Skip to main content
SpringerLink
Log in

Behçet Disease

  • Chapter
Uveitis: An Update

Abstract

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.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 54.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Evereklioglu C. Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol. 2005;50:297–350.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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. 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.

    CAS  PubMed  Google Scholar 

  5. Keino H, Okada AA. Behçet’s disease: global epidemiology of an old Silk Road disease. Br J Ophthalmol. 2007;91(12):1573–4.

    PubMed Central  PubMed  Google Scholar 

  6. Azizlerli G, Kose AA, Sarica R, et al. Prevalence of Behçet’s disease in Istanbul, Turkey. Int J Dermatol. 2003;42:803–6.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  8. Tüzün Y, Yurdakul S, Mat MC, et al. Epidemiology of Behçet’s syndrome in Turkey. Int J Dermatol. 1996;35:618–20.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  11. Khairallah M, Accorinti M, Muccioli C, Kahloun R, Kempen JH. Epidemiology of Behçet disease. Ocul Immunol Inflamm. 2012;20:324–35.

    CAS  PubMed  Google Scholar 

  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. 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.

    PubMed Central  PubMed  Google Scholar 

  14. Kitaichi N, Ohno S. Behçet disease in children. Int Ophthalmol Clin. 2008;48:87–91.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  16. Tugal-Tutkun I. Behçet disease in the developing world. Int Ophthalmol Clin. 2010;50:87–98.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  18. Kone-Paut I, Gorchakoff-Molinas A, Weschler B, et al. Paediatric Behçet’s disease in France. Ann Rheum Dis. 2002;61:655–6.

    PubMed Central  CAS  PubMed  Google Scholar 

  19. Okada AA. Behçet’s disease: general concepts and recent advances. Curr Opin Ophthalmol. 2006;17:551–6.

    PubMed  Google Scholar 

  20. Tugal-Tutkun I. Behçet’s uveitis. Middle East Afr J Ophthalmol. 2009;16:219–24.

    PubMed Central  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  22. Deuter CM, Kötter I, Wallace GR, et al. Behçet’s disease: ocular effects and treatment. Prog Retin Eye Res. 2008;27:111–36.

    CAS  PubMed  Google Scholar 

  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. Marshall SE. Behçet’s disease. Best Pract Res Clin Rheumatol. 2004;18:291–311.

    CAS  PubMed  Google Scholar 

  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. 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.

    PubMed Central  PubMed  Google Scholar 

  27. Gül A. Behçet’s disease as an autoinflammatory disorder. Curr Drug Targets Inflamm Allergy. 2005;4:81–3.

    PubMed  Google Scholar 

  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. 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.

    PubMed Central  CAS  PubMed  Google Scholar 

  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.

    PubMed Central  CAS  PubMed  Google Scholar 

  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. 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.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  34. Wakabayashi T, Morimura Y, Miyamoto Y, et al. Changing patterns of intraocular inflammatory disease in Japan. Ocul Immunol Inflamm. 2003;11:277–86.

    PubMed  Google Scholar 

  35. Kitamei H, Kitaichi N, Namba K, et al. Clinical features of intraocular inflammation in Hokkaido, Japan. Acta Ophthalmol. 2009;87:424–8.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  39. Zamir E, Bodaghi B, Tugal-Tutkun I, et al. Conjunctival ulcers in Behçet’s disease. Ophthalmology. 2003;110:1137–41.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed Central  PubMed  Google Scholar 

  42. Yang P, Fang W, Meng Q, et al. Clinical features of Chinese patients with Behçet’s disease. Ophthalmology. 2008;115:312–18.e4.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  44. Zaidi AA, Ying GS, Daniel E, et al. Hypopyon in patients with uveitis. Ophthalmology. 2010;117:366–72.

    PubMed Central  PubMed  Google Scholar 

  45. Sachdev N, Kapali N, Singh R, et al. Spectrum of Behçet’s disease in the Indian population. Int Ophthalmol. 2009;29:495–501.

    PubMed  Google Scholar 

  46. Saleh OA, Birnbaum AD, Tessler HH, et al. Behçet uveitis in the American midwest. Ocul Immunol Inflamm. 2012;20:12–7.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  49. Tugal-Tutkun I. Imaging in the diagnosis and management of Behçet disease. Int Ophthalmol Clin. 2012;52:183–90.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  51. Tugal-Tutkun I, Gupta V, Cunningham ET. Differential diagnosis of Behçet uveitis. Ocul Immunol Inflamm. 2013;21(5):337–50.

    PubMed  Google Scholar 

  52. Atmaca LS. Fundus changes associated with Behçet’s disease. Graefes Arch Clin Exp Ophthalmol. 1989;227:340–4.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed Central  CAS  PubMed  Google Scholar 

  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. 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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  64. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008;67:1656–62.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed Central  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  69. Teoh SC, Hogan AC, Dick AD, et al. Mycophenolate mofetil for the treatment of uveitis. Am J Ophthalmol. 2008;146:752–60.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  71. Tanabe K. Calcineurin inhibitors in renal transplantation: what is the best option? Drugs. 2003;63:1535–48.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed Central  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed Central  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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. 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.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  98. Elewaut D, Matucci-Cerinic M. Treatment of ankylosing spondylitis and extra-articular manifestations in everyday rheumatology practice. Rheumatology (Oxford). 2009;48:1029–35.

    CAS  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  101. Mushtaq B, Saeed T, Situnayake RD, et al. Adalimumab for sight-threatening uveitis in Behçet’s disease. Eye (Lond). 2007;21:824–5.

    CAS  Google Scholar 

  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.

    PubMed Central  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed Central  CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  116. Mamo FG. The rate of visual loss in Behçet’s disease. Arch Ophthalmol. 1970;84:451–2.

    CAS  PubMed  Google Scholar 

  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.

    PubMed Central  CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed Central  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed Central  PubMed  Google Scholar 

  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.

    PubMed Central  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Ophthalmology, Koc University, School of Medicine, Istanbul, Turkey

    Sumru Onal MD, FEB. Ophth

  2. Department of Ophthalmology, V.K. Foundation, American Hospital, Istanbul, Turkey

    Sumru Onal MD, FEB. Ophth

  3. Department of Ophthalmology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey

    Ilknur Tugal-Tutkun MD

Authors
  1. Sumru Onal MD, FEB. Ophth
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ilknur Tugal-Tutkun MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ilknur Tugal-Tutkun MD .

Editor information

Editors and Affiliations

  1. Uveitis and Ocular Pathology Department, Sankara Nethralaya, Chennai, India

    Jyotirmay Biswas

  2. Department of Uvea and Intraocular Inflammation, Sankara Nethralaya, Chennai, India

    Parthopratim Dutta Majumder

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer India

About this chapter

Cite this chapter

Onal, S., Tugal-Tutkun, I. (2016). Behçet Disease. In: Biswas, J., Majumder, P. (eds) Uveitis: An Update. Springer, New Delhi. https://doi.org/10.1007/978-81-322-2295-8_3

Download citation

  • DOI: https://doi.org/10.1007/978-81-322-2295-8_3

  • Publisher Name: Springer, New Delhi

  • Print ISBN: 978-81-322-2294-1

  • Online ISBN: 978-81-322-2295-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation