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继发于家族性地中海热的霜样树枝状视网膜血管炎,类似视网膜中央静脉阻塞

Frosted Branch Angiitis Secondary to Familial Mediterranean Fever Resembling Central Retinal Vein Occlusion.

作者信息

Ozates Serdar, Ozdal Pınar Çakar, Teke Mehmet Yasin

机构信息

Ankara Ulucanlar Eye Education and Research Hospital, Ankara, Turkey.

出版信息

Case Rep Ophthalmol Med. 2016;2016:2916027. doi: 10.1155/2016/2916027. Epub 2016 Dec 4.

DOI:10.1155/2016/2916027
PMID:28044118
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5164904/
Abstract

. To report a case of unilateral frosted branch angiitis (FBA) resembling central retinal vein occlusion associated with Familial Mediterranean Fever (FMF). . A 32-year-old woman presented with progressive, painless vision loss in her left eye lasting for 2 days. She was clinically diagnosed with FMF 2 months ago. The best-corrected visual acuity (BCVA) was 20/20 in her right eye and there was light perception in the left. Ophthalmologic examination revealed severe retinal vasculitis showing clinical features of FBA in the left eye. 64 mg/day oral methylprednisolone was started. A significant improvement in retinal vasculitis was observed in two weeks. However, BCVA did not increase significantly due to subhyaloid premacular hemorrhage. Argon laser posterior hyaloidotomy was performed. One week after hyaloidotomy, visual acuity improved to 20/20 and intravitreal hemorrhage disappeared. Four months after the first attack, FBA recurred. Oral methylprednisolone dosage was increased to 64 mg/day and combined with azathioprine 150 mg. At the end of 12-month follow-up, the BCVA was 20/25 and development of epiretinal membrane was observed in the left eye. . Frosted branch angiitis may occur with gene abnormalities as an underlying condition. Our case showed that FMF might be a causative disease.

摘要

报告一例类似视网膜中央静脉阻塞的单侧霜样树枝状视网膜血管炎(FBA),其与家族性地中海热(FMF)相关。一名32岁女性,左眼渐进性、无痛性视力丧失2天。她于2个月前临床诊断为FMF。右眼最佳矫正视力(BCVA)为20/20,左眼仅存光感。眼科检查发现左眼有严重的视网膜血管炎,表现为FBA的临床特征。开始口服甲泼尼龙64mg/天。两周后观察到视网膜血管炎有显著改善。然而,由于玻璃膜下黄斑前出血,BCVA未显著提高。进行了氩激光后玻璃体切割术。玻璃体切割术后一周,视力提高到20/20,玻璃体内出血消失。首次发作四个月后,FBA复发。口服甲泼尼龙剂量增加到64mg/天,并联合硫唑嘌呤150mg。在12个月随访结束时,BCVA为20/25,左眼观察到视网膜前膜形成。霜样树枝状视网膜血管炎可能以基因异常为潜在病因发生。我们的病例表明FMF可能是致病疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/638e/5164904/12d7ca62cc0d/CRIOPM2016-2916027.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/638e/5164904/b7d3998fefb5/CRIOPM2016-2916027.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/638e/5164904/b3d58ef92175/CRIOPM2016-2916027.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/638e/5164904/12d7ca62cc0d/CRIOPM2016-2916027.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/638e/5164904/b7d3998fefb5/CRIOPM2016-2916027.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/638e/5164904/b3d58ef92175/CRIOPM2016-2916027.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/638e/5164904/12d7ca62cc0d/CRIOPM2016-2916027.003.jpg

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