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.

A case of intestinal Behcet’s disease under Adalimumab

“…[1] Endoscopic evaluation demonstrates characteristic findings of which can be discrete lesions, large (> 1 cm), and single to few in number. [1] Presence of typical lesions with systemic BD has been shown to be 100% specific for intestinal BD based on diagnostic criteria proposed by Cheon et al [2] Treatment of intestinal BD is similar to that of inflammatory bowel diseases. Sulfasalazine and 5-aminosalicyclic acid have been the mainstay of therapy.…”

“…Sulfasalazine and 5-aminosalicyclic acid have been the mainstay of therapy. [1] Treatment in the acute setting of intestinal BD flares is primarily corticosteroid therapy with additional steroid sparing medications such as azathioprine. [4] Since the advent of TNFα inhibitor therapy, these medications have been used to control moderate to severe intestinal BD.…”

“…The most common presenting symptoms are abdominal pain, nausea, vomiting, and gastrointestinal bleeding. [1] Intestinal involvement of BD usually appears within 4-6 years following the onset of oral ulcerative lesions. [5] Overall, BD can present at any location in the gastrointestinal tract.…”

“…BD is most common in native populations of Eurasia and East Asia while gastrointestinal manifestations of BD are more prevalent among East Asian populations. [1,2] Anti-TNF (Anti-tumor necrosis factor) agents are increasingly being used for severe manifestations of BD. [3] …”

Microbial Infections as an Etiology of Behcet’s Disease

“…The prevalence of BD in Turkey is particularly high at 80-420 ⁄ 100,000 (Azizlerli et al, 2003). In Egypt the prevalence is at 7.6/100,000 Skef et al, 2015). BD starts in the third and fourth decade of life and runs a more severe course in men and in those with a younger age of onset (Gül, 2005).…”