Unilateral cancer-associated retinopathy: diagnosis, serology and treatment
Unilateral cancer-associated retinopathy: diagnosis, serology and treatment
复制标题
DOI：
10.1007/s10633-017-9605-y
复制
发表时间：
2017-12-01
影响因子：
1.4
通讯作者：
Leroy, Bart P.
中科院分区：
文献类型：
--
作者：
Roels, Dimitri;Ueno, Shinji;Leroy, Bart P.
报告一例单侧癌症相关性视网膜病变（CAR）患者，经积极的癌症治疗联合类固醇和利妥昔单抗治疗后，临床、血清学和视网膜电图（ERG）恢复正常。一位45岁的女性，自6周前开始出现进行性眼盲、眼恐怖及相对的右眼中心暗点。双眼BCVA均为1.0。生物显微镜检查、IOP和眼底检查无异常。此外，色觉、自体荧光成像、OCT和EOG均正常。视野显示右眼中央灵敏度降低。ERG表现为单侧、负性联合和ON双极反应。疑似CAR诊断。在诊断为右侧卵巢腺癌后，进行了根治性卵巢切除术和子宫切除术，随后进行了辅助化疗。全身PET扫描显示无转移。开始利妥昔单抗单克隆抗体联合皮质类固醇治疗。该患者针对TRPM 1（一种在ON双极细胞中表达的瞬时受体电位阳离子通道）的血清自身抗体检测呈阳性。治疗期间，症状逐渐改善，ERG恢复正常。血清学证实自身抗体完全清除。虽然罕见，但单侧CAR确实发生，在临床高度怀疑的情况下，肿瘤学检查是强制性的。结合类固醇和利妥昔单抗的侵袭性癌症治疗可导致临床和ERG表型正常化，清除抗视网膜抗体。
To report a case of unilateral cancer-associated retinopathy (CAR) with clinical, serological and electroretinogram (ERG) normalization after aggressive cancer treatment combined with steroids and rituximab.Work-up included extensive clinical and electrophysiological testing. Also, serological work-up for antiretinal antibodies and oncological screening was organized.A 45-year-old female presented with progressive photopsias, photophobia and relative central scotoma in the right eye since 6 weeks prior. BCVA was 1.0 in both eyes. Biomicroscopy, IOP and fundus exam were unremarkable. Also, colour vision, autofluorescence imaging, OCT and EOG were normal. Visual fields showed decreased central sensitivity in the right eye. ERG showed a unilateral, electronegative combined and ON-bipolar response. A diagnosis of CAR was suspected. After a diagnosis of an adenocarcinoma of the right ovary, radical ovariectomy and hysterectomy were performed, followed by adjuvant chemotherapy. A whole-body PET scan revealed no metastasis. Treatment with rituximab monoclonal antibodies in combination with corticosteroids was initiated. The patient tested positive for serum autoantibodies against TRPM1, a transient receptor potential cation channel expressed in ON-bipolar cells. During treatment, there was progressive improvement in symptoms and the ERG normalized. Serology confirmed complete clearance of autoantibodies.Although rare, unilateral CAR does occur and in cases with high clinical suspicion an oncological work-up is mandatory. Aggressive cancer treatment combined with steroids and rituximab can lead to normalization of the clinical and ERG phenotype, with clearing of antiretinal antibodies.