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Nonparaneoplastic Autoimmune Retinopathy: Scoping Review and Suggested Reporting Guidelines
Daniel Adamkiewicz1, Sruthi Arepalli1, Kubra Sarici1
1Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia, USA.
Ocular Immunology and Inflammation
|December 16, 2025
Summary
Diagnostic approaches for nonparaneoplastic autoimmune retinopathy (npAIR) show variability. While electroretinography and antibody testing are common, advanced methods like widefield autofluorescence and genetic testing are underutilized, hindering disease understanding.
Area of Science:
- Ophthalmology
- Autoimmune Diseases
- Retinal Disorders
Background:
- Nonparaneoplastic autoimmune retinopathy (npAIR) is a rare condition affecting vision.
- Current diagnostic criteria and approaches for npAIR lack standardization.
- Understanding diagnostic trends is crucial for improving patient outcomes.
Purpose of the Study:
- To analyze trends in the diagnostic methods used for nonparaneoplastic autoimmune retinopathy (npAIR).
- To identify common and underutilized diagnostic tools in npAIR literature.
Main Methods:
- A systematic literature search was conducted on PubMed for npAIR clinical reports from January 2016 to September 2025.
- Articles were reviewed to assess diagnostic criteria and categorized into case reports or case series.
- Data on diagnostic tests, author subspecialty, and patient numbers were extracted.
Main Results:
- 77 articles (755 cases) were analyzed, with contributions from uveitis, inherited retinal disease (IRD), and retina specialists.
- Electroretinography and anti-retinal antibody testing were reported in over 80% of publications.
- Fundus autofluorescence (FAF) and genetic testing were less frequently utilized, particularly widefield FAF and genetic testing in non-IRD specialist studies.
Conclusions:
- Variability in classification and incomplete reporting challenge the diagnosis of npAIR.
- Widespread use of electroretinography and antibody testing contrasts with underutilization of advanced techniques like widefield FAF and genetic testing.
- Updating diagnostic criteria using these underused tools can improve understanding of npAIR's natural history.
