagnoses, but solely treated <40% of patients. Severe TED was treated more often with surgery and systemic immunologic therapies than moderate disease, but was less likely to respond to treatment. These results reinforce that moderate-to-severe TED is difficult to treat with an unmet medical need in the United States.

Bilateral retinal detachment after chimeric antigen receptor T-cell therapy

Key Points CAR T-cell targeting of leukemic infiltrates in the optic nerve and retina caused retinal detachment as a presentation of pseudoprogression. Treatment of this intraocular inflammation with intravitreal triamcinolone and orbital radiation led to marked improvement in visual acuity.

Diplopia and proptosis due to isolated lateral rectus plasmacytoma in a patient with multiple myeloma

Orbital involvement of multiple myeloma (MM) is uncommon, with most of those reported cases occurring at the time of initial diagnosis of MM. We present a case of an extramedullary plasmacytoma involving only the right lateral rectus of a patient who had been in disease remission. The patient presented with new-onset diplopia and an abduction deficit of the right eye, with mild proptosis. In light of her past medical history of MM, an orbital MRI was obtained. The MRI demonstrated an isolated finding of eccentric enlargement of the right lateral rectus muscle limited to the muscle belly with sparing of the tendinous insertions, leading to diagnosis of plasmacytoma. Patching of the involved eye to alleviate the symptoms of diplopia was instituted. Chemotherapy was initiated, followed by orbital radiation and stem-cell transplantation for coexisting systemic disease. The orbital symptoms of proptosis and diplopia resolved within 1 month of treatment.