Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Apr 7, 2015; 21(13): 3801-3812
Published online Apr 7, 2015. doi: 10.3748/wjg.v21.i13.3801
Gastrointestinal Behçet's disease: A review
Wasseem Skef, Matthew J Hamilton, Thurayya Arayssi
Wasseem Skef, Department of Medicine, St. Elizabeth’s Medical Center, Boston, MA 02135, United States
Matthew J Hamilton, Division of Gastroenterology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA 02115, United States
Thurayya Arayssi, Weill Cornell Medical College in Qatar, Doha 24144, Qatar
Author contributions: Skef W performed the literature review and wrote the paper; Hamilton MJ and Arayssi T provided expertise and critical revision of the manuscript.
Conflict-of-interest: The authors report no conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Dr. Thurayya Arayssi, Weill Cornell Medical College in Qatar, Doha 24144, Qatar.
Telephone: +974-44-928329 Fax: +974-44-928377
Received: November 15, 2014
Peer-review started: November 17, 2014
First decision: December 26, 2014
Revised: January 14, 2015
Accepted: January 30, 2015
Article in press: January 30, 2015
Published online: April 7, 2015

Behçet’s disease (BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and skin lesions. Prevalence of BD is highest in countries along the ancient silk road from the Mediterranean basin to East Asia. By comparison, the prevalence in North American and Northern European countries is low. Gastrointestinal manifestations of Behçet’s disease are of particular importance as they are associated with significant morbidity and mortality. Although ileocecal involvement is most commonly described, BD may involve any segment of the intestinal tract as well as the various organs within the gastrointestinal system. Diagnosis is based on clinical criteria - there are no pathognomonic laboratory tests. Methods for monitoring disease activity on therapy are available but imperfect. Evidence-based treatment strategies are lacking. Different classes of medications have been successfully used for the treatment of intestinal BD which include 5-aminosalicylic acid, corticosteroids, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody therapy. Like inflammatory bowel disease, surgery is reserved for those who are resistant to medical therapy. A subset of patients have a poor disease course. Accurate methods to detect these patients and the optimal strategy for their treatment are not known at this time.

Keywords: Behçet syndrome, Behçet disease, Upper gastrointestinal tract, Inflammatory bowel disease, Lower gastrointestinal tract, Ulcer

Core tip: Behçet’s disease is an uncommon subtype of inflammatory bowel disease. It can present with a wide array of clinical manifestations that may mimic other diseases including Crohn’s disease. Establishing the diagnosis remains a challenge and clinicians must be aware of the relevant clinical manifestations and diagnostic considerations. The optimal medical management is limited by the lack of rigorous clinical trial data.