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      Updated treatment strategies for intestinal Behçet’s disease

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          Abstract

          Behçet’s disease (BD) is a chronic, idiopathic, relapsing immune-mediated disease involving multiple organs, and is characterized by recurrent oral and genital ulcers, ocular disease, gastrointestinal ulcers, vascular diseases, and skin lesions. In particular, gastrointestinal involvement in BD is followed by severe complications, including massive bleeding, bowel perforation, and fistula, which can lead to significant morbidity and mortality. However, the management of intestinal BD has not yet been properly established. Intestinal BD patients with a severe clinical course experience frequent disease aggravations and often require recurrent corticosteroid and/or immunomodulatory therapies, or even surgery. However, a considerable number of patients with intestinal BD are often refractory to conventional therapies such as corticosteroids and immunomodulators. Recently, there has been a line of evidence suggesting that biologics such as infliximab and adalimumab are effective in treating intestinal BD. Moreover, new biologics targeting proteins other than tumor necrosis factor α are emerging and are under active investigation. Therefore, in this paper, we review the current therapeutic strategies and new clinical data for the treatment of intestinal BD.

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          Most cited references109

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          Behçet's disease.

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            Virus interference. I. The interferon.

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              Mechanisms of action of cyclosporine.

              Cyclosporine (cyclosporin A, CsA) has potent immunosuppressive properties, reflecting its ability to block the transcription of cytokine genes in activated T cells. It is well established that CsA through formation of a complex with cyclophilin inhibits the phosphatase activity of calcineurin, which regulates nuclear translocation and subsequent activation of NFAT transcription factors. In addition to the calcineurin/NFAT pathway, recent studies indicate that CsA also blocks the activation of JNK and p38 signaling pathways triggered by antigen recognition, making CsA a highly specific inhibitor of T cell activation. Here we discuss the action of CsA on JNK and p38 activation pathways. We also argue the potential of CsA and its natural counterparts as pharmacological probes.
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                Author and article information

                Journal
                Korean J Intern Med
                Korean J. Intern. Med
                KJIM
                The Korean Journal of Internal Medicine
                The Korean Association of Internal Medicine
                1226-3303
                2005-6648
                January 2018
                8 December 2017
                : 33
                : 1
                : 1-19
                Affiliations
                [1 ]Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
                [2 ]Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
                Author notes
                Correspondence to Jae Hee Cheon, M.D. Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Korea Tel: +82-2-2228-1990 Fax: +82-2-393-6884 E-mail: geniushee@ 123456yuhs.ac

                This paper was contributed by Korean Association for the Study of Intestinal Diseases.

                Article
                kjim-2017-377
                10.3904/kjim.2017.377
                5768550
                29207867
                9cc8cac9-069b-49f9-923e-c129a3628180
                Copyright © 2018 The Korean Association of Internal Medicine

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 17 November 2017
                : 20 November 2017
                Categories
                Review

                Internal medicine
                intestinal behcet disease,biological products,tumor necrosis factor-alpha,infliximab,adalimumab

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