Department of Ophthalmology, American University of Beirut, Beirut, Lebanon.
Uveitis and Behçet Disease Service, University of Health Sciences, Department of Ophthalmology, Ulucanlar Eye Training and Research Hospital, Ankara, Turkey.
Eye (Lond). 2022 Nov;36(11):2157-2162. doi: 10.1038/s41433-021-01822-5. Epub 2021 Nov 1.
To analyse the entity of retinal vasculitis, including frosted branch angiitis (FBA), or retina vascular occlusion in patients with familial Mediterranean fever (FMF).
Retrospective collaborative case series using invitation by email to uveitis specialists around the Mediterranean basin. This series was combined with a literature review. Exclusion criteria included infectious diseases, Behçet's disease or other autoimmune diseases.
A total of 16 patients (21 eyes) had FMF and retinal vasculitis (FBA 11 patients, mild retinal vasculitis 5 patients). The mean age at onset of vasculitis was 29.5 ± 13.4 (range 9-62) with a female to male ratio of 9 to 7. In 19 eyes treated with various forms of corticosteroid and/or immunosuppression, the mean initial spectacle-corrected visual acuity improved from 6/194 to 6/10.5 at the last mean follow-up of 29.0 ± 34.9 months (p < 0.001). The most common FEVR mutations were M680I and M694V. In addition, retinal vascular occlusions included one case of central retinal artery occlusion and one case of branch retinal artery occlusion.
FBA and milder forms of retinal vasculitis are associated with FMF. Therapy involves an increase in colchicine dosage in early cases, a long period of oral corticosteroid, intravitreal dexamethasone implant or periocular corticosteroid in select cases, and combination therapy with systemic immunosuppression in severe cases. FMF needs to be included in the differential diagnosis of retinal vasculitis.
分析家族性地中海热（FMF）患者视网膜血管炎的实体，包括霜样树枝状血管炎（FBA）或视网膜血管阻塞。
采用电子邮件邀请地中海盆地周围的葡萄膜炎专家进行回顾性合作病例系列研究。该系列研究结合了文献复习。排除标准包括传染病、贝切特病或其他自身免疫性疾病。
共 16 名（21 只眼）患者患有 FMF 和视网膜血管炎（FBA11 例，轻度视网膜血管炎 5 例）。血管炎发病的平均年龄为 29.5±13.4 岁（范围 9-62 岁），女性与男性的比例为 9:7。在 19 只接受各种形式皮质类固醇和/或免疫抑制治疗的眼中，初始矫正视力从 6/194 提高到 6/10.5，平均最后随访时间为 29.0±34.9 个月（p<0.001）。最常见的 FEVR 突变是 M680I 和 M694V。此外，视网膜血管阻塞包括 1 例中央视网膜动脉阻塞和 1 例分支视网膜动脉阻塞。
FBA 和较轻微的视网膜血管炎与 FMF 有关。治疗包括早期增加秋水仙碱剂量、长期口服皮质类固醇、在选择病例中使用玻璃体内地塞米松植入物或眶周皮质类固醇，以及在严重病例中联合使用全身免疫抑制治疗。FMF 需要纳入视网膜血管炎的鉴别诊断。
