副肿瘤性视网膜病变的发病机制及临床特点

副肿瘤性视网膜病变(PR)是一种少见的副肿瘤综合征,因肿瘤表达异常抗原,产生自身抗体与视网膜自身抗原发生交叉免疫反应,导致病变.PR包括癌症相关性视网膜病变(CAR)、黑色素瘤相关性视网膜病变(MAR)及双侧弥漫性葡萄膜黑色素细胞增生症(BDUMP).目前发现多种导致CAR的自身抗体,如抗恢复蛋白(recoverin)抗体、抗α烯醇化酶抗体及抗转导蛋白-α(transducin-α)抗体等,可以与视网膜相应的自身抗原结合;导致MAR的自身抗体最主要为抗TRPM1抗体,可以与视网膜双极细胞离子通道的TRPM1结合;导致BDUMP的抗体目前只发现抗recoverin抗体和抗热休克蛋白70(HSP70)抗体.PR的临床表现主要为短期内双眼视力下降及夜盲等,眼底表现可正常或轻度异常,视网膜电图(electroretinogram,ERG)表现为不同类型的异常,其中BDUMP有双眼多发橘红色斑块等特殊表现.临床表现往往因自身抗体类型的不同而存在差异,主要原因在于针对自身抗原的不同生理作用,表现为发病时间、进展速度、严重程度及ERG表现等.目前对于PR的治疗主要为治疗原发肿瘤,及针对自身抗体进行糖皮质激素、血浆置换及免疫球蛋白等单独及联合的治疗.

The mechanism and clinical features of paraneoplastic retinopathy

Paraneoplastic retinopathy (PR) is a rare form of paraneoplastic syndrome,it occurs when autoantibodies of neoplasms cross-react with autoantigens in normal retina.PR includes carcinoma-associated retinopathy (CAR),melanoma-associated retinopathy (MAR) and bilateral diffuse uveal melanocytic proliferation (BDUMP).Many kinds of autoantigens are involved in the process of CAR,like recoverin,α-enolase,α-transducin and so on.TRPM1 is a component of an ion channel of bipolar cell,it plays an important role in MAR.There are fewer kinds of antibodies associated with BDUMP,only anti-recoverin and anti-heat shock protein 70 (HSP70) antibodies were found in one particular research.The clinical manifestation of PR includes rapid vision loss of both eyes,photopsia and nyctalopia.The fundus can be normal or mild abnormal.Electroretinogram (ERG) presents various abnormalities based on the type of PR and autoantibody.The treatment of PR mainly targets on the original tumor,meanwhile glucocoticoids,plasmapheresis and intravenous immune globulin are also applied singly or combinedly in clinic.