Abstract
PURPOSE: To provide a comprehensive overview of the existing understanding regarding the clinical characteristics, diagnostic investigation, and treatment strategies for autoimmune retinopathy (AIR). To emphasize the lack of consensus in the field and the ongoing controversies regarding best practices. METHODS: Narrative review of the literature on PubMed and Google Scholar databases. RESULTS: AIR comprises a group of rare autoimmune disorders causing retinal degeneration, characterized by rapid vision deterioration linked to circulating anti-retinal autoantibodies (ARAs). The spectrum includes primary autoimmune diseases and associations with retinitis pigmentosa or various malignancies, raising questions about the causative role of antibodies. Non-paraneoplastic AIR patients may be younger on average than paraneoplastic AIR patients and may have a higher likelihood of being female and having a history of autoimmune disease. Diagnosing AIR is challenging due to its complex pathophysiology, overlapping phenotypes, the absence of standardized diagnostic criteria, and the limited availability of specialized serological testing for ARAs. Despite decades of research progress, the exact mechanisms underlying ocular immune privilege breakdown and the autoimmune attack on retinal cells in AIR remain unknown. CONCLUSIONS: The diagnosis and management of AIR present ongoing challenges, with no international consensus on diagnostic criteria or treatment protocols. While several authors consider the identification of circulating ARAs crucial for diagnosis, there is disagreement on the pathogenicity of specific antibodies, including anti-recoverin. The lack of randomized controlled trials and a universally accepted treatment protocol for AIR further contribute to uncertainties in its management. Despite recent advances, AIR remains an enigmatic condition, necessitating further research to establish standardized diagnostic and therapeutic guidelines.