Guidelines for the Diagnosis, Management, and Study of Autoimmune Retinopathy from the American Academy of Ophthalmology's Task Force.

Purpose: The American Academy of Ophthalmology created a task force to advance the understanding of autoimmune retinopathy (AIR) and provide guidelines on the diagnosis and management of this complex disorder.

Design: A search on PubMed and Google Scholar of English-language studies was conducted without date restrictions. The Task Force reviewed the current literature and formulated an expert consensus on the management of AIR as well as recommendations for future efforts to improve our understanding of this condition.

Results: Key clinical and imaging features are discussed, and a new diagnostic framework is proposed based on likelihood of AIR (probable AIR, possible AIR, and unlikely AIR) to provide a more standardized approach for categorizing disease. Patients who possess all the following features can be categorized as having probable AIR: (1) signs of disease progression based on subjective symptoms and objective testing within six months, (2) examination with less than 1+ anterior chamber or vitreous cell/haze, (3) optical coherence tomography (OCT) with outer retinal disruption and loss of the external limiting membrane/outer retinal bands/ellipsoid zone often relatively sparing the fovea, (4) characteristic fundus autofluorescence (FAF) abnormalities, (5) full field ERG with reduction of both rod and cone responses, and (6) positive anti-retinal antibodies. Those with some but not all of these features, or with otherwise atypical presentations, can be classified as possible AIR. Features that would make AIR unlikely and should elicit strong suspicion for alternative diagnoses are: (1) slowly progressive symptoms or changes on testing taking place over years, (2) retinal examination with bone spicules, retinal vascular sheathing, or retinal hemorrhages, (3) examination with more than 1+ anterior chamber or vitreous cell/haze, (4) OCT changes predominantly at the level of the RPE or areas of focal/sharply delineated outer retinal/RPE atrophy, (5) fluorescein angiography with diffuse retinal vasculitis or large areas of non-perfusion, or (6) a normal full-field electroretinogram (even with an abnormal multifocal electroretinogram).

Conclusions: These criteria will allow for better classification of patients reported in the literature and improve communication between clinicians. Further study is necessary to optimize the approach for managing AIR and will require collaborative multi-center efforts.