Behçet Disease

  • Sumru Onal
  • Ilknur Tugal-Tutkun


Behçet disease is a multisystem disorder characterized by relapsing inflammation of unknown origin [1, 2]. The disease has first been described as a distinct clinical entity by a Turkish dermatologist, Prof. Hulusi Behçet, as a triple symptom complex of oral and genital ulcers and hypopyon iritis in 1937 [3]. The disease is now accepted as an obliterative and necrotizing systemic vasculitis involving different organ systems and leading to a wide range of clinical manifestations. The usual course of the disease is characterized by recurrent inflammatory episodes. The eye is the most commonly involved vital organ [1]. Uveitis associated with Behçet disease, which will be referred to as Behçet uveitis throughout this chapter, represents one of the most difficult forms of uveitis to treat. Despite the use of robust medical therapy with corticosteroids and conventional immunomodulatory and biologic agents, some patients with Behçet uveitis still end up with poor vision, and Behçet uveitis remains as a potentially blinding disease.


Retinal Nerve Fiber Layer Macular Hole Cystoid Macular Edema Anterior Uveitis Ocular Involvement 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Evereklioglu C. Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol. 2005;50:297–350.PubMedGoogle Scholar
  2. 2.
    Yazici H, Fresko I, Yurdakul S. Behçet’s syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol. 2007;3(3):148–55.PubMedGoogle Scholar
  3. 3.
    Behçet H. Über rezidivierende aphthöse durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatol Wochenschr. 1937;105:1152–7.Google Scholar
  4. 4.
    Verity DH, Marr JE, Ohno S, et al. Behçet’s disease, the Silk Road and HLA-B51: historical and geographical perspectives. Tissue Antigens. 1999;54(3):213–20.PubMedGoogle Scholar
  5. 5.
    Keino H, Okada AA. Behçet’s disease: global epidemiology of an old Silk Road disease. Br J Ophthalmol. 2007;91(12):1573–4.PubMedCentralPubMedGoogle Scholar
  6. 6.
    Azizlerli G, Kose AA, Sarica R, et al. Prevalence of Behçet’s disease in Istanbul, Turkey. Int J Dermatol. 2003;42:803–6.PubMedGoogle Scholar
  7. 7.
    Cakir N, Dervis E, Benian O, et al. Prevalence of Behçet’s disease in rural western Turkey: a preliminary report. Clin Exp Rheumatol. 2004;22 Suppl 34:S53–5.PubMedGoogle Scholar
  8. 8.
    Tüzün Y, Yurdakul S, Mat MC, et al. Epidemiology of Behçet’s syndrome in Turkey. Int J Dermatol. 1996;35:618–20.PubMedGoogle Scholar
  9. 9.
    Idil A, Gurler A, Boyvat A, et al. The prevalence of Behçet’s disease above the age of 10 years. The results of a pilot study conducted at the Park Primary Health Care Center in Ankara, Turkey. Ophthalmic Epidemiol. 2002;9:325–31.PubMedGoogle Scholar
  10. 10.
    Yurdakul S, Günaydin I, Tüzün Y, et al. The prevalence of Behçet’s syndrome in a rural area in Northern Turkey. J Rheumatol. 1988;15:820–2.PubMedGoogle Scholar
  11. 11.
    Khairallah M, Accorinti M, Muccioli C, Kahloun R, Kempen JH. Epidemiology of Behçet disease. Ocul Immunol Inflamm. 2012;20:324–35.PubMedGoogle Scholar
  12. 12.
    Zouboulis CC. Epidemiology of Adamantiades-Behçet’s disease. In: Zierhut M, Ohno S, editors. Immunology of Behçet’s disease. Lisse: Swets & Zeitlinger Publ; 2003. p. 1–16.Google Scholar
  13. 13.
    Calamia KT, Wilson FC, Icen M, et al. Epidemiology and clinical characteristics of Behçet’s disease in the US: a population-based study. Arthritis Rheum. 2009;61:600–4.PubMedCentralPubMedGoogle Scholar
  14. 14.
    Kitaichi N, Ohno S. Behçet disease in children. Int Ophthalmol Clin. 2008;48:87–91.PubMedGoogle Scholar
  15. 15.
    Saricaoglu H, Karadogan SK, Bayazit N, et al. Clinical features of late-onset Behçet’s disease: report of nine cases. Int J Dermatol. 2006;45:1284–7.PubMedGoogle Scholar
  16. 16.
    Tugal-Tutkun I. Behçet disease in the developing world. Int Ophthalmol Clin. 2010;50:87–98.PubMedGoogle Scholar
  17. 17.
    Tugal-Tutkun I, Urgancıoglu M. Childhood-onset uveitis in Behçet disease: a descriptive study of 36 cases. Am J Ophthalmol. 2003;136:1114–9.PubMedGoogle Scholar
  18. 18.
    Kone-Paut I, Gorchakoff-Molinas A, Weschler B, et al. Paediatric Behçet’s disease in France. Ann Rheum Dis. 2002;61:655–6.PubMedCentralPubMedGoogle Scholar
  19. 19.
    Okada AA. Behçet’s disease: general concepts and recent advances. Curr Opin Ophthalmol. 2006;17:551–6.PubMedGoogle Scholar
  20. 20.
    Tugal-Tutkun I. Behçet’s uveitis. Middle East Afr J Ophthalmol. 2009;16:219–24.PubMedCentralPubMedGoogle Scholar
  21. 21.
    Alpsoy E, Donmez L, Onder M, et al. Clinical features and natural course of Behçet’s disease in 661 cases: a multicenter study. Br J Dermatol. 2007;157:901–6.PubMedGoogle Scholar
  22. 22.
    Deuter CM, Kötter I, Wallace GR, et al. Behçet’s disease: ocular effects and treatment. Prog Retin Eye Res. 2008;27:111–36.PubMedGoogle Scholar
  23. 23.
    Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003;82:60–76.Google Scholar
  24. 24.
    Marshall SE. Behçet’s disease. Best Pract Res Clin Rheumatol. 2004;18:291–311.PubMedGoogle Scholar
  25. 25.
    The International Study Group for Behçet’s Disease. Evaluation of diagnostic (‘classification’) criteria in Behçet’s disease – towards internationally agreed criteria. Br J Rheumatol. 1992;31:299–308.Google Scholar
  26. 26.
    Chang HK, Kim SY. Survey and validation of the criteria for Behcet’s disease recently used in Korea: a suggestion for modification of the International Study Group criteria. J Korean Med Sci. 2003;18:88–92.PubMedCentralPubMedGoogle Scholar
  27. 27.
    Gül A. Behçet’s disease as an autoinflammatory disorder. Curr Drug Targets Inflamm Allergy. 2005;4:81–3.PubMedGoogle Scholar
  28. 28.
    Gul A, Ohno S. Genetics of Behçet’s disease. In: Yazici Y, Yazici H, editors. Behçet’s syndrome. New York: Springer; 2010. p. 265–76.Google Scholar
  29. 29.
    Remmers EF, Cosan F, Kirino Y, et al. Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet’s disease. Nat Genet. 2010;42:698–702.PubMedCentralPubMedGoogle Scholar
  30. 30.
    Kirino Y, Zhou Q, Ishigatsubo Y, et al. Targeted resequencing implicates the familial Mediterranean fever gene MEFV and the toll-like receptor 4 gene TLR4 in Behçet disease. Proc Natl Acad Sci U S A. 2013;110:8134–9.PubMedCentralPubMedGoogle Scholar
  31. 31.
    Nakae K, Masaki F, Inaba G, et al. Recent epidemiologic features of Behçet’s disease in Japan. In: Wechsler B, Godeau P, editors. Behçet’s disease. Amsterdam: Elsevier Science; 1993. p. 145–51.Google Scholar
  32. 32.
    Yoshida A, Kawashima H, Motoyama Y, et al. Comparison of patients with Behçet’s disease in the 1980s and 1990s. Ophthalmology. 2004;111:810–5.PubMedGoogle Scholar
  33. 33.
    Akiyama K, Numaga J, Yoshida A, et al. Statistical analysis of endogenous uveitis at Tokyo University Hospital. Jpn J Ophthalmol. 2006;50:69–71.PubMedGoogle Scholar
  34. 34.
    Wakabayashi T, Morimura Y, Miyamoto Y, et al. Changing patterns of intraocular inflammatory disease in Japan. Ocul Immunol Inflamm. 2003;11:277–86.PubMedGoogle Scholar
  35. 35.
    Kitamei H, Kitaichi N, Namba K, et al. Clinical features of intraocular inflammation in Hokkaido, Japan. Acta Ophthalmol. 2009;87:424–8.PubMedGoogle Scholar
  36. 36.
    Kotake S, Furudate N, Sasamoto Y, et al. Characteristics of endogenous uveitis in Hokkaido, Japan. Graefes Arch Clin Exp Ophthalmol. 1997;235:5–9.PubMedGoogle Scholar
  37. 37.
    Ando K, Fujino Y, Hijikata K, et al. Epidemiological features and visual prognosis of Behçet’s disease. Jpn J Ophthalmol. 1999;43:312–7.PubMedGoogle Scholar
  38. 38.
    Kazokoglu H, Onal S, Tugal-Tutkun I, et al. Demographic and clinical features of uveitis in tertiary centers in Turkey. Ophthalmic Epidemiol. 2008;15:285–93.PubMedGoogle Scholar
  39. 39.
    Zamir E, Bodaghi B, Tugal-Tutkun I, et al. Conjunctival ulcers in Behçet’s disease. Ophthalmology. 2003;110:1137–41.PubMedGoogle Scholar
  40. 40.
    Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, et al. Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol. 2004;138:373–80.PubMedGoogle Scholar
  41. 41.
    Kitaichi N, Miyazaki A, Iwata D, et al. Ocular features of Behcet’s disease: an international collaborative study. Br J Ophthalmol. 2007;91:1579–82.PubMedCentralPubMedGoogle Scholar
  42. 42.
    Yang P, Fang W, Meng Q, et al. Clinical features of Chinese patients with Behçet’s disease. Ophthalmology. 2008;115:312–18.e4.PubMedGoogle Scholar
  43. 43.
    Khairallah M, Attia S, Yahia SB, et al. Pattern of uveitis in Behçet’s disease in a referral center in Tunisia, North Africa. Int Ophthalmol. 2009;29:135–41.PubMedGoogle Scholar
  44. 44.
    Zaidi AA, Ying GS, Daniel E, et al. Hypopyon in patients with uveitis. Ophthalmology. 2010;117:366–72.PubMedCentralPubMedGoogle Scholar
  45. 45.
    Sachdev N, Kapali N, Singh R, et al. Spectrum of Behçet’s disease in the Indian population. Int Ophthalmol. 2009;29:495–501.PubMedGoogle Scholar
  46. 46.
    Saleh OA, Birnbaum AD, Tessler HH, et al. Behçet uveitis in the American midwest. Ocul Immunol Inflamm. 2012;20:12–7.PubMedGoogle Scholar
  47. 47.
    Barra C, Belfort Júnior R, Abreu MT, et al. Behçet’s disease in Brazil--a review of 49 cases with emphasis on ophthalmic manifestations. Jpn J Ophthalmol. 1991;35:339–46.PubMedGoogle Scholar
  48. 48.
    Tugal-Tutkun I, Cingü K, Kir N, et al. Use of laser flare-cell photometry to quantify intraocular inflammation in patients with Behçet uveitis. Graefes Arch Clin Exp Ophthalmol. 2008;246:1169–77.PubMedGoogle Scholar
  49. 49.
    Tugal-Tutkun I. Imaging in the diagnosis and management of Behçet disease. Int Ophthalmol Clin. 2012;52:183–90.PubMedGoogle Scholar
  50. 50.
    Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, et al. Neovascularization of the optic disc in Behçet’s disease. Jpn J Ophthalmol. 2006;50:256–65.PubMedGoogle Scholar
  51. 51.
    Tugal-Tutkun I, Gupta V, Cunningham ET. Differential diagnosis of Behçet uveitis. Ocul Immunol Inflamm. 2013;21(5):337–50.PubMedGoogle Scholar
  52. 52.
    Atmaca LS. Fundus changes associated with Behçet’s disease. Graefes Arch Clin Exp Ophthalmol. 1989;227:340–4.PubMedGoogle Scholar
  53. 53.
    Onal S, Tugal-Tutkun I, Neri P, et al. Optical coherence tomography imaging in uveitis. Int Ophthalmol. 2013. doi: 10.1007/s10792-013-9822-7.PubMedGoogle Scholar
  54. 54.
    Toker E, Kazokoğlu H, Acar N. High dose intravenous steroid therapy for severe posterior segment uveitis in Behçet’s disease. Br J Ophthalmol. 2002;86:521–3.PubMedCentralPubMedGoogle Scholar
  55. 55.
    Yalçindag FN, Can E, Ozdemir O. Intravenous methylprednisolone pulse therapy for acute posterior segment uveitis attacks in Behçet’s disease. Ann Ophthalmol (Skokie). 2007;39:194–7.Google Scholar
  56. 56.
    Karacorlu M, Mudun B, Ozdemir H. Intravitreal triamcinolone acetonide for the treatment of cystoid macular edema secondary to Behçet disease. Am J Ophthalmol. 2004;138:289–91.PubMedGoogle Scholar
  57. 57.
    Tuncer S, Yilmaz S, Urgancioglu M, et al. Results of intravitreal triamcinolone acetonide (IVTA) injection for the treatment of panuveitis attacks in patients with Behçet disease. J Ocul Pharmacol Ther. 2007;23:395–401.PubMedGoogle Scholar
  58. 58.
    Markomichelakis N, Delicha E, Masselos S, et al. Intravitreal infliximab for sight-threatening relapsing uveitis in Behçet disease: a pilot study in 15 patients. Am J Ophthalmol. 2012;154:534–41.e1.PubMedGoogle Scholar
  59. 59.
    Sfikakis PP, Theodossiadis PG, Katsiari CG, et al. Effect of infliximab on sight-threatening panuveitis in Behçet’s disease. Lancet. 2001;358:295–6.PubMedGoogle Scholar
  60. 60.
    Sfikakis PP, Kaklamanis PH, Elezoglou A, et al. Infliximab for recurrent, sight-threatening ocular inflammation in Adamantiades-Behçet disease. Ann Intern Med. 2004;140:404–6.PubMedGoogle Scholar
  61. 61.
    Sfikakis PP, Markomichelakis N, Alpsoy E, et al. Anti-TNF therapy in the management of Behcet’s disease--review and basis for recommendations. Rheumatology (Oxford). 2007;46:736–41.Google Scholar
  62. 62.
    Markomichelakis N, Delicha E, Masselos S, et al. A single infliximab infusion vs corticosteroids for acute panuveitis attacks in Behçet’s disease: a comparative 4-week study. Rheumatology (Oxford). 2011;50:593–7.Google Scholar
  63. 63.
    Jabs DA, Rosenbaum JT, Foster CS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol. 2000;130:492–513.PubMedGoogle Scholar
  64. 64.
    Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008;67:1656–62.PubMedGoogle Scholar
  65. 65.
    Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med. 1990;322:281–5.PubMedGoogle Scholar
  66. 66.
    Masuda K, Urayama A, Kogure M, et al. Double-masked trial of cyclosporin versus colchicine and long-term open study of cyclosporin in Behçet’s disease. Lancet. 1989;1:1093–6.PubMedGoogle Scholar
  67. 67.
    Ozyazgan Y, Yurdakul S, Yazici H, et al. Low dose cyclosporin A versus pulsed cyclophosphamide in Behçet’s syndrome: a single masked trial. Br J Ophthalmol. 1992;76:241–3.PubMedCentralPubMedGoogle Scholar
  68. 68.
    Akman-Demir G, Ayranci O, Kurtuncu M, et al. Cyclosporine for Behçet’s uveitis: is it associated with an increased risk of neurological involvement? Clin Exp Rheumatol. 2008;26 Suppl 50:S84–90.PubMedGoogle Scholar
  69. 69.
    Teoh SC, Hogan AC, Dick AD, et al. Mycophenolate mofetil for the treatment of uveitis. Am J Ophthalmol. 2008;146:752–60.PubMedGoogle Scholar
  70. 70.
    Sobrin L, Christen W, Foster CS. Mycophenolate mofetil after methotrexate failure or intolerance in the treatment of scleritis and uveitis. Ophthalmology. 2008;115:1416–21.PubMedGoogle Scholar
  71. 71.
    Tanabe K. Calcineurin inhibitors in renal transplantation: what is the best option? Drugs. 2003;63:1535–48.PubMedGoogle Scholar
  72. 72.
    Mochizuki M, Masuda K, Sakane T, et al. A multicenter clinical open trial of FK 506 in refractory uveitis, including Behçet’s disease: Japanese FK 506 Study Group on Refractory Uveitis. Transplant Proc. 1991;23:3343–6.PubMedGoogle Scholar
  73. 73.
    Yurdakul S, Mat C, Tüzün Y, et al. A double-blind trial of colchicine in Behçet’s syndrome. Arthritis Rheum. 2001;44:2686–92.PubMedGoogle Scholar
  74. 74.
    Tessler HH, Jennings T. High-dose short-term chlorambucil for intractable sympathetic ophthalmia and Behçet’s disease. Br J Ophthalmol. 1990;74:353–7.PubMedCentralPubMedGoogle Scholar
  75. 75.
    Mudun BA, Ergen A, Ipcioglu SU, et al. Short-term chlorambucil for refractory uveitis in Behçet’s disease. Ocul Immunol Inflamm. 2001;9:219–29.PubMedGoogle Scholar
  76. 76.
    Kötter I, Zierhut M, Eckstein AK, et al. Human recombinant interferon alfa-2a for the treatment of Behçet’s disease with sight threatening posterior or panuveitis. Br J Ophthalmol. 2003;87:423–31.PubMedCentralPubMedGoogle Scholar
  77. 77.
    Kötter I, Günaydin I, Zierhut M, et al. The use of interferon alpha in Behçet disease: review of the literature. Semin Arthritis Rheum. 2004;33:320–35.PubMedGoogle Scholar
  78. 78.
    Wechsler B, Bodaghi B, Huong DL, et al. Efficacy of interferon alfa-2a in severe and refractory uveitis associated with Behçet’s disease. Ocul Immunol Inflamm. 2000;8:293–301.PubMedGoogle Scholar
  79. 79.
    Hamuryudan V, Ozyazgan Y, Fresko Y, et al. Interferon alfa combined with azathioprine for the uveitis of Behçet’s disease: an open study. Isr Med Assoc J. 2002;4(Suppl):928–30.PubMedGoogle Scholar
  80. 80.
    Tugal-Tutkun I, Güney-Tefekli E, Urgancioglu M. Results of interferon-alfa therapy in patients with Behçet uveitis. Graefes Arch Clin Exp Ophthalmol. 2006;244:1692–5.PubMedGoogle Scholar
  81. 81.
    Krause L, Altenburg A, Pleyer U, et al. Longterm visual prognosis of patients with ocular Adamantiades-Behçet’s disease treated with interferon-alpha-2a. J Rheumatol. 2008;35:896–903.PubMedGoogle Scholar
  82. 82.
    Gueudry J, Wechsler B, Terrada C. Long-term efficacy and safety of low-dose interferon alpha2a therapy in severe uveitis associated with Behçet disease. Am J Ophthalmol. 2008;146:837.e1–44.e1.Google Scholar
  83. 83.
    Sobaci G, Erdem U, Durukan AH, et al. Safety and effectiveness of interferon alpha-2a in treatment of patients with Behçet’s uveitis refractory to conventional treatments. Ophthalmology. 2010;117:1430–5.PubMedGoogle Scholar
  84. 84.
    Onal S, Kazokoglu H, Koc A, et al. Long-term efficacy and safety of low-dose and dose-escalating interferon alfa-2a therapy in refractory Behçet uveitis. Arch Ophthalmol. 2011;129:288–94.PubMedGoogle Scholar
  85. 85.
    Yalçindağ FN, Uzun A. Results of interferon alpha-2a therapy in patients with Behcet’s disease. J Ocul Pharmacol Ther. 2012;28:439–43.PubMedGoogle Scholar
  86. 86.
    Deuter CM, Zierhut M, Möhle A, et al. Long-term remission after cessation of interferon-α treatment in patients with severe uveitis due to Behçet’s disease. Arthritis Rheum. 2010;62:2796–805.PubMedGoogle Scholar
  87. 87.
    Ohno S, Nakamura S, Hori S. Efficacy, safety, and pharmacokinetics of multiple administration of infliximab in Behçet’s disease with refractory uveoretinitis. J Rheumatol. 2004;31:1362–8.PubMedGoogle Scholar
  88. 88.
    Tugal-Tutkun I, Mudun A, Urgancioglu M, et al. Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in Behçet’s disease: an open-label trial. Arthritis Rheum. 2005;52:2478–84.PubMedGoogle Scholar
  89. 89.
    Abu El-Asrar AM, Abboud EB, Aldibhi H, et al. Long-term safety and efficacy of infliximab therapy in refractory uveitis due to Behçet’s disease. Int Ophthalmol. 2005;26:83–92.PubMedGoogle Scholar
  90. 90.
    Niccoli L, Nannini C, Benucci M, et al. Long-term efficacy of infliximab in refractory posterior uveitis of Behcet’s disease: a 24-month follow-up study. Rheumatology (Oxford). 2007;46:1161–4.Google Scholar
  91. 91.
    Accorinti M, Pirraglia MP, Paroli MP, et al. Infliximab treatment for ocular and extraocular manifestations of Behçet’s disease. Jpn J Ophthalmol. 2007;51:191–6.PubMedGoogle Scholar
  92. 92.
    Al-Rayes H, Al-Swailem R, Al-Balawi M, et al. Safety and efficacy of infliximab therapy in active Behcet’s uveitis: an open-label trial. Rheumatol Int. 2008;29:53–7.PubMedGoogle Scholar
  93. 93.
    Okada AA, Goto H, Ohno S, et al. Multicenter study of infliximab for refractory uveoretinitis in Behçet disease. Arch Ophthalmol. 2012;130:592–8.PubMedGoogle Scholar
  94. 94.
    Al Rashidi S, Al Fawaz A, Kangave D, et al. Long-term clinical outcomes in patients with refractory uveitis associated with Behçet disease treated with infliximab. Ocul Immunol Inflamm. 2013;21(6):468–74.PubMedGoogle Scholar
  95. 95.
    Tabbara KF, Al-Hemidan AI. Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet disease. Am J Ophthalmol. 2008;146:845–50.e1.PubMedGoogle Scholar
  96. 96.
    Yamada Y, Sugita S, Tanaka H, et al. Comparison of infliximab versus ciclosporin during the initial 6-month treatment period in Behçet disease. Br J Ophthalmol. 2010;94:284–8.PubMedGoogle Scholar
  97. 97.
    Sugita S, Yamada Y, Mochizuki M. Relationship between serum infliximab levels and acute uveitis attacks in patients with Behcet disease. Br J Ophthalmol. 2011;95:549–52.PubMedGoogle Scholar
  98. 98.
    Elewaut D, Matucci-Cerinic M. Treatment of ankylosing spondylitis and extra-articular manifestations in everyday rheumatology practice. Rheumatology (Oxford). 2009;48:1029–35.Google Scholar
  99. 99.
    Kakkassery V, Mergler S, Pleyer U. Anti-TNF-alpha treatment: a possible promoter in endogenous uveitis? Observational report on six patients: occurrence of uveitis following etanercept treatment. Curr Eye Res. 2010;35:751–6.PubMedGoogle Scholar
  100. 100.
    Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behçet’s disease: a double blind, placebo controlled study. J Rheumatol. 2005;32:98–105.PubMedGoogle Scholar
  101. 101.
    Mushtaq B, Saeed T, Situnayake RD, et al. Adalimumab for sight-threatening uveitis in Behçet’s disease. Eye (Lond). 2007;21:824–5.Google Scholar
  102. 102.
    van Laar JA, Missotten T, van Daele PL, et al. Adalimumab: a new modality for Behçet’s disease? Ann Rheum Dis. 2007;66:565–6.PubMedCentralPubMedGoogle Scholar
  103. 103.
    Bawazeer A, Raffa LH, Nizamuddin SH. Clinical experience with adalimumab in the treatment of ocular Behçet disease. Ocul Immunol Inflamm. 2010;18:226–32.PubMedGoogle Scholar
  104. 104.
    Leccese P, Latanza L, D'Angelo S, et al. Efficacy of switching to adalimumab in a patient with refractory uveitis of Behçet’s disease to infliximab. Clin Exp Rheumatol. 2011;29(4 Suppl 67):S93.PubMedGoogle Scholar
  105. 105.
    Olivieri I, Leccese P, D'Angelo S, et al. Efficacy of adalimumab in patients with Behçet’s disease unsuccessfully treated with infliximab. Clin Exp Rheumatol. 2011;29(4 Suppl 67):S54–7.PubMedGoogle Scholar
  106. 106.
    Perra D, Alba MA, Callejas JL, et al. Adalimumab for the treatment of Behçet’s disease: experience in 19 patients. Rheumatology (Oxford). 2012;51:1825–31.Google Scholar
  107. 107.
    Mesquida M, Victoria Hernández M, Llorenç V, et al. Behçet disease-associated uveitis successfully treated with golimumab. Ocul Immunol Inflamm. 2013;21:160–2.PubMedGoogle Scholar
  108. 108.
    Gül A, Tugal-Tutkun I, Dinarello CA, et al. 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. 2012;71:563–6.PubMedGoogle Scholar
  109. 109.
    Sadreddini S, Noshad H, Molaeefard M, et al. Treatment of retinal vasculitis in Behcet’s disease with rituximab. Modern Rheumatol Jpn Rheum Assoc. 2008;18:306–8.Google Scholar
  110. 110.
    Davatchi F, Shams H, Rezaipoor M, et al. Rituximab in intractable ocular lesions of Behcet’s disease; randomized single-blind control study (pilot study). Int J Rheum Dis. 2010;13:246–52.PubMedGoogle Scholar
  111. 111.
    Hirano T, Ohguro N, Hohki S, et al. A case of Behçet’s disease treated with a humanized anti-interleukin-6 receptor antibody, tocilizumab. Mod Rheumatol. 2012;22:298–302.PubMedGoogle Scholar
  112. 112.
    Buggage RR, Levy-Clarke G, Sen HN, et al. A Double-masked, randomized study to investigate the safety and efficacy of daclizumab to treat the ocular complications related to Behçet’s disease. Ocul Immunol Inflamm. 2007;15:63–70.PubMedCentralPubMedGoogle Scholar
  113. 113.
    Dick A, Tugal-Tutkun I, Foster S, et al. Secukinumab in the treatment of noninfectious uveitis: results of three randomized, controlled clinical trials. Ophthalmology. 2013;120:777–87.PubMedGoogle Scholar
  114. 114.
    Onal S, Savar F, Akman M, et al. Vision- and health-related quality of life in patients with Behçet uveitis. Arch Ophthalmol. 2010;128:1265–71.PubMedGoogle Scholar
  115. 115.
    Sakai T, Watanabe H, Kuroyanagi K, et al. Health- and vision-related quality of life in patients receiving infliximab therapy for Behçet uveitis. Br J Ophthalmol. 2013;97:338–42.PubMedGoogle Scholar
  116. 116.
    Mamo FG. The rate of visual loss in Behçet’s disease. Arch Ophthalmol. 1970;84:451–2.PubMedGoogle Scholar
  117. 117.
    Mishima S, Masuda K, Izawa Y, et al. Mochizuki M, Namba K. Behçet’s disease in Japan: ophthalmological aspects. Trans Am Ophthalmol Soc. 1979;77:225–79.PubMedCentralPubMedGoogle Scholar
  118. 118.
    Cingu AK, Onal S, Urgancioglu M, et al. 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. 2012;20(6):423–8.PubMedGoogle Scholar
  119. 119.
    Shahram F, Davatchi F, Nadji A, et al. Recent epidemiological data on Behçet’s disease in Iran: the 2001 survey. Adv Exp Med Biol. 2003;528:31–6.PubMedGoogle Scholar
  120. 120.
    Kump LI, Moeller KL, Reed GF, et al. Behçet’s disease: comparing 3 decades of treatment response at the National Eye Institute. Can J Ophthalmol. 2008;43:468–72.PubMedCentralPubMedGoogle Scholar
  121. 121.
    Taylor SR, Singh J, Menezo V, et al. Behçet disease: visual prognosis and factors influencing the development of visual loss. Am J Ophthalmol. 2011;152:1059–66.PubMedGoogle Scholar
  122. 122.
    Kaçmaz RO, Kempen JH, Newcomb C, et al. Ocular inflammation in Behçet disease: incidence of ocular complications and of loss of visual acuity. Am J Ophthalmol. 2008;146:828–36.PubMedCentralPubMedGoogle Scholar
  123. 123.
    Yazici H, Tüzün Y, Pazarli H, et al. Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis. 1984;43:783.PubMedCentralPubMedGoogle Scholar
  124. 124.
    Demiroglu H, Barista I, Dundar S. Risk factor assessment and prognosis of eye involvement in Behçet’s disease in Turkey. Ophthalmology. 1997;104:701–5.PubMedGoogle Scholar
  125. 125.
    Yu HG, Kim MJ, Sewoong F. Fluorescein angiography and visual acuity in active uveitis with Behçet disease. Ocul Immunol Inflamm. 2009;17:41–6.PubMedGoogle Scholar
  126. 126.
    Takeuchi M, Hokama H, Tsukahara R, et al. Risk and prognostic factors of poor visual outcome in Behcet’s disease with ocular involvement. Graefes Arch Clin Exp Ophthalmol. 2005;243:1147–52.PubMedGoogle Scholar

Copyright information

© Springer India 2016

Authors and Affiliations

  1. 1.Department of OphthalmologyKoc University, School of MedicineIstanbulTurkey
  2. 2.Department of OphthalmologyV.K. Foundation, American HospitalIstanbulTurkey
  3. 3.Department of OphthalmologyIstanbul University, Istanbul Faculty of MedicineIstanbulTurkey

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