Guijarro Andrea, Muñoz Nelida, Alejandre Nicolas, Recuero Sheila, Sanchez-Pernaute Olga, Carreño Ester
Hospital Universitario Fundacion Jimenez Diaz, Madrid, Spain.
Eur J Ophthalmol. 2020 Dec 21:1120672120981874. doi: 10.1177/1120672120981874.
Acute zonal occult outer retinopathy (AZOOR) is a rare syndrome characterized by sudden onset of photopsia, scotomas, and abnormal electrophysiological tests, predominantly affecting young women. Although its pathogenesis remains unknown, auto-reactivity to retinal components is thought to mediate tissue damage. A 42-year-old woman presented with symptoms and examination consistent with the diagnosis of AZOOR. She was treated with azathioprine for 5 years. In spite of the immunosuppressive treatment, clear progression in the visual field, autofluorescence, electrophysiological tests and optical coherence tomography was observed. Treatment with intravenous immunoglobulins (IVIg) and subcutaneous Abatacept was subsequently started with little efficacy. Hereby, we present a case of progressive AZOOR despite aggressive immunosuppression with 10-year follow up. Currently, there is no consensus regarding management of AZOOR, and the convenience of administering aggressive immunosuppression remains uncertain.
急性区域性隐匿性外层视网膜病变（AZOOR）是一种罕见的综合征，其特征为突然出现闪光感、暗点以及异常的电生理检查结果，主要影响年轻女性。尽管其发病机制尚不清楚，但对视网膜成分的自身反应性被认为介导了组织损伤。一名42岁女性出现了与AZOOR诊断相符的症状和检查结果。她接受了硫唑嘌呤治疗5年。尽管进行了免疫抑制治疗，但仍观察到视野、自发荧光、电生理检查和光学相干断层扫描有明显进展。随后开始使用静脉注射免疫球蛋白（IVIg）和皮下注射阿巴西普治疗，但疗效甚微。在此，我们报告一例尽管进行了积极免疫抑制治疗仍进展的AZOOR病例，并进行了10年随访。目前，关于AZOOR 的治疗尚无共识，积极免疫抑制治疗的便利性仍不确定。
