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Behçet Syndrome: Gastrointestinal Involvement

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Behçet Syndrome

Abstract

Vasculitic as well as inflammatory characteristics of gastrointestinal Behçet syndrome (GIBS) make it a distinct pattern of bowel inflammation and a bridging disease between vasculitis and inflammatory bowel disease (IBD). Reports indicate that the prevalence of gastrointestinal involvement of Behçet syndrome (BS) shows geographic variation. Since no true population surveys are available, at least some of this variation could be due to selection bias. The usually reported prevalence is less than 1% among patients from Western countries and the Middle East, while this frequency becomes 10% or even higher in the Far East. As in Crohn’s disease (CD), GIBS is seen most commonly in the ileocecal area. Lesions in the esophagus and stomach are distinctly rare. Although endoscopic morphology and distribution pattern of GI ulcerations may help in differentiation between CD and GIBS, it is sometimes difficult to differentiate GIBS from CD unless other stigmata of either condition are present. Among the mucocutaneous signs of BS, genital ulcer is a fairly specific sign of BS; however, unless clearly figured out from the patients’ history, existence of genital ulcers in a short period of time makes its sensitivity very poor. Thus far, complications like perforation and bleeding are more common in GIBS (25–50%) than in CD (∼2%). Management of GIBS is very similar to CD and is based on extrapolation from the IBD experience. As in IBD anti-TNF agents are now being used for refractory cases. This suggests that rather than the differentiation, prediction of complications may be more vital in IBD and BS clinics. Surgical treatment is reserved for complications like perforation, bleeding, obstruction, and stubborn fistula formation. Since relapses are more frequent without medical treatment, partly because of the pathergy phenomenon, surgical resection should be conservative and be applied with optimal postoperative medical treatment.

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References

  1. Bechgaard P Et tilfaelds af recidiverende aphtos stomatitis ledsaget af conjunctivitis og ulcerationer paa genitalia og hud. Ugeskr Laeger. 1940;102:1019–23.

    Google Scholar 

  2. Molodecky NA, Soon IS, Rabi DM, Ghali WA, Ferris M, Chernoff G, et al. Increasing incidence and prevalence of the inflammatory bowel diseases with time, based on systematic review. Gastroenterology. 2012;142:46–54.

    Article  PubMed  Google Scholar 

  3. Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behçet’s disease (Behçet syndrome). Semin Arthritis Rheum. 1979;8(4):223–60.

    Article  CAS  PubMed  Google Scholar 

  4. Lee SK, Kim WH. Diagnostic challenges in Asia: intestinal Behçet’s disease. Falk Symp. 2006;151:1–13.

    Google Scholar 

  5. Yang SK. Intestinal Behçet’s disease. Intest Res. 2005;3(1):1–10.

    Article  Google Scholar 

  6. Kaklamani VG, Vaiopoulos G, Kaklamanis PG. Behçet’s disease. Semin Arthritis Rheum. 1998;27(4):197–217.

    Article  CAS  PubMed  Google Scholar 

  7. Hatemi I, Hatemi G, Çelik AF. Gastrointestinal involvement in Behçet disease. Rheum Dis Clin N Am. 2018;44(1):45–64.

    Article  Google Scholar 

  8. O'Duffy JD, Carney JA, Deodhar S. Behçet’s disease. Report of 10 cases, 3 with new manifestations. Ann Intern Med. 1971;75:561–70.

    Article  CAS  PubMed  Google Scholar 

  9. Yamamato T, Toyokkawa H, Matsubara JT. A nation-wide survey of Behçet’s disease in Japan. Jpn J opthalmol. 1974;18:282–90.

    Google Scholar 

  10. Chamberlain MA. Behcet’s syndrome in 32 patients in Yorkshire. Ann Rheum Dis. 1977;36:491–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Eun HC, Chung H, Choi SJ. Clinical analysis of 114 patients with Behçet’s disease. J Korean Med Assoc. 27:933–9.

    Google Scholar 

  12. Jankowski J, Crombie I, Jankowski R. Behçet’s syndrome in Scotland. Postgrad Med J. 1992;68:566–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Dilsen N, Konice M, Aral O, et al. Risk factors of vital organ involvement in Behçet’s disease. In: Weschle B, Godeau F, editors. Behçet’s disease. Proceedings of the sixty International conference on Behçet’s disease. Amsterdam: Excrepta Medica. p. 165–9.

    Google Scholar 

  14. Yurdakul S, Tüzüner N, Yurdakul I, Hamuryudan V, Yazici H. Gastrointestinal involvement in Behçet’s syndrome: a controlled study. Ann Rheum Dis. 1996;55:208–10.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Gürler A, Boyvat A, Türsen U. Clinical manifestations of Behçet’s disease: an analysis of 2147 patients. Yonsei Med J. 1997;38:423–7.

    Article  PubMed  Google Scholar 

  16. Bang D, Yoon KH, Chung HG, Choi EH, Lee ES, Lee S. Epidemiological and clinical features of Behçet’s disease in Korea. Yonsei Med J. 1997;38:428–36.

    Article  CAS  PubMed  Google Scholar 

  17. Bang DS, Oh SH, Lee KH, Lee ES, Lee SN. Influence of sex on patients with Behçet’s disease in Korea. J Korean Med Sci. 2003;18:231–5.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Tursen U, Gurler A, Boyvat A. Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet’s disease. Int J Dermatol. 2003;42:346–51.

    Article  PubMed  Google Scholar 

  19. Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, 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 

  20. Yi SW, Kim JH, Lim KY, Bang D, Lee S, Lee ES. The Behcet’s Disease Quality of Life: reliability and validity of the Korean version. Yonsei Med J. 2008;49:698–704.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Alli N, Gur G, Yalcin B, Hayran M. Patient characteristics in Behçet disease: a retrospective analysis of 213 Turkish patients during 2001–4. Am J Clin Dermatol. 2009;10:411–8.

    Article  PubMed  Google Scholar 

  22. Neves FS, Caldas CA, Lage LV, Goldenstein-Schainberg C, Gonçalves CR. Faraway from the silk route: demographic and clinical features of Behçet’s disease in 106 Brazilian patients. Clin Rheumatol. 2009;28:543–6.

    Article  PubMed  Google Scholar 

  23. El Menyawi MM, Raslan HM, Edrees A. Clinical features of Behcet’s disease in Egypt. Rheumatol Int. 2009;29:641–6.

    Article  PubMed  Google Scholar 

  24. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M, et al. Behcet’s disease in Iran: analysis of 6500 cases. Int J Rheum Dis. 2010;13:367–73.

    Article  PubMed  Google Scholar 

  25. Oliveira AC, Buosi AL, Dutra LA, de Souza AW. Behçet disease: clinical features and management in a Brazilian tertiary hospital. J Clin Rheumatol. 2011;17:416–20.

    Article  PubMed  Google Scholar 

  26. Zhang Z, He F, Shi Y. Behcet’s disease seen in China: analysis of 334 cases. Rheumatol Int. 2013;33:645–8.

    Article  CAS  PubMed  Google Scholar 

  27. Kobayashi T, Kishimoto M, Swearingen CJ, Filopoulos MT, Ohara Y, Tokuda Y, et al. Differences in clinical manifestations, treatment, and concordance rates with two major sets of criteria for Behçet’s syndrome for patients in the US and Japan: data from a large, three-center cohort study. Mod Rheumatol. 2013;23:547–53.

    Article  PubMed  Google Scholar 

  28. Singal A, Chhabra N, Pandhi D, Rohatgi J. Behcet’s disease in India: A dermatological perspective. Indian J Dermatol Venereol Leprol. 2013;79:199–204.

    Article  Google Scholar 

  29. Olivieri I, Leccese P, Padula A, Nigro A, Palazzi C, Gilio M, et al. High prevalence of Behçet’s disease in southern Italy. Clin Exp Rheumatol. 2013;31:28–31.

    PubMed  Google Scholar 

  30. Mohammad A, Mandl T, Sturfelt G, Segelmark M. Incidence, prevalence and clinical characteristics of Behcet’s disease in southern Sweden. Rheumatology (Oxford). 2013;52:304–10.

    Article  Google Scholar 

  31. Sibley C, Yazici Y, Tascilar K, Khan N, Bata Y, Yazici H, et al. Behçet syndrome manifestations and activity in the United States versus Turkey a cross-sectional cohort comparison. J Rheumatol. 2014;41:1379–84.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Kim DY, Choi MJ, Cho S, Kim DW, Bang D. Changing clinical expression of Behçet disease in Korea during three decades (1983–2012): chronological analysis of 3674 hospital-based patients. Br J Dermatol. 2014;170:458–61.

    Article  CAS  PubMed  Google Scholar 

  33. Rodríguez-Carballeira M, Alba MA, Solans-Laqué R, Castillo MJ, Ríos-Fernández R, Larrañaga JR, et al. Registry of the Spanish network of Behçet’s disease: a descriptive analysis of 496 patients. Clin Exp Rheumatol. 2014;32:S33–9.

    PubMed  Google Scholar 

  34. Khabbazi A, Noshad H, Shayan FK, Kavandi H, Hajialiloo M, Kolahi S. Demographic and clinical features of Behcet’s disease in Azerbaijan. Int J Rheum Dis. 2014;

    Google Scholar 

  35. Hamzaoui A, Jaziri F, Ben Salem T, Said Imed Ben Ghorbel F, Lamloum M, Smiti Khanfir M, et al. Comparison of clinical features of Behcet disease according to age in a Tunisian cohort. Acta Med Iran. 2014;52:748–51.

    PubMed  Google Scholar 

  36. Ndiaye M, Sow AS, Valiollah A, Diallo M, Diop A, Alaoui RA, et al. Behçet’s disease in black skin. A retrospective study of 50 cases in Dakar. J Dermatol Case Rep. 2015, 31(9):98–102.

    Google Scholar 

  37. Lennikov A, Alekberova Z, Goloeva R, Kitaichi N, Denisov L, Namba K, et al. Single center study on ethnic and clinical features of Behcet’s disease in Moscow, Russia. Clin Rheumatol. 2015;34:321–7.

    Article  PubMed  Google Scholar 

  38. Ajose FO, Adelowo O, Oderinlo O. Clinical presentations of Behçet’s disease among Nigerians: a 4-year prospective study. Int J Dermatol. 2015;54:889–97.

    Article  CAS  PubMed  Google Scholar 

  39. Bonitsis NG, Luong Nguyen LB, LaValley MP, Papoutsis N, Altenburg A, Kötter I, et al. Gender-specific differences in Adamantiades-Behçet’s disease manifestations: an analysis of the German registry and meta-analysis of data from the literature. Rheumatology (Oxford). 2015;54:121–33.

    Article  CAS  Google Scholar 

  40. Kirino Y, Ideguchi H, Takeno M, Suda A, Higashitani K, Kunishita Y, et al. Continuous evolution of clinical phenotype in 578 Japanese patients with Behçet’s disease: a retrospective observational study. Arthritis Res Ther. 2016;3(18):217.

    Article  Google Scholar 

  41. Nanthapisal S, Klein NJ, Ambrose N, Eleftheriou D, Brogan PA. Paediatric Behçet’s disease: a UK tertiary centre experience. Clin Rheumatol. 2016;35:2509–16.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Cansu DÜ, Kaşifoğlu T, Korkmaz C. Do clinical findings of Behçet’s disease vary by gender?: A single-center experience from 329 patients. Eur J Rheumatol. 2016;3:157–60.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Ryu HJ, Seo MR, Choi HJ, Baek HJ. Clinical phenotypes of Korean patients with Behcet disease according to gender, age at onset, and HLA-B51. Korean J Intern Med. 2018;33(5):1025–31.

    Article  CAS  PubMed  Google Scholar 

  44. Hatemi I, Esatoglu SN, Hatemi G, et al. Characteristics,treatment, and long-term outcome of gastrointestinal involvement in Behcet’s syndrome: a strobe-compliant observational study from a dedicated multidisciplinary center. Medicine (Baltimore). 2016;95:e3348.

    Article  CAS  Google Scholar 

  45. Davatchi F, Chams-Davatchi C, Shams H, Nadji A, Faezi T, Akhlaghi M, et al. Adult Behcet’s disease in Iran: analysis of 6075 patients. Int J Rheum Dis. 2016;19(1):95–103.

    Article  CAS  PubMed  Google Scholar 

  46. Gallizzi R, Pidone C, Cantarini L, Finetti M, Cattalini M, Filocamo G, et al. A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry. Pediatr Rheumatol Online J. 2017;15(1):84.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Ghembaza ME, Bouabdallah N, Lounici A. Behçet disease in Western Algeria. Med Sante Trop. 2017;27(1):101–4.

    CAS  PubMed  Google Scholar 

  48. Han M, Jung YS, Kim WH, Cheon JH, Park S. Incidence and clinical outcomes of intestinal Behçet’s disease in Korea, 2011–2014: a nationwide population-based study. J Gastroenterol. 2017;52:920–8.

    Article  PubMed  Google Scholar 

  49. Mizushima Y, Inaba G, Mimura Y, Ono S. Diagnostic criteria for Behçet’s disease in 1987, and guidelines for treating Behçet’s disease. Saishin Igaku. 1988;43:382–91.

    Google Scholar 

  50. Yashiro, Cheon JH, Kim JH, Lee SK, Kim TI, Lee YC, et al. The prevalence and clinical characteristics of esophageal involvement in patients with Behçet’s disease: a single center experience in Korea. J Korean Med Sci. 2009;24(1):52–6.

    Article  Google Scholar 

  51. O’Duffy JD, Carney A, Deodhar S. Behçet’s disease: report of 10 cases, 3 with new manifestation. Ann Intern Med. 1971;75:561–70.

    Article  PubMed  Google Scholar 

  52. Chamberlain MA. Behçet’s syndrome in 32 patients in Yokshire. Ann Rheum Dis. 1977;36:491–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. Bayraktar Y, Ozaslan E, Van Thiel DH. Gastrointestinal manifestations of Behçet’s disease. J Clin Gastroenterol. 2000;30(2):144–54.

    Article  CAS  PubMed  Google Scholar 

  54. Yazici H, Tuzun Y, Pazarli H, Yurdakul S, Yalcin B. Muftuoglu A Behçet’s disease as seen in Turkey. Haematologica. 1980;65(3):381–3.

    CAS  PubMed  Google Scholar 

  55. Chang HK, Kim JW. The clinical features of Behçet’s disease in Yongdong district: analysis of a cohort followed from 1997 to 2001. J Korean Med Sci. 2002;17:784–9.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Bottomley WW, Dakkak M, Walton S, Bennett JR. Esophageal involvement in Behçet’s disease. Is endoscopy necessary? Dig Dis Sci. 1992;37(4):594–7.

    Article  CAS  PubMed  Google Scholar 

  57. Houman MH, Ben Ghorbel I, Lamloum M, Khanfir M, Braham A, Haouet S, et al. Esophageal involvement in Behçet’s disease. Yonsei Med J. 2002;43(4):457–60.

    Article  PubMed  Google Scholar 

  58. Kasahara Y, Tanaka S, Nishino M, Umemura H, Shiraha S, Kuyama T. Intestinal involvement in Behçet’s disease: review of 136 surgical cases in the Japanese literature. Dis Colon Rectum. 1981;24(2):103–6.

    Article  CAS  PubMed  Google Scholar 

  59. Ning-Sheng L, Ruay-Sheng L, Kuo-Chih T. High frequency of unusual gastric/duodenal ulcers in patients with Behçet’s disease in Taiwan: a possible correlation of MHC molecules with the development of gastric/duodenal ulcers. Clin Rheumatol. 2005;24(5):516–20.

    Article  PubMed  Google Scholar 

  60. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet’s disease. N Engl J Med. 1999;341(17):1284–91.

    Article  CAS  PubMed  Google Scholar 

  61. Yazici H, Tuzun Y, Pazarli H, Yurdakul S, Ozyazgan Y, Ozdogan 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(6):783–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Choi IJ, Kim JS, Cha SD, Jung HC, Park JG, Song IS, et al. Long-term clinical course and prognostic factors in intestinal Behçet’s disease. Dis Colon Rectum. 2000;43(5):692–700.

    Article  CAS  PubMed  Google Scholar 

  63. Lee CR, Kim WH, Cho YS, Kim MH, Kim JH, Park IS, et al. Colonoscopic findings in intestinal Behçet’s disease. Inflamm Bowel Dis. 2001;7(3):243–9.

    Article  CAS  PubMed  Google Scholar 

  64. Kim DK, Yang SK, Byeon JS, Myung SJ, Jo JY, Choi KD, et al. Clinical manifestations and course of intestinal Behçet’s disease: an analysis in relation to disease subtypes. Intest Res. 2005;3(1):48–54.

    Article  Google Scholar 

  65. Lang BA, Laxer RM, Thorner P, Greenberg M, Silverman ED. Pediatric onset of Behçet’s syndrome with myositis: case report and literature review illustrating unusual features. Arthritis Rheum. 1990;33(3):418–25.

    Article  CAS  PubMed  Google Scholar 

  66. Wu PS, Chen HL, Yang YH, Jeng YM, Lee PI, Chang MH. Intestinal Behçet’s disease presenting as neonatal onset chronic diarrhea in an 11-month-old male baby. Eur J Pediatr. 2005;164(8):523–5.

    Article  PubMed  Google Scholar 

  67. Fujikawa S, Suemitsu T. Behçet’s disease in children: a nationwide retrospective survey in Japan. Acta Paediatr Jpn. 1997;39(2):285–9.

    Article  CAS  PubMed  Google Scholar 

  68. Tabata M, Tomomasa T, Kaneko H, Morikawa A. Intestinal Behçet’s disease: a case report and review of Japanese reports in children. J Pediatr Gastroenterol Nutr. 1999;29(4):477–81.

    Article  CAS  PubMed  Google Scholar 

  69. Ng FH, Cheung TC, Chow KC, Wong SY, Ng WF, Chan HC, et al. Repeated intestinal perforation caused by an incomplete form of Behçet’s syndrome. J Gastroenterol Hepatol. 2001;16(8):935–9.

    Article  CAS  PubMed  Google Scholar 

  70. Isik B, Ara C, Kirimlioglu H, Sogutlu G, Yilmaz M, Yilmaz S, et al. Single or multiple perforations with varying locations as a complication of intestinal Behçet’s disease: report of three cases. Scand J Gastroenterol. 2005;40(5):599–603.

    Article  PubMed  Google Scholar 

  71. Pirildar T, Keser G, Tunc E, Alkanat M, Tuncyurek M, Doganavsargil E. An unusual presentation of Behçet’s disease: intestinal perforation. Clin Rheumatol. 2001;20(1):61–2.

    Article  CAS  PubMed  Google Scholar 

  72. Lee RG. The colitis of Behçet’s syndrome. Am J Surg Pathol. 1986;10(12):888–93.

    Article  CAS  PubMed  Google Scholar 

  73. Lee SK, Kim BK, Kim TI, Kim WH. Differential diagnosis of intestinal Behçet’s Disease and Crohn’s disease by colonoscopic findings. Endoscopy. 2009;41(1):9–16.

    Article  CAS  PubMed  Google Scholar 

  74. Iwama T, Utzunomiya J. Anal complication in Behçet’s syndrome. Jpn J Surg. 1977;7(3):114–7.

    Article  CAS  PubMed  Google Scholar 

  75. Kim SU, Cheon JH, Lim JS, Paik SH, Kim SK, Lee SK, et al. Massive gastrointestinal bleeding due to aneurysmal rupture of ileo-colic artery in a patient with Behçet’s disease. Korean J Gastroenterol. 2007;49(6):400–4.

    PubMed  Google Scholar 

  76. Smith JA, Siddiqui D. Intestinal Behçet’s disease presenting as a massive acute lower gastrointestinal bleed. Dig Dis Sci. 2002;47(3):517–21.

    Article  PubMed  Google Scholar 

  77. Kim WH, Cho YS, Yoo HM, Park IS, Park EC, Lim JG. Quality of life in Korean patients with inflammatory bowel diseases: ulcerative colitis, Crohn’s disease and intestinal Behçet’s disease. Int J Color Dis. 1999;14(1):52–7.

    Article  CAS  Google Scholar 

  78. Best WR, Becktel JM, Singleton JW, Kern F Jr. Development of a Crohn’s disease activity index. National cooperative Crohn’s disease study. Gastroenterology. 1976;70(3):439–44.

    Article  CAS  PubMed  Google Scholar 

  79. Cheon JH, Han DS, Park JY, Ye BD, Jung SA, Park YS, Kim YS, Kim JS, Nam CM, Kim YN, Yang SK, Kim WH, Korean IBD Study Group. Development, validation, and responsiveness of a novel disease activity index for intestinal Behçet’s disease. Inflamm Bowel Dis. 2011;17(2):605–13.

    Article  PubMed  Google Scholar 

  80. Mori S, Yoshihira A, Kawamura H, Takeuchi A, Hashimoto T, Inaba G. Esophageal involvement in Behçet’s disease. Am J Gastroenterol. 1983;78(9):548–53.

    CAS  PubMed  Google Scholar 

  81. Yashiro K, Nagasako K, Hasegawa K, Maruyama M, Suzuki S, Obata H. Esophageal lesions in intestinal Behçet’s disease. Endoscopy. 1986;18(2):57–60.

    Article  CAS  PubMed  Google Scholar 

  82. Anti M, Marra G, Rapaccini GL, Barone C, Manna R, Bochicchio GB, et al. Esophageal involvement in Behçet’s syndrome. J Clin Gastroenterol. 1986;8(5):514–9.

    Article  CAS  PubMed  Google Scholar 

  83. Brodie TE, Ochsner JL. Behçet’s syndrome with ulcerative oesophagitis: report of the first case. Thorax. 1973;28(5):637–40.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  84. Morimoto Y, Tanaka Y, Itoh T, Yamamoto S, Kurihara Y, Nishikawa K. Esophagobronchial fistula in a patient with Behçet’s disease: report of a case. Surg Today. 2005;35(8):671–6.

    Article  PubMed  Google Scholar 

  85. Celik AF, Hatemi I. Gastrointestinal involvement of Behçet’s syndrome. Turkiye Klinikleri J Int Med Sci. 2005;1:48–54.

    Google Scholar 

  86. Kural-Seyahi E, Ozdogan H, Yurdakul S, Ugurlu S, Ozyazgan Y, Mat C, et al. The outcome of the children with Behçet’s syndrome. Clin Exp Rheumatol. 2004;22.(Suppl 34:116a.

    Google Scholar 

  87. Hatemi I, Hatemi G, Celik AF, Melikoglu M, Arzuhal N, Mat C, et al. Frequency of pathergy phenomenon and other features of Behçet’s syndrome among patients with inflammatory bowel disease. Clin Exp Rheumatol. 2008;26.(Suppl 50:S91–5.

    CAS  PubMed  Google Scholar 

  88. Iscimen A, Imren S, Serdaroglu S, Kutlar M, et al. The significance of genital scars in the diagnosis of Behçet’s syndrome. In: 11th European congress of rheumatology. Athens, Greece; June 28 to July 4, 1987. p. F327.

    Google Scholar 

  89. Mat CM, Goksungur N, Engin B, Yurdakul S, Yazici H. The frequency of scarring after ulcers in Behçet’s syndrome: a prospective study. Int J Dermatol. 2006;45:554–6.

    Article  PubMed  Google Scholar 

  90. Fresko I, Ugurlu S, Ozbakır F, Celik A, Yurdakul S, Hamuryudan V, et al. Anti-Saccharomyces cerevisiae antibodies (ASCA) in Behçet’s syndrome. Clin Exp Rheumatol. 2005;23(Suppl 38):S67–70.

    CAS  PubMed  Google Scholar 

  91. Byeong GK, You SK, Joo SK, Hyun CJ, In SS. Diagnostic role of anti-Saccharomyces cerevisiae mannan antibodies combined with anti-neutrophil cytoplasmic antibodies in patients with inflammatory bowel diseases. Dis Colon Rectum. 2002;45:1062–9.

    Article  Google Scholar 

  92. Kim JS, Lim SH, Choi IJ, Moon H, Jung HC, Song IS, et al. Prediction of the clinical course of Behçet’s colitis according to macroscopic classification by colonoscopy. Endoscopy. 2000;32(8):635–40.

    Article  CAS  PubMed  Google Scholar 

  93. Pulimood AB, Ramakrishna BS, Kurian G, Peter S, Mathan MM. Endoscopic mucosal biopsies are useful in distinguishing granulomatous colitis due to Crohn’s disease from tuberculosis. Gut. 1999;45:537–41.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  94. Cigerciogulları E, Goksel S, Dogusoy B, Erdamar S, Celik AF, Erzin Y, et al. An analysis of the reliability of detection and diagnostic value of various pathologic features in Crohn’s disease. Virchows Arch. 2005;447(2):P5O7.

    Google Scholar 

  95. Kim DH, Cheon JH. Intestinal Behçet’s disease: a true inflammatory bowel disease or merely an intestinal complication of systemic vasculitis? Yonsei Med J. 2016;57(1):22–32.

    Article  CAS  PubMed  Google Scholar 

  96. Ben Ghorbel I, Ennaifer R, Lamloum M, Khanfir M, Miled M, Houman MH. Budd–Chiari syndrome associated with Behçet’s disease. Gastroenterol Clin Biol. 2008;32(3):316–20.

    Article  CAS  PubMed  Google Scholar 

  97. Seyahi E, Caglar E, Ugurlu S, Kantarci F, Hamuryudan V, Sonsuz A, Melikoglu M, Yurdakul S, Yazici H. An outcome survey of 43 patients with Budd-Chiari syndrome due to Behçet’s syndrome followed up at a single, dedicated center. Semin Arthritis Rheum. 2015;44(5):602–9.

    Article  PubMed  Google Scholar 

  98. Han M, Jung YS, Kim WH, Cheon JH, Park S. Cancer risk in patients with intestinal Behçet’s disease: a Nationwide Population-Based Study. Gut Liver. 2018;12(4):433–9.

    Article  PubMed  PubMed Central  Google Scholar 

  99. Park JJ, Cheon JH, Kim TI, Kim WH. Correlation of erythrocyte sedimentation rate and C-reactive protein with clinical disease activity in intestinal Behçet’s disease. Gut. 2009;58(suppl 2):A461.

    Google Scholar 

  100. Muftuoglu AU, Yazici H, Yurdakul S, Tuzun Y, Pazarli H, Gungen G, et al. Behçet’s disease. Relation of serum C-reactive protein and erythrocyte sedimentation rates to disease activity. Int J Dermatol. 1986;25(4):235–9.

    Article  CAS  PubMed  Google Scholar 

  101. Quinton J-F, Sendid B, Reumaux D, Cortot A, Grandbastien B, Charrier G, et al. Anti-Saccharomyces cerevisiae mannan antibodies combined with antineutrophil cytoplasmic auto-antibodies in inflammatory bowel disease: prevalence and diagnostic role. Gut. 1988;42:788–91.

    Article  Google Scholar 

  102. Choi CH, Kim TI, Kim BC, Shin SJ, Lee SK, Kim WH, et al. Anti-Saccharomyces cerevisiae antibody in intestinal Behçet’s disease patients: relation to clinical course. Dis Colon Rectum. 2006;49(12):1849–59.

    Article  PubMed  Google Scholar 

  103. Krause I, Monselise Y, Milo G, Weinberger A. Anti-Saccharomyces cerevisiae antibodies – a novel serologic marker for Behçet’s disease. Clin Exp Rheumatol. 2002;20(Suppl 26):S21–4.

    CAS  PubMed  Google Scholar 

  104. Makharia GK, Sachdev V, Gupta R, Lal S, Pandey RM. Anti-Saccharomyces cerevisiae antibody does not differentiate between Crohn’s disease and intestinal tuberculosis. Dig Dis Sci. 2007;52(1):33–9.

    Article  PubMed  Google Scholar 

  105. Kim SW, Jung YS, Ahn JB, Shin ES, Jang HW, Lee HJ, et al. Identification of genetic susceptibility loci for intestinal Behçet’s disease. Sci Rep. 2017;7:39850.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  106. Chang DK, Kim JJ, Choi H, Eun CS, Han DS, Byeon JS, et al. Double balloon endoscopy in small intestinal Crohn’s disease and other inflammatory diseases such as cryptogenic multifocal ulcerous stenosing enteritis (CMUSE). Gastrointest Endosc. 2007;66(Suppl 3):S96–8.

    Article  PubMed  Google Scholar 

  107. Hamdulay SS, Cheent K, Ghosh C, Stocks J, Ghosh S, Haskard DO. Wireless capsule endoscopy in the investigation of intestinal Behçet’s syndrome. Rheumatology (Oxford). 2008;47(8):1231–4.

    Article  CAS  Google Scholar 

  108. Gubler C, Bauerfeind P. Intestinal Behçet’s disease diagnosed by capsule endoscopy. Endoscopy. 2005;37(7):689.

    Article  CAS  PubMed  Google Scholar 

  109. Goldstein JL, Eisen GM, Lewis B, Gralnek IM, Zlotnick S, Fort JG. Video capsule endoscopy to prospectively assess small bowel injury with celecoxib, naproxen plus omeprazole, and placebo. Clin Gastroenterol Hepatol. 2005;3(2):133–41.

    Article  PubMed  Google Scholar 

  110. Graham DY, Opekun AR, Willingham FF, Qureshi WA. Visible small-intestinal mucosal injury in chronic NSAID users. Clin Gastroenterol Hepatol. 2005;3(1):55–9.

    Article  PubMed  Google Scholar 

  111. Thach BT, Cummings NA. Behçet’s syndrome with “aphthous colitis”. Arch Intern Med. 1976;136(6):705–9.

    Article  CAS  PubMed  Google Scholar 

  112. Lee KS, Kim SJ, Lee BC, Yoon DS, Lee WJ, Chi HS. Surgical treatment of intestinal Behçet’s disease. Yonsei Med J. 1997;38(6):455–60.

    Article  CAS  PubMed  Google Scholar 

  113. Lebwohl O, Forde KA, Berdon WE, Morrison S, Challop R. Ulcerative esophagitis and colitis in a pediatric patient with Behçet’s syndrome. Response to steroid therapy. Am J Gastroenterol. 1977;68(6):550–5.

    CAS  PubMed  Google Scholar 

  114. Kim JH, Choi BI, Han JK, Choo SW, Han MC. Colitis in Behçet’s disease: characteristics on double-contrast barium enema examination in 20 patients. Abdom Imaging. 1994;19(2):132–6.

    Article  CAS  PubMed  Google Scholar 

  115. Chung SY, Ha HK, Kim JH, Kim KW, Cho N, Cho KS, et al. Radiologic findings of Behçet’s syndrome involving the gastrointestinal tract. Radiographics. 2001;21(4):911–24.

    Article  CAS  PubMed  Google Scholar 

  116. Korman U, Cantasdemir M, Kurugoglu S, Mihmanli I, Soylu N, Hamuryudan V, et al. Enteroclysis findings of intestinal Behçet’s disease: a comparative study with Crohn disease. Abdom Imaging. 2003;28(3):308–12.

    Article  CAS  PubMed  Google Scholar 

  117. Ha HK, Lee HJ, Yang SK, Ki WW, Yoon KH, Shin YM, et al. Intestinal Behçet’s syndrome: CT features of patients with and patients without complications. Radiology. 1998;209(2):449–54.

    Article  CAS  PubMed  Google Scholar 

  118. Moreno N, Ripollés T, Paredes JM, Ortiz I, Martínez MJ, López A, et al. Usefulness of abdominal ultrasonography in the analysis of endoscopic activity in patients with Crohn’s disease: changes following treatment with immunomodulators and/or anti-TNF antibodies. J Crohns Colitis. 2014;8(9):1079–87.

    Article  PubMed  Google Scholar 

  119. de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behçet’s disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum. 2009;61:1287–96.

    Article  PubMed  CAS  Google Scholar 

  120. Maldini C, Lavalley MP, Cheminant M, et al. Relationships of HLA-B51 or B5 genotype with Behcet’s disease clinical characteristics: systematic review and meta-analyses of observational studies. Rheumatology (Oxford). 2012;51:887–900.

    Article  Google Scholar 

  121. Gullberg E, Soderholm JD. Peyer’s patches and M cells as potential sites of the inflammatory onset in Crohn’s disease. Ann N Y Acad Sci. 2006;1072:218–32.

    Article  CAS  PubMed  Google Scholar 

  122. Isomoto H, Shikuwa S, Suematsu T, Migita K, Ito M, Kohno S. Ileal lesions in Behçet’s disease originate in Peyer’s patches: findings on magnifying endoscopy. Scand J Gastroenterol. 2008;43(2):249–50.

    Article  PubMed  Google Scholar 

  123. Takada Y, Fujita Y, Igarashi M, Katsumata T, Okabe H, Saigenji K, et al. Intestinal Behçet’s disease pathognomonic changes in intramucosal lymphoid tissues and effect of a “rest cure” on intestinal lesions. J Gastroenterol. 1997;32(5):598–604.

    Article  CAS  PubMed  Google Scholar 

  124. Nara K, Kurokawa MS, Chiba S, Yoshikawa H, Tsukikawa S, Matsuda T, et al. Involvement of innate immunity in the pathogenesis of intestinal Behçet’s disease. Clin Exp Immunol. 2008;152(2):245–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  125. Hayasaki N, Ito M, Suzuki T, Ina K, Ando T, Kusugami K, et al. Neutrophilic phlebitis is characteristic of intestinal Behçet’s disease and simple ulcer syndrome. Histopathology. 2004;45(4):377–83.

    Article  CAS  PubMed  Google Scholar 

  126. Kobayashi M, Ito M, Nakagawa A, Matsushita M, Nishikimi N, Sakurai T, et al. Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behçet disease. Histopathology. 2000;36(4):362–71.

    Article  CAS  PubMed  Google Scholar 

  127. Zimmerman GA, Prescott SM, McIntyre TM. Endothelial cell interactions with granulocytes: tethering and signaling molecules. Immunol Today. 1992;13(3):93–100.

    Article  CAS  PubMed  Google Scholar 

  128. Senturk T, Aydintug O, Kuzu I, Duzgun N, Tokgoz G, Gurler A, et al. Adhesion molecule expression in erythema nodosum-like lesions in Behçet’s disease. A histopathological and immunohistochemical study. Rheumatol Int. 1998;18(2):51–7.

    Article  CAS  PubMed  Google Scholar 

  129. Lakhanpal S, Tani K, Lie JT, Katoh K, Ishigatsubo Y, Ohokubo T. Pathologic features of Behçet’s syndrome: a review of Japanese autopsy registry data. Hum Pathol. 1985;16(8):790–5.

    Article  CAS  PubMed  Google Scholar 

  130. Direskeneli H. Behçet’s disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum Dis. 2001;60(11):996–1002.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  131. Naganuma M, Iwao Y, Inoue N, Hisamatsu T, Imaeda H, Ishii H, et al. Analysis of clinical course and long-term prognosis of surgical and nonsurgical patients with intestinal Behçet’s disease. Am J Gastroenterol. 2000;95(10):2848–51.

    CAS  PubMed  Google Scholar 

  132. Imamura Y, Kurokawa MS, Yoshikawa H, Nara K, Takada E, Masuda C, et al. Involvement of Th1 cells and heat shock protein 60 in the pathogenesis of intestinal Behçet’s disease. Clin Exp Immunol. 2005;139(2):371–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  133. Ferrante A, Ciccia F, Principato A, et al. ATh1 but not a Th17 response is present in the gastrointestinal involvement of Behçet’s disease. Clin Exp Rheumatol. 2010;28:S27–30.

    PubMed  Google Scholar 

  134. Ortiz-Ferna’ndez L, Garcı’a-Lozano JR, Montes-Cano MA, et al. Association of haplotypes of the TLR8 locus with susceptibility to Crohn’s and Behçet’s diseases. Clin Exp Rheumatol. 2015;33:S117–22.

    Google Scholar 

  135. Sfikakis PP. Behçet’s disease: a new target for anti-tumour necrosis factor treatment. Ann Rheum Dis. 2002;61(Suppl 2):ii51–3.

    Article  PubMed  PubMed Central  Google Scholar 

  136. Uyar FA, Saruhan-Direskeneli G, Gül A. Common Crohn’s disease predisposing variants of the CARD15/NOD2 genes are not associated with Behçet’s disease in Turkey. Clin Exp Rheumatol. 2004;22(Suppl 34):S50–2.

    CAS  PubMed  Google Scholar 

  137. Ahmad T, Zhang L, Gogus F, Verity D, Wallace G, Madanat W, et al. CARD15 polymorphisms in Behçet’s disease. Scand J Rheumatol. 2005;34:233–7.

    Article  CAS  PubMed  Google Scholar 

  138. Ozen SC, Dagli U, Kilic MY, Toruner M, Celik Y, Ozkan M, et al. NOD2/CARD15, NOD1/CARD4, and ICAM-1 gene polymorphism in Turkish patients with inflammatory bowel disease. J Gastroenterol. 2006;41:304–10.

    Article  CAS  PubMed  Google Scholar 

  139. Uyar FA, Over-Hamzaoglu H, Ture F, Gul A, Tozun N, Saruhan-Direskeneli G. Distribution of common CARD15 variants in patients sporadic Crohn’s disease cases from Turkey. Dig Dis Sci. 2006;51(4):706–10.

    Article  PubMed  Google Scholar 

  140. Kobayashi T, Sudo Y, Okamura S, Ohashi S, Urano F, Hosoi T, et al. Monozygotic twins concordant for intestinal Behçet’s disease. J Gastroenterol. 2005;40(4):421–5.

    Article  PubMed  Google Scholar 

  141. Hamuryudan V, Yurdakul S, Ozbakir F, Yazici H, Hekim H. Monozygotic twins concordant for Behçet’s syndrome. Arthritis Rheum. 1991;34(8):1071–2.

    Article  CAS  PubMed  Google Scholar 

  142. Kim SW, Jung YS, Ahn JB, Shin ES, Jang HW, Lee HJ, Il Kim T, Kim DY, Bang D, Kim WH, Cheon JH. Identification of genetic susceptibility loci for intestinal Behçet’s disease. Sci Rep. 2017;7:39850.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  143. Soy M, Erken E, Konca K, Ozbek S. Smoking and Behçet’s disease. Clin Rheumatol. 2000;19:508–9.

    Article  CAS  PubMed  Google Scholar 

  144. Kaklamani VG, Markkomichelakis N, Kaklamanis PG. Could nicotine be beneficial for Behçet’s disease. Clin Rheumatol. 2002;21:341–2.

    Article  CAS  PubMed  Google Scholar 

  145. Edward JB, Koepsell TD, Prera DR, Inuni TS. Risk of ulcerative colitis among former and current cigarette smokers. N Engl J Med. 1987;316:707–10.

    Article  Google Scholar 

  146. Somerville KW, Logan RFA, Edmond M, Langman MJS. Smoking and Crohn’s disease. Br Med J. 1984;289:954–6.

    Article  CAS  Google Scholar 

  147. Ananthakrishnan AN. Epidemiology and risk factors for IBD. Nat Rev Gastroenterol Hepatol. 2015;12:205–17.

    Article  PubMed  Google Scholar 

  148. Fresko I, Hamuryudan V, Demir M, Hizli N, Sayman H, Melikoglu M, et al. Intestinal permeability in Behçet’s syndrome. Ann Rheum Dis. 2001;60:65–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  149. Koc B, Aymelek S, Sonmez A, Yilmaz MI, Kocar H. Increased sucrose permeability in Behçet’s disease. Rheumatol Int. 2004;24(6):347–50.

    Article  PubMed  Google Scholar 

  150. Kobayashi K, Ueno F, Bito S, Iwao Y, Fukushima T, Hiwatashi N, et al. Development of consensus statements for the diagnosis and management of intestinal Behçet’s disease using a modified Delphi approach. J Gastroenterol. 2007;42(9):737–45.

    Article  PubMed  Google Scholar 

  151. Shin SJ, Kim BC, Park SY, Kim TI, Kim WH. Systemic manifestations of Behçet’s disease in diagnosis of intestinal Behçet’s disease. Gut. 2005;55:A120.

    Google Scholar 

  152. Jung HC, Rhee PL, Song IS, Choi KW, Kim CY. Temporal changes in the clinical type or diagnosis of Behçet’s colitis in patients with aphthoid or punched-out colonic ulcerations. J Korean Med Sci. 1991;6(4):313–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  153. Cheon JH, Kim ES, Shin SJ, et al. Development and Validation of Novel Diagnostic Criteria for Intestinal Behçet’s Disease in Korean Patients with Ileo-colonic Ulcers. Am J Gastroenterol. 2009;104(10):2492–9.

    Article  PubMed  Google Scholar 

  154. Yim CW, White RH. Behçet’s syndrome in a family with inflammatory bowel disease. Arch Intern Med. 1985;145(6):1047–50.

    Article  CAS  PubMed  Google Scholar 

  155. Kim DH, Cheon JH. Intestinal Behçet’s disease: a true inflammatory bowel disease or merely an intestinal complication of systemic vasculitis? Yonsei Med J. 2016;57(1):22–32.

    Article  CAS  PubMed  Google Scholar 

  156. Af C, Hatemi I, Hatemi G, Satir E, Erzin Y, Sisman G, et al. A comparative study of clinical, endoscopic and histologic findings in patients with gastrointestinal behçet’s disease and crohn’s disease. Clin Exp Dermatol. 2010;28(4 SUPPL 60):S–111.

    Google Scholar 

  157. Hatemi I, Erzin Y, Kochan K, Aygun G, Dogusoy G, Erdamar S, et al. Celik Comparison of Crohn’s disease and intestinal tuberculosis by clinical, laboratory, endoscopic, radiologic and histologic parameters. J Crohn’s Coli. 2012;6:76–7.

    Article  Google Scholar 

  158. Kim ES, Chung WC, Lee KM, Lee BI, Choi H, Han SW, et al. A case of intestinal Behçet’s disease similar to Crohn’s colitis. J Korean Med Sci. 2007;22:918–22.

    Article  PubMed  PubMed Central  Google Scholar 

  159. Naganuma M, Iwao Y, Kashiwagi K, Funakoshi S, Ishii H, Hibi T. A case of Behçet’s disease accompanied by colitis with longitudinal ulcers and granuloma. J Gastroenterol Hepatol. 2002;17(1):105–8.

    Article  PubMed  Google Scholar 

  160. Tolia V, Abdullah A, Thirumoorthi MC, Chang CH. A case of Behçet’s disease with intestinal involvement due to Crohn’s disease. Am J Gastroenterol. 1989;84(3):322–5.

    CAS  PubMed  Google Scholar 

  161. Smith GE, Kime LR, Pitcher JL. The colitis of Behçet’s disease: a separate entity? Colonoscopic findings and literature review. Am J Dig Dis. 1973;18(11):987–1000.

    Article  CAS  PubMed  Google Scholar 

  162. International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335(8697):1078–80.

    Google Scholar 

  163. Jung HC, Rhee PL, Song IS, Choi KW, Kim CY. Temporal changes in the clinical type or diagnosis of Behcet’s colitis in patients with aphthoid or punched-out colonic ulcerations. J Korean Med Sci. 1991;6:313–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  164. Erzin Y, Esatoglu SN, Hatemi I, Demir N, Dogusoy G, Erdamar S, et al. Comparative retrospective assessment of prospectively recorded endoscopic and histological findings between CD and GI-TB; the first Eastern European registry data. J Crohn’s Coli. 2014;8(Supplement 1):171.

    Article  Google Scholar 

  165. Balthazar EJ, Gordon R, Hulnick D. Ileocecal tuberculosis: CT and radiologic evaluation. AJR Am J Roentgenol. 1990;154(3):499–503.

    Article  CAS  PubMed  Google Scholar 

  166. Erzin Y, Hatemi I, Kuskucu M, Aygun G, Midilli K, Esatoglu SN, et al. What is the real value of acid-fast staining, PCR, and culture of endoscopically obtained tissue samples in diagnosis of gastrointestinal tuberculosis in patients with ileocecal inflammation? Comparative data from an Eastern European registry. J Crohn’s Coli. 2014;8:103–4.

    Article  Google Scholar 

  167. Puspok A, Keiner H, Oberhuber G. Clinical, endoscopic, and histologic spectrum of nonsteroidal anti-inflammatory drug-induced lesions in the colon. Dis Colon Rectum. 2000;43:685–91.

    Article  CAS  PubMed  Google Scholar 

  168. Laine L, Connors LG, Reicin A, Hawkey CJ, Burgos-Vagas R, Schnitzer TJ, et al. Serious lower gastrointestinal clinical events with nonselective NSAID or Coxib use. Gastroenterology. 2003;124:288–92.

    Article  CAS  PubMed  Google Scholar 

  169. Celik AF, Pamuk ON, Melikoglu M, Yazici H. How to diagnose Behçet’s and intestinal Behçet’s disease? In: Tozun N, Mantzaris DU, Schölmerich J, editors. IBD 2007-achievements in research and clinical practice. Dordrecht: Springer; 2008. p. 118–28.

    Chapter  Google Scholar 

  170. Kurahara K, Matsumoto T, Iida M, Honda K, Yao T, Fujishima M. Clinical and endoscopic features of nonsteroidal anti-inflammatory drug-induced colonic ulcerations. Am J Gastroenterol. 2001;96(2):473–80.

    Article  CAS  PubMed  Google Scholar 

  171. Xu N, Yu Z, Cao X, Wang Z, Yan M. Characteristics of nonsteroidal anti-inflammatory drugs (NSAIDs)-induced small bowel injury identified by single-balloon endoscopy or capsule endoscopy. Med Sci Monit. 2017;23:5237–45.

    Article  PubMed  PubMed Central  Google Scholar 

  172. Vavricka SR, Spigaglia SM, Rogler G, Pittet V, Michetti P, Felley C, Swiss IBD Cohort Study Group, et al. Systematic evaluation of risk factors for diagnostic delay in inflammatory bowel disease. Inflamm Bowel Dis. 2012;18(3):496–505.

    Article  PubMed  Google Scholar 

  173. Wang YF, Ou-Yang Q, Xia B, Liu LN, Gu F, Zhou KF, et al. Multicenter case-control study of the risk factors for ulcerative colitis in China. World J Gastroenterol. 2013;19(11):1827–33.

    Article  PubMed  PubMed Central  Google Scholar 

  174. Brandt LJ, Feuerstadt P, Longstreth GF, Boley SJ, American College of Gastroenterology. ACG clinical guideline: epidemiology, risk factors, patterns of presentation, diagnosis, and management of colon ischemia. Am J Gastroenterol. 2015;110(1):18–44.

    Article  PubMed  Google Scholar 

  175. Zuckerman GR, Prakash C, Merriman RB, Sawhney MS, DeSchryver-Kecskemeti K, Clouse RE. The colon single-stripe sign and its relationship to ischemic colitis. Am J Gastroenterol. 2003;98(9):2018–22.

    Article  PubMed  Google Scholar 

  176. Yilmaz M, Bilir YA, Aygün G, Erzin Y, Ozturk R, Celik AF. Prospectiveobservational study on antibiotic-associated bloody diarrhea: report of 21 cases with a long-term follow-up from Turkey. Eur J Gastroenterol Hepatol. 2012;24(6):688–94.

    Article  CAS  PubMed  Google Scholar 

  177. Esatoglu SN, Hatemi G, Salihoglu A, Hatemi I, Soysal T, Celik AF. A reappraisal of the association between Behçet’s disease, myelodysplastic syndrome and the presence of trisomy 8: a systematic literature review. Clin Exp Rheumatol. 2015;33:S145–51.

    PubMed  Google Scholar 

  178. Pallis AG, Mouzas IA, Vlachonikolis IG. The inflammatory bowel disease questionnaire: a review of its national validation studies. Inflamm Bowel Dis. 2004;10(3):261–9.

    Article  CAS  PubMed  Google Scholar 

  179. Park KD, Bang D, Lee ES, Lee SH, Lee S. Clinical study on death in Behçet’s disease. J Korean Med Sci. 1993;8(4):241–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  180. Ozguler Y, Leccese P, Christensen R, Esatoglu SN, Bang D, Bodaghi B, et al. Management of major organ involvement of Behçet’s syndrome: a systematic review for update of the EULAR recommendations. Rheumatology (Oxford). 2018; https://doi.org/10.1093/rheumatology/key242.

    Article  PubMed  Google Scholar 

  181. Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77(6):808–18.

    PubMed  Google Scholar 

  182. Chung MJ, Cheon JH, Kim SU, et al. Response rates to medical treatments and long-term clinical outcomes of nonsurgical patients with intestinal Behçet disease. J Clin Gastroenterol. 2010;44:116–22.

    Google Scholar 

  183. Yoo HM, Han KH, Kim PS, Kim WH, Kang JK, Park IS, et al. Clinical features of intestinal Behçet’s disease and therapeutic effects of sulfasalazine. Korean J Gastroenterol. 1997;29:465–72.

    Google Scholar 

  184. Houman MH, Hamzaoui K. Promising new therapies for Behçet’s disease. Eur J Intern Med. 2006;17(3):163–9.

    Article  CAS  PubMed  Google Scholar 

  185. Sonta T, Araki Y, Koubokawa M, Tamura Y, Ochiai T, Harada N, et al. The beneficial effect of mesalazine on esophageal ulcers in intestinal Behçet’s disease. J Clin Gastroenterol. 2000;30(2):195–9.

    Article  CAS  PubMed  Google Scholar 

  186. Jung YS, Hong SP, Kim TI, et al. Long-term clinical outcomes and factors predictive of relapse after 5-aminosalicylate or sulfasalazine therapy in patients with intestinal Behcet disease. J Clin Gastroenterol. 2012;46:e38–45.

    Article  CAS  PubMed  Google Scholar 

  187. Park JJ, Kim WH, Cheon JH. Outcome predictors for intestinal Behçet’s disease. Yonsei Med J. 2013;54:1084–90.

    Article  PubMed  PubMed Central  Google Scholar 

  188. Jung YS, Cheon JH, Hong SP, Kim TI, Kim WH. Clinical outcomes and prognostic factors for thiopurine maintenance therapy in patients with intestinal Behcet’s disease. Inflamm Bowel Dis. 2012;18:7507.

    Google Scholar 

  189. Hatemi I, Hatemi G, Pamuk ON, Erzin Y, Celik AF. TNF-alpha antagonists and thalidomide for the management of gastrointestinal Behçet’s syndrome refractory to the conventional treatment modalities: a case series and review of the literature. Clin Exp Rheumatol. 2015;33:S129–37.

    PubMed  Google Scholar 

  190. Iwata S, Saito K, Yamaoka K Tsujimura S, Nawata M, Hanami K, et al. Efficacy of combination therapy of anti-TNF-alpha antibody infliximab and methotrexate in refractory entero-Behçet’s disease. Mod Rheumatol. 2011;21:184–91.

    Article  CAS  PubMed  Google Scholar 

  191. Hanauer SB. Crohn’s disease: step up or top down therapy. Best Pract Res Clin Gastroenterol. 2003;17(1):131–7.

    Article  PubMed  Google Scholar 

  192. Mussack T, Landauer N, Ladurner R, Schiemann U, Goetzberger M, Burchardi C, et al. Successful treatment of cervical esophageal perforation in Behçet’s disease with drainage operation and infliximab. Am J Gastroenterol. 2003;98(3):703–4.

    Article  PubMed  Google Scholar 

  193. Ju JH, Kwok SK, Seo SH, Yoon CH, Kim HY, Park SH. Successful treatment of life-threatening intestinal ulcer in Behçet’s disease with infliximab: rapid healing of Behçet’s ulcer with infliximab. Clin Rheumatol. 2007;26(8):1383–5.

    Article  PubMed  Google Scholar 

  194. Tanida S, Inoue N, Kobayashi K, Naganuma M, Hirai F, Iizuka B, et al. Adalimumab for the treatment of Japanese patients with intestinal Behcet’s disease. Clin Gastroenterol Hepatol. 2015;13:9408 e3.

    Article  CAS  Google Scholar 

  195. Ma D, Zhang CJ, Wang RP, Wang L, Yang H. Etanercept in the treatment of intestinal Behçet’s disease. Cell Biochem Biophys. 2014;69:735–9.

    Article  CAS  PubMed  Google Scholar 

  196. 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-TNFa monoclonal antibodies. J Gastroenterol. 2014;49:156–62.

    Article  CAS  PubMed  Google Scholar 

  197. Rossi G, Moretta A, Locatelli F. Autologous hematopoietic stem cell transplantation for severe/refractory intestinal Behçet’s disease. Blood. 2004;103(2):748–50.

    Article  CAS  PubMed  Google Scholar 

  198. Yamato K. Successful cord blood stem cell transplantation for myelodysplastic syndrome with Behçet’s disease. Int J Hematol. 2003;77(1):82–5.

    Article  PubMed  Google Scholar 

  199. Soysal T, Salihoğlu A, Esatoğlu SN, Gültürk E, Eşkazan AE, Hatemi G, et al. Bone marrow transplantation for Behçet’s disease: a case report and systematic review of the literature. Rheumatology (Oxford). 2014;53(6):1136–41.

    Article  CAS  Google Scholar 

  200. Matsukawa M, Yamasaki T, Kouda T, Kurihara M. Endoscopic therapy with absolute ethanol for postoperative recurrent ulcers in intestinal Behçet’s disease, and simple ulcers. J Gastroenterol. 2001;36(4):255–8.

    Article  CAS  PubMed  Google Scholar 

  201. Sayek I, Aran O, Uzunalimoglu B, Hersek E. Intestinal Behçet’s disease: surgical experience in seven cases. Hepato-Gastroenterology. 1991;38(1):81–3.

    CAS  PubMed  Google Scholar 

  202. Lida M, Kobayayashi H, Matsumoto T, Okada M, Fuchigami T, Yao T, et al. Postoperative recurrence in patients with intestinal Behçet’s disease. Dis Colon Rectum. 1994;37(1):16–21.

    Article  Google Scholar 

  203. Bozkurt M, Torin G, Aksakal B, Ataoglu O. Behçet’s disease and surgical intervention. Int J Dermatol. 1992;31(8):571–3.

    Article  CAS  PubMed  Google Scholar 

  204. Bradbury AW, Milne AA, Murie JA. Surgical aspects of Behçet’s disease. Br J Surg. 1994;81(12):1712–21.

    Article  CAS  PubMed  Google Scholar 

  205. Park YE, Cheon JH, Park J, Lee JH, Lee HJ, Park SJ, et al. The outcomes and risk factors of early reoperation after initial intestinal resective surgery in patients with intestinal Behçet’s disease. Int J Color Dis. 2017;32(4):591–4.

    Article  Google Scholar 

  206. Park JW, Park Y, Park SJ, Kim TI, Kim WH, Cheon JH. Development of a novel endoscopic scoring system to predict relapse after surgery in intestinal Behçet’s disease. Gut Liver. 2018; https://doi.org/10.5009/gnl17547.

    Article  PubMed  PubMed Central  Google Scholar 

  207. Lee HW, Cheon JH, Lee HJ, Park SJ, Hong SP, Kim TI, Postoperative Effects of Thiopurines in Patients with Intestinal Behçet’s Disease, et al. Dig Dis Sci. 2015;60(12):3721–7.

    Article  CAS  PubMed  Google Scholar 

  208. Park YE, Cheon JH, Park J, Lee JH, Lee HJ, Park SJ, Kim TI, Kim WH, et al. The outcomes and risk factors of early reoperation after initial intestinal respective surgery in patients with intestinal Behçet’s disease. Int J Color Dis. 2017;32:591–4.

    Article  Google Scholar 

  209. Mosli MH, Zou G, Garg SK, Feagan SG, MacDonald JK, Chande N, et al. C-Reactive protein, fecal calprotectin, and stool lactoferrin for detection of endoscopic activity in symptomatic inflammatory bowel disease patients: a systematic review and meta-analysis. Am J Gastroenterol. 2015;110:802–19.

    Article  PubMed  Google Scholar 

  210. Kim DH, Park Y, Kim B, Kim SW, Park SJ, Hong SP, et al. Fecal calprotectin as a non-invasive biomarker for intestinal involvement of Behçet’s disease. J Gastroenterol Hepatol. 2017;32(3):595–601.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea

    Jae Hee Cheon

  2. Division of Gastroenterology, Department of Internal Medicine, University of Istanbul-Cerrahpasa, Cerrahpasa School of Medicine, Istanbul, Turkey

    Ibrahim Hatemi & Aykut Ferhat Çelik

Authors
  1. Jae Hee Cheon
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  2. Ibrahim Hatemi
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  3. Aykut Ferhat Çelik
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Correspondence to Aykut Ferhat Çelik .

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Editors and Affiliations

  1. School of Medicine, New York University, New York, NY, USA

    Yusuf Yazici

  2. Department of Internal Medicine, Istanbul University, Istanbul, Turkey

    Gulen Hatemi

  3. Department of Rheumatology, Istanbul University, Istanbul, Turkey

    Emire Seyahi

  4. Department of Medicine, Academic Hospital, Istanbul, Turkey

    Hasan Yazici

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Cheon, J.H., Hatemi, I., Çelik, A.F. (2020). Behçet Syndrome: Gastrointestinal Involvement. In: Yazici, Y., Hatemi, G., Seyahi, E., Yazici, H. (eds) Behçet Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-24131-5_9

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  • DOI: https://doi.org/10.1007/978-3-030-24131-5_9

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-24130-8

  • Online ISBN: 978-3-030-24131-5

  • eBook Packages: MedicineMedicine (R0)

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