Department of Ophthalmology and Visual Science, Chiba University Graduate School of Medicine, Inohana 1-8-1, Chuo-ku, Chiba, 260-8670, Japan.
Department of Ophthalmology, Keio University, Tokyo, Japan.
BMC Ophthalmol. 2020 Apr 6;20(1):132. doi: 10.1186/s12886-020-01414-z.
Evidence-based criteria for the treatment of autoimmune retinopathy (AIR) have not been established. The pathology and clinical features of each antibody causing AIR, and its long-term course are still undetermined. We report our findings in a case of non-paraneoplastic AIR (npAIR) that developed in the fellow eye 10 years after the onset in the first eye.
Our patient had photophobia in both eyes and a rapidly progressing visual field defect in his right eye at the initial examination. He was diagnosed with non-paraneoplastic AIR based on the clinical findings and immunoblot analyses for anti-retinal antibodies, and he was treated with steroids. Ten years later, a visual field defect developed in the fellow eye, and a diagnosis of npAIR was made. Immunoblot analyses were positive for anti-α-enolase antibodies. He was treated with steroids, immunosuppressants, and plasma exchange. However, the response to the treatment was poor and both eyes eventually became blind.
As best we know, this is the first case report of npAIR that developed in the fellow eye over 10 years after the development in the first eye. Long-term follow-up and a search for tumor lesions are necessary in cases of npAIR. Further understanding of the long-term course of AIR can contribute to an understanding of the pathology and treatment of npAIR.
目前尚未确立治疗自身免疫性视网膜炎（AIR）的循证标准。每种引起 AIR 的抗体的病理和临床特征及其长期病程尚不确定。我们报告了一例非副肿瘤性 AIR（npAIR）病例，该患者在第一只眼发病 10 年后，对侧眼出现该病。
我们的患者双眼畏光，右眼视野缺损迅速进展，初次检查时被诊断为非副肿瘤性 AIR，依据为临床检查结果和抗视网膜抗体免疫印迹分析，并接受了类固醇治疗。10 年后，对侧眼出现视野缺损，诊断为 npAIR。免疫印迹分析显示抗α-烯醇化酶抗体阳性。他接受了类固醇、免疫抑制剂和血浆置换治疗。但治疗反应不佳，最终双眼失明。
据我们所知，这是首例第一只眼发病 10 年后对侧眼发生 npAIR 的病例报告。在 npAIR 病例中需要进行长期随访和肿瘤病变检查。进一步了解 AIR 的长期病程有助于了解 npAIR 的病理和治疗。
