Nonparaneoplastic Autoimmune Retinopathy: Scoping Review and Suggested Reporting Guidelines

To investigate trends in the diagnostic approach to nonparaneoplastic autoimmune retinopathy (npAIR).

We queried PubMed for clinical reports on npAIR published between January 2016 and September 2025. Articles were assessed to determine criteria used to establish diagnosis of npAIR using a standardized grading system. Articles were categorized as case reports (≤3 patients) or case series (>3 patients).

36 case reports and 41 case series met eligibility criteria (755 total cases). Author subspecialty included 34% uveitis, 20% inherited retinal disease (IRD), 16% general retina, 10% miscellaneous, and 19% unknown specialty. Over 80% of publications reported electroretinography and anti-retinal antibody testing for diagnosis of npAIR. Fundus autofluorescence (FAF) was performed in 67% of case reports and at least one patient in 51% of case series. Widefield FAF was used in 19% of case reports and in at least one patient in 20% of case series. Genetic testing was reported in 22% of case reports and in at least one patient in 27% of case series. Studies with an IRD specialist as first or last author most commonly used genetic testing (35%).

Literature on npAIR is hampered by variability in classification schemes and incomplete reporting. Nonspecific electroretinography testing and antiretinal antibody testing are widely employed while widefield autofluorescence testing and genetic testing are not commonly used. Expanded access to these tools provides an opportunity to update diagnostic criteria of npAIR. Improved classification will permit us to better understand the natural history of disease.