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. 2020 Jul;55(7):679-700.
doi: 10.1007/s00535-020-01690-y. Epub 2020 May 7.

Evidence-based diagnosis and clinical practice guidelines for intestinal Behçet's disease 2020 edited by Intractable Diseases, the Health and Labour Sciences Research Grants

Kenji Watanabe  1 , Satoshi Tanida  2 , Nagamu Inoue  3 , Reiko Kunisaki  4 , Kiyonori Kobayashi  5 , Masakazu Nagahori  6 , Katsuhiro Arai  7 , Motoi Uchino  8 , Kazutaka Koganei  9 , Taku Kobayashi  10 , Mitsuhiro Takeno  11 , Fumiaki Ueno  12 , Takayuki Matsumoto  13 , Nobuhisa Mizuki  14 , Yasuo Suzuki  15 , Tadakazu Hisamatsu  16
Affiliations

Evidence-based diagnosis and clinical practice guidelines for intestinal Behçet's disease 2020 edited by Intractable Diseases, the Health and Labour Sciences Research Grants

Kenji Watanabe et al. J Gastroenterol. 2020 Jul.

Abstract

Behçet's disease (BD) is an intractable systemic inflammatory disease characterized by four main symptoms: oral and genital ulcers and ocular and cutaneous involvement. The Japanese diagnostic criteria of BD classify intestinal BD as a specific disease type. Volcano-shaped ulcers in the ileocecum are a typical finding of intestinal BD, and punched-out ulcers can be observed in the intestine or esophagus. Tumor necrosis factor inhibitors were first approved for the treatment of intestinal BD in Japan and have been used as standard therapy. In 2007 and 2014, the Japan consensus statement for the diagnosis and management of intestinal BD was established. Recently, evidence-based JSBD (Japanese Society for BD) Clinical Practice Guidelines for BD (Japanese edition) were published, and the section on intestinal BD was planned to be published in English. Twenty-eight important clinical questions (CQs) for diagnosis (CQs 1-6), prognosis (CQ 7), monitoring and treatment goals (CQs 8-11), medical management and general statement (CQs 12-13), medical treatment (CQs 14-22), and surgical treatment (CQs 23-25) of BD and some specific situations (CQs 26-28) were selected as unified consensus by the members of committee. The statements and comments were made following a search of published scientific evidence. Subsequently, the levels of recommendation were evaluated based on clinical practice guidelines in the Medical Information Network Distribution Service. The degree of agreement was calculated using anonymous voting. We also determined algorithms for diagnostic and therapeutic approaches for intestinal BD. The present guidelines will facilitate decision making in clinical practice.

Keywords: Behçet’s disease; Consensus; Evidence; Guideline; Intestinal behçet’s disease.

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Conflict of interest statement

The authors have the following financial conflicts of interest regarding this manuscript. KW: advisory role for Takeda Pharmaceutical Corporation (TPC), commercial research funding from EA Pharma Corporation (EPC), Abbvie GK (AGK), Mitsubishi Tanabe Pharma (MTP), Kyorin Pharmaceutical Corporation (KPC) and JIMRO Corporation (JC), honoraria from EPC, AGK, MTP, KPC, JC, Mochida Pharmaceutical Corporation (MPC), Janssen Pharmaceutical (JP), TPC and Zeria Pharmaceutical Corporation (ZPO); ST: commercial research funding from EPC and AGK; AK: honoraria from MTP; TK: advisory role for AGK, Eli Lilly, JP, TPC and Robarts Clinical Trials, commercial research funding from EPC, Thermo Fisher Scientific, Alfresa Pharma (AP), Nippon Kayaku Co. Ltd. (NK) and Asahi Kasei Medical (AKM), honoraria from AGK, AP, EPC, JP, JC, NK, MPC, Pfizer Japan Inc. (PJI), TPC, MTP and ZPO; YS: commercial research funding from AGK, MTP, JC, MPC, Kissei Pharmaceutical Corporation and NK. honoraria from AGK, MTP, ZPO, MPC, KPC, JP and EPC; TH: commercial research funding from EPC, MTP, AGK, Daiichi-Sankyo Co. Ltd., TPC, PJI, MPC, NK, KPC, JC, Astellas Pharma Inc., AKM, ZPO and Otsuka Pharmaceutical Co. Ltd, honoraria from EPC, AGK, Celgene K.K., JP, PJI, TPC, MTP, KPC, JC, MPC and Nichi-lko Pharmaceutical Co., Ltd.

Figures

Fig. 1
Fig. 1
Typical ileocecal ulcer in a patient with Behçet’s disease
Fig. 2
Fig. 2
Algorithm for a definite diagnosis of intestinal BD. BD Behçet’s disease, NSAIDs non-steroidal anti-inflammatory drugs
Fig. 3
Fig. 3
Algorithm for the treatment of intestinal BD. 5-ASA 5-aminosalicylic acid, 6-MP 6-mercaptopurine, AZA azathioprine, MTX methotrexate, TNF tumor necrosis factor, TPN total parenteral nutrition
See this image and copyright information in PMC

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