Skip to main content

Advertisement

Log in

Behçet’s Syndrome

  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Behçet’s syndrome is a systemic vasculitis with an unknown etiology affecting the small and large vessels of the venous and arterial systems. At least two clusters of disease expression have been described. The first includes superficial vein thrombosis, deep vein thrombosis, and dural sinus thrombi. The second includes acne, arthritis, and enthesitis. The presence of these clusters suggests there may be more than one disease mechanism operative in this complex disorder. Recent European League Against Rheumatism guidelines are useful for the management of the disease in organ systems distinct from the vascular, neurological, and gastrointestinal systems. This is because of a lack of controlled studies evaluating such vascular, neurological, and gastrointestinal complications.

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.

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Behçet H: Uber rezidivierende, aphthose, dürch ein Virus verursachte Geshwure am Munde, am Auge und an den Genitalien [in German]. Dematologische Wochenschrift 1937, 36:1152–1157.

    Google Scholar 

  2. Yurdakul S, Hamuryudan V, Fresko I, et al.: Behçet’s syndrome. In Rheumatology, edn 4. Edited by Hochberg MC, Silman AJ, Smolen YS, et al. Philadelphia, PA: Mosby Elsevier; 2008:1561–1565.

    Google Scholar 

  3. 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.

    Article  CAS  PubMed  Google Scholar 

  4. 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 [in Turkish]. Cerrahpasa Tip Fak Derg 1981, 12:509–514.

    Google Scholar 

  5. Yurdakul S, Gunaydin I, Tuzun Y, et al.: The prevalence of Behçet’s syndrome in a rural area in northern Turkey. J Rheumatol 1988, 15:820–822.

    CAS  PubMed  Google Scholar 

  6. Ozen S, Karaaslan Y, Ozdemir O, et al.: Prevalence of juvenile chronic arthritis and familial Mediterranean fever in Turkey: a field study. J Rheumatol 1998, 25:2445–2449.

    CAS  PubMed  Google Scholar 

  7. Jaber L, Milo G, Halpern GJ, et al.: Prevalence of Behçet’s disease in an Arab community in Israel. Ann Rheum Dis 2002, 61:365–366.

    Article  CAS  PubMed  Google Scholar 

  8. 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. Clin Exp Rheumatol 2006, 24(5 Suppl 42):S125.

    CAS  PubMed  Google Scholar 

  9. Shimizu T, Ehrlich GE, Inaba G, et al.: Behçet disease (Behçet syndrome). Semin Arthritis Rheum 1979, 8:223–260.

    Article  CAS  PubMed  Google Scholar 

  10. Yazici H, Chamberlain MA, Tuzun Y, et al.: A comparative study of the pathergy among Turkish and British patients with Behçet’s disease. Ann Rheum Dis 1984, 43:74–75.

    Article  CAS  PubMed  Google Scholar 

  11. 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. Ann Rheum Dis 1984, 43:783–789.

    Article  CAS  PubMed  Google Scholar 

  12. Mat C, Göksugur N, Engin B, et al.: The frequency of scarring after genital ulcers in Behçet’s syndrome: a prospective study. Int J Dermatol 2006, 45:554–556.

    Article  PubMed  Google Scholar 

  13. Ergun T, Gurbuz O, Dogusoy G, et al.: Histopathologic features of the spontaneous pustular lesions of Behçet’s syndrome. Int J Dermatol 1998, 37:194–196.

    Article  CAS  PubMed  Google Scholar 

  14. Tüzün Y, Yazici H, Pazarli H, et al.: The usefulness of the nonspecific skin hyperreactivity (the pathergy test) in Behçet’s disease in Turkey. Acta Derm Venereol 1979, 59:77–79.

    PubMed  Google Scholar 

  15. Mat C, Demirkesen C, Melikoglu M, et al.: Behçet’s syndrome. In Handbook of Systemic Autoimmune Diseases. The Skin in Autoimmune Diseases. Edited by Sarzi-Puttini P, Doria A, Girolomini G, Kuhn A. Philadelphia, PA: Elsevier; 2006:185–206.

    Chapter  Google Scholar 

  16. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, et al.: Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol 2004, 138:373–380.

    Article  PubMed  Google Scholar 

  17. Yurdakul S, Yazici H, Tuzun Y, et al.: The arthritis of Behçet’s disease: a prospective study. Ann Rheum Dis 1983, 42:505–515.

    Article  CAS  PubMed  Google Scholar 

  18. 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. Ann Rheum Dis 2001, 60:1074–1076.

    Article  CAS  PubMed  Google Scholar 

  19. Muftuoglu AU, Yurdakul S, Yazici H, et al.: Vascular involvement in Behçet disease: a review of 129 cases. In Recent Advances in Behcet Disease. Edited by Lehner T, Barnes CG. London: Royal Society of Medicine Services; 1986:255–260.

    Google Scholar 

  20. Lie JT: Vascular involvement in Behçet’s disease: arterial and venous and vessels of all sizes. J Rheumatol 1992, 19:341–343.

    CAS  PubMed  Google Scholar 

  21. Hamuryudan V, Yurdakul S, Moral F, et al.: Pulmonary arterial aneurysms in Behçet’s syndrome: a report of 24 cases. Br J Rheumatol 1994, 33:48–51.

    Article  CAS  PubMed  Google Scholar 

  22. Seyahi E, Melikoglu M, Akman C, et al.: Pulmonary vascular involvement in Behçet’s syndrome (BS) [abstract]. Arthritis Rheum 2007, 56(Suppl):853.

    Google Scholar 

  23. Seyahi E, Ugurlu S, Cumali R, et al.: Atherosclerosis in Behçet’s syndrome. Semin Arthritis Rheum 2008, 38:1–12.

    Article  CAS  PubMed  Google Scholar 

  24. 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.

    Article  PubMed  Google Scholar 

  25. Webb CJ, Moots RJ, Swift AC: Ear, nose and throat manifestations of Behçet's disease: a review. J Laryngol Otol 2008, 122:1279–1283.

    Article  CAS  PubMed  Google Scholar 

  26. Korman U, Cantasdemir M, Kurugoglu S, et al.: Enteroclysis findings of intestinal Behcet disease: a comparative study with Crohn disease. Abdom Imaging 2003, 28:308–312.

    Article  CAS  PubMed  Google Scholar 

  27. Melikoglu M, Kural-Seyahi E, Tascilar K, et al.: The unique features of vasculitis in Behçet’s syndrome. Clin Rev Allergy Immunol 2008, 35:40–46.

    Article  CAS  PubMed  Google Scholar 

  28. Gül A, Inanç M, Ocal L, et al.: Familial aggregation of Behçet’s disease in Turkey. Ann Rheum Dis 2000, 59:622–625.

    Article  PubMed  Google Scholar 

  29. Gül A: Behçet’s disease as an autoinflammatory disorder. Curr Drug Targets Inflamm Allergy 2005, 4:81–83.

    Article  PubMed  Google Scholar 

  30. Yazici H, Fresko I: Behçet’s disease and other autoinflammatory conditions: what’s in a name? Clin Exp Rheumatol 2005, 23(4 Suppl 38):S1–S2.

    PubMed  Google Scholar 

  31. Tunc R, Saip S, Siva A, et al.: Cerebral venous thrombosis is associated with major vessel disease in Behçet’s syndrome. Ann Rheum Dis 2004, 63:1693–1694.

    Article  CAS  PubMed  Google Scholar 

  32. Hatemi G, Bahar H, Uysal S, et al.: The pustular skin lesions in Behcet’s syndrome are not sterile. Ann Rheum Dis 2004, 63:1450–1452.

    Article  CAS  PubMed  Google Scholar 

  33. Fresko I, Ugurlu S, Ozbakir F, et al.: Anti-Saccharomyces cerevisiae antibodies (ASCA) in Behçet’s syndrome. Clin Exp Rheumatol 2005, 23(4 Suppl 38):S67–S70.

    CAS  PubMed  Google Scholar 

  34. Muftuoglu AU, Yazici H, Yurdakul S, et al.: Behçet’s disease. Relation of serum C-reactive protein and erythrocyte sedimentation rates to disease activity. Int J Dermatol 1986, 25:235–239.

    Article  CAS  PubMed  Google Scholar 

  35. Criteria for diagnosis of Behçet’s disease. International Study Group for Behçet’s Disease. Lancet 1990, 335:1078–1080.

    Google Scholar 

  36. O’Neill TW, Rigby AS, Silman AJ, et al.: Validation of the International Study Group criteria for Behcet’s disease. Br J Rheumatol 1994, 33:115–117.

    Article  PubMed  Google Scholar 

  37. Yazici H, Seyahi E, Yurdakul S: Behçet’s syndrome is not so rare: why do we need to know? Arthritis Rheum 2008, 58:3640–3643.

    Article  PubMed  Google Scholar 

  38. • 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.

    Article  Google Scholar 

  39. Aktulga E, Altac M, Muftuoglu A, et al.: A double blind study of colchicine in Behçet’s disease. Haematologica 1980, 65:399–402.

    CAS  PubMed  Google Scholar 

  40. Yurdakul S, Mat C, Tuzun Y, et al.: A double-blind trial of colchicine in Behçet’s syndrome. Arthritis Rheum 2001, 44:2686–2692.

    Article  CAS  PubMed  Google Scholar 

  41. Yazici H, Pazarli H, Barnes CG, et al.: A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med 1990, 322:281–285.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  43. 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.

    Article  Google Scholar 

  44. 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. Ann Intern Med 1998, 128:443–450.

    CAS  PubMed  Google Scholar 

  45. 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. Arch Dermatol 2002, 138:467–471.

    Article  CAS  PubMed  Google Scholar 

  46. Gueudry J, Wechsler B, Terrada C, et al.: Long-term efficacy and safety of low-dose interferon alpha2a therapy in severe uveitis associated with Behçet disease. Am J Ophthalmol 2008, 146:837.e1–844.e1.

    Article  Google Scholar 

  47. 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.

    Article  CAS  Google Scholar 

  48. 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 

  49. • 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). Ann Rheum Dis 2008, 67:1656–1662. This is a very good review of the current information on treatment of BS.

    Google Scholar 

Download references

Disclosure

Dr. Yusuf Yazici has served as a consultant for Bristol-Myers Squibb, Celgene Corp., Centocor Ortho Biotech, Genentech, Roche, and UCB.

Dr. Yurdakul has received travel support from Bristol-Myers Squibb.

Dr. Hasan Yazici has received travel support and served as a speaker/consultant for Schering-Plough, Wyeth, Bristol-Myers Squibb, and Celgene Corp.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Yusuf Yazici.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yazici, Y., Yurdakul, S. & Yazici, H. Behçet’s Syndrome. Curr Rheumatol Rep 12, 429–435 (2010). https://doi.org/10.1007/s11926-010-0132-z

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11926-010-0132-z

Keywords

Navigation