摘要自身免疫性视网膜病变(AIR)是一种较为罕见的免疫性视网膜病，以视力下降、视物暗点、视野缺损、光感受器功能障碍为主要特点。AIR分为副肿瘤性AIR(pAIR)和非肿瘤性AIR(npAIR)，其中pAIR又分为癌相关性视网膜病变(CAR)、黑色素瘤相关性视网膜病变(MAR)和双眼弥漫性葡萄膜黑色素细胞增生症(BDUMP)。各AIR分类患者外周血往往存在循环性抗视网膜抗体，并伴有视网膜电图异常，但眼底检查无显著异常(BDUMP除外)。在AIR患者血清中已鉴定出抗恢复蛋白抗体和抗α烯醇化酶抗体等多种抗视网膜抗体，但部分AIR患者血清抗视网膜抗体亦可呈阴性反应。目前AIR诊断标准及实验室检查的标准尚不统一，同时患者个体间的临床检查表现存在较大的差异，容易导致误诊和漏诊。因此，在做出推测性诊断之前，需要行全面的检查以排除其他可能的原因。目前AIR各型的治疗方案不统一，多数临床医生采用的治疗方法为各种免疫调节组合治疗，包含全身或局部使用皮质类固醇、静脉注射免疫球蛋白、血浆置换和使用抗代谢药物或CD20单克隆抗体。本文就AIR各分型的临床特点、血清视网膜自身抗体检测、鉴别诊断及治疗预后进行综述，以期提高临床医师和研究工作者对AIR的认识，实现AIR的早期诊断和治疗。

abstractsAutoimmune retinopathy (AIR) is a rare immune retinopathy characterized by decreased visual acuity, scotoma, visual field defect, and photoreceptor dysfunction.AIR is divided into paraneoplastic AIR (pAIR) and non-neoplastic AIR (npAIR). pAIR is further divided into cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and bilateral diffuse uveal melanocytic proliferation (BDUMP). Circulating anti-retinal antibodies often exist in peripheral blood of patients with various types of AIR, accompanied by electroretinogram abnormalities, but no significant abnormality in fundus examination (except BDUMP). A variety of anti-retinal antibodies such as anti-recoverin protein antibody and anti-α-enolase antibody have been identified in the serum of AIR patients.However, anti-retinal antibodies can also be negative in some AIR patients' serum.At present, the diagnostic criteria and laboratory examination criteria for AIR are not uniform, and there are large differences in clinical examination performance among patients, which may lead to misdiagnosis and missed diagnosis.Therefore, a thorough examination is required to rule out other possible causes before making a speculative diagnosis.So far, the treatments for different types of AIR are not unified.Most clinicians choose a combination of various immunomodulatory therapies, including systemic or topical application of corticosteroids, intravenous immunoglobulin, plasmapheresis, and the use of antimetabolites or anti-CD20 monoclonal antibody.The clinical characteristics of different AIR types, serum anti-retinal autoantibodies detection, differential diagnosis and treatment prognosis of AIR were reviewed in this article to improve the understanding of clinicians and researchers toward the disease, and to achieve early diagnosis and early treatment of AIR.