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Naganuma M, Takeno M, Çelik AF, Moots R, Pinton P, Hisamatsu T. Assessment of IL-6 Pathway Inhibition in Gastrointestinal Behçet's Disease from Immunological and Clinical Perspectives. Biomedicines 2025; 13:247. [PMID: 39857830 PMCID: PMC11761229 DOI: 10.3390/biomedicines13010247] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2024] [Revised: 01/13/2025] [Accepted: 01/17/2025] [Indexed: 01/27/2025] Open
Abstract
Behçet's disease is an autoinflammatory disorder characterized by relapsing and remitting vasculitis that can manifest in various forms, including gastrointestinal Behçet's disease (GIBD). Its complications (e.g., intestinal perforation) are among the primary causes of morbidity and mortality. GIBD pathogenesis involves the enhanced production of certain cytokines, e.g., tumor necrosis factor α and interleukin-6 (IL-6), which could serve as a target for potential therapies. This review provides an overview of GIBD, including the diagnosis and immunopathogenesis as it is currently understood, and evaluates the emerging role of the inhibition of IL-6 (classic and trans-signaling) as an alternative treatment option for patients with GIBD. Given the current paucity of data, we reflected on the potential of IL-6 inhibitors such as tocilizumab and olamkicept based on immunopathogenic considerations and available clinical data in patients with inflammatory bowel disease (IBD), in whom clinical response or remission was induced. The selective inhibition of IL-6 trans-signaling may bring new impetus to the development of this drug class, particularly regarding safety. Still, the benefits of IL-6 inhibitors for patients with GIBD need to be evaluated in appropriate proof-of-concept studies. The clinical outcomes of IL-6 inhibitors in IBD are promising and may suggest their potential relevance in GIBD.
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Affiliation(s)
- Makoto Naganuma
- Third Department of Internal Medicine, Kansai Medical University, Hirakata 573-1191, Japan;
| | - Mitsuhiro Takeno
- Department of Allergy and Rheumatology, Nippon Medical School Musashi Kosugi Hospital, Kawasaki 211-8533, Japan;
| | - Aykut Ferhat Çelik
- Cerrahpaşa Medical Faculty, Istanbul University-Cerrahpaşa, Fatih, Istanbul 34320, Türkiye;
| | - Robert Moots
- Faculty of Heath Social Care and Medicine, Edge Hill University, Ormskirk, Lancashire L39 4QP, UK;
- Department of Rheumatology, Aintree University Hospital, Liverpool L9 7AL, UK
| | - Philippe Pinton
- Clinical and Translational Sciences, Ferring Pharmaceuticals, 2770 Kastrup, Denmark;
| | - Tadakazu Hisamatsu
- Department of Gastroenterology and Hepatology, Kyorin University School of Medicine, Tokyo 181-0004, Japan
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Jobran AWM, Janem A, Alhor T, Idris M, Nabilsi M, Mughrabi S, Alfakhry A, Ashhab H. Behçet's disease presented with obscure gastrointestinal bleeding: A Palestinian case report. Clin Case Rep 2024; 12:e9348. [PMID: 39206074 PMCID: PMC11347930 DOI: 10.1002/ccr3.9348] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2024] [Revised: 07/20/2024] [Accepted: 07/29/2024] [Indexed: 09/04/2024] Open
Abstract
Key Clinical Message It is important to recognize and manage gastrointestinal symptoms in patients with BD, as they are linked to high rates of mortality and morbidity. BD can affect any part of the gastrointestinal tract, and prompt diagnosis and appropriate treatment are essential to prevent complications. Additionally, the diagnosis of BD is primarily based on clinical factors due to the lack of pathognomonic laboratory tests. Abstract Behçet's disease (BD) is an idiopathic, chronic, and relapsing multi-systemic vasculitis characterized by recurrent skin lesions and eye disease. There is no pathognomonic laboratory testing; the diagnosis is made mainly on clinical factors. BD symptoms affecting the gastrointestinal (GI) system are particularly important as they are linked to high rates of mortality and morbidity. Although ileocecal involvement is most frequently mentioned, BD can affect any part of the GI tract. There are techniques for keeping track of disease activity during treatment, but they are not ideal. We present a case of a 38-year-old male patient admitted to the ICU with a history of black tarry stools of 1-month duration, fresh blood per rectum 5 to 7 times a day associated with fever, chills, and back and nonspecific joint pain for five days. The patient also experienced left eye episcleritis while being hospitalized, which was later confirmed to be BD.
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Affiliation(s)
| | | | - Tareq Alhor
- Gastroenterology fellowAl Ahli HospitalHebronPalestine
| | | | | | | | | | - Hazem Ashhab
- Faculty of MedicineAl Quds UniversityJerusalemPalestine
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Park JB, Han SJ, Lee SB, Kim DH, Cheon JH, Hwang SW, Ye BD, Yang SK, Park SJ, Park SH. Optimal Treatment Approaches to Intestinal Behçet's Disease Complicated by Myelodysplastic Syndrome: The KASID and KSBD Multicenter Study. Yonsei Med J 2024; 65:265-275. [PMID: 38653565 PMCID: PMC11045345 DOI: 10.3349/ymj.2023.0321] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/08/2023] [Revised: 12/01/2023] [Accepted: 01/02/2024] [Indexed: 04/25/2024] Open
Abstract
PURPOSE Studies on intestinal Behçet's disease (BD) complicated by myelodysplastic syndrome (MDS) are rare, and no established therapeutic guidelines exist. This study aimed to evaluate the clinical presentation and outcomes of patients with intestinal BD complicated by MDS (intestinal BD-MDS) and suggest a treatment strategy. MATERIALS AND METHODS Data from patients with intestinal BD-MDS from four referral centers in Korea who were diagnosed between December 2000 and December 2022 were retrospectively analyzed. Clinical features and prognosis of intestinal BD-MDS compared with age-, sex-matched intestinal BD without MDS were investigated. RESULTS Thirty-five patients with intestinal BD-MDS were included, and 24 (70.6%) had trisomy 8. Among the 35 patients, 23 (65.7%) were female, and the median age at diagnosis for intestinal BD was 46.0 years (range, 37.0-56.0 years). Medical treatments only benefited eight of the 32 patients, and half of the patients underwent surgery due to complications. Compared to 70 matched patients with intestinal BD alone, patients with intestinal BD-MDS underwent surgery more frequently (51.4% vs. 24.3%; p=0.010), showed a poorer response to medical and/or surgical treatment (75.0% vs. 11.4%; p<0.001), and had a higher mortality (28.6% vs. 0%; p<0.001). Seven out of 35 patients with intestinal BD-MDS underwent hematopoietic stem cell transplantation (HSCT), and four out of the seven patients had a poor response to medical treatment prior to HSCT, resulting in complete remission of both diseases. CONCLUSION Patients with intestinal BD-MDS frequently have refractory diseases with high mortalities. HSCT can be an effective treatment modality for medically refractory patients with intestinal BD-MDS.
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Affiliation(s)
- Jung-Bin Park
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - So Jung Han
- Department of Internal Medicine and Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- Division of Gastroenterology, Department of Internal Medicine, National Health Insurance Service Ilsan Hospital, Goyang, Korea
| | - Seung Bum Lee
- Department of Gastroenterology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea
| | - Dong Hyun Kim
- Division of Gastroenterology, Department of Internal Medicine, Chonnam National University Hospital, Gwangju, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Sung Wook Hwang
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Byong Duk Ye
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Suk-Kyun Yang
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Soo Jung Park
- Department of Internal Medicine and Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
| | - Sang Hyoung Park
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
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Hyun HK, Park J, Park SJ, Park JJ, Kim TI, Lee JS, Lee HW, Kim BK, Park JY, Kim DY, Ahn SH, Kim SU, Cheon JH. Neither hepatic steatosis nor fibrosis is associated with clinical outcomes in patients with intestinal Behçet's disease. Eur J Gastroenterol Hepatol 2024; 36:445-451. [PMID: 38417062 DOI: 10.1097/meg.0000000000002711] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 03/01/2024]
Abstract
BACKGROUND Behçet's disease (BD) and nonalcoholic fatty liver disease (NAFLD) are chronic inflammatory diseases that share pathogenetic mechanisms. In this study, we investigated whether NAFLD influences the clinical outcomes in patients with intestinal BD. METHODS Patients with intestinal BD and available hepatic steatosis index (HSI) and fibrosis-4 (FIB-4) scores were recruited between 2005 and 2022. An HSI of ≥30 and FIB-4 of ≥1.45 were used to diagnose hepatic steatosis and significant liver fibrosis, respectively. The primary outcomes were intestinal BD-related hospitalization, surgery, emergency room visits, or the first use of corticosteroids, immunomodulators, or biologic agents for intestinal BD. RESULTS A total of 780 patients with BD were selected. The prevalence of hepatic steatosis and significant liver fibrosis were 72.3% and 8.8%, respectively. Multivariate analysis showed that younger age, prior smoking history, concomitant skin lesions, higher white blood cell count, and lower serum albumin levels were independently associated with an increased risk of clinical relapse (all P < 0.05), whereas hepatic steatosis and significant liver fibrosis were not (hazard ratio [HR] = 1.164, 95% confidence interval [CI] 0.923-1.468; P = 0.199 for hepatic steatosis; HR = 0.982, 95% CI 0.672-1.436; P = 0.927 for significant liver fibrosis). CONCLUSION Hepatic steatosis and liver fibrotic burden were not independently associated with clinical outcomes in patients with intestinal BD.
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Affiliation(s)
- Hye Kyung Hyun
- Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin
| | - Jihye Park
- Department of Internal Medicine, Yonsei University College of Medicine
| | - Soo Jung Park
- Department of Internal Medicine, Yonsei University College of Medicine
| | - Jae Jun Park
- Department of Internal Medicine, Yonsei University College of Medicine
| | - Tae Il Kim
- Department of Internal Medicine, Yonsei University College of Medicine
- Graduate School of Medical Science, Brain Korea 21 Project, Yonsei University College of Medicine
| | - Jae Seung Lee
- Department of Internal Medicine, Yonsei University College of Medicine
- Yonsei Liver Center, Severance Hospital, Seoul, Korea
| | - Hye Won Lee
- Department of Internal Medicine, Yonsei University College of Medicine
- Yonsei Liver Center, Severance Hospital, Seoul, Korea
| | - Beom Kyung Kim
- Department of Internal Medicine, Yonsei University College of Medicine
- Yonsei Liver Center, Severance Hospital, Seoul, Korea
| | - Jun Yong Park
- Department of Internal Medicine, Yonsei University College of Medicine
- Yonsei Liver Center, Severance Hospital, Seoul, Korea
| | - Do Young Kim
- Department of Internal Medicine, Yonsei University College of Medicine
- Yonsei Liver Center, Severance Hospital, Seoul, Korea
| | - Sang Hoon Ahn
- Department of Internal Medicine, Yonsei University College of Medicine
- Yonsei Liver Center, Severance Hospital, Seoul, Korea
| | - Seung Up Kim
- Department of Internal Medicine, Yonsei University College of Medicine
- Yonsei Liver Center, Severance Hospital, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine, Yonsei University College of Medicine
- Graduate School of Medical Science, Brain Korea 21 Project, Yonsei University College of Medicine
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Zu X, Xiong S, Lu Y, Zhang N, Xu S, Feng R, Chen B, Zeng Z, Chen M, He Y. Predicting Factors of Long-term Outcome of Gastrointestinal Behçet's Disease: A Chinese Retrospective Study. Clin Ther 2024; 46:201-207. [PMID: 38296718 DOI: 10.1016/j.clinthera.2023.12.012] [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] [Received: 08/05/2023] [Revised: 12/13/2023] [Accepted: 12/14/2023] [Indexed: 02/02/2024]
Abstract
PURPOSE Behçet's disease (BD) is a complex disorder affecting multiple systems and organs, and gastrointestinal BD is poorly understood. We aimed to identify factors influencing the long-term outcomes of patients with gastrointestinal BD. METHODS Consecutive patients with gastrointestinal BD were analyzed retrospectively. Data on the following clinical characteristics were collected: sex, age at diagnosis, symptoms, endoscopic findings, medical treatments, and surgery. Mucosal healing and surgical rates at 1, 2, and 5 years were evaluated. Log-rank test and Cox proportional hazards regression models were used to evaluate the factors affecting long-term outcomes. FINDINGS Baseline data of 175 patients with gastrointestinal BD were included. The mean (SD) age at diagnosis was 38.3 (12.9) years. The typical clinical symptoms were oral ulcer (72.6%), abdominal pain (71.4%), and weight loss (41.1%). The most commonly involved location was the ileocecum; isolated oval ulcer was the most common ulcer type. Seventeen patients (9.7%) underwent 18 surgeries after inclusion. The cumulative surgical rates were 8.6% (n/N = 15/175), 8.6% (n/N = 15/175), and 9.1% (n/N = 16/175) in 1, 2, and 5 years, respectively. Data from 101 patients who underwent at least 2 endoscopies were included in the analysis for mucosal healing. Kaplan-Meier curve showed that the cumulative mucosal healing rates at 1, 2, and 5 years were 34.7% (n/N = 35/101), 41.6% (n/N = 42/101), and 61.4% (n/N = 62/101), respectively. We compared cumulative mucosal healing rates between 4 treatment groups, including 5-aminosalicylic acid (3% [n/N = 3/101]), mono-immunosuppressant (31.7% [n/N = 32/101]), combined therapy (36.6% [n/N = 37/101]), and escalation therapy (28.7% [n/N = 29/101]), and found that mono-immunosuppressant achieved earlier mucosal healing than combined therapy (P = 0.0008) and escalation therapy (P = 0.0008). The univariate analysis showed that moderate to severe disease activity (P = 0.013, P = 0.004), diameter of the maximal ulcer >4 cm (P = 0.002), and nonsimple esophageal involvement (P < 0.001) were risk factors, and number of ulcers between 2 and 5 was the protective factor of mucosal healing (P = 0.001). Multivariate regression analysis indicated that nonsimple esophageal involvement (P < 0.001) and the maximal ulcer >4 cm (P = 0.041) were independent risk factors of mucosal healing. IMPLICATIONS Most patients with gastrointestinal BD need long-term treatment to achieve mucosal healing. The location and size of ulcers have a significant impact on the mucosal healing of gastrointestinal BD.
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Affiliation(s)
- Xiaoman Zu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Shanshan Xiong
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Yaming Lu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Ning Zhang
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Shu Xu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Rui Feng
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Baili Chen
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Zhirong Zeng
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Minhu Chen
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Yao He
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
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Park MY, Yoon YS, Park JH, Lee JL, Yu CS. Short- and long-term outcomes of surgical treatment in patients with intestinal Behcet's disease. World J Gastrointest Surg 2024; 16:429-437. [PMID: 38463356 PMCID: PMC10921202 DOI: 10.4240/wjgs.v16.i2.429] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2023] [Revised: 11/29/2024] [Accepted: 01/15/2024] [Indexed: 02/25/2024] Open
Abstract
BACKGROUND Behcet's disease (BD), a chronic vasculitic disorder affecting multiple organs, is characterized by recurrent oral and genital ulcers, arthritis, vasculitis, and intestinal ulcers. Although intestinal involvement of BD is common in East Asia, the efficacy and long-term outcomes of surgical treatment of intestinal BD still remain to be established. AIM To evaluate the postoperative clinical course of intestinal BD and determine factors associated with its recurrence. METHODS Data from patients who underwent surgical treatment for intestinal BD between January 2010 and August 2021 were retrospectively reviewed. Patients' demographics, clinical features, postoperative course, complications, and follow-up data were evaluated. RESULTS We analyzed 39 surgeries in 31 patients. The mean patient age was 45.1 years, and the mean interval between the diagnosis of intestinal BD and surgical treatment was 4.9 years (range 1.0-8.0 years). The most common indication for surgery was medical intractability (n = 16, 41.0%), followed by fistula or abscess (n = 11, 28.2%). Laparoscopic approaches were used in 19 patients (48.7%), and 5 patients (12.8%) underwent emergency surgeries. The most common surgical procedure was ileocecal resection (n = 18, 46.2%), followed by right colectomy (n = 11, 28.2%). A diverting stoma was created in only one patient (2.6%). During a mean follow-up period of 45 (range 8-72) months, eight cases (20.5%) of recurrence in five patients required reoperation. The interval between operations was 12.1 months (range 6.3-17.8 mo). Four patients (10.3%) experienced recurrence within 1 year postoperatively, and all eight recurrences occurred within 2 years of the initial surgery. The reoperation rates at 1 and 3 years were 10.3% and 20.5%, respectively. A redo ileocolic anastomosis was performed in all recurrent cases. In multivariate Cox regression analysis, emergency surgery [hazard ratio (HR) 9.357, 95% confidence interval (CI): 1.608-54.453, P = 0.013] and elevated C-reactive protein (CRP) levels (HR 1.154, 95%CI: 1.002-1.328, P = 0.047), but not medication use, were predictors of recurrence. CONCLUSION Surgical resection is a feasible treatment option for complicated BD. Reoperation is associated with severe inflammatory conditions, reflected by increased CRP levels and the requirement for emergency surgery.
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Affiliation(s)
- Min Young Park
- Division of Colon and Rectal Surgery, Department of Surgery, Yonsei University College of Medicine, Seoul 03722, South Korea
| | - Yong Sik Yoon
- Division of Colon and Rectal Surgery, Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul 05505, South Korea
| | - Jae Ha Park
- Division of Colon and Rectal Surgery, Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul 05505, South Korea
| | - Jong Lyul Lee
- Division of Colon and Rectal Surgery, Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul 05505, South Korea
| | - Chang Sik Yu
- Division of Colon and Rectal Surgery, Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul 05505, 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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Park J, Park SJ, Park JJ, Kim TI, Cheon JH. Long-term clinical outcomes of intestinal Behçet's disease: A 30-year cohort study at a tertiary hospital in South Korea. J Gastroenterol Hepatol 2023; 38:386-392. [PMID: 36346041 DOI: 10.1111/jgh.16056] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/05/2022] [Revised: 10/15/2022] [Accepted: 11/05/2022] [Indexed: 11/11/2022]
Abstract
BACKGROUND AND AIM We aimed to identify the long-term clinical outcomes of and prognostic factors for intestinal Behçet's disease (BD). METHODS A cohort of 780 patients with intestinal BD between 1997 and 2021 was investigated to determine long-term clinical outcomes and prognostic factors at an inflammatory bowel disease clinic at Severance Hospital, Seoul, Korea. RESULTS During the median follow-up period of 12.7 ± 7.2 years, 5-aminosalicylic acids, corticosteroids, immunomodulators, and anti-tumor necrosis factor-alpha (TNF-α) agents were required in 94.9%, 67.2%, 43.8%, and 14.6% of the patients, respectively. The cumulative rates of anti-TNF-α use were 3.7%, 7.5%, 8.5%, 12.1%, 17.6%, and 24.0%, and those for abdominal surgery were 5.7%, 10.9%, 12.6%, 16.5%, 21.6%, and 28.3%, at 1, 3, 5, 10, 20, and 30 years, respectively, after initial diagnosis of intestinal BD. The cumulative rates of hospitalization were 11.8%, 21.9%, 27.9%, 38.8%, 54.4%, and 74.8%, and those of emergency room visits were 10.0%, 19.8%, 22.7%, 31.6%, 50.0%, and 65.0% at 1, 3, 5, 10, 20, and 30 years. Older age at primary diagnosis, previous appendectomy history, higher disease activity index for intestinal Behçet's disease score, systemic BD, multiple intestinal ulcers, deep intestinal ulcers, higher C-reactive protein, lower hemoglobin, and lower albumin levels were associated with poor prognosis. Married status, higher body mass index, oral ulceration, and arthritis were negatively associated with poor prognosis. CONCLUSIONS Data on the long-term clinical outcomes of intestinal BD and their prognostic factors could guide physicians in patient monitoring and in optimizing individualized treatment.
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Affiliation(s)
- Jihye Park
- Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.,Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - Soo Jung Park
- Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.,Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - Jae Jun Park
- Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.,Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - Tae Il Kim
- Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.,Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - Jae Hee Cheon
- Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.,Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
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Wang Z, Zu X, Xiong S, Mao R, Qiu Y, Chen B, Zeng Z, Chen M, He Y. The Role of Colchicine in Different Clinical Phenotypes of Behcet Disease. Clin Ther 2023; 45:162-176. [PMID: 36732153 DOI: 10.1016/j.clinthera.2023.01.004] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2022] [Revised: 12/29/2022] [Accepted: 01/11/2023] [Indexed: 02/04/2023]
Abstract
PURPOSE Behcet disease (BD) is a multisystemic disorder characterized by variable clinical manifestations that affect nearly all systems and organs. Colchicine, an alkaloid plant extract, is considered as the first-line therapy for gout, pericarditis, and familial Mediterranean fever. However, the role of colchicine in the treatment of different clinical phenotypes of BD has not been clearly described. This narrative review summarizes the clinical use of colchicine in BD. METHODS All relevant literature from 1980 to March 2021 was searched in PubMed, MEDLINE, and Cochrane Library. The Medical Subject Heading terms and related words that were searched are as follows: Behcet's disease, Behcet's syndrome, BD, colchicine, management, treatment, and therapy. FINDINGS BD is an autoimmune systemic vasculitis with various clinical phenotypes, with involvement of skin mucosa, joints, eyes, and gastrointestinal, vascular, and neurologic systems. Colchicine has been used for centuries, acts by binding to tubulin to prevent the mitotic process, and has anti-inflammatory, antitumor, and antifibrotic properties. Colchicine has been reported to be an effective option for the treatment of skin, mucosal, and joint involvement in patients with certain BD clinical phenotypes. IMPLICATIONS Colchicine reduces the severity of certain clinical phenotypes and may improve the overall disease activity index in patients with BD. More randomized clinical trials are needed to confirm the value of colchicine in the treatment of BD, and further elucidation of the mechanisms is also needed, which may reveal new application of colchicine that has been used for centuries.
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Affiliation(s)
- Zeyuan Wang
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China; Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China
| | - Xiaoman Zu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Shanshan Xiong
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Ren Mao
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Yun Qiu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Baili Chen
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Zhirong Zeng
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Minhu Chen
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Yao He
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
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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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He K, Wu D. The treatment principles and targets for intestinal Behcet's disease. Therap Adv Gastroenterol 2023; 16:17562848231167283. [PMID: 37113192 PMCID: PMC10126606 DOI: 10.1177/17562848231167283] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2023] [Accepted: 03/14/2023] [Indexed: 04/29/2023] Open
Abstract
Behcet's disease (BD) is a chronic and recurrent systemic vasculitis involving large, medium and small blood vessels as well as arteries and veins. BD with predominant gastrointestinal manifestations is diagnosed as intestinal BD, which is associated with severe complications such as massive gastrointestinal hemorrhage, perforation, and obstruction. Recently, treat-to-target (T2T) strategies have been successfully used in many chronic diseases and been suggested in the management of BD, while there are no related reviews about the global treatment strategy including treatment principles and targets for intestinal BD in detail. Herein, we review the treatment principles from the aspects of departments of Rheumatology and Gastroenterology. In addition, treatment targets of intestinal BD are reviewed from three aspects such as evaluable markers, effective markers and potency-ratio markers. Some definitions and conceptions from inflammatory bowel disease (IBD) bring us reference and enlightenments.
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Affiliation(s)
- Kun He
- Department of Gastroenterology, Peking Union
Medical College Hospital, Chinese Academy of Medical Sciences and Peking
Union Medical College, Beijing, China
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13
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Joo YY, Lee BI, Kim SJ, Lee HH, Kim JS, Park JM, Cho YS, Lee KM, Kim SW, Choi H, Choi MG. Clinical Course of Patients with Intestinal Behçet's Disease According to Consensus-Based Diagnostic Categories. Gut Liver 2022; 16:746-753. [PMID: 34916337 PMCID: PMC9474495 DOI: 10.5009/gnl210308] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2021] [Revised: 09/23/2021] [Accepted: 09/24/2021] [Indexed: 11/04/2022] Open
Abstract
BACKGROUND/AIMS There have been few studies regarding the prognosis of intestinal Behçet's disease (iBD) patients according to consensus-based diagnostic categories, which reflects the typicality of intestinal ulcers, the presence of oral ulcers, and the accompanying systemic manifestations. METHODS The medical records of patients who had ileocolonic ulcers with a clinical impression of iBD were reviewed. The patients were categorized according to the diagnostic algorithm at the time of diagnosis. Adverse events were defined as major surgery or admission related to iBD deterioration. RESULTS A total of 163 patients were included in the study. The male-to-female ratio was 1:1.2, and the mean age at the time of diagnosis was 48.9±15.9 years. The numbers of patients who met the definite, probable, suspected, and nondiagnostic iBD criteria were 19 (11.7%), 61 (37.4%), 38 (23.3%), and 45 (27.6%), respectively. The event-free survival of patients with definite, probable, and suspected iBD was significantly shorter than that of patients with nondiagnostic iBD (p=0.026), while there was no significant difference among the definite iBD, probable iBD, and suspected iBD groups (p=0.596). After excluding patients with nondiagnostic iBD, multivariate analysis showed that anemia, fever, colonic involvement other than the ileocecum, and accompanying hematologic disorders at the time of diagnosis were significantly associated with the development of adverse events. CONCLUSIONS The clinical course of patients with definite, probable, and suspected iBD is distinguished from that of patients with nondiagnostic iBD, but patients with definite, probable, and suspected iBD share similar clinical courses.
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Affiliation(s)
- Yu Young Joo
- Division of Gastroenterology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Bo-In Lee
- Division of Gastroenterology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
- Catholic Photomedicine Research Institute, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Seung-Jun Kim
- Division of Gastroenterology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Han Hee Lee
- Division of Gastroenterology, Department of Internal Medicine, Yeouido St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Jin Su Kim
- Division of Gastroenterology, Department of Internal Medicine, Eunpyeong St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Jae Myung Park
- Division of Gastroenterology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
- Catholic Photomedicine Research Institute, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Young-Seok Cho
- Division of Gastroenterology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
- Catholic Photomedicine Research Institute, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Kang Moon Lee
- Division of Gastroenterology, Department of Internal Medicine, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Sang Woo Kim
- Division of Gastroenterology, Department of Internal Medicine, Uijeongbu St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Hwang Choi
- Division of Gastroenterology, Department of Internal Medicine, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Myung-Gyu Choi
- Division of Gastroenterology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
- Catholic Photomedicine Research Institute, College of Medicine, The Catholic University of Korea, Seoul, Korea
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14
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Zhan S, Liu C, Li N, Li T, Tian Z, Zhao M, Wu D, Chen M, Zeng Z, Zhuang X. Anti-TNF-α agents for refractory intestinal Behçet's disease: case series and meta-analysis. Therap Adv Gastroenterol 2022; 15:17562848221116666. [PMID: 36082178 PMCID: PMC9445467 DOI: 10.1177/17562848221116666] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/27/2022] [Accepted: 07/11/2022] [Indexed: 02/04/2023] Open
Abstract
Background Behçet's disease (BD) is a relapsing systemic immune disorder, and intestinal BD is a significant cause of mortality in patients with BD. Conventional therapeutic strategies for intestinal BD showed unsatisfactory outcomes, especially in those patients with refractory subtypes. In recent years, biologic agents have exhibited promising results in this field. While the sample sizes of existing studies were limited, the results were heterogeneous. Objectives This study aimed to observe the efficacy of different biologics in clinical symptomatic improvement and intestinal mucosal healing. Design This is a study including the report of case series and meta-analysis. Data sources and methods This meta-analysis was conducted following the PRISMA guidelines. Free-text words and subject terms, including 'Behcet's Syndrome', 'Biologics', 'Tumor Necrosis Factor Antagonist', were used to systematically research the relevant studies in the electronic databases (PubMed, Web of Science, Embase, and Cochrane Library). All retrieved articles were from inception to July 2021, and the data from our institution were also included in this meta-analysis. A double arcsine transformation was performed to stabilize the variance of the original ratio. Heterogeneity was evaluated via Q-test and I 2 statistics. Random-effects or fixed-effects model was used to calculate the pooled parameters, and the results were presented as forest plots with 95% confidence intervals. Results Twelve studies were included, but only antitumor necrosis factor alpha (anti-TNF-α) agents were prescribed as biologicals for refractory intestinal BD. The symptom improvement rates at our institution ranged from 57.1 to 81.8%, and the mucosal healing rates were from 20 to 60% in different therapeutic periods. A total of 514 patients were enrolled in the meta-analysis, and the synthesized ratios showed that 59.8% (n = 377) and 73.7% (n = 317) of patients who received maintenance therapy with anti-TNF-α agents could achieve clinical symptomatic improvement during short-term (10-14 weeks) and long-term (48-54 weeks) periods, respectively. Furthermore, 77.8% (n = 229) of patients with intestinal BD maintained therapeutic efficacy for a longer time (100 weeks). In addition, 60.9% (n = 120) of the patients achieved sustained intestinal mucosal healing during a long-term follow-up (24-52 weeks). Conclusion Anti-TNF-α treatment is effective in treating refractory intestinal BD but more studies are required to evaluate the effects of new biologics for intestinal BD in the near future. Registration This study has been registered on PROSPERO, the ID is CRD42022329211. Plain language summary Anti-TNF-α agents are effective and safe in patients with intestinal Behçet's disease Behçet's disease (BD) is a disease affecting several organs including the gastrointestinal tract. Nowadays, the efficacy of existing therapy strategies is still unsatisfactory and some patients are suffering from repeated attacks of the disease. We noticed that a new kind of medicine, called antitumor necrosis factor alpha (anti-TNF-α) agents, was applied to these patients recently. The therapeutic efficacy is not convincing enough to evaluate since the number of patients receiving this new medicine was small in every individual study. Regarding this, we conducted a research to learn about the efficacy of this medicine at our own institution. Besides, we composed the results of other studies in an appropriate way. Then, we drew a conclusion on the exact efficacy of anti-TNF-α agents after the data analysis. We unveiled that the anti-TNF-α agents appeared both effective and safe in the management of intestinal BD patients when the classical therapy failed. More than half of the patients could achieve discomfort remission when they got the therapy of the new medicine at our institution. We also found that intestinal ulcers in most patients improved after they received the treatment. All in all, it offered another foothold for getting relief in these patients who were caught in this mire.
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Affiliation(s)
- Shukai Zhan
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Caiguang Liu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Na Li
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Tong Li
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Zhenyi Tian
- Department of Gastroenterology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Min Zhao
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Dongxuan Wu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Minhu Chen
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Zhirong Zeng
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, No. 58 Zhongshan Road 2, Guangzhou, 510080, Guangdong, China
| | - Xiaojun Zhuang
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, No. 58 Zhongshan Road 2, Guangzhou, 510080, Guangdong, China
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15
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Ciscato A, Sadeghi S, Speziale A, Hosseini M, Hu J. Behcet's Disease Mimicking Crohn's Disease: A Diagnostic Pitfall in GI Tract Biopsies. Int J Surg Pathol 2022:10668969221113477. [PMID: 35898181 DOI: 10.1177/10668969221113477] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Behcet's disease is a rare entity. It's a multi-systemic inflammatory disease of unknown etiology characterized by recurrent ulcers and vasculitis, mainly including the oral cavity, eyes, gastrointestinal tract and joints. Crohn's disease is a chronic inflammatory disorder that may affect the same organs as Behcet's disease, however more frequently the gastrointestinal tract. Distinguishing Behcet's disease from Crohn's disease can be challenging due to the overlapping clinical presentation and similar morphology features on pathology biopsy specimens. This is a case report of a 32-year-old female who first presented at the emergency department with fatigue, weight loss, arthralgia, and erythema nodosum. The patient was admitted for oral ulcers, skin rash, genital ulcers, and melena one month later. Treatment with prednisone was started at the time and further workup for Behcet's disease versus inflammatory bowel disease was started. Her esophagus biopsy shows granulomatous-like vasculitis, and her colon biopsies show overlapping features with Crohn's disease. Herein, we present a rare and interesting case in which Behcet's disease mimics inflammatory bowel disease on the gastrointestinal tract biopsies but with some unique findings and diagnostic pitfalls for gastrointestinal tract vasculitis and ulceration.
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Affiliation(s)
- Andreas Ciscato
- Department of Pathology, 8784University of California San Diego, La Jolla, CA, USA
| | - Saha Sadeghi
- Department of Pathology, 21380Sharp Memorial Hospital, San Diego, CA, USA
| | - Alissa Speziale
- Gastroenterology, 7123Sharp Rees-Stealy Medical Center, San Diego, CA, USA
| | - Mojgon Hosseini
- Department of Pathology, 8784University of California San Diego, La Jolla, CA, USA
| | - Jingjing Hu
- Department of Pathology, 8784University of California San Diego, La Jolla, CA, USA
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16
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Liu Z, Yang C, Bai X, Shen K, Qiao L, Wang Q, Yang H, Qian J. Clinical features and prognosis of patients with gastrointestinal Behcet's disease-like syndrome and myelodysplastic syndrome with and without trisomy 8. Semin Arthritis Rheum 2022; 55:152039. [DOI: 10.1016/j.semarthrit.2022.152039] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/20/2022] [Revised: 05/23/2022] [Accepted: 05/31/2022] [Indexed: 11/28/2022]
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Gupta S, Allegretti JR. Mimics of Crohn's Disease. Gastroenterol Clin North Am 2022; 51:241-269. [PMID: 35595413 DOI: 10.1016/j.gtc.2021.12.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/21/2023]
Abstract
Crohn's disease is a chronic inflammatory disease that can affect any portion of the gastrointestinal tract. Associated symptoms can vary based on the severity of disease, extent of involvement, presence of extraintestinal manifestations, and development of complications. Diagnosis is based on a constellation of findings. Many diseases can mimic Crohn's disease and lead to diagnostic conundrums. These include entities associated with the gastrointestinal luminal tract, vascular disease, autoimmune processes, various infections, malignancies and complications, drug- or treatment-induced conditions, and genetic diseases. Careful consideration of possible causes is necessary to establish the correct diagnosis.
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Affiliation(s)
- Sanchit Gupta
- Division of Gastroenterology, Hepatology, and Endoscopy, Brigham and Women's Hospital, 850 Boyslton Street, Suite 201, Chestnut Hill, MA 02467, USA; Harvard Medical School, 25 Shattuck Street, Boston, MA 02115, USA
| | - Jessica R Allegretti
- Division of Gastroenterology, Hepatology, and Endoscopy, Brigham and Women's Hospital, 850 Boyslton Street, Suite 201, Chestnut Hill, MA 02467, USA; Harvard Medical School, 25 Shattuck Street, Boston, MA 02115, USA.
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18
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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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19
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Prevention of well-leg compartment syndrome following lengthy medical operations in the lithotomy position. Surg Open Sci 2020; 3:16-21. [PMID: 33305248 PMCID: PMC7709791 DOI: 10.1016/j.sopen.2020.10.001] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2020] [Revised: 09/26/2020] [Accepted: 10/17/2020] [Indexed: 11/23/2022] Open
Abstract
Purpose Compartment syndrome that occurs after lengthy surgery in the lithotomy position is known as well-leg compartment syndrome. It has serious consequences for patients, including amyotrophic renal failure, limb loss, and sometimes even death. This study aimed to identify effective preventive measures against well-leg compartment syndrome using a retrospective cohort study of 1,951 patients (985 and 966 in the prevention and control groups, respectively). Material and methods The following preventive interventions were analyzed: (1) changing from the lithotomy position to the open-leg position, (2) removing lower leg pressure caused by the lithotomy position, (3) limiting leg elevation based on the height of the right atrium, (4) horizontally repositioning the operating table every 3 hours, and (5) decompressing the contact area of the lower leg in the lithotomy position during operation. Results Eight cases of well-leg compartment syndrome occurred in the control group, whereas no well-leg compartment syndrome occurred in the prevention group. Conclusion These findings suggest that the five interventions assessed can prevent the development of well-leg compartment syndrome.
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20
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Lip A, Stelfox HT, Au S. A mixed methods study to effectively utilize trigger tools in the ICU. J Crit Care 2020; 61:57-62. [PMID: 33096346 DOI: 10.1016/j.jcrc.2020.09.033] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/29/2020] [Revised: 08/23/2020] [Accepted: 09/28/2020] [Indexed: 11/16/2022]
Abstract
PURPOSE This study aimed to create a trigger tool for our intensive care units (ICUs) to support our departmental quality improvement efforts. METHODS We compiled an initial list of triggers used in an ICU setting through literature review. We used a modified Delphi method to develop a unique set of triggers. An expert panel was selected for multidisciplinary and multi-site representation from four adult medical-surgical ICUs of a Canadian city. Respondents ranked triggers on a Likert scale based on its likelihood of being associated with adverse event (sensitivity to harm), and likelihood of being associated with suboptimal ICU processes (specificity for internal recommendations). OUTCOMES Our literature search yielded 10 articles and 59 triggers. Completion of the rating process resulted in 12 items that achieved consensus. Triggers included specific clinical, hospital-acquired infection, medication related, and procedural occurrences. One additional trigger (cardiopulmonary arrest) which consistently held high scores but did not achieve multidisciplinary consensus, was also included. CONCLUSIONS We used the modified Delphi process to derive consensus-selected triggers to identify ICU specific adverse events with opportunity for improvement in local care. This methodology can be adopted by other centers looking to introduce trigger tools in a manner selective to their practice needs.
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Affiliation(s)
- Alyssa Lip
- Department of Medicine, University of Calgary, Calgary, Alberta, Canada.
| | - Henry T Stelfox
- Department of Critical Care Medicine, University of Calgary, Calgary, Alberta, Canada; O'Brien Institute for Public Health, University of Calgary, Calgary, Alberta, Canada; Alberta Health Services-Calgary Zone, Calgary, Alberta, Canada
| | - Selena Au
- Department of Critical Care Medicine, University of Calgary, Calgary, Alberta, Canada
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Wu JHC, Langford B, Ha R, Garber G, Daneman N, Johnstone J, McIsaac W, Sharpe S, Tu K, Schwartz KL. Defining appropriate antibiotic prescribing in primary care: A modified Delphi panel approach. JOURNAL OF THE ASSOCIATION OF MEDICAL MICROBIOLOGY AND INFECTIOUS DISEASE CANADA = JOURNAL OFFICIEL DE L'ASSOCIATION POUR LA MICROBIOLOGIE MEDICALE ET L'INFECTIOLOGIE CANADA 2020; 5:61-69. [PMID: 36338183 PMCID: PMC9602887 DOI: 10.3138/jammi.2019-0023] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/20/2019] [Accepted: 12/05/2019] [Indexed: 06/16/2023]
Abstract
BACKGROUND Antimicrobial overuse contributes to antimicrobial resistance. In the ambulatory setting, where more than 90% of antibiotics are dispensed, there are no Canadian benchmarks for appropriate use. This study aims to define the expected appropriate outpatient antibiotic prescribing rates for three age groups (<2, 2-18, >18 years) using a modified Delphi method. METHODS We developed an online questionnaire to solicit from a multidisciplinary panel (community-academic family physicians, adult-paediatric infectious disease physicians, and antimicrobial stewardship pharmacists) what percentage of 23 common clinical conditions would appropriately be treated with systemic antibiotics followed with in-person meetings to achieve 100% consensus. RESULTS The panelists reached consensus for one condition online and 22 conditions face-to-face, which took an average of 2.6 rounds of discussion per condition (range, min-max 1-5). The consensus for appropriate systemic antibiotic prescribing rates were, for pneumonia, pyelonephritis, non-purulent skin and soft tissue infections (SSTI), other bacterial infections, and reproductive tract infections, 100%; urinary tract infections, 95%-100%; prostatitis, 95%; epididymo-orchitis, 85%-88%; chronic obstructive pulmonary disease, 50%; purulent SSTI, 35%-50%; otitis media, 30%-40%; pharyngitis, 18%-40%; acute sinusitis, 18%-20%; chronic sinusitis, 14%; bronchitis, 5%-8%; gastroenteritis, 4%-5%; dental infections, 4%; eye infections, 1%; otitis externa, 0%-1%; and asthma, common cold, influenza, and other non-bacterial infections (0%). (Note that some differed by age group.). CONCLUSIONS This study resulted in expert consensus for defined levels of appropriate antibiotic prescribing across a broad set of outpatient conditions. These results can be applied to community antimicrobial stewardship initiatives to investigate the level of inappropriate use and set targets to optimize antibiotic use.
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Affiliation(s)
| | | | - Rita Ha
- Public Health Ontario, Toronto, Ontario, Canada
| | - Gary Garber
- Public Health Ontario, Toronto, Ontario, Canada
- Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
| | - Nick Daneman
- Public Health Ontario, Toronto, Ontario, Canada
- Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
| | - Jennie Johnstone
- Public Health Ontario, Toronto, Ontario, Canada
- Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
| | - Warren McIsaac
- Department of Family and Community Medicine, University of Toronto, Toronto, Ontario, Canada
- Sinai Health System, Toronto, Ontario, Canada
| | - Sally Sharpe
- Four Villages Community Health Centre, Toronto, Ontario, Canada
| | - Karen Tu
- Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
- North York General Hospital, Toronto, Ontario, Canada
| | - Kevin L Schwartz
- Public Health Ontario, Toronto, Ontario, Canada
- Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada
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Feng R, Xu PP, Chen BL, Mao R, Zhang SH, Qiu Y, Zeng ZR, Chen MH, He Y. CYP2C19 polymorphism has no correlation with the efficacy and safety of thalidomide in the treatment of immune-related bowel disease. J Dig Dis 2020; 21:98-103. [PMID: 31916702 DOI: 10.1111/1751-2980.12842] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/14/2018] [Revised: 12/21/2019] [Accepted: 12/23/2019] [Indexed: 12/11/2022]
Abstract
OBJECTIVE To explore the relationship between hepatic cytochrome P450 2C19 (CYP2C19) gene polymorphisms and the effectiveness and safety of thalidomide in the treatment of patients with immune-related bowel disease (IRBD). METHODS CYP2C19 variants in 79 patients treated with thalidomide were analyzed using a polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). The clinical response and adverse events of the thalidomide treatment were recorded. The potential influences of the CYP2C19 genotype polymorphisms on the clinical efficacy and adverse events of thalidomide were then investigated. RESULTS Altogether 79 patients with IRBD (70 with Crohn's disease, three with ulcerative colitis and six with Behcet's disease) receiving thalidomide therapy were recruited from January 2013 to February 2015 in a tertiary IBD center in China. Overall, 21.5% (17/79) of these patients had CYP2C19 poor metabolizers genotype (PM). The overall response rate and the incidence of adverse events of CYP2C19 extensive metabolizers genotype were not significantly different from that of the PM when IRBD patients were treated with thalidomide (P = 0.517 and 0.816, respectively). CONCLUSION CYP2C19 polymorphisms do not seem to be associated with efficacy of thalidomide and the incidence of adverse events in treating IRBD.
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Affiliation(s)
- Rui Feng
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Ping Ping Xu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China.,Department of Gastroenterology, The First Affiliated Hospital of Wannan Medical College, Wuhu, Anhui Province, China
| | - Bai Li Chen
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Ren Mao
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Sheng Hong Zhang
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Yun Qiu
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Zhi Rong Zeng
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Min Hu Chen
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Yao He
- Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
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23
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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: 49] [Impact Index Per Article: 9.8] [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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24
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Kinoshita H, Nishioka H, Ikeda A, Ikoma K, Sameshima Y, Ohi H, Tatsuno M, Kouyama J, Kawamoto C, Mitsui T, Tamura Y, Hashimoto Y, Nishio M, Ogashiwa T, Saigusa Y, Maeda S, Kimura H, Kunisaki R, Koike K. Remission induction, maintenance, and endoscopic outcome with oral 5-aminosalicylic acid in intestinal Behçet's disease. J Gastroenterol Hepatol 2019; 34:1929-1939. [PMID: 31017728 DOI: 10.1111/jgh.14690] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/25/2019] [Revised: 04/12/2019] [Accepted: 04/15/2019] [Indexed: 02/06/2023]
Abstract
BACKGROUND AND AIM Oral 5-aminosalicylic acid (5-ASA) is recommended for the therapy of mild to moderate intestinal Behçet's disease (BD). However, the induction remission efficacy and endoscopic outcomes of 5-ASA are unknown. We investigated remission induction at 8 weeks, endoscopic outcomes until 52 weeks, and event-free survival at 52 weeks in patients with intestinal BD treated with 5-ASA. METHODS Forty-one patients with intestinal BD were treated with oral 5-ASA. Clinical remission was evaluated with the Crohn's disease activity index (CDAI). The endoscopic response was evaluated using the modified global gastrointestinal endoscopic assessment scores. Rescue therapy-free survival and surgery-free survival at 52 weeks were estimated, and predictive factors for a clinical response at weeks 8 and 52 were identified. RESULTS Seven patients (17%) withdrew 5-ASA early (≤ 8 weeks) because of adverse events. At week 8, clinical efficacy could be accurately evaluated in 28 patients, and the response and remission rates were 61% and 57%, respectively, using the CDAI. Endoscopic evaluation was achieved in 17 patients up to 52 weeks, and the endoscopic response and remission rates were 71% and 35%, respectively. The probabilities of rescue therapy-free survival and surgery-free survival were 73% and 100%, respectively, at 52 weeks in all 41 patients. The predictive factors for therapeutic effectiveness at week 8 were a higher baseline C-reactive protein level and CDAI, but they were negative predictive factors for a 52-week response. CONCLUSIONS 5-ASA is effective for clinical and endoscopic induction and maintaining a response in patients with mild to moderate intestinal BD.
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Affiliation(s)
- Hiroto Kinoshita
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan.,Department of Gastroenterology, The University of Tokyo, Tokyo, Japan
| | - Hitomi Nishioka
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Aya Ikeda
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Kyoko Ikoma
- Department of Gastroenterology, Idzuro Imamura Hospital, Kagoshima, Japan
| | - Yoichi Sameshima
- Department of Gastroenterology, Idzuro Imamura Hospital, Kagoshima, Japan
| | - Hidehisa Ohi
- Department of Gastroenterology, Idzuro Imamura Hospital, Kagoshima, Japan
| | - Mizuki Tatsuno
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Junka Kouyama
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Chiaki Kawamoto
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Tomohiro Mitsui
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Yuko Tamura
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Yu Hashimoto
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Masashi Nishio
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Tsuyoshi Ogashiwa
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Yusuke Saigusa
- Department of Biostatistics, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Shin Maeda
- Department of Gastroenterology, Department of Medicine, Yokohama City University, Yokohama, Japan
| | - Hideaki Kimura
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Reiko Kunisaki
- Inflammatory Bowel Disease Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Kazuhiko Koike
- Department of Gastroenterology, The University of Tokyo, Tokyo, Japan
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25
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Elrobaa I, Elserhy M, Ahmad M, Subidar M, Alhadi H. Al Wakra emergency presentation of Behcet's disease. AME Case Rep 2019; 3:34. [PMID: 31616862 DOI: 10.21037/acr.2019.08.02] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2019] [Accepted: 08/06/2019] [Indexed: 11/06/2022]
Abstract
This is atypical presentation of Behcet's disease and it's important for emergency physician and doctors who are working in acute medicine. We reported a case of a young patient presenting back pain concerning the right leg, a result of extensive bilateral deep vein thrombosis (DVT) in both legs that extended to inferior vena cava (IVC) and renal veins. After a bundle of investigations and medical examinations, the case was diagnosed as Behcet's disease with an atypical presentation. Al Wakra emergency presentation of Behcet's disease: low back pain with lower limb pain that result of DVT which is one of vascular complication of Behcet's disease.
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Affiliation(s)
- Islam Elrobaa
- Al Wakra Emergency Department, Al Wakra Hospital, Hamad Medical Corporation, Al Wakrah, Qatar
| | - Mohamed Elserhy
- Al Wakra Emergency Department, Al Wakra Hospital, Hamad Medical Corporation, Al Wakrah, Qatar
| | - Muayad Ahmad
- Al Wakra Emergency Department, Al Wakra Hospital, Hamad Medical Corporation, Al Wakrah, Qatar
| | - Mohamed Subidar
- Al Wakra Emergency Department, Al Wakra Hospital, Hamad Medical Corporation, Al Wakrah, Qatar
| | - Hassan Alhadi
- Al Wakra Emergency Department, Al Wakra Hospital, Hamad Medical Corporation, Al Wakrah, Qatar
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26
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Shahram F, Mæhlen MT, Akhlaghi M, Davatchi F, Liao YJ, Weyand CM. Geographical variations in ocular and extra-ocular manifestations in Behçet's disease. Eur J Rheumatol 2019; 6:199-206. [PMID: 31329543 DOI: 10.5152/eurjrheum.2019.18215] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2019] [Accepted: 05/16/2019] [Indexed: 01/12/2023] Open
Abstract
OBJECTIVE Behçet's disease (BD) is a rare vasculitis that results in multi-organ inflammatory disease. At-risk populations are most prevalent in the Middle East and East Asia. Clinical data on BD in Western countries, especially in the United States, are scarce. We have compared clinical patterning of BD vasculitis in two geographically defined patient cohorts in the Western United States and Iran. METHODS Comparative analysis of a retrospective cohort of 56 patients with BD evaluated at Stanford University Hospital between 2000 and 2016 and a cohort of 163 patients from the BD Registry at Tehran University of Medical Sciences. Clinical, demographic, laboratory, and treatment data were available. Comparisons were performed using descriptive statistics, Student's t-test, and χ2-test. RESULTS The Stanford patients with BD were significantly younger at disease onset, had a higher proportion of females, and had longer disease duration than Iranian patients with BD. Genital ulcers, skin, joint, neurological, vascular, cardiopulmonary manifestations were all significantly more common in the Stanford cohort and 38% of Stanford patients had four or more organ systems involved compared with approximately 10% of Iranian patients. In contrast, Stanford patients had fewer ocular lesions (Stanford 21.4% vs. Iran 53.4% p<0.05), with the biggest difference seen for retinal vasculitis. CONCLUSION Patients with BD from the Western US have a more severe disease course when compared to Iranian patients with BD, as demonstrated by earlier onset and a higher rate of multi-organ involvement. The high risk of Iranian patients with BD developing vasculitis of ocular structures suggests distinct pathomechanisms driving ocular versus extra-ocular BD.
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Affiliation(s)
- Farhad Shahram
- Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA.,Behcet's Disease Unit, Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Marthe T Mæhlen
- Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA.,Department of Rheumatology, Diakonhjemmet Hospital, Oslo, Norway
| | - Massoomeh Akhlaghi
- Behcet's Disease Unit, Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Fereydoun Davatchi
- Behcet's Disease Unit, Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Yaping Joyce Liao
- Department of Ophthalmology and Department of Neurology, Stanford University School of Medicine, Stanford, CA, USA
| | - Cornelia M Weyand
- Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA
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27
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Goloeva RG, Alekberova ZS, Lisitsyna TA, Stepanova EA, Orlova LP. [Intestinal manifestations of Behçet's disease]. TERAPEVT ARKH 2019; 91:111-119. [PMID: 32598685 DOI: 10.26442/00403660.2019.05.000247] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2020] [Indexed: 11/22/2022]
Abstract
Behçet's disease (BD) is a systemic vasculitis of unknown origin, characterized by recurrences of the ulcerative process in the oral cavity and on the genitals, inflammatory damage of the eyes, joints, vessels and other organs. The severity and prognosis of BD determines organ pathology. Intestinal manifestations of BD (intestinal BD) are the least studied. Its verification in BD is complicated by the variety of clinical manifestations, their similarity with inflammatory bowel diseases, the lack of informative laboratory tests, pathognomonic endoscopic and histological signs. Intestinal BD can lead to serious complications (massive bleeding, intestinal perforation and fistula formation), which can not only significantly reduce the quality of the patient's life, but also cause death. Treatment of intestinal BD is not standardized; it is mainly empirical and conducted courses. The purpose of therapy is to achieve clinical remission, healing of intestinal ulcers and prevention of surgery. The article presents a case of severe refractory intestinal BD, requiring twice emergency surgical care - removal of half and then the whole of the colon because of multiple perforations. A brief review of the literature is given and diagnostic difficulties of intestinal BD are discussed.
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Affiliation(s)
- R G Goloeva
- V.A. Nasonova Scientific and Research Institute of Rheumatology
| | - Z S Alekberova
- V.A. Nasonova Scientific and Research Institute of Rheumatology
| | - T A Lisitsyna
- V.A. Nasonova Scientific and Research Institute of Rheumatology
| | | | - L P Orlova
- A.N. Ryzhikh State Scientific Centre of Coloproctology
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28
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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] [MESH Headings] [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 Behçet'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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29
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Shivashankar R, Lichtenstein GR. Mimics of Inflammatory Bowel Disease. Inflamm Bowel Dis 2018; 24:2315-2321. [PMID: 29947781 DOI: 10.1093/ibd/izy168] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/24/2017] [Indexed: 12/31/2022]
Abstract
Inflammatory bowel disease (IBD) may present with nonspecific symptoms and diagnostic findings. Therefore, many diseases may mimic the clinical symptoms, endoscopic findings, and histologic features of IBD. In this paper, we will review mimics of IBD, dividing the diseases that can imitate IBD into noninfectious and infectious causes. For each disease state, we will discuss the clinical symptoms and endoscopic and histologic features. Finally, we will discuss how to distinguish these distinct disorders from IBD.
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Affiliation(s)
- Raina Shivashankar
- Division of Gastroenterology and Hepatology, Thomas Jefferson University, Philadelphia, PA
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30
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Tominaga K, Kamimura K, Takahashi K, Yokoyama J, Terai S. A case of Behçet's disease with various gastrointestinal findings. Clin J Gastroenterol 2018; 11:354-358. [PMID: 29667077 DOI: 10.1007/s12328-018-0860-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/06/2018] [Accepted: 04/07/2018] [Indexed: 01/18/2023]
Abstract
Behçet's disease (BD) is a systemic inflammatory disorder involving the oral and genital mucosa, skin, eyes, gastrointestinal tract, joints, vasculature, and nervous. The gastrointestinal lesions accompanying with BD can extend along the full length of the tract, from the mouth to the anus, and show various clinical characteristics which is difficult to be differentiate from the other inflammatory bowel diseases including ulcerative colitis and Crohn's disease. Hence, the differential diagnosis is difficult and can affect treatment outcomes. We report here, a BD case showing varied gastrointestinal lesions and endoscopic findings. The information, endoscopic findings, differential diagnosis, and treatment approach in this case might help physicians to distinguish from other diseases and to manage similar cases.
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Affiliation(s)
- Kentaro Tominaga
- Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, 1-757 Asahimachi-Dori, Chuo-Ku, Niigata, 9518510, Japan
| | - Kenya Kamimura
- Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, 1-757 Asahimachi-Dori, Chuo-Ku, Niigata, 9518510, Japan.
| | - Kazuya Takahashi
- Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, 1-757 Asahimachi-Dori, Chuo-Ku, Niigata, 9518510, Japan
| | - Junji Yokoyama
- Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, 1-757 Asahimachi-Dori, Chuo-Ku, Niigata, 9518510, Japan
| | - Shuji Terai
- Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, 1-757 Asahimachi-Dori, Chuo-Ku, Niigata, 9518510, Japan
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31
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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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32
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Feng R, Chao K, Chen SL, Sun CH, Qiu Y, Chen BL, Mao R, He Y, Cao QH, Xue L, Zeng ZR, Zhang SH, Chen MH. Heat shock protein family A member 6 combined with clinical characteristics for the differential diagnosis of intestinal Behçet's disease. J Dig Dis 2018; 19:350-358. [PMID: 29851304 DOI: 10.1111/1751-2980.12613] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2017] [Revised: 05/24/2018] [Accepted: 05/28/2018] [Indexed: 12/11/2022]
Abstract
OBJECTIVE To investigate the role of heat shock protein family A member 6 (HSPA6) expression alone and in combination with clinical characteristics in distinguishing intestinal Behçet's disease (BD) from Crohn's disease (CD) with ileocolonic involvement. METHODS Patients diagnosed with either intestinal BD or CD were enrolled. Their clinical characteristics, disease activity, laboratory test results including hypersensitive C-reactive protein (hsCRP) and erythrocyte sedimentation rate (ESR), endoscopic, pathological and radiological features were retrospectively analyzed. Enzyme-linked immunosorbent assay was applied to measure serum HSPA6 levels. RESULTS Among intestinal BD patients, abdominal pain and diarrhea were the leading gastrointestinal symptoms. Submucosal lymphocyte infiltration was the most common pathological finding. Computed tomography enterography features involved number of segments of less than 4 and bowel wall thickening. Independent factors were round/ellipsoid intestinal ulcer (P < 0.001), number of ulcers ≤5 (P = 0.050), elevated ulcer margin (P = 0.019), absence of aphthous ulcer (P = 0.005), bowel wall thickening >13 mm (P < 0.001) and serum HSPA6 level >3.725 ng/mL (P = 0.008) for the differential diagnosis between intestinal BD and CD. Serum HSPA6 expression was significantly elevated in intestinal BD (0.72 ± 0.39 ng/mL) compared with CD (0.50 ± 0.24 ng/mL, P = 0.000) and healthy controls (0.38 ± 0.37 ng/mL, P = 0.000). CONCLUSION HSPA6 in combination with clinical, radiological and pathological characteristics is useful in distinguishing intestinal BD from CD with ileocolonic involvement.
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Affiliation(s)
- Rui Feng
- Department of Gastroenterology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Kang Chao
- Department of Gastroenterology, The Sixth Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Shu Ling Chen
- Department of Ultrasonography, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Can Hui Sun
- Department of Radiology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Yun Qiu
- Department of Gastroenterology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Bai Li Chen
- Department of Gastroenterology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Ren Mao
- Department of Gastroenterology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Yao He
- Department of Gastroenterology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Qing Hua Cao
- Department of Pathology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Ling Xue
- Department of Pathology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Zhi Rong Zeng
- Department of Gastroenterology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Sheng Hong Zhang
- Department of Gastroenterology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - Min Hu Chen
- Department of Gastroenterology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
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Jo JH, Park SJ, Cheon JH, Kim TI, Kim WH. Rediscover the clinical value of small intestinal bacterial overgrowth in patients with intestinal Behçet's disease. J Gastroenterol Hepatol 2018; 33:375-379. [PMID: 28666309 DOI: 10.1111/jgh.13855] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2017] [Accepted: 06/25/2017] [Indexed: 01/14/2023]
Abstract
BACKGROUND AND AIMS Many patients with intestinal Behçet's disease (BD) still suffer from gastrointestinal symptoms despite the disease being in endoscopic or radiological remission. Previous studies report that small intestinal bacterial overgrowth (SIBO) can be associated with inflammatory bowel disease. However, there have been no reports about SIBO in patients with intestinal BD. We sought to identify the frequency of SIBO in patients with inactive intestinal BD by hydrogen breath test (HBT) and to investigate the efficacy of rifaximin as a treatment for SIBO. METHODS Twenty-five patients with intestinal BD who had gastrointestinal symptoms even in endoscopic or radiological remission status were enrolled between January 2012 and January 2016. The patients filled out a questionnaire regarding their subjective gastrointestinal symptoms and took an HBT. Patients with positive HBT results were recommended to take 800-mg rifaximin daily for 14 days. RESULTS Nine patients (9/25, 36%) had a positive HBT test. Eight (8/9, 88.9%) were women, and their mean age was 48.7 years. The most common symptom was abdominal distension (8/9, 88.9%), followed by abdominal discomfort (6/9, 66.7%). Rifaximin was prescribed to the nine patients with positive HBT, but two patients refused to take the medication. Four weeks later, six of the seven patients taking rifaximin (85.7%) reported symptom improvement, and none of them reported adverse events. CONCLUSIONS More than one-third of the patients with inactive intestinal BD who had gastrointestinal symptoms were accompanied by SIBO using HBT. Rifaximin might be an effective and safe drug to treat these patients.
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Affiliation(s)
- Jung Hyun Jo
- 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 Hee Cheon
- 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
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Park YE, Cheon JH. Updated treatment strategies for intestinal Behçet's disease. Korean J Intern Med 2018; 33:1-19. [PMID: 29207867 PMCID: PMC5768550 DOI: 10.3904/kjim.2017.377] [Citation(s) in RCA: 28] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2017] [Accepted: 11/20/2017] [Indexed: 02/08/2023] Open
Abstract
Behçet's disease (BD) is a chronic, idiopathic, relapsing immune-mediated disease involving multiple organs, and is characterized by recurrent oral and genital ulcers, ocular disease, gastrointestinal ulcers, vascular diseases, and skin lesions. In particular, gastrointestinal involvement in BD is followed by severe complications, including massive bleeding, bowel perforation, and fistula, which can lead to significant morbidity and mortality. However, the management of intestinal BD has not yet been properly established. Intestinal BD patients with a severe clinical course experience frequent disease aggravations and often require recurrent corticosteroid and/or immunomodulatory therapies, or even surgery. However, a considerable number of patients with intestinal BD are often refractory to conventional therapies such as corticosteroids and immunomodulators. Recently, there has been a line of evidence suggesting that biologics such as infliximab and adalimumab are effective in treating intestinal BD. Moreover, new biologics targeting proteins other than tumor necrosis factor α are emerging and are under active investigation. Therefore, in this paper, we review the current therapeutic strategies and new clinical data for the treatment of intestinal BD.
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Affiliation(s)
- Yong Eun Park
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Lee HJ, Kim JH, Kim SW, Joo HA, Lee HW, Kim YS, Park SJ, Hong SP, Kim TI, Kim WH, Kim YH, Cheon JH. Proteomic Analysis of Serum Amyloid A as a Potential Marker in Intestinal Behçet's Disease. Dig Dis Sci 2017; 62:1953-1962. [PMID: 28523576 DOI: 10.1007/s10620-017-4606-y] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/21/2016] [Accepted: 05/04/2017] [Indexed: 12/19/2022]
Abstract
BACKGROUND/AIMS Data regarding biomarkers to understand disease pathogenesis and to assess disease activity of intestinal Behçet's disease (BD) are limited. Therefore, we aimed to investigate the differentially expressed proteins in sera from patients with intestinal BD and to search for biomarkers using mass spectrometry-based proteomic analysis. METHODS Serum samples were pooled for the screening study, and two-dimensional electrophoresis (2-DE) was performed to characterize the proteins present in intestinal BD patients. Candidate protein spots were identified using matrix-assisted laser desorption/ionization tandem time-of-flight mass spectrometry (MALDI-TOF/TOF MS) and bioinformatic analysis. To validate the proteomic results, serum samples from an independent cohort were assessed by enzyme-linked immunosorbent assay. RESULTS Pooled serum samples were used for 2-DE, and approximately 400 protein spots were detected in the sera of intestinal BD patients. Of the 22 differentially expressed proteins, 3 were successfully identified using MALDI-TOF/TOF MS. The three up-regulated proteins identified in the intestinal BD group included fibrin, apolipoprotein A-IV, and serum amyloid A (SAA). Serum SAA in intestinal BD patients (2.76 ± 2.50 ng/ml) was significantly higher than that in controls (1.68 ± 0.90 ng/ml, p = 0.007), which is consistent with the proteomic results. In addition, the level of IL-1β in patients with intestinal BD (8.96 ± 1.23 pg/ml) was higher than that in controls (5.40 ± 0.15 pg/ml, p = 0.009). SAA released by HT-29 cells was markedly increased by tumor necrosis factor-α (TNF-α) and lipopolysaccharides stimulation. CONCLUSIONS Our proteomic analysis revealed that SAA was up-regulated in intestinal BD patients.
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Affiliation(s)
- Hyun Jung Lee
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Jae Hyun Kim
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
- Brain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea
| | - Seung Won Kim
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
- Brain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea
- Severance Biomedical Science Institute, Yonsei University College of Medicine, Seoul, Korea
| | - Hyun Ah Joo
- Department of Biochemistry, Yonsei University, Seoul, Korea
| | - Hye Won Lee
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - You Sun Kim
- Department of Internal Medicine, Inje University College of Medicine, Seoul, Korea
| | - Soo Jung Park
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Sung Pil Hong
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Tae Il Kim
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Won Ho Kim
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Young-Ho Kim
- Department of Medicine, Samsung Medical Centre, Sungkyunkwan University School of Medicine, Seoul, 135-710, Republic of Korea.
| | - Jae Hee Cheon
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.
- Brain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea.
- Severance Biomedical Science Institute, Yonsei University College of Medicine, Seoul, Korea.
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Han M, Jung YS, Kim WH, Cheon JH, Park S. Incidence and clinical outcomes of intestinal Behçet's disease in Korea, 2011-2014: a nationwide population-based study. J Gastroenterol 2017; 52:920-928. [PMID: 28028610 DOI: 10.1007/s00535-016-1300-3] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2016] [Accepted: 12/12/2016] [Indexed: 02/04/2023]
Abstract
BACKGROUND Intestinal Behçet's disease (BD) is very rare, and epidemiologic data regarding BD are scarce. Moreover, there have been no population-based studies focusing on intestinal BD. We conducted a nationwide population-based study to examine the incidence and clinical course of Korean patients with intestinal BD. METHODS We collected data on 365 patients diagnosed with intestinal BD from 2011 to 2014 using the Health Insurance Review and Assessment Services claims database. We analyzed the incidence and clinical outcomes, including cumulative rates of bowel resection, hospitalization, and medication use. RESULTS During the study period, the average annual incidence for intestinal BD was 0.18 per 105 population, and the proportion of cases with intestinal involvement was 3.9%. At 1 and 4 years after diagnosis, the cumulative rates of surgery were 5.0 and 10.9%, respectively, whereas those of hospitalization were 27.8 and 32.4%, respectively. The 1- and 4-year cumulative rates of requirements for medications were 39.8 and 49.1% for moderate- to high-dose corticosteroids, 33.6 and 42.1% for immunomodulators, and 3.5 and 6.8% for biologics, respectively. The cumulative probability of corticosteroid and immunomodulator use was higher in male than in female patients and in younger (<40 years) than in older (≥40 years) patients. However, there were no significant differences in the cumulative probabilities of surgery, hospitalization, and biologics use according to sex and age group. CONCLUSIONS The incidence of intestinal BD in Korea is quite low and has been stable in recent years.
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Affiliation(s)
- Minkyung Han
- Department of Public Health, Graduate School, Yonsei University, Seoul, Republic of Korea
| | - Yoon Suk Jung
- Division of Gastroenterology, Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
| | - Won Ho Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.
| | - Sohee Park
- Department of Biostatistics, Graduate School of Public Health, Yonsei University, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.
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Lim KI, Yang DH, Ryoo E. Behçet's disease with multiple splenic abscesses in a child. Intest Res 2017; 15:422-428. [PMID: 28670241 PMCID: PMC5478769 DOI: 10.5217/ir.2017.15.3.422] [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: 05/31/2016] [Revised: 08/31/2016] [Accepted: 09/12/2016] [Indexed: 01/30/2023] Open
Abstract
We report the case of a 5-year-old male patient with multiple aseptic splenic abscesses associated with Behçet's disease. The patient visited Gachon University Gil Hospital with fever, abdominal pain, and acute watery and bloody diarrhea, and reported a 2-year history of chronic abdominal pain and intermittent watery diarrhea. He was treated with antibiotics at a local clinic for fever and cervical lymph node swelling. Additionally, he had recurrent stomatitis. A colonoscopy showed multiple well-demarcated ulcerations throughout the colon, and abdominal computed tomography showed multiple splenic abscesses. Pathergy and HLA-B51 tests were positive. Investigations did not reveal any infectious organisms in the aspirate obtained via ultrasound-guided fine needle aspiration. After steroid treatment, all symptoms and multiple aseptic splenic abscesses resolved. However, oral ulcers, genital ulcers, and abdominal pain recurred after tapering the steroids. Infliximab treatment improved the patient's symptoms. However, 5 months after the treatment, the symptoms recurred. The treatment was changed to include adalimumab. Subsequently, the patient's symptoms resolved and colonoscopic findings improved. No recurrence was noted after 3 months of follow-up.
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Affiliation(s)
- Kyung In Lim
- Department of Pediatrics, Gachon University Gil Hospital, Incheon, Korea
| | - Dong Hwa Yang
- Department of Pediatrics, Gachon University Gil Hospital, Incheon, Korea
| | - Eell Ryoo
- Department of Pediatrics, Gachon University Gil Hospital, Incheon, Korea
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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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Hisamatsu T, Hayashida M. Treatment and outcomes: medical and surgical treatment for intestinal Behçet's disease. Intest Res 2017; 15:318-327. [PMID: 28670228 PMCID: PMC5478756 DOI: 10.5217/ir.2017.15.3.318] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/12/2017] [Revised: 03/19/2017] [Accepted: 03/20/2017] [Indexed: 12/12/2022] Open
Abstract
Behçet's disease (BD) is a chronic relapsing disease involving multiple organ systems. BD is characterized clinically by oral and genital aphthae, cutaneous lesions, and ophthalmological, neurological, and/or gastrointestinal manifestations. It is widely recognized that the presence of intestinal lesions may be a poor prognostic factor in intestinal BD, increasing the risk of surgery and decreasing the quality of life. Despite this, the management of intestinal BD has not been standardized. Empirical therapies including 5-aminosalicylic acid and corticosteroids have been used anecdotally to treat intestinal BD, but recent studies have provided evidence for the efficacy of anti-tumor necrosis factor α monoclonal antibodies. The development of agents targeting tumor necrosis factor α continues, it seems likely that they will change the therapeutic strategy and clinical outcomes of intestinal BD and inflammatory bowel disease. Monitoring disease activity such as endoscopic evaluation will become more important to obtain better outcomes. Here, we review current and future perspectives in the treatment and outcomes of intestinal BD.
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Affiliation(s)
- Tadakazu Hisamatsu
- The Third Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan
| | - Mari Hayashida
- The Third Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan
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Interleukin-33 regulates intestinal inflammation by modulating macrophages in inflammatory bowel disease. Sci Rep 2017; 7:851. [PMID: 28404987 PMCID: PMC5429815 DOI: 10.1038/s41598-017-00840-2] [Citation(s) in RCA: 82] [Impact Index Per Article: 10.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2017] [Accepted: 03/15/2017] [Indexed: 12/19/2022] Open
Abstract
Interleukin 33 (IL-33) that signals through the ST2 receptor has emerged as a critical modulator in several inflammatory disorders, including inflammatory bowel disease (IBD). However, the precise mechanisms by which IL-33 modulates IBD are controversial. The aim of this study was thus to clarify the role of IL-33 in IBD. The plasma levels of IL-33 were significantly decreased, but soluble ST2 levels were increased in patients with IBD compared to healthy individuals. Moreover, IL-33 restored goblet cell numbers and induced macrophage switching from the M1 to the M2 phenotype. These effects were sufficient to ameliorate colitis in dextran sodium sulfate, trinitrobenzene sulfonic acid, and peritoneal cavity cell transfer models. IL-33 facilitated goblet cell restoration via modulating macrophages toward the M2 phenotype. In addition, wound healing was significantly faster in IL-33-treated human monocyte-derived macrophages than in control cells, which could be attributed to increased polarisation into M2 macrophages. We found that patients with IBD show decreased serum levels of IL-33 compared with healthy individuals and that IL-33 can attenuate colitis and aid tissue repair in mice. The mechanism by which IL-33 exerts these effects appears to involve the stimulation of differentiation of goblet cells and M2 macrophages.
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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
- Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Donato Rigante
- Institute of Pediatrics, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Largo A. Gemelli 8, 00168 Rome, Italy
| | - Vincenzo Venerito
- Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Claudia Fabiani
- Department of Ophthalmology, Humanitas Clinical and Research Center, Via Manzoni 56, Rozzano, 20089 Milan, Italy
| | - Rossella Franceschini
- Ophthalmology and Neurosurgery Department, University of Siena, Viale Bracci 1, 53100 Siena, Italy
| | - Michele Barone
- Section of Gastroenterology, Department of Emergency and Organ Transplantation, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Giovanni Lapadula
- Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Mauro Galeazzi
- Research 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
- Research 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
- Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Luca Cantarini
- Research 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
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Özşeker B, Şahin C, Özşeker HS, Efe SC, Kav T, Bayraktar Y. The Role of Fecal Calprotectin in Evaluating Intestinal Involvement of Behçet's Disease. DISEASE MARKERS 2016; 2016:5423043. [PMID: 27642216 PMCID: PMC5013222 DOI: 10.1155/2016/5423043] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/28/2016] [Revised: 05/31/2016] [Accepted: 06/02/2016] [Indexed: 01/28/2023]
Abstract
One of the regions of involvement of Behçet's disease (BD), a systematic inflammatory vasculitis with unknown etiology, is the gastrointestinal (GI) tract. Upper GI endoscopy, colonoscopy, and capsule endoscopy are frequently used methods to diagnose the intestinal involvement of BD. The aim of this study was to investigate the role of fecal calprotectin (FC) in the evaluation of intestinal involvement in BD. Material and Method. A total of 30 patients who were diagnosed with BD and had no GI symptoms and 25 individuals in the control group were included in this study. Results. Levels of FC were statistically significantly higher in patients with BD compared to the control group (p < 0.001). The correlation analysis performed including FC and markers of disease activity revealed a positive and statistically significant correlation between FC level and CRP and erythrocyte sedimentation rate (r: 0.255, p < 0.049, and r: 0.404, p < 0.001, resp.). FC levels in patients who were detected to have ulcers in the terminal ileum and colon in the colonoscopic examination were statistically significantly higher compared to the patients with BD without intestinal involvement (p = 0.01). Conclusion. The measurement of FC levels, in patients with BD who are asymptomatic for GI involvement, may be helpful to detect the possible underlying intestinal involvement.
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Affiliation(s)
- Burak Özşeker
- Division of Gastroenterology, Department of Internal Medicine, School of Medicine, Muğla Sıtkı Koçman University, 48000 Muğla, Turkey
| | - Cem Şahin
- Department of Internal Medicine, School of Medicine, Muğla Sıtkı Koçman University, 48000 Muğla, Turkey
| | - Havva Solak Özşeker
- Department of Pathology, Education and Research Hospital, Muğla Sıtkı Koçman University, 48000 Muğla, Turkey
| | - S. Cumali Efe
- Gastroenterology Unit, Batman State Hospital, 72070 Batman, Turkey
| | - Taylan Kav
- Department of Internal Medicine, Division of Gastroenterology, School of Medicine, Hacettepe University, 06230 Ankara, Turkey
| | - Yusuf Bayraktar
- Department of Internal Medicine, Division of Gastroenterology, School of Medicine, Hacettepe University, 06230 Ankara, Turkey
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Hernández-Chan GS, Ceh-Varela EE, Sanchez-Cervantes JL, Villanueva-Escalante M, Rodríguez-González A, Pérez-Gallardo Y. Collective intelligence in medical diagnosis systems: A case study. Comput Biol Med 2016; 74:45-53. [DOI: 10.1016/j.compbiomed.2016.04.016] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2015] [Revised: 04/26/2016] [Accepted: 04/26/2016] [Indexed: 11/26/2022]
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Abstract
Inflammatory bowel disease (IBD) is a chronic inflammatory disease of the gastrointestinal tract and includes both Crohn's disease and ulcerative colitis. Patients with IBD often present with abdominal pain, diarrhea, and rectal bleeding but may also have a wide variety of other symptoms such as weight loss, fever, nausea, vomiting, and possibly obstruction. Given that the presentation of IBD is not specific, the differential diagnosis is broad and encompasses a wide spectrum of diseases, many of which can mimic and/or even coexist with IBD. It is important for physicians to differentiate symptoms due to refractory IBD from symptoms due to IBD mimics when a patient is not responding to standard IBD treatment. Many of the various IBD mimics include infectious etiologies (viral, bacterial, mycobacterial, fungal, protozoal, and helminthic infections), vascular causes, other immune causes including autoimmune etiologies, drug-induced processes, radiation-induced, and other etiologies such as small intestinal bacterial overgrowth, diverticulitis, and bile acid malabsorption. Thoughtful consideration and evaluation of these potential etiologies through patient history and physical examination, as well as appropriate tests, endoscopic evaluation, and cross-sectional imaging is required to evaluate any patient presenting with symptoms consistent with IBD.
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Wang YN, Li T, Gu JR. Treatment of intestinal Behcet's disease with infliximab: A case report and literature review. Shijie Huaren Xiaohua Zazhi 2016; 24:1302-1306. [DOI: 10.11569/wcjd.v24.i8.1302] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Behcet's disease (BD) is a kind of systemic chronic vascular inflammatory disease. BD often affects the stomach and intestinal tract, resulting in gastrointestinal bleeding, perforation and ulcers. Tumor necrosis factor α (TNF-α) antagonists can relieve the symptoms of refractory BD patients, and infliximab as a TNF-α antagonist in the treatment of intestinal Behcet's disease is rarely reported. Here we report the diagnosis and treatment of a patient with intestinal Behcet's disease, and the efficacy and safety of infliximab in the treatment of intestinal Behcet's disease are analyzed.
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Arimoto J, Endo H, Kato T, Umezawa S, Fuyuki A, Uchiyama S, Higurashi T, Ohkubo H, Nonaka T, Takeno M, Ishigatsubo Y, Sakai E, Matsuhashi N, Nakajima A. Clinical value of capsule endoscopy for detecting small bowel lesions in patients with intestinal Behçet's disease. Dig Endosc 2016; 28:179-85. [PMID: 26459917 DOI: 10.1111/den.12552] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/15/2015] [Revised: 09/14/2015] [Accepted: 09/25/2015] [Indexed: 12/22/2022]
Abstract
BACKGROUND AND AIM To evaluate the clinical value of capsule endoscopy (CE) in patients with intestinal Behçet's disease (BD). METHODS The present study was a case-control pilot study conducted in intestinal BD patients and healthy volunteers. A total of 19 patients with intestinal BD (intestinal BD group) and 19 healthy volunteers (control group) matched for age and sex were enrolled. Frequency, number of small bowel lesions per subject, and Lewis score were comparatively evaluated between the two groups. RESULTS Of the 19 patients with intestinal BD, 18 (94.7%) had reddened lesions, 15 (78.9%) had erosions, and nine (47.4%) had ulcers. There were significant differences in the frequency of reddened lesions (P < 0.0001), erosions (P < 0.0001) and ulcers (P = 0.0011) between the two groups. The difference in the number of small bowel lesions between the two groups was also statistically significant. The median Lewis score in the intestinal BD group was significantly higher than that in the control group (intestinal BD group 237 (0-768) vs. control group 8 (0-135); P < 0.0001). Analysis according to the location in the small bowel revealed that the frequency of ulcers tended to increase towards the distal intestine. CONCLUSION This is the first CE study conducted to examine small bowel involvement in intestinal BD patients. Our results suggest that CE evaluation is necessary, in addition to colonoscopy, in all intestinal BD patients.
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Affiliation(s)
| | | | | | | | | | | | | | | | | | - Mitsuhiro Takeno
- Division of Internal Medicine and Clinical Immunology, Yokohama City University School of Medicine, Yokohama
| | - Yoshiaki Ishigatsubo
- Division of Internal Medicine and Clinical Immunology, Yokohama City University School of Medicine, Yokohama
| | - Eiji Sakai
- Department of Gastroenterology, Kanto Medical Center NTT EC, Tokyo, Japan
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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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Lee HW, Cheon JH, Lee HJ, Park SJ, Hong SP, Kim TI, Kim WH. Postoperative Effects of Thiopurines in Patients with Intestinal Behçet's Disease. Dig Dis Sci 2015; 60:3721-3727. [PMID: 26199149 DOI: 10.1007/s10620-015-3799-1] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/18/2015] [Accepted: 07/04/2015] [Indexed: 12/31/2022]
Abstract
BACKGROUND No studies have heretofore specifically focused on the efficacy of postoperative thiopurine therapy in intestinal Behçet's disease (BD). We conducted this study to assess the clinical effects of postoperative thiopurines in patients with intestinal BD. METHODS We reviewed the medical records of all patients with intestinal BD who had undergone bowel resection surgery in a single tertiary academic medical center between 1991 and 2013. The cumulative probabilities of clinical recurrence were calculated using the Kaplan-Meier method, and predictive factors for recurrence were assessed by multivariate analysis. RESULTS A total of 77 patients with intestinal BD received 5-ASA (n = 50, 64.9%) or thiopurine (n = 27, 35.1%) therapy after surgery at our center. The postoperative recurrence rate was lower in patients who received postoperative thiopurines (P = 0.050). The hazard ratio for recurrence was 0.636 (95% confidence interval 0.130-1.016, P = 0.053) for postoperative thiopurine use compared with postoperative 5-ASA. However, the rates of re-operation, re-admission, and death were not significantly different between the 5-ASA and thiopurine groups. CONCLUSIONS Thiopurine therapy after surgery exhibited a modest effect in preventing recurrence in intestinal BD patients. More patients, long-term follow-up, and a randomized controlled design are necessary to validate the effectiveness of postoperative thiopurines in patients with this disease.
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Affiliation(s)
- Hye Won Lee
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea.
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea.
| | - Hyun Jung Lee
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Soo Jung Park
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Sung Pil Hong
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Tae Il Kim
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Won Ho Kim
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Efficacy of Adalimumab in a Girl with Refractory Intestinal Behcet's Disease. Case Rep Rheumatol 2015; 2015:716138. [PMID: 26609459 PMCID: PMC4644826 DOI: 10.1155/2015/716138] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2015] [Revised: 06/10/2015] [Accepted: 06/14/2015] [Indexed: 01/21/2023] Open
Abstract
We describe our experience with a juvenile patient who had refractory intestinal Behcet's disease that responded to adalimumab, a fully humanized antibody against soluble TNF-α and its receptor. The patient, a 13-year-old girl, presented with oral aphthous ulcers, vulvar pain, and rashes on the lower extremities. She gradually developed a low-grade fever, abdominal pain, diarrhea, and hematochezia. Lower gastrointestinal endoscopy revealed ulcers in the terminal ileum, consistent with intestinal Behcet's disease. Methylprednisolone pulse therapy was initiated, after which the symptoms transiently improved, but, during the corticosteroid taper, the abdominal pain recurred. The symptoms resolved soon after the administration of adalimumab. Of importance, the dose of corticosteroids was successfully reduced without exacerbation during 8 months of observation. This is the first reported case in which adalimumab was used for pediatric gastrointestinal Behcet's disease. Adalimumab is a good choice for intestinal Behcet's disease refractory to conventional treatment.
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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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