SPLIT OUTER PLEXIFORM LAYER APPEARANCE REPRESENTS AN ACQUIRED CONE DYSFUNCTION PHENOTYPE of AUTOIMMUNE RETINOPATHY

Abstract

Purpose:Autoimmune retinopathy (AIR) is a poorly understood condition with a wide phenotypic spectrum and heterogeneous findings on clinical examination and imaging. In this study, the authors characterize the clinical features of a recently described phenotypic subtype of AIR with a distinct split outer plexiform layer appearance on OCT.Methods:This was a retrospective, observational study of patients with a diagnosis of AIR seen at the University of Iowa between January 2007 and September 2023 who were found to have decreased visual acuity, central scotoma on visual field testing, split outer plexiform layer appearance on OCT, and abnormal light-adapted electroretinogram, consistent with an acquired cone dysfunction. Clinical course and multimodal imaging and physiologic testing were reviewed.Results:Ten patients were identified in the retrospective cohort. All patients presented with bilateral, asymmetric, subacute, and progressive central vision loss. A diagnosis of cancer was present or subsequently diagnosed in 5 out of 10 patients (50%). Overall, 40% of patients underwent treatment with immunosuppressives, and one patient underwent tumor resection, with partial visual recovery seen.Conclusion:Patients with split outer plexiform layer appearance on OCT have an acquired cone dysfunction that is important to recognize as treatment in some cases may result in improved or stabilized visual function.