Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Treatment of Behçet’s syndrome

Ellison L Smith, MD
Yusuf Yazici, MD
Section Editor
Peter A Merkel, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


Behçet’s syndrome is an inflammatory disease characterized by recurrent oral aphthous ulcers and numerous potential systemic manifestations. These include genital ulcers, ocular disease, skin lesions, neurologic disease, vascular disease, and arthritis.

Most clinical manifestations of Behçet’s syndrome are believed to be due to vasculitis. Among the systemic vasculitides, Behçet’s syndrome is remarkable for its ability to involve blood vessels of all sizes—small, medium, and large—on both the arterial and venous sides of the circulation.

The treatment of Behçet’s syndrome will be reviewed here. The pathogenesis, clinical manifestations, and diagnosis are discussed separately. (See "Pathogenesis of Behçet's syndrome" and "Clinical manifestations and diagnosis of Behçet's syndrome".)


The literature on the treatment of Behçet’s syndrome consists primarily of case reports and small case series, with few follow-up studies to confirm findings of preliminary reports and few randomized clinical trials.

In a systematic review from the Cochrane Database of trials conducted up until 1998, only 10 single- or double-blind trials fit the inclusion criteria [1]. The authors noted a lack of comparability across trials that precluded the pooling of data. The robustness of their analysis was hampered by the small number of studies.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Dec 08, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Saenz A, Ausejo M, Shea B, et al. Pharmacotherapy for Behcet's syndrome. Cochrane Database Syst Rev 2000; :CD001084.
  2. Yurdakul S, Mat C, Tüzün Y, et al. A double-blind trial of colchicine in Behçet's syndrome. Arthritis Rheum 2001; 44:2686.
  3. Hatemi G, Silman A, Bang D, et al. Management of Behçet disease: a systematic literature review for the European League Against Rheumatism evidence-based recommendations for the management of Behçet disease. Ann Rheum Dis 2009; 68:1528.
  4. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis 2008; 67:1656.
  5. Pipitone N, Olivieri I, Cantini F, et al. New approaches in the treatment of Adamantiades-Behçet's disease. Curr Opin Rheumatol 2006; 18:3.
  6. Barnes CG. Treatment of Behcet's syndrome. Rheumatology (Oxford) 2006; 45:245.
  7. Mat C, Yurdakul S, Uysal S, et al. A double-blind trial of depot corticosteroids in Behçet's syndrome. Rheumatology (Oxford) 2006; 45:348.
  8. Kaklamani VG, Kaklamanis PG. Treatment of Behçet's disease--an update. Semin Arthritis Rheum 2001; 30:299.
  9. Aktulga E, Altaç M, Müftüoglu A, et al. A double blind study of colchicine in Behçet's disease. Haematologica 1980; 65:399.
  10. Kazokoglu H, Saatçi O, Cuhadaroglu H, Eldem B. Long-term effects of cyclophosphamide and colchicine treatment in Behçet's disease. Ann Ophthalmol 1991; 23:148.
  11. Davatchi F, Sadeghi Abdollahi B, Tehrani Banihashemi A, et al. Colchicine versus placebo in Behçet's disease: randomized, double-blind, controlled crossover trial. Mod Rheumatol 2009; 19:542.
  12. Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behçet's syndrome. N Engl J Med 1990; 322:281.
  13. Hamuryudan V, Ozyazgan Y, Hizli N, et al. Azathioprine in Behcet's syndrome: effects on long-term prognosis. Arthritis Rheum 1997; 40:769.
  14. Saadoun D, Wechsler B, Terrada C, et al. Azathioprine in severe uveitis of Behçet's disease. Arthritis Care Res (Hoboken) 2010; 62:1733.
  15. 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.
  16. Hamza M, Meddeb S, Mili I, Ouertani A. [Bolus of cyclophosphamide and methylprednisolone in uveitis in Behçet's disease. Preliminary results with the use of new criteria of evaluation]. Ann Med Interne (Paris) 1992; 143:438.
  17. Du LT, Fain O, Wechsler B, et al. [Value of "bolus" cyclophosphamide injections in Behçet's disease. Experience of 17 cases]. Presse Med 1990; 19:1355.
  18. Atmaca LS, Batioğlu F. The efficacy of cyclosporin-a in the treatment of Behçet's disease. Ophthalmic Surg 1994; 25:321.
  19. Sajjadi H, Soheilian M, Ahmadieh H, et al. Low dose cyclosporin-A therapy in Behçet's disease. J Ocul Pharmacol 1994; 10:553.
  20. Whitcup SM, Salvo EC Jr, Nussenblatt RB. Combined cyclosporine and corticosteroid therapy for sight-threatening uveitis in Behçet's disease. Am J Ophthalmol 1994; 118:39.
  21. BenEzra D, Cohen E, Chajek T, et al. Evaluation of conventional therapy versus cyclosporine A in Behçet's syndrome. Transplant Proc 1988; 20:136.
  22. Kötter I, Günaydin I, Batra M, et al. CNS involvement occurs more frequently in patients with Behçet's disease under cyclosporin A (CSA) than under other medications--results of a retrospective analysis of 117 cases. Clin Rheumatol 2006; 25:482.
  23. Sakane T, Takeno M. Novel approaches to Behçet's disease. Expert Opin Investig Drugs 2000; 9:1993.
  24. Marshall SE. Behçet's disease. Best Pract Res Clin Rheumatol 2004; 18:291.
  25. 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.
  26. Arida A, Fragiadaki K, Giavri E, Sfikakis PP. Anti-TNF agents for Behçet's disease: analysis of published data on 369 patients. Semin Arthritis Rheum 2011; 41:61.
  27. Sfikakis PP, Theodossiadis PG, Katsiari CG, et al. Effect of infliximab on sight-threatening panuveitis in Behçet's disease. Lancet 2001; 358:295.
  28. Hassard PV, Binder SW, Nelson V, Vasiliauskas EA. Anti-tumor necrosis factor monoclonal antibody therapy for gastrointestinal Behçet's disease: a case report. Gastroenterology 2001; 120:995.
  29. Rozenbaum M, Rosner I, Portnoy E. Remission of Behçet's syndrome with TNFalpha blocking treatment. Ann Rheum Dis 2002; 61:283.
  30. Wechsler B, Sablé-Fourtassou R, Bodaghi B, et al. Infliximab in refractory uveitis due to Behçet's disease. Clin Exp Rheumatol 2004; 22:S14.
  31. Sarwar H, McGrath H Jr, Espinoza LR. Successful treatment of long-standing neuro-Behçet's disease with infliximab. J Rheumatol 2005; 32:181.
  32. Benitez-del-Castillo JM, Martinez-de-la-Casa JM, Pato-Cour E, et al. Long-term treatment of refractory posterior uveitis with anti-TNFalpha (infliximab). Eye (Lond) 2005; 19:841.
  33. Lanthier N, Parc C, Scavennec R, et al. Infliximab in the treatment of posterior uveitis in Behçet's disease. Long term follow up in four patients. Presse Med 2005; 34:916.
  34. Ohno S, Nakamura S, Hori S, et al. Efficacy, safety, and pharmacokinetics of multiple administration of infliximab in Behçet's disease with refractory uveoretinitis. J Rheumatol 2004; 31:1362.
  35. Rosenbaum JT. Blind insight: eyeing anti-tumor necrosis factor treatment in uveitis associated with Behçet's disease. J Rheumatol 2004; 31:1241.
  36. 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.
  37. 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.
  38. Giardina A, Ferrante A, Ciccia F, et al. One year study of efficacy and safety of infliximab in the treatment of patients with ocular and neurological Behçet's disease refractory to standard immunosuppressive drugs. Rheumatol Int 2011; 31:33.
  39. Iwata S, Saito K, Yamaoka K, et al. Efficacy of combination therapy of anti-TNF-α antibody infliximab and methotrexate in refractory entero-Behçet's disease. Mod Rheumatol 2011; 21:184.
  40. 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.
  41. Pipitone N, Olivieri I, Padula A, et al. Infliximab for the treatment of Neuro-Behçet's disease: a case series and review of the literature. Arthritis Rheum 2008; 59:285.
  42. Lindstedt EW, Baarsma GS, Kuijpers RW, van Hagen PM. Anti-TNF-alpha therapy for sight threatening uveitis. Br J Ophthalmol 2005; 89:533.
  43. Capella MJ, Foster CS. Long-term efficacy and safety of infliximab in the treatment of Behçet's disease. Ocul Immunol Inflamm 2012; 20:198.
  44. Takeuchi M, Asukata Y, Kawagoe T, et al. Infliximab monotherapy versus infliximab and colchicine combination therapy in patients with Behçet's disease. Ocul Immunol Inflamm 2012; 20:193.
  45. Cantini F, Niccoli L, Nannini C, et al. Efficacy of infliximab in refractory Behçet's disease-associated and idiopathic posterior segment uveitis: a prospective, follow-up study of 50 patients. Biologics 2012; 6:5.
  46. Lee JH, Cheon JH, Jeon SW, et al. Efficacy of infliximab in intestinal Behçet's disease: a Korean multicenter retrospective study. Inflamm Bowel Dis 2013; 19:1833.
  47. Takeuchi M, Kezuka T, Sugita S, et al. Evaluation of the long-term efficacy and safety of infliximab treatment for uveitis in Behçet's disease: a multicenter study. Ophthalmology 2014; 121:1877.
  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.
  49. Bawazeer A, Raffa LH, Nizamuddin SH. Clinical experience with adalimumab in the treatment of ocular Behçet disease. Ocul Immunol Inflamm 2010; 18:226.
  50. 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:S54.
  51. Calvo Catalá J, Campos Fernández C, Rueda Cid A, et al. [Efficacy of adalimumab in Behçet's disease. Description of 6 cases]. Reumatol Clin 2011; 7:258.
  52. Díaz-Llopis M, Salom D, Garcia-de-Vicuña C, et al. Treatment of refractory uveitis with adalimumab: a prospective multicenter study of 131 patients. Ophthalmology 2012; 119:1575.
  53. 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.
  54. Pivetti-Pezzi P, Accorinti M, Pirraglia MP, et al. Interferon alpha for ocular Behçet's disease. Acta Ophthalmol Scand 1997; 75:720.
  55. Mochizuki M. Immunotherapy for Behçet's disease. Int Rev Immunol 1997; 14:49.
  56. Nichols JC, Ince A, Akduman L, Mann ES. Interferon-alpha 2a treatment of neuro-Behcet disease. J Neuroophthalmol 2001; 21:109.
  57. Zouboulis CC, Orfanos CE. Treatment of Adamantiades-Behçet disease with systemic interferon alfa. Arch Dermatol 1998; 134:1010.
  58. Hamuryudan V, Moral F, Yurdakul S, et al. Systemic interferon alpha 2b treatment in Behçet's syndrome. J Rheumatol 1994; 21:1098.
  59. 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.
  60. Deuter CM, Kötter I, Günaydin I, et al. [Ocular involvement in Behçet's disease: first 5-year-results for visual development after treatment with interferon alfa-2a]. Ophthalmologe 2004; 101:129.
  61. 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.
  62. Alpsoy E, Yilmaz E, Başaran E. Interferon therapy for Behçet's disease. J Am Acad Dermatol 1994; 31:617.
  63. O'Duffy JD, Calamia K, Cohen S, et al. Interferon-alpha treatment of Behçet's disease. J Rheumatol 1998; 25:1938.
  64. Kuemmerle-Deschner JB, Tzaribachev N, Deuter C, et al. Interferon-alpha--a new therapeutic option in refractory juvenile Behçet's disease with CNS involvement. Rheumatology (Oxford) 2008; 47:1051.
  65. Yalçindağ FN, Uzun A. Results of interferon alpha-2a therapy in patients with Behcet's disease. J Ocul Pharmacol Ther 2012; 28:439.
  66. Kötter I, Günaydin I, Zierhut M, Stübiger N. The use of interferon alpha in Behçet disease: review of the literature. Semin Arthritis Rheum 2004; 33:320.
  67. Kötter I, Vonthein R, Zierhut M, et al. Differential efficacy of human recombinant interferon-alpha2a on ocular and extraocular manifestations of Behçet disease: results of an open 4-center trial. Semin Arthritis Rheum 2004; 33:311.
  68. 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.
  69. 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.
  70. 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.
  71. Adler YD, Mansmann U, Zouboulis CC. Mycophenolate mofetil is ineffective in the treatment of mucocutaneous Adamantiades-Behçet's disease. Dermatology 2001; 203:322.
  72. Shugaiv E, Tüzün E, Mutlu M, et al. Mycophenolate mofetil as a novel immunosuppressant in the treatment of neuro-Behçet's disease with parenchymal involvement: presentation of four cases. Clin Exp Rheumatol 2011; 29:S64.
  73. 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.
  74. Ehrlich GE. Behçet disease and the emergence of thalidomide. Ann Intern Med 1998; 128:494.
  75. 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.
  76. Seider N, Beiran I, Scharf J, Miller B. Intravenous immunoglobulin therapy for resistant ocular Behçet's disease. Br J Ophthalmol 2001; 85:1287.
  77. Russell AI, Lawson WA, Haskard DO. Potential new therapeutic options in Behçet's syndrome. BioDrugs 2001; 15:25.
  78. Boyvat A, Sişman-Solak C, Gürler A. Long-term effects of interferon alpha 2A treatment in Behçet's disease. Dermatology 2000; 201:40.
  79. Lockwood CM, Hale G, Waldman H, Jayne DR. Remission induction in Behçet's disease following lymphocyte depletion by the anti-CD52 antibody CAMPATH 1-H. Rheumatology (Oxford) 2003; 42:1539.
  80. Sharquie KE, Najim RA, Abu-Raghif AR. Dapsone in Behçet's disease: a double-blind, placebo-controlled, cross-over study. J Dermatol 2002; 29:267.
  81. Matsuda T, Ohno S, Hirohata S, et al. Efficacy of rebamipide as adjunctive therapy in the treatment of recurrent oral aphthous ulcers in patients with Behçet's disease: a randomised, double-blind, placebo-controlled study. Drugs R D 2003; 4:19.
  82. Hensel M, Breitbart A, Ho AD. Autologous hematopoietic stem-cell transplantation for Behçet's disease with pulmonary involvement. N Engl J Med 2001; 344:69.
  83. Rossi G, Moretta A, Locatelli F. Autologous hematopoietic stem cell transplantation for severe/refractory intestinal Behcet disease. Blood 2004; 103:748.
  84. Tomonari A, Tojo A, Takahashi T, et al. Resolution of Behçet's disease after HLA-mismatched unrelated cord blood transplantation for myelodysplastic syndrome. Ann Hematol 2004; 83:464.
  85. Stanford M, Whittall T, Bergmeier LA, et al. Oral tolerization with peptide 336-351 linked to cholera toxin B subunit in preventing relapses of uveitis in Behcet's disease. Clin Exp Immunol 2004; 137:201.
  86. Alpsoy E, Er H, Durusoy C, Yilmaz E. The use of sucralfate suspension in the treatment of oral and genital ulceration of Behçet disease: a randomized, placebo-controlled, double-blind study. Arch Dermatol 1999; 135:529.
  87. Tellier Z. Intravenous immunoglobulin in eye involvement. Clin Rev Allergy Immunol 2005; 29:295.
  88. Namba K, Sonoda KH, Kitamei H, et al. Granulocytapheresis in patients with refractory ocular Behcet's disease. J Clin Apher 2006; 21:121.
  89. Cantarini L, Vitale A, Scalini P, et al. Anakinra treatment in drug-resistant Behcet's disease: a case series. Clin Rheumatol 2015; 34:1293.
  90. Alpsoy E. Behçet's disease: treatment of mucocutaneous lesions. Clin Exp Rheumatol 2005; 23:532.
  91. Taylor J, Glenny AM, Walsh T, et al. Interventions for the management of oral ulcers in Behçet's disease. Cochrane Database Syst Rev 2014; :CD011018.
  92. Chams-Davatchi C, Barikbin B, Shahram F, et al. Pimecrolimus versus placebo in genital aphthous ulcers of Behcet's disease: a randomized double-blind controlled trial. Int J Rheum Dis 2010; 13:253.
  93. 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; 1:1093.
  94. Gardner-Medwin JM, Smith NJ, Powell RJ. Clinical experience with thalidomide in the management of severe oral and genital ulceration in conditions such as Behçet's disease: use of neurophysiological studies to detect thalidomide neuropathy. Ann Rheum Dis 1994; 53:828.
  95. Hatemi G, Melikoglu M, Tunc R, et al. Apremilast for Behçet's syndrome--a phase 2, placebo-controlled study. N Engl J Med 2015; 372:1510.
  96. Kim B, LeBoit PE. Histopathologic features of erythema nodosum--like lesions in Behçet disease: a comparison with erythema nodosum focusing on the role of vasculitis. Am J Dermatopathol 2000; 22:379.
  97. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med 1999; 341:1284.
  98. Reed JB, Morse LS, Schwab IR. High-dose intravenous pulse methylprednisolone hemisuccinate in acute Behçet retinitis. Am J Ophthalmol 1998; 125:409.
  99. Tuncer S, Yilmaz S, Urgancioglu M, Tugal-Tutkun I. 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.
  100. Levy-Clarke G, Jabs DA, Read RW, et al. Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology 2014; 121:785.
  101. 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:S93.
  102. Mushtaq B, Saeed T, Situnayake RD, Murray PI. Adalimumab for sight-threatening uveitis in Behçet's disease. Eye (Lond) 2007; 21:824.
  103. Calvo-Río V, Blanco R, Beltrán E, et al. Anti-TNF-α therapy in patients with refractory uveitis due to Behçet's disease: a 1-year follow-up study of 124 patients. Rheumatology (Oxford) 2014; 53:2223.
  104. 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; 4:928.
  105. Deuter CM, Kötter I, Günaydin I, et al. Efficacy and tolerability of interferon alpha treatment in patients with chronic cystoid macular oedema due to non-infectious uveitis. Br J Ophthalmol 2009; 93:906.
  106. Davatchi F, Sadeghi Abdollahi B, Shams H, et al. Combination of pulse cyclophosphamide and azathioprine in ocular manifestations of Behcet's disease: longitudinal study of up to 10 years. Int J Rheum Dis 2014; 17:444.
  107. Davatchi F, Shams H, Shahram F, et al. Methotrexate in ocular manifestations of Behcet's disease: a longitudinal study up to 15 years. Int J Rheum Dis 2013; 16:568.
  108. Jap A, Chee SP. Immunosuppressive therapy for ocular diseases. Curr Opin Ophthalmol 2008; 19:535.
  109. Kalra S, Silman A, Akman-Demir G, et al. Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. J Neurol 2014; 261:1662.
  110. Ward EM, Woodward TA, Mazlumzadeh M, Calamia KT. Gastrointestinal disease in Behçet's disease. Adv Exp Med Biol 2003; 528:459.
  111. Travis SP, Czajkowski M, McGovern DP, et al. Treatment of intestinal Behçet's syndrome with chimeric tumour necrosis factor alpha antibody. Gut 2001; 49:725.
  112. Kram MT, May LD, Goodman S, Molinas S. Behçet's ileocolitis: successful treatment with tumor necrosis factor-alpha antibody (infliximab) therapy: report of a case. Dis Colon Rectum 2003; 46:118.
  113. Kinoshita H, Kunisaki R, Yamamoto H, et al. Efficacy of infliximab in patients with intestinal Behçet's disease refractory to conventional medication. Intern Med 2013; 52:1855.
  114. Hisamatsu T, Ueno F, Matsumoto T, et al. The 2nd edition of consensus statements for the diagnosis and management of intestinal Behçet's disease: indication of anti-TNFα monoclonal antibodies. J Gastroenterol 2014; 49:156.
  115. Altiparmak MR, Tanverdi M, Pamuk ON, et al. Glomerulonephritis in Behçet's disease: report of seven cases and review of the literature. Clin Rheumatol 2002; 21:14.
  116. Akpolat T, Akkoyunlu M, Akpolat I, et al. Renal Behçet's disease: a cumulative analysis. Semin Arthritis Rheum 2002; 31:317.
  117. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behçet disease. Curr Opin Rheumatol 2005; 17:1.
  118. Hamuryudan V, Er T, Seyahi E, et al. Pulmonary artery aneurysms in Behçet syndrome. Am J Med 2004; 117:867.
  119. Adler S, Baumgartner I, Villiger PM. Behçet's disease: successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis. Arthritis Care Res (Hoboken) 2012; 64:607.
  120. Iscan ZH, Vural KM, Bayazit M. Compelling nature of arterial manifestations in Behcet disease. J Vasc Surg 2005; 41:53.
  121. Lê Thi Huong D, Wechsler B, Papo T, et al. Arterial lesions in Behçet's disease. A study in 25 patients. J Rheumatol 1995; 22:2103.
  122. Tuzun H, Seyahi E, Arslan C, et al. Management and prognosis of nonpulmonary large arterial disease in patients with Behçet disease. J Vasc Surg 2012; 55:157.
  123. Hosaka A, Miyata T, Shigematsu H, et al. Long-term outcome after surgical treatment of arterial lesions in Behçet disease. J Vasc Surg 2005; 42:116.
  124. Park JH, Chung JW, Joh JH, et al. Aortic and arterial aneurysms in behçet disease: management with stent-grafts--initial experience. Radiology 2001; 220:745.
  125. Nitecki SS, Ofer A, Karram T, et al. Abdominal aortic aneurysm in Behçet's disease: new treatment options for an old and challenging problem. Isr Med Assoc J 2004; 6:152.
  126. Ma WG, Zheng J, Zhu JM, et al. Aortic regurgitation caused by Behçet's disease: surgical experience during an 11-year period. J Card Surg 2012; 27:39.
  127. Mader R, Ziv M, Adawi M, et al. Thrombophilic factors and their relation to thromboembolic and other clinical manifestations in Behçet's disease. J Rheumatol 1999; 26:2404.
  128. Desbois AC, Wechsler B, Resche-Rigon M, et al. Immunosuppressants reduce venous thrombosis relapse in Behçet's disease. Arthritis Rheum 2012; 64:2753.
  129. Saadoun D, Wechsler B, Resche-Rigon M, et al. Cerebral venous thrombosis in Behçet's disease. Arthritis Rheum 2009; 61:518.
  130. Tayer-Shifman OE, Seyahi E, Nowatzky J, Ben-Chetrit E. Major vessel thrombosis in Behçet's disease: the dilemma of anticoagulant therapy - the approach of rheumatologists from different countries. Clin Exp Rheumatol 2012; 30:735.
  131. 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.
  132. Yazici H, Başaran G, Hamuryudan V, et al. The ten-year mortality in Behçet's syndrome. Br J Rheumatol 1996; 35:139.
  133. Park KD, Bang D, Lee ES, et al. Clinical study on death in Behçet's disease. J Korean Med Sci 1993; 8:241.
  134. Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behçet's disease. Arthritis Rheum 2010; 62:2806.
  135. Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain 1999; 122 ( Pt 11):2171.
  136. Kidd D, Steuer A, Denman AM, Rudge P. Neurological complications in Behçet's syndrome. Brain 1999; 122 ( Pt 11):2183.
  137. Farahangiz S, Sarhadi S, Safari A, Borhani-Haghighi A. Magnetic resonance imaging findings and outcome of neuro-Behçet's disease: the predictive factors. Int J Rheum Dis 2012; 15:e142.