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Liu Y, Gao F, Yang DQ, Jiao Y. Intestinal Behçet's disease: A review of clinical diagnosis and treatment. World J Gastrointest Surg 2024; 16:1493-1500. [PMID: 38983357 PMCID: PMC11230016 DOI: 10.4240/wjgs.v16.i6.1493] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/15/2024] [Revised: 04/13/2024] [Accepted: 04/26/2024] [Indexed: 06/27/2024] Open
Abstract
Behçet's disease (BD) is a chronic inflammatory disorder prone to frequent recurrences, with a high predilection for intestinal involvement. However, the efficacy and long-term effects of surgical treatment for intestinal BD are unknown. In the current issue of World J Gastrointest Surg, Park et al conducted a retrospective analysis of 31 patients with intestinal BD who received surgical treatment. They found that elevated C-reactive protein levels and emergency surgery were poor prognostic factors for postoperative recurrence, emphasizing the adverse impact of severe inflammation on the prognosis of patients with intestinal BD. This work has clinical significance for evaluating the postoperative condition of intestinal BD. The editorial attempts to summarize the clinical diagnosis and treatment of intestinal BD, focusing on the impact of adverse factors on surgical outcomes. We hope this review will facilitate more precise postoperative management of patients with intestinal BD by clinicians.
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Affiliation(s)
- Ying Liu
- Department of General Surgery, The Affiliated Hospital to Changchun University of Chinese Medicine, Changchun 130021, Jilin Province, China
| | - Feng Gao
- Department of General Surgery, The Affiliated Hospital to Changchun University of Chinese Medicine, Changchun 130021, Jilin Province, China
| | - Ding-Quan Yang
- Department of Gastrointestinal and Colorectal Surgery, China-Japan Union Hospital of Jilin University, Changchun 130033, Jilin Province, China
| | - Yan Jiao
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
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Chang JY, Park SJ, Park JJ, Kim TI, Cheon JH, Park J. Impact of age at diagnosis on long-term prognosis in patients with intestinal Behçet's disease. J Gastroenterol Hepatol 2024; 39:519-526. [PMID: 38149352 DOI: 10.1111/jgh.16449] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/02/2023] [Revised: 11/22/2023] [Accepted: 12/03/2023] [Indexed: 12/28/2023]
Abstract
BACKGROUND AND AIM Although age at disease onset is considered to be a significant factor in the prognosis of Crohn's disease, little is known about its influence on the long-term prognosis of those with intestinal Behçet's disease (BD). This study aimed to evaluate the long-term clinical outcomes of patients with intestinal BD according to age of disease onset. METHODS Patients diagnosed with intestinal BD at < 18, 18-60, and > 60 years of age were classified into early-onset, adult-onset, and late-onset groups, respectively. The influence of disease onset time on clinical prognosis, including specific medical requirements, BD-related intestinal surgery, hospitalization, and emergency room visits, was compared using the log-rank test in a large cohort of patients with intestinal BD. RESULTS Among 780 patients, 21 (2.7%), 672 (86.2%), and 87 (11.1%) comprised the early-onset, adult-onset, and late-onset groups, respectively. Patients in the early-onset group were more likely to require immunosuppressants than those in the adult-onset group (P = 0.048). Nine (42.9%), 158 (23.5%), and 18 (20.7%) patients in the early-onset, adult-onset, and late-onset groups, respectively, underwent intestinal resection. The early-onset group exhibited a higher risk for intestinal resection than the late-onset (P = 0.043) and adult-onset (P = 0.030) groups. The late-onset group exhibited a higher risk for BD-related hospitalization than the adult-onset group (P = 0.023). CONCLUSIONS Age at diagnosis affected the clinical course of intestinal BD, including intestinal surgery, hospitalization, and specific medical requirements. Different treatment strategies should be established according to age at diagnosis.
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Affiliation(s)
- Ji Young Chang
- Department of Health Promotion Medicine, Ewha Womans University Seoul Hospital, Seoul, South Korea
| | - Soo Jung Park
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
- Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
- Inflammatory Bowel Disease Center, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - Jae Jun Park
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
- Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
- Inflammatory Bowel Disease Center, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - Tae Il Kim
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
- Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
- Inflammatory Bowel Disease Center, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - Jae Hee Cheon
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
- Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
- Inflammatory Bowel Disease Center, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
- Brain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, South Korea
| | - Jihye Park
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
- Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
- Inflammatory Bowel Disease Center, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
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Manuelyan Z, Butt E, Parupudi S. Gastrointestinal Behçet's disease: Manifestations, diagnosis, and management. Dis Mon 2024; 70:101674. [PMID: 38185603 DOI: 10.1016/j.disamonth.2023.101674] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2024]
Abstract
Behçet's disease (BD) is a rare, inflammatory vascular disorder with recurrent oral and genital aphthous ulcers, along with ocular and cutaneous manifestations. Gastrointestinal (GI) BD may involve any portion of the GI tract. However, it is commonly described in the terminal ileum, followed by the ileocecal region. Diagnosis is challenging given lack of pathognomonic tests; therefore, it is based on clinical criteria. Management of intestinal BD includes different classes of medications including corticosteroids, 5-aminosalicylic acid, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody agents. In this review, we aim to focus on intestinal BD and provide details of clinical manifestations, diagnosis and therapeutic options of intestinal BD from gastroenterology viewpoint.
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Affiliation(s)
- Zara Manuelyan
- Department of Internal Medicine, Division of Gastroenterology and Hepatology, The University of Texas Medical Branch at Galveston, Galveston, TX 77550, USA
| | - Edward Butt
- Department of Internal Medicine, The University of Texas Medical Branch at Galveston, 301 University Blvd, Galveston, TX 77550, USA.
| | - Sreeram Parupudi
- Department of Internal Medicine, Division of Gastroenterology and Hepatology, The University of Texas Medical Branch at Galveston, Galveston, TX 77550, USA
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Abstract
Behçet's disease is a rare and poorly understood vasculitis affecting blood vessels of all types and sizes. Uveitis and oral and genital ulcers represent the typical clinical triad. Populations along the ancient trading route connecting the Mediterranean basin with the Middle and Far East are most affected. Up to a quarter of the cases has a pediatric onset, typically around puberty. The aim of the treatment is early intervention to control inflammation, with symptom relief and prevention of relapses, damage, and complications. The heterogeneous clinical presentation often requires a multidisciplinary and tailored approach. Ocular, neurological, gastrointestinal, and vascular involvement is associated with a worse prognosis and needs more aggressive treatments. In young patients with expected prolonged disease, treatment should also focus on preventive measures and lifestyle advice. In recent years, the pharmacological armamentarium has grown progressively, although only a limited number of drugs are currently authorized for pediatric use. Most evidence for these drugs still derives from adult studies and experience; these are prescribed as off-label medications and are only available as adult formulations. Corticosteroids frequently represent the mainstay for the management of the initial acute phases, but their potential serious adverse effects limit their use to short periods. Different conventional disease-modifying anti-rheumatic drugs have long been used. Many other biologic drugs targeting different cytokines such as interleukin-1, interleukin-6, and interleukin-17 and treatments with small molecules including the phosphodiesterase 4 and Janus kinase inhibitors are emerging as novel promising therapeutic agents. In recent years, a growing interest has developed around anti-tumor necrosis factor agents that have often proven to be effective in severe cases, especially in those with a gastrointestinal and ocular involvement.
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Han SJ, Kang EA, Park J, Park SJ, Park JJ, Kim TI, Kim WH, Cheon JH. Risk Factors for Surgery in Patients with Intestinal Behçet's Disease During Anti-Tumor Necrosis Factor-Alpha Therapy. Yonsei Med J 2023; 64:111-116. [PMID: 36719018 PMCID: PMC9892539 DOI: 10.3349/ymj.2022.0264] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/08/2022] [Revised: 12/12/2022] [Accepted: 12/13/2022] [Indexed: 01/20/2023] Open
Abstract
PURPOSE Behçet's disease (BD) is a chronic inflammatory immune-mediated disease involving multiorgan systems. Gastrointestinal (GI) manifestations of BD include abdominal pain, vomiting, GI bleeding, fistula formation, obstruction, and perforation that might require surgery. Recently, anti-tumor necrosis factor-alpha (anti-TNF-α) therapy has been shown to have favorable outcomes in patients with intestinal BD who are refractory to conventional therapy. This study sought to figure out the risk factors for undergoing surgery during anti-TNF-α therapy in patients with intestinal BD. MATERIALS AND METHODS In this retrospective analysis of intestinal BD patients who were treated with anti-TNF-α, we collected the baseline patient data including comorbidities, clinical, endoscopic, and radiologic characteristics, and the Disease Activity Index for Intestinal Behçet's Disease at the time of anti-TNF-α initiation. Each potential risk factor was compared. For multivariate analysis, Cox regression was used. RESULTS A total of 62 patients were considered eligible for analysis, and 15 of them (24.1%) underwent surgery. In univariate analysis, the presence of extraintestinal manifestation, such as joint symptoms and erythrocyte sedimentation rate (ESR), were significantly associated with surgery during therapy. In multivariate analysis, drug response within 4 weeks [hazard ratio (HR), 64.59], skin and joint manifestation (HR, 10.23 and HR, 6.22), geographic ulcer (HR, 743.97), and ESR >42.5 mm/h (HR, 9.16) were found to be factors predictive of undergoing surgery during anti-TNF-α therapy. CONCLUSION We found five risk factors predictive of surgery in patients with intestinal BD receiving anti-TNF-α therapy, which can guide physicians in selecting appropriate patients between anti-TNF-α therapy and early surgery.
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Affiliation(s)
- So Jung Han
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Eun Ae Kang
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jihye Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Soo Jung Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Jun Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Tae Il Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Won Ho Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea.
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Hou CC, Ye JF, Ma HF, Guan JL. Clinical characteristics and risk factors of intestinal involvement in Behçet's syndrome patients: a cross-sectional study from a single center. Orphanet J Rare Dis 2021; 16:132. [PMID: 33731182 PMCID: PMC7972242 DOI: 10.1186/s13023-021-01772-x] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2020] [Accepted: 03/09/2021] [Indexed: 12/15/2022] Open
Abstract
BACKGROUND Intestinal Behçet's syndrome (BS) has high morbidity and mortality rates with serious complications. The purpose of this study was to investigate the clinical characteristics and laboratory parameters of intestinal and mucocutaneous BS patients and analyze the risk factors of intestinal involvement in BS patients. METHODS A retrospective analysis was used to collect the demographic data and laboratory parameters from 97 intestinal and 154 mucocutaneous BS patients. Univariate and multivariate logistic regression analyses were used to investigate the risk factors of intestinal involvement in BS patients. RESULTS The most common clinical manifestations of first onset in intestinal BS patients were oral ulceration (100.00%), followed by genital ulcers (62.89%) and erythema nodule (28.87%), gastrointestinal lesions (28.87%), pseudofolliculitis (25.77%), fever (17.53%), arthritis (16.49%), ocular involvement (5.15%), while the least common were vascular involvement (2.06%) and hematologic involvement involvement (2.06%). The most common intestinal segment involved in intestinal BS patients was terminal ileum (30.9%), followed by ileocecal (18.6%), colon (15.5%). By univariate logistic regression analysis, gender, age at hospitalization, age of disease onset, BDCAF, T-SPOT, fever, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), leukocyte, erythrocyte, hemoglobin (HGB), neutrophil-to-lymphocyte ratio, serum amyloid A, complement 3, albumin, total cholesterol, high-density lipoprotein and interleukin 6 (IL-6) were found all risk factors of intestinal involvement in BS patients (P < 0.05 or P = 0.00). Moreover, gender (male), BDCAF (≥ 2), ESR (≥ 15 mm/H), CRP (> 10 mg/L), HGB (< 130 g/L) and IL-6 (> 7 pg/ml) were found the independent risk factors of intestinal involvement in BS patients (all P < 0.05). CONCLUSIONS More attention shall be paid to gender, BDCAF, ESR, CRP, HGB and IL-6 in BS patients. When gender (male), BDCAF (≥ 2), ESR (≥ 15 mm/H), CRP (> 10 mg/L), HGB (< 130 g/L) and IL-6 (> 7 pg/ml) being observed, it may reminds that the presence of intestinal involvement in BS patients.
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Affiliation(s)
- Cheng-cheng Hou
- Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan’an west Road, Shanghai, 200040 People’s Republic of China
| | - Jing-fen Ye
- Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan’an west Road, Shanghai, 200040 People’s Republic of China
| | - Hai-fen Ma
- Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan’an west Road, Shanghai, 200040 People’s Republic of China
| | - Jian-long Guan
- Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan’an west Road, Shanghai, 200040 People’s Republic of China
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Gong L, Zhang YL, Sun LX, Chen GR, Wu D. Mucosal healing in intestinal Behçet's disease: A systematic review and meta-analysis. J Dig Dis 2021; 22:83-90. [PMID: 33314672 DOI: 10.1111/1751-2980.12965] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/20/2020] [Revised: 12/02/2020] [Accepted: 12/08/2020] [Indexed: 12/11/2022]
Abstract
OBJECTIVES Behçet's disease (BD) is a chronic inflammatory disease characterized by recurrent oral aphthous ulcers, intestinal lesions, genital ulcers, uveitis, and skin lesions. Evidence regarding mucosal healing for the prognosis of intestinal BD is scarce. The aim of this systematic review and meta-analysis was to determine the association between mucosal healing and long-term outcomes of patients with intestinal BD. METHODS Relevant studies were identified in a comprehensive search of PubMed, Cochrane Library, and EMBASE databases. Studies reporting long-term outcomes of mucosal healing in patients with intestinal BD were included. Pooled risk ratio (RR) and 95% confidence interval (CI) for disease recurrence and surgery were calculated using the Mantel-Haenszel random-effects models. Heterogeneity among the eligible studies was evaluated using the Q test and I2 statistics. RESULTS Of the 4785 studies initially identified, 8 were finally included. The pooled RR for the association between mucosal healing and disease recurrence was 0.41 (95% CI 0.30-0.57, P < 0.001). For the association between mucosal healing and the risk of surgery, the pooled RR was 0.33 (95% CI 0.17-0.63, P < 0.001). Confounding factors were adjusted in one study, whereas other studies only reported a crude association between mucosal healing and long-term outcomes without adjustment. CONCLUSIONS Mucosal healing is associated with a decreased risk of recurrence and surgery in intestinal BD. However, more studies are required given a small number of currently eligible studies and insufficient adjustment for confounding factors.
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Affiliation(s)
- Liang Gong
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Yue Lun Zhang
- Medical Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Lu Xi Sun
- Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Guo Rong Chen
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Dong Wu
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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8
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Cheon JH. Advances in Management of Intestinal Behçet's Disease: A Perspective From Gastroenterologists. JOURNAL OF RHEUMATIC DISEASES 2021; 28:4-16. [PMID: 37476392 PMCID: PMC10324954 DOI: 10.4078/jrd.2021.28.1.4] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2020] [Revised: 08/09/2020] [Accepted: 08/10/2020] [Indexed: 07/22/2023]
Abstract
Intestinal Behçet's disease (intestinal BD) is a rare chronic inflammatory disorder of the intestine that is characterized by recurrent intestinal manifestations with other systemic features of BD. Intestinal BD is diagnosed when a typically shaped ulcer is observed in the gastrointestinal tract, and the clinical findings meet the diagnostic criteria for BD. Owing to the small number of patients, intestinal BD is easily underestimated. On the other hand, but it often requires surgical treatment because of severe complications, including intestinal perforations or massive bleeding. The same treatment strategies used for inflammatory bowel diseases, such as Crohn's disease and ulcerative colitis, are used for intestinal BD. 5-Aminosalicylic acids, corticosteroids, and immunomodulators are considered conventional therapies, but a considerable number of patients eventually become unresponsive to these pharmaceutical treatments. Recently, biologic agents, such as anti-tumor necrosis factor-alpha inhibitors, have also been suggested as a new treatment option for intestinal BD. This article reviews the pathogenesis and diagnosis of intestinal BD and the current treatment strategies that are expected to be useful for rheumatologic specialists.
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Affiliation(s)
- Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Watanabe K, Tanida S, Inoue N, Kunisaki R, Kobayashi K, Nagahori M, Arai K, Uchino M, Koganei K, Kobayashi T, Takeno M, Ueno F, Matsumoto T, Mizuki N, Suzuki Y, Hisamatsu T. Evidence-based diagnosis and clinical practice guidelines for intestinal Behçet's disease 2020 edited by Intractable Diseases, the Health and Labour Sciences Research Grants. J Gastroenterol 2020; 55:679-700. [PMID: 32377946 PMCID: PMC7297851 DOI: 10.1007/s00535-020-01690-y] [Citation(s) in RCA: 36] [Impact Index Per Article: 7.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/25/2020] [Accepted: 04/08/2020] [Indexed: 02/04/2023]
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.
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Affiliation(s)
- Kenji Watanabe
- Department of Intestinal Inflammation Research, Hyogo College of Medicine, Hyogo, Japan
| | - Satoshi Tanida
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Aichi, Japan
| | - Nagamu Inoue
- Centers for Preventive Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Reiko Kunisaki
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Kanagawa, Japan
| | - Kiyonori Kobayashi
- Research and Development Center for New Medical Frontiers, Kitasato University, School of Medicine, Kanagawa, Japan
| | - Masakazu Nagahori
- Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University, Tokyo, Japan
| | - Katsuhiro Arai
- Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan
| | - Motoi Uchino
- Department of Inflammatory Bowel Disease, Division of Surgery, Hyogo College of Medicine, Hyogo, Japan
| | - Kazutaka Koganei
- Department of Inflammatory Bowel Disease, Yokohama Municipal Citizen’s Hospital, Kanagawa, Japan
| | - Taku Kobayashi
- Center for Advanced IBD Research and Treatment, Kitasato University Kitasato Institute Hospital, Tokyo, Japan
| | - Mitsuhiro Takeno
- Department of Allergy and Rheumatology, Nippon Medical School Musashi Kosugi Hospital, Kanagawa, Japan
| | - Fumiaki Ueno
- Center for Gastroenterology and Inflammatory Bowel Disease, Ofuna Chuo Hospital, Kanazawa, Japan
| | - Takayuki Matsumoto
- Division of Gastroenterology, Department of Medicine, Iwate Medical University, Iwate, Japan
| | - Nobuhisa Mizuki
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Kanagawa, Japan
| | - Yasuo Suzuki
- Inflammatory Bowel Disease Center, Toho University Sakura Medical Center, Chiba, Japan
| | - Tadakazu Hisamatsu
- Department of Gastroenterology and Hepatology, Kyorin University School of Medicine, Tokyo, Japan
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Park JW, Park Y, Park SJ, Kim TI, Kim WH, Cheon JH. Development of a Novel Endoscopic Scoring System to Predict Relapse after Surgery in Intestinal Behçet's Disease. Gut Liver 2018; 12:674-681. [PMID: 29938457 PMCID: PMC6254620 DOI: 10.5009/gnl17547] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/04/2017] [Revised: 04/01/2018] [Accepted: 04/27/2018] [Indexed: 01/28/2023] Open
Abstract
Background/Aims The cumulative surgery rate and postoperative relapse of intestinal Behcet’s disease (BD) have been reported to be high. This study aimed to establish a scoring system based on follow-up endoscopic findings that can predict intestinal BD recurrence after surgery. Methods Fifty-four patients with intestinal BD who underwent surgery due to bowel complications and underwent follow-up colonoscopy were retrospectively investigated. Their clinical data, including colonoscopic findings, were retrieved. Classification and regression tree analysis was used to develop an appropriate endoscopic classification model that can explain the postsurgical recurrence of intestinal BD most accurately based on the following classification: e0, no lesions; e1, solitary ulcer <20 mm in size; e2, solitary ulcer ≥ 20 mm in size; and e3, multiple ulcers regardless of size. Results Clinical relapse occurred in 37 patients (68.5%). Among 38 patients with colonoscopic recurrence, only 29 patients had clinically relapsed. Multivariate analysis identified higher disease activity index for intestinal BD at colonoscopy (hazard ratio [HR], 1.013; 95% confidence interval [CI], 1.005 to 1.021; p=0.002) and colonoscopic recurrence (HR, 2.829; 95% CI, 1.223 to 6.545; p=0.015) as independent risk factors for clinical relapse of intestinal BD. Endoscopic findings were classified into four groups, and multivariate analysis showed that the endoscopic score was an independent risk factor of clinical relapse (p=0.012). The risk of clinical relapse was higher in the e3 group compared to the e0 group (HR, 6.284; 95% CI, 2.036 to 19.391; p=0.001). Conclusions This new endoscopic scoring system could predict clinical relapse in patients after surgical resection of intestinal BD.
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Affiliation(s)
- Jung Won Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Yehyun Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Soo Jung Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Tae Il Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Won Ho Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Abstract
Behçet disease (BD) is a variable vessel vasculitis that can involve several organs and systems. Gastrointestinal (GI) involvement has an acute exacerbating course with ulcers, most commonly in the ileocolonic area. These ulcers can be large and deep, causing perforation and massive bleeding. This article highlights the current knowledge on the epidemiology, clinical findings, diagnosis, and management of GI involvement of BD, with emphasis on recent findings.
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Affiliation(s)
- Ibrahim Hatemi
- Division of Gastroenterology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Koca Mustafa Pasa Mahallesi, Cerrahpaşa Caddesi No:53, 34096 Fatih/Istanbul, Turkey
| | - Gulen Hatemi
- Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Koca Mustafa Paşa Mahallesi, Cerrahpasa Caddesi No:53, 34096 Fatih/Istanbul, Turkey
| | - Aykut Ferhat Çelik
- Division of Gastroenterology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Koca Mustafa Pasa Mahallesi, Cerrahpaşa Caddesi No:53, 34096 Fatih/Istanbul, Turkey.
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12
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Lee HJ, Cheon JH. Optimal diagnosis and disease activity monitoring of intestinal Behçet's disease. Intest Res 2017; 15:311-317. [PMID: 28670227 PMCID: PMC5478755 DOI: 10.5217/ir.2017.15.3.311] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2016] [Revised: 01/20/2017] [Accepted: 01/23/2017] [Indexed: 12/15/2022] Open
Abstract
Intestinal Behçet's disease (BD), generally accepted as a type of inflammatory bowel disease (IBD), could be diagnosed when patients with BD have objectively documented gastrointestinal symptoms and intestinal ulcerations. Similar to IBD, intestinal BD has an unpredictable disease course with relapse and remission and is often related to a poor prognosis. However, there is no single gold standard for assessment of the disease activity of intestinal BD, and its diagnosis and management depend heavily on expert opinions. The Korean IBD Study Group recently developed novel diagnostic criteria based on colonoscopy findings and clinical manifestations using a modified Delphi process to overcome drawbacks of previously used consensus for the diagnosis of intestinal BD. In addition, the recently developed disease activity index for intestinal BD, consisting of a relatively simple 8-point index, could also help in determining treatment strategies and monitoring therapeutic responses. In this review, the progress in the diagnosis and disease activity measurement of intestinal BD will be discussed.
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Affiliation(s)
- Hyun Jung Lee
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Kim DH, Park Y, Kim B, Kim SW, Park SJ, Hong SP, Kim TI, Kim WH, Cheon JH. Fecal calprotectin as a non-invasive biomarker for intestinal involvement of Behçet's disease. J Gastroenterol Hepatol 2017; 32:595-601. [PMID: 27521492 DOI: 10.1111/jgh.13530] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 08/09/2016] [Indexed: 12/17/2022]
Abstract
BACKGROUND AND AIM The diagnostic and prognostic values of fecal calprotectin (FC) levels in patients with inflammatory bowel diseases have been proven. However, little is known about the usefulness of FC measurement in predicting intestinal involvement of Behçet's disease (BD). METHODS Forty-four consecutive patients with systemic BD who underwent colonoscopy for the evaluation of gastrointestinal symptoms were prospectively enrolled between November 2012 and March 2014 in a single tertiary medical center. Fecal specimens from the patients were obtained the day before bowel cleansing and 3 months after colonoscopy. RESULTS Twenty-five patients showed intestinal ulcerations on colonoscopy (12 [48.0%] typical and 13 [52.0%] atypical ulcerations). The median FC level in the intestinal BD group was significantly higher than that in the non-diagnostic group (112.53 [6.86-1604.39] vs 31.64 [5.46-347.60] µg/g, respectively, P = 0.003). Moreover, the typical ulceration group showed a significantly higher median FC level than the atypical ulceration group in patients with intestinal BD (435.995 [75.65-1604.39] vs 71.42 [6.86-476.94] µg/g, respectively, P = 0.033). Multivariate analysis revealed higher FC as an independent predictor of intestinal BD (OR = 38.776; 95% CI = 2.306-652.021; P = 0.011). The cut-off level of FC for predicting intestinal BD was 68.89 µg/g (76% sensitivity and 79% specificity). The absolute changes between fecal calprotectin levels and the disease activity index of intestinal BD from initial diagnosis of intestinal BD to 3 months after diagnosis were significantly correlated (Pearson's correlation coefficient = 0.470, P = 0.027). CONCLUSION The FC level might serve as a non-invasive surrogate marker of intestinal involvement of BD.
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Affiliation(s)
- Duk Hwan Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea.,Digestive disease center, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, Korea
| | - Yehyun Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Bun Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Seung Won Kim
- Severance Biomedical Science Institute, Yonsei University College of Medicine, Seoul, Korea.,Brain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea
| | - Soo Jung Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Sung Pil Hong
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Tae Il Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Won Ho Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea.,Severance Biomedical Science Institute, Yonsei University College of Medicine, Seoul, Korea.,Brain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea
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Lopalco G, Rigante D, Venerito V, Fabiani C, Franceschini R, Barone M, Lapadula G, Galeazzi M, Frediani B, Iannone F, Cantarini L. Update on the Medical Management of Gastrointestinal Behçet's Disease. Mediators Inflamm 2017; 2017:1460491. [PMID: 28210071 PMCID: PMC5292121 DOI: 10.1155/2017/1460491] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2016] [Accepted: 01/04/2017] [Indexed: 02/07/2023] Open
Abstract
Behçet's disease (BD) is a multisystemic disorder of unknown etiology mainly defined by recurrent oral aphthosis, genital ulcers, and chronic relapsing bilateral uveitis, all of which represent the "stigmata" of disease. However, many other organs including the vascular, neurological, musculoskeletal, and gastrointestinal systems can be affected. The gastrointestinal involvement in Behçet's disease (GIBD), along with the neurological and vascular ones, represents the most feared clinical manifestation of BD and shares many symptoms with inflammatory bowel diseases, such as Crohn's disease and ulcerative colitis. Consequently, the differential diagnosis is often a daunting task, albeit the presence of typical endoscopic and pathologic findings may be a valuable aid to the exact diagnosis. To date, there are no standardized medical treatments for GIBD; therefore therapy should be tailored to the single patient and based on the severity of the clinical features and their complications. This work provides a digest of all current experience and evidence about pharmacological agents suggested by the medical literature as having a potential role for managing the dreadful features of GIBD.
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Affiliation(s)
- Giuseppe Lopalco
- 1Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Donato Rigante
- 2Institute of Pediatrics, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Largo A. Gemelli 8, 00168 Rome, Italy
| | - Vincenzo Venerito
- 1Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Claudia Fabiani
- 3Department of Ophthalmology, Humanitas Clinical and Research Center, Via Manzoni 56, Rozzano, 20089 Milan, Italy
| | - Rossella Franceschini
- 4Ophthalmology and Neurosurgery Department, University of Siena, Viale Bracci 1, 53100 Siena, Italy
| | - Michele Barone
- 5Section of Gastroenterology, Department of Emergency and Organ Transplantation, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Giovanni Lapadula
- 1Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Mauro Galeazzi
- 6Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale Bracci 1, 53100 Siena, Italy
| | - Bruno Frediani
- 6Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale Bracci 1, 53100 Siena, Italy
| | - Florenzo Iannone
- 1Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Luca Cantarini
- 6Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale Bracci 1, 53100 Siena, Italy
- *Luca Cantarini:
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15
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Kim DH, Cheon JH. Intestinal Behçet's Disease: A True Inflammatory Bowel Disease or Merely an Intestinal Complication of Systemic Vasculitis? Yonsei Med J 2016; 57:22-32. [PMID: 26632379 PMCID: PMC4696957 DOI: 10.3349/ymj.2016.57.1.22] [Citation(s) in RCA: 59] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/19/2015] [Indexed: 12/18/2022] Open
Abstract
Behçet's disease (BD) is a multi-systemic inflammatory disorder of an unknown etiology and shows a chronic recurrent clinical course. When the disease involves the alimentary tract, it is called intestinal BD because of its clinical importance. Intestinal BD is more frequently reported in East Asian countries than in Western or Middle Eastern countries. While any part of the gastrointestinal tract can be involved, the most common location of intestinal BD is the ileocecal area. A few, large, deep ulcerations with discrete border are characteristic endoscopic findings of intestinal BD. Currently, there is no single gold standard test or pathognomonic finding of intestinal BD. However, recently developed novel diagnostic criteria and a disease activity index have helped in assessing intestinal BD. As intestinal BD shares a lot of characteristics with inflammatory bowel disease, including genetic background, clinical manifestations, and therapeutic strategies, distinguishing between the two diseases in clinical practice is quite difficult. However, biologic agents such as anti-tumor necrosis factor α antibody shows a considerable efficacy similar to inflammatory bowel disease cases. It is important to distinguish and treat those two disease entities separately from the standpoint of precise medicine. Clinicians should require comprehensive knowledge regarding the similarities and differences between intestinal BD and inflammatory bowel disease for making an accurate clinical decision.
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Affiliation(s)
- Duk Hwan Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
- Digestive Disease Center, CHA Bundang Medical Center, CHA University, Seongnam, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
- Severance Biomedical Science Institute, Yonsei University College of Medicine, Seoul, Korea
- Brain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea.
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Abstract
PURPOSE OF REVIEW This review discusses recent scientific developments in the diagnosis, treatment, and prognosis of intestinal Behçet's disease. RECENT FINDINGS Gastrointestinal involvement is a major cause of morbidity and mortality in Behçet's disease. Patient clinical data are scarce because of the rarity of the disease; however, novel diagnostic criteria and disease activity indices have been developed recently to aid treatment of Behçet's disease patients. Current therapies include 5-aminosalicylic acids, corticosteroids, immunomodulators, or antitumor necrosis factor alpha agents. Antitumor necrosis factor alpha agents can achieve clinical responses and remission in patients that were previously nonresponsive to corticosteroids or immunomodulators. Clinical variables, including young age and higher disease activity at the time of diagnosis, volcano-type ulcers, absence of mucosal healing, higher C-reactive protein levels, prior history of surgery, and lack of initial response to medical therapy, can be regarded as poor prognostic factors. SUMMARY Previously, the diagnosis and management of intestinal Behçet's disease depended upon the expertise of individual clinicians; however, more standardized medical assessments and improved treatment regimens for Behçet's disease patients are evolving.
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Abstract
Behcet disease (BD) is a chronic, multisystem, inflammatory disease characterized by variable clinical manifestations involving systemic vasculitis of both the small and large blood vessels. The majority of BD patients present with recurrent oral ulcers in combination with other manifestations of the disease, including genital ulcers, skin lesions, arthritis, uveitis, thrombophlebitis, gastrointestinal or central nervous system involvement. Gastrointestinal BD occurs in 3 to 25% of the BD patients and shares many clinical characteristics with inflammatory bowel disease (IBD). Consequently, the differentiation between IBD and gastrointestinal manifestation of BD is very difficult. Intestinal BD should be considered in patients who present with abdominal pain, diarrhea, weight loss, and rectal bleeding who are susceptible or at a risk for intestinal BD.
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Affiliation(s)
- Anne B Chin
- Department of Surgery, Medstar Georgetown University Hospital, Washington, District of Columbia
| | - Anjali S Kumar
- Section of Colon and Rectal Surgery, Medstar Washington Hospital Center, Washington, District of Columbia
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Surgical Treatment and Outcomes in Patients With Intestinal Behçet Disease: Long-term Experience of a Single Large-Volume Center. Dis Colon Rectum 2015; 58:575-81. [PMID: 25944429 DOI: 10.1097/dcr.0000000000000373] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
BACKGROUND Few studies have examined the surgical treatment of intestinal Behçet disease. Consequently, there is currently no standard surgical treatment for intestinal Behçet disease. Instead, treatment is empirical and symptom based. OBJECTIVE Our aim was to evaluate the clinical course after surgery and determine the appropriate surgical options for intestinal Behçet disease. DESIGN Medical charts of patients who underwent surgery for intestinal Behçet were retrospectively reviewed. SETTINGS The study was conducted at a tertiary referral center. PATIENTS Ninety-one patients who underwent surgical treatment for intestinal Behçet disease between January 1995 and December 2012 were included in this study. MAIN OUTCOME MEASURES Primary outcomes measured were patient demographics, clinical characteristics, operative and postoperative outcomes, and long-term follow-up data. RESULTS Surgical treatment was mainly in response to intractability to medical treatment (56.0%), and 19.8% of patients underwent an emergency operation. Surgery was performed laparoscopically in 33.0% of the patients. Most patients received an ileocecectomy (39.6%) or a right hemicolectomy (34.1%). Twenty-eight patients (30.8%) experienced postoperative morbidities, and 8 patients (8.8%) required reoperations. There were 3 deaths. Reoperation was required for recurrent disease in 32 patients during the long-term follow-up, and the 5-year cumulative reoperation rate was 31.2% (95% CI, 20.4%-42.0%). Among those requiring a second operation, 53.1% were segmental colonic resections that included the previous anastomosis. From multivariable Cox regression analysis, independent predictors of surgical recurrence included postoperative use of steroids (HR = 2.85 (95% CI, 1.21-6.75); p = 0.02), postoperative complications (HR = 2.42 (95% CI, 1.12-5.22); p = 0.03), and BMI (HR per 1-kg/m increase in BMI = 0.90 (95% CI, 0.82-0.99); p = 0.04). LIMITATIONS This study was designed retrospectively and had a small sample size. CONCLUSIONS Patients treated surgically for intestinal Behçet disease frequently have postoperative complications and the need for a stoma and have a high risk of recurrence.
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Skef W, Hamilton MJ, Arayssi T. Gastrointestinal Behçet's disease: A review. World J Gastroenterol 2015; 21:3801-3812. [PMID: 25852265 PMCID: PMC4385527 DOI: 10.3748/wjg.v21.i13.3801] [Citation(s) in RCA: 125] [Impact Index Per Article: 12.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2014] [Revised: 01/14/2015] [Accepted: 01/30/2015] [Indexed: 02/07/2023] Open
Abstract
Behçet’s disease (BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and skin lesions. Prevalence of BD is highest in countries along the ancient silk road from the Mediterranean basin to East Asia. By comparison, the prevalence in North American and Northern European countries is low. Gastrointestinal manifestations of Behçet’s disease are of particular importance as they are associated with significant morbidity and mortality. Although ileocecal involvement is most commonly described, BD may involve any segment of the intestinal tract as well as the various organs within the gastrointestinal system. Diagnosis is based on clinical criteria - there are no pathognomonic laboratory tests. Methods for monitoring disease activity on therapy are available but imperfect. Evidence-based treatment strategies are lacking. Different classes of medications have been successfully used for the treatment of intestinal BD which include 5-aminosalicylic acid, corticosteroids, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody therapy. Like inflammatory bowel disease, surgery is reserved for those who are resistant to medical therapy. A subset of patients have a poor disease course. Accurate methods to detect these patients and the optimal strategy for their treatment are not known at this time.
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Mucosal healing predicts the long-term prognosis of intestinal Behçet's disease. Dig Dis Sci 2014; 59:2529-35. [PMID: 24838499 DOI: 10.1007/s10620-014-3198-z] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/14/2014] [Accepted: 04/30/2014] [Indexed: 12/23/2022]
Abstract
BACKGROUND Mucosal healing (MH) has emerged as a therapeutic goal in the treatment of inflammatory bowel disease; however, little is known about the impact of MH on the prognosis of intestinal Behçet's disease (BD). AIM We investigated whether MH could predict the prognosis of intestinal BD. METHODS We retrospectively reviewed the medical records of 80 patients with intestinal BD who underwent colonoscopy within 3 months after clinical remission. The clinical recurrence rate according to the presence or absence of MH was evaluated using the Kaplan-Meier method and the log-rank test. In order to evaluate MH as an independent prognostic factor, a multivariate analysis using Cox proportional hazards regression model was performed including other potential factors for the relapse of intestinal BD. RESULTS The number of patients with active ulcers at the time of clinical remission was 57 (71.3%), while 23 patients (28.7%) were experiencing MH. In the active ulcer group, 39 patients (68.4%) experienced recurrence during the follow-up period, whereas 7 patients (30.4%) recurred in the MH group. The cumulative recurrence rate was significantly higher in the active ulcer group than in the MH group (P < 0.001). A multivariate analysis identified active ulcers at the time of clinical remission as an independent predictive factor for relapse. CONCLUSION Our study demonstrates that MH is an independent factor predictive of long-term prognosis of intestinal BD. MH might be the ultimate therapeutic goal in the treatment of intestinal BD.
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Chen CY, Chang SW, Tsai CH. Suspected Behçet's disease presenting with enterocutaneous fistula formation and lower gastrointestinal bleeding after appendectomy. FORMOSAN JOURNAL OF SURGERY 2014. [DOI: 10.1016/j.fjs.2013.10.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022] Open
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Hatemi G, Merkel PA, Hamuryudan V, Boers M, Direskeneli H, Aydin SZ, Yazici H. Outcome measures used in clinical trials for Behçet syndrome: a systematic review. J Rheumatol 2014; 41:599-612. [PMID: 24488418 DOI: 10.3899/jrheum.131249] [Citation(s) in RCA: 49] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Behçet syndrome (BS) is a multisystem vasculitis that is most active during young adulthood, causing serious disability and significant impairment in quality of life. Differences in the disease course, severity, and organ involvement between patients, depending on the age at presentation and sex, makes it impossible to determine a single management strategy. The diversity and variability in the outcome measures used in clinical trials in BS makes it difficult to compare the results or inform physicians about the best management strategy for individual patients. There is a large unmet need to determine or develop validated outcome measures for use in clinical trials in BS that are acceptable to researchers and regulatory agencies. We conducted a systematic review to describe the outcomes and outcome measures that have been used in clinical trials in BS. This review revealed the diversity and variability in the outcomes and outcome measures and the lack of standard definitions for most outcomes and rarity of validated outcome tools for disease assessment in BS. This systematic literature review will identify domains and candidate instruments for use in a Delphi exercise, the next step in the development of a core set of outcome measures that are properly validated and widely accepted by the collaboration of researchers from many different regions of the world and from different specialties, including rheumatology, ophthalmology, dermatology, gastroenterology, and neurology.
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Affiliation(s)
- Gulen Hatemi
- From the Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey; Division of Rheumatology and Department of Epidemiology, University of Pennsylvania, Philadelphia, PA, USA; Department of Clinical Epidemiology, VU University Medical Center, Amsterdam, The Netherlands; Department of Rheumatology, Marmara University School of Medicine, Istanbul; Department of Rheumatology, Istanbul Medeniyet University, Goztepe Training and Research Hospital, Istanbul, Turkey
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Abstract
Behçet's disease (BD) is a multisystem inflammatory disorder that presents as recurrent oral and genital ulcers in conjunction with other dermatological and ocular manifestations. The prevalence of BD is higher in Middle and East Asia than in Western countries. Intestinal BD is a specific subtype of BD, characterized by intestinal ulcers and associated gastrointestinal symptoms. Similar to inflammatory bowel disease, intestinal BD exhibits a fluctuating disease course with repeated episodes of relapse and remission that necessitate adequate maintenance therapy after achievement of clinical remission. Medical treatment of intestinal BD is largely empirical since well-controlled studies have been difficult to perform due to the heterogeneity and rarity of the disease. To date, 5-aminosalicylic acid, systemic corticosteroids, and immunosuppressants have been used anecdotally to treat intestinal BD. The clinical course of intestinal BD shows considerable variability, and the exact point at which more potent agents such as immunosuppressants should be used has not yet been elucidated. Given the difficulty in predicting which patients will experience complicated disease courses and the fact that these drugs are related with certain risk resulting from immunosuppression, proper identification of prognostic factors in intestinal BD may allow physicians to implement tailored medical therapy and individualized patient monitoring based on risk stratification. In this review, the impact of baseline characteristics on the long-term course of intestinal BD, prognostic factors during various medical therapies, and outcome predictors related to surgery will be discussed.
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Affiliation(s)
- Jae Jun Park
- Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea.
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Kovacs DB, Ray DK, Dasgupta K, Borowski DW. Intestinal complications of Behçet's disease. BMJ Case Rep 2013; 2013:bcr-2013-200253. [PMID: 23917369 DOI: 10.1136/bcr-2013-200253] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
We report a case of a young female patient with long-standing oral and genital Behçet's disease (BD), who presented with progressive severe colonic inflammation and perforation, requiring multiple laparotomies. The case had ultimately a favourable outcome despite posing a number of diagnostic and therapeutic challenges. Intestinal complications, although rare, should be considered as important differential diagnoses in patients with BD presenting with abdominal pain, and is a difficult-to-prove differential diagnosis to Crohn's disease.
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Affiliation(s)
- D Botond Kovacs
- Department of Colorectal Surgery, University Hospital North Tees, Stockton on Tees, UK
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Affiliation(s)
- Hye Won Lee
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Won Ho Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Lee HJ, Kim YN, Jang HW, Jeon HH, Jung ES, Park SJ, Hong SP, Kim TI, Kim WH, Nam CM, Cheon JH. Correlations between endoscopic and clinical disease activity indices in intestinal Behcet's disease. World J Gastroenterol 2012; 18:5771-8. [PMID: 23155319 PMCID: PMC3484347 DOI: 10.3748/wjg.v18.i40.5771] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2012] [Revised: 07/04/2012] [Accepted: 07/18/2012] [Indexed: 02/06/2023] Open
Abstract
AIM: To develop a novel endoscopic severity model of intestinal Behcet's disease (BD) and to evaluate its feasibility by comparing it with the actual disease activity index for intestinal Behcet's disease (DAIBD).
METHODS: We reviewed the medical records of 167 intestinal BD patients between March 1986 and April 2011. We also investigated the endoscopic parameters including ulcer locations, distribution, number, depth, shape, size and margin to identify independent factors associated with DAIBD. An endoscopic severity model was developed using significant colonoscopic variables identified by multivariate regression analysis and its correlation with the DAIBD was evaluated. To determine factors related to the discrepancy between endoscopic severity and clinical activity, clinical characteristics and laboratory markers of the patients were analyzed.
RESULTS: A multivariate regression analysis revealed that the number of intestinal ulcers (≥ 2, P = 0.031) and volcanoshaped ulcers (P = 0.001) were predictive factors for the DAIBD. An endoscopic severity model (Y) was developed based on selected endoscopic variables as follows: Y = 47.44 + 9.04 × non-Ileocecal area + 11.85 ×≥ 2 of intestinal ulcers + 5.03 × shallow ulcers + 12.76 × deep ulcers + 4.47 × geographic-shaped ulcers + 26.93 × volcano-shaped ulcers + 8.65 ×≥ 20 mm of intestinal ulcers. However, endoscopic parameters used in the multivariate analysis explained only 18.9% of the DAIBD variance. Patients with severe DAIBD scores but with moderately predicted disease activity by the endoscopic severity model had more symptoms of irritable bowel syndrome (21.4% vs 4.9%, P = 0.026) and a lower rate of corticosteroid use (50.0% vs 75.6%, P = 0.016) than those with severe DAIBD scores and accurately predicted disease by the model.
CONCLUSION: Our study showed that the number of intestinal ulcers and volcano-shaped ulcers were predictive factors for severe DAIBD scores. However, the correlation between endoscopic severity and DAIBD (r = 0.434) was weak.
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Jung YS, Yoon JY, Hong SP, Kim TI, Kim WH, Cheon JH. Influence of age at diagnosis and sex on clinical course and long-term prognosis of intestinal Behcet's disease. Inflamm Bowel Dis 2012; 18:1064-71. [PMID: 21793128 DOI: 10.1002/ibd.21833] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/09/2011] [Accepted: 06/23/2011] [Indexed: 12/21/2022]
Abstract
BACKGROUND The aim of this study was to examine the influence of age at diagnosis and sex on the clinical course and long-term prognosis of intestinal Behcet's disease (BD). METHODS We reviewed the medical records of 291 patients with intestinal BD who underwent regular follow-up at a single tertiary academic medical center. The patients were divided into two groups according to their age at diagnosis of intestinal BD or sex. The cumulative probabilities of operation, admission, corticosteroid use, and immunosuppressant use after diagnosis were analyzed using the Kaplan-Meier method and a log-rank test. RESULTS Of the 291 patients, 154 (52.9%) were diagnosed with intestinal BD when younger than 40 years old, and 132 (45.4%) were male. Younger age at diagnosis was associated with a higher leukocyte count, C-reactive protein (CRP) level, and disease activity index for intestinal BD, and with a greater prevalence of volcano-shaped ulcers and a definite diagnostic subtype. Moreover, the cumulative probabilities of operation, admission, and corticosteroid use were significantly higher in the younger group. Male sex was associated with a higher CRP level and a greater prevalence of volcano-shaped ulcers. However, there were no significant differences in cumulative probabilities of operation, admission, corticosteroid use, and immunosuppressant use according to sex. CONCLUSIONS In intestinal BD, younger age at diagnosis is associated with a more severe clinical course and a poorer prognosis. However, although some clinical manifestations at initial diagnosis tend to be more severe in male patients, clinical outcomes do not differ significantly according to sex.
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Affiliation(s)
- Yoon Suk Jung
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Wu QJ, Zhang FC, Zhang X. Adamantiades-Behcet's disease-complicated gastroenteropathy. World J Gastroenterol 2012; 18:609-15. [PMID: 22363131 PMCID: PMC3281217 DOI: 10.3748/wjg.v18.i7.609] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/02/2011] [Revised: 10/12/2011] [Accepted: 12/31/2011] [Indexed: 02/06/2023] Open
Abstract
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.
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Abstract
BACKGROUND To date, no studies have been conducted to evaluate the potential benefits of early surgery in patients with intestinal Behçet disease. OBJECTIVE We investigated the long-term clinical outcomes in patients with intestinal Behçet disease first diagnosed at surgery ("early surgery") compared with those requiring surgical resection during the course of the disease ("late surgery"). DESIGN This is a retrospective cohort study. SETTINGS This study was conducted at a single tertiary academic medical center. PATIENTS We reviewed the medical records of 272 consecutive patients with intestinal Behçet disease between March 1986 and August 2010. MAIN OUTCOME MEASURES The cumulative probabilities of clinical recurrence and reoperation after operation were the main outcomes measures. RESULTS Forty of 272 patients were first diagnosed with intestinal Behçet disease at surgery (early surgery); the remaining 232 were diagnosed clinically, with 62 undergoing surgery during their follow-up after clinical diagnosis (late surgery). The cumulative probabilities of postoperative clinical recurrence and reoperation were significantly lower in the early-surgery group than in the late-surgery group (p = 0.045 and p = 0.003). In multivariate analysis, early surgery was the only independent factor significantly associated with a reduced probability of reoperation (HR 0.26; 95% CI 0.10-0.71; p = 0.008). However, when we analyzed only the patients who underwent surgery because of chronic symptoms, early surgery was not associated with lower cumulative clinical recurrence and reoperation rates (p = 0.896 and p = 0.492). LIMITATIONS We analyzed the clinical characteristics retrospectively, and the number of patients was insufficient to reach a decisive conclusion. CONCLUSIONS : According to the current study, the patients with intestinal Behçet disease undergoing early surgery showed better prognoses in comparison with those undergoing late surgery. Early surgery may represent a valid approach in the initial management of the patients with intestinal Behçet disease, at least in the subset of the patients with acute symptoms.
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Jung YS, Yoon JY, Lee JH, Jeon SM, Hong SP, Kim TI, Kim WH, Cheon JH. Prognostic factors and long-term clinical outcomes for surgical patients with intestinal Behcet's disease. Inflamm Bowel Dis 2011; 17:1594-602. [PMID: 21674717 DOI: 10.1002/ibd.21517] [Citation(s) in RCA: 60] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/19/2010] [Accepted: 09/07/2010] [Indexed: 12/21/2022]
Abstract
BACKGROUND To date there have been few studies regarding the efficacy of surgical treatment and related prognostic factors following intestinal resection in patients with intestinal Behcet's disease (BD). Here we investigated the long-term clinical outcomes and related prognostic factors after surgical treatment for intestinal BD. METHODS We reviewed the medical records of 72 patients with intestinal BD who underwent surgery between March 1986 and May 2010. Prognostic factors were identified by univariate analysis using the Kaplan-Meier method, the log-rank test, and multivariate analysis using Cox proportional hazards regression models. RESULTS Recurrence after surgical treatment was observed in 42 (58.3%) patients and reoperations were performed in 22 (30.6%) patients. The cumulative recurrence rates after surgical treatment were 29.2% at 2 years and 47.2% at 5 years; the cumulative reoperation rates were 12.5% at 2 years and 22.2% at 5 years. Multivariate analysis identified volcano-shaped ulcers, higher C-reactive protein (CRP) levels (≥ 4.4 mg/dL), and the presence of intestinal perforations detected by pathology as independent predictive factors for recurrence. Moreover, volcano-shaped ulcers, higher CRP levels (≥ 4.4 mg/dL), and a history of postoperative steroid therapy were independent predictive factors for reoperation. CONCLUSIONS According to the current study, volcano-shaped ulcers, higher CRP levels, a history of postoperative steroid therapy, and the presence of intestinal perforations detected by pathology were factors of a poor prognosis. Careful follow-up is required in surgical patients with these risk factors, who are at high risk for recurrence and reoperation.
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Affiliation(s)
- Yoon Suk Jung
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Cheon JH, Han DS, Park JY, Ye BD, Jung SA, Park YS, Kim YS, Kim JS, Nam CM, Kim YN, Yang SK, Kim WH. Development, validation, and responsiveness of a novel disease activity index for intestinal Behçet's disease. Inflamm Bowel Dis 2011; 17:605-13. [PMID: 20848515 DOI: 10.1002/ibd.21313] [Citation(s) in RCA: 81] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
BACKGROUND There is no disease activity index (DAI) currently available that can objectively assess the disease status of intestinal Behçet's disease (BD). The aim of this study was to develop a novel specific DAI for intestinal BD through a prospective study. METHODS Items included in the index were produced and graded by experts in intestinal BD. Two separate cohorts of patients (weighting and validation cohorts) with intestinal BD were prospectively enrolled, and their clinical and laboratory data were collected. Through weighting items by multiple regression modeling, the DAI for intestinal BD (DAIBD) was derived using the weighting cohort and validated using the validation cohort. The index's responsiveness was evaluated using a longitudinal cohort derived from the weighting cohort at a follow-up visit 2-3 months later. RESULTS A total of 110 patients with intestinal BD were enrolled in the weighting cohort. Eight of the 17 items selected by the experts accounted for 86.8% of the physician's global assessment (PGA) variance. The DAIBD calculated with gradations of weighted items correlated more strongly with PGA (r=0.850) than did the Crohn's disease activity index (CDAI) (r=0.649) in 50 patients of the validation cohort. Furthermore, quiescent, mild, moderate, and severe could be discriminated using the best cutoff scores. The DAIBD showed much higher responsiveness than did the CDAI (r=0.812 vs. 0.645, respectively) in 109 patients in the longitudinal cohort. CONCLUSIONS This novel DAIBD is an easy, valid, and responsive index to assess disease activity and can be useful to physicians who treat intestinal BD.
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Affiliation(s)
- Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Moon CM, Cheon JH, Shin JK, Jeon SM, Bok HJ, Lee JH, Park JJ, Hong SP, Kim TI, Kim NK, Kim WH. Prediction of free bowel perforation in patients with intestinal Behçet's disease using clinical and colonoscopic findings. Dig Dis Sci 2010; 55:2904-11. [PMID: 20094787 DOI: 10.1007/s10620-009-1095-7] [Citation(s) in RCA: 39] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/05/2009] [Accepted: 12/04/2009] [Indexed: 12/11/2022]
Abstract
BACKGROUND Gastrointestinal tract involvement in Behçet's disease (BD) often requires surgical intervention due to serious complications such as intestinal perforation, fistula formation, or massive bleeding. AIM The aims of this study were to investigate the clinical and surgical features of free bowel perforation and to determine the risk factors associated with this complication in intestinal BD patients. METHODS We reviewed the medical records of 129 patients with intestinal BD treated from September 1988 to September 2008. Among them, 33 patients had intestinal perforations and all underwent emergent or elective laparotomy. RESULTS The mean age of the patients with bowel perforation was 34.8 ± 15.6 years (range 12-70 years) with a sex ratio of 2.3:1 (male:female). Twenty-seven (81.8%) patients were diagnosed with intestinal BD preoperatively, whereas six (18.2%) patients were diagnosed by pathological examination after operation. Fourteen (42.4%) patients experienced postoperative recurrence of intestinal BD and 11 (33.3%) underwent reoperation. Multivariate Cox hazard regression analysis identified younger age (≤ 25 years) at diagnosis (HR = 3.25; 95% CI, 1.41-7.48, p = 0.006), history of prior laparotomy (HR = 5.53; 95% CI, 2.25-13.56, p = 0.0001), and volcano-shaped intestinal ulcers (HR = 2.84; 95% CI, 1.14-7.08, p = 0.025) as independent risk factors for free bowel perforation in intestinal BD. CONCLUSIONS According to the results of our study, patients diagnosed with intestinal BD younger than 25 years, who had a history of prior laparotomy or volcano-shaped intestinal ulcers have an increased risk of free bowel perforation.
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Affiliation(s)
- Chang Mo Moon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, 250 Seongsanno, Seodaemun-gu, Seoul, 120-752, Republic of Korea
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Response rates to medical treatments and long-term clinical outcomes of nonsurgical patients with intestinal Behçet disease. J Clin Gastroenterol 2010; 44:e116-22. [PMID: 20054283 DOI: 10.1097/mcg.0b013e3181c8a50f] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
BACKGROUND The aims of this study were to evaluate the efficacy of medical treatments and to identify factors to predict clinical outcome of intestinal Behçet disease (BD) during medical treatment. METHODS We performed a retrospective review of the medical records of 93 patients who were diagnosed and medically treated with intestinal BD at Severance Hospital, Seoul, Korea from 1992 to 2007. A therapeutic response was evaluated 8 weeks after the initiation of medical treatment, and cumulative recurrence and surgery rates were also assessed during long-term follow-up. RESULTS The initial remission rate at 8 weeks after treatment was 66.7%. During the follow-up period, cumulative recurrence rates for intestinal BD were 24.9% at 2 years and 43.0% at 5 years. The recurrence rate was significantly higher in patients with apparent gastrointestinal symptoms at their initial presentation, volcano-type and deep intestinal ulcers, and those who failed to achieve complete remission during the initial treatment. Cumulative rates for surgery were 6.7% at 2 years and 15.1% at 5 years. The typical type of ulcers was the only predictive factor for the likelihood of surgery. CONCLUSIONS Our study demonstrates that a considerable number of patients experience disease relapse during follow-up despite a high-remission rate after medical treatment for intestinal BD. Careful observation and intensive treatment should be carried out, especially in patients without complete remission after initial treatment, with deep and volcano-shaped ulcers, or with apparent gastrointestinal symptoms at the time of diagnosis.
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Kim MC, Shin SJ, Lim SG, Lee KR, Woo H, Choi SJ, Jo JS, Eum JH, Cha DY, Hwang JC, Lee KM, Lee KJ, Kim JH. Clinical Course of Intestinal Behçet's Disease according to the Characteristics of Ulcer in Colonoscopy. Intest Res 2010. [DOI: 10.5217/ir.2010.8.1.40] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 03/29/2023] Open
Affiliation(s)
- Min Cheul Kim
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Sung Jae Shin
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Sun Gyo Lim
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Kyung Rok Lee
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Hak Woo
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Sang Jo Choi
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Jung Soo Jo
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Jung Ho Eum
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Dong Youb Cha
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Jae Chul Hwang
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Ki Myung Lee
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Kwang Jae Lee
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
| | - Jin Hong Kim
- Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
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Jung YS, Cheon JH, Park JJ, Moon CM, Kim ES, Lee JH, Kim SW, Kim JH, Hong SP, Kim TI, Kim WH. Correlation of genotypes for thiopurine methyltransferase and inosine triphosphate pyrophosphatase with long-term clinical outcomes in Korean patients with inflammatory bowel diseases during treatment with thiopurine drugs. J Hum Genet 2009; 55:121-3. [PMID: 19960028 DOI: 10.1038/jhg.2009.125] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
There is a lack of research describing the associations between thiopurine methyltransferase (TPMT)/inosine triphosphate pyrophosphatase (ITPA) genotypes and long-term clinical outcomes. We investigated whether TPMT/ITPA genotypes predicted long-term clinical response in Korean patients with inflammatory bowel diseases (IBDs) undergoing thiopurine treatment. A total of 204 patients with IBD in whom thiopurine treatment was indicated were enrolled and categorized by TPMT and ITPA genotypes. Long-term follow-up clinical data for these patients were analyzed with specific focus on disease relapse. Of the 204 patients, 162 (79.4%) patients using thiopurines achieved remission and were included in an analysis of long-term clinical outcomes. There were no significant differences in disease relapse-free survival between wild and mutant types of TPMT (P=0.903) or ITPA (P=0.392), according to the results of the log-rank analysis. Our study suggests that TPMT and ITPA genotypes may not affect the rates of disease relapse in IBD patients treated with thiopurines. Further studies are indicated to confirm the utility of TPMT/ITPA genotyping to guide clinicians formulating individualized treatments for IBD patients requiring thiopurine therapy.
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Affiliation(s)
- Yoon Suk Jung
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Ariyachaipanich A, Berkelhammer C, Nicola H. Intestinal Behçet's disease: maintenance of remission with adalimumab monotherapy. Inflamm Bowel Dis 2009; 15:1769-71. [PMID: 19177427 DOI: 10.1002/ibd.20869] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
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Ebert EC. Gastrointestinal manifestations of Behçet's disease. Dig Dis Sci 2009; 54:201-7. [PMID: 18594975 DOI: 10.1007/s10620-008-0337-4] [Citation(s) in RCA: 66] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/01/2008] [Accepted: 05/06/2008] [Indexed: 12/13/2022]
Abstract
Behçet's disease is a rare vasculitis diagnosed by the presence of recurrent oral ulcers and two of the following: genital ulcers, typical eye lesions, typical skin lesions, and positive pathergy test. It is most commonly seen in countries along the ancient silk road from Eastern Asia to the Mediterranean Basin. Young adults between the second and fourth decades of life are mainly affected, with abdominal pain being the most common symptom. The ileocecal region is most commonly affected, with ulcerations that may penetrate or perforate. Rarely, the esophagus and stomach may have ulcerations. Bowel wall thickening is the most common finding on computed tomography (CT) scan. Pathology shows a vasculitis mainly involving the small veins or, alternatively, nonspecific inflammation. Corticosteroids, with or without other immunosuppressive drugs, are used for severe eye disease. Their use in intestinal disease is largely empirical. Surgery may be required for perforation. Behçet's disease runs a chronic, unpredictable course with exacerbations and remissions which decrease in frequency and severity over time. Death is mainly due to major vessel disease and neurological involvement.
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Affiliation(s)
- Ellen C Ebert
- UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08903, USA.
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Schneider A, Merikhi A, Frank BB. Autoimmune disorders: gastrointestinal manifestations and endoscopic findings. Gastrointest Endosc Clin N Am 2006; 16:133-51. [PMID: 16546029 DOI: 10.1016/j.giec.2006.01.013] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
The gastrointestinal tract can be involved in many autoimmune disorders, and women are affected more than men in most of the disease processes discussed. As this article outlines, gastrointestinal manifestations can be either part of the clinical presentation or complications of treatment. Depending on the disease process and the severity of symptoms, gastrointestinal evaluation and treatment can have an important role in the management of these diseases.
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Affiliation(s)
- Alison Schneider
- Division of Gastroenterology, Drexel University College of Medicine, Philadelphia, PA 19107, USA
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Rhee SH, Kim YB, Lee ES. Comparison of Behcet's disease and recurrent aphthous ulcer according to characteristics of gastrointestinal symptoms. J Korean Med Sci 2005; 20:971-6. [PMID: 16361807 PMCID: PMC2779329 DOI: 10.3346/jkms.2005.20.6.971] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
Behcet's disease (BD) is a multisystemic chronic inflammatory disease. It is characterized by recurrent oral and genital ulcers, uveitis, skin lesions and other manifestations, including neurologic, vascular, joint, and gastrointestinal ulcers of variable severity. Recurrent aphthous ulcer (RAU) represents a very common, but poorly understood, mucosal disorder. If a patient of RAU without any other typical symptoms of BD has gastrointestinal symptoms, it is difficult to distinguish this RAU from true BD with gastrointestinal involvement. Because pathognomonic clinical features and tools are absent, the differential diagnosis of these two diseases relies on the characteristic clinical features and the judgement of an experienced physician. Sixty-five out of a total 960 RAU patients and forty-four of 556 BD patients with gastrointestinal symptoms between January 1996 and December 2003 participated in this study. All were evaluated with esophagogastroduodenoscopy and colonoscopy. Clinical, endoscopic and histopathologic findings were analyzed and ELISA tests were conducted to detect serum levels of ASCA and pANCA. No significant difference was found between the two groups. Differential diagnosis between RAU with gastrointestinal symptoms and BD with gastrointestinal involvement requires further prospective, large-scale study.
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Affiliation(s)
- Seung-Ho Rhee
- Department of Dermatology, Ajou University School of Medicine, Suwon, Korea
| | - Young-Bae Kim
- Department of Pathology, Ajou University School of Medicine, Suwon, Korea
| | - Eun-So Lee
- Department of Dermatology, Ajou University School of Medicine, Suwon, Korea
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Alva S, Abir F, Longo WE. Colorectal manifestations of collagen vascular disease. Am J Surg 2005; 189:685-93. [PMID: 15910721 DOI: 10.1016/j.amjsurg.2004.11.036] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2004] [Revised: 11/15/2004] [Accepted: 11/15/2004] [Indexed: 11/27/2022]
Abstract
BACKGROUND The collagen vascular diseases are a collection of conditions, which are thought to be secondary to pathologic alterations in the immune system. Deposition of immune complexes in blood vessel walls resulting in either ischemia or thrombosis is the most widely accepted pathologic mechanism. The lack of familiarity with this subgroup of disease can lead to unnecessary surgical intervention. DATA SOURCES A Medline search was performed of all the English-language literature. Further references were obtained through cross-referencing the bibliography cited in each work. CONCLUSION Clinical manifestations are varied and complications include constipation, fecal incontinence, pseudoobstruction, perforation, hemorrhage, and mesenteric ischemia. Colorectal manifestations typically follow dermal presentations. Management should be conservative especially for pseudo-obstructions. Surgical intervention increases morbidity and should be chosen when absolutely necessary. Because of the high incidence of colorectal malignancies in patients with dermatomyositis, aggressive screening should be performed.
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Affiliation(s)
- Suraj Alva
- Department of Surgery, Yale University School of Medicine, PO Box 208062, New Haven, CT 06520-8062, USA
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Passam FH, Diamantis ID, Perisinaki G, Saridaki Z, Kritikos H, Georgopoulos D, Boumpas DT. Intestinal ischemia as the first manifestation of vasculitis. Semin Arthritis Rheum 2004; 34:431-41. [PMID: 15305242 DOI: 10.1016/j.semarthrit.2003.12.004] [Citation(s) in RCA: 39] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
OBJECTIVE To summarize current knowledge regarding the diagnosis and management of gastrointestinal vasculitis. METHODS Three cases of gastrointestinal vasculitis with acute abdominal ischemia as their first manifestation are presented. Underlying diseases were microscopic polyangiitis, systemic lupus erythematosus (SLE), and polyarteritis nodosa (PAN). Relevant English-language articles collected from the PubMed database were reviewed. RESULTS Among the angiitides, PAN, SLE, and Henoch-Schönlein are those most commonly accompanied by gastrointestinal complications. Intestinal vasculitis usually occurs when there is evidence of generalized disease activity. Abdominal computerized tomography is a valuable tool for diagnosing intestinal ischemia and suspected vasculitis. CONCLUSIONS In young patients presenting with intestinal ischemia, it is essential to assess the possibility of an underlying systemic disease. With prompt initiation of immunosuppressive treatment, surgery may be avoided. Prognosis is improved when there is minimal delay in surgical intervention.
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Affiliation(s)
- Freda H Passam
- Division of Internal Medicine, University Hospital of Crete, Heraklion, Greece
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Espinet E, Muñoz-Navas M, Súbtil JC, de la Riva S, Betés M, Fernández-Urién I, Carretero C. [Utility of endoscopy in digestive hemorrhage due to vasculitis]. GASTROENTEROLOGIA Y HEPATOLOGIA 2004; 27:403-7. [PMID: 15461938 DOI: 10.1016/s0210-5705(03)70487-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
Abstract
Vasculitides constitute a heterogeneous group of diseases characterized by inflammation of blood vessels. The skin is mainly affected, although the gastrointestinal tract mucosa can also be involved. The contribution of endoscopy in these cases has not been clearly determined. We report three cases of systemic vasculitis (polyarteritis nodosa, Schonlein-Henoch purpura and Behcet's disease) presenting with acute digestive bleeding. Endoscopy was an effective technique for completing the diagnosis and in establishing an effective nonsurgical therapeutic approach in these potentially lethal cases of gastrointestinal hemorrhage.
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Affiliation(s)
- E Espinet
- Unidad de Endoscopia, Servicio de Digestivo, Clínica Universitaria, Universidad de Navarra, Pamplona, Navarra, Spain.
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Barp EA, Mandracchia VJ, House MR. Behçet's disease: a case report. Clin Podiatr Med Surg 2002; 19:519-25, vi-vii. [PMID: 12471859 DOI: 10.1016/s0891-8422(02)00035-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/18/2023]
Abstract
While Behçet's disease is an uncommon presentation to a podiatric clinical setting, it is nonetheless a fascinating and poorly understood disease as demonstrated by the current and historical research available about the topic. Much debate remains about the disease cause, course and treatment goals. Only through further research into the specific components of this multisystem disease will medicine be more fully able to address the needs of the patient. This discussion should offer the podiatric physician an overview of the disease as well as available treatment options.
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Affiliation(s)
- Eric A Barp
- Broadlawns Medical Center, Des Moines, IA 50314, USA
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