Abstract
Behçet’s syndrome (BS) is a vasculitis involving several organ systems including the eyes. Ocular involvement is one of the most disabling complications of BS, causing loss of vision that may progress to blindness if left untreated. The typical form of ocular involvement is a relapsing and remitting panuveitis and retinal vasculitis. Initial attacks may spontaneously improve and subsequently disappear in a few weeks but tend to recur if left untreated. Destructive and recurrent attacks, especially with posterior segment and retina involvement, may cause irreversible ocular structural changes and permanent damage in sensory retina, resulting in loss of vision. The risk of irreversible damage to ocular tissue which may result in loss of vision warrants early and intensive treatment especially in patients at high risk such as young men who tend to follow an aggressive disease course. The management strategy involves rapid suppression of inflammation during the attacks and prevention of recurrent attacks. Local and systemic measures including immunosuppressives, corticosteroids, and biologic agents are used for this purpose. Surgery may be required in selected cases. The prognosis of eye involvement has greatly improved over the last decades with the effective use of immunosuppressives.
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References
Tugal-Tutkun I, Gupta V, Cunningham ET (2013) Differential diagnosis of Behçet uveitis. Ocul Immunol Inflamm 21(5):337–350
Tugal-Tutkun I, Onal S, Ozyazgan Y, Soylu M, Akman M (2013) Validity and agreement of uveitis experts in interpretation of ocular photographs for diagnosis of Behçet uveitis. Ocul Immunol Inflamm. 2013 Dec 11. [Epub ahead of print]
Yurdakul S, Yazıcı Y (2010) Epidmiology of Behçet’s syndrome and regional differences in disease expression. In: Yazıcı H, Yazıcı Y (eds) Behçet’s syndrome. Springer, New York, pp 35–52
Mishima, Masuda K, Izawa Y, Mochizuki M, Namba K (1979) Behçet’s disease in Japan: ophthalmological aspect. Trans Am Ophthalmol Soc 77:225–279
Yang P, Fang W, Meng Q, Ren Y, Xing L, Kijlstra A (2008) Clinical features of Chinese patients with Behçet’s disease. Ophthalmology 115(2):312–318.e4
Yazici H, Fresko I, Yurdakul S (2007) Behçet’s syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 3(3):148–155
Kazokoğlu H, Onal S, Tuğal-Tutkun İ et al (2008) Demographic and clinical features of uveitis in tertiary centers in Turkey. Ophthalmic Epidemiol 15:285–293
Yazıcı H, Tüzün Y, Pazarlı H et al (1984) Influence of age of onset and patient’s sex on the prevalence and severity of manifestation of Behçet’s syndrome. Ann Rheum Dis 43:783–789
Kural-Seyahi E, Fresko İ, Seyahi N et al (2003) The long-term mortality and morbidity of Behçet’s syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine 82:60–76
Tuğal-Tutkun I, Onal S, Altan-Yaycioğlu R, Hüseyin Altunbaş H, Urgancioğlu M (2004) Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol 138:373–380
Jabs DA, Nussenblatt RB, Rosenbaum JT (2005) Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol 140:509–516
Pazarlı H, Ozyazgan Y, Aktunc T (1989) Clinical observations on hypopyon attacks of Behçet’s disease in Turkey. In: Seventh international conference on Behçet’s disease (abstracts), Rochester. MN. 14–15 Sept
Nussenblatt RB, Whitcup SM, Palestine AG (2004) Behçet’s disease. In: Nussenblatt RB, Whitcup SM, Palestine AG (eds) Uveitis: fundamentals and clinical practice. Mosby, Philadelphia, pp 350–371
Ehrlich GE (1997) Vasculitis in Behçet’s disease. Int Rev Immunol 14:81–88
Mullaney J, Collum LM (1985) Ocular vasculitis in Behçet’s disease: a pathological and immunohistochemical study. Int Ophthalmol 7:183–191
Mamo JG (1970) The rate of visual loss in Behçet’s disease. Arc Ophthalmol 84:451–452
Cingu AK, Onal S, Urgancioglu M, Tugal-Tutkun I (2012) Comparison of presenting features and three-year disease course in Turkish patients with Behçet uveitis who presented in the early 1990s and the early 2000s. Ocul Immunol Inflamm 20(6):423–428
Markomichelakis N, Delicha E, Masselos S, Sfikakis PP (2012) Intravitreal infliximab for sight-threatening relapsing uveitis in Behçet disease: a pilot study in 15 patients. Am J Ophthalmol 154(3):534–541
Yazıcı H, Pazarlı H, Barnes CG et al (1990) A controlled trail of azathioprine in Behçet’s syndrome. N England J Med 322:281–285
Hamuryudan V, Ozyazgan Y, Hızlı N et al (1997) Azathioprine in Behçet’s syndrome: effects on long-term prognosis. Arthritis Rheum 40:769–774
Ozyazgan Y, Yurdakul S, Yazici H, Tuzun B, Işcimen A, Tuzun Y et al (1992) Low dose cyclosporin A versus pulsed cyclophosphamide in Behçet’s syndrome: a single masked trial. Br J Ophthalmol 76:241–243
Masuda K, Nakajima A, Urayama A, Nakae K et al (1989) Double-masked trial of cyclosporin versus colchicines and long-term open study of cyclosporin in Behçet’s disease. Lancet 1:1093–1096
BenEzra D, Cohen E, Chajek T et al (1988) Evaluation of conventional therapy versus cyclosporin A in Behçet’s syndrome. Transplant Proc 20(3 Suppl 4):136–143
Durand JM, Soubeyrand J (1994) Interferon-alpha 2b for refractory ocular Behçet’s disease. Lancet 344:333
Kotter I, Eckstein AK, Stübiger N et al (1998) Treatment of ocular symptoms of Behçet’s disease with interferon alpha 2a: a pilot study. Br J Ophthalmol 82:488–494
Kotter I, Zıerhut M, Eckstein AK, VontheinR NT, Gunaydın I et al (2003) Human recombinant interferon alfa-2a for the treatment of Behçet’s disease with sight threatening posterior or panuveitis. Br J Ophthalmol 87:423–431
Kotter I, Deuter C, Stubiger N, Zierhut M (2005) Interferon-a (IFN-a) application versus tumor necrosis factor-a antagonism for ocular Behçet’s disease: focusing more on IFN. J Rheumatol 32:1633
Tugal –Tutkun I, Güney–Tefekli E, Urgancioğlu M (2006) Results of interferon-alpha therapy in patients with Behçet’s uveitis. Graefes Arch Clin Exp Ophthalmol 244:1692–1695
Bodaghi B, Gendron G, Wechsler B (2007) Efficacy of interferon alpha in the treatment of refractory and sight threatening uveitis: a retrospective monocentric study of 45 patients. Br J Ophthalmol 91:335–339
Onal S, Kazokoglu H, Koc A et al (2011) Long-term efficacy and safety of low-dose and dose-escalating interferon alfa-2a therapy in refractory Behçet uveitis. Arch Ophthalmol 129:288–294
Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P et al (2001) Effect of infliximab on sight-threatening panuveitis in Behçet’s disease. Lancet 358:295–296
Ohno S, Nakamura S, Hori S et al (2004) Efficacy, safety and pharmacokinetics of multiple administration of infliximab in Behçet’s disease with refractory uveoretinitis. J Rheumatol 31:1362–1368
Tugal-Tutkun I, Mudun A, Urgancıoğlu M et al (2005) Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroid in Behçet’s disease: an open-label trial. Arthritis Rheum 52:2478–2484
Bodaghi B, Bui Quoc E, Wechsler B et al (2005) Therapeutic use of infliximab in sight threatening uveitis: retrospective analysis of efficacy, safety, and limiting factors. Ann Rheum Dis 64:962–964
Sfikakis PP, Markomichelakis N, Alpsoy E et al (2007) Anti TNF therapy in the management of Behçet’s disease—review and basis for recommendations. Rheumatology (Oxford) 46:736–741
Mushtaq B, Saeed T, Situnayake RD, Murray PI (2007) Adalimumab for sight-threatening uveitis in Behçet’s disease. Eye 21:824–825
Markomichelakis N, Delicha E, Masselos S, Fragiadaki K, Kaklamanis P, Sfikakis PP (2011) A single infliximab infusion vs corticosteroids for acute panuveitis attacks in Behçet’s disease: a comparative 4-week study. Rheumatology (Oxford) 50(3):593–597
Okada AA, Goto H, Ohno S, Mochizuki M (2012) Multicenter study of infliximab for refractory uveoretinitis in Behçet disease. Arch Ophthalmol 130(5):592–598
Kawaguchi T, Sugita S, Yamada Y, Miyanaga M, Mochizuki M (2010) Regression of optic disc neovascularization in patients with Behçet’s uveoretinitis after infliximab therapy. J Ocul Pharmacol Ther 26(6):627–630
Keino H, Okada AA, Watanabe T, Taki W (2011) Decreased ocular inflammatory attacks and background retinal and disc vascular leakage in patients with Behcet’s disease on infliximab therapy. Br J Ophthalmol 95(9):1245–1250
Mushtaq B, Saeed T, Situnayake RD, Murray PI (2007) Adalimumab for sight-threatening uveitis in Behçet’s disease. Eye (Lond) 21(6):824–825
Bawazeer A, Raffa LH, Nizamuddin SH (2010) Clinical experience with adalimumab in the treatment of ocular Behçet disease. Ocul Immunol Inflamm 18(3):226–232
Gül A, Tugal-Tutkun I, Dinarello CA, Reznikov L, Esen BA, Mirza A, Scannon P, Solinger A (2012) Interleukin-1β-regulating antibody XOMA 052 (gevokizumab) in the treatment of acute exacerbations of resistant uveitis of Behcet’s disease: an open-label pilot study. Ann Rheum Dis 71(4):563–566
Emmi G, Silvestri E, Cameli AM, Bacherini D, Vannozzi L, Squatrito D, Emmi L (2013) Anakinra for resistant Behçet uveitis: why not? Clin Exp Rheumatol 31(3 Suppl 77):152–153
Ugurlu S, Ucar D, Seyahi E, Hatemi G, Yurdakul S (2012) Canakinumab in a patient with juvenile Behcet’s syndrome with refractory eye disease. Ann Rheum Dis 71(9):1589–1591
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The authors thank Hasan Yazıcı for critical reading of the manuscript.
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Ozyazgan, Y., Ucar, D., Hatemi, G. et al. Ocular Involvement of Behçet’s Syndrome: a Comprehensive Review. Clinic Rev Allerg Immunol 49, 298–306 (2015). https://doi.org/10.1007/s12016-014-8425-z
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DOI: https://doi.org/10.1007/s12016-014-8425-z