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      The 2nd edition of consensus statements for the diagnosis and management of intestinal Behçet’s disease: indication of anti-TNFα monoclonal antibodies

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          Abstract

          Background

          Clinical evidence regarding intestinal Behçet’s disease (BD) management is lacking and intestinal lesions are a poor prognostic factor. In 2007, the Japan consensus statement for diagnosis and management of intestinal BD was developed. Recently, the efficacy of anti-tumor necrosis factor (TNF)α monoclonal antibodies (mAbs), and infliximab (IFX) was reported and adalimumab (ADA) was approved for intestinal BD in Japan. This study renewed consensus-based practice guidelines for diagnosis and treatment of intestinal BD focusing on the indication of anti-TNFα mAbs.

          Methods

          An expert panel of Japanese gastroenterology and rheumatology specialists was involved. Clinical statements for ratings were extracted from the literature, a professional group survey, and by an expert panel discussion, which rated clinical statements on a nine-point scale. After the first round of ratings, a panelist meeting discussed areas of disagreement and clarified areas of uncertainty. The list of clinical statements was revised after the panelist meeting and a second round of ratings was conducted.

          Results

          Fifteen relevant articles were selected. Based on the first edition consensus statement, improved clinical statements regarding indications for anti-TNFα mAbs use were developed. After a two-round modified Delphi approach, the second edition of consensus statements was finalized.

          Conclusions

          In addition to standard therapies in the first edition, anti-TNFα mAbs (ADA and IFX) should be considered as a standard therapy for intestinal BD. Colchicines, thalidomide, other pharmacological therapy, endoscopic therapy, and leukocytapheresis were deemed experimental therapies.

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          Most cited references19

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          Behçet's disease.

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            Efficacy of infliximab for induction and maintenance of remission in intestinal Behçet's disease.

            Intestinal Behçet disease (BD) is characterized by intestinal inflammation with round and oval ulcers associated with gastrointestinal symptoms. Although several cases have been reported that infliximab is effective for induction of remission, the efficacy of infliximab for maintaining remission is unknown. Six cases with fulminant intestinal BD were treated with infliximab. All patients were steroid-dependent and refractory to immunosuppressants; 3 patients were treated with 6-mercaptopurine, 1 patient with azathioprine, 1 patient with cyclosporine A, and 1 patient with methotrexate. Four patients achieved remission by infliximab and all of these patients maintained remission with scheduled treatments of infliximab, with the longest duration of remission being about 3 years. Another 2 patients with ileal ulceration required surgery; however, 1 patient has maintained remission by scheduled treatment of infliximab for 2 years after surgery. Infliximab appears to offer an option for fulminant intestinal BD to induce and maintain remission, although a randomized control trial is needed.
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              Anti-tumor necrosis factor monoclonal antibody therapy for gastrointestinal Behçet's disease: a case report.

              Behçet's disease (BD) is a multisystem immune-mediated inflammatory disorder that involves the intestine in 3%-26% of cases. Corticosteroids, 5-aminosalicylic acid derivatives, immunomodulators, and more recently thalidomide and pentoxifylline have been used to treat BD with varying degrees of success. Tumor necrosis factor (TNF)-alpha is believed to play a pivotal role in this T helper cell type 1 (Th1)-mediated disease. Infliximab, a chimeric monoclonal antibody to TNF-alpha, has been demonstrated to be an effective therapy for Crohn's disease and rheumatoid arthritis, 2 other Th1-mediated disorders. We describe a patient with chronically active, steroid-dependent BD involving the gastrointestinal tract who received 4 doses of infliximab during a 6-month period. Because most of her symptoms were gastrointestinal, the Crohn's Disease Activity Index (CDAI) was used to assess response. A rapid and dramatic improvement in both gastrointestinal and extraintestinal symptoms was observed. The CDAI score decreased from 270 points (preinfusion) to 13 points by week 2, and remission was sustained despite complete withdrawal of steroids. Colonoscopy performed 10 weeks after the first infusion showed marked endoscopic and histologic improvement. This report suggests that infliximab may be an effective new therapy for gastrointestinal BD, and perhaps other manifestations of BD as well.
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                Author and article information

                Contributors
                +81-3-33531211 , +81-3-33572778 , hisamachi@a7.keio.jp
                Journal
                J Gastroenterol
                J. Gastroenterol
                Journal of Gastroenterology
                Springer Japan (Tokyo )
                0944-1174
                1435-5922
                18 August 2013
                18 August 2013
                2014
                : 49
                : 156-162
                Affiliations
                [ ]Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582 Japan
                [ ]Center for Digestive and Liver Diseases, Ohfuna Chuo Hospital, Kamakura, Japan
                [ ]Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
                [ ]Department of Gastroenterology, Kitasato University East Hospital, Sagamihara, Japan
                [ ]Department of Surgery, Yokohama Municipal Citizen’s Hospital, Yokohama, Japan
                [ ]Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
                [ ]Department of Gastroenterology, Fukuoka University Chikushi Hospital, Chikushino, Japan
                [ ]Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University, Tokyo, Japan
                [ ]Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
                [ ]Division of Allergy and Rheumatology, St. Luke’s International Hospital, Tokyo, Japan
                [ ]Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Yokohama, Japan
                [ ]Department of Pathology and Laboratory Medicine, Hirosaki City Hospital, Hirosaki, Japan
                [ ]Center for Preventive Medicine, School of Medicine, Keio University, Tokyo, Japan
                [ ]Center for Advanced IBD Research and Treatment, Kitasato University Kitasato Institute Hospital, Tokyo, Japan
                Article
                872
                10.1007/s00535-013-0872-4
                3895195
                23955155
                21aac73d-9b4f-49a8-8536-52bfc180453d
                © The Author(s) 2013

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.

                History
                : 4 July 2013
                : 6 August 2013
                Categories
                Rapid Communication
                Custom metadata
                © Springer Japan 2014

                Gastroenterology & Hepatology
                intestinal behçet’s disease,consensus statements,anti-tnfα mab

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