S-129 1 Division of Gastroenterology, and 2 Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Turkey; 3 Division of Rheumatology, Department of Internal Medicine, Trakya University Medical School, Edirne, Turkey. Ibrahim Hatemi, MD , PhD Gulen Hatemi, MD, PhD Omer Nuri Pamuk, MD, PhD Yusuf Erzin, MD, PhD Aykut Ferhat Celik, MD, PhD Please address correspondence to: Ibrahim Hatemi, Sakayik sokak, Zeynep Cemal ap. 11/19, Tesvikiye, 34365 Istanbul, Turkey. E-mail: ihatemi@yahoo.com Received on May 1, 2015; accepted in revised form on August 31, 2015. Clin Exp Rheumatol 2015; 33 (Suppl. 94): S129-S137. © Copyright CliniCal and ExpErimEntal rhEumatology 2015. Key words: Behçet’s disease, Behçet’s syndrome, management, intestinal involvement, infammatory bowel disease, infiximab, etanercept, adalimumab, thalidomide Competing interests: none declared. ABSTRACT Objective. Gastrointestinal involvement of Behçet’s syndrome is usually treated with glucocorticoids, 5-aminosalicyl- ic acid compounds and azathioprine. However, some patients are refractory to these conventional therapy modali- ties. In this paper we report our expe- rience on 13 patients with gastrointes- tinal involvement of Behçet’s syndrome who were refractory to the conventional therapy and who were treated with TNF- alpha antagonists and/or thalidomide. Methods. We reviewed the charts of our Behçet’s syndrome patients with gastrointestinal involvement and iden- tifed those who were treated with TNF- alpha antagonists and/or thalidomide. Demographic features, previous and concomitant drugs, previous surgery, time to remission and duration of remis- sion were tabulated. We also performed a systematic review of publications on gastrointestinal involvement of Behçet’s syndrome patients treated with TNF- alpha antagonists and/or thalidomide. Results. Among our 64 patients with gastrointestinal involvement of Behçet’s syndrome, we identifed 13 (20%) (7 women, 6 men, mean age 27.4 ± 9.4) who had been treated with TNF-alpha antagonists and/or thalidomide. Their previous medications were glucocor- ticoids (13/13), azathioprine (13/13), 5-aminosalicylic acid derivatives (3/13) and budesonide (1/13). Clinical and endoscopic remission was obtained in 10 patients. One patient died with sepsis. The systematic literature search revealed 91 cases who had used TNF- alpha antagonists and 15 who had used thalidomide. Among the patients who had received TNF-alpha antagonists, clinical remission was obtained in 47/91 patients (51%), while endoscopic remission was observed in 21/46 (45%) who had a control colonoscopy. Conclusion. One ffth of our Behçet’s syndrome patients with gastrointestinal involvement were refractory to conven- tional treatment modalities. Remission was obtained with TNF-alpha antago- nists and/or thalidomide in about 75 % of the cases. Introduction Gastrointestinal involvement of Be- hçet’s syndrome (GIBS) is character- ised by gastrointestinal ulcers that are located mainly in the ileocecal region. These ulcers can also be located in the other colonic segments, small intes- tine, oesophagus or duodenum. GIBS may cause abdominal pain, diarrhoea, fever or weight loss. The main compli- cations are perforation, bleeding and fstula formation (1). Surgical interven- tions can be necessary in some patients due to acute complications such as acute abdomen or bleeding and in oth- ers due to persistent symptoms despite medical therapy (2). The prevalence of GIBS shows differences depending on the geographic distribution. It is more frequent in the Far East including Ja- pan and Korea and the United States, compared to the Middle East and Eu- rope (3). This difference may be related to several factors such as genetic and environmental factors, as well as the diagnostic methods and criteria used (1). The management of GIBS is simi- lar to infammatory bowel diseases in terms of the medications used. The conventional treatment modalities are glucocorticoids, azathioprine, salazo- pyrine and other 5-aminosalicylic acid (5-ASA) derivatives (4). Some patients with GIBS can be refractory to these agents, necessitating other medical op- tions. TNF-alpha antagonists and tha- lidomide are the main options in such cases. TNF-alpha antagonists have been used with increasing frequency TNF-alpha antagonists and thalidomide for the management of gastrointestinal Behçet’s syndrome refractory to the conventional treatment modalities: a case series and review of the literature I. Hatemi 1 , G. Hatemi 2 , O.N. Pamuk 3 , Y. Erzin 1 , A.F. Celik 1