Behçet Syndrome: Gastrointestinal Involvement

  • Jae Hee Cheon
  • Ibrahim Hatemi
  • Aykut Ferhat ÇelikEmail author


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.


Anastomotic site ulcers Azathioprine Behçet’s disease Crohn’s disease Diagnostic criteria for GIBD Enteroclysis Esophageal disease Fistulas Genetic Environment Gastrointestinal involvement Ileocecal disease Inflammatory bowel disease Intestinal pathology Intestinal ulcers NSAID enterocolitis Intestinal tuberculosis Myelodysplastic syndrome Prognosis Radiology Rectal involvement Recto-sigmoidoscopy Smoking Stomach involvement Surgical management Top-down therapy 


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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Jae Hee Cheon
    • 1
  • Ibrahim Hatemi
    • 2
  • Aykut Ferhat Çelik
    • 2
    Email author
  1. 1.Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of MedicineSeoulSouth Korea
  2. 2.Division of Gastroenterology, Department of Internal Medicine, University of Istanbul-Cerrahpasa, Cerrahpasa School of MedicineIstanbulTurkey

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