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Thieroff G, Bartholow SJ, Kilian A. Development of Behçet's disease on maintenance therapy for autoimmune hepatitis. BMJ Case Rep 2024; 17:e259199. [PMID: 39694650 DOI: 10.1136/bcr-2023-259199] [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: 12/20/2024] Open
Abstract
Behçet's disease is a clinical diagnosis with variable presentations. Liver involvement is rare in the absence of vascular complications. We describe a patient diagnosed with Hashimoto's thyroiditis and autoimmune hepatitis on azathioprine who developed extensive aphthous ulcers approximately 10 years later. She was initially diagnosed with an overlap autoimmune connective tissue disorder in the setting of a positive anti-Ro antibody. Symptoms did not resolve with prednisone, hydroxychloroquine or mycophenolate mofetil. She subsequently developed pseudofolliculitis, erythema nodosum-like lesions, large cutaneous ulcers and pathergy phenomenon at sites of venipuncture consistent with Behçet's disease. She was successfully treated with infliximab, azathioprine, colchicine and apremilast. This case represents an unusual sequence of autoimmune conditions and a diagnostic challenge.
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Affiliation(s)
- George Thieroff
- Rheumatology, Saint Louis University, Saint Louis, Missouri, USA
| | | | - Adam Kilian
- Internal Medicine, Saint Louis University School of Medicine, Saint Louis, Missouri, USA
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Hemdan M, Abdel Mageed SS, Abulsoud AI, Faraag AHI, Zaki MB, Mansour RM, Raouf AA, Ali MA, Mohammed OA, Salman A, Salah AN, Abdel-Reheim MA, Doghish AS. Approaches based on miRNAs in Behçet's Disease: Unveiling pathogenic mechanisms, diagnostic strategies, and therapeutic applications. Life Sci 2024; 354:122950. [PMID: 39128821 DOI: 10.1016/j.lfs.2024.122950] [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: 05/27/2024] [Revised: 07/16/2024] [Accepted: 08/05/2024] [Indexed: 08/13/2024]
Abstract
Behçet's Disease (BD) is an intricate medical puzzle, captivating researchers with its enigmatic pathogenesis. This complex ailment, distinguished by recurrent mouth and genital lesions, eye irritation, and skin injuries, presents a substantial obstacle to therapeutic research. This review explores the complex interaction of microRNAs (miRNAs) with BD, highlighting their crucial involvement in the disease's pathophysiology. miRNAs, recognized for regulatory influence in diverse biological processes, hold a pivotal position in the molecular mechanisms of autoimmune diseases, such as BD. The exploration begins with examining miRNA biogenic pathways and functions, establishing a foundational understanding of their regulatory mechanisms. Shifting to the molecular landscape governing BD, the review highlights miRNA-mediated impacts on critical signaling pathways like Notch, nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB), and protein kinase B (AKT)/mammalian target of rapamycin (mTOR), offering insights into intricate pathophysiological mechanisms. Dissecting the immunological landscape reveals the profound influence of miRNAs on BD, shedding light on the intricate modulation of immune responses and offering novel perspectives on disease etiology and progression. Beyond molecular intricacies, the review explores the clinical relevance of miRNAs in BD, emphasizing their potential as diagnostic and prognostic indicators. The discussion extends to the promising realm of miRNA-based therapeutic interventions, highlighting their potential in alleviating symptoms and altering disease progression. This comprehensive review, serving as a valuable resource for researchers, clinicians, and stakeholders, aims to decipher the intricate molecular tapestry of BD and explore the therapeutic potential of miRNAs.
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Affiliation(s)
- Mohamed Hemdan
- School of Biotechnology, Badr University in Cairo (BUC), Badr City, Cairo 11829, Egypt
| | - Sherif S Abdel Mageed
- Pharmacology and Toxicology Department, Faculty of Pharmacy, Badr University in Cairo (BUC), Badr City, Cairo 11829, Egypt
| | - Ahmed I Abulsoud
- Biochemistry Department, Faculty of Pharmacy, Heliopolis University, Cairo 11785, Egypt; Biochemistry and Molecular Biology Department, Faculty of Pharmacy (Boys), Al-Azhar University, Nasr City, Cairo 11231, Egypt
| | - Ahmed H I Faraag
- School of Biotechnology, Badr University in Cairo (BUC), Badr City, Cairo 11829, Egypt; Botany and Microbiology Department, Faculty of Science, Helwan University, Helwan 11795, Egypt
| | - Mohamed Bakr Zaki
- Department of Biochemistry, Faculty of Pharmacy, University of Sadat City, Menoufia 32897, Egypt
| | - Reda M Mansour
- Zoology and Entomology Department, Faculty of Science, Helwan University, Helwan 11795, Egypt; Biology Department, School of Biotechnology, Badr University in Cairo, Badr City, Cairo 11829, Egypt
| | - Ahmed Amr Raouf
- Pharmacology and Toxicology Department, Faculty of Pharmacy, Badr University in Cairo (BUC), Badr City, Cairo 11829, Egypt
| | - Mohamed A Ali
- School of Biotechnology, Badr University in Cairo (BUC), Badr City, Cairo 11829, Egypt
| | - Osama A Mohammed
- Department of Pharmacology, College of Medicine, University of Bisha, Bisha 61922, Saudi Arabia
| | - Aya Salman
- Department of Biochemistry, Faculty of Pharmacy, Egyptian Russian University, Cairo, Egypt
| | - Akram N Salah
- Microbiology and Immunology Department, Faculty of Pharmacy, Badr University in Cairo (BUC), Badr City, Cairo 11829, Egypt
| | - Mustafa Ahmed Abdel-Reheim
- Department of Pharmaceutical Sciences, College of Pharmacy, Shaqra University, Shaqra 11961, Saudi Arabia; Department of Pharmacology and Toxicology, Faculty of Pharmacy, Beni-Suef University, Beni Suef 62521, Egypt.
| | - Ahmed S Doghish
- Department of Biochemistry, Faculty of Pharmacy, Badr University in Cairo (BUC), Badr City, Cairo 11829, Egypt; Faculty of Pharmacy (Boys), Al-Azhar University, Nasr City, Cairo 11231, Egypt.
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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 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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Dai A, Kim SJ. Systemic calcineurin inhibitors tacrolimus and voclosporin: A review of off-label dermatologic uses. J Am Acad Dermatol 2024; 90:358-367. [PMID: 37307993 DOI: 10.1016/j.jaad.2023.05.074] [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: 08/22/2022] [Revised: 05/08/2023] [Accepted: 05/18/2023] [Indexed: 06/14/2023]
Abstract
BACKGROUND Systemic calcineurin inhibitors, cyclosporine, tacrolimus, and voclosporin, have been utilized in various dermatologic conditions. Although there have been numerous off-label dermatologic indications with published guidelines for cyclosporine, there is no established strong consensus for tacrolimus and voclosporin. OBJECTIVE To conduct a review of off-label use of systemic tacrolimus and voclosporin in various dermatoses to better inform treatment methods. METHODS A literature search was conducted using PubMed and Google Scholar. Relevant clinical trials, observational studies, case series, and reports regarding off-label dermatologic uses of systemic tacrolimus and voclosporin were included. RESULTS Tacrolimus shows promise for numerous dermatologic conditions, including psoriasis, atopic dermatitis/eczema, pyoderma gangrenosum, chronic urticaria, and Behcet's disease. Randomized controlled trial data are only available for voclosporin in psoriasis, which showed efficacy but did not meet noninferiority to cyclosporine. LIMITATIONS Data were limited and extracted from published papers. Studies differed in methodology, and nonstandardized outcomes limited the conclusions drawn. CONCLUSIONS In comparison to cyclosporine, tacrolimus can be considered for treatment-refractory disease or in patients with cardiovascular risk factors or inflammatory bowel disease. Voclosporin has only been utilized in psoriasis currently, and clinical trials in psoriasis show voclosporin's efficacy. Voclosporin can be considered for patients with lupus nephritis.
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Affiliation(s)
- Annie Dai
- Department of Dermatology, Baylor College of Medicine, Houston, Texas
| | - Soo Jung Kim
- Department of Dermatology, Baylor College of Medicine, Houston, Texas.
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Ono R, Tominaga T, Nonaka T, Takamura Y, Oishi K, Shiraishi T, Hashimoto S, Noda K, Sawai T, Okano S, Nagayasu T. Intestinal Behçet's and suspected intestinal Behçet's disease: a report of four surgical cases. Surg Case Rep 2024; 10:3. [PMID: 38165549 PMCID: PMC10761645 DOI: 10.1186/s40792-023-01798-2] [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: 08/31/2023] [Accepted: 12/20/2023] [Indexed: 01/04/2024] Open
Abstract
BACKGROUND Intestinal Behçet's disease (BD) is often associated with ulceration that requires surgery, including perforation and abscess formation. However, no consensus has been reached on the optimal extent of resection or treatment strategy. This study reviewed four cases of intestinal or suspected intestinal BD. CASE PRESENTATIONS In Case 1, a 74-year-old woman diagnosed with BD 2 years earlier was treated with anti-tumor necrosis factor α antibody (Infliximab) and steroids. She had oral and pubic ulcers. After close investigation of abdominal pain, perforation of the gastrointestinal tract was suspected and surgery was performed. Multiple perforating ulcers and abscesses were found in the distal ileum, and the small intestine was resected. Postoperatively, the patient was treated with an increased steroid dose and symptoms have remained stable. Case 2 involved a 69-year-old woman with oral and pubic ulcers, ocular ulcer, and skin lesions. She experienced sudden onset of abdominal pain during treatment for lymphoma. She showed multiple perforating ulcers throughout the ileum and underwent resection of the small intestine and ileostomy. Upper abdominal pain appeared during postoperative treatment for high-output syndrome. The patient underwent omentoplasty after perforation of the upper gastrointestinal tract was diagnosed. Postoperatively, anti-interleukin-1 beta antibodies (canakinumab) was administered to control the disease. Case 3 involved an 81-year-old, previously healthy woman. She presented to her previous physician with complaints of pubic ulcer, hemorrhage and abdominal pain. Colonoscopy showed multiple ulcers throughout the entire colon. Steroid therapy was started, but bleeding proved difficult to control and total proctocolectomy was performed. Histopathology revealed multiple perforating ulcers and BD was diagnosed. Postoperatively, the patient remains under steroid control. Case 4 involved a 43-year-old man with abdominal pain who showed abscess formation in the ileocecal region. After excision of the ileocecal area, multiple ulcers were diagnosed. Two years later, abdominal pain recurred and free air was found in the abdomen on close imaging. Emergency anastomotic resection was performed due to ulceration and perforation of the anastomosis. CONCLUSIONS Intestinal BD may flare up after surgical treatment and require multiple surgeries. Introducing pharmacotherapy as soon as possible after surgical treatment is important to control the disease.
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Affiliation(s)
- Rika Ono
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Tetsuro Tominaga
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.
| | - Takashi Nonaka
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Yuma Takamura
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Kaido Oishi
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Toshio Shiraishi
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Shintaro Hashimoto
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Keisuke Noda
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Terumitsu Sawai
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Shinji Okano
- Department of Pathology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Takeshi Nagayasu
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
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Bellamy CO, Burt AD. Liver in Systemic Disease. MACSWEEN'S PATHOLOGY OF THE LIVER 2024:1039-1095. [DOI: 10.1016/b978-0-7020-8228-3.00015-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/05/2025]
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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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Samreen I, Darji P, Genobaga S, Doosetty S, Mohta T, Maity G, Vue C, Nakka S, Umeh C. Pulmonary Artery Aneurysm in Behcet Disease: Medical, Endovascular or Surgical Intervention. Cureus 2023; 15:e49368. [PMID: 38146562 PMCID: PMC10749288 DOI: 10.7759/cureus.49368] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/22/2023] [Indexed: 12/27/2023] Open
Abstract
Behçet's disease is a chronic inflammatory condition that predominantly affects the body's blood vessels, exhibiting various clinical manifestations and complications. The exact cause remains unclear, but genetic predisposition, immune responses, and vascular activation are believed to contribute to its development. This disease is more prevalent in certain geographic regions and primarily affects young adults, particularly males. Pulmonary aneurysm, a complication of Behçet's disease, is the leading cause of mortality in Behcet disease. In this review, we summarize the complications of Behcet disease with a focus on pulmonary artery aneurysms. We discussed the medical, endovascular, and surgical management of pulmonary aneurysms in Behcet disease and the indications and outcomes of the different treatment options. Corticosteroids and cyclophosphamide remain the preferred first-line therapy. However, clinical improvement with infliximab or adalimumab, tumor necrosis factor-alpha (TNFα) blocking agents, have been reported after treatment failure with recommended first-line agents. In patients who fail medical therapy or those with life-threatening hemoptysis, endovascular or surgical intervention is the next option. Endovascular interventions include pulmonary artery embolization with coils or acrylic glue and using plugs, occluders, or stents. Endovascular interventions usually have fewer adverse effects than surgery. Although the risk of surgical procedures is high in pulmonary artery aneurysms, it could be a life-saving procedure in patients with life-threatening hemoptysis. Surgical options, including pulmonary artery ligation, aneurysmorrhaphy, segmentectomy, lobectomy, or pneumonectomy are available. However, the results of surgical therapy for Behçet aneurysms are often disappointing.
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Affiliation(s)
- Isha Samreen
- Internal Medicine, Hemet Global Medical Center, Hemet, USA
| | - Puja Darji
- Internal Medicine, Hemet Global Medical Center, Hemet, USA
| | | | | | - Tamanna Mohta
- Internal Medicine, Hemet Global Medical Center, Hemet, USA
| | - Gargi Maity
- Internal Medicine, Hemet Global Medical Center, Hemet, USA
| | - Chong Vue
- Internal Medicine, Hemet Global Medical Center, Hemet, USA
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Yoo JW, Jo SI, Shin DW, Park JW, Kim SE, Lim H, Kang HS, Moon SH, Kim MK, Kim SY, Hwang SW, Soh JS. Clinical Usefulness of Immune Profiling for Differential Diagnosis between Crohn's Disease, Intestinal Tuberculosis, and Behcet's Disease. Diagnostics (Basel) 2023; 13:2904. [PMID: 37761270 PMCID: PMC10529363 DOI: 10.3390/diagnostics13182904] [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: 08/01/2023] [Revised: 09/06/2023] [Accepted: 09/08/2023] [Indexed: 09/29/2023] Open
Abstract
It is important to make a differential diagnosis between inflammatory diseases of the bowel with similar clinical and endoscopic features. The profiling of immune cells could be helpful for accurately diagnosing inflammatory bowel diseases. We compared immune marker expression between Crohn's disease (CD), intestinal Behcet's disease (BD), and intestinal tuberculosis (TB) and evaluated the usefulness of immune profiling in differentiating between these diseases. Biopsy specimens were acquired around ulcerations on the terminal ileum or cecum from five patients with each disease. Panel 1 included multiplex immunohistochemistry staining for CD8, CD4, Foxp3, CD20, programmed death-1, and granzyme B. CD56, CD68, CD163, CD11c, and HLA-DR were analyzed in panel 2. The differences in cytotoxic T cells (CD8+CD4-Fopx3-CD20-), helper T cells (CD8-CD4+Fopx3-CD20-), and regulatory T cells (CD8-CD4+Fopx3+CD20-) were also not significant. However, M1 macrophage (CD68+CD163-HLA-DR-) cell densities were significantly higher in intestinal BD than in other diseases. The expression level of dendritic cells (CD56-CD68-CD163-CD11c+HLA-DR+) was highest in intestinal TB and lowest in intestinal BD. The expression of immune cells, including M1 macrophages and dendritic cells, was different between CD, intestinal BD, and intestinal TB. Immune profiling can be helpful for establishing differential diagnoses of inflammatory bowel diseases.
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Affiliation(s)
- Ji Won Yoo
- Department of Internal Medicine, Kirk Kerkorian School of Medicine, University of Nevada, Las Vegas, NV 89557, USA;
| | - Su In Jo
- PrismCDX Co., Ltd., Hwaseong-si 18469, Republic of Korea;
| | - Dong Woo Shin
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, University of Hallym College of Medicine, Anyang 14068, Republic of Korea; (D.W.S.); (J.W.P.); (S.-E.K.); (H.L.); (H.S.K.); (S.-H.M.)
| | - Ji Won Park
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, University of Hallym College of Medicine, Anyang 14068, Republic of Korea; (D.W.S.); (J.W.P.); (S.-E.K.); (H.L.); (H.S.K.); (S.-H.M.)
| | - Sung-Eun Kim
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, University of Hallym College of Medicine, Anyang 14068, Republic of Korea; (D.W.S.); (J.W.P.); (S.-E.K.); (H.L.); (H.S.K.); (S.-H.M.)
| | - Hyun Lim
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, University of Hallym College of Medicine, Anyang 14068, Republic of Korea; (D.W.S.); (J.W.P.); (S.-E.K.); (H.L.); (H.S.K.); (S.-H.M.)
| | - Ho Suk Kang
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, University of Hallym College of Medicine, Anyang 14068, Republic of Korea; (D.W.S.); (J.W.P.); (S.-E.K.); (H.L.); (H.S.K.); (S.-H.M.)
| | - Sung-Hoon Moon
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, University of Hallym College of Medicine, Anyang 14068, Republic of Korea; (D.W.S.); (J.W.P.); (S.-E.K.); (H.L.); (H.S.K.); (S.-H.M.)
| | - Min Kyu Kim
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, Republic of Korea;
| | - Sang-Yeob Kim
- Convergence Medicine Research Center, Asan Institute for Life Sciences, Asan Medical Center, Seoul 05505, Republic of Korea;
| | - Sung Wook Hwang
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, Republic of Korea;
| | - Jae Seung Soh
- Department of Internal Medicine, Hallym University Sacred Heart Hospital, University of Hallym College of Medicine, Anyang 14068, Republic of Korea; (D.W.S.); (J.W.P.); (S.-E.K.); (H.L.); (H.S.K.); (S.-H.M.)
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11
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Curtin BF, Hill KL, Bhattacharya S, Powers A, Venkatesan A, Bagi P, Joyal E, Alimchandani M, Goldbach-Mansky R, Grayson P, Quezado M, Sibley C, Heller T. Clinical, Endoscopic, and Histopathologic Gastrointestinal Disease in an American Cohort With Behçet's Disease. Clin Transl Gastroenterol 2023; 14:e00591. [PMID: 37114910 PMCID: PMC10461961 DOI: 10.14309/ctg.0000000000000591] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/20/2022] [Accepted: 04/10/2023] [Indexed: 04/29/2023] Open
Abstract
INTRODUCTION Behçet's disease (BD) is a chronic systemic vasculitis characterized by oral and genital ulcers, uveitis, and skin lesions. Patients with BD may develop gastrointestinal (GI) disease; however, characterization of GI disease in American cohorts is lacking. In this article, we present clinical, endoscopic, and histopathologic GI findings in an American cohort of patients with BD. METHODS Patients with established BD were evaluated prospectively at the National Institutes of Health. Demographic and clinical data were collected including BD manifestations and GI symptoms. Endoscopy with histopathologic sampling was performed for both clinical and research indications with written consent. RESULTS Eighty-three patients were evaluated. The majority were female (83.1%) and white (75.9%). Mean age was 36 ± 14.8 years. GI symptoms were reported in 75% of cohort with nearly half of reporting abdominal pain (48.2%) and significant numbers reporting acid reflux, diarrhea, and nausea/vomiting. Esophagogastroduodenoscopy was performed in 37 patients; erythema and ulcers were the most common found abnormalities. Colonoscopy was performed in 32 patients with abnormalities including polyps, erythema, and ulcers. Endoscopy was normal in 27% of esophagogastroduodenoscopies and 47% of colonoscopies. Vascular congestion was demonstrated on the majority of random biopsies throughout the GI tract. Inflammation was not highly prevalent on random biopsies except in the stomach. Wireless capsule endoscopy was performed on 18 patients; ulcers and strictures were the most common abnormalities. DISCUSSION GI symptoms were common in this cohort of American patients with BD. Endoscopic examination was often normal; however, histopathologic examination demonstrated vascular congestion throughout the GI tract.
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Affiliation(s)
- Bryan F. Curtin
- Digestive Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA
| | - Kareen L. Hill
- Translational Hepatology Section, Liver Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA
| | - Sumona Bhattacharya
- Digestive Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA
| | - Astin Powers
- Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - Aradhana Venkatesan
- Radiology and Imaging Sciences Department, Clinical Center, National Institutes of Health, Bethesda, Maryland, USA
| | - Preet Bagi
- Digestive Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA
| | - Elizabeth Joyal
- Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA
| | - Meghna Alimchandani
- Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA
| | - Raphaela Goldbach-Mansky
- Translational Autoinflammatory Diseases AU2 Section, National Institute of Arthritis andMusculoskeletal and Skin Diseases and National Institute of Allergy and Infectious Diseases, Bethesda, Maryland, USA
| | - Peter Grayson
- Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA
| | - Martha Quezado
- Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - Cailin Sibley
- Translational Autoinflammatory Diseases AU2 Section, National Institute of Arthritis andMusculoskeletal and Skin Diseases and National Institute of Allergy and Infectious Diseases, Bethesda, Maryland, USA
| | - Theo Heller
- Translational Hepatology Section, Liver Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA
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Hamilton J, Milenkovski N, Martin K, Tully E, Peng C, Hayes I. Rare causes of abdominal pain: a primer for the admitting general surgeon. ANZ J Surg 2023; 93:1773-1779. [PMID: 37350226 DOI: 10.1111/ans.18570] [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: 12/18/2022] [Revised: 05/11/2023] [Accepted: 06/07/2023] [Indexed: 06/24/2023]
Abstract
The broad uptake of the acute surgical unit (ASU) model of surgical care in Australia has resulted in general surgeons becoming increasingly involved in the management of patients with acute abdominal pain (AAP), some of whom will be labelled as having non-specific abdominal pain (NSAP) (Kinnear N, Jolly S, Herath M, et al. The acute surgical unit: An updated systematic review and meta-analysis. review. Int. J. Surg. 2021;94:106109; Lehane CW, Jootun RN, Bennett M, Wong S, Truskett P. Does an acute care surgical model improve the management and outcome of acute cholecystitis? ANZ J. Surg. 2010;80:438-42). NSAP patients lack a clear diagnosis of surgical pathology based on standard clinical, laboratory and imaging work-up, although they may require ASU admission for pain control and assessment. This article provides a review of uncommon conditions, presenting as AAP, that could possibly be mis-labelled as NSAP, with a focus on aspects of the presentation that may aid diagnosis and management including specific demographic features, clinical findings, key investigations and initial treatment priorities for ASU clinicians. Ultimately, most of the conditions discussed will not require surgical intervention, however, they require a diagnosis to be made and initial treatment planning before on-referral to the appropriate specialty. For the on-call general surgeon, some knowledge of these conditions and an index of suspicion are invaluable for the prompt diagnosis and efficient management of these patients.
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Affiliation(s)
- Jordan Hamilton
- Department of General Surgical Specialties, The Royal Melbourne Hospital, Melbourne, Victoria, Australia
- Department of Surgery, The University of Melbourne, Melbourne, Victoria, Australia
| | - Nicole Milenkovski
- Department of General Surgical Specialties, The Royal Melbourne Hospital, Melbourne, Victoria, Australia
| | - Katherine Martin
- Department of General Surgical Specialties, The Royal Melbourne Hospital, Melbourne, Victoria, Australia
| | - Emma Tully
- Department of General Surgical Specialties, The Royal Melbourne Hospital, Melbourne, Victoria, Australia
| | - Calvin Peng
- Department of General Surgical Specialties, The Royal Melbourne Hospital, Melbourne, Victoria, Australia
| | - Ian Hayes
- Department of General Surgical Specialties, The Royal Melbourne Hospital, Melbourne, Victoria, Australia
- Department of Surgery, The University of Melbourne, Melbourne, Victoria, Australia
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13
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Li R, Li X, Zhou H, Shi Y, Wang F, Wu T, Liang J. Successful treatment of a refractory intestinal Behcet's disease with an oncology history by Vedolizumab: a case report and literature review. Front Immunol 2023; 14:1205046. [PMID: 37287984 PMCID: PMC10242066 DOI: 10.3389/fimmu.2023.1205046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2023] [Accepted: 05/10/2023] [Indexed: 06/09/2023] Open
Abstract
Objective Behçet's Disease (BD) is an intractable systemic vasculitis. When accompanied by intestinal symptoms, the prognosis is usually poor. 5-Aminosalicylic acid (5-ASA), corticosteroids, immunosuppressive drugs, and anti-tumor necrosis factor-α (anti-TNF-α) biologics are standard therapies to induce or maintain remission for intestinal BD. However, they might not be effective in refractory cases. Safety should also be considered when patients have an oncology history. Regarding the pathogenesis of intestinal BD and the specific targeting effect of vedolizumab (VDZ) on the inflammation of the ileum tract, previous case reports suggested that VDZ might be a potential treatment for refractory intestinal BD. Methods We report a 50-year-old woman patient with intestinal BD who had oral and genital ulcers, joint pain, and intestinal involvement for about 20 years. The patient responds well to anti-TNF-α biologics but not to conventional drugs. However, biologics treatment was discontinued due to the occurrence of colon cancer. Results VDZ was intravenously administered at a dose of 300 mg at 0, 2, and 6 weeks and then every eight weeks. At the 6-month follow-up, the patient reported significant improvement in abdominal pain and arthralgia. We observed complete healing of intestinal mucosal ulcers under endoscopy. However, her oral and vulvar ulcers remained unresolved, which disappeared after adding thalidomide. Conclusion VDZ may be a safe and effective option for refractory intestinal BD patients who do not respond well to conventional treatments, especially those with an oncology history.
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Affiliation(s)
| | | | | | | | | | - Tong Wu
- *Correspondence: Tong Wu, ; Jie Liang,
| | - Jie Liang
- *Correspondence: Tong Wu, ; Jie Liang,
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14
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van der Geest KS, Slijkhuis BG, Tomelleri A, Gheysens O, Jiemy WF, Piccolo C, Nienhuis P, Sandovici M, Brouwer E, Glaudemans AW, Mulder DJ, Slart RH. Positron Emission Tomography Imaging in Vasculitis. Cardiol Clin 2023; 41:251-265. [PMID: 37003681 DOI: 10.1016/j.ccl.2023.01.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/21/2023]
Abstract
Systemic vasculitides comprise a group of autoimmune diseases affecting blood vessels. [18F]-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography (FDG-PET/CT) plays an important role in the diagnosis and therapeutic monitoring of vasculitides affecting large-sized and medium-sized vessels. FDG-PET/CT also provides complementary information to other vascular imaging tools. The resolution and sensitivity of newer generation scanners continues to increase, hereby improving the ability of FDG-PET/CT to accurately assess the full disease extent in patients with vasculitis. Novel tracers targeting specific immune cells will allow for more detailed detection of vascular infiltrates.
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15
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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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Yang H, Zhang H, Liu W, Han W, Guo T, Lai Y, Tan B, Wang C, Chen M, Gao X, Ran Z, Liu Z, Wu K, Cao Q, Qian J. Computed tomography enterography increases the ability of endoscopy to differentiate Crohn's disease from intestinal Behçet's disease. Front Med (Lausanne) 2022; 9:900458. [PMID: 36059846 PMCID: PMC9433799 DOI: 10.3389/fmed.2022.900458] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/08/2022] [Accepted: 07/19/2022] [Indexed: 11/13/2022] Open
Abstract
Background Distinguishing Crohn's disease (CD) and intestinal Behçet's disease (BD) is difficult in clinical practice. Aim To evaluate the ability of CT enterography (CTE) to enhance the diagnostic value of endoscopy in differentiating CD from intestinal BD and to establish differential diagnosis models. Methods A total of 113 patients with CD and 70 patients with intestinal BD from seven tertiary inflammatory bowel disease centers were enrolled. The univariate and multivariate analyses were used by SAS software version 9.2. Three differential scoring models based on the multivariate analysis of endoscopic features alone (model 1), endoscopic features combined with clinical symptoms (model 2), and endoscopic features combined with clinical symptoms and CTE (model 3) were established. Results The results showed that model 2 increased the efficacy of model 1 in differential diagnosis and model 3 had the highest accuracy of 84.15% at a cutoff value of two points. The scoring of model 3 was as follows: genital ulcer (−3 points), skin lesions (−3 points), oval ulcer (-2 points), longitudinal ulcer (1 point), number of ulcers > 5 (3 points), inflammatory polyps (2 points), mucosal severe enhancement (2 points), and fibrofatty proliferation (1 point). Conclusion Clinical symptoms and CTE increased the ability of endoscopy to differentiate CD from intestinal BD.
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Affiliation(s)
- Hong Yang
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy Medical Sciences and Peking Union Medical College, Beijing, China
| | - Huimin Zhang
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy Medical Sciences and Peking Union Medical College, Beijing, China
| | - Wei Liu
- Department of Radiology, Peking Union Medical College Hospital, Chinese Academy Medical Sciences and Peking Union Medical College, Beijing, China
| | - Wei Han
- Department of Epidemiology and Biostatistics, Institute of Basic Medical Sciences, Peking Union Medical College, Chinese Academy of Medical Sciences, School of Basic Medicine, Beijing, China
| | - Tao Guo
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yamin Lai
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy Medical Sciences and Peking Union Medical College, Beijing, China
| | - Bei Tan
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy Medical Sciences and Peking Union Medical College, Beijing, China
| | - Congling Wang
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy Medical Sciences and Peking Union Medical College, Beijing, China
| | - Minhu Chen
- Department of Gastroenterology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
| | - Xiang Gao
- Department of Gastroenterology, The Sixth Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
| | - Zhihua Ran
- Division of Gastroenterology and Hepatology, Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, Shanghai Inflammatory Bowel Disease Research Center, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
| | - Zhanju Liu
- Department of Gastroenterology, The Shanghai Tenth People's Hospital, Tongji University, Shanghai, China
| | - Kaichun Wu
- State Key Laboratory of Cancer Biology, National Clinical Research Center for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Qian Cao
- Department of Gastroenterology, Sir Run Run Shaw Hospital, College of Medicine Zhejiang University, Hangzhou, China
| | - Jiaming Qian
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy Medical Sciences and Peking Union Medical College, Beijing, China
- *Correspondence: Jiaming Qian
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Kawaguchi T, Fukata M, Omori T, Kiyohara H, Sugimoto S, Nanki K, Sujino T, Mikami Y, Kanai T. Efficacy of Calcineurin Inhibitors for Induction of Remission in Intestinal Behçet's Disease. CROHN'S & COLITIS 360 2022; 4:otac017. [PMID: 36777415 PMCID: PMC9802215 DOI: 10.1093/crocol/otac017] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/17/2022] [Indexed: 11/15/2022] Open
Abstract
Background The efficacy of calcineurin inhibitors (CNIs) for induction of remission in intestinal Behçet's disease (intestinal BD) has not been explored. Methods A multicenter retrospective case series study of patients with active intestinal BD treated with CNIs (cyclosporin and tacrolimus) was conducted. Results Of 16 patients, 12 (75%) showed a clinical response and 5 (31.3%) achieved clinical remission after 2 weeks of CNI treatment. Similar efficacy of CNIs was observed even in 7 patients refractory to antitumor necrosis factor-alpha therapies. Endoscopic improvement was observed in 11 of 12 patients. Conclusions CNIs may be promising treatment options for refractory intestinal BD.
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Affiliation(s)
- Takaaki Kawaguchi
- Address correspondence to: Takaaki Kawaguchi, MD, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan ()
| | - Masayuki Fukata
- Center for Inflammatory Bowel Disease, Division of Gastroenterology, Department of Internal Medicine, Tokyo Yamate Medical Center, Tokyo, Japan
| | - Teppei Omori
- Department of Internal Medicine, Institute of Gastroenterology, Tokyo Women’s Medical University, Tokyo, Japan
| | - Hiroki Kiyohara
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Shinya Sugimoto
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Kosaku Nanki
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Tomohisa Sujino
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Yohei Mikami
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Takanori Kanai
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
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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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Hou CC, Luo D, Bao HF, Ye JF, Ma HF, Shen Y, Zou J, Guan JL. Clinical heterogeneity of ocular Behçet's syndrome versus intestinal Behçet's syndrome: a cross-sectional study from Shanghai Behçet's syndrome database. Arthritis Res Ther 2022; 24:98. [PMID: 35488313 PMCID: PMC9052578 DOI: 10.1186/s13075-022-02782-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/15/2021] [Accepted: 04/19/2022] [Indexed: 12/15/2022] Open
Abstract
BACKGROUND Behçet's syndrome (BS) is a rare variant vasculitis which can involve the eyes and gastrointestinal systems. However, ocular involvement rarely overlaps with intestinal lesions. This study aimed to compare the clinical characteristics and laboratory parameters of ocular BS and intestinal BS patients in China and analyze the differences between two key phenotypes to verify the heterogeneous conditions in BS patients. METHODS A retrospective analysis was used to collect the demographic data, clinical characteristics, endoscopic findings, and laboratory parameters from 135 ocular BS and 174 intestinal BS patients. The Mann-Whitney U test and Pearson chi-square or continuity correction was used to analyze the differences between two groups. RESULTS Among 916 BS patients enrolled in this study, ocular BS and intestinal BS accounted for 14.74% (135 cases) and 19.00% (174 cases), respectively. Ocular and intestinal involvements overlapped in only 7 cases (0.76%). Male gender (74.8% vs. 51.1%, P=0.00), erythema nodosum (45.9% vs. 32.2%, P=0.01), and vascular involvement (6.7% vs. 1.7%, P=0.03) were more frequent in the ocular BS group compared with the intestinal BS group. On the contrary, hematologic involvement (7.5% vs. 0.0%, P=0.00) and fever (17.8% vs. 4.4%, P=0.00) were more frequent in the intestinal BS group compared with the ocular BS group. Additionally, the inflammation markers including ESR [26.5 (16.0-41.5) vs. 9.0 (5.0-15.0) mm/H, P=0.00], CRP [14.8 (4.8-33.0) vs. 4.1 (1.6-8.3) mg/L, P=0.00], serum amyloid A [27.4 (10.8-92.3) vs. 11.3 (6.0-24.0) mg/L, P=0.00], and interleukin 6 [8.4 (1.7-18.7) vs. 1.7 (1.5-3.2) pg/mL, P=0.00] were higher in the intestinal BS group than those in the ocular BS group, respectively. CONCLUSIONS Ocular BS was more prevalent in male patients and more likely to manifest with erythema nodosum and vascular involvement, while intestinal BS tends to have fever and hematologic disorders with higher inflammation markers. Ocular BS and intestinal BS are two distinct clinical phenotypes and very rarely overlapped.
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Affiliation(s)
- Cheng-cheng Hou
- Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan’an West Road, Shanghai, 200040 People’s Republic of China
| | - Dan Luo
- Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan’an West Road, Shanghai, 200040 People’s Republic of China
| | - Hua-fang Bao
- Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan’an West Road, Shanghai, 200040 People’s Republic of China
| | - Jing-fen Ye
- Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan’an West Road, Shanghai, 200040 People’s Republic of China
| | - Hai-fen Ma
- Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan’an West Road, Shanghai, 200040 People’s Republic of China
| | - Yan Shen
- Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan’an West Road, Shanghai, 200040 People’s Republic of China
| | - Jun Zou
- Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan’an West Road, Shanghai, 200040 People’s Republic of China
| | - Jian-long Guan
- Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan’an West Road, Shanghai, 200040 People’s Republic of China
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FACANALI CBG, FACANALI JUNIOR MR, RIBEIRO JUNIOR U, QUEIROZ NSF, SOBRADO JUNIOR CW, SAFATLE-RIBEIRO AV. SMALL BOWEL IS LARGELY AFFECTED IN BEHÇET’S DISEASE: A LONG-TERM FOLLOW-UP OF GASTROINTESTINAL SYMPTOMS. ARQUIVOS DE GASTROENTEROLOGIA 2022; 59:117-122. [PMID: 35442321 DOI: 10.1590/s0004-2803.202200001-20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/25/2021] [Accepted: 10/07/2021] [Indexed: 11/21/2022]
Abstract
ABSTRACT Background Behçet’s disease is a rare immune-mediated disorder that can affect the gastrointestinal tract. The prevalence and extension of small bowel involvement is largely unknown. Objective The aim of this study was to describe the small bowel lesions diagnosed by double-balloon enteroscopy (DBE) and to verify if these findings were associated to the presence of gastrointestinal symptoms and disease activity after long-term follow-up. Methods This study included 19 Behçet’s disease patients who underwent DBE. After a mean follow-up of 15 years the endoscopic findings were associated to the presence of gastrointestinal symptoms, disease activity and current therapy through collection of electronic medical records. Results A total of 63.2% patients were female and the mean age was 37 years at the time of DBE. Mean disease duration at baseline was 24 years. 11 patients had no gastrointestinal symptoms and eight patients presented either abdominal pain, gastrointestinal bleeding or diarrhea. The average procedure time was 1 hour and 30 minutes and the ileum was achieved in all patients but one. Small bowel ulcers were diagnosed in 78.9%, with 63.1% of jejunal involvement. Two patients presented only small bowel edema and two were normal by DBE. Eight patients had concomitant gastric ulcers. Gastrointestinal symptoms prior to DBE were present in 36.8% of the patients and, after follow-up, all of them persisted with some of the symptoms. Bleeding was reported by three patients at baseline and persisted in only one patient. The frequency of treatment with steroids and immunomodulators was 31.6% and 57.9% at baseline, respectively, and 21% in both at the end of the follow-up. No patient was treated with biologics at the time of the DBE procedure and the current rate of biologic use is 21%. Conclusion Small bowel involvement in Behçet’s disease was frequently demonstrated by DBE even in asymptomatic patients. Understanding clinical evolution of the disease over the years and the impact of such diagnosis still represents a challenge, possibly with the need for novel treatment.
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Nguyen A, Upadhyay S, Javaid MA, Qureshi AM, Haseeb S, Javed N, Cormier C, Farooq A, Sheikh AB. Behcet's Disease: An In-Depth Review about Pathogenesis, Gastrointestinal Manifestations, and Management. Inflamm Intest Dis 2022; 6:175-185. [PMID: 35083283 DOI: 10.1159/000520696] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/15/2021] [Accepted: 11/03/2021] [Indexed: 11/19/2022] Open
Abstract
Background Behcet's disease (BD) is a complex inflammatory vascular disorder that follows a relapsing-remitting course with diverse clinical manifestations. The prevalence of the disease varies throughout the globe and targets different age-groups. There are many variations of BD; however, intestinal BD is not only more common but has many signs and symptoms. Summary BD is a relapsing-remitting inflammatory vascular disorder with multiple system involvement, affecting vessels of all types and sizes that targets young adults. The etiology of BD is unknown but many factors including genetic mechanisms, vascular changes, hypercoagulability, and dysregulation of immune function are believed to be responsible. BD usually presents with signs and symptoms of ulcerative disease of the small intestine; endoscopy being consistent with the clinical manifestations. The mainstay of treatment depends upon the severity of the disease. Corticosteroids are recommended for severe forms of the disease and aminosalicylic acids are used in maintaining remission in mild to moderate forms of the disease. Key Messages In this review, we have tried to summarize in the present review the clinical manifestations, differential diagnoses, and management of intestinal BD. Hopefully, this review will enable health policymakers to ponder over establishing clear endpoints for treatment, surveillance investigations, and creating robust algorithms.
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Affiliation(s)
- Anthony Nguyen
- Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA
| | - Shubhra Upadhyay
- Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA
| | | | - Abdul Moiz Qureshi
- Department of Internal Medicine, Shifa College of Medicine, Shifa Tameer-e-Millat University, Islamabad, Pakistan
| | - Shahan Haseeb
- Department of Internal Medicine, Shifa College of Medicine, Shifa Tameer-e-Millat University, Islamabad, Pakistan
| | - Nismat Javed
- Department of Internal Medicine, Shifa College of Medicine, Shifa Tameer-e-Millat University, Islamabad, Pakistan
| | - Christopher Cormier
- Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA
| | - Asif Farooq
- Department of Family and Community Medicine, Texas Tech Health Sciences Center, Lubbock, Texas, USA
| | - Abu Baker Sheikh
- Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA
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22
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Arbrile M, Radin M, Rossi D, Menegatti E, Baldovino S, Sciascia S, Roccatello D. Vedolizumab for the Management of Refractory Behçet's Disease: From a Case Report to New Pieces of Mosaic in a Complex Disease. Front Immunol 2021; 12:769785. [PMID: 34759935 PMCID: PMC8573273 DOI: 10.3389/fimmu.2021.769785] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2021] [Accepted: 10/04/2021] [Indexed: 12/12/2022] Open
Abstract
Objectives When treating Behçet’s disease (BD), anti-tumor necrosis factor (TNF)-α agents have become a second-line therapy when conventional immunosuppressive drugs have failed. However, in the case of failure of treatment with anti-TNFα drugs, further options are limited. Based on previous reports of the efficacy of vedolizumab (VDZ) in inflammatory bowel diseases, we decided to administer VDZ to treat a patient with intestinal BD. Methods We present the case of a 49-year-old female patient with BD. Her clinical manifestations included erythema nodosum, oro-genital ulcers, positive Pathergy test, positive HLA-B51, and biopsy-proven intestinal BD. The patient was unsuccessfully treated with conventional immunosuppressive and several biological agents. Results Treatment with VDZ was started intravenously at a dose of 300 mg at 0, 2, and 6 weeks and then every 4 weeks. After the second dose of VDZ, the patient reported a marked improvement of intestinal BD and a concomitant amelioration of arthralgia, erythema nodosum lesions and aphthosis. Clinical remission was achieved at 6 months after starting VDZ. Conclusion VDZ might represent a valid option to treat patients with BD who are non-responsive to standard treatments or anti-TNFα agents, particularly, those cases with intestinal involvement.
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Affiliation(s)
- Marta Arbrile
- Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, Complex Structure with University Management (SCDU) Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy
| | - Massimo Radin
- Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, Complex Structure with University Management (SCDU) Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy
| | - Daniela Rossi
- Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, Complex Structure with University Management (SCDU) Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy
| | - Elisa Menegatti
- Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, Complex Structure with University Management (SCDU) Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy
| | - Simone Baldovino
- Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, Complex Structure with University Management (SCDU) Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy
| | - Savino Sciascia
- Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, Complex Structure with University Management (SCDU) Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy
| | - Dario Roccatello
- Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, Complex Structure with University Management (SCDU) Nephrology and Dialysis, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy
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23
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Kang EA, Park JW, Park Y, Park SJ, Kim TI, Kim WH, Cho MS, Cheon JH. C-reactive protein is associated with postoperative outcomes in patients with intestinal Behçet's disease. BMC Gastroenterol 2021; 21:362. [PMID: 34620099 PMCID: PMC8496041 DOI: 10.1186/s12876-021-01922-2] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2021] [Accepted: 09/09/2021] [Indexed: 11/25/2022] Open
Abstract
BACKGROUND Patients with intestinal Behçet's disease (BD) frequently undergo intestinal resections, which significantly affects postoperative morbidity and mortality. The aim of this study was to identify the association between C-reactive protein (CRP) levels and postoperative outcomes in patients with intestinal BD who underwent surgical bowel resection. METHODS Patients who were diagnosed with intestinal BD and underwent intestinal surgery due to BD at Severance Hospital between November 2005 and April 2018 were retrospectively investigated. Clinical relapse was defined as a disease activity index of BD (DAIBD) > 40, existence of newly added medications, re-hospitalization, or re-operation related to intestinal BD. The relationship between CRP level and postoperative outcomes was analyzed, and a receiver operating characteristic (ROC) curve was drawn to specify a cut-off value. RESULTS Ninety patients with intestinal BD were included. Among them, 44 were male (48.9%), and the median age at diagnosis was 38 years (range, 11-69 years). The median total disease follow-up duration was 130 months (range, 3-460 months). Forty patients (44.4%) underwent laparoscopic surgery. A higher CRP level immediately after surgery was significantly associated with postoperative complications (OR 1.01, 95% CI 1.004-1.018, p < 0.01), re-operation (hazard ratio [HR] 1.01, 95% CI 1.005-1.020, p < 0.01), and re-admission (HR 1.01, 95% CI 1.006-1.017 p < 0.01). The ROC curve showed that CRP predicts the risk of postoperative complications (p < 0.01) at a cut-off value of 41.9% with a sensitivity of 60.0% and specificity of 67.7%. CONCLUSIONS Postoperative CRP levels in patients with intestinal BD undergoing surgical resection were associated with postoperative outcomes.
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Affiliation(s)
- Eun Ae Kang
- Gastroenterology and Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea
| | - Jung Won Park
- Gastroenterology and Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea
| | - Yehyun Park
- Gastroenterology and Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea
| | - Soo Jung Park
- Gastroenterology and Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea
| | - Tae Il Kim
- Gastroenterology and Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea
| | - Won Ho Kim
- Gastroenterology and Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea
| | - Min Soo Cho
- Division of Colon and Rectal Surgery, Department of Surgery, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.
| | - Jae Hee Cheon
- Gastroenterology and Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.
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Yazici Y, Hatemi G, Bodaghi B, Cheon JH, Suzuki N, Ambrose N, Yazici H. Behçet syndrome. Nat Rev Dis Primers 2021; 7:67. [PMID: 34531393 DOI: 10.1038/s41572-021-00301-1] [Citation(s) in RCA: 84] [Impact Index Per Article: 21.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 08/11/2021] [Indexed: 12/17/2022]
Abstract
Behçet syndrome is a systemic vasculitis with an unknown aetiology affecting the small and large vessels of the venous and arterial systems. The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest that multiple pathological pathways are involved in Behçet syndrome. These disease features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 as a genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bona fide disease, especially in non-endemic regions, suggests that other factors must also be operative in Behçet syndrome. Despite lacking a clear aetiological mechanism and definition, management of manifestations that include major vascular disease, eye disease and central nervous system involvement has improved with the help of new technology. Furthermore, even with our incomplete understanding of disease mechanisms, the prognoses of patients with Behçet syndrome, including those with eye disease, continue to improve. New treatment options and a better understanding of the underlying pathogenesis for various manifestations of this condition are required to further improve the management of the disease, which will improve patient quality of life.
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Affiliation(s)
- Yusuf Yazici
- Division of Rheumatology, New York University School of Medicine, New York, NY, USA.
| | - Gulen Hatemi
- Division of Rheumatology, Department of Internal Medicine and Behçet's Disease Research Center, Istanbul University - Cerrahpasa, Istanbul, Turkey
| | - Bahram Bodaghi
- Sorbonne University, IHU FOReSIGHT, Pitié-Salpêtrière Hospital UMR 7211 UPMC/CNRS, U972 INSERM, Transimmunom Laboratory of Excellence Sorbonne University, Paris, France
| | - Jae Hee Cheon
- Department of Internal Medicine, Yonsei University College of Medicine, Seodaemun-gu, Seoul, Korea
| | - Noburu Suzuki
- Department of Immunology and Medicine, St. Marianna University School of Medicine, Miyamae-ku, Kawasaki, Japan
| | | | - Hasan Yazici
- (Rheumatology) Academic Hospital Istanbul, Istanbul, Turkey
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25
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Wang Y, Geng P, Yin J, Xiao Q, Xiong J, Ling B, Wang H, Xu J. Recurrent high fever with macular papules in an elderly male: a case report. Immunol Res 2021; 69:612-617. [PMID: 34417959 DOI: 10.1007/s12026-021-09227-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2021] [Accepted: 08/14/2021] [Indexed: 11/26/2022]
Affiliation(s)
- Ying Wang
- Dalian Medical University, DalianLiaoning, 116044, China
| | - Ping Geng
- Department of Emergency Medicine, Northern Jiangsu People's Hospital, Clinical Medical College, Yangzhou University, Yangzhou, 225001, Jiangsu, China.
| | - Jian Yin
- Department of gastroenterology, Northern Jiangsu People's Hospital, Clinical Medical College, Yangzhou University, Jiangsu, 225001, Yangzhou, China
| | - Qin Xiao
- Department of pathology, Northern Jiangsu People's Hospital, Clinical Medical College, Yangzhou University, Jiangsu, 225001, Yangzhou, China
| | - Jiali Xiong
- Dalian Medical University, DalianLiaoning, 116044, China
| | - Bingyu Ling
- Department of Emergency Medicine, Northern Jiangsu People's Hospital, Clinical Medical College, Yangzhou University, Yangzhou, 225001, Jiangsu, China
| | - Huihui Wang
- Department of Emergency Medicine, Northern Jiangsu People's Hospital, Clinical Medical College, Yangzhou University, Yangzhou, 225001, Jiangsu, China
| | - Jiyang Xu
- Department of Emergency Medicine, Northern Jiangsu People's Hospital, Clinical Medical College, Yangzhou University, Yangzhou, 225001, Jiangsu, China
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26
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Kim JM, Kang JG, Kim S, Cheon JH. Deep-learning system for real-time differentiation between Crohn's disease, intestinal Behçet's disease, and intestinal tuberculosis. J Gastroenterol Hepatol 2021; 36:2141-2148. [PMID: 33554375 DOI: 10.1111/jgh.15433] [Citation(s) in RCA: 19] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/18/2020] [Revised: 11/14/2020] [Accepted: 01/31/2021] [Indexed: 12/13/2022]
Abstract
BACKGROUND AND AIM Pattern analysis of big data can provide a superior direction for the clinical differentiation of diseases with similar endoscopic findings. This study aimed to develop a deep-learning algorithm that performs differential diagnosis between intestinal Behçet's disease (BD), Crohn's disease (CD), and intestinal tuberculosis (ITB) using colonoscopy images. METHODS The typical pattern for each disease was defined as a typical image. We implemented a convolutional neural network (CNN) using Pytorch and visualized a deep-learning model through Gradient-weighted Class Activation Mapping. The performance of the algorithm was evaluated using the area under the receiver operating characteristic curve (AUROC). RESULTS A total of 6617 colonoscopy images of 211 CD, 299 intestinal BD, and 217 ITB patients were used. The accuracy of the algorithm for discriminating the three diseases (all-images: 65.15% vs typical images: 72.01%, P = 0.024) and discriminating between intestinal BD and CD (all-images: 78.15% vs typical images: 85.62%, P = 0.010) was significantly different between all-images and typical images. The CNN clearly differentiated colonoscopy images of the diseases (AUROC from 0.7846 to 0.8586). Algorithmic prediction AUROC for typical images ranged from 0.8211 to 0.9360. CONCLUSION This study found that a deep-learning model can discriminate between colonoscopy images of intestinal BD, CD, and ITB. In particular, the algorithm demonstrated superior discrimination ability for typical images. This approach presents a beneficial method for the differential diagnosis of the diseases.
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Affiliation(s)
- Jung Min Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Jun Gu Kang
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Sungwon Kim
- Department of Radiology, Research Institute of Radiological Science and Center for Clinical Image Data Science, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Republic of Korea.,Brain Korea 21 PLUS Project for Medical Science, Yonsei University College of Medicine, Seoul, Republic of Korea
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27
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Yan J, Yan Y, Young A, Yan Z, Yan Z. Effectiveness and Safety of Chinese Medicine Decoctions for Behcet's Disease: A Systematic Review and Meta-Analysis. EVIDENCE-BASED COMPLEMENTARY AND ALTERNATIVE MEDICINE : ECAM 2021; 2021:8202512. [PMID: 34335839 PMCID: PMC8313333 DOI: 10.1155/2021/8202512] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 04/10/2021] [Revised: 06/22/2021] [Accepted: 07/03/2021] [Indexed: 11/26/2022]
Abstract
BACKGROUND Behcet's disease (BD) is an autoimmune disease of systemic vasculitis with an unclear pathogenesis. Although western medicines remain the mainstay interventions, effectiveness and safety are significant challenges. Complementary and alternative medicine, including herbal medicine, are gaining more attention. Chinese medicine decoctions, which have been used for centuries, are the most common form of traditional therapies. OBJECTIVE The purpose of the review was to evaluate the effectiveness and safety of Chinese medicine decoctions in the treatment of BD. METHODS Randomized controlled trials (RCTs) for BD treatment with Chinese medicine decoctions were searched in six electronic databases until March 2021. Primary outcomes were total effective rate, recovery rate, and recurrence rate. Secondary outcomes were clinical feature scores (oral ulcers, eye lesions, genital ulcers, skin lesions, arthropathies, fever, and pathergy reactions) and laboratory index levels (erythrocyte sedimentation rate, C-reactive protein, and immunoglobulin A). The risk of bias was assessed with the Cochrane Handbook, and a meta-analysis was performed with RevMan 5.4.1. RESULTS Sixteen RCTs with 924 patients were included in the review. The meta-analysis indicated that Chinese medicine decoctions were effective for BD when compared with control groups for all the primary outcomes and 7/10 of the secondary outcomes. Adverse events were reported in 11 of the 16 RCTs, with the Chinese medicine decoctions possibly having fewer adverse events than western drugs. This review included a range of classical prescriptions. An additional meta-analysis of modified Gancao Xiexin Decoction for BD treatment was conducted. Gancao Xiexin decoction is also discussed as a representative prescription, as well as high-frequency herbs, and warrants further exploration for individualized medicine and pharmacology. CONCLUSION Chinese medicine decoctions have the potential to be effective and safe for treating BD. However, additional well-designed RCTs are needed to confirm the findings because of the unsatisfactory quality of the included studies.
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Affiliation(s)
- Jingxian Yan
- Chinese Medical College, Tianjin University of Traditional Chinese Medicine, Tianjin 301617, China
| | - Yi Yan
- Xinglin College, Liaoning University of Traditional Chinese Medicine, Shenyang 110167, China
| | - Andrew Young
- Department of Diagnostic Sciences, Arthur Dugoni School of Dentistry, University of the Pacific, Stockton, CA, USA
| | - Zhiyong Yan
- Department of Surgery, Handan First Hospital, Handan 056000, China
| | - Zhimin Yan
- Department of Oral Medicine, Peking University School and Hospital of Stomatology, Beijing 100081, China
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28
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Alpsoy E, Bozca BC, Bilgic A. Behçet Disease: An Update for Dermatologists. Am J Clin Dermatol 2021; 22:477-502. [PMID: 34061323 DOI: 10.1007/s40257-021-00609-4] [Citation(s) in RCA: 22] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/04/2021] [Indexed: 12/13/2022]
Abstract
Behçet disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology with the clinical features of oral and genital ulcers, cutaneous vasculitic lesions, ocular, articular, vascular, gastrointestinal, neurologic, urogenital and cardiac involvement. BD usually appears around the third or fourth decade of life. Gender distribution is roughly equal. The disease is much more frequent in populations along the ancient 'Silk Road', extending from Eastern Asia to countries in the Middle East and the Mediterranean, compared with Western countries, but has universal distribution. Mucocutaneous manifestations are the clinical hallmarks of BD. The diagnostic criteria widely used in the disease's diagnosis are based on mucocutaneous manifestations because of their high sensitivity and/or specificity. Genetic factors are the key driver of BD pathogenesis, and HLA-B51 antigen is the strongest genetic susceptibility factor. Streptococcus sanguinis (S. sanguinis) or microbiome change can trigger innate immune system-mediated inflammation sustained by adaptive immune responses. Epistatic interaction between HLA-B51 and endoplasmic reticulum aminopeptidase 1 (ERAP1) in antigen-presenting cells disrupt T-cell homeostasis leading to downregulation of Tregs and expansion of Th1 and Th17. Thus, neutrophil activation and intense neutrophil infiltration of the affected organs develop in the early stage of inflammation. BD has a variable clinical course with unpredictable exacerbations and remissions. The disease is associated with a high mortality rate, especially in young male patients, and large-vessel, neurological, gastrointestinal system and cardiac involvement are the most important causes of death. The principal aim of treatment should be to prevent irreversible organ damage, especially during the disease's early, active phase. A better understanding of the disease's pathogenesis has provided important information on its management. New drugs, especially apremilast and anti-TNF-α agents are effective in the management of BD and have the potential to improve patients' quality of life, prognosis and survival.
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Affiliation(s)
- Erkan Alpsoy
- Department of Dermatology and Venereology, Akdeniz University School of Medicine, 07059, Antalya, Turkey.
| | - Burcin Cansu Bozca
- Department of Dermatology and Venereology, Akdeniz University School of Medicine, 07059, Antalya, Turkey
| | - Asli Bilgic
- Department of Dermatology and Venereology, Akdeniz University School of Medicine, 07059, Antalya, Turkey
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Sun L, Liu J, Jin X, Wang Z, Li L, Bai W, Yang Y, Wu C, Chen W, Xu S, Zheng J, Zheng W. Perioperative management with biologics on severe aortic valve regurgitation caused by Behçet syndrome: the experience from a single center. Ther Adv Chronic Dis 2021; 12:20406223211026753. [PMID: 34221307 PMCID: PMC8221692 DOI: 10.1177/20406223211026753] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2020] [Accepted: 06/02/2021] [Indexed: 11/15/2022] Open
Abstract
Background To investigate the efficacy and safety of biologics in the perioperative management of severe aortic valve regurgitation (AR) caused by Behçet syndrome (BS). Methods We retrospectively analyzed 20 patients with severe AR caused by BS who were all treated with biologics during the perioperative period of cardiac surgeries in our center between February 2016 and October 2020. Results A total of 20 patients with severe AR were enrolled, including 19 males and 1 female, with a mean age of 39.1 ± 8.8 years and a median course of 8 [interquartile range (IQR) 5.25-10.00] years. Before biologic administration, 92.9% of the patients who underwent aortic valve replacement had failed conventional therapy and developed postoperative paravalvular leakage (PVL) at a median interval of 4 months. Biologics were administered with background glucocorticoids (GCs) and immunosuppressants during the perioperative period for 22 aortic valve surgeries, including preoperatively with a median interval of 3.5 (IQR 2.75-4.25) months in 13 cases and within 3 months postoperatively in 9 cases. After a median follow up of 21 (IQR 15-32) months, 2 out of 13 cases (15.4%) preoperatively, and 1 out of 9 cases (11.1%) postoperatively treated with biologics developed PVL, and the rest were event free. The Behçet's Disease Current Activity Form score improved significantly (7 versus 0, median, p < 0.0001). Decrease of erythrocyte sedimentation rate [25.0 (IQR 11.00-36.25) mm/h versus 6.5 (IQR 4.0-8.8) mm/h, p < 0.001], and C-reactive protein [20.77 (IQR 7.19-29.58) mg/l versus 1.53 (IQR 0.94-2.92) mg/l, p = 0.001] were achieved rapidly and effectively. The GC dosage tapered from 40 (IQR 30-60) mg/d to 10 (IQR 5-11.25) mg/d, p < 0.0001. Immunosuppressants were tapered in number and dosage in 6 (30%) and 20 patients (100%), respectively. No serious adverse event was observed. Conclusion Our study suggests that biologics were effective and well tolerated for the perioperative management of severe and refractory AR caused by BS, which significantly reduced the occurrence of postoperative PVL and had favorable GC- and immunosuppressant-sparing effect.
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Affiliation(s)
- Luxi Sun
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
| | - Jinjing Liu
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
| | - Xiufeng Jin
- Beijing Anzhen Hospital, Beijing Institute of Heart, Lung & Vascular Diseases, Beijing Aortic Disease Center, Capital Medical University, Beijing, China
| | - Zhimian Wang
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
| | - Lu Li
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
| | - Wei Bai
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
| | - Yunjiao Yang
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
| | - Chanyuan Wu
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
| | - Wei Chen
- Department of Cardiology, Peking Union Medical College Hospital, Beijing, China
| | - Shangdong Xu
- Beijing Anzhen Hospital, Beijing Institute of Heart, Lung & Vascular Diseases, Beijing Aortic Disease Center, Capital Medical University, Beijing, China
| | - Jun Zheng
- Beijing Anzhen Hospital, Beijing Institute of Heart, Lung & Vascular Diseases, Beijing Aortic Disease Center, Capital Medical University, Chaoyang-qu, Beijing, China
| | - Wenjie Zheng
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, No.1 Shuaifuyuan Hutong, Dongcheng-qu, Beijing 100730, China
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Zeng L, Meng WJ, Wen ZH, Chen YL, Wang YF, Tang CW. Management and outcomes of surgical patients with intestinal Behçet’s disease and Crohn’s disease in southwest China. World J Clin Cases 2021; 9:3858-3868. [PMID: 34141742 PMCID: PMC8180223 DOI: 10.12998/wjcc.v9.i16.3858] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/02/2020] [Revised: 03/01/2021] [Accepted: 04/06/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Gastrointestinal involvement in Behçet's disease (GIBD) and Crohn’s disease (CD) are inflammatory diseases sharing a considerable number of similarities. However, different from CD, the operative and postoperative management of GIBD remains largely empirical because of the lack of comprehensive treatment guidelines.
AIM To compare surgical patients with GIBD and those with CD in a medical center and identify notable clinical features and effective postoperative treatment for surgical patients with GIBD.
METHODS We searched patients diagnosed with CD and GIBD who underwent operations for gastrointestinal complications from 2009 to 2015 at West China Hospital of Sichuan University. A total of 10 surgical patients with GIBD and 106 surgical patients with CD were recruited. Information including demographic data, medication, and operative and postoperative parameters were collected and analyzed. As the incidence of surgical GIBD is low, their detailed medical records were reviewed and compared to previous studies. Moreover, the prognoses of CD and GIBD were evaluated respectively between groups treated with biological and non-biological agents.
RESULTS Indication for first surgery was often acute intestinal perforation for GIBD patients (7/10 vs 0/106, P < 0.001), whereas intestinal fistulae (0/10 vs 44/106, P = 0.013) and ileus (0/10 vs 40/106, P = 0.015) were the indications for surgical CD patients. Approximately 40% of patients with GIBD and 23.6% of patients with CD developed postoperative complications, 50% of patients with GIBD and 38.7% of patients with CD had recurrence postoperatively, and 40% (4/10) of patients with GIBD and 26.4% (28/106) of patients with CD underwent reoperations. The average period of postoperative recurrence was 7.87 mo in patients with Behçet's disease (BD) and 10.43 mo in patients with CD, whereas the mean duration from first surgery to reoperation was 5.75 mo in BD patients and 18.04 mo in CD patients. Surgical patients with GIBD more often used corticosteroids (6/10 vs 7/106, P < 0.001) and thalidomide (7/10 vs 9/106, P < 0.001) postoperatively, whereas surgical patients with CD often used infliximab (27/106), azathioprine, or 6-mercaptopurine (74/106) for maintenance therapy.
CONCLUSION Patients suffering GIBD require surgery mostly under emergency situations, which may be more susceptible to recurrence and reoperation and need more aggressive postoperative treatment than patients with CD.
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Affiliation(s)
- Li Zeng
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Wen-Jian Meng
- Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Zhong-Hui Wen
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Yi-Long Chen
- West China Biomedical Big Data Center, West China Hospital/West China School of Medicine, Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Yu-Fang Wang
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Cheng-Wei Tang
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
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Park J, Jeong D, Chung YW, Han S, Kim DH, Yu J, Cheon JH, Ryu JH. Proteomic analysis-based discovery of a novel biomarker that differentiates intestinal Behçet's disease from Crohn's disease. Sci Rep 2021; 11:11019. [PMID: 34040049 PMCID: PMC8155054 DOI: 10.1038/s41598-021-90250-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2020] [Accepted: 05/05/2021] [Indexed: 12/25/2022] Open
Abstract
Intestinal Behçet's disease (BD) and Crohn's disease (CD) present similar manifestations, but there are no specific diagnostic tests to differentiate them. We used a proteomic approach to discover novel diagnostic biomarkers specific to intestinal BD. Colon mucosa tissue samples were obtained from patients with intestinal BD or CD using colonoscopy-guided biopsy of the affected bowel. Peptides from seven intestinal BD and seven CD patients were extracted and labeled using tandem mass tag (TMT) reagents. The labeled peptides were identified and quantified using liquid chromatography-tandem mass spectrometry (LC-MS/MS). The proteins were further validated using immunohistochemical (IHC) analysis with tissue samples and an ELISA test with serum samples from 20 intestinal BD and 20 CD patients. Using TMT/LC-MS/MS-based proteomic quantification, we identified 39 proteins differentially expressed between intestinal BD and CD. Beta-2 glycoprotein 1 (APOH) and maltase-glucoamylase (MGAM) showed higher intensity in the IHC staining of intestinal BD tissues than in CD tissues. The serum MGAM level was higher in intestinal BD patients. Proteomic analysis revealed that some proteins were differentially expressed in patients with intestinal BD compared with those with CD. Differential MGAM expression in intestinal BD suggests its role as a potential novel diagnostic biomarker.
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Affiliation(s)
- Jihye Park
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Daeun Jeong
- Severance Biomedical Science Institute, 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
| | - Youn Wook Chung
- Severance Biomedical Science Institute, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
- Airway Mucus Institute, Yonsei University College of Medicine, Seoul, 03722, Korea
| | - Seunghan Han
- Severance Biomedical Science Institute, 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
| | - Da Hye Kim
- Severance Biomedical Science Institute, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Jongwook Yu
- Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, 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, 03722, Korea.
- Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea.
| | - Ji-Hwan Ryu
- Severance Biomedical Science Institute, 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.
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Kötter I, Lötscher F. Behçet's Syndrome Apart From the Triple Symptom Complex: Vascular, Neurologic, Gastrointestinal, and Musculoskeletal Manifestations. A Mini Review. Front Med (Lausanne) 2021; 8:639758. [PMID: 33898481 PMCID: PMC8063110 DOI: 10.3389/fmed.2021.639758] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2020] [Accepted: 03/12/2021] [Indexed: 12/13/2022] Open
Abstract
Behçet‘s Syndrome (BS) is a variable vessel vasculitis according to the Chapel Hill Consensus Nomenclature (1) and may thus affect any organ, including major and minor arterial and venous vessels to a varying degree and with varying frequency. Although the main features of BS are recurrent oral and genital aphthous ulcers, cutaneous lesions, ocular inflammation and arthritis—major vessel and life—or organ threatening involvement of internal organs and the central and peripheral nervous system occur. In general, BS in Europe appears to form six phenotypes of clinical manifestations (2), which are (1) mucocutaneous only, (2) predominant arthritis/articular involvement, (3) vascular phenotype, (4) ocular manifestations, which are most likely associated with CNS manifestations and HLA-B51, (5) dominant parenchymal CNS manifestations (being associated with the ocular ones), and (6) gastrointestinal involvement. Mucocutaneous manifestations are present in almost all patients/all phenotypes. In the following review, we summarize the current knowledge concerning vascular, neurologic, gastrointestinal and musculoskeletal manifestations of the disease.
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Affiliation(s)
- Ina Kötter
- Division of Rheumatology and Inflammatory Rheumatic Diseases, University Hospital Hamburg Eppendorf and Clinic for Rheumatology and Immunology Bad Bramstedt, Bad Bramstedt, Germany
| | - Fabian Lötscher
- Department of Rheumatology and Immunology, Inselspital Bern, University of Bern, Bern, Switzerland
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Hou CC, Ye JF, Ma HF, Guan JL. Clinical characteristics and risk factors of intestinal involvement in Behçet's syndrome patients: a cross-sectional study from a single center. Orphanet J Rare Dis 2021; 16:132. [PMID: 33731182 PMCID: PMC7972242 DOI: 10.1186/s13023-021-01772-x] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2020] [Accepted: 03/09/2021] [Indexed: 12/15/2022] Open
Abstract
BACKGROUND Intestinal Behçet's syndrome (BS) has high morbidity and mortality rates with serious complications. The purpose of this study was to investigate the clinical characteristics and laboratory parameters of intestinal and mucocutaneous BS patients and analyze the risk factors of intestinal involvement in BS patients. METHODS A retrospective analysis was used to collect the demographic data and laboratory parameters from 97 intestinal and 154 mucocutaneous BS patients. Univariate and multivariate logistic regression analyses were used to investigate the risk factors of intestinal involvement in BS patients. RESULTS The most common clinical manifestations of first onset in intestinal BS patients were oral ulceration (100.00%), followed by genital ulcers (62.89%) and erythema nodule (28.87%), gastrointestinal lesions (28.87%), pseudofolliculitis (25.77%), fever (17.53%), arthritis (16.49%), ocular involvement (5.15%), while the least common were vascular involvement (2.06%) and hematologic involvement involvement (2.06%). The most common intestinal segment involved in intestinal BS patients was terminal ileum (30.9%), followed by ileocecal (18.6%), colon (15.5%). By univariate logistic regression analysis, gender, age at hospitalization, age of disease onset, BDCAF, T-SPOT, fever, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), leukocyte, erythrocyte, hemoglobin (HGB), neutrophil-to-lymphocyte ratio, serum amyloid A, complement 3, albumin, total cholesterol, high-density lipoprotein and interleukin 6 (IL-6) were found all risk factors of intestinal involvement in BS patients (P < 0.05 or P = 0.00). Moreover, gender (male), BDCAF (≥ 2), ESR (≥ 15 mm/H), CRP (> 10 mg/L), HGB (< 130 g/L) and IL-6 (> 7 pg/ml) were found the independent risk factors of intestinal involvement in BS patients (all P < 0.05). CONCLUSIONS More attention shall be paid to gender, BDCAF, ESR, CRP, HGB and IL-6 in BS patients. When gender (male), BDCAF (≥ 2), ESR (≥ 15 mm/H), CRP (> 10 mg/L), HGB (< 130 g/L) and IL-6 (> 7 pg/ml) being observed, it may reminds that the presence of intestinal involvement in BS patients.
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Affiliation(s)
- Cheng-cheng Hou
- Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan’an west Road, Shanghai, 200040 People’s Republic of China
| | - Jing-fen Ye
- Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan’an west Road, Shanghai, 200040 People’s Republic of China
| | - Hai-fen Ma
- Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan’an west Road, Shanghai, 200040 People’s Republic of China
| | - Jian-long Guan
- Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan’an west Road, Shanghai, 200040 People’s Republic of China
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Gendreau S, Porcher R, Thoreau B, Paule R, Maurier F, Goulenok T, Frumholtz L, Bernigaud C, Ingen-Housz-Oro S, Mekinian A, Audemard-Verger A, Gaillet A, Perard L, Samson M, Sonneville R, Arlet JB, Mirouse A, Kahn JE, Charpentier J, Hachulla É, Hummel A, Pires T, Carron PL, Durel CA, Jourde W, Puechal X, Lega JC, Sarrot-Reynauld F, Tieulie N, Diot E, Guillevin L, Terrier B. Characteristics and risk factors for poor outcome in patients with systemic vasculitis involving the gastrointestinal tract. Semin Arthritis Rheum 2021; 51:436-441. [PMID: 33711774 DOI: 10.1016/j.semarthrit.2021.03.002] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/26/2020] [Revised: 02/04/2021] [Accepted: 03/01/2021] [Indexed: 01/13/2023]
Abstract
BACKGROUND Gastrointestinal (GI) involvement was described to be a poor prognostic factor in systemic necrotizing vasculitis. Its prognostic significance may vary according to clinical presentation and vasculitis subtype. AIMS This study investigated risk-factors associated to poor outcome in GI-involvement of vasculitis. METHODS Patients with systemic vasculitis as defined by the 2012 Chapel Hill Consensus Conference and presenting with GI involvement were retrospectively included. Baseline characteristics, treatments and outcome were recorded. Primary endpoint was a composite of admission to intensive care unit (ICU), emergency surgical procedure, or death. RESULTS Two hundred and thirteen patients were included. Vasculitis were distributed as follows: 41% IgA vasculitis, 27% ANCA-associated vasculitis, 17% polyarteritis nodosa (PAN), and 15% other vasculitis. Eighty-three (39%) patients fulfilled the composite primary endpoint within 6 months. Predictive factors associated with the primary endpoint included PAN subtype (OR 3.08, 95% CI 1.29-7.34), performance status (OR 1.40, 1.05-1.87), use of morphine (OR 2.51, 0.87-7.24), abdominal guarding (OR 3.08, 1.01-9.37), ileus (OR 2.29, 0.98-5.32), melena (OR 2.74, 1.17-6.42), increased leukocytes (per G/L, OR 1.05, 1.00-1.10), low hemoglobin (per g/dL, OR 0.80, 0.71-0.91) and increased CRP (log mg/L, OR 1.21, 0.94-1.56). A risk prediction model for the achievement of primary endpoint had a very good performance [C-statistics 0.853 (0.810 to 0.895], and for overall survival as well. CONCLUSIONS Vasculitis presenting with GI involvement have a poor outcome in more than one third of cases. An easy-to-use risk prediction model had a very good performance to predict the admission to ICU, emergency surgical procedure, or death.
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Affiliation(s)
- Ségolène Gendreau
- Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
| | - Raphael Porcher
- Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité, Hôtel-Dieu, Paris, France
| | - Benjamin Thoreau
- Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
| | - Romain Paule
- Department of Internal Medicine, Hôpital Foch, Suresnes, France
| | - François Maurier
- Department of Internal Medicine, Hôpital Sainte-Blandine De Metz, Metz, France
| | - Tiphaine Goulenok
- Department of Internal Medicine, Hôpital Bichat - Claude-Bernard, AP-HP, Paris, France
| | - Laure Frumholtz
- Department of Dermatology, Hôpital Saint-Louis, AP-HP, Paris, France
| | | | | | - Arsène Mekinian
- Department of Internal Medicine, Hôpital Saint-Antoine, AP-HP, Paris, France
| | | | - Antoine Gaillet
- Intensive-care unit, Hôpital Bichat-Claude-Bernard, AP-HP, Paris, France
| | - Laurent Perard
- Department of Internal Medicine, Centre Hospitalier Saint Joseph Saint Luc, Lyon, France
| | - Maxime Samson
- Department of Clinical Immunology and Internal Medicine, CHU de Dijon, Dijon, France
| | - Romain Sonneville
- Intensive-care unit, Hôpital Bichat-Claude-Bernard, AP-HP, Paris, France
| | - Jean-Benoît Arlet
- Department of Internal Medicine, Hôpital européen Georges Pompidou, AP-HP, Paris, France
| | | | - Jean-Emmanuel Kahn
- Department of Internal Medicine, Hôpital Ambroise Paré, AP-HP, Boulogne, France
| | | | - Éric Hachulla
- Department of Internal Medicine and Clinical Immunology, Hôpital Claude Huriez, Lille, France
| | - Aurélie Hummel
- Department of Nephrology, Hôpital Necker, AP-HP, Paris, France
| | - Thomas Pires
- Department of Internal Medicine, CHU Bordeaux, Bordeaux, France
| | | | - Cécile-Audrey Durel
- Department of Internal Medicine, Hôpital Edouard Herriot, CHU Lyon, Lyon, France
| | - Wendy Jourde
- Department of Internal Medicine, CHU Bordeaux, Bordeaux, France
| | - Xavier Puechal
- Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
| | - Jean-Christophe Lega
- Department of Internal Medicine, Hôpital Edouard Herriot, CHU Lyon, Lyon, France
| | | | | | - Elisabeth Diot
- Department of Internal Medicine and Clinical Immunology, CHRU Tours, Tours, France
| | - Loïc Guillevin
- Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
| | - Benjamin Terrier
- Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France.
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Malek Mahdavi A, Khabbazi A, Hajialilo M. Long-term outcome and predictors of remission in Behçet's disease in daily practice. Mod Rheumatol 2021; 31:1148-1157. [PMID: 33560927 DOI: 10.1080/14397595.2021.1886623] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
BACKGROUND Many factors can influence the response to treatment and prognosis of Behçet's disease (BD). Identifying the predictors of response to treatment can improve the quality and decrease the cost of medical care. This analytical study was performed to identify factors affecting the remission and outcome in BD patients with long-term follow-up. METHODS A total of 245 BD patients aged over 16 years were followed for at least 12 months and visited at least three times a year were included. The outcome was assessed by the number of patients who were in sustained and long-term remission, had lost the primary criteria of BD for at least 12 months, were asymptomatic, and developed the sequela of disease or deceased. Sustained remission was defined as being in remission for at least six months. Long-term remission was defined as remission for ≥ 5 years. RESULTS Mean age and mean duration of follow-up were 35.1 ± 10.7 years and 92.3 months, respectively. At the end of follow-up, 63.2% of the patients lost the criteria of BD, 51.8% of the cases were in sustained remission, and 36.2% of them were asymptomatic. Predictors of sustained remission were adherence to therapy and treatment for more than six years. Having genital ulcers and treatment with methotrexate were associated with non-remission. Predictor of long-term remission was remission induction in the first two years of the treatment. Treatment with methotrexate was associated with non-remission. Poor outcome was observed in 31.8% of patients. Male sex, obesity, and having severe disease were the risk factors of poor outcome. CONCLUSION Achieving remission in BD is not inaccessible. Treatment with conventional and biologic disease-modifying antirheumatic drugs may cause sustained and long-term remission. Adherence to treatment, remission induction during the two years after the diagnosis and treatment for at least six years have significant role.
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Affiliation(s)
- Aida Malek Mahdavi
- Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
| | - Alireza Khabbazi
- Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
| | - Mehrzad Hajialilo
- Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
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Yildiz M, Haslak F, Adrovic A, Sahin S, Koker O, Barut K, Kasapcopur O. Pediatric Behçet's Disease. Front Med (Lausanne) 2021; 8:627192. [PMID: 33614684 PMCID: PMC7886701 DOI: 10.3389/fmed.2021.627192] [Citation(s) in RCA: 27] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2020] [Accepted: 01/06/2021] [Indexed: 12/14/2022] Open
Abstract
Behçet's Disease (BD) is a systemic vasculitis firstly described as a disorder causing aphthous lesion in oral and genital mucosae and uveitis. The disease has an extremely unique distribution characterized by the highest incidence in communities living along the historical Silk road. Although our understanding of the etiopathogenesis of BD has expanded over time, there are still lots of unidentified points in the underlying mechanisms of the disease. The accepted opinion in the light of the current knowledge is that various identified and/or unidentified infectious and/or environmental triggers can take a role as a trigger in individuals with genetic susceptibility. Although the disease usually develops in young adulthood, it is reported that about 15-20% of all Behçet's patients develop in childhood. Pediatric BD differs from adult BD not only with the age of onset but also in the frequency and distribution of clinical findings, disease severity and outcome. While gastrointestinal system involvement, neurological findings, arthralgia and positive family history are more common in children, genital lesions and vascular lesions are more common in adult patients. In addition, a better disease outcome with lower severity score and activity index has been reported in children. The diagnosis of the disease is made according to clinical findings. It can be challenging to diagnose the disease due to the absence of a specific diagnostic test, and the long time interval from the first finding of the disease to the full-blown disease phenotype in pediatric cases. Therefore, many classification criteria have been proposed so far. The widely accepted ones are proposed by the International Study Group. The new sets of classification criteria which is the only one for pediatric BD were also developed for pediatric cases by the PEDBD group. The primary goal for the treatment is preventing the organ damages by suppressing the ongoing inflammation and forestalling the disease flares. The treatment of the BD can be onerous due to its multisystemic nature and a multidisciplinary approach is essential for the management of the patients. In this review article, the definition, clinical findings, epidemiology, etiopathogenesis, and treatment will be discussed.
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Affiliation(s)
| | | | | | | | | | | | - Ozgur Kasapcopur
- Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey
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Gong L, Zhang YL, Sun LX, Chen GR, Wu D. Mucosal healing in intestinal Behçet's disease: A systematic review and meta-analysis. J Dig Dis 2021; 22:83-90. [PMID: 33314672 DOI: 10.1111/1751-2980.12965] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/20/2020] [Revised: 12/02/2020] [Accepted: 12/08/2020] [Indexed: 12/11/2022]
Abstract
OBJECTIVES Behçet's disease (BD) is a chronic inflammatory disease characterized by recurrent oral aphthous ulcers, intestinal lesions, genital ulcers, uveitis, and skin lesions. Evidence regarding mucosal healing for the prognosis of intestinal BD is scarce. The aim of this systematic review and meta-analysis was to determine the association between mucosal healing and long-term outcomes of patients with intestinal BD. METHODS Relevant studies were identified in a comprehensive search of PubMed, Cochrane Library, and EMBASE databases. Studies reporting long-term outcomes of mucosal healing in patients with intestinal BD were included. Pooled risk ratio (RR) and 95% confidence interval (CI) for disease recurrence and surgery were calculated using the Mantel-Haenszel random-effects models. Heterogeneity among the eligible studies was evaluated using the Q test and I2 statistics. RESULTS Of the 4785 studies initially identified, 8 were finally included. The pooled RR for the association between mucosal healing and disease recurrence was 0.41 (95% CI 0.30-0.57, P < 0.001). For the association between mucosal healing and the risk of surgery, the pooled RR was 0.33 (95% CI 0.17-0.63, P < 0.001). Confounding factors were adjusted in one study, whereas other studies only reported a crude association between mucosal healing and long-term outcomes without adjustment. CONCLUSIONS Mucosal healing is associated with a decreased risk of recurrence and surgery in intestinal BD. However, more studies are required given a small number of currently eligible studies and insufficient adjustment for confounding factors.
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Affiliation(s)
- Liang Gong
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Yue Lun Zhang
- Medical Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Lu Xi Sun
- Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Guo Rong Chen
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Dong Wu
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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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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Halabi H, AlDabbagh A, Alamoudi A. Gastrointestinal Manifestations of Rheumatic Diseases. SKILLS IN RHEUMATOLOGY 2021:475-499. [DOI: 10.1007/978-981-15-8323-0_23] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
Abstract
AbstractSLE may involve any part of the gastrointestinal (GI) tract as well as the liver.
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Behcet's Disease with Upper GI Bleeding. Case Rep Emerg Med 2020; 2020:2983209. [PMID: 32257459 PMCID: PMC7103031 DOI: 10.1155/2020/2983209] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2019] [Accepted: 02/21/2020] [Indexed: 12/04/2022] Open
Abstract
Introduction. Behcet's disease is a multisystem disease. In sub-Saharan Africa, the prevalence of this disease is not known, with only one case report from Ethiopia. Case Presentation. We describe a case of a 29-year-old Ethiopian male who presented to the emergency room of Tikur Anbessa specialized hospital with 4 days history of back pain, recurrent history of oral and genital ulcers, right eye blindness, chronic cerebral vein thrombosis, gastrointestinal bleeding, aortic aneurysm with dissection, and positive pathergy test. He is retrospectively diagnosed with Behcet's disease according to both the International Criteria for Behcet's Disease (ICBD) and the International Study Group (ISG) consensus. Conclusion. Even if Behcet's disease is rare in sub-Saharan Africa, it is important to know the clinical presentation for timely diagnosis and urgent management.
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An Overview of Conventional and Recent Treatment Options for Behcet’s Disease. CURRENT TREATMENT OPTIONS IN RHEUMATOLOGY 2020. [DOI: 10.1007/s40674-020-00143-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
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Yıldız M, Köker O, Adrovic A, Şahin S, Barut K, Kasapçopur Ö. Pediatric Behçet's disease - clinical aspects and current concepts. Eur J Rheumatol 2020; 7:S38-S47. [PMID: 31556871 PMCID: PMC7004268 DOI: 10.5152/eurjrheum.2019.19121] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2019] [Accepted: 07/30/2019] [Indexed: 12/14/2022] Open
Abstract
Behçet's Disease was first described by a Turkish dermatologist, Hulusi Behçet, in 1937 as a triple symptom complex; aphthous stomatitis, genital ulcers, and uveitis. Today, in light of current trials and experiments, we know that the disease may have a wider involvement with a multisystemic recurrent course, causing significant morbidity and mortality. However, there are still unanswered questions, particularly about Pediatric Behçet's Disease. Although several immunological and genetic associations have been demonstrated, the real etiologic mechanism of the disease is unclear. The diagnosis is difficult due to its rarity in childhood, the lack of validation of the diagnostic criteria obtained from adult studies, and the inadequacy of large case-controlled studies. Also, the management is challenging and controversial due to the various geographic distribution of clinical spectrum. New therapeutic options under development in light of pathogenetic hypothesis seem to be promising.
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Affiliation(s)
- Mehmet Yıldız
- Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa School of Medicine, İstanbul, Turkey
| | - Oya Köker
- Department of Pediatric Rheumatology, İstanbul University, İstanbul School of Medicine, İstanbul, Turkey
| | - Amra Adrovic
- Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa School of Medicine, İstanbul, Turkey
| | - Sezgin Şahin
- Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa School of Medicine, İstanbul, Turkey
| | - Kenan Barut
- Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa School of Medicine, İstanbul, Turkey
| | - Özgür Kasapçopur
- Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa School of Medicine, İstanbul, Turkey
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Shen Y, Ma HF, Yang YL, Guan JL. Ulcerative intestinal tuberculosis case as a complication of treatment by infliximab for intestinal Behçet's disease: A case report. Medicine (Baltimore) 2019; 98:e17652. [PMID: 31651888 PMCID: PMC6824802 DOI: 10.1097/md.0000000000017652] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
RATIONALE Intestinal Behçet's disease (BD) is characterized by intestinal ulcerations and gastrointestinal symptoms. Ulcerative intestinal tuberculosis (TB) is usually with dyspepsia, abdominal pain, vomiting, and weight loss. The 2 diseases exhibit similar clinical manifestations, but the most critical aspects of their clinical courses and required treatments are not at all similar. PATIENT CONCERNS We present a case in which a patient with intestinal Behçet's disease developed a de novo ulcerative intestinal TB infection after the start of anti-tumor necrosis factor-α treatment. This was despite histopathologic examination without caseous necrosis granuloma and negative for acid-fast staining and latent TB screen. DIAGNOSES Intestinal Behçet's disease and intestinal TB. INTERVENTIONS The patient was treated with quadruple antituberculous chemotherapy, comprising rifapentine, isoniazid, ethambutol, and pyrazinamide. OUTCOMES At follow-up about 3 months, the therapy of oral antituberculous drugs and thalidomide was continued and the patient's condition had stabilized. LESSONS This case illustrates the importance of closely monitoring patients who are on infliximab for possible onset of TB, even without abdominal symptoms, and with negative screening results for latent TB.
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Affiliation(s)
- Yan Shen
- Rheumatology and Immunology Department
| | | | - Yan-li Yang
- Medical Imaging Department, Huadong Hospital, Fudan University, Shanghai 200040, PR China
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Tang SJ, Wu R. Ilececum: A Comprehensive Review. Can J Gastroenterol Hepatol 2019; 2019:1451835. [PMID: 30854348 PMCID: PMC6378086 DOI: 10.1155/2019/1451835] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/21/2018] [Revised: 11/18/2018] [Accepted: 12/09/2018] [Indexed: 02/07/2023] Open
Abstract
For gastrointestinal endoscopists, the ileocecum is the finishing line during colonoscopy and it is identified by three endoscopic landmarks: terminal ileum, ileocecal valve, and the appendiceal orifice. Although ileal intubation is recommended during routine screening colonoscopy, it is not required in most cases of screening colonoscopy. Ileal intubation is indicated in certain circumstances such as suspected inflammatory bowel disease and GI bleeding. There is much pathology that can be observed within the ileocecum. Careful and systematic examination should be stressed during GI endoscopic training and practice. In this review, the authors demonstrate its anatomy, endoscopic findings, and pathologies.
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Affiliation(s)
- Shou-jiang Tang
- Division of Digestive Diseases, Department of Medicine, University of Mississippi Medical Center, USA
| | - Ruonan Wu
- Division of Digestive Diseases, Department of Medicine, University of Mississippi Medical Center, USA
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Suzuki T, Horita N, Takeuchi M, Ishido T, Mizuki Y, Mizuki R, Kawagoe T, Shibuya E, Yuta K, Yamane T, Hayashi T, Meguro A, Ishido M, Minegishi K, Yoshimi R, Kirino Y, Kato S, Arimoto J, Fukumoto T, Ishigatsubo Y, Kurosawa M, Takeno M, Kaneko T, Mizuki N. Clinical features of early-stage possible Behçet’s disease patients with a variant-type major organ involvement in Japan. Mod Rheumatol 2018; 29:640-646. [DOI: 10.1080/14397595.2018.1494501] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Affiliation(s)
- Takeharu Suzuki
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Nobuyuki Horita
- Department of Pulmonology, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Masaki Takeuchi
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Takehito Ishido
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Yuki Mizuki
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Ryuta Mizuki
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Tatsukata Kawagoe
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Etsuko Shibuya
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Kentaro Yuta
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Takahiro Yamane
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Takahiko Hayashi
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Akira Meguro
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Mizuho Ishido
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Kaoru Minegishi
- Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Ryusuke Yoshimi
- Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Yohei Kirino
- Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Shingo Kato
- Department of Gastroenterology and Hepatology, Yokohama City University School of Medicine, Yokohama, Japan
| | - Jun Arimoto
- Department of Gastroenterology and Hepatology, Yokohama City University School of Medicine, Yokohama, Japan
| | - Takeshi Fukumoto
- Gene Expression and Regulation Program, The Wistar Institute, Philadelphia, PA, USA
| | | | - Michiko Kurosawa
- Department of Epidemiology and Environmental Health, Juntendo University Faculty of Medicine, Tokyo, Japan
| | - Mitsuhiro Takeno
- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
| | - Takeshi Kaneko
- Department of Pulmonology, Yokohama City University Graduate School of Medicine, Yokohama, Japan
| | - Nobuhisa Mizuki
- Department of Ophthalmology and Visual Science, Yokohama City University Graduate School of Medicine, Yokohama, Japan
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Park JW, Park Y, Park SJ, Kim TI, Kim WH, Cheon JH. Development of a Novel Endoscopic Scoring System to Predict Relapse after Surgery in Intestinal Behçet's Disease. Gut Liver 2018; 12:674-681. [PMID: 29938457 PMCID: PMC6254620 DOI: 10.5009/gnl17547] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/04/2017] [Revised: 04/01/2018] [Accepted: 04/27/2018] [Indexed: 01/28/2023] Open
Abstract
Background/Aims The cumulative surgery rate and postoperative relapse of intestinal Behcet’s disease (BD) have been reported to be high. This study aimed to establish a scoring system based on follow-up endoscopic findings that can predict intestinal BD recurrence after surgery. Methods Fifty-four patients with intestinal BD who underwent surgery due to bowel complications and underwent follow-up colonoscopy were retrospectively investigated. Their clinical data, including colonoscopic findings, were retrieved. Classification and regression tree analysis was used to develop an appropriate endoscopic classification model that can explain the postsurgical recurrence of intestinal BD most accurately based on the following classification: e0, no lesions; e1, solitary ulcer <20 mm in size; e2, solitary ulcer ≥ 20 mm in size; and e3, multiple ulcers regardless of size. Results Clinical relapse occurred in 37 patients (68.5%). Among 38 patients with colonoscopic recurrence, only 29 patients had clinically relapsed. Multivariate analysis identified higher disease activity index for intestinal BD at colonoscopy (hazard ratio [HR], 1.013; 95% confidence interval [CI], 1.005 to 1.021; p=0.002) and colonoscopic recurrence (HR, 2.829; 95% CI, 1.223 to 6.545; p=0.015) as independent risk factors for clinical relapse of intestinal BD. Endoscopic findings were classified into four groups, and multivariate analysis showed that the endoscopic score was an independent risk factor of clinical relapse (p=0.012). The risk of clinical relapse was higher in the e3 group compared to the e0 group (HR, 6.284; 95% CI, 2.036 to 19.391; p=0.001). Conclusions This new endoscopic scoring system could predict clinical relapse in patients after surgical resection of intestinal BD.
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Affiliation(s)
- Jung Won Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Yehyun Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Soo Jung Park
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Tae Il Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Won Ho Kim
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Hee Cheon
- Department of Internal Medicine and Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Korea
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Abstract
Behçet disease (BD) is a variable vessel vasculitis that can involve several organs and systems. Gastrointestinal (GI) involvement has an acute exacerbating course with ulcers, most commonly in the ileocolonic area. These ulcers can be large and deep, causing perforation and massive bleeding. This article highlights the current knowledge on the epidemiology, clinical findings, diagnosis, and management of GI involvement of BD, with emphasis on recent findings.
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Affiliation(s)
- Ibrahim Hatemi
- Division of Gastroenterology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Koca Mustafa Pasa Mahallesi, Cerrahpaşa Caddesi No:53, 34096 Fatih/Istanbul, Turkey
| | - Gulen Hatemi
- Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Koca Mustafa Paşa Mahallesi, Cerrahpasa Caddesi No:53, 34096 Fatih/Istanbul, Turkey
| | - Aykut Ferhat Çelik
- Division of Gastroenterology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Koca Mustafa Pasa Mahallesi, Cerrahpaşa Caddesi No:53, 34096 Fatih/Istanbul, Turkey.
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High Incidence of Gastrointestinal Ulceration and Cytogenetic Aberration of Trisomy 8 as Typical Features of Behçet's Disease Associated with Myelodysplastic Syndrome: A Series of 16 Consecutive Chinese Patients from the Shanghai Behçet's Disease Database and Comparison with the Literature. BIOMED RESEARCH INTERNATIONAL 2018; 2018:8535091. [PMID: 29854799 PMCID: PMC5941803 DOI: 10.1155/2018/8535091] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/11/2018] [Revised: 02/24/2018] [Accepted: 03/04/2018] [Indexed: 12/16/2022]
Abstract
This study aimed to investigate the characteristics of Chinese patients with Behçet disease (BD) and myelodysplastic syndrome (MDS) and explore the role played by trisomy 8. This was a retrospective study of patients with BD and MDS from the Shanghai Behçet's disease database who were diagnosed between October 2012 and July 2017. There were 805 patients with BD and 16 also had MDS. Trisomy 8 was examined in patients with BD-MDS and some patients with gastrointestinal (GI) BD. Patients with BD and MDS (16/805; 2%) were more likely to be female and older; display fever and intestinal lesions; have lower leukocyte count, hemoglobin, platelet count; and show higher C-reactive protein and erythrocyte sedimentation rate (ESR) than patients with BD without MDS (all P < 0.05). Trisomy 8 was common (81.3%) in patients with BD-MDS. Ulcers in the ileocecal region were more frequently seen in intestinal patients with BD-MDS than in BD without MDS (90.0% versus 48.9%; P = 0.032). GI ulceration is common in patients with BD-MDS. Cytogenetic aberrations, especially trisomy 8, may play a role in the pathogenesis of intestinal involvement in patients with BD-MDS.
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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: 27] [Impact Index Per Article: 3.9] [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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