Kalogeropoulos Dimitrios, Lotery Andrew John, Pavesio Carlos, Kalogeropoulos Chris, Kanavaros Panagiotis, Afshar Farid, Shawkat Fatima, De Salvo Gabriella
Southampton Eye Unit, University Hospital Southampton, 103 Tremona Rd, Southampton, SO16 6HU, UK.
Faculty of Medicine, University of Southampton, Southampton, UK.
Int Ophthalmol. 2025 Aug 18;45(1):341. doi: 10.1007/s10792-025-03696-y.
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.
Narrative review of the literature on PubMed and Google Scholar databases.
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.
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.
全面概述目前对自身免疫性视网膜病变（AIR）的临床特征、诊断检查及治疗策略的认识。强调该领域缺乏共识以及关于最佳实践的持续争议。
对PubMed和谷歌学术数据库中的文献进行叙述性综述。
AIR是一组导致视网膜变性的罕见自身免疫性疾病，其特征是与循环抗视网膜自身抗体（ARA）相关的快速视力下降。其范围包括原发性自身免疫性疾病以及与色素性视网膜炎或各种恶性肿瘤的关联，这引发了关于抗体致病作用的疑问。非副肿瘤性AIR患者的平均年龄可能比副肿瘤性AIR患者年轻，女性患病率和自身免疫性疾病病史的可能性可能更高。由于其复杂的病理生理学、重叠的表型、缺乏标准化诊断标准以及ARA专门血清学检测的可用性有限，诊断AIR具有挑战性。尽管经过数十年的研究进展，但AIR中眼免疫赦免破坏和视网膜细胞自身免疫攻击的确切机制仍不清楚。
AIR的诊断和管理目前仍面临挑战，在诊断标准或治疗方案上没有国际共识。虽然一些作者认为识别循环ARA对诊断至关重要，但对于包括抗恢复蛋白在内的特定抗体的致病性存在分歧。缺乏针对AIR的随机对照试验和普遍接受的治疗方案进一步加剧了其管理的不确定性。尽管最近取得了进展，但AIR仍然是一种神秘的疾病，需要进一步研究以建立标准化的诊断和治疗指南。
