Purpose: In response to the coronavirus (COVID-19) pandemic, teprotumumab production was temporarily halted with resources diverted toward vaccine production. Many patients who initiated treatment with teprotumumab for thyroid eye disease were forced to deviate from the standard protocol. This study investigates the response of teprotumumab when patients receive fewer than the standard 8-dose regimen. Methods: This observational cross-sectional cohort study included patients from 15 institutions with active or minimal to no clinical activity thyroid eye disease treated with the standard teprotumumab infusion protocol. Patients were included if they had completed at least 1 teprotumumab infusion and had not yet completed all 8 planned infusions. Data were collected before teprotumumab initiation, within 3 weeks of last dose before interruption, and at the visit before teprotumumab reinitiation. The primary outcome measure was reduction in proptosis more than 2 mm. Secondary outcome measures included change in clinical activity score (CAS), extraocular motility restriction, margin reflex distance-1 (MRD1), and reported adverse events. Results: The study included 74 patients. Mean age was 57.8 years, and 77% were female. There were 62 active and 12 minimal to no clinical activity patients. Patients completed an average of 4.2 teprotumumab infusions before interruption. A significant mean reduction in proptosis (–2.9 mm in active and –2.8 mm in minimal to no clinical activity patients, P < 0.01) was noted and maintained during interruption. For active patients, a 3.4-point reduction in CAS (P < 0.01) and reduction in ocular motility restriction (P < 0.01) were maintained during interruption. Conclusions: Patients partially treated with teprotumumab achieve significant reduction in proptosis, CAS, and extraocular muscle restriction and maintain these improvements through the period of interruption.

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PurposeWe report an update on a recently published case of uncontrolled hypertension secondary to immunoglobulin A (IgA) nephropathy resulting in massive bilateral retinal and choroidal infarction.ObservationsIn our previous report, we presented a 30-year old female with end-stage renal disease who complained of painless vision loss after many missed hemodialysis. The patient was found to be in hypertensive crisis resulting in massive retinal and choroidal infarction with severe vision loss in both eyes. The patient was treated with pan-retinal photocoagulation (PRP) with intravitreal Bevacizumab and was subsequently lost to follow-up. In this update, we report the complications that followed. After many months, she presented to clinic with a blind painful right eye. She was found to have a further decrease in vision with neovascular glaucoma in the right eye and a tractional retinal detachment in the left eye. The patient ultimately elected for enucleation of her right eye. Immunohistopathology revealed IgA deposition, confirming the presumed diagnosis of IgA nephropathy, previously unconfirmable through renal biopsy.Conclusions and ImportanceThere is a strong association between severity of retinopathy and level of kidney function. Although a rare presentation, hypertensive retinopathy is a common complication of end-stage renal disease and can be a devastating process as emphasized by this report. Those with auto-immune renal disease, such as IgA nephropathy, are at higher risk for retino-choroidal complications. It should remind all ophthalmologists and clinicians on the necessity of closer eye examinations for these patients, particularly for those with auto-immune renal disease.

Alpha-gal syndrome is characterized by an IgEmediated hypersensitivity response to the carbohydrate galactose-alpha-1,3-galactose. It can manifest as both an immediate drug allergy to pharmaceuticals containing alpha-gal as well as a delayed hypersensitivity response to the ingestion of mammalian meat products. In the United States, sensitization to alpha-gal is thought to occur from tick bites and can cause severe angioedema, most commonly periorbitally and periorally, with high rates of anaphylaxis. We present a unique case of chronic periorbital edema in a carnivorous individual after acquiring a new red meat allergy from Alpha-Gal Syndrome.A lpha-gal syndrome is an immune-mediated hypersensitivity response to the carbohydrate galactose-alpha-1,3-galactose (alpha-gal), a sugar molecule found in nonprimate mammals. [1][2][3][4][5] It includes both a drug allergy, characterized by immediate hypersensitivity responses to injected pharmaceutical products containing alpha-gal, and a food allergy, characterized by delayed reactions after ingestion of red mammalian meat. [1][2][3][4][5] Currently, we believe the sensitization begins with a bite from the Lone Star tick (Amblyomma Americanum), which increases expression of alpha-gal sugar in its saliva after a blood meal from nonprimate mammals such as the deer. [1][2][3][4]6 After a bite, the sugar molecule is transferred to humans, triggering an immune system reaction that later produces mild to severe allergic reactions to red meat such as beef, pork and lamb in 2-6 hours. 6,7 Immune responses in humans are variable and influenced by cofactors and the dose and form of alpha-gal consumed; however, a majority of patients present with cutaneous symptoms characterized by urticaria, angioedema, and pruritis. 8,9 Diagnoses can be challenging given