Skip to main content

Advertisement

Log in

Ocular Involvement of Behçet’s Syndrome: a Comprehensive Review

  • Published:
Clinical Reviews in Allergy & Immunology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8

Similar content being viewed by others

References

  1. Tugal-Tutkun I, Gupta V, Cunningham ET (2013) Differential diagnosis of Behçet uveitis. Ocul Immunol Inflamm 21(5):337–350

    Article  PubMed  Google Scholar 

  2. 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]

  3. 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

  4. Mishima, Masuda K, Izawa Y, Mochizuki M, Namba K (1979) Behçet’s disease in Japan: ophthalmological aspect. Trans Am Ophthalmol Soc 77:225–279

    PubMed Central  CAS  PubMed  Google Scholar 

  5. 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

    Article  PubMed  Google Scholar 

  6. 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

    Article  PubMed  Google Scholar 

  7. 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

    Article  PubMed  Google Scholar 

  8. 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

    Article  PubMed Central  PubMed  Google Scholar 

  9. 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

    Article  PubMed  Google Scholar 

  10. 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

    Article  PubMed  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. 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

  13. 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

    Google Scholar 

  14. Ehrlich GE (1997) Vasculitis in Behçet’s disease. Int Rev Immunol 14:81–88

    Article  CAS  PubMed  Google Scholar 

  15. Mullaney J, Collum LM (1985) Ocular vasculitis in Behçet’s disease: a pathological and immunohistochemical study. Int Ophthalmol 7:183–191

    Article  CAS  PubMed  Google Scholar 

  16. Mamo JG (1970) The rate of visual loss in Behçet’s disease. Arc Ophthalmol 84:451–452

    Article  CAS  Google Scholar 

  17. 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

    Article  PubMed  Google Scholar 

  18. 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

    Article  CAS  PubMed  Google Scholar 

  19. 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

    Article  Google Scholar 

  20. Hamuryudan V, Ozyazgan Y, Hızlı N et al (1997) Azathioprine in Behçet’s syndrome: effects on long-term prognosis. Arthritis Rheum 40:769–774

    Article  CAS  PubMed  Google Scholar 

  21. 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

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  22. 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

    Article  CAS  PubMed  Google Scholar 

  23. 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

    CAS  PubMed  Google Scholar 

  24. Durand JM, Soubeyrand J (1994) Interferon-alpha 2b for refractory ocular Behçet’s disease. Lancet 344:333

    Article  CAS  PubMed  Google Scholar 

  25. 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

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  26. 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

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  27. 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

    PubMed  Google Scholar 

  28. 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

    Article  PubMed  Google Scholar 

  29. 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

    Article  PubMed Central  PubMed  Google Scholar 

  30. 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

    Article  CAS  PubMed  Google Scholar 

  31. 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

    Article  CAS  PubMed  Google Scholar 

  32. 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

    CAS  PubMed  Google Scholar 

  33. 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

    Article  CAS  PubMed  Google Scholar 

  34. 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

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  35. 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

    Article  CAS  Google Scholar 

  36. Mushtaq B, Saeed T, Situnayake RD, Murray PI (2007) Adalimumab for sight-threatening uveitis in Behçet’s disease. Eye 21:824–825

    Article  CAS  PubMed  Google Scholar 

  37. 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

    Article  CAS  Google Scholar 

  38. 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

    Article  CAS  PubMed  Google Scholar 

  39. 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

    Article  CAS  PubMed  Google Scholar 

  40. 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

    Article  PubMed  Google Scholar 

  41. Mushtaq B, Saeed T, Situnayake RD, Murray PI (2007) Adalimumab for sight-threatening uveitis in Behçet’s disease. Eye (Lond) 21(6):824–825

    Article  CAS  Google Scholar 

  42. 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

    Article  CAS  PubMed  Google Scholar 

  43. 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

    Article  PubMed  Google Scholar 

  44. 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

    PubMed  Google Scholar 

  45. 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

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors thank Hasan Yazıcı for critical reading of the manuscript.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Yusuf Yazici.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

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

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12016-014-8425-z

Keywords

Navigation