Towards an Imaging-centric Definition of Non-paraneoplastic Autoimmune Retinopathy

Abstract

Purpose: We explored characteristic imaging features of npAIR to augment diagnostic criteria. Methods: This is a retrospective cohort study of patients with npAIR evaluated at the Emory Eye Center between 2013-2019. 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 (42%) patients were female, with median (range) age of 59 years (45-85 years). Median baseline visual acuity was 20/30 (20/20 - HM). Disease was asymmetric in 11 (92%) subjects. 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 FAF abnormality, ultimately developing characteristic speckled perimacular hypoautofluorescence. On OCT imaging, 18 (86%) eyes 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. Our findings augment diagnostic criteria to improve specificity and accessibility of testing for npAIR.