Diagnosis and management of intestinal Behçet's disease

Abstract

Behçet's disease (BD) is a chronic relapsing disease with multiple organ system involvement characterized clinically by oral and genital aphthae, cutaneous lesions, and ophthalmological, neurological, and/or gastrointestinal manifestations. Little clinical evidence is available regarding the management of patients with intestinal BD, despite recognition that the presence of intestinal lesions is a poor prognostic factor, causing perforation and massive bleeding. Many recent case reports have suggested that anti-tumor necrosis factor alpha (TNF)α monoclonal antibodies (mAbs) are effective in patients with intestinal BD. Adalimumab, a fully human anti-TNFα mAb, has been approved in Japan for the treatment of intestinal BD. Here, we review the pathogenesis, diagnosis and management of intestinal BD, including evidence of the efficacy of anti-TNFα mAbs.

Keywords: Adalimumab; Anti-TNFα mAb; Intestinal Behçet’s disease; Trisomy 8.

Fig. 1

Gastrointestinal lesions in BD. a A typical giant oval-shaped deep punched-out ulcer in the ileocecal area. b An atypical oval-shaped ulcer in the middle part of the esophagus in a patient with intestinal BD. c A discrete ulcer in the small intestine detected by capsule endoscopy in a MDS patient associated with trisomy 8

Fig. 2

Differential diagnosis of intestinal BD. a Annular ulcers in patients with active TB. b Longitudinal ulcers and a cobblestone appearance in a patient with CD

Fig. 3

IFX treatment can induce ‘mucosal healing’ of intestinal BD lesions. a A typical giant oval-shaped ulcer observed before initiation of IFX. b Dramatic improvement of ileocecal lesions after treatment with IFX (the patient was reported by Maruyama et al. [72])