Autoimmune retinopathy associated with systemic lupus erythematosus: A diagnostic dilemma.

Visual loss in systemic lupus erythematosus (SLE) due to autoimmune retinopathy (AIR) is rare and easily misdiagnosed as hydroxychloroquine retinopathy. We report the rare clinical presentation of severe visual loss in a patient with SLE due to nonparaneoplastic AIR as differentiated from hydroxychloroquine toxicity. A 70-year-old female diagnosed and treated for lupus for 17 years and had been taking hydroxychloroquine for 15 years. Over the past 2 years, she developed progressive peripheral visual loss oculus uterque which rapidly advanced in the latter 6 months. Hydroxychloroquine toxicity was initially suspected, but diagnostic testing revealed a retinal degeneration. Antiretinal autoantibody testing using Western blot analysis revealed autoantibodies against 44-kDa, 46-kDa (anti-enolase), and 68-kDa proteins. Visual acuity improved in the first 6 months of treatment with mycophenolate mofetil. Our case suggests that AIR should be considered in the differential diagnosis of rapid, severe visual loss in patients with hydroxychloroquine treatment.

Treat-and-Extend Therapy Using Aflibercept for Neovascular Age-related Macular Degeneration: A Prospective Clinical Trial.

PURPOSE: To determine the efficacy and durability of aflibercept used in a treat-and-extend (TAE) regimen for neovascular age-related macular degeneration (NVAMD). DESIGN: Multicenter, prospective, open label, noncomparative, interventional study. METHODS: Forty eyes of 40 patients with treatment-naïve NVAMD were managed with a TAE regimen of intravitreal aflibercept. The main endpoints were the change in mean and median best-corrected visual acuity from baseline at years 1 and 2. Other endpoints included mean number of annual injections and treatment intervals. RESULTS: Thirty-five (87.5%) and 31 patients (77.5%) completed year 1 and year 2, respectively. The mean letter gain was 7.2 (P < .001) and 2.4 (P = .269) letters at 1 and 2 years, respectively, from a mean baseline of 58.9 letters (20/63 Snellen equivalent). The median visual gain was 11.5 and 7.5 letters at 1 and 2 years, respectively, from a median baseline of 59.0 letters (20/63 Snellen equivalent). The mean number of injections was 8.0 and 6.5 during the first and second year, respectively. Twelve-week or longer treatment intervals were used in 35% and 38% of patients during the first- and second-year time points, respectively. CONCLUSION: Intravitreal aflibercept TAE therapy led to significant visual improvement in eyes with NVAMD at 1 year, with some loss in the visual gains at the end of year 2 that was not related to loss of exudative control. TAE therapy with aflibercept is a rational strategy to reduce treatments and clinic evaluations over 2 years with satisfactory outcomes.

Select Features of Diabetic Retinopathy on Swept-Source Optical Coherence Tomographic Angiography Compared With Fluorescein Angiography and Normal Eyes.

IMPORTANCE: Optical coherence tomographic angiography (OCTA) is a recently developed noninvasive imaging technique that can visualize the retinal and choroidal microvasculature without the injection of exogenous dyes. OBJECTIVE: To evaluate the potential clinical utility of OCTA using a prototype swept-source OCT (SS-OCT) device and compare it with fluorescein angiography (FA) for analysis of the retinal microvasculature in diabetic retinopathy. DESIGN, SETTING, AND PARTICIPANTS: Prospective, observational cross-sectional study conducted at a tertiary care academic retina practice from November 2013 through November 2014. A cohort of diabetic and normal control eyes were imaged with a prototype SS-OCT system. The stage of diabetic retinopathy was determined by clinical examination. Imaging was performed using angiographic 3 × 3-mm and 6 × 6-mm SS-OCT scans to generate 3-dimensional en-face OCT angiograms for each eye. Two trained Boston Image Reading Center readers reviewed and graded FA and OCTA images independently. MAIN OUTCOMES AND MEASURES: The size of the foveal nonflow zone and the perifoveal intercapillary area on OCTA were measured in both normal and diabetic eyes using Boston Image Reading Center image analysis software. RESULTS: The study included 30 patients with diabetes (mean [SD] age, 55.7 [10] years) and 6 control individuals (mean [SD] age, 55.1 [6.4] years). A total of 43 diabetic and 11 normal control eyes were evaluated with OCTA. Fluorescein angiography was performed in 17 of 43 diabetic eyes within 8 weeks of the OCTA. Optical coherence tomographic angiography was able to identify a mean (SD) of 6.4 (4.0) microaneurysms (95% CI, 4.4-8.5), while FA identified a mean (SD) of 10 (6.9) microaneurysms (95% CI, 6.4-13.5). The exact intraretinal depth of microaneurysms on OCTA was localized in all cases (100%). The sensitivity of OCTA in detecting microaneuryms when compared with FA was 85% (95% CI, 53-97), while the specificity was 75% (95% CI, 21-98). The positive predictive value and the negative predictive value were 91% (95% CI, 59-99) and 60% (95% CI, 17-92), respectively. CONCLUSIONS AND RELEVANCE: Optical coherence tomographic angiography enables noninvasive visualization of macular microvascular pathology in eyes with diabetic retinopathy. It identified fewer microaneurysms than FA, but located their exact intraretinal depth. Optical coherence tomographic angiography also allowed the precise and reproducible delineation of the foveal nonflow zone and perifoveal intercapillary area. Evaluation of OCTA may be of clinical utility in the evaluation and grading of diabetic eye disease.

Analysis of Choroidal and Retinal Vasculature in Inherited Retinal Degenerations Using Optical Coherence Tomography Angiography.

BACKGROUND AND OBJECTIVE: To describe the retinal vasculature and choriocapillaris, as well as the transition zone between the diseased and healthy tissue, in eyes with inherited retinal degenerations using optical coherence tomography angiography (OCTA). PATIENTS AND METHODS: Patients with inherited retinal degenerations were recruited for OCTA imaging. Retinal vasculature was assessed for increased intercapillary space and foveal avascular zone abnormalities. Choriocapillaris, retinal pigment epithelium (RPE), and photoreceptor disruption were noted, and the borders were evaluated to speculate which layers become affected first. RESULTS: Fourteen eyes of seven subjects with inherited retinal degenerations were included. All eyes (100%) demonstrated retinal thinning and increased intercapillary spaces overlying focal outer retinal changes. In all eyes, the region of choriocapillaris changes was smaller than the region of overlying RPE and photoreceptor alteration, suggesting the vascular loss was secondary. CONCLUSION: OCTA is able to provide highly detailed vascular information in eyes with inherited retinal degenerations and may be useful to better understand the pathogenesis of these diseases.

DETECTION OF MICROVASCULAR CHANGES IN EYES OF PATIENTS WITH DIABETES BUT NOT CLINICAL DIABETIC RETINOPATHY USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To evaluate the ability of optical coherence tomography angiography to detect early microvascular changes in eyes of diabetic individuals without clinical retinopathy. METHODS: Prospective observational study of 61 eyes of 39 patients with diabetes mellitus and 28 control eyes of 22 age-matched healthy subjects that received imaging using optical coherence tomography angiography between August 2014 and March 2015. Eyes with concomitant retinal, optic nerve, and vitreoretinal interface diseases and/or poor-quality images were excluded. Foveal avascular zone size and irregularity, vessel beading and tortuosity, capillary nonperfusion, and microaneurysm were evaluated. RESULTS: Foveal avascular zone size measured 0.348 mm² (0.1085-0.671) in diabetic eyes and 0.288 mm² (0.07-0.434) in control eyes (P = 0.04). Foveal avascular zone remodeling was seen more often in diabetic than control eyes (36% and 11%, respectively; P = 0.01). Capillary nonperfusion was noted in 21% of diabetic eyes and 4% of control eyes (P = 0.03). Microaneurysms and venous beading were noted in less than 10% of both diabetic and control eyes. Both diabetic and healthy control eyes demonstrated tortuous vessels in 21% and 25% of eyes, respectively. CONCLUSION: Optical coherence tomography angiography was able to image foveal microvascular changes that were not detected by clinical examination in diabetic eyes. Changes to the foveal avascular zone and capillary nonperfusion were more prevalent in diabetic eyes, whereas vessel tortuosity was observed with a similar frequency in normal and diabetic eyes. Optical coherence tomography angiography may be able to detect diabetic eyes at risk of developing retinopathy and to screen for diabetes quickly and noninvasively before the systemic diagnosis is made.

Visualization of the Retinal Vasculature Using Wide-Field Montage Optical Coherence Tomography Angiography.

BACKGROUND AND OBJECTIVE: To evaluate the technique of wide-field montage optical coherence tomography angiography (OCTA) for improved visualization of the retinal vasculature in the posterior pole. PATIENTS AND METHODS: In this prospective case series, five eyes (three healthy and two diseased) of three patients were imaged using a novel technique to create wide-field montage OCTA images by combining nine 3 × 3-mm OCTA scans from the prototype AngioVue software of the RTVue XR (Optovue, Fremont, CA). Features seen on montage OCTA were compared to those visualized on fluorescein angiography (FA) and single-scan 8 × 8-mm OCTA. RESULTS: The montage OCTA showed the retinal vasculature in the greatest detail. The technique successfully detected vascular abnormalities seen on FA, and in some instances, montage OCTA visualized pathology that was not detected by FA and 8 × 8-mm OCTA. CONCLUSION: Wide-field montage OCTA imaging is a noninvasive, high-resolution technique to improve visualization of the retinal vasculature in the posterior pole.

Imaging in diabetic retinopathy.

While the primary method for evaluating diabetic retinopathy involves direct and indirect ophthalmoscopy, various imaging modalities are of significant utility in the screening, evaluation, diagnosis, and treatment of different presentations and manifestations of this disease. This manuscript is a review of the important imaging modalities that are used in diabetic retinopathy, including color fundus photography, fluorescein angiography, B-scan ultrasonography, and optical coherence tomography. The article will provide an overview of these different imaging techniques and how they can be most effectively used in current practice.

Oral eplerenone for treatment of chronic central serous chorioretinopathy: a case series.

BACKGROUND AND OBJECTIVE: To evaluate the effect of oral eplerenone on subretinal fluid, visual acuity, and choroidal thickness in patients with chronic central serous chorioretinopathy (CSCR). PATIENTS AND METHODS: Retrospective review of all patients (14 eyes of 14 patients) monitored for a minimum of 3 months with chronic CSCR who were treated with oral eplerenone in a single multi-physician retina practice. Visual acuity, dilated funduscopic examination, and spectral-domain ocular coherence tomography (OCT) with enhanced depth imaging (EDI) were obtained at each visit. Measurement of subfoveal fluid (SFF) height and choroidal thickness were performed. Two-tailed paired t test was used to calculate statistical significance of pre- and post-treatment variables. RESULTS: At 1 month, 10 of 14 eyes had decreased SFF height on OCT and two eyes had complete resolution of SFF. Mean SFF height decreased from 130 µm to 62 µm (P = .05). Mean choroidal thickness decreased from 315 µm to 282 µm (P = .07). Mean visual acuity improved from logMAR 0.41 to 0.40. At 3 months, 13 of 14 (93%) had decreased SFF on OCT, and nine eyes (64%) had complete resolution of SFF. Mean SFF height decreased to 21 µm (P = .004). Mean choroidal thickness decreased to 253 µm (P = .10). Mean visual acuity improved to logMAR 0.28 (P = .02). CONCLUSION: Oral eplerenone may be effective in treating patients with chronic CSCR.

Publication rates of registered clinical trials in macular degeneration.

PURPOSE: The purpose of this study was to evaluate the rate of publication of registered clinical trials concerning age-related macular degeneration (AMD). METHODS: The National Institutes of Health's ClinicalTrials.gov registry was searched to identify all trials concerning AMD. Trials that were actively recruiting, not interventional, terminated, or did not actually concern AMD were excluded. Only trials completed 2 or more years before this analysis was started were included, to allow for adequate time to pass before publication was expected to occur. PubMed.gov was then searched to evaluate the publication status of each study. RESULTS: Three hundred and eight-six studies were initially identified, and 64 (16.5%) were included in the final evaluation. Three hundred and twenty-one studies were not included for the following reasons: 171 did not involve AMD or were not interventional; 141 were not completed by January 1, 2007; and 9 trials were terminated. Of the 64 trials included, 35 (54%) were published. Early phase trials were published at a lower rate (41.6% [15/36]) than late-phase trials (71.4% [20/28]). This difference was statistically significant (P = 0.02). The sponsor type, date of study initiation, and location did not influence the publication rate. CONCLUSION: Using broad study parameters, 54% of registered interventional clinical trials in AMD have been reported in the peer-reviewed literature.