Department of Ophthalmology, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, Israel.
Turk J Ophthalmol. 2022 Aug 25;52(4):286-290. doi: 10.4274/tjo.galenos.2022.69337.
Familial Mediterranean fever (FMF) is a rare autoinflammatory disorder. Ocular involvement is rare. The full spectrum and response to treatment is poorly understood. An 18-year-old girl previously diagnosed with FMF presented with sudden loss of vision in the left eye (LE). Best-corrected visual acuity (BCVA) in the LE was finger counting at 1.5 meters. Angiitis with mild "frosting," hemi-central retinal vein occlusion (HCRVO), and acute outer retina rupture (ORR) were observed in the LE. Systemic steroids were initiated immediately. The ORR was sealed 2 weeks later while vision improved to 6/15 (near vision: J2) 5 months later. No recurrences were observed over 5 years of follow-up. We report a rare manifestation of frosted branch angiitis with concomitant HCRVO and ORR in a young patient with FMF. Closure of ORR was attained and vision recovered after treatment with high-dose steroids.
家族性地中海热（FMF）是一种罕见的自身炎症性疾病。眼部受累较为罕见，其全貌和治疗反应尚不清楚。一位 18 岁的女孩既往被诊断为 FMF，左眼（LE）突然失明。LE 的最佳矫正视力（BCVA）为在 1.5 米处只能指数辨认。在 LE 中观察到血管炎伴轻微“霜样”改变、半中心性视网膜静脉阻塞（HCRVO）和急性外层视网膜破裂（ORR）。立即开始给予全身类固醇治疗。2 周后 ORR 封闭，5 个月后视力提高至 6/15（近视力：J2）。在 5 年的随访中未观察到复发。我们报告了一例在 FMF 年轻患者中罕见的霜样分支血管炎伴同时发生的 HCRVO 和 ORR 的病例。通过大剂量类固醇治疗，ORR 闭合，视力恢复。
