伴有免疫功能受损的急性视网膜坏死综合征的临床特征及预后

目的 观察长期应用免疫抑制药物后发生的急性视网膜坏死综合征（ARNS）患者的临床特征及预后。 方法 回顾分析经裂隙灯显微镜、前置镜、血清学检查、荧光素眼底血管造影检查明确ARN诊断，病史及相关检查证实伴有全身免疫功能受损的患者8例12只眼的临床资料。患者中男性2例，女性6例，年龄35～54岁，平均年龄44.2岁。伴有血液病2例、胸腺肿瘤2例（1例合并脑炎)、脑膜脑炎2例、系统性红斑狼疮（SLE）1例、急性肺炎1例；发病前均长期应用免疫抑制药物。5只眼视力低于0.05，占41.7%；3只眼视力为0.05～0.3，占25.0%；4只眼视力为0.3～1.0，占33.3%。确诊ARN后根据不同病情行抗病毒药物、激光光凝和（或）手术治疗。治疗后随访3～36个月，平均随访时间10.8个月。 结果 所有患者发病时眼部症状及葡萄膜炎表现较轻，视网膜血管炎和坏死灶进展较慢。7只眼视网膜血管炎累及4个象限，2只眼介于1～2个象限,3只眼不超过1个象限。10只眼视网膜坏死灶侵及周边部及中周部视网膜，占83.4％；2只眼视网膜坏死灶侵及周边部至后极部，占16.6%。随访结束时7只眼视力不同程度提高，最终视力0.05以下者4只眼，占33.3%； 0.05～0.3者2只眼，占16.7%；0.3～1.0者6只眼，占50.0%。 结论 伴有全身免疫功能受损的ARN患者病史明确，临床特征典型，但眼部症状及葡萄膜炎表现较轻，视网膜血管炎和坏死灶进展较慢；及时确诊和治疗可以使患者获得有用视力。

Clinical features and treatment outcomes of acute retinal necrosis syndrome in immunocompromised patients

Objective To evaluate the clinical features and treatment outcomes of acute retinalnecrosis syndrome(ARNS)which caused by long-term usage of immunosuppressent drug.MethodsThe clinical data of 8 patients(12 eyes)with immunocompromised-related ARNS were retrospectivelyreviewed.The diagosis was made by medical history,slit-lamp microscopy,pre-set lenses check,serologicexamination and fluorescein fundus angiography.The patients(2 males and 6 females)aged from 35 to 54years,with the mean age of 44.2 years.Previous medical history included hematopathy(2 cases),thymustumor(2 cases,one also with meningoencephalitis),meningoencephalitis (2 cases),systemic lupuserythematosus(1 case)and acute pneumonia(1 case).All patients received immunosuppressent therapyfor a long time before ARNS occurred.The visual acuity was<0.05(5 eyes,41.7%),or 0.05-0.3(3eyes,25.0%),or 0.3-1.0(4 eyes,33.3%).Those ARNS patients received antiviral therapy,laserphotocoagulation and(or)surgery therapy.The mean followed-up period was 10.8 months(from 3 to 36months).Results The eye sympotoms and uveitis of all patients were very mild,and their retinalvasculitis and retinal necrosis progressed slowly.Retinal vasculitis was involved in 4 quadrants(7 eyes),or 1-2 quadrants(2 eyes),or<1 quadrant(3 eyes).Retinal necrosis extended from peripheral retina tomid-periphcral retina(10 eyes,83.4%),or from peripheral retina to posterior pole(2 eyes,16.6%).Atthe end of the follow-up period,the visual acuity of 7 eyes(58.3%)showed different degree ofimprovement.The follow-up visual acuity was<0.05(4 eyes,33.3%),or 0.05-0.3(2 eyes,16.6%),or 0.3-1.0(6 eyes,50%).Conclusions Immunocompromised ARNS patients had valid medical historyand typical clinical features.However the eye sympotoms and uveitis were very mild.retinal vasculitis andretinal necrosis progressed slowly in this study.Early diagnosis and prompt therapy may save the visualacuity of those patients.