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Medical Management of Behçet’s Syndrome

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Behçet’s Syndrome

Abstract

The current treatment of Behçet’s syndrome (BS) is directed at the suppression of symptoms and prevention of organ damage. The choice of the therapeutic agent depends on the type and severity of existing symptoms as well as the prognostic factors. Men and those developing the disease at a younger age need to be treated more aggressively since they are at increased risk for a more severe disease course. There is an indication that early treatment with azathioprine improves the long-term prognosis in this group of patients. Colchicine, azathioprine, cyclosporine A, interferon alpha (IFN alpha), and corticosteroids are the most frequently used drugs in the management of BS. Their efficacy has been documented by controlled trials that consistently target mucocutaneous symptoms, joint, and eye involvement. Thus, no formal data are available for the management of vascular, gastrointestinal, and central nervous system disease. TNF alpha inhibitors appear to be promising new agents for the treatment of serious manifestations that are resistant to conventional agents. Recently, a group of experts developed nine European recommendations that target all physicians involved in the management of BS.

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References

  1. Yurdakul S, Yazici H (2008) Behçet’s syndrome. Best Pract Res Clin Rheumatol 22:793–809

    Article  PubMed  Google Scholar 

  2. Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, Yurdakul S, Yazici H (2003) The long-term mortality and morbidity of Behçet’s syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 82:60–76

    Article  Google Scholar 

  3. Hamuryudan V, Ozyazgan Y, Hizli N, Mat C, Yurdakul S, Tuzun Y, Senocak M, Yazici H (1997) Azathioprine in Behçet’s syndrome: effects on long-term prognosis. Arthritis Rheum 40:769–774

    Article  PubMed  CAS  Google Scholar 

  4. Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, Houman MH, Kotter I, Olivieri I, Salvarani C et al (2008) EULAR recommendations for the management of Behçet’s disease. Ann Rheum Dis 67:1656–1662

    Article  PubMed  CAS  Google Scholar 

  5. Ben-Chetrit E, Levy M (1998) Colchicine: 1998 update. Semin Arthritis Rheum 28:48–59

    Article  PubMed  CAS  Google Scholar 

  6. Matsumura N, Mizushima Y (1975) Leucocyte movement and colchicine treatment in Behçet’s disease. Lancet 2:813

    Article  PubMed  CAS  Google Scholar 

  7. Mizushima Y, Matsumura N, Mori M, Shimizu T, Fukushima B, Mimura Y, Saito K, Sugiura S (1037) Colchicine in Behçet’s disease. Lancet 1977:2

    Google Scholar 

  8. Aktulga E, Altac M, Muftuoglu A, Ozyazgan Y, Pazarli H, Tuzun Y, Yalcin B, Yazici H, Yurdakul S (1980) A double blind study of colchicine in Behçet’s disease. Haematologica 65:399–402

    PubMed  CAS  Google Scholar 

  9. Yurdakul S, Mat C, Tuzun Y, Ozyazgan Y, Hamuryudan V, Uysal O, Senocak M, Yazici H (2001) A double-blind trial of colchicine in Behçet’s syndrome. Arthritis Rheum 44:2686–2692

    Article  PubMed  CAS  Google Scholar 

  10. Rosselet E, Saudan Y, Zenklusen G (1968) Effects of azathioprine (“Imuran”) in Behçet’s disease. Preliminary therapeutic results. Ophthalmologica 156:218–226

    Article  PubMed  CAS  Google Scholar 

  11. Yazici H, Pazarli H, Barnes CG, Tuzun Y, Ozyazgan Y, Silman A, Serdaroglu S, Oguz V, Yurdakul S, Lovatt GE et al (1990) A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med 322:281–285

    Article  PubMed  CAS  Google Scholar 

  12. Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, Houman MH, Kotter I, Olivieri I, Salvarani C et al (2009) Management of Behçet’s disease: a systematic literature review for the EULAR evidence based recommendations for the management of Behçet’s disease. Ann Rheum Dis 68:1528–1534

    Article  PubMed  CAS  Google Scholar 

  13. Hamuryudan V, Ozyazgan Y, Fresko Y, Mat C, Yurdakul S, Yazici H (2002) Interferon alfa combined with azathioprine for the uveitis of Behçet’s disease: an open study. Isr Med Assoc J 4:928–930

    PubMed  CAS  Google Scholar 

  14. Hamuryudan V, Er T, Seyahi E, Akman C, Tuzun H, Fresko I, Yurdakul S, Numan F, Yazici H (2004) Pulmonary artery aneurysms in Behçet’s syndrome. Am J Med 117:867–870

    Article  PubMed  Google Scholar 

  15. Kaklamani VG, Kaklamanis PG (2001) Treatment of Behçet’s disease–an update. Semin Arthritis Rheum 30:299–312

    Article  PubMed  CAS  Google Scholar 

  16. BenEzra D, Cohen E, Chajek T, Friedman G, Pizanti S, de Courten C, Harris W (1988) Evaluation of conventional therapy versus cyclosporine A in Behçet’s syndrome. Transplant Proc 20:136–143

    PubMed  CAS  Google Scholar 

  17. Saenz A, Ausejo M, Shea B, Wells G, Welch V, Tugwell P (2000) Pharmacotherapy for Behçet’s syndrome. Cochrane Database Syst Rev (2) CD001084

    Google Scholar 

  18. Ozyazgan Y, Yurdakul S, Yazici H, Tuzun B, Iscimen A, Tuzun Y, Aktunc T, Pazarli H, Hamuryudan V, Muftuoglu A (1992) Low dose cyclosporin A versus pulsed cyclophosphamide in Behçet’s syndrome: a single masked trial. Br J Ophthalmol 76:241–243

    Article  PubMed  CAS  Google Scholar 

  19. Masuda K, Nakajima A, Urayama A, Nakae K, Kogure M, Inaba G (1989) Double-masked trial of cyclosporin versus colchicine and long-term open study of cyclosporin in Behçet’s disease. Lancet 1:1093–1096

    Article  PubMed  CAS  Google Scholar 

  20. Cantini F, Salvarani C, Niccoli L, Padula A, Arena AI, Bellandi F, Macchioni P, Olivieri I (1999) Treatment of thrombophlebitis of Behçet’s disease with low dose cyclosporin A. Clin Exp Rheumatol 17:391–392

    PubMed  CAS  Google Scholar 

  21. Muftuoglu AU, Pazarli H, Yurdakul S, Yazici H, Ulku BY, Tuzun Y, Serdaroglu S, Altug E, Bahcecioglu H, Gungen G (1987) Short term cyclosporin A treatment of Behçet’s disease. Br J Ophthalmol 71:387–390

    Article  PubMed  CAS  Google Scholar 

  22. Akpolat T, Diri B, Oguz Y, Yilmaz E, Yavuz M, Dilek M (2003) Behçet’s disease and renal failure. Nephrol Dial Transplant 18:888–891

    Article  PubMed  CAS  Google Scholar 

  23. Kotake S, Higashi K, Yoshikawa K, Sasamoto Y, Okamoto T, Matsuda H (1999) Central nervous system symptoms in patients with Behçet’s disease receiving cyclosporine therapy. Ophthalmology 106:586–589

    Article  PubMed  CAS  Google Scholar 

  24. Kato Y, Numaga J, Kato S, Kaburaki T, Kawashima H, Fujino Y (2001) Central nervous system symptoms in a population of Behçet’s disease patients with refractory uveitis treated with cyclosporine A. Clin Exp Ophthalmol 29:335–336

    Article  CAS  Google Scholar 

  25. Kotter I, Gunaydin I, Batra M, Vonthein R, Stubiger N, Fierlbeck G, Melms A (2006) 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 25:482–486

    Article  PubMed  Google Scholar 

  26. Akman-Demir G, Ayranci O, Kurtuncu M, Vanli EN, Mutlu M, Tugal-Tutkun I (2008) Cyclosporine for Behçet’s uveitis: is it associated with an increased risk of neurological involvement? Clin Exp Rheumatol 26:S84–S90

    PubMed  CAS  Google Scholar 

  27. Perri AJ 3rd, Hsu S (2003) A review of thalidomide’s history and current dermatological applications. Dermatol Online J 9:5

    PubMed  Google Scholar 

  28. Direskeneli H, Ergun T, Yavuz S, Hamuryudan V, Eksioglu-Demiralp E (2008) Thalidomide has both anti-inflammatory and regulatory effects in Behçet’s disease. Clin Rheumatol 27:373–375

    Article  PubMed  CAS  Google Scholar 

  29. Saylan T, Saltik I (1982) Thalidomide in the treatment of Behçet’s syndrome. Arch Dermatol 118:536

    Article  PubMed  CAS  Google Scholar 

  30. Jorizzo JL, Schmalstieg FC, Solomon AR Jr, Cavallo T, Taylor RS 3rd, Rudloff HB, Schmalstieg EJ, Daniels JC (1986) Thalidomide effects in Behçet’s syndrome and pustular vasculitis. Arch Intern Med 146:878–881

    Article  PubMed  CAS  Google Scholar 

  31. Denman AM, Graham E, Howe L, Denman EJ, Lightman S (1993) Low dose thalidomide treatment of Behçet’s syndrome. In: Wechsler B, Godeau P (eds) Behçet’s disease. Proceedings of the 6th ınternational conference on Behçet’s disease. Excerpta Medica, Amsterdam, pp 649–653

    Google Scholar 

  32. Hamza MH (1986) Treatment of Behçet’s disease with thalidomide. Clin Rheumatol 5:365–371

    Article  PubMed  CAS  Google Scholar 

  33. Brik R, Shamali H, Bergman R (2001) Successful thalidomide treatment of severe infantile Behçet’s disease. Pediatr Dermatol 18:143–145

    Article  PubMed  CAS  Google Scholar 

  34. Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S, Zwingenberger K, Yazici H (1998) Thalidomide in the treatment of the mucocutaneous lesions of the Behçet’s syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med 128:443–450

    PubMed  CAS  Google Scholar 

  35. Zangari M, Anaissie E, Barlogie B, Badros A, Desikan R, Gopal AV, Morris C, Toor A, Siegel E, Fink L et al (2001) Increased risk of deep-vein thrombosis in patients with multiple myeloma receiving thalidomide and chemotherapy. Blood 98:1614–1615

    Article  PubMed  CAS  Google Scholar 

  36. Kobayashi K, Ueno F, Bito S, Iwao Y, Fukushima T, Hiwatashi N, Igarashi M, Iizuka BE, Matsuda T, Matsui T et al (2007) Development of consensus statements for the diagnosis and management of intestinal Behçet’s disease using a modified Delphi approach. J Gastroenterol 42:737–745

    Article  PubMed  Google Scholar 

  37. Mat C, Yurdakul S, Uysal S, Gogus F, Ozyazgan Y, Uysal O, Fresko I, Yazici H (2006) A double-blind trial of depot corticosteroids in Behçet’s syndrome. Rheumatology (Oxford) 45:348–352

    Article  CAS  Google Scholar 

  38. O’Duffy JD, Robertson DM, Goldstein NP (1984) Chlorambucil in the treatment of uveitis and meningoencephalitis of Behçet’s disease. Am J Med 76:75–84

    Article  PubMed  Google Scholar 

  39. Mudun BA, Ergen A, Ipcioglu SU, Burumcek EY, Durlu Y, Arslan MO (2001) Short-term chlorambucil for refractory uveitis in Behçet’s disease. Ocul Immunol Inflamm 9:219–229

    Article  PubMed  CAS  Google Scholar 

  40. Palmer RG, Dore CJ, Denman AM (1984) Chlorambucil-induced chromosome damage to human lymphocytes is dose-dependent and cumulative. Lancet 1:246–249

    Article  PubMed  CAS  Google Scholar 

  41. Tabbara KF (1983) Chlorambucil in Behçet’s disease. A reappraisal. Ophthalmology 90:906–908

    PubMed  CAS  Google Scholar 

  42. Sakane T, Mochizuki M, Inaba G, Masuda K (1995) A phase II study of FK506 (tacrolimus) on refractory uveitis associated with Behçet’s disease and allied conditions. Ryumachi 35:802–813

    PubMed  CAS  Google Scholar 

  43. Davatchi F, Shahram F, Chams H, Jamshidi AR, Nadji A, Chams C, Akbarian M, Gharibdoost F (2003) High dose methotrexate for ocular lesions of Behçet’s disease. Preliminary short-term results. Adv Exp Med Biol 528:579–584

    Article  PubMed  CAS  Google Scholar 

  44. Adler YD, Mansmann U, Zouboulis CC (2001) Mycophenolate mofetil is ineffective in the treatment of mucocutaneous Adamantiades-Behçet’s disease. Dermatology 203:322–324

    Article  PubMed  CAS  Google Scholar 

  45. Mumcu G, Inanc N, Yavuz S, Direskeneli H (2007) The role of infectious agents in the pathogenesis, clinical manifestations and treatment strategies in Behçet’s disease. Clin Exp Rheumatol 25:S27–S33

    PubMed  CAS  Google Scholar 

  46. Calguneri M, Kiraz S, Ertenli I, Benekli M, Karaarslan Y, Celik I (1996) The effect of prophylactic penicillin treatment on the course of arthritis episodes in patients with Behçet’s disease. A randomized clinical trial. Arthritis Rheum 39:2062–2065

    Article  PubMed  CAS  Google Scholar 

  47. Calguneri M, Ertenli I, Kiraz S, Erman M, Celik I (1996) Effect of prophylactic benzathine penicillin on mucocutaneous symptoms of Behçet’s disease. Dermatology 192:125–128

    Article  PubMed  CAS  Google Scholar 

  48. Mumcu G, Ergun T, Elbir Y, Eksioglu-Demiralp E, Yavuz S, Atalay T, Direskeneli H (2005) Clinical and immunological effects of azithromycin in Behçet’s disease. J Oral Pathol Med 34:13–16

    Article  PubMed  CAS  Google Scholar 

  49. Kaneko F, Oyama N, Nishibu A (1997) Streptococcal infection in the pathogenesis of Behçet’s disease and clinical effects of minocycline on the disease symptoms. Yonsei Med J 38:444–454

    PubMed  CAS  Google Scholar 

  50. Kahraman O, Celebi-Onder S, Kamali S (2003) Long term course of deep vein thrombosis in patients with Behçet’s disease. In: Proceedings of the American College of Rheumatology 67th annual meeting, Orlando, FL, Wiley, NJ, p S385

    Google Scholar 

  51. Ahn JK, Lee YS, Jeon CH, Koh EM, Cha HS (2008) Treatment of venous thrombosis associated with Behçet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clin Rheumatol 27:201–205

    Article  PubMed  Google Scholar 

  52. Sharquie KE, Najim RA, Abu-Raghif AR (2002) Dapsone in Behçet’s disease: a double-blind, placebo-controlled, cross-over study. J Dermatol 29:267–279

    PubMed  CAS  Google Scholar 

  53. Matsuda T, Ohno S, Hirohata S, Miyanaga Y, Ujihara H, Inaba G, Nakamura S, Tanaka S, Kogure M, Mizushima Y (2003) 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 4:19–28

    Article  PubMed  CAS  Google Scholar 

  54. Alpsoy E, Er H, Durusoy C, Yilmaz E (1999) The use of sucralfate suspension in the treatment of oral and genital ulceration of Behçet’s disease: a randomized, placebo-controlled, double-blind study. Arch Dermatol 135:529–532

    Article  PubMed  CAS  Google Scholar 

  55. de Merieux P, Spitler LE, Paulus HE (1981) Treatment of Behçet’s syndrome with levamisole. Arthritis Rheum 24:64–70

    Article  PubMed  Google Scholar 

  56. Wolf RE, Fudenberg HH, Welch TM, Spitler LE, Ziff M (1977) Treatment of Bechcet’s syndrome with transfer factor. JAMA 238:869–871

    Article  PubMed  CAS  Google Scholar 

  57. Davies UM, Palmer RG, Denman AM (1988) Treatment with acyclovir does not affect orogenital ulcers in Behçet’s syndrome: a randomized double-blind trial. Br J Rheumatol 27:300–302

    Article  PubMed  CAS  Google Scholar 

  58. Tasli L, Mat C, De Simone C, Yazici H (2006) Lactobacilli lozenges in the management of oral ulcers of Behçet’s syndrome. Clin Exp Rheumatol 24:S83–S86

    PubMed  CAS  Google Scholar 

  59. Ergun T, Gurbuz O, Yurdakul S, Hamuryudan V, Bekiroglu N, Yazici H (1997) Topical cyclosporine-A for treatment of oral ulcers of Behçet’s syndrome. Int J Dermatol 36:720

    Article  PubMed  CAS  Google Scholar 

  60. Kose O, Dinc A, Simsek I (2009) Randomized trial of pimecrolimus cream plus colchicine tablets versus colchicine tablets in the treatment of genital ulcers in Behçet’s disease. Dermatology 218:140–145

    Article  PubMed  CAS  Google Scholar 

  61. Hamuryudan V, Yurdakul S, Rosenkaimer F, Yazici H (1991) Inefficacy of topical alpha interferon alpha interferon in the treatment of oral ulcers of Behçet’s syndrome: a randomized, double blind trial. Br J Rheumatol 30:395–396

    PubMed  CAS  Google Scholar 

  62. Bacanli A, Yerebakan Dicle O, Parmaksizoglu B, Yilmaz E, Alpsoy E (2006) Topical granulocyte colony-stimulating factor for the treatment of oral and genital ulcers of patients with Behçet’s disease. J Eur Acad Dermatol Venereol 20:931–935

    Article  PubMed  CAS  Google Scholar 

  63. Yasui K, Ohta K, Kobayashi M, Aizawa T, Komiyama A (1996) Successful treatment of Behçet’s disease with pentoxifylline. Ann Intern Med 124:891–893

    PubMed  CAS  Google Scholar 

  64. Simsek H, Dundar S, Telatar H (1991) Treatment of Behçet’s disease with indomethacin. Int J Dermatol 30:54–57

    Article  PubMed  CAS  Google Scholar 

  65. Takeuchi A, Mori M, Hashimoto A, Chihara T (1984) Efficacy of oxaprozin in the treatment of articular symptoms of Behçet’s disease. Clin Rheumatol 3:397–399

    Article  PubMed  CAS  Google Scholar 

  66. Moral F, Hamuryudan V, Yurdakul S, Yazici H (1995) Inefficacy of azapropazone in the acute arthritis of Behçet’s syndrome: a randomized, double blind, placebo controlled study. Clin Exp Rheumatol 13:493–495

    PubMed  CAS  Google Scholar 

  67. Tsambaos D, Eichelberg D, Goos M (1986) Behçet’s syndrome: treatment with recombinant leukocyte alpha-interferon. Arch Dermatol Res 278:335–336

    Article  PubMed  CAS  Google Scholar 

  68. Hamuryudan V, Moral F, Yurdakul S, Mat C, Tuzun Y, Ozyazgan Y, Direskeneli H, Akoglu T, Yazici H (1994) Systemic interferon alpha 2b treatment in Behçet’s syndrome. J Rheumatol 21:1098–1100

    PubMed  CAS  Google Scholar 

  69. Demiroglu H, Ozcebe OI, Barista I, Dundar S, Eldem B (2000) Interferon alfa-2b, colchicine, and benzathine penicillin versus colchicine and benzathine penicillin in Behçet’s disease: a randomised trial. Lancet 355:605–609

    Article  PubMed  CAS  Google Scholar 

  70. Horton R (2000) Retraction: interferon alfa-2b...in Behçet’s disease. Lancet 356:1292

    Article  PubMed  CAS  Google Scholar 

  71. Alpsoy E, Durusoy C, Yilmaz E, Ozgurel Y, Ermis O, Yazar S, Basaran E (2002) Interferon alfa-2a in the treatment of Behçet’s disease: a randomized placebo-controlled and double-blind study. Arch Dermatol 138:467–471

    Article  PubMed  CAS  Google Scholar 

  72. Kotter I, Zierhut M, Eckstein AK, Vonthein R, Ness T, Gunaydin I, Grimbacher B, Blaschke S, Meyer-Riemann W, Peter HH et al (2003) Human recombinant interferon alfa-2a for the treatment of Behçet’s disease with sight threatening posterior or panuveitis. Br J Ophthalmol 87:423–431

    Article  PubMed  CAS  Google Scholar 

  73. Kotter I, Gunaydin I, Zierhut M, Stubiger N (2004) The use of interferon alpha in Behçet’s disease: review of the literature. Semin Arthritis Rheum 33:320–335

    Article  PubMed  CAS  Google Scholar 

  74. Kotter I, Vonthein R, Zierhut M, Eckstein AK, Ness T, Gunaydin I, Grimbacher B, Blaschke S, Peter HH, Stubiger N (2004) Differential efficacy of human recombinant interferon-alpha2a on ocular and extraocular manifestations of Behçet’s disease: results of an open 4-center trial. Semin Arthritis Rheum 33:311–319

    Article  PubMed  CAS  Google Scholar 

  75. Tugal-Tutkun I, Guney-Tefekli E, Urgancioglu M (2006) Results of interferon-alfa therapy in patients with Behçet’s uveitis. Graefes Arch Clin Exp Ophthalmol 244:1692–1695

    Article  PubMed  CAS  Google Scholar 

  76. Gueudry J, Wechsler B, Terrada C, Gendron G, Cassoux N, Fardeau C, Lehoang P, Piette JC, Bodaghi B (2008) Long-term efficacy and safety of low-dose interferon alpha2a therapy in severe uveitis associated with Behçet’s disease. Am J Ophthalmol 146:837–844, e831

    Article  PubMed  CAS  Google Scholar 

  77. Krause L, Altenburg A, Pleyer U, Kohler AK, Zouboulis CC, Foerster MH (2008) Longterm visual prognosis of patients with ocular Adamantiades-Behçet’s disease treated with interferon-alpha-2a. J Rheumatol 35:896–903

    PubMed  CAS  Google Scholar 

  78. Deuter CM, Kotter I, Gunaydin I, Zierhut M, Stubiger N (2004) Ocular involvement in Behçet’s disease: first 5-year-results for visual development after treatment with interferon alfa-2a. Ophthalmologe 101:129–134

    Article  PubMed  CAS  Google Scholar 

  79. Krause L, Kohler AK, Altenburg A, Papoutsis N, Zouboulis CC, Pleyer U, Stroux A, Foerster MH (2009) Ocular involvement in Adamantiades-Behçet’s disease in Berlin, Germany. Graefes Arch Clin Exp Ophthalmol 247:661–666

    Article  PubMed  Google Scholar 

  80. Deuter C, Kotter I, Mohle A, Vonthein R, Stubiger N, Zierhut M (2009) Long-term observations of vision and relapse-free intervals in patients with severe uveitis due to Behçet’s disease treated with interferon alfa. ARVO Abstract 2009 No. 2694

    Google Scholar 

  81. Deuter CM, Kotter I, Wallace GR, Murray PI, Stubiger N, Zierhut M (2008) Behçet’s disease: ocular effects and treatment. Prog Retin Eye Res 27:111–136

    Article  PubMed  CAS  Google Scholar 

  82. Guillaume-Czitrom S, Berger C, Pajot C, Bodaghi B, Wechsler B, Kone-Paut I (2007) Efficacy and safety of interferon-alpha in the treatment of corticodependent uveitis of paediatric Behçet’s disease. Rheumatology (Oxford) 46:1570–1573

    Article  CAS  Google Scholar 

  83. Kuemmerle-Deschner JB, Tzaribachev N, Deuter C, Zierhut M, Batra M, Koetter I (2008) Interferon-alpha – a new therapeutic option in refractory juvenile Behçet’s disease with CNS involvement. Rheumatology (Oxford) 47:1051–1053

    Article  CAS  Google Scholar 

  84. Hauschild A, Kahler KC, Schafer M, Fluck M (2008) Interdisciplinary management recommendations for toxicity associated with interferon-alfa therapy. J Dtsch Dermatol Ges 6:829–837, 829–838

    PubMed  Google Scholar 

  85. Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN (2001) Effect of infliximab on sight-threatening panuveitis in Behçet’s disease. Lancet 358:295–296

    Article  PubMed  CAS  Google Scholar 

  86. Benitez-del-Castillo JM, Martinez-de-la-Casa JM, Pato-Cour E, Mendez-Fernandez R, Lopez-Abad C, Matilla M, Garcia-Sanchez J (2005) Long-term treatment of refractory posterior uveitis with anti-TNFalpha (infliximab). Eye 19:841–845

    Article  PubMed  CAS  Google Scholar 

  87. Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, Hamuryudan V, Yazici H (2005) Short term trial of etanercept in Behçet’s disease: a double blind, placebo controlled study. J Rheumatol 32:98–105

    Article  PubMed  CAS  Google Scholar 

  88. Tabbara KF, Al-Hemidan AI (2008) Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet’s disease. Am J Ophthalmol 146:845–850, e841

    Article  PubMed  CAS  Google Scholar 

  89. Ribi C, Sztajzel R, Delavelle J, Chizzolini C (2005) Efficacy of TNF alpha blockade in cyclophosphamide resistant neuro-Behçet’s disease. J Neurol Neurosurg Psychiatry 76:1733–1735

    Article  PubMed  CAS  Google Scholar 

  90. Sarwar H, McGrath H Jr, Espinoza LR (2005) Successful treatment of long-standing neuro-Behçet’s disease with infliximab. J Rheumatol 32:181–183

    PubMed  Google Scholar 

  91. Alty JE, Monaghan TM, Bamford JM (2007) A patient with neuro-Behçet’s disease is successfully treated with etanercept: further evidence for the value of TNFalpha blockade. Clin Neurol Neurosurg 109:279–281

    Article  PubMed  Google Scholar 

  92. Belzunegui J, Lopez L, Paniagua I, Intxausti JJ, Maiz O (2008) Efficacy of infliximab and adalimumab in the treatment of a patient with severe neuro-Behçet’s disease. Clin Exp Rheumatol 26:S133–S134

    PubMed  CAS  Google Scholar 

  93. Kikuchi H, Aramaki K, Hirohata S (2008) Effect of infliximab in progressive neuro-Behçet’s syndrome. J Neurol Sci 272:99–105

    Article  PubMed  CAS  Google Scholar 

  94. Sfikakis PP, Kaklamanis PH, Elezoglou A, Katsilambros N, Theodossiadis PG, Papaefthimiou S, Markomichelakis N (2004) Infliximab for recurrent, sight-threatening ocular inflammation in Adamantiades-Behçet’s disease. Ann Intern Med 140:404–406

    PubMed  CAS  Google Scholar 

  95. Ohno S, Nakamura S, Hori S, Shimakawa M, Kawashima H, Mochizuki M, Sugita S, Ueno S, Yoshizaki K, Inaba G (2004) Efficacy, safety, and pharmacokinetics of multiple administration of infliximab in Behçet’s disease with refractory uveoretinitis. J Rheumatol 31:1362–1368

    PubMed  CAS  Google Scholar 

  96. Tugal-Tutkun I, Mudun A, Urgancioglu M, Kamali S, Kasapoglu E, Inanc M, Gul A (2005) 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 52:2478–2484

    Article  PubMed  CAS  Google Scholar 

  97. Tognon S, Graziani G, Marcolongo R (2007) Anti-TNF-alpha therapy in seven patients with Behçet’s uveitis: advantages and controversial aspects. Ann N Y Acad Sci 1110:474–484

    Article  PubMed  CAS  Google Scholar 

  98. Sfikakis PP, Markomichelakis N, Alpsoy E, Assaad-Khalil S, Bodaghi B, Gul A, Ohno S, Pipitone N, Schirmer M, Stanford M et al (2007) Anti-TNF therapy in the management of Behçet’s disease–review and basis for recommendations. Rheumatology (Oxford) 46:736–741

    Article  CAS  Google Scholar 

  99. Song IH, Appel H, Haibel H, Loddenkemper C, Braun J, Sieper J, Rudwaleit M (2008) New onset of Crohn’s disease during treatment of active ankylosing spondylitis with etanercept. J Rheumatol 35:532–536

    PubMed  CAS  Google Scholar 

  100. Lim LL, Fraunfelder FW, Rosenbaum JT (2007) Do tumor necrosis factor inhibitors cause uveitis? A registry-based study. Arthritis Rheum 56:3248–3252

    Article  PubMed  CAS  Google Scholar 

  101. Foeldvari I, Nielsen S, Kummerle-Deschner J, Espada G, Horneff G, Bica B, Olivieri AN, Wierk A, Saurenmann RK (2007) Tumor necrosis factor-alpha blocker in treatment of juvenile idiopathic arthritis-associated uveitis refractory to second-line agents: results of a multinational survey. J Rheumatol 34:1146–1150

    PubMed  CAS  Google Scholar 

  102. Lockwood CM, Hale G, Waldman H, Jayne DR (2003) Remission induction in Behçet’s disease following lymphocyte depletion by the anti-CD52 antibody CAMPATH 1-H. Rheumatology (Oxford) 42:1539–1544

    Article  CAS  Google Scholar 

  103. Buggage RR, Levy-Clarke G, Sen HN, Ursea R, Srivastava SK, Suhler EB, Altemare C, Velez G, Ragheb J, Chan CC et al (2007) 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 15:63–70

    Article  PubMed  CAS  Google Scholar 

  104. Sadreddini S, Noshad H, Molaeefard M, Noshad R (2008) Treatment of retinal vasculitis in Behçet’s disease with rituximab. Mod Rheumatol 18:306–308

    Article  PubMed  CAS  Google Scholar 

  105. Botsios C, Sfriso P, Furlan A, Punzi L, Dinarello CA (2008) Resistant Behçet’s disease responsive to anakinra. Ann Intern Med 149:284–286

    PubMed  Google Scholar 

  106. De Cata A, Intiso D, Bernal M, Molinaro F, Mazzoccoli G, D’Alessandro V, Greco A, Curci S, Sperandeo M, Frusciante V et al (2007) Prolonged remission of neuro-Behçet’s disease following autologous transplantation. Int J Immunopathol Pharmacol 20:91–96

    PubMed  Google Scholar 

  107. Rossi G, Moretta A, Locatelli F (2004) Autologous hematopoietic stem cell transplantation for severe/refractory intestinal Behçet’s disease. Blood 103:748–750

    Article  PubMed  CAS  Google Scholar 

  108. Maurer B, Hensel M, Max R, Fiehn C, Ho AD, Lorenz HM (2006) Autologous haematopoietic stem cell transplantation for Behçet’s disease with pulmonary involvement: analysis after 5 years of follow up. Ann Rheum Dis 65:127–129

    Article  PubMed  CAS  Google Scholar 

  109. Tomonari A, Tojo A, Takahashi T, Iseki T, Ooi J, Takahashi S, Nagamura F, Uchimaru K, Asano S (2004) Resolution of Behçet’s disease after HLA-mismatched unrelated cord blood transplantation for myelodysplastic syndrome. Ann Hematol 83:464–466

    Article  PubMed  Google Scholar 

  110. Marmont AM, Gualandi F, Piaggio G, Podesta M (2006) Teresa van Lint M, Bacigalupo A, Nobili F: allogeneic bone marrow transplantation (BMT) for refractory Behçet’s disease with severe CNS involvement. Bone Marrow Transplant 37:1061–1063

    Article  PubMed  CAS  Google Scholar 

  111. Nonami A, Takenaka K, Sumida C, Aizawa K, Kamezaki K, Miyamoto T, Harada N, Nagafuji K, Teshima T, Harada M (2007) Successful treatment of myelodysplastic syndrome (MDS)-related intestinal Behçet’s disease by up-front cord blood transplantation. Intern Med 46:1753–1756

    Article  PubMed  Google Scholar 

  112. Daikeler T, Kotter I, Bocelli Tyndall C, Apperley J, Attarbaschi A, Guardiola P, Gratwohl A, Jantunen E, Marmont A, Porretto F et al (2007) Haematopoietic stem cell transplantation for vasculitis including Behçet’s disease and polychondritis: a retrospective analysis of patients recorded in the European bone marrow transplantation and European league against rheumatism databases and a review of the literature. Ann Rheum Dis 66:202–207

    Article  PubMed  Google Scholar 

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Hamuryudan, V., Kötter, I. (2010). Medical Management of Behçet’s Syndrome. In: Yazıcı, Y., Yazıcı, H. (eds) Behçet’s Syndrome. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-5641-5_19

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