自身免疫性视网膜病变的临床特征与研究进展

Clinical features and research progress of autoimmune retinopathy

自身免疫性视网膜病变(AIR)是一组罕见的自身免疫相关的视网膜变性疾病,考虑与血清自身抗体和视网膜抗原发生交叉免疫反应有关,主要包括癌症相关性视网膜病变、黑色素瘤相关性视网膜病变以及非副肿瘤性自身免疫性视网膜病变等。该类疾病以无痛性进行性视力下降、视网膜电图检查明显异常以及血清抗视网膜抗体检测阳性为主要临床特征。由于AIR发病率较低,早期临床症状隐匿,且缺乏特异敏感的实验室指标加大了疾病诊断的难度,但及时的诊断及治疗对避免形成不可逆性视网膜免疫损伤有重要意义。

Autoimmune retinopathy(AIR)is an umbrella term for a group of rare autoimmune retinal degenerative disease presumably caused by cross-reactivity of serum autoantibodies directed against ratinal antigens,and include cancer-associated retinopathy,melanoma-associated retinopathy and non-paraneoplastic autoimmune retinopathy.Common feature of AIR include progressively painless vision loss with abnormal electrophysiology responses associated with positive anti-retinal antibodies.They present a clinical diagnosis challenge on account of the rare incidence,unobvious clinical symptoms and lack of specific and sensitive biological markers.Early diagnosis and treatment may play a critical role to avoid the irreversible immunological retinal damage.