Best Practice & Research Clinical Rheumatology
Volume 22, Issue 5 , Pages 793-809 , October 2008

Behçet's syndrome

  • Sebahattin Yurdakul, MD (Professor of Medicine, Department of Medicine, Division of Rheumatology)

      Affiliations

    • Corresponding Author InformationCorresponding author. Atakoy 9 Kisim D 12 Daire 12 Bakirkoy 34156 Istanbul, Turkey, Tel.: +90 212 4143240; Fax: +90 212 5890808.
  • Hasan Yazici, MD (Professor and Chief of Department of Medicine and Division of Rheumatology)

References 

  1. Behçet H. Uber rezidivierende, aphthose, dürch ein Virus verursachte Geshwure am Munde, am Auge und an den Genitalien. Dermatologische Wochenschrift. 1937;36:1152–1157
  2. Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. The New England Journal of Medicine. 1999;41:1284–1291
  3. Yurdakul S, Hamuryudan V, Fresko I, Yazıcı H. Behçet's syndrome. In:  Hochberg MC,  Silman AJ,  Smolen YS, et al. editor. Rheumatology. 4th edn.. Philadelphia: Mosby Elsevier; 2008;p. 1561–1565
  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;54:213–220
  5. Demirhindi O, Yazici H, Binyildiz P, et al. Silivri Fener koyu ve yoresinde Behçet hastaligi sikligi ve bu hastaligin toplum icinde taranabilmesinde kullanabilecek bir yontem. Cerrahpaşa Tip Fakültesi Dergisi. 1981;12:509–514[in Turkish]
  6. Yurdakul S, Gunaydin I, Tuzun Y, et al. The prevalence of Behçet's syndrome in a rural area in northern Turkey. The Journal of Rheumatology. 1988;15:820–822
  7. 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 Epidemiology. 2002;9:325–331
  8. Azizlerli G, Kose AA, Sarica R, et al. Prevalence of Behçet's disease in Istanbul, Turkey. International Journal of Dermatology. 2003;42:803–806
  9. Cakir N, Dervis E, Benian O, et al. Prevalence of Behçet's disease in rural western Turkey: a preliminary report. Clinical and Experimental Rheumatology. 2004;22(4 Suppl. 34):S53–S55
  10. Ozen S, Karaaslan Y, Ozdemir O, et al. Prevalence of juvenile chronic arthritis and familial Mediterranean fever in Turkey: a field study. The Journal of Rheumatology. 1998;25:2445–2449
  11. Yamamoto S, Toyokawa H, Matsubara J, et al. A nation wide survey of Behçet's disease in Japan. Japanese Journal of Ophthalmology. 1974;18:282–290
  12. Hirohata T, Kuratsune M, Nomura A, Jimi S. Prevalence of Behçet's syndrome in Hawai. Hawaii Medical Journal. 1975;34:244–246
  13. Chamberlain MA. Behçet's syndrome in 32 patients in Yorkshire. Annals of the Rheumatic Diseases. 1977;36:491–499
  14. Gonzalez-Gay MA, Garcia-Porrua C, Branas F, et al. Epidemiologic and clinical aspects of Behçet's disease in a defined area of Northwestern Spain, 1988–1997. The Journal of Rheumatology. 2000;27:703–707
  15. Graña J, Sánchez-Meizoso MO, Galdo F. Epidemiological aspects of Behçet's disease in Galicia. The Journal of Rheumatology. 2001;28:2565–2566
  16. Mok CC, Cheung TC, Ho CT, et al. Behçet's disease in southern Chinese patients. The Journal of Rheumatology. 2002;29:1689–1693
  17. Al-Rawi ZS, Neda AH. Prevalence of Behçet's disease among Iraqis. Advances In Experimental Medicine and Biology. 2003;528:37–41
  18. Jaber L, Milo G, Halpern GJ, et al. Prevalence of Behçet's disease in an Arab community in Israel. Annals of the Rheumatic Diseases. 2002;61:365–366
  19. Papoutsis NG, Abdel-Naser MB, Altenburg A, et al. Prevalence of Adamantiades-Behçet's disease in Germany and the municipality of Berlin: results of a nationwide survey. Clinical and Experimental Rheumatology. 2006;24(5 Suppl. 42):S125
  20. Salvarani C, Pipitone N, Catanoso MG, et al. Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: a seventeen-year population-based study. Arthritis and Rheumatism. 2007;57:171–178
  21. Wilson FC, Icen M, Crowson CS, et al. Incidence and prevalence of Behçet's disease in a north American population. Arthritis and Rheumatism. 2007;56(Suppl.):abstract F 34
  22. Mahr AD, Belarbi L, Wechsler B, et al. Epidemiology of Behçet's disease in a multiethnic French population. Arthritis and Rheumatism. 2007;56(Suppl.):abstract no 1244
  23. Poon W, Verity DH, Larkin GL, et al. Behcet's disease in patients of West African and Afro-Caribbean origin. British Journal of Ophthalmology. 2003;87:876–878
  24. Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behçet disease (Behçet syndrome). Seminars in Arthritis and Rheumatism. 1979;8:223–260
  25. Yurdakul S, Tuzuner N, Yurdakul I, et al. Gastrointestinal involvement in Behçet's syndrome: a controlled study. Annals of the Rheumatic Diseases. 1996;55:208–210
  26. Yazıcı H, Chamberlain MA, Tuzun Y, et al. A comparative study of the pathergy among Turkish and British patients with Behçet's disease. Annals of the Rheumatic Diseases. 1984;43:74–75
  27. Yazici H, Tuzun 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. Annals of the Rheumatic Diseases. 1984;43:783–789
  28. Kone-Paut I, Yurdakul S, Bahabri SA, et al. Clinical features of Behçet's disease in children: an international colloborative study of 86 cases. The Journal of Pediatrics. 1998;132:721–725
  29. Mat C, Göksugur N, Engin B, et al. The frequency of scarring after genital ulcers in Behçet's syndrome: a prospective study. International Journal of Dermatology. 2006;45:554–556
  30. Ergun T, Gurbuz O, Dogusoy G, et al. Histopathologic features of the spontaneous pustular lesions of Behçet's syndrome. International Journal of Dermatology. 1998;37:194–196
  31. Tüzün Y, Yazıcı H, Pazarlı H, et al. The usefulness of the nonspecific skin hyperreactivity (The pathergy test) in Behçet's disease in Turkey. Acta Dermatovener (Stockholm). 1979;59:77–79
  32. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, et al. Uveitis in Behçet disease: an analysis of 880 patients. American Journal of Ophthalmology. 2004;138:373–380
  33. Behçet H, Gozcu N. Uç nahiyede nuksi tavazzular yapan ve hususi bir virus tesiriyle umumi intan hasil ettigine kanaatimiz artan (Entite morbide) hakkinda. Deri Hastaliklari ve Frengi Kliniği Arsivi. 1938;5:1863–1873[in Turkish]
  34. Yurdakul S, Yazici H, Tuzun Y, et al. The arthritis of Behçet's disease: a prospective study. Annals of the Rheumatic Diseases. 1983;42:505–515
  35. Yazici H, Tuzlaci M, Yurdakul S. A controlled survey of sacroiliitis in Behçet's disease. Annals of the Rheumatic Diseases. 1981;40:558–559
  36. Diri E, Mat C, Hamuryudan V, et al. Papulopustular skin lesions are seen more frequently in patients with Behçet's syndrome who have arthritis: a controlled and masked study. Annals of the Rheumatic Diseases. 2001;60:1074–1076
  37. Hatemi G, Fresko I, Tascilar K, Yazici H. Enthesopathy is increased among Behçet's syndrome patients with acne and arthritis: an ultrasonographic study. Arthritis and Rheumatism. 2008;58(5):1539–1545
  38. Muftuoglu AU, Yurdakul S, Yazici H, et al. Vascular involvement in Behçet disease: a review of 129 cases. In:  Lehner T,  Barnes CG editor. Recent advances in Behcet Disease. London: Royal Society of Medicine Services Ltd; 1986;p. 255–260
  39. Lie JT. Vascular involvement in Behçet's disease: arterial and venous and vessels of all sizes. The Journal of Rheumatology. 1992;19:341–343
  40. Koc Y, Gullu I, Akpek G, et al. Vascular involvement in Behçet's disease. The Journal of Rheumatology. 1992;19:402–410
  41. Sarica-Kucukoglu R, Akdag-Kose A, Kayabali M, et al. Vascular involvement in Behçet's disease: a retrospective analysis of 2319 cases. International Journal of Dermatology. 2006;45:919–921
  42. Düzgün N, Ateş A, Aydintuğ OT, et al. Characteristics of vascular involvement in Behçet's disease. Scandinavian Journal of Rheumatology. 2006;35:65–68
  43. Bayraktar Y, Ozaslan E, Van Thiel DH. Gastrointestinal manifestations of Behcet's disease. Journal of Clinical Gastroenterology. 2000;30:144–154
  44. Hamuryudan V, Yurdakul S, Moral F, et al. Pulmonary arterial aneurysms Behçet's syndrome: a report of 24 cases. British Journal of Rheumatology. 1994;33:48–51
  45. Hamuryudan V, Er T, Seyahi E, et al. Pulmonary artery aneurysms in Behçet syndrome. American Journal of Medicine. 2004;117:867–870
  46. Seyahi E, Melikoglu M, Akman C, et al. Pulmonary vascular involvement in Behçet's syndrome (BS). Arthritis and Rheumatism. 2007;56(Suppl.):abstract no 853
  47. Ozkan M, Emel O, Ozdemir M, et al. M-mode, 2-D and Doppler echocardiographic study in 65 patients with Behçet's syndrome. European Heart Journal. 1992;13:638–641
  48. Seyahi E, Ugurlu S, Cumali R, et al. Atherosclerosis in Behçet's syndrome. Seminars in Arthritis and Rheumatism. 2008;38:1–12
  49. Akman-Demir G, Serdaroglu P, Tasci B. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behcet Study Group. Brain. 1999;122:2171–2182
  50. Siva A, Kantarci OH, Saip S, et al. Behçet's disease: diagnostic and prognostic aspects of neurological involvement. Journal of Neurology. 2001;248:95–103
  51. Korman U, Cantasdemir M, Kurugoglu S, et al. Enteroclysis findings of intestinal Behcet disease: a comparative study with Crohn disease. Abdominal Imaging. 2003;28:308–312
  52. Melikoglu M, Altiparmak MR, Fresko I, et al. A reappraisal of amyloidosis in Behçet's syndrome. Rheumatology (Oxford). 2001;40:212–215
  53. Cetinel B, Akpinar H, Tufek I, et al. Bladder involvement in Behçet's syndrome. The Journal of Urology. 1999;161:52–56
  54. Melikoglu M, Kural-Seyahi E, Tascilar K, et al. The unique features of vasculitis in Behçet's syndrome. Clinical Reviews in Allergy & Immunology. 2008;35:40–46
  55. Kobayashi M, Ito M, Nakagawa A, et al. Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behçet disease. Histopathology. 2000;36:362–371
  56. Melikoglu M, Uysal S, Kreuger JG, et al. Characterization of the divergent wound-healing responses occurring in the pathergy reaction and normal healthy volunteers. Journal of Immunology. 2006;177:6415–6421
  57. Krause I, Weinberger A. Behçet's disease. Current Opinion In Rheumatology. 2008;20:82–87
  58. Matsumoto T, Uekusa T, Fukuda Y. Vasculo-Behçet's disease: a pathological study of eight cases. Human Pathology. 1991;22:45–51
  59. Lee KH, Chung HS, Kim HS, et al. Human alpha-enolase from endothelial cells as a target antigen of anti-endothelial cell antibody in Behçet's disease. Arthritis and Rheumatism. 2003;48:2025–2035
  60. Okuniki Y, Usui Y, Takeuchi M, et al. Proteomic survey of Behçet's disease with uveitis: selenium binding protein is a novel autoantigen in Behçet's disease. Experimental Eye Research. 2007;84:823–831
  61. Dalghous AM, Freysdottir J, Fortune F, et al. Expression of cytokines, chemokines and chemokine receptors in oral ulcers of pateints with Behçet's disease (BD) and recurrent aphthous stomatitis is Th1 associated, although Th2 association is also observed in patients with BD. Scandinavian Journal of Rheumatology. 2006;35:472–475
  62. Espinosa G, Font J, Tassies D, et al. Vascular involvement in Behçet's disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin. The American Journal of Medicine. 2002;112:37–43
  63. Hatemi G, Bahar H, Uysal S, et al. The pustular skin lesions in Behçet's syndrome are not sterile. Annals of the Rheumatic Diseases. 2004;63:1450–1452
  64. Gul A, Inanç M, Ocal L, et al. Familial aggregation of Behçet's disease in Turkey. Annals of the Rheumatic Diseases. 2000;59:622–625
  65. Karasneh J, Gul A, Ollier WE, et al. Whole-genome screening for susceptibility genes in multicase families with Behçet's disease. Arthritis and Rheumatism. 2005;52:1836–1842
  66. Gul A. Behçet's disease as an autoinflammatory disorder. Current Drug Targets. Inflammation and Allergy. 2005;4:81–83
  67. Yazici H, Fresko I. Behçet's disease and other autoinflammatory conditions: what's in a name?. Clinical and Experimental Rheumatology. 2005;23(4 Suppl. 38):S1–S2
  68. Ryan JG, Goldbach-Masky R. The spectrum of autoinflammatory disease: recent bench to bedside observations. Current Opinion in Rheumatology. 2008;20:66–75
  69. Çakır N, Yazıcı H, Chamberlain MA, et al. Response to intradermal injection of monosodium urate crystals in Behçet's syndrome. Annals of the Rheumatic Diseases. 1991;50:634–636
  70. Tunc R, Saip S, Siva A, Yazici H. Cerebral venous thrombosis is associated with major vessel disease in Behçet's syndrome. Annals of the Rheumatic Diseases. 2004;63:1693–1694
  71. Fresko I, Ugurlu S, Ozbakir F, et al. Anti-Saccharomyces cerevisiae antibodies (ASCA) in Behçet's syndrome. Clinical and Experimental Rheumatology. 2005;23(4 Suppl. 38):S67–S70
  72. Muftuoglu AU, Yazici H, Yurdakul S, et al. Behçet's disease. Relation of serum C-reactive protein and erythrocyte sedimentation rates to disease activity. International Journal of Dermatology. 1986;25:235–239
  73. International Study Group for Behçet's Disease . Criteria for diagnosis of Behçet's disease. Lancet. 1990;335:1078–1080
  74. O'Duffy JD. Suggested criteria for diagnosis of Behcet's disease. The Journal of Rheumatology. 1974;1(Suppl. 1):Abstr 32:18
  75. Mason RM, Barnes CG. Behcet's syndrome with arthritis. Annals of the Rheumatic Diseases. 1969;28:95–103
  76. Behçet's Disease Research Committee of Japan . Behçet's disease: guide to diagnosis of Behçet's disease. Japanese Journal of Ophthalmology. 1974;18:291–294
  77. Dilsen N, Konice M, Aral O. Our diagnostic criteria of Behcet's disease-an overview. In: T Lehner (ed) Recent advances in Behcet's Disease. International Congress Series pp 177–180, 1986.
  78. Cheng Zhang X-Q. Chinese Journal of Internal Medicine. 1980;19:15–20[in Chinese]
  79. O'Neill TW, Rigby AS, Silman AJ, Barnes CG. Validation of the International Study Group criteria for Behcet's disease. British Journal of Rheumatology. 1994;33:115–117
  80. Tunc R, Uluhan A, Melikoglu M, et al. A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome. Clinical and Experimental Rheumatology. 2001;19(5 Suppl. 24):S45–S47
  81. 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
  82. Aktulga E, Altac M, Muftuoglu A, et al. A double blind study of colchicine in Behçet's disease. Haematologica. 1980;65:399–402
  83. Yurdakul S, Mat C, Tuzun Y, et al. A double-blind trial of colchicine in Behçet's syndrome. Arthritis and Rheumatism. 2001;44:2686–2692
  84. Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behçet's syndrome. The New England Journal of Medicine. 1990;322:281–285
  85. Hamuryudan V, Ozyazgan Y, Hizli N, et al. Azathioprine in Behçet's syndrome: effects on long-term prognosis. Arthritis and Rheumatism. 1997;40:769–774
  86. Hamuryudan V, Ozyazgan Y, Fresko Y, et al. Interferon alfa combined with azathioprine for the uveitis of Behçet's disease: an open study. The Israel Medical Association journal: IMAJ. 2002;4(11 Suppl):928–930
  87. Masuda K, Nakajima A, Urayama A, et al. Double-masked trial of cyclosporin versus colchicine and long-term open study of cyclosporin in Behçet's disease. Lancet. 1989;333:1093–1096
  88. Hamuryudan V, Mat C, Saip S, et al. Thalidomide in the treatment of the mucocutaneous lesions of the Behçet syndrome. A randomized, double-blind, placebo-controlled trial. Annals of Internal Medicine. 1998;128:443–450
  89. Moral F, Hamuryudan V, Yurdakul S, Yazici H. Inefficacy of azapropazone in the acute arthritis of Behçet's syndrome: a randomized, double blind, placebo controlled study. Clinical and Experimental Rheumatology. 1995;13:493–495
  90. Alpsoy E, Durusoy C, Yilmaz E, et al. Interferon alfa-2a in the treatment of Behçet disease: a randomized placebo-controlled and double-blind study. Archives of Dermatology. 2002;138:467–471
  91. 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;46:736–741
  92. Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. The Journal of Rheumatology. 2005;32:98–105
  93. Mat C, Yurdakul S, Uysal S, et al. Double-blind trial of depot corticosteroids in Behçet's syndrome. Rheumatology (Oxford). 2006;45:348–352
  94. Denman AM, Hollingworth P, Webster ADB, et al. Failure of transfer factor in the treatment of Behçet's syndrome: a double-blind trial. In:  Lehner T,  Barnes CG editor. Behçet's Syndrome. London: Academic Press; 1979;p. 227–289
  95. Davies UM, Palmer RG, Denman AM. Treatment with acyclovir does not affect orogenital ulcers in Behcet's syndrome: a randomized double-blind trial. British Journal of Rheumatology. 1988;27:300–302
  96. 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. Ocular Immunology and Inflammation. 2007;15:63–70
  97. Sharquie KE, Najim RA, Abu-Raghif AR. Dapsone in Behçet's disease: a double-blind, placebo-controlled, cross-over study. The Journal of Dermatology. 2002;29:267–279
  98. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet's disease: report of a task force of the European Standing Committee for International Clinical Studies Including Therapeutics (ESCISIT). Annals of the Rheumatic Diseases, in press.

PII: S1521-6942(08)00094-6

doi: 10.1016/j.berh.2008.08.005

Best Practice & Research Clinical Rheumatology
Volume 22, Issue 5 , Pages 793-809 , October 2008