Clinical Features of Japanese Patients With Anti-α-enolase Antibody?Positive Autoimmune Retinopathy: Novel Subtype of Multiple Drusen
Clinical Features of Japanese Patients With Anti-α-enolase Antibody?Positive Autoimmune Retinopathy: Novel Subtype of Multiple Drusen
复制标题
日本抗α-烯醇化酶抗体阳性自身免疫性视网膜病变患者的临床特征：多发性玻璃膜疣的新亚型
DOI：
10.1016/j.ajo.2018.08.044
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发表时间：
2018
影响因子：
4.2
通讯作者：
Ishida Susumu
中科院分区：
文献类型：
--
作者：
Ando Ryo;Saito Wataru;Kanda Atsuhiro;Kase Satoru;Fujinami Kaoru;Sugahara Michitaka;Nakamura Yosuke;Eguchi Shuichiro;Mori Shohei;Noda Kousuke;Shinoda Kei;Ishida Susumu
目的探讨抗α-烯醇化酶抗体阳性的日本自身免疫性视网膜病变（anti-烯醇化酶AIR）患者的临床特点。设计多中心回顾性观察病例系列。方法选取25例日本抗烯醇化酶AIR患者49只眼，其中女性16例，男性9例，平均初诊年龄60.8岁。评估眼底特征、视野、光谱域光学相干断层扫描（SD-OCT）、视网膜电图（ERG）、最佳矫正视力（BCVA）和系统性肿瘤的并发症。用免疫组化方法检测1例去核眼α-烯醇化酶的蛋白定位。结果将患者分为3组：多发性肾盂（48%）、视网膜变性（36%）和眼底正常（16%）。德鲁森大小不一，从小的沉积物到卵黄样病变。SD-OCT图像显示视网膜色素上皮（RPE）下方呈圆顶状高反射率，与drusen相对应。视野检查显示环状暗斑最常见（39%）。视杆系统和/或单闪视锥反应显示81%的眼睛反应下降。杆状体和锥体系统的联合反应表明，变性组的a波振幅明显低于衰老组（P= 0.005）。随访期间，80%的眼睛BCVA得到改善或维持。30%的患者检出恶性或良性肿瘤。RPE和感光层α-烯醇化酶免疫阳性。结论日本抗烯醇化酶AIR患者的特征为drusen亚型，文献中很少有描述。不同的眼底特征和不同的功能严重程度可能是由于抗体介导的RPE和光感受器细胞损伤所致。
PurposeTo evaluate clinical features of Japanese patients with anti-α-enolase antibody–positive autoimmune retinopathy (anti-enolase AIR).DesignMulticenter retrospective observational case series.MethodsForty-nine eyes of 25 Japanese anti-enolase AIR patients (16 female and 9 male; mean age at first visit, 60.8 years) were included. Fundus characteristics, perimetry, spectral-domain optical coherence tomography (SD-OCT), electroretinography (ERG), best-corrected visual acuity (BCVA), and complicating systemic tumors were assessed. Protein localization of α-enolase was examined by immunohistochemistry in an enucleated eye of 1 patient.ResultsPatients were classified into 3 groups: multiple drusen (48%), retinal degeneration (36%), and normal fundus (16%). Drusen varied in size from small deposits to vitelliform-like lesions. Images on SD-OCT revealed dome-shaped hyperreflectivity beneath the retinal pigment epithelium (RPE), corresponding to drusen. Perimetry showed that ring scotoma was the most frequent (39%). Rod-system and/or single-flash cone responses revealed decreased responses in 81% of the eyes. Combined rod and cone system responses demonstrated significantly lower a-wave amplitudes in the degeneration group than in the drusen group (P= .005). BCVA was improved or maintained in 80% of the eyes during follow-up. Malignant or benign tumors were detected in 30% of patients. The RPE and photoreceptor layers were immunopositive for α-enolase.ConclusionsThe drusen subtype, scarcely described in the literature, is suggested to characterize Japanese patients with anti-enolase AIR. The different funduscopic features with different functional severities may have resulted from antibody-mediated damage to RPE as well as photoreceptor cells.