Gastrointestinal Behçet's disease: a review

Abstract

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 disease; Behçet syndrome; Inflammatory bowel disease; Lower gastrointestinal tract; Ulcer; Upper gastrointestinal tract.

Figure 1

Algorithm for diagnosis of intestinal Behçet’s disease. ¹Typical: < 5 ulcers, oval in shape, deep, discrete borders located in ileocecal region; ²Atypical: Ulcerations that do not satisfy all criteria for typical. Adapted from Cheon et al[88]. BD: Behçet’s disease.