Intestinal Behçetʼs Disease Does Not Follow the Silk Road

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Introduction: Behçet's disease (BD) is a chronic multi-system inflammatory disease with an unknown etiology and carries a high morbidity. It is most prevalent in Eastern Asia and along the Mediterranean basin, an area referred to as “The Silk Road.” The diagnosis of BD is largely based on the International Study Group (ISG) criteria which are more specific than sensitive. ISG criteria do not include intestinal manifestations, a feature more commonly seen in the West. Pathophysiology of BD is attributed to an environmental trigger in a genetically susceptible host, and likely explains the different manifestations seen here in the West. Intestinal BD is one of several findings that are not typically seen along “The Silk Road”. Herein we report a rare case of Intestinal BD and compare Western and Traditional BD. Case Report: A 25-year-old male with a history of painful oral apthous ulcers, pericarditis and diffuse papulopustular rash presented to the ED with two terminal ileal perforations (Figure 1). Pathology demonstrated mucosal necrosis with active inflammation and no chronic inflammatory changes. Post-surgical laboratory studies showed an elevated CRP of 35.57 mg/dL, ESR of 82mm/h, and a positive anti-Saccharomyces cerevisiae antibody. Rhematological workup including: ANA, RF, PR3 antibody, MPO antibody, ANCA, SSA, SSB, Smith antibody, SCL-70 and anti-Jo-1 antibodies were all negative. Pathergy test was negative. His pericarditis symptoms improved with colchicine and prednisone prior to discharge.Figure 1Discussion: Our patient did not meet the current ISG criteria for traditional BD, however, he clearly showed findings typically seen in western patients with BD which include intestinal manifestions, cardiac involvment and lack of pathergy reaction and ocular changes. Our investigation demonstrates that the clinical manifestations common to this disorder vary among geographic and ethnic populations (Table 1). Commonly used criteria for the diagnosis of BD may not be sensitive for some populations such as Western BD, potentially leading to underdiagnoses and mismanagement. Recognition and select inclusion of these differences may be one way to assist with diagnosing Western BD in the future. As our knowledge of BD continues to evolve, so must the population-specific criteria used to define BD.Table 1: Prevelance of Clincial Features in Western and Traditional Behçet's Disaease