Abstract:
Cancer-associated retinopathy (CAR) is a rare paraneoplastic disorder mediated by auto-antibodies that cross-react with retinal antigens leading to gradual visual defects. Early diagnosis and initiation of treatment is crucial to avoid permanent visual loss. Although most patients with CAR respond to intravenous steroids and intravenous immunoglobulin (IVIG), there are some cases refractory to the aforementioned treatment strategies. The present study describes a case of CAR in a patient with ovarian cancer that was initially resistant to most treatment regimens (chemotherapy, steroids, IVIG). Treatment with rituximab at 375 mg/m2 and oral cyclophosphamide was administered and the patient showed marked improvement of visual acuity. Electroretinogram showed a 40 and 10% improvement in scotopic and photopic vision, respectively. Notably, at the most recent follow up, the patient was still in remission. In conclusion, treatment with intravenous rituximab and oral cyclophosphamide is a promising treatment option for those cases of CAR that do not respond to steroids, immunomodulatory agents and IVIG.
摘要:
癌症相关视网膜病变(CAR)是一种罕见的副肿瘤疾病,由自身抗体介导,与视网膜抗原交叉反应,导致逐渐的视觉缺陷。早期诊断和开始治疗对于避免永久性视力丧失至关重要。尽管大多数CAR患者对静脉注射类固醇和静脉注射免疫球蛋白(IVIG)有反应,有一些病例难以采用上述治疗策略.本研究描述了一例卵巢癌患者的CAR,该患者最初对大多数治疗方案(化疗,类固醇,IVIG)。给予375mg/m2的利妥昔单抗和口服环磷酰胺治疗,患者的视力显着改善。视网膜电图显示暗视和明视视力有40%和10%的改善,分别。值得注意的是,在最近的后续行动中,患者仍处于缓解状态。总之,静脉注射利妥昔单抗和口服环磷酰胺治疗是对类固醇无反应的CAR病例的有希望的治疗选择,免疫调节剂和IVIG。