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Suzuki Y, Hagiwara T, Kobayashi M, Morita K, Shimamoto T, Hibi T. Is adalimumab safe and effective in patients with intestinal Behcet's disease in real-world practice? Intest Res 2020; 19:301-312. [PMID: 32814420 PMCID: PMC8322033 DOI: 10.5217/ir.2020.00013] [Citation(s) in RCA: 22] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/11/2020] [Accepted: 06/15/2020] [Indexed: 12/11/2022] Open
Abstract
Background/Aims The safety and effectiveness of adalimumab was demonstrated in a phase 3 trial in Japanese patients with intestinal Behçet’s disease. The aim of this study was to evaluate the long-term safety and effectiveness of adalimumab in Japanese patients with intestinal Behçet’s disease. Methods This prospective, all-case, post-marketing study was conducted at 254 centers in Japanese patients with intestinal Behçet’s disease receiving adalimumab. The primary endpoint was incidence of adverse drug reactions. Effectiveness endpoints included global improvement rating and change in C-reactive protein levels. Results Of the 473 registered patients, 462 and 383 included in the safety and effectiveness populations were administered adalimumab for a mean of 515.3 and 579.5 days, respectively. Overall, 395 patients (85.5%) received adalimumab at the recommended dose. Adverse drug reactions and serious adverse drug reactions were reported in 120 (25.97%) and 51 (11.04%) patients, respectively. The incidence of adverse drug reactions was significantly higher in patients with comorbidities (P<0.0001), patients taking concomitant oral corticosteroids (P<0.0001), and those not self-administering adalimumab (P=0.0257). At study end, global improvement rating was “effective” (n = 156, 40.7%) or “markedly effective” (n = 168, 43.9%) in 324 patients (overall effective, 84.6%). Mean C-reactive protein levels (mg/dL) decreased from 1.96 at baseline (n = 324) to 0.58 at week 24 (n = 208) and 0.25 at week 156 (n = 37). Conclusions This large real-world study confirmed the long-term safety and effectiveness of adalimumab in patients with intestinal Behçet’s disease. No new safety concerns were identified. (Clinical trial registration number: NCT01960790)
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Affiliation(s)
- Yasuo Suzuki
- Department of Internal Medicine, Sakura Medical Center, Toho University, Chiba, Japan
| | | | | | | | | | - Toshifumi Hibi
- Center for Advanced IBD Research and Treatment, Kitasato University Kitasato Institute Hospital, Tokyo, Japan
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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: 48] [Impact Index Per Article: 9.6] [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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Iwamoto J, Murakami M, Konishi N, Monma T, Ueda H, Yara S, Hirayama T, Ikegami T, Honda A, Matsuzaki Y. Effects of the Concomitant Use of Low-dose Clarithromycin with an Anti-TNFα Antibody in a Patient with Intestinal Behçet Disease. Intern Med 2018; 57:339-342. [PMID: 29093413 PMCID: PMC5827312 DOI: 10.2169/internalmedicine.9322-17] [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] [Indexed: 11/17/2022] Open
Abstract
A 66-year-old Japanese male with a history of Behçet disease exhibited oral and genital ulcers, and a round deep ileocecal ulcer. He was treated with a combination of mesalazine and 20 mg/day of prednisolone (PSL), but was only partially responsive to PSL and we were not able to reduce the steroid dosage. Adalimumab was also administered. However, the ulcer was not completely responsive, and weaning the patient off PSL remained impossible. In contrast, additional treatment with clarithromycin completely healed the refractory active ulcer and left only a scar. Furthermore, the ulcer has since maintained the scar stage despite successfully weaning the patient from PSL.
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Affiliation(s)
- Junichi Iwamoto
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Masashi Murakami
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Naoki Konishi
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Tadakuni Monma
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Hajime Ueda
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Shoichiro Yara
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Takeshi Hirayama
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Tadashi Ikegami
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Akira Honda
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
| | - Yasushi Matsuzaki
- Department of Gastroenterology, Tokyo Medical University Ibaraki Medical Center, Japan
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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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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: 24] [Impact Index Per Article: 3.0] [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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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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Cheon JH, Kim WH. An update on the diagnosis, treatment, and prognosis of intestinal Behçet's disease. Curr Opin Rheumatol 2015; 27:24-31. [PMID: 25405821 DOI: 10.1097/bor.0000000000000125] [Citation(s) in RCA: 55] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
PURPOSE OF REVIEW This review discusses recent scientific developments in the diagnosis, treatment, and prognosis of intestinal Behçet's disease. RECENT FINDINGS Gastrointestinal involvement is a major cause of morbidity and mortality in Behçet's disease. Patient clinical data are scarce because of the rarity of the disease; however, novel diagnostic criteria and disease activity indices have been developed recently to aid treatment of Behçet's disease patients. Current therapies include 5-aminosalicylic acids, corticosteroids, immunomodulators, or antitumor necrosis factor alpha agents. Antitumor necrosis factor alpha agents can achieve clinical responses and remission in patients that were previously nonresponsive to corticosteroids or immunomodulators. Clinical variables, including young age and higher disease activity at the time of diagnosis, volcano-type ulcers, absence of mucosal healing, higher C-reactive protein levels, prior history of surgery, and lack of initial response to medical therapy, can be regarded as poor prognostic factors. SUMMARY Previously, the diagnosis and management of intestinal Behçet's disease depended upon the expertise of individual clinicians; however, more standardized medical assessments and improved treatment regimens for Behçet's disease patients are evolving.
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Affiliation(s)
- Jae Hee Cheon
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
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Yun HG, Kim HY, Kim DY, Lim YJ. Successful treatment of intractable bleeding caused by radiation-induced hemorrhagic gastritis using oral prednisolone: a case report. Cancer Res Treat 2014; 47:334-8. [PMID: 25327495 PMCID: PMC4398121 DOI: 10.4143/crt.2013.114] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2013] [Accepted: 10/31/2013] [Indexed: 12/25/2022] Open
Abstract
Radiation-induced hemorrhagic gastritis is an intractable and dangerous condition. We describe a 59-year-old female patient with radiation-induced hemorrhagic gastritis. The patient underwent postoperative radiation therapy with a dose of 54 Gy in 30 fractions after a radical operation for a Klatskin tumor. Radiation volume included the gastric antrum. Approximately three months after radiation therapy, she was admitted for melena and anemia. Esophagogastroduodenoscopy showed an area of bleeding in the gastric antrum that was so diffuse that effective laser coagulation was not feasible. After failure of various treatments and transfusion of 7,040 mL of packed red blood cells, we successfully stopped the hemorrhage using oral prednisolone treatment. Based on this case, we think that oral prednisolone treatment can be tried as a first treatment for potentially life-threatening radiation-induced hemorrhagic gastritis.
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Affiliation(s)
- Hyong Geun Yun
- Department of Radiation Oncology, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang, Korea
| | - Hong Yong Kim
- Department of Surgery, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang, Korea
| | - Do Yeun Kim
- Department of Internal Medicine, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang, Korea
| | - Yun Jeong Lim
- Department of Internal Medicine, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang, Korea
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Hisamatsu T, Naganuma M, Matsuoka K, Kanai T. Diagnosis and management of intestinal Behçet's disease. Clin J Gastroenterol 2014; 7:205-12. [PMID: 24883128 PMCID: PMC4037558 DOI: 10.1007/s12328-014-0488-0] [Citation(s) in RCA: 46] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2014] [Accepted: 04/01/2014] [Indexed: 12/12/2022]
Abstract
Behçet’s disease (BD) is a chronic relapsing disease with multiple organ system involvement characterized clinically by oral and genital aphthae, cutaneous lesions, and ophthalmological, neurological, and/or gastrointestinal manifestations. Little clinical evidence is available regarding the management of patients with intestinal BD, despite recognition that the presence of intestinal lesions is a poor prognostic factor, causing perforation and massive bleeding. Many recent case reports have suggested that anti-tumor necrosis factor alpha (TNF)α monoclonal antibodies (mAbs) are effective in patients with intestinal BD. Adalimumab, a fully human anti-TNFα mAb, has been approved in Japan for the treatment of intestinal BD. Here, we review the pathogenesis, diagnosis and management of intestinal BD, including evidence of the efficacy of anti-TNFα mAbs.
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Affiliation(s)
- Tadakazu Hisamatsu
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582 Japan
| | - Makoto Naganuma
- Center for Diagnostic and Therapeutic Endoscopy, School of Medicine, Keio University, Tokyo, Japan
| | - Katsuyoshi Matsuoka
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582 Japan
| | - Takanori Kanai
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582 Japan
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Wu QJ, Zhang FC, Zhang X. Adamantiades-Behcet's disease-complicated gastroenteropathy. World J Gastroenterol 2012; 18:609-15. [PMID: 22363131 PMCID: PMC3281217 DOI: 10.3748/wjg.v18.i7.609] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/02/2011] [Revised: 10/12/2011] [Accepted: 12/31/2011] [Indexed: 02/06/2023] Open
Abstract
Adamantiades-Behcet’s disease (ABD) is a chronic, relapsing, systemic vasculitis of unknown etiology. It is more prevalent in populations along the ancient Silk Road from Eastern Asia to the Mediterranean Basin, and most frequently affects young adults between the second and fourth decades of life. ABD-complicated gastroenteropathy is a significant cause of morbidity and mortality, with abdominal pain as the most common symptom. The ileocecal region is affected predominantly, with ulcerations that may lead to penetration and/or perforation, whereas other parts of the gastrointestinal system including the esophagus and stomach can also be affected. Endoscopy is useful to locate the site and extent of the lesions, and tissue biopsy is often warranted to examine the histopathology that is often suggestive of underlying vasculitis of small veins/venules or, alternatively in some cases, nonspecific inflammation. Bowel wall thickening is the most common finding on computed tomography scan. Treatment is largely empirical since well-controlled studies are difficult to conduct due to the heterogeneity of the disease, and the unpredictable course with exacerbation and remission. Corticosteroids with or without other immunosuppressive drugs, such as cyclophosphamide, azathioprine, sulfasalazine, tumor necrosis factor α antagonist or thalidomide should be applied before surgery, except in emergency.
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Abstract
PURPOSE OF REVIEW To alert physician to timely recognition and current treatment of recurrent hypopyon iridocyclitis or panuveitis in ocular Behçet disease (OBD). RECENT FINDINGS Interferon-α, rituximab, intravitreal triamcinolone, and biological response modifiers by tumor necrosis factor inhibitors such as infliximab and adalimumab are being used increasingly for the treatment of severe sight-threatening ocular inflammation including retinal vasculitis and cystoid macular edema (CME). SUMMARY Biological agents offer tremendous potential in the treatment of OBD. Given that OBD predominantly afflicts the younger adults in their most productive years, dermatologist, rheumatologist, internist, or general practitioners supervising patients with oculo-articulo-oromucocutaneous syndromes should be aware of systemic Behçet disease. Early recognition of ocular involvement is important and such patients should strongly be instructed to visit immediately an ophthalmologist, as uveitis management differs from extraocular involvements with high ocular morbidity from sight-threatening complications due to relapsing inflammatory attacks in the posterior segment of the eye. A single infliximab infusion should be considered for the control of acute panuveitis, whereas repeated long-term infliximab infusions were proved to be more effective in reducing the number of episodes in refractory uveoretinitis with faster regression and complete remission of CME.
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Affiliation(s)
- Cem Evereklioglu
- Department of Ophthalmology, Erciyes University Medical Faculty, Kayseri, Turkey
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12
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Kawata K, Kobayashi Y, Souda K, Kawamura K, Takahashi Y, Noritake H, Watanabe S, Suehiro T, Kamiya M, Yamashita S, Ushio T, Nakamura H. Hemorrhagic radiation gastritis successfully treated with repeated intra-arterial steroid infusions. Clin J Gastroenterol 2010; 4:34-8. [PMID: 26190619 DOI: 10.1007/s12328-010-0191-8] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2010] [Accepted: 11/09/2010] [Indexed: 02/06/2023]
Abstract
Intra-arterial steroid infusion therapy has previously been shown to be effective for inflammatory bowel disease; however, few cases in which it has been used for the treatment of hemorrhagic radiation gastritis have been reported. We report the case of a 70-year-old Japanese man with hemorrhagic radiation gastritis induced by radiation therapy for para-aortic lymph node metastases of hepatocellular carcinoma. Two months after completing radiation therapy, acute persistent bleeding occurred in the gastric irradiation area. Although argon plasma coagulation was performed five times over a month, the bleeding continued and the patient showed persistent anemia that required 50 units of blood transfusion. Finally, the patient was given intra-arterial steroid infusions through the right gastric artery and the right gastroepiploic artery. After three intra-arterial steroid infusions, the melena stopped, and the anemia no longer progressed. Hemorrhagic radiation gastritis was successfully treated with repeated intra-arterial steroid infusions through the regional vessels to the gastric mucosa. Repeated intra-arterial steroid infusions could be a clinically useful option for the treatment of intractable bleeding from radiation gastritis.
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Affiliation(s)
- Kazuhito Kawata
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan.
| | - Yoshimasa Kobayashi
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Kenichi Souda
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Kinya Kawamura
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Yurimi Takahashi
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Hidenao Noritake
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Shinya Watanabe
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Tomoyuki Suehiro
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Mika Kamiya
- Department of Radiology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Shuhei Yamashita
- Department of Radiology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Takasuke Ushio
- Department of Radiology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Hirotoshi Nakamura
- Hepatology Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashiku, Hamamatsu, Shizuoka, 431-3192, Japan
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Ebert EC. Gastrointestinal manifestations of Behçet's disease. Dig Dis Sci 2009; 54:201-7. [PMID: 18594975 DOI: 10.1007/s10620-008-0337-4] [Citation(s) in RCA: 66] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/01/2008] [Accepted: 05/06/2008] [Indexed: 12/13/2022]
Abstract
Behçet's disease is a rare vasculitis diagnosed by the presence of recurrent oral ulcers and two of the following: genital ulcers, typical eye lesions, typical skin lesions, and positive pathergy test. It is most commonly seen in countries along the ancient silk road from Eastern Asia to the Mediterranean Basin. Young adults between the second and fourth decades of life are mainly affected, with abdominal pain being the most common symptom. The ileocecal region is most commonly affected, with ulcerations that may penetrate or perforate. Rarely, the esophagus and stomach may have ulcerations. Bowel wall thickening is the most common finding on computed tomography (CT) scan. Pathology shows a vasculitis mainly involving the small veins or, alternatively, nonspecific inflammation. Corticosteroids, with or without other immunosuppressive drugs, are used for severe eye disease. Their use in intestinal disease is largely empirical. Surgery may be required for perforation. Behçet's disease runs a chronic, unpredictable course with exacerbations and remissions which decrease in frequency and severity over time. Death is mainly due to major vessel disease and neurological involvement.
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Affiliation(s)
- Ellen C Ebert
- UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08903, USA.
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Karakaya K, Comert M, Numanoglu G. Multiple perforations along the transverse colon as a rare presentation of intestinal Behcet's disease: a case report. Clinics (Sao Paulo) 2009; 64:1231-3. [PMID: 20037714 PMCID: PMC2797595 DOI: 10.1590/s1807-59322009001200016] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Affiliation(s)
- Kemal Karakaya
- Department of General Surgery, Zonguldak Karaelmas University - Zonguldak, Turkey
- Tel: 90 372 2612776
| | - Mustafa Comert
- Department of General Surgery, Zonguldak Karaelmas University - Zonguldak, Turkey
| | - Gamze Numanoglu
- Department of Pathology, Zonguldak Karaelmas University - Zonguldak, Turkey
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Garcia-Porrua C, Gutierrez-Duque O, Soto S, Garcia-Rodeja E, Gonzalez-Gay MA. Localized Vasculitis of the Gastrointestinal Tract. Semin Arthritis Rheum 2006; 35:403-6. [PMID: 16765718 DOI: 10.1016/j.semarthrit.2006.03.001] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Abstract
BACKGROUND AND OBJECTIVES Isolated vasculitis of the gastrointestinal (GI) tract is a rare entity. Endoscopic biopsies have low sensitivity to diagnose intestinal vasculitis, even though the endoscopic findings may be suggestive of this condition. Our aims were to describe a case of biopsy-proven colonic leukocytoclastic vasculitis and review the literature. METHODS A patient with biopsy-proven colonic leukocytoclastic vasculitis is described. A Medline database search of cases with localized GI vasculitis between January 1985 and September 2005 was conducted. RESULTS A 32-year-old man was admitted to the hospital because of abdominal pain and diarrhea. A colonic biopsy showed leukocytoclastic vasculitis. There are very few articles on leukocytoclastic GI vasculitis as a separate disease, and most of them emphasize the difficulty in classification. Unlike our case, in former cases of localized vasculitis a diagnosis was made after surgery. Although our patient had steroid-refractory biopsy-proven isolated intestinal vasculitis, treatment with intravenous cyclophosphamide resulted in rapid resolution of symptoms and surgery was not required. CONCLUSIONS In patients with abdominal pain a diagnosis of intestinal vasculitis should be considered. Immunosuppressive therapy allowed our patient to avoid surgery and may be similarly beneficial in other similar cases.
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Isik B, Ara C, Kirimlioglu H, Sogutlu G, Yilmaz M, Yilmaz S, Kirimlioglu V. Single or multiple perforations with varying locations as a complication of intestinal Behçet's disease: report of three cases. Scand J Gastroenterol 2005; 40:599-603. [PMID: 16036514 DOI: 10.1080/00365520510012127] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Intestinal ulcers in Behçet's disease (BD) tend to cause perforation with significant morbidity. The optimal surgical procedure in such cases is controversial and the postoperative period can be eventful with an unpredictable course. We report three cases of perforations with varying locations in three patients with long-standing Behçet's disease. Two patients required two and one patient required four operations. It is emphasized that the clinician must be alert in a patient with BD when abdominal symptoms accompany the clinical picture. As soon as the diagnosis is reached, surgical intervention with limited resection must be performed. Endoscopic examination and careful medication play major roles in the follow-up.
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Affiliation(s)
- Burak Isik
- Department of General Surgery, Inonu University Faculty of Medicine, Turgut Ozal Medical Center, Malatya, Turkey.
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Abstract
Behçet's disease (BD) is a chronic, relapsing, systemic inflammatory vasculitis of unknown aetiology with a myriad of immunological and pathological consequences. Patients with BD are clustered along the ancient silk road, extending from Far-East Asia to Turkey. The disease affects both genders of all ages from infants to the elderly. It is a long-term, cyclical disease and such patients may have symptom-free periods of weeks, months or years that are interrupted by exacerbations of varying intensities lasting a few days, weeks or months. Clinical features include oral aphthae, genital ulcers, ocular inflammation, skin lesions, as well as articular, vascular, neurological, pulmonary, gastrointestinal, renal and genitourinary manifestations. The main histopathological finding is a widespread vasculitis of the arteries and veins of any size or thrombophilia according to the site of involvement. BD may start with just one or two small symptoms but other symptoms may gradually appear over the years. Recurrent ocular inflammation, which occurs in approximately 50% of cases, is the major morbidity that may eventually lead to blindness. The treatment of BD is usually symptomatic and palliative. Therefore, the main objectives are to relieve symptoms associated with mucocutaneous lesions and arthritis, to modify the course of the disease, to control inflammatory eye disease, clinically suppress the inflammation and vasculitis, to prevent recurrences and thus, prevent irreversible damage. The choice of treatment is based on the severity of systemic involvement, clinical presentation and the site affected. The preferred treatment modalities are combined drug therapy and include topical therapies as well as systemic corticosteroids, NSAIDs, colchicine, dapsone and immunosuppressive and cytotoxic agents. Such therapies are tailored to the individual patient depending on clinical manifestations. Thalidomide, tacrolimus, IFN-alpha and anti-TNF monoclonal antibody have recently attracted attention as novel therapeutic approaches.
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Affiliation(s)
- Cem Evereklioglu
- Department of Ophtalmology, Erciyes University Medical Faculty, Kayseri, Turkey.
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