Autoimmune retinopathy: findings and limitations from optical coherence tomography angiography
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(2020) 6:64 Raevis et al. Int J Retin Vitr https://doi.org/10.1186/s40942-020-00267-4
Open Access
ORIGINAL ARTICLE
Autoimmune retinopathy: findings and limitations from optical coherence tomography angiography Joseph Raevis* , Tyler Etheridge, Spencer Cleland and Mihai Mititelu
Abstract Background and objective: To report novel findings and limitations from optical coherence tomography angiography (OCTA) in the evaluation of autoimmune retinopathy (AIR). Study design: We 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. Results: The 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). Conclusion: This 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. Keywords: Autoimmune retinopathy, Cancer associated retinopathy, Melanoma associated retinopathy, Optical coherence tomography angiography, OCTA, vessel density, retinal thickness, foveal avascular zone Background 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]. *Correspondence: [email protected] Department of Ophthalmology and Visual Sciences, University of Wisconsin School of Medicine and Public Health, 206 University Ave 2870, Madison, WI, USA
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 also be seen with genitourinary cancers [4]. Visual symptoms precede the diagnosis of a systemic malignancy in approximately 50% of cases, highlighting the need for a systemic workup whenever there is suspicion for CAR [1, 3, 5]. MAR is frequently observed in patients with a p
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