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 provided 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 the 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 6 months; (2) examination with <1+ anterior chamber cells, vitreous cells, or vitreous haze; (3) 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 abnormalities; (5) full-field electroretinogram (ERG) with reduction of both rod and cone responses; and (6) positive antiretinal 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 as follows: (1) slowly progressive symptoms or changes on testing taking place over the years; (2) retinal examination with bone spicules, retinal vascular sheathing, or retinal hemorrhages; (3) examination with >1+ anterior chamber cells, vitreous cells, or vitreous haze; (4) OCT changes predominantly at the level of the retinal pigment epithelium (RPE) or areas of focal/sharply delineated outer retinal/RPE atrophy; (5) fluorescein angiography with diffuse retinal vasculitis or large areas of nonperfusion; or (6) a normal full-field ERG (even with an abnormal multifocal ERG).

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 multicenter efforts.

FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.