Abstract
Background
To evaluate the long-time progression of retinal vasculitis in Behçet patients using the fluorescein angiography (FA) scoring system.
Methods
Retrospective study was conducted for 71 eyes of 43 patients who met the study criteria. All patients completed at least 2 years of follow-up. FA was taken during the periods of active retinal vasculitis and the quiescent phase, and analyzed using a FA scoring system. Among nine categories, the four most prevalent FA signs were evaluated: optic disc hyperfluorescence (score 0–3), macular edema (score 0–4), retinal vascular staining and/or leakage (score 0–7), and capillary leakage (score 0–10).
Results
Mean number of total active inflammations was 2.6 ± 1.5 times during mean 55.0 ± 20.0 months. Mean scores at the first active inflammation were 1.8 ± 1.0 for optic disc hyperfluorescence, 2.4 ± 1.0 for macular edema, 5.3 ± 2.1 for retinal vascular staining and/or leakage, and 5.8 ± 3.2 for capillary leakage. Mean total FA score was 17.4 ± 6.8. Mean scores at the first quiescent phase were 0.6 ± 0.4 for optic disc hyperfluorescence, 1.1 ± 1.2 for macular edema, 3.8 ± 1.9 for retinal vascular staining and/or leakage, and 3.5 ± 3.5 for capillary leakage. Mean total FA score was 9.1 ± 5.0. Mean scores for each active inflammation and quiescent phase were not significantly changed, and mean FA scores were significantly reduced in quiescent phase (P = 0.003 for optic disc hyperfluorescence, P = 0.005 for macular leakage, P = 0.010 for retinal vascular staining and/or leakage, P = 0.008 for capillary leakage, and P = 0.018 for total FA score; paired t-test).
Conclusions
Retinal vasculitis of Behçet patients did not significantly progress during long-term follow-up.
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References
Deuter CM, Kötter I, Wallace GR, Murray PI, Stübiger N, Zierhut M (2008) Behçet’s disease: ocular effects and treatment. Prog Retin Eye Res 27:111–136
George RK, Chan C, Whitcup SM, Nussenblatt RB (1997) Ocular immunopathology of Behçet’s disease. Surv Ophthalmol 42:157–162
Chajek T, Fainaru M (1975) Behçet’s disease. Report of 41 cases and a review of the literature. Medicine 54:179–196
Kontogianis V, Powell RJ (2000) Behçet’s disease. Postgrad Med J 76:629–637
Wong RC, Ellis CN, Diaz LA (1984) Behçet’s disease. Int J Dermatol 23:25–32
Llopis MD, Cervera M, Menezo JL (1990) Cyclosporin A treatment of Behçet’s disease: a long term study. Curr Eye Res 9:17–23
Evereklioglu C (2005) Current concepts in the etiology and treatment of Behçet’s disease. Surv Ophthalmol 50:297–350
Atmaca LS (1989) Fundus changes associated with Behçet’s disease. Graefes’s Arch Clin Exp Ophthalmol 227:340–344
Atmaca LS, Batioglu F (1994) The efficacy of cyclosporine A in the treatment of Behçet’s disease. Ophthalmic Surg 25:321–327
Ozdal PC, Ortaç S, Taşkintuna I, Taşkintuna I, Firat E (2001) Posterior segment involvement in ocular Behçet’s disease. Eur J Ophthalmol 12:424–431
Altenburg A, Papoustis N, Orawa H, Martus P, Krause L, Zouboulis CC (2006) Epidemiology and clinical manifestations of Adamantiades–Behçet’s disease in Germany—current pathogenetic concepts and therapeutic possibilities. J Dtsch Dermatol Ges 4:49–64
Yazici H, Tuzun Y, Pazarli H, Yurdakul S, Ozyazgan Y, Ozdoğan H, Serdaroğlu S, Ersanli M, Ulkü BY, Müftüoğlu AU (1984) Influence of age of onset and patient’s sex on prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis 43:783–789
Yazici H, Barnes CG (1991) Practical treatment recommendations for pharmacotherapy of Behçet’s syndrome. Drugs 42:796–804
Ben-Ezra D, Cohen E (1986) Treatment and visual prognosis in Behçet’s disease. Br J Ophthalmol 70:589–592
Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, Huseyin Altunbas H, Urgancioglu M (2004) Uveitis in Behçet’s disease: an analysis of 880 patients. Am J Ophthalmol 138:373–380
Atmaca LS, Sonmez PA (2003) Fluorescein and indocyanine green angiography findings in Behçet’s disease. Br J Ophthalmol 87:1466–1468
Gedik S, Akova Y, Yilmaz G, Bozbeyoğlu S (2005) Indocyanine green and fundus fluorescein angiographic findings in patients with active ocular Behçet’s disease. Ocular Immunol Inflamm 13:51–58
Yu HG, Kim MJ, Oh FS (2009) Fluorescein angiography and visual acuity in active uveitis with Behçet’s disease. Ocular Immunol Inflamm 17:41–46
Tugal-Tutkun I, Herbort CP, Khairallah M, The Angiography Scoring for Uveitis Working Group (ASUWOG) (2010) Scoring of dual fluorescein and ICG inflammatory angiographic signs for the grading of posterior segment inflammation (dual fluorescein and ICG angiographic scoring system for uveitis). Int Ophthalmol 30:539–553
The International Study Group for Behçet’s disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 1335:1078–1080
Jabs DA, Nussenblatt RB, Rosenbaum JT, The Standardization of Uveitis Nomenclature (SUN) Working Group (2005) Standardization of uveitis nomenclature for reporting clinical data. Results of the first international workshop. Am J Ophthalmol 140:509–516
Spitznas M (2006) Understanding fluorescein angiography. Springer, Berlin, pp 73–99
Nussenblatt R (2010) Uveitis: fundamentals and clinical practice, 4th edn. Elsevier, Philadelphia, pp 42–71
Verity DH, Vaughan RW, Madanat W, Kondeatis E, Zureikat H, Fayyad F, Kanawati CA, Ayesh I, Stanford MR, Wallace GR (1999) Factor V Leiden mutation is associated with ocular involvement in Behçet’s disease. Am J Ophthalmol 128:352–356
Takeuchi M, Hokama H, Tsukahara R, Kezuka T, Goto H, Sakai J, Usui M (2005) Risk and prognostic factors of poor visual outcome in Behçet’s disease with ocular involvement. Graefes Arch Clin Exp Ophthalmol 243:1147–1152
Gueudry J, Wechsler B, Terrada C, Gendron G, Cassoux N, Fardeau C, Lehoang P, Piette JC, Bodaghi B (2008) Long-term efficacy and safety of low-dose interferon alpha2a therapy in severe uveitis associated with Behçet disease. Am J Ophthalmol 146:837–844
Deuter CM, Zierhut M, Möhle A, Vonthein R, Stöbiger N, Kötter I (2010) Long-term remission after cessation of interferon-α treatment in patients with severe uveitis due to Behçet’s disease. Arthritis Rheum 62:2796–2805
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Design and conduct of study (HM Kang, SC Lee); collection of data (HM Kang); management, analysis, and interpretation of data (HM Kang and CS Lee); and preparation, review, and approval of the manuscript (HM Kang and SC Lee)
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The abstract of this article was presented as a poster in the 110th annual congress of the Korean Ophthalmological Society in KINTEX, Seoul, Republic of Korea, November, 2013
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Kang, H.M., Lee, S.C. Long-term progression of retinal vasculitis in Behçet patients using a fluorescein angiography scoring system. Graefes Arch Clin Exp Ophthalmol 252, 1001–1008 (2014). https://doi.org/10.1007/s00417-014-2637-z
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DOI: https://doi.org/10.1007/s00417-014-2637-z