J Rheum Dis.  2021 Jan;28(1):4-16. 10.4078/jrd.2021.28.1.4.

Advances in Management of Intestinal Behçet’s Disease: A Perspective From Gastroenterologists

• Cheon JH

• KMID: 2510337
• DOI: http://doi.org/10.4078/jrd.2021.28.1.4

Abstract

Intestinal Behçet’s disease (intestinal BD) is a rare chronic inflammatory disorder of the intestine that is characterized by recurrent intestinal manifestations with other systemic features of BD. Intestinal BD is diagnosed when a typically shaped ulcer is observed in the gastrointestinal tract, and the clinical findings meet the diagnostic criteria for BD. Owing to the small number of patients, intestinal BD is easily underestimated. On the other hand, but it often requires surgical treatment because of severe complications, including intestinal perforations or massive bleeding. The same treatment strategies used for inflammatory bowel diseases, such as Crohn’s disease and ulcerative colitis, are used for intestinal BD. 5-Aminosalicylic acids, corticosteroids, and immunomodulators are considered conventional therapies, but a considerable number of patients eventually become unresponsive to these pharmaceutical treatments. Recently, biologic agents, such as anti-tumor necrosis factor-alpha inhibitors, have also been suggested as a new treatment option for intestinal BD. This article reviews the pathogenesis and diagnosis of intestinal BD and the current treatment strategies that are expected to be useful for rheumatologic specialists.

Keyword

Behçet’s disease; Intestinal Behçet’s disease; Therapeutic strategy; Anti-TNF-α inhibitor

Figure

• 

  Figure 1 Diagnosis of intestinal Behçet’s disease (BD) (adapted from the article of Cheon et al. Am J Gastroenterol 2009;104:2492-9 [10]). *Single or few deep oval-shaped ulcers with discrete margins in the ileocecal area, †aphthous, shallow ulcers.

• 

  Figure 2 Treatment algorithm based on the disease severity (adapted from the article of Lee et al. Intest Res 2013;11:155-60 [39]). DAIBD: Disease Activity Index of intestinal Behçet’s disease, 5-ASA: 5-Aminosalicylic acids, AZA: azathioprine, 6-MP: 6-mercaptopurine, MTX: methotrexate, IMM: immunomodulator.

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