TOWARD AN IMAGING-CENTRIC DEFINITION OF NONPARANEOPLASTIC AUTOIMMUNE RETINOPATHY.

PURPOSE: To explore characteristic imaging features of nonparaneoplastic autoimmune retinopathy (npAIR) to augment diagnostic criteria. METHODS: This is a retrospective cohort study of patients with npAIR evaluated at the Emory Eye Center between 2013 and 2019. Multimodal fundus images were evaluated to characterize the evolution of the disease. RESULTS: Twenty-one eyes of 12 patients were classified as having npAIR. Five patients (42%) were female, with median (range) age of 59 years (45-85 years). Median baseline visual acuity was 20/30 (20/20 to hand motions). Disease was asymmetric in 11 patients (92%). Common imaging findings included absence of bone spicules (86% of affected eyes), presence of attenuated vessels (86%), and speckled hypoautofluorescence in perimacular and perivenular regions. Three eyes were noted to present early with subtle splotchy fundus autofluorescence abnormality, ultimately developing characteristic speckled perimacular hypoautofluorescence. On optical coherence tomography, 18 eyes (86%) had loss of outer retinal bands with relative foveal sparing and a tapered transition zone. CONCLUSION: Many eyes with npAIR exhibit a subacute, asymmetric, generalized photoreceptor degeneration featuring outer retinal atrophy with relative foveal sparing, retinal vascular attenuation, absence of bone spicules, and speckled hypoautofluorescence often in a perimacular and perivenular distribution. Findings of this study augment diagnostic criteria to improve specificity and accessibility of testing for npAIR.

The impact of COVID-19: variations in volumes and characteristics of retina surgeries.

BACKGROUND: In concordance with medical recommendations in response to COVID-19, Emory Healthcare limited surgical procedures starting March 16, 2020. We investigated the impact of these recommendations on the number, types, and urgency of surgical retina cases. METHODS: We conducted a retrospective review of all surgical patients at the Retina division of the Emory Eye Center from February 17-April 12, 2020 and during the same time period in 2019 and 2018. The demographics of patients and the number, types and urgency of retina surgeries were collected. Descriptive statistics for each variable were reported. Univariate analysis was carried out using the chi-square test or Fisher's exact test for categorical covariates. RESULTS: From February 17-March 15 to March 16-April 12, 2020, total surgeries decreased from 87 to 34. Emergent cases, occurring within 7 days of surgical order placement, decreased from 23 to 18 (p = 0.0056), and urgent cases, occurring within 21 days of surgical order placement, decreased from 26 to 4 (p = 0.0380). From March 16-April 12, 2019 there were 62 surgeries: 21 emergent (34%), 14 urgent (23%). From March 16-April 12, 2018 there were 68 surgeries: 15 emergent (22%), 21 urgent (30%). After March 16, 2020, average patient age decreased from 39.4 to 25.7 years. There were no statistically significant differences in racial make-up or insurance coverage for those having surgery prior to versus after March 16, 2020. CONCLUSION: National recommendations for ophthalmologic surgeries during COVID-19 disproportionately affected older patients and patients with urgent cases at our tertiary care academic medical center. These results may inform the ophthalmologic field of the potential effects of pandemics such as COVID-19 on the surgical retina care of patients.

A UNIQUE INDOCYANINE GREEN ANGIOGRAPHY FEATURE IN SARCOIDOSIS.

PURPOSE: To describe a unique indocyanine green angiography (ICGA) feature in sarcoidosis-associated choroidal vasculitis in a 33-year-old woman. METHODS: Case report. RESULTS: A 33-year-old woman with sarcoidosis was referred for the evaluation of blurry vision in both eyes. On ocular examination of both eyes, slit-lamp biomicroscopy and ophthalmoscopy revealed 0.5+ anterior chamber cell, 1+ vitreous cell, and no vitreous haze. Also noted were areas of venous perivascular sheathing and multiple (3-5) inferior snowballs. Fluorescein angiography showed optic disk hyperfluorescence, retinal phlebitis, and hyperfluorescence with late leakage in the periphery (Figure 1). Indocyanine green angiography showed small hypofluorescent spots. Also noted on the ICGA images were multiple, sharp, linear hyperfluorescent areas signifying areas of choroidal vasculitis (Figure 2). The patient was referred to rheumatology for the initiation of immunomodulatory therapy. CONCLUSION: To the best of our knowledge, this ICGA pattern of multiple, sharp, linear hyperfluorescent areas has not been previously described in patients with sarcoidosis. This ICGA pattern represents areas of sarcoidosis associated choroidal vasculitis.

Characterization of Fluorescein Angiography Features in Pediatric Patients with Uveitis.

PURPOSE: To characterize fluorescein angiography (FA) features in pediatric uveitis. METHODS: A retrospective cross-sectional study of pediatric uveitis patients in Atlanta, GA, who had FA imaging between May 2009 and August 2020. RESULTS: A total of 137 eyes of 88 patients were evaluated. The mean FA score in anterior uveitis was lower than that in other categories (p = .0093). The mean FA score in the clinically active group was higher than that in the clinically inactive group (p = .0057). The mean FA score in eyes 20/40 or better was lower than that in other categories (p = .0292). Worse visual acuity was associated with retinal vascular staining/leakage, retinal staining/pooling, and neovascularization elsewhere. Non-infectious uveitis more often demonstrated optic disc hyperfluorescence, retinal vascular staining/leakage, capillary leakage, and pinpoint leaks, while infectious uveitis more often demonstrated retinal staining/pooling. CONCLUSION: A standardized quantitative FA scoring system can be useful in the characterization of uveitis in pediatric patients.

Visual Morbidity and Outcomes of Scleritis Associated with Intraocular Inflammation Compared to Isolated Scleritis.

PURPOSE: To compare visual outcomes, ocular complications and therapies for patients with scleritis-associated intraocular inflammation (SAI) and patients with isolated scleritis (IS). RESULTS: A total of 52 patients (36 with SAI and 16 with IS) were reviewed. Mean age (standard deviation) at presentation was 48.4 years old (± 15.4) in the SAI group and 53 years old (± 17.1) in the IS group (p = .37). Visual acuity was worse at presentation and last visit for patients with SAI compared to IS (p = .04). Patients in the SAI group developed greater posterior segment complications than in the IS group (p = .002). CONCLUSIONS: Scleritis with intraocular inflammation was associated with a higher rate of visual morbidity compared to isolated scleritis. More aggressive management strategies may be needed for patients who present with scleritis associated with inflammation.

Morphological characteristics of eyes with neovascular age-related macular degeneration and good long-term visual outcomes after anti-VEGF therapy.

PURPOSE: To analyse the morphological characteristics of eyes with neovascular age-related macular degeneration (AMD) with good long-term visual acuity after anti-VEGF (vascular endothelial growth factor) therapy. METHODS: Retrospective, observational study of 175 patients with neovascular AMD with >5 years of follow-up after initiating anti-VEGF therapy. Spectral-domain optical coherence tomography images were assessed for thickness of pigment epithelial detachment (PED), subretinal hyper-reflective material (SHRM), subretinal fluid and subfoveal choroidal, as well as the integrity of the outer retinal bands. RESULTS: The final analysis cohort included 203 eyes (175 patients) followed for a mean of 7.84±1.70 years (range: 5-11). The maximum PED thickness in the foveal central subfield (FCS) was significantly lower (p<0.001) in the poor vision group (13.11 µm) compared with the intermediate (86.25 µm) or good (97.92 µm) vision groups, respectively. In contrast, the maximum thickness of SHRM in the FCS was significantly thicker (p<0.001) in eyes with poor vision (149.46 µm) compared with eyes with intermediate vision (64.37 µm) which in turn were significantly thicker (p<0.001) than eyes with good vision (9.35 µm). The good vision group also had better continuity of all outer retinal bands (external limiting membrane, ellipsoid zone, and retinal pigment epithelium) compared with the other two groups (all p<0.001). CONCLUSION: A thicker PED and thinner SHRM were correlated with better vision in eyes with neovascular AMD following long-term anti-VEGF therapy. If replicated in future prospective studies, these findings may have implications for design of optimal anatomic endpoints for neovascular AMD treatment.

Diabetic retinopathy in African-Americans with end-stage kidney disease: a cross-sectional study on prevalence and impact on quality of life.

INTRODUCTION: The prevalence, severity, and quality of life (QoL) impact of diabetic retinopathy (DR) among African-Americans (AAs) with end-stage kidney disease (ESKD) undergoing dialysis are unknown. RESEARCH DESIGN AND METHODS: A cross-sectional study was conducted on 93 AA adults with diabetes and ESKD. The diagnosis of DR was based on a review of medical records and/or a positive photograph with a portable hand-held device reviewed by both artificial intelligence software and a retinal specialist. QoL, physical disability social determinants of health (SDoHs) were assessed by standardized questionnaires. RESULTS: The prevalence of DR was 75%, with 33% of participants having mild, 9.6% moderate and 57.4% severe DR. A total of 43% had normal visual acuity; 45% had moderate visual impairment; and 12% had severe visual impairment. We found a high burden of disease, multiple SDoH challenges, and low QoL and general health among patients with ESKD. The presence of DR had no significant impact on physical health and QoL compared with participants without DR. CONCLUSIONS: DR is present in 75% of AA patients with diabetes and ESKD on haemodialysis. ESKD has a significant burden on general health and QoL; however, DR has a minor additional impact on the overall physical health and QoL in people with ESKD.

Progress in the diagnosis of ocular sarcoidosis.

Sarcoidosis is a multisystem granulomatous inflammation that affects multiple organ systems. The spectrum of extraocular and ocular involvement is wide and may precede systemic involvement. The diagnosis of ocular sarcoidosis relies on a combination of clinical findings, laboratory investigations, and radiographic findings. These include but are not limited to serum angiotensin-converting enzyme (ACE), lysozyme, plain-film radiographs of the chest, computed tomography (CT) scans of the chest, pulmonary function testing, bronchoalveolar lavage, and retinal imaging among others. In this review, we highlight current and evolving systemic investigations and approaches to ophthalmic imaging when considering the diagnosis of ocular sarcoidosis.

Health Literacy, Medication Adherence, and Quality of Life in Patients With Uveitis.

PURPOSE: To evaluate health literacy, medication adherence, and quality of life (QOL) in uveitis patients. METHODS: Cross-sectional quality improvement study using questionnaires of health literacy (Short Assessment of Health Literacy), adherence (Beliefs about Medicine Questionnaire), and QOL (12-item Short Form Health Survey). RESULTS: Sixty patients were surveyed: 57% women, 80% identified as Black, and 42% with schooling beyond high school. Forty-three percent of the patients had poor health literacy, with lower scores among those with less schooling (p < .01). Necessity scores were higher for multiple providers (p = .02). Necessity (p = .03) and Concerns (p < .01) scores were higher for patients seeing a rheumatologist. Patients had lower physical (p < .01) and mental QOL (p < .01) than the general US population. CONCLUSIONS: Uveitis patients reported many barriers to care. Despite strong perceptions of treatment necessity, there were significant concerns, especially among patients seeing a rheumatologist. We recommend multidisciplinary care, individualized education, and counseling regarding medication safety and the necessity to improve adherence.

Cytopathology of Vitreous Specimens in Acute Retinal Necrosis.

PURPOSE: To report the cytopathology of vitreous biopsy samples in patients with acute retinal necrosis (ARN) who underwent pars plana vitrectomy (PPV). We also describe two patients with unique clinical courses, cytopathologic findings, and immune response. METHODS: A retrospective review of patients with ARN who developed retinal detachment (RD) and underwent PPV from 22011 to 2019 at the Emory Eye Center was performed to assess cytopathology findings of vitreous biopsy samples. Patient demographics and laboratory testing including aqueous humor PCR for viral pathogens were recorded. Additional clinical details abstracted included intravitreal injections, surgical procedures, and vitreous cytopathological reports including immunohistochemistry findings. RESULTS: Fourteen eyes of 12 patients with RD were reviewed. Ten eyes showed HSV DNA (71%) and 4 demonstrated VZV DNA (29%). All eyes received intravitreal antivirals (i.e. ganciclovir or foscarnet) with a median of 8.5 intravitreal injections per eye. Diagnoses prompting PPV included tractional RD in 14 eyes (100%), rhegmatogenous RD in 8 eyes (57%), vitreous hemorrhage in 4 eyes (29%) and vitreous opacity in 4 (29%). Ophthalmic pathology reports showed lymphocyte populations in 10 eyes (71%) with a CD3 + T-cell predominance in two patients where immunohistochemistry of CD3+ and CD20+ for T- and B-cell populations was performed. Observed immune cell populations included macrophages or histiocytes (11 eyes, 79%) and polymorphonuclear cells in 4 eyes (29%). Initial median VA was 2.5 (IQR 2.0-3.0) and improved to 2.0 (IQR 1.48-3.00, p = .48) at 6-months and 1.8 (IQR 1.2-3.0, p = .45) at 12 months follow-up. CONCLUSIONS: Our cohort of ARN patients undergoing PPV show a spectrum of immunologic findings with the majority demonstrating a lymphocytic response. Histiocytes, macrophages, and PMNs were also observed. Cytopathologic and immunologic studies suggest that both innate and adaptive immunity are responsible for the clinical disease findings observed in ARN. The variability of the response to treatment in patients with ARN may reflect patient-to-patient differences in their antigen-specific immune response. Understanding the immunologic response associated with ARN may provide valuable information regarding the dosing and timing of treatment.

Clinical Correlation between Acute Exudative Polymorphous Paraneoplastic Vitelliform Maculopathy and Metastatic Melanoma Disease Activity: A 48-month Longitudinal Case Report.

PURPOSE: Longitudinal evaluation of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM) following diagnosis and treatment of metastatic melanoma. METHODS: Case report of a 47-year-old male with unknown primary metastatic melanoma and AEPPVM monitored before and during melanoma treatment using clinical exam, retinal imaging, and electroretinograms (ERG). Genetic testing and autoantibody panels were performed. RESULTS: He presented within a month of metastatic melanoma diagnosis with numerous bilateral vitelliform lesions in the posterior pole, consistent with AEPPVM. Metastatic disease was treated with immunotherapy, radiosurgery, and radiation over 48 months. Maculopathy and metastatic disease improved and worsened in parallel. Genetic testing was negative for bestrophin-1. An autoantibody panel was positive for anti-recoverin and transducin-α. CONCLUSION: AEPPVM is an uncommon paraneoplastic retinopathy found in patients with metastatic malignancy. To our knowledge, this is the first report demonstrating a temporal association between metastatic disease activity and quantifiable changes in retinal imaging over a 4-year period.

Risk Factors for Retinal Detachment in Acute Retinal Necrosis.

PURPOSE: Retinal detachment (RD) is associated with poor visual outcomes in patients with acute retinal necrosis (ARN). This research was undertaken to assess the risk factors for RD in ARN. DESIGN: Retrospective cohort study. SUBJECTS: Patients diagnosed with ARN at a tertiary referral center from 2010 to 2020. METHODS: A chart review was performed for all clinical and surgical encounters. Univariate and multivariate logistic analyses of demographic and clinical variables associated with RD were performed. Survival analyses with Kaplan-Meier estimates were performed to compare the time to RD in herpes simplex virus (HSV)- and varicella zoster virus (VZV)-associated ARN. MAIN OUTCOME MEASURES: Demographic information, clinical information (including visual acuity [VA]), intraocular pressure (IOP), intraocular inflammation level, the extent of retinitis, incidence and timing of retinal detachment, date of diagnosis, and treatments performed (including intravitreal injections of antiviral medications). RESULTS: Fifty-four eyes of 47 patients who were diagnosed with ARN were included, with equal proportions of eyes (n = 27; 50%) with VZV-ARN and HSV-ARN. Patients with VZV-ARN were, on average, older, more likely to be men, and more likely to be immunosuppressed compared with patients with HSV-ARN. The clinical characteristics, including the initial VA, initial IOP, anterior segment inflammation, clock hours, and posterior extent of retinitis, were similar between eyes with VZV- and HSV-ARN. In the univariate analysis of clinical and demographic variables associated with the development of RD, initial VA (P = 0.0083) and greater clock hours of retinitis (P = 0.009) were significantly associated with RD. These 2 variables remained significant in the multivariate logistic regression; worse VA at presentation had an odds ratio of 2.34 (95% confidence interval [CI], 1.01-5.44; P = 0.042), and greater clock hours of retinitis had an odds ratio of 1.23 (95% CI, 1.02-1.47; P = 0.025). A Kaplan-Meier survival analysis demonstrated no statistical difference in RD-free survival between HSV- and VZV-ARN. CONCLUSIONS: Patients with VZV-ARN were more likely to be older, male, and immunosuppressed compared with those with HSV-ARN, although no clear difference was observed in RD by viral etiology. Poor initial VA and clock hours of retinitis were significantly associated with RD development and may be relevant for patient counseling and prognosis.

Retinal vasculitis: A framework and proposal for a classification system.

Retinal vasculitis, a poorly understood process involving inflammation or ischemia of the retinal vessel wall, may occur in association with a systemic process, although it can also be isolated to the retina. Because of the limited ability to perform histopathological studies on retinal vessels, there is no gold standard for diagnosis. Thus, there is utility in creating a classification system for retinal vasculitis and improving diagnostic strategies for this disease. We provide a framework for understanding retinal vasculitis based on size, location, and etiology. We hope that this information can be implemented in the clinical setting to provide some diagnostic strategies for this often confusing entity.

Clinical course and outcomes of COVID-19 in rheumatic disease patients: a case cohort study with a diverse population.

OBJECTIVE: To determine clinical course and outcomes in rheumatic disease patients with coronavirus disease 2019 (COVID-19) and compare results to uninfected patients. METHODS: We conducted a case cohort study of autoimmune disease patients with COVID-19 (confirmed by severe acute respiratory syndrome coronavirus 2 PCR) from February 1, 2020, to July 31, 2020, and compared them in a 1:3 ratio with uninfected patients who were matched based on race, age, sex, and comorbidity index. Patient demographics, clinical course, and outcomes were compared among these patient groups. RESULTS: A total of 70 rheumatic disease patients with COVID-19 (mean age, 56.6 years; 64% African American) were identified. The 34 (49%) patients who were hospitalized used oral glucocorticoids more frequently than those treated as outpatients (p < 0.01). All 10 patients using anti-TNFα medications were treated as outpatients (p < 0.01). Those hospitalized with COVID-19 more often required ICU admission (17 (50%) vs 27 (26%), p = 0.01) and intubation (10 (29%) vs 6 (6%), p < 0.01) than uninfected patients and had higher mortality rates (6 (18%) vs 3 (3%), p < 0.01). Of the six COVID-19 patients who died, only one was of African ancestry (p = 0.03). CONCLUSION: Rheumatic disease patients infected with COVID-19 were more likely to require ICU admission, ventilation, and died more frequently versus uninfected patients with autoimmune disease. Patients on anti-TNFα medications were hospitalized less frequently, while those on chronic glucocorticoids were hospitalized more frequently. These findings have important implications for medication choice in rheumatic disease patients during the ongoing spread of COVID-19. Key Points • We show that hospitalized rheumatic disease patients with COVID-19 have poorer outcomes including ICU admission, ventilation, and death compared to hospitalized rheumatic disease patients not infected with COVID-19. • This study adds further support regarding protective effects of anti-TNFα medications in COVID-19 disease course, with 0 of 10 of these patients required hospitalization.

Description of symptom course in a telemedicine monitoring clinic for acute symptomatic COVID-19: a retrospective cohort study.

OBJECTIVE: Describe the disease course in a cohort of outpatients with COVID-19 and evaluate factors predicting duration of symptoms. DESIGN: Retrospective cohort study. SETTING: Telemedicine clinic at a large medical system in Atlanta, Georgia. PARTICIPANTS: 337 patients with acute COVID-19. Exclusion criteria included intake visit more than 10 days after symptom onset and hospitalisation prior to intake visit. MAIN OUTCOME MEASURES: Symptom duration in days. RESULTS: Common symptoms at intake visit are upper respiratory (73% cough, 55% loss of smell or taste, 57% sinus congestion, 32% sore throat) and systemic (66% headache, 64% body aches, 53% chills, 30% dizziness, 36% fever). Day of symptom onset was earliest for systemic and upper respiratory symptoms (median onset day 1 for both), followed by lower respiratory symptoms (day 3, 95% CI 2 to 4), with later onset of gastrointestinal symptoms (day 4, 95% CI 3 to 5), when present. Cough had the longest duration when present with median 17 days (95% CI 15 to 21), with 42% not resolved at final visit. Loss of smell or taste had the second longest duration with 14 days (95% CI 12 to 17), with 38% not resolved at final visit. Initial symptom severity is a significant predictor of symptom duration (p<0.01 for multiple symptoms). CONCLUSIONS: COVID-19 illness in outpatients follows a pattern of progression from systemic symptoms to lower respiratory symptoms and persistent symptoms are common across categories. Initial symptom severity is a significant predictor of disease duration for most considered symptoms.

Discharge characteristics and care transitions of hospitalized patients with COVID-19.

Little is known about the follow-up healthcare needs of patients hospitalized with coronavirus disease 2019 (COVID-19) after hospital discharge. Due to the unique circumstances of providing transitional care in a pandemic, post-discharge providers must adapt to specific needs and limitations identified for the care of COVID-19 patients. In this study, we conducted a retrospective chart review of all hospitalized COVID-19 patients discharged from an Emory Healthcare Hospital in Atlanta, GA from March 26 to April 21, 2020 to characterize their post-discharge care plans. A total of 310 patients were included in the study (median age 58, range: 23-99; 51.0% female; 69.0% African American). The most common presenting comorbidities were hypertension (200, 64.5%), obesity (BMI≥30) (138, 44.5%), and diabetes mellitus (112, 36.1%). The median length of hospitalization was 5 days (range: 0-33). Sixty-seven patients (21.6%) were admitted to the intensive care unit and 42 patients (13.5%) received invasive mechanical ventilation. The most common complications recorded at discharge were electrolyte abnormalities (124, 40.0%), acute kidney injury (86, 27.7%) and sepsis (55, 17.7%). The majority of patients were discharged directly home (281, 90.6%). Seventy-five patients (24.2%) required any home service including home health and home oxygen therapy. The most common follow-up need was an appointment with a primary care provider (258, 83.2%). Twenty-four patients (7.7%) had one or more visit to an ED after discharge and 16 patients (5.2%) were readmitted. To our knowledge, this is the first large study to report on post-discharge medical care for COVID-19 patients.

Diagnostic retinal biopsy in the management of secondary non-CNS vitreoretinal lymphoma masquerading as viral retinitis: a case report.

BACKGROUND: Intraocular lymphoma accounts for fewer than 1% of intraocular tumors. When the posterior segment is involved, it can be further classified as vitreoretinal or choroidal lymphoma. Vitreoretinal lymphoma (VRL) can rarely masquerade as an infectious retinitis making diagnosis and management challenging. RESULTS: A 73-year-old woman with a history of non-central nervous system (CNS) involving diffuse large B-cell lymphoma (DLBCL) was referred for worsening blurry vision-visual acuity of count figures at 2 ft-in her right eye for 8 months. Dilated fundus examination of the right eye was significant for retinal whitening and dot-blot hemorrhages, which was concerning for a viral retinitis and guided initial management. Secondary intraocular lymphoma was also considered. The retinal disease continued to progress despite intravitreal and systemic antiviral therapy, and a diagnostic vitrectomy was inconclusive. A retinal biopsy was then performed, which showed DLBCL, confirming a diagnosis of secondary VRL. Three subsequent treatments with intravitreal methotrexate led to regression of the VRL. CONCLUSIONS: Our case highlights the utility of a retinal biopsy after an inconclusive diagnostic vitrectomy in a challenging scenario of VRL to establish a diagnosis and initiate successful treatment. A multidisciplinary team of providers was essential for diagnosis, comprehensive workup, medical and surgical management of the patient.