Abstract
Background and objectiveTo report novel findings and limitations from optical coherence tomography angiography (OCTA) in the evaluation of autoimmune retinopathy (AIR).Study designWe retrospectively reviewed features of five patients diagnosed with AIR and five controls. OCTA scans were obtained and manually segmented to provide accurate measurements of foveal avascular zone (FAZ), vessel density, and retinal thickness at different levels.ResultsThe total retina and superficial vessel density throughout the whole scan were similar between AIR and controls (p = 0.14 and p = 0.11), whereas deep vessel density was decreased in AIR compared controls (p = 0.02). Decreased vessel density was most pronounced in the parafoveal and perifoveal areas (p = 0.01 and p = 0.01). AIR patients also had reduction of total retinal thickness in the perifoveal zone (p = 0.03), corresponding to outer retinal thinning (p = 0.001).ConclusionThis small series shows that AIR patients have reduced deep vessel density, particularly in the parafoveal and perifoveal regions and a decrease in macular thickness. These findings show correlation with the classic “flying saucer” sign seen on OCT.
Highlights
Background and objectiveTo report novel findings and limitations from optical coherence tomography angiography (OCTA) in the evaluation of autoimmune retinopathy (AIR)
AIR is classified as non-paraneoplastic AIR and paraneoplastic [1], with the latter being further classified as cancer associated retinopathy (CAR) and melanoma associated retinopathy (MAR) [2]
Every OCTA image, for both AIR and control groups, contained several artifacts, including decentration of the Early Treatment Diabetic Retinopathy Study (ETDRS) grid, eye movement, refractive shift, defocus, shadowing, z-offset, tilt, projection, and blink lines (Additional file 1: Table S1). For both AIR and controls the artifacts were mild to moderate in severity, with the exception of the OCTA scan for patient “AIR 5”, which contained one severe artifact, related to the patient’s inability to fixate due to light perception vision
Summary
Background and objectiveTo report novel findings and limitations from optical coherence tomography angiography (OCTA) in the evaluation of autoimmune retinopathy (AIR). Autoimmune retinopathies (AIR) are a heterogenous and often underdiagnosed group of degenerative retinal diseases causing vision loss in the absence of posterior pole pathology such as inherited-retinal disease or overt inflammation. AIR is classified as non-paraneoplastic AIR (npAIR) and paraneoplastic (pAIR) [1], with the latter being further classified as cancer associated retinopathy (CAR) and melanoma associated retinopathy (MAR) [2]. Non-paraneoplastic autoimmune retinopathy is the most common subtype and is frequently associated with an underlying autoimmune etiology such as thyroid or connective tissue diseases. Acute zonal occult outer retinopathy is a considered subtype of npAIR and can show a trizonal pattern of retinal and retinal pigment epithelium (RPE) degeneration [3]. CAR is the most common paraneoplastic retinopathy and is associated with small cell lung cancer but can be seen with genitourinary cancers [4]. MAR is frequently observed in patients with a prior diagnosis of cutaneous or uveal melanoma [3]
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