Familial Mediterranean fever associated frosted branch angiitis, retinal vasculitis and vascular occlusion,Eye

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Familial Mediterranean fever associated frosted branch angiitis, retinal vasculitis and vascular occlusion
Eye ( IF 2.8 ) Pub Date : 2021-11-01 , DOI: 10.1038/s41433-021-01822-5
Hana A Mansour ₁ , Pinar Ç Ozdal ₂ , Sibel Kadayifcilar ₃ , Ilknur Tugal-Tutkun ₄ , Hilal Eser-Ozturk ₅ , F Nilüfer Yalçındağ ₆ , Harry Petrushkin _{7,

8} , Errol W Chan _{9,

10} , Soukaina Belfaiza ₁₁ , Remzi Karadag _{12,

13} , Sirel Gür Güngör ₁₄ , Maurizio Battaglia Parodi ₁₅ , Ahmad M Mansour _{1,

16}

Affiliation

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.
Department of Ophthalmology, Hacettepe University, Ankara, Turkey.
Department of Ophthalmology, Istanbul University, Istanbul, Turkey.
Department of Ophthalmology, Ondokuz Mayıs University, Samsun, Turkey.
Department of Ophthalmology, University of Ankara, Ankara, Turkey.
Uveitis and Scleritis Service, Moorfields Eye Hospital, London, UK.
Pediatric Uveitis Service, Great Ormond St Hospital, London, UK.
Department of Ophthalmology and Visual Sciences, McGill University, Montreal, Canada.
Department of Ophthalmology, National University Health System, Singapore, Thailand.
Department of Ophthalmology, Mohammed V Military Hospital, Rabat, Morocco.
RK Eye, Aesthetic and Health Services, Kadikoy, Istanbul, Turkey.
Veni Vidi Eye Center, Caddebostan, Kadikoy, Istanbul, Turkey.
Department of Ophthalmology, Başkent University, Ankara, Turkey.
Department of Ophthalmology, University Vita-Salute, IRCCS Ospedale San Raffaele, Milan, Italy.
Department of Ophthalmology, Rafic Hariri University Hospital, Beirut, Lebanon.

Objectives

To analyse the entity of retinal vasculitis, including frosted branch angiitis (FBA), or retina vascular occlusion in patients with familial Mediterranean fever (FMF).

Methods

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.

Results

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.

Conclusion

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.

中文翻译：

家族性地中海热相关的霜枝血管炎、视网膜血管炎和血管闭塞

目标

分析家族性地中海热（FMF）患者视网膜血管炎的实体，包括霜枝血管炎（FBA）或视网膜血管闭塞。

方法

通过电子邮件邀请地中海盆地葡萄膜炎专家进行回顾性合作病例系列。该系列与文献综述相结合。排除标准包括传染病、白塞氏病或其他自身免疫性疾病。

结果

共有 16 例患者（21 只眼）患有 FMF 和视网膜血管炎（FBA 11 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），女性与男性的比例为 9 比 7。在接受各种形式的皮质类固醇和/或免疫抑制剂治疗的 19 只眼睛中，平均初始眼镜矫正视力在最后一次平均随访 29.0 ± 34.9 个月时，从 6/194 提高到 6/10.5 ( 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视网膜中央动脉阻塞1例和视网膜分支动脉阻塞1例。