Differential Diagnosis: Intestinal Behçet’s Disease

Abstract

Behçet’s disease (BD) is a chronic relapsing multisystemic vasculitic disorder characterized by recurrent oral and genital ulcers, ocular lesions, skin manifestations, arthritis, as well as vascular, neurologic, and intestinal involvement. The prevalence of BD is higher in the Middle and East Asia than in Western countries. Intestinal BD is a specific subtype of BD, characterized by intestinal ulcers and associated gastrointestinal symptoms. The frequency of GI involvement varies depending on geographic location, ranging from 3 to 50 %. Symptomatic or documented intestinal involvement is rare in Mediterranean patients with BD, whereas it is common in East Asian countries including Korea and Japan. The most frequently affected site is the ileocecal region, and common clinical symptoms include abdominal pain, diarrhea, and bleeding. Although the diagnosis of systemic BD is usually made according to the criteria suggested by the Behçet’s Disease Research Committee of Japan or the International Study Group for Behçet’s Disease, adequate diagnosis of intestinal BD using these criteria is limited due to various extraintestinal manifestations that emerge at different time points throughout the disease course. Therefore, novel diagnostic criteria for intestinal BD were recently proposed, which may be particularly useful in patients with ileocolonic ulcers that do not fully satisfy the diagnostic criteria for systemic BD (Fig. 3.1). Histologic findings of intestinal BD are generally nonspecific, showing lymphocytic or neutrophilic infiltration rather than vasculitis (Fig. 3.2).