Hana A Mansour1, Pinar Ç Ozdal2, Sibel Kadayifcilar3, Ilknur Tugal-Tutkun4, Hilal Eser-Ozturk5, F Nilüfer Yalçındağ6, Harry Petrushkin7,8, Errol W Chan9,10, Soukaina Belfaiza11, Remzi Karadag12,13, Sirel Gür Güngör14, Maurizio Battaglia Parodi15, Ahmad M Mansour16,17. 1. Department of Ophthalmology, American University of Beirut, Beirut, Lebanon. 2. Uveitis and Behçet Disease Service, University of Health Sciences, Department of Ophthalmology, Ulucanlar Eye Training and Research Hospital, Ankara, Turkey. 3. Department of Ophthalmology, Hacettepe University, Ankara, Turkey. 4. Department of Ophthalmology, Istanbul University, Istanbul, Turkey. 5. Department of Ophthalmology, Ondokuz Mayıs University, Samsun, Turkey. 6. Department of Ophthalmology, University of Ankara, Ankara, Turkey. 7. Uveitis and Scleritis Service, Moorfields Eye Hospital, London, UK. 8. Pediatric Uveitis Service, Great Ormond St Hospital, London, UK. 9. Department of Ophthalmology and Visual Sciences, McGill University, Montreal, Canada. 10. Department of Ophthalmology, National University Health System, Singapore, Thailand. 11. Department of Ophthalmology, Mohammed V Military Hospital, Rabat, Morocco. 12. RK Eye, Aesthetic and Health Services, Kadikoy, Istanbul, Turkey. 13. Veni Vidi Eye Center, Caddebostan, Kadikoy, Istanbul, Turkey. 14. Department of Ophthalmology, Başkent University, Ankara, Turkey. 15. Department of Ophthalmology, University Vita-Salute, IRCCS Ospedale San Raffaele, Milan, Italy. 16. Department of Ophthalmology, American University of Beirut, Beirut, Lebanon. ammansourmd@gmail.com. 17. Department of Ophthalmology, Rafic Hariri University Hospital, Beirut, Lebanon. ammansourmd@gmail.com.
Abstract
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.
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.