Update on autoimmune retinopathy: diagnosis and management

Autoimmune retinopathy (AIR) is a rare but vision-threatening disorder characterized by retinal damage through humoral or cellular immune mechanisms. The purpose of this review is to summarize our current understanding on imaging, antibody testing, and immunosuppressive therapies for AIR.

AIR includes paraneoplastic forms - such as cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR) - as well as a nonparaneoplastic (np) variant (npAIR). Definitive diagnosis of AIR is challenging due the similar clinical findings between AIR and inherited retinal dystrophies and the lack of specific laboratory testing. Therefore, a multimodal diagnostic approach including electroretinography, fundus imaging, visual field testing, genetic testing, and serologic assays is necessary to support a diagnosis of AIR. Early diagnosis and treatment with systemic or local immunomodulators may preserve retinal function and lead to better visual outcomes.

This review highlights current diagnostic approach and treatment strategies in AIR. Advances in biomarkers and precision immunotherapy may improve diagnosis and outcomes.