Parc Christine E, Azan Elodie, Bonnel Sébastien, Sahel José Alain, Kaplan Josseline, Thirkill Charles E
Department of Ophthalmology, Hôpital Foch, Suresnes, France.
Ophthalmic Genet. 2006 Jun;27(2):57-61. doi: 10.1080/13816810600678097.
Reports on cancer-associated retinopathies (CAR) have increased in recent years as the autoimmune reactions responsible have become better understood. The 23-kDalton autoantigen 'recoverin' was the first retinal antigen implicated in CAR, but others have since been described. We report an additional case involving an autoantigen other than recoverin, with five-year follow-up of a 50-year-old woman suffering from the CAR syndrome, with ocular abnormalities restricted to cone dysfunction. The patient had a history of laryngeal carcinoma surgically removed 18 months prior to presenting to the ophthalmologist with photophobia and decreased vision in both eyes. The patient's abnormal retinal hypersensitivity included antibody activity with two retinal antigens approximating 40 kD, located within the outer segments of the photoreceptor layer. To our knowledge, this is the first description of a cancer-associated, cone-specific dystrophy involving an abnormal amount of immunologic activity with two retinal proteins approximating 40 kD, that may prove to be isoforms of the same antigen.
近年来,随着对相关自身免疫反应的深入了解,关于癌症相关性视网膜病变(CAR)的报告有所增加。23千道尔顿的自身抗原“恢复蛋白”是首个被认为与CAR有关的视网膜抗原,但此后又发现了其他抗原。我们报告了另外一例涉及除恢复蛋白之外的自身抗原的病例,对一名患有CAR综合征的50岁女性进行了为期五年的随访,其眼部异常仅限于视锥细胞功能障碍。该患者有喉癌病史,在出现畏光和双眼视力下降症状并就诊于眼科医生之前18个月,喉癌已通过手术切除。患者异常的视网膜超敏反应包括与两种约40kD的视网膜抗原的抗体活性,这些抗原位于光感受器层的外段。据我们所知,这是首次描述一种与癌症相关的、视锥细胞特异性营养不良,涉及与两种约40kD的视网膜蛋白异常的免疫活性,这两种蛋白可能是同一抗原的异构体。