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Attia DHS, Alkaffas M, Eissa M, Rashed L, Khattab RAM, Elzanaty R, Khattab RA, Samy LA. Protein tyrosine phosphatase non-receptor type 2 (PTPN2) gene polymorphisms (rs2542151, rs7234029) in Egyptian Behçet's disease patients: a preliminary report. Clin Rheumatol 2024; 43:3439-3448. [PMID: 39320556 PMCID: PMC11489223 DOI: 10.1007/s10067-024-07128-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/20/2024] [Revised: 07/13/2024] [Accepted: 08/29/2024] [Indexed: 09/26/2024]
Abstract
Single nucleotide polymorphisms (SNPs) of the protein tyrosine phosphatase non-receptor type 2 (PTPN2) gene have been documented to be linked with several autoimmune disorders including Behçet's disease (BD). PTPN2 SNPs rs2542151 and rs7234029 have been assessed using real-time PCR in 96 BD patients and 50 controls matched by age and gender. Patients were categorized into groups according to the disease phenotypes and severity. A total of 94.8% of patients were males. The patients' mean age at onset was 26.1 ± 8 years. The median (IQR) disease duration was 8.5(4-13) years. No difference was observed between the patients and controls concerning the frequency of the two SNPs' different genotypes, models, and alleles. Moreover, neither disease phenotypes nor severity were associated with rs2542151 or rs7234029 SNPs. PTPN2 rs2542151 and rs7234029 SNPs do not seem to have associations with BD occurrence, phenotypes, or severity in the Egyptian patients. Key Points • PTPN2 rs2542151 and rs7234029 SNPs do not seem to have associations with BD occurrence, phenotypes, or severity in the Egyptian patients. • Further studies involving a larger sample size with variable clinical diversity are recommended to verify the results.
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Affiliation(s)
- Doaa H S Attia
- Rheumatology and Rehabilitation Department, Faculty of Medicine, Cairo University Hospitals, Saray El Manial Street, El Manial, Cairo, 11956, Egypt.
| | - Marwa Alkaffas
- Medical Biochemistry and Molecular Biology Department, Faculty of Medicine, Cairo University, Saray El Manial Street, El Manial, Cairo, 11956, Egypt
| | - Mervat Eissa
- Rheumatology and Rehabilitation Department, Faculty of Medicine, Cairo University Hospitals, Saray El Manial Street, El Manial, Cairo, 11956, Egypt
| | - Laila Rashed
- Medical Biochemistry and Molecular Biology Department, Faculty of Medicine, Cairo University, Saray El Manial Street, El Manial, Cairo, 11956, Egypt
| | - Rasha A M Khattab
- Clinical and Chemical Pathology Department, Faculty of Medicine, Beni-Suef University, Beni-Suef, Egypt
| | - Radwa Elzanaty
- Ophthalmology Department, Faculty of Medicine, Cairo University, Saray El Manial Street, El Manial, Cairo, 11956, Egypt
| | - Rabab A Khattab
- Ophthalmology Department, Helwan University Student's Hospital, Cairo, Egypt
| | - Lamees A Samy
- Clinical and Chemical Pathology Department, Faculty of Medicine, Cairo University Hospitals, Saray El Manial Street, El Manial, Cairo, 11956, Egypt
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Peters KS, Schuman SG, Sengupta S, Maheswaranathan M, Gospe SM. Papilledema and Combined Central Retinal Artery and Central Retinal Vein Occlusion as a Manifestation of Behçet Disease. J Neuroophthalmol 2024; 44:e371-e373. [PMID: 37418630 DOI: 10.1097/wno.0000000000001941] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/09/2023]
Affiliation(s)
- Katherine S Peters
- Departments of Ophthalmology (KSP, SGS, SMG) and Neurology (SS); and Division of Rheumatology (MM), Duke University Medical Center, Durham, North Carolina
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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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Dantas MAS, Graneiro AL, Cavalcante R, Felipez LM. A Challenging Case of Oral Ulcers and Gastrointestinal Bleeding: Crohn's or Behçet's Disease. Case Rep Pediatr 2023; 2023:4705638. [PMID: 37830113 PMCID: PMC10567328 DOI: 10.1155/2023/4705638] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2022] [Revised: 09/05/2023] [Accepted: 09/26/2023] [Indexed: 10/14/2023] Open
Abstract
Introduction. Differentiating Crohn's disease (CD) and Behçet's disease (BD) with gastrointestinal (GI) manifestations can be clinically challenging, as current diagnostic criteria are not clear between both conditions and multiple symptoms could overlap. Case Presentation. The patient is an 8-year-old boy of Brazilian descent, who initially presented with a 1-year history of painful oral ulcers. Before presenting to the hospital, he had been treated for periodic fever, aphthous stomatitis, pharyngitis, and adenitis and placed on steroids, with relapsing symptoms on attempts to wean the doses. The initial workup was largely unremarkable. Buccal biopsies showed no granulomas, and the ophthalmologic exam was normal. Infectious and rheumatological tests were negative. Prometheus IBD sgi testing showed a pattern consistent with CD; however, the patient had multiple negative endoscopies, colonoscopies, and capsule endoscopies. He developed intermittent bloody stools and severe malnutrition and did not respond to infliximab, colchicine, or methotrexate. After a large GI bleed, a 4th colonoscopy was performed, which showed large round ulcers in the terminal ileum, and no granulomas. He was started on ustekinumab with clinical improvement. One month later, he developed bilateral hip effusion and meningismus, being diagnosed with aseptic meningitis secondary to COVID-19. He improved, but in one month developed worsening symptoms, and MRV showed extensive venous sinus thrombosis. The patient was started on enoxaparin, methylprednisolone, and colchicine, with resolution of the thrombus on a 3-month follow-up. The patient's overall symptoms remained controlled with clinical and biochemical remission on monthly ustekinumab. Discussion and Conclusion. Our patient had a challenging clinical course, with nonspecific systemic and intestinal manifestations which proved difficult to differentiate between BD and CD. Given endoscopic findings and the worsening of an auto-inflammatory reaction in the central nervous system after COVID-19 in a patient with controlled GI symptoms, the most likely diagnosis is BD.
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Affiliation(s)
- Marina A. S. Dantas
- Department of Pediatric Hospital Medicine, Medical University of South Carolina, Charleston, SC, USA
| | - Ana Luiza Graneiro
- Department of Allergy and Immunology, Nicklaus Children's Hospital, Miami, FL, USA
| | - Rodrigo Cavalcante
- Department of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
| | - Lina Maria Felipez
- Department of Gastroenterology, Nicklaus Children's Hospital, Miami, FL, USA
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Sun FSK, Chiu NSY, Chung HY. Potential gastrointestinal Behcet's disease flare after treatment with anti-interleukin 17a therapy. BMC Rheumatol 2023; 7:25. [PMID: 37553722 PMCID: PMC10408212 DOI: 10.1186/s41927-023-00344-9] [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: 11/02/2022] [Accepted: 07/07/2023] [Indexed: 08/10/2023] Open
Abstract
BACKGROUND Behcet's disease (BD) is a systemic disease characterized by recurrent oral and genital ulcers. The underlying disease pathway likely involves interleukin (IL)-17 A, a proinflammatory cytokine that is implicated in Behcet's uveitis. Secukinumab is an anti-IL-17 A drug that may have an emerging role in the treatment of refractory BD. This is the first known case report of gastrointestinal BD flare up after anti-IL-17 A therapy. CASE PRESENTATION We presented a case of BD with cutaneous and articular features being treated with secukinumab. After the third dose of loading secukinumab, the patient developed acute lower abdominal pain required hospital admission. Urgent computer tomography (CT) abdomen showed fatty stranding of caecum. Colonoscopy with caecal showed increased number of inflammatory cells in lamina propria. Secukinumab was stopped and patient was started on medium dose steroid. His abdominal symptoms resolved after treatment. CONCLUSIONS This case report illustrates a case of gastrointestinal (GI) BD presenting as acute inflammatory colitis after the use of secukinumab. Therefore, anti-IL-17 A agents should be used cautiously in patients with GI BD, and preferably guided by a phenotype-tailored approach.
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Affiliation(s)
- Frances Sze Kei Sun
- Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Hong Kong, China
| | - Nicole Sau Yan Chiu
- Division of Rheumatology, Chiron Medical Group, 26/F, 9 Queen's Road, Central, Hong Kong, China
| | - Ho Yin Chung
- Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Hong Kong, China.
- Division of Rheumatology, Chiron Medical Group, 26/F, 9 Queen's Road, Central, Hong Kong, China.
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Adiga A, Hussain N, Bateman J. Behcet's intestinal disease confirmation: telemedicine images demonstrating stomal ulceration facilitate prompt assessment. Clin Rheumatol 2023; 42:1215-1216. [PMID: 36564555 PMCID: PMC9788656 DOI: 10.1007/s10067-022-06489-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2022] [Revised: 12/14/2022] [Accepted: 12/15/2022] [Indexed: 12/24/2022]
Affiliation(s)
- Aditya Adiga
- grid.6572.60000 0004 1936 7486Birmingham Medical School, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
| | - Naveed Hussain
- grid.6572.60000 0004 1936 7486Birmingham Medical School, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
| | - James Bateman
- grid.6572.60000 0004 1936 7486Birmingham Medical School, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
- grid.6572.60000 0004 1936 7486Institute of Clinical Sciences, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
- grid.439674.b0000 0000 9830 7596Department of Rheumatology, Royal Wolverhampton Hospitals NHS Trust, Wolverhampton, UK
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Abstract
OBJECTIVE Behçet's disease is a rare and incurable condition where the body's immune system attacks healthy tissue. Behçet's can cause blood clots and ulcerations to form in every organ and system in the body, including deep and painful genital ulceration. The psychological impact of the disease on intimacy and relationships is unexplored. This study aimed to explore how the disease impacts on female patients' intimate partner relationships. METHODS Participants were seven female, UK patients with Behçet's disease who were in committed relationships. In depth, semi-structured interviews lasting approximately 80 minutes were conducted via video conferencing about participants' sexual functioning and intimate relationships. Data was analysed using Interpretative Phenomenological Analysis. RESULTS Patients' sexual relationships and intimacy were impacted by issues due to lack of knowledge of symptoms (misdiagnosis of herpes), issues due to symptoms (genital manifestations, painful sex, exhaustion), difficulties communicating with medical professionals, medication, partner support, and support from fellow Behçet's patients. DISCUSSION Female Behçet's patients are at risk of developing psychological problems with intimacy due to symptoms, lack of knowledge of symptoms, and negative impacts of medication. Intimacy needs to be discussed in medical consultations so these issues can be addressed before a lack of intimacy negatively impacts relationships.
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Affiliation(s)
- Fay Sweeting
- Department of Psychology, Bournemouth University, Poole, UK
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Vuyyuru SK, Kedia S, Sahu P, Ahuja V. Immune-mediated inflammatory diseases of the gastrointestinal tract: Beyond Crohn's disease and ulcerative colitis. JGH Open 2022; 6:100-111. [PMID: 35155819 PMCID: PMC8829105 DOI: 10.1002/jgh3.12706] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/24/2021] [Revised: 12/26/2021] [Accepted: 12/29/2021] [Indexed: 12/17/2022]
Abstract
Immune-mediated inflammatory diseases (IMIDs) are a diverse group of complex inflammatory diseases that result from dysregulated immune pathways and can involve any system of the human body. Inflammatory bowel disease (IBD) is one such disease involving the gastrointestinal (GI) system. With high prevalence in the West and increasing incidence in newly industrialized countries, IBD poses a significant burden on health care. IMIDs of the GI system other than IBD can have similar clinical features, causing diagnostic and therapeutic challenges. Although these disorders share a common pathophysiology, the defects can occur anywhere in the complex network of cytokines, inflammatory mediators, and innate and adaptive systems, leading to unregulated inflammation. Precise knowledge about them will help determine the possible targeted therapy. Thus, it is essential to distinguish these disorders from IBD. This review describes various IMIDs of the GI tract that mimic IBD.
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Affiliation(s)
- Sudheer K Vuyyuru
- Department of GastroenterologyAll India Institute of Medical SciencesNew DelhiIndia
| | - Saurabh Kedia
- Department of GastroenterologyAll India Institute of Medical SciencesNew DelhiIndia
| | - Pabitra Sahu
- Department of GastroenterologyAll India Institute of Medical SciencesNew DelhiIndia
| | - Vineet Ahuja
- Department of GastroenterologyAll India Institute of Medical SciencesNew DelhiIndia
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Kidambi TD, Liang Y, Motarjem P, Mirza R, Han A, Hein M, Singh D, Lin JL, Banciu-Odell C, Toroussian R, Lew MW, Manoukian S. Episodic, severe abdominal pain due to isolated jejunal Crohn's disease. Clin J Gastroenterol 2021; 14:1084-1089. [PMID: 33904109 DOI: 10.1007/s12328-021-01421-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/18/2021] [Accepted: 04/16/2021] [Indexed: 10/21/2022]
Abstract
Small bowel Crohn's disease can present with episodic, relapsing, and remitting symptoms and delays in the diagnosis are common. We present a case of a young woman with three years of intermittent abdominal pain and nausea with negative previous evaluations. On presentation, inflammatory markers were elevated, and repeat imaging showed jejunal inflammation, with histopathological examination showing non-caseating granulomas of the small bowel consistent with Crohn's disease. This case highlights the importance of gastroenterologist recognizing the alarm signs in a patient with unexplained symptoms and adds to the literature on the clinical presentation of a rare diagnosis of isolated jejunal Crohn's disease.
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Affiliation(s)
- Trilokesh D Kidambi
- Division of Gastroenterology, Department of Medicine, City of Hope, 1500 E Duarte Road, Duarte, CA, 91010, USA.
| | - Yu Liang
- Department of Pathology, City of Hope, Duarte, CA, USA
| | | | - Rizvan Mirza
- Department of Radiology, City of Hope, Duarte, CA, USA
| | - Alice Han
- Division of Gastroenterology, Department of Medicine, City of Hope, 1500 E Duarte Road, Duarte, CA, 91010, USA
| | - Marjorie Hein
- Division of Gastroenterology, Department of Medicine, City of Hope, 1500 E Duarte Road, Duarte, CA, 91010, USA
| | - Dupinder Singh
- Division of Gastroenterology, Department of Medicine, City of Hope, 1500 E Duarte Road, Duarte, CA, 91010, USA
| | - James L Lin
- Division of Gastroenterology, Department of Medicine, City of Hope, 1500 E Duarte Road, Duarte, CA, 91010, USA
| | - Cornelia Banciu-Odell
- Division of Hospital Medicine, Department of Medicine, City of Hope, Duarte, CA, USA
| | | | - Michael W Lew
- Department of Anesthesiology, City of Hope, Duarte, CA, USA
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Francois J, Moulinet T, Neiter E, Ehrardt A, Conart JB, Angioi-Duprez K. [Behçet's disease : Description and analysis of a French single-center retrospective study of 51 patients]. J Fr Ophtalmol 2021; 44:711-717. [PMID: 33741217 DOI: 10.1016/j.jfo.2020.04.064] [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: 12/09/2019] [Revised: 04/23/2020] [Accepted: 04/24/2020] [Indexed: 11/16/2022]
Abstract
PURPOSE To evaluate if the presence of uveitis in Behçet's disease (BD) is associated with a particular clinical phenotype and to analyze the prognostic impact of a missed diagnosis of BD at the time the uveitis is diagnosed. MATERIEL AND METHODS Ophthalmologic and systemic clinical features of 51 patients with BD were recorded retrospectively. We compared the clinical phenotype of patients with ocular manifestations with those without ocular manifestations. The patients were divided into two groups depending on the progression of their visual acuity: "decreased visual acuity" versus "stable or improved visual acuity." RESULTS In the group of patients with ocular involvement, there was a mean 2.3 systemic manifestations, vs. 3.2 in the group without ocular manifestations (P=0.004). When BD was diagnosed prior to the onset of uveitis, we counted fewer patients in the "decreased visual acuity" group in comparison with the patients who had no prior diagnosis of BD at the onset of the uveitis (91.3% in the "decreased visual acuity" group, P=0.04). The time before initiation of immunosuppressive treatment or a biological agent was shorter for these patients (4.4 vs. 39.3 months, P=0.007). CONCLUSION It appears that different phenotypes exist according to whether or not the BD patient has ocular involvement. Moreover, the visual prognosis is better if the uveitis occurs in patients who have already been diagnosed with BD, due to earlier initiation of immunosuppressive therapy.
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Affiliation(s)
- Julie Francois
- Département d'ophtalmologie, Centre Hospitalier Universitaire de Nancy, Vandœuvre-lès-Nancy, France.
| | - Thomas Moulinet
- Département de médecine interne, Centre Hospitalier Universitaire de Nancy, Vandœuvre-lès-Nancy, France
| | - Estelle Neiter
- Département d'ophtalmologie, Centre Hospitalier Universitaire de Nancy, Vandœuvre-lès-Nancy, France
| | - Alix Ehrardt
- Département d'ophtalmologie, Centre Hospitalier Universitaire de Nancy, Vandœuvre-lès-Nancy, France
| | - Jean-Baptiste Conart
- Département d'ophtalmologie, Centre Hospitalier Universitaire de Nancy, Vandœuvre-lès-Nancy, France
| | - Karine Angioi-Duprez
- Département d'ophtalmologie, Centre Hospitalier Universitaire de Nancy, Vandœuvre-lès-Nancy, France
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Cicero G, Ascenti G, Blandino A, Pallio S, Abate C, D’Angelo T, Mazziotti S. Magnetic Resonance Imaging of the Anal Region: Clinical Applications. J Clin Imaging Sci 2020; 10:76. [PMID: 33274120 PMCID: PMC7708963 DOI: 10.25259/jcis_180_2020] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2020] [Accepted: 11/05/2020] [Indexed: 12/18/2022] Open
Abstract
Over the past years, magnetic resonance imaging (MRI) has become a cornerstone in evaluating anal canal and adjacent tissues due to its safeness, the three-dimensional and comprehensive approach, and the high soft-tissue resolution. Several diseases arising in the anal canal can be assessed through MRI performance, including congenital conditions, benign pathologies, and malignancies. Good knowledge of the normal anatomy and MRI technical protocols is, therefore, mandatory for appropriate anal pathology evaluation. Radiologists and clinicians should be familiar with the different clinical scenarios and the anatomy of the structures involved. This pictorial review presents an overview of the diseases affecting the anal canal and the surrounding structures evaluated with dedicated MRI protocol.
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Affiliation(s)
- Giuseppe Cicero
- Department of Biomedical Sciences and Morphological and Functional Imaging, University of Messina, Messina, Italy
| | - Giorgio Ascenti
- Department of Biomedical Sciences and Morphological and Functional Imaging, University of Messina, Messina, Italy
| | - Alfredo Blandino
- Department of Biomedical Sciences and Morphological and Functional Imaging, University of Messina, Messina, Italy
| | - Socrate Pallio
- Department of Digestive Diseases Endoscopy Unit, University of Messina, Messina, Italy
| | - Claudia Abate
- Department of Biomedical Sciences and Morphological and Functional Imaging, University of Messina, Messina, Italy
| | - Tommaso D’Angelo
- Department of Biomedical Sciences and Morphological and Functional Imaging, University of Messina, Messina, Italy
| | - Silvio Mazziotti
- Department of Biomedical Sciences and Morphological and Functional Imaging, University of Messina, Messina, Italy
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Park Y, Cheon JH. Update on the Treatment of Behcet's Disease of the Small Bowel with Biologic Agents. Curr Gastroenterol Rep 2020; 22:24. [PMID: 32193746 DOI: 10.1007/s11894-020-00759-1] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
PURPOSE OF REVIEW The treatment of intestinal Behcet's disease (BD) is challenging, and one-third of patients require surgery due to failure of conventional therapies. Anti-tumor necrosis factor-α (TNF-α) and other new biologics have been actively investigated for managing intestinal BD. In this article, we review the updated experiences and up-to-date clinical data on anti-TNF-α and other biologics for the management of intestinal BD. RECENT FINDINGS Recent prospective studies have proved the efficacy and safety of infliximab and adalimumab for treating intestinal BD. Recent studies with other biologics such as anti-interleukin (IL)-1 (anakinra and canakinumab) and anti-IL-6 (tocilizumab) have shown promising results in patients with systemic, including intestinal, BD. Both infliximab and adalimumab can be useful in managing patients with intestinal BD, especially severe or refractory cases, with a similar efficacy and safety profile. More evidence for anakinra, canakinumab, tocilizumab, anti-IL-17 (secukinumab), and anti-IL-12/23 (ustekinumab) in intestinal BD is required.
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Affiliation(s)
- Yehyun Park
- Department of Internal Medicine, Institute of Gastroenterology, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, Republic of Korea
| | - Jae Hee Cheon
- Department of Internal Medicine, Institute of Gastroenterology, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, Republic of Korea.
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Zhang L, Wu TT. Inflammatory Bowel Disease. SURGICAL PATHOLOGY OF NON-NEOPLASTIC GASTROINTESTINAL DISEASES 2019:373-424. [DOI: 10.1007/978-3-030-15573-5_15] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Hakim S, Ramireddy S, Amin M, Gebara S, Cappell MS. Preoperative Misdiagnosis of Intestinal Behçet's Syndrome as Crohn's Disease Based on Superficial Colonoscopic Biopsies: Case Report and Systematic Review. Dig Dis Sci 2018; 63:3509-3515. [PMID: 30151660 DOI: 10.1007/s10620-018-5248-4] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2018] [Accepted: 08/09/2018] [Indexed: 01/30/2023]
Affiliation(s)
- Seifeldin Hakim
- Division of Gastroenterology and Hepatology, MOB #602, Department of Medicine, William Beaumont Hospital, 3535 W. Thirteen Mile Rd, Royal Oak, MI, 48073, USA
| | - Srinivas Ramireddy
- Division of Gastroenterology and Hepatology, MOB #602, Department of Medicine, William Beaumont Hospital, 3535 W. Thirteen Mile Rd, Royal Oak, MI, 48073, USA
| | - Mitual Amin
- Department of Pathology, William Beaumont Hospital, Royal Oak, MI, 48073, USA
- Oakland University William Beaumont School of Medicine, Royal Oak, MI, 48073, USA
| | - Souheil Gebara
- Division of Pediatric Gastroenterology, Department of Pediatrics, Beaumont Children's Hospital, Royal Oak, MI, 48073, USA
- Oakland University William Beaumont School of Medicine, Royal Oak, MI, 48073, USA
| | - Mitchell S Cappell
- Division of Gastroenterology and Hepatology, MOB #602, Department of Medicine, William Beaumont Hospital, 3535 W. Thirteen Mile Rd, Royal Oak, MI, 48073, USA.
- Oakland University William Beaumont School of Medicine, Royal Oak, MI, 48073, USA.
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Naidu H, Szeto W, Kissin E, Farraye FA. MAGIC Syndrome in a Patient With Crohn's Disease. Inflamm Bowel Dis 2018; 24:664-665. [PMID: 29462377 DOI: 10.1093/ibd/izx016] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Affiliation(s)
- Harini Naidu
- Section of Gastroenterology, Emory University Hospital, Atlanta, Georgia
| | - Winnie Szeto
- Section of Gastroenterology, St. Elizabeth's Hospital, Cambridge, Massachusetts
| | - Eugene Kissin
- Section of Rheumatology, Boston Medical CenterBoston, Massachusetts
| | - Francis A Farraye
- Section of Gastroenterology, Boston Medical CenterBoston, Massachusetts
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16
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Valenti S, Gallizzi R, De Vivo D, Romano C. Intestinal Behçet and Crohn's disease: two sides of the same coin. Pediatr Rheumatol Online J 2017; 15:33. [PMID: 28427473 PMCID: PMC5397832 DOI: 10.1186/s12969-017-0162-4] [Citation(s) in RCA: 58] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2017] [Accepted: 04/11/2017] [Indexed: 12/11/2022] Open
Abstract
Behçet's disease (BD) and Crohn's disease (CD) are chronic immune-mediated, inflammatory disorders affecting many different systems (joints, skin, eyes, gastrointestinal and biliary tracts). Both disorders have fluctuating courses and when gastrointestinal symptoms are prevalent, differential diagnosis can be difficult. BD involves the gastrointestinal tract in 10-15% of cases with localized lesions in the ileocecal region. The clinical picture is heterogeneous with various clusters of disease expression. CD is a chronic inflammatory disorder, which can affect any part of the intestinal tract, as well as extra-intestinal tissue. Factors that contribute towards the pathogenesis of both disease include the host's genetic profile, and immune system, and environmental factors such as the gut microbiota. The aim of this manuscript is to provide a narrative review of clinical features of BD and CD, highlighting the importance of differential diagnosis and therapeutic approach, especially in the presence of gastrointestinal involvement. A comprehensive search of published literature using the Pubmed ( http://www.ncbi.nlm.nih.gov/pubmed/ ) database was carried out to identify all articles published in English from 1999 to October 2016, using 4 key terms: "Behçet Disease", "Intestinal Behçet's Disease", "Crohn's Disease" and" Inflammatory Bowel Disease".
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Affiliation(s)
- Simona Valenti
- Unit of Pediatrics, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of Messina, Viale Consolare Valeria, 98124 Messina, Italy
| | - Romina Gallizzi
- Unit of Pediatrics, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of Messina, Viale Consolare Valeria, 98124 Messina, Italy
| | - Dominique De Vivo
- Unit of Pediatrics, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of Messina, Viale Consolare Valeria, 98124 Messina, Italy
| | - Claudio Romano
- Unit of Pediatrics, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of Messina, Viale Consolare Valeria, 98124 Messina, Italy
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Gallizzi R, De Vivo D, Valenti S, Pidone C, Romeo C, Caruso R, Romano C. Intestinal and neurological involvement in Behcet disease: a clinical case. Ital J Pediatr 2017; 43:33. [PMID: 28388936 PMCID: PMC5383959 DOI: 10.1186/s13052-017-0350-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/18/2017] [Accepted: 03/24/2017] [Indexed: 01/30/2023] Open
Abstract
Background Behcet’s disease (BD) is a chronic immune-mediated, inflammatory disorder which may affect a number of different systems (oral and genital mucosa, eyes, skin, vascular district, joints, gastrointestinal tract and nervous system). Neurological manifestations are present in 5–10%, and gastrointestinal tract involvement in 10–15% of cases. The simultaneous involvement of two systems, neurological and gastrointestinal tract, is very rare and represents the aim of our case report. Case presentation We describe a case of a 12-year-old girl with neurological (endocranial hypertension, papilledema, retinal vasculitis) and gastrointestinal tract (terminal ileum and cecum inflammation) involvement and with a history of recurrent oral aphthosis; therefore, according to both International Criteria for Behcet’s Disease (ICBD) and Paediatric Behcet’s Disease criteria (PEDBD) the diagnosis of BD was confirmed. Conclusions This case report is one of the few described in literature with simultaneous involvement of the two systems, neurological and gastrointestinal tract, in paediatric BD. The diagnosis is really difficult because there is no specific diagnostic test. We think that our clinical case should help clinicians to suspect a BD with an unusual onset.
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Affiliation(s)
- Romina Gallizzi
- Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatrics, University of Messina, Messina, Italy.
| | - Dominique De Vivo
- Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatrics, University of Messina, Messina, Italy
| | - Simona Valenti
- Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatrics, University of Messina, Messina, Italy
| | - Caterina Pidone
- Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatrics, University of Messina, Messina, Italy
| | - Carmelo Romeo
- Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatric Surgery, University of Messina, Messina, Italy
| | - Rosario Caruso
- Department of Human Pathology in Adulthood and Childhood "G. Barresi", Section of Anatomic Pathology, University of Messina, Messina, Italy
| | - Claudio Romano
- Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatrics, University of Messina, Messina, Italy
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Pereira MS, Munerato MC. Oral Manifestations of Inflammatory Bowel Diseases: Two Case Reports. Clin Med Res 2016; 14:46-52. [PMID: 26864508 PMCID: PMC4851452 DOI: 10.3121/cmr.2015.1307] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2015] [Revised: 11/17/2015] [Accepted: 12/14/2015] [Indexed: 01/07/2023]
Abstract
Inflammatory bowel diseases (IBD) are known as chronic inflammatory disorders of the digestive tract, represented mainly by Crohn's disease (CD) and ulcerative colitis (UC). Among the main oral manifestations of IBD are cobblestoning of the oral mucosa, labial swellings with vertical fissures, pyostomatitis vegetans, angular cheilitis, perioral erythema, and glossitis. In this sense, understanding these nosological entities by dentists would help reach early and differential diagnosis. Thus, two case reports are presented and discussed based on theoretical references obtained by a literature review. The first case report refers to an adult patient whose IBD diagnosis was established after stomatological assessment. The second case was a patient with CD diagnosed in childhood with characteristic oral lesions.
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Affiliation(s)
- Manoela Seadi Pereira
- Faculdade de Odontologia da Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Brazil
| | - Maria Cristina Munerato
- Faculdade de Odontologia da Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Brazil Hospital de Clínicas de Porto Alegre (HCPA), Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Brazil
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Pathogenesis of Behçet's disease: autoinflammatory features and beyond. Semin Immunopathol 2015; 37:413-8. [PMID: 26068404 DOI: 10.1007/s00281-015-0502-8] [Citation(s) in RCA: 54] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2015] [Accepted: 05/25/2015] [Indexed: 12/14/2022]
Abstract
Behçet's disease (BD) is an inflammatory disorder of unknown aetiology characterised by recurrent attacks affecting the mucocutaneous tissues, eyes, joints, blood vessels, brain and gastrointestinal tract. It is a multifactorial disease classified as a variable vessel vasculitis, and several environmental triggers may induce inflammatory episodes in genetically susceptible individuals. BD has several autoinflammatory features including recurrent self-limited clinical manifestations overlapping with monogenic autoinflammatory disorders, significant host predisposition and abnormally increased inflammatory response, with a robust innate component. Human leukocyte antigen (HLA)-B*51 is the strongest susceptibility factor described so far affecting the disease risk and typical phenotype. Non-HLA genetic associations such as endoplasmic reticulum aminopeptidase 1 (ERAP1), interleukin 23 receptor (IL23R) and IL10 variations suggest that BD shares susceptibility genes and inflammatory pathways with spondyloarthritis. Although genomewide association studies revealed an increased risk associated with recessively inherited ERAP1 variations in HLA-B*51 positive patients, it is not clear yet whether certain peptide-HLA allele combinations result in an adaptive response by a self-antigen-directed cytotoxic response or an innate response by modulating an NK cell activity or causing an unfolded protein response. Understanding of major histocompatibility complex (MHC) Class I-driven inflammatory response is expected to provide insights for the development of better treatment and remission-induction options in BD as well as in ankylosing spondylitis (AS) and psoriasis.
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Skef W, Hamilton MJ, Arayssi T. Gastrointestinal Behçet's disease: A review. World J Gastroenterol 2015; 21:3801-3812. [PMID: 25852265 PMCID: PMC4385527 DOI: 10.3748/wjg.v21.i13.3801] [Citation(s) in RCA: 129] [Impact Index Per Article: 12.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2014] [Revised: 01/14/2015] [Accepted: 01/30/2015] [Indexed: 02/07/2023] Open
Abstract
Behçet’s disease (BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and skin lesions. Prevalence of BD is highest in countries along the ancient silk road from the Mediterranean basin to East Asia. By comparison, the prevalence in North American and Northern European countries is low. Gastrointestinal manifestations of Behçet’s disease are of particular importance as they are associated with significant morbidity and mortality. Although ileocecal involvement is most commonly described, BD may involve any segment of the intestinal tract as well as the various organs within the gastrointestinal system. Diagnosis is based on clinical criteria - there are no pathognomonic laboratory tests. Methods for monitoring disease activity on therapy are available but imperfect. Evidence-based treatment strategies are lacking. Different classes of medications have been successfully used for the treatment of intestinal BD which include 5-aminosalicylic acid, corticosteroids, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody therapy. Like inflammatory bowel disease, surgery is reserved for those who are resistant to medical therapy. A subset of patients have a poor disease course. Accurate methods to detect these patients and the optimal strategy for their treatment are not known at this time.
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