Adamantiades-Behcet's disease (ABD) is a chronic,relapsing,systemic vasculitis of unknown etiology.It is more prevalent in populations along the ancient Silk Road from Eastern Asia to the Mediterranean Basin,and most frequently affects young adults between the second and fourth decades of life.ABD-complicated gastroenteropathy is a significant cause of morbidity and mortality,with abdominal pain as the most common symptom.The ileocecal region is affected predominantly,with ulcerations that may lead to penetration and/or perforation,whereas other parts of the gastrointestinal system including the esophagus and stomach can also be affected.Endoscopy is useful to locate the site and extent of the lesions,and tissue biopsy is often warranted to examine the histopathology that is often suggestive of underlying vasculitis of small veins/venules or,alternatively in some cases,nonspecific inflammation.Bowel wall thickening is the most common finding on computed tomography scan.Treatment is largely empirical since well-controlled studies are difficult to conduct due to the heterogeneity of the disease,and the unpredictable course with exacerbation and remission.Corticosteroids with or without other immunosuppressive drugs,such as cyclophosphamide,azathioprine,sulfasalazine,tumor necrosis factor α antagonist or thalidomide should be applied before surgery,except in emergency.
Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China
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