scholarly journals Behcet’s disease: a review

2021 ◽  
Author(s):  
Daniela Ann Reyes-Weaver ◽  
Kevin Luis Plata-Jimenez ◽  
Raul Melo-Acevedo
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Age Of Onset ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Vascular Lesions ◽  
Severe Condition ◽  
Behcet's Disease ◽  
Oral Ulcers

Behcet's disease (BD) is a complex systemic vasculitis with an etiopathogenesis that remains unclear. It has a strong geographic association as well as a genetic propensity linked to the HLA-B51 factor and interactions between genetic and environmental factors. The typical age of onset is 25 to 40 years old and it is more common in men, who also have a more severe condition. Occlusive vasculitis is the hallmark of this condition, which can affect vessels of all diameters. Oral ulcers, genital ulcers, skin lesions, pathergy reaction as well as involvement of other systems and organs such as ophthalmic, neurological and vascular lesions, among others, are used to make the diagnosis. Uncontrolled neutrophil activation, activation of the humoral and cell immune systems, toxic proteins and infectious agents such as herpes simplex and streptococci are all involved in their pathophysiology. Due to the heterogeneity and several systems affected, the treatment is individualized and focused on treating each clinical manifestation.

scholarly journals Aortic thrombosis in a patient with a late diagnosis of Behcet's disease

2015 ◽  
Vol 14 (2) ◽  
pp. 193-196
Author(s):  
Ana Bittencourt Detanico ◽  
Marcelo Luiz Brandão ◽  
Ly de Freitas Fernandes ◽  
Carolina Parreira Ribeiro Camelo ◽  
Juliano Ricardo Santana dos Santos
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Thromboangiitis Obliterans ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Arterial Involvement ◽  
Genital Ulcers

Behcet's disease is form of systemic vasculitis of unknown etiology. One surprising feature is that arterial involvement is less common in this disease than venous forms, accounting for 1 to 7% of patients. In 7 to 30% of cases the vascular symptoms precede the clinical diagnosis of Behcet. We describe the case of a patient with Thromboangiitis Obliterans who was treated with lumbar sympathectomy and an aortobiiliac bypass. Around 1 year later he was diagnosed with Behcet's disease after presenting with oral ulcers, genital ulcers and scaling lesions on the hands.

scholarly journals Behcet’s Disease in an Adult Male From Nepal: A Case Report

2021 ◽  
Author(s):  
Madan Basnet ◽  
Abisha Phudong ◽  
Kamal Gautam ◽  
Bishnu Pathak ◽  
Suman Gaire ◽  
...  
Keyword(s):  
Case Report ◽  
Blood Vessels ◽  
Behçet’S Disease ◽  
Adult Male ◽  
Acute Inflammation ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Behcet's Disease

Behcet’s Disease is a rare systemic vasculitis characterized by recurrent episodes of acute inflammation affecting blood vessels of all sizes. Symptoms include orogenital apthosis, cutaneous skin lesions, and uveitis. We present the case of a 38-year-old Nepalese man with Behcet’s Disease. In Nepal, Behcet’s Disease may still be under-reported.

Ethiopathogenesis of Behçet's Disease

2018 ◽  
pp. 324-328
Keyword(s):  
Behçet’S Disease ◽  
Genetic Factors ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Th2 Cells ◽  
Innate And Adaptive Immunity ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Th 1

Behçet’s disease (BD) is a chronic multisystem inflammatory disease characterized mainly by recurrent oral ulcers, ocular inflammation, genital ulcers, and skin lesions, presenting with remission and exacerbations. The pathogenesis of BD has not been fully elucidated since it has a broad spectrum of phenotypic heterogeneity and complex immunopathogenesis. BD has been considered that both environmental and genetic factors contribute to its onset and development. Recent advances in molecular biology and genetics show us that particular genes encompass both innate and adaptive immunity, and confirm the importance of the predominant polarization towards helper T cell (Th)1 versus Th2 cells, and the involvement of Th17 cells in BD.

Epidemiology, Prevalence, Incidence in Behçet's Disease

2018 ◽  
pp. 321-323
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Medical Community ◽  
Aphthous Ulcer ◽  
Ocular Involvement ◽  
Behcet's Disease ◽  
Ancient Silk Road

Behçet’s disease (BD) was first brought to the attention of the modern medical community by Hulusi Behçet in 1937. The disease is a systemic vasculitis characterized by a recurrent aphthous ulcer, genital ulcer, uveitis, and skin lesions. It is observed more common and severe throughout the ancient Silk Road from East Asia to the Mediterranean. Ocular involvement is reported in 25-75% of patients with BD. In most cases, it may progress to blindness if left untreated. The aim of this review is to investigate the epidemiology, prevalence, and incidence of BD.

scholarly journals Diagnosis/Classification Criteria for Behcet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Fereydoun Davatchi
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Far East ◽  
Skin Lesions ◽  
Historical Background ◽  
Vascular Lesions ◽  
Behcet's Disease ◽  
Pathergy Test ◽  
Eye Lesions

Historical Background. The ISG criteria for Behcet's, created in 1990, have excellent specificity, but lack sensitivity. The International Criteria for Behcet's Disease (ICBD) was created in 2006, as replacement to ISG. The aim of this study was to compare their performance. ISG and ICBD Criteria. For ISG oral aphthosis is mandatory. The presence of any two of the following (genital aphthosis, skin lesions, eye lesions, and positive pathergy test) will diagnose/classify the patient as BD. For ICBD, vascular lesions were added, while oral aphthosis is no more mandatory. Getting 3 or more points diagnose/classify the patient as BD (genital aphthosis 2 points, eye lesions 2 points, and the remaining each one point). Performance and Comparison of ISG and ICBD. Their sensitivity, specificity, and accuracy (percent agreement), were tested in three independent cohort of patients from Far-East (China), Middle-East (Iran), and Europe (Germany). The sensitivity for ISG was respectively 65.4%, 78.1%, 83.7% and for ICBD 87%, 98.2%, and 96.5%. The specificity for ISG was 99.2%, 98.8%, 89.5% and for ICBD 94.1%, 95.6%, and 73.7%. The accuracy for ISG was 74.2%, 85.5%, 85.5% and for ICBD 88.9%, 97.3%, and 89.5%. Conclusion. ICBD has better sensitivity, and accuracy than ISG.

scholarly journals Behçet’s Disease as a Model of Venous Thrombosis

2010 ◽  
Vol 4 (1) ◽  
pp. 71-77 ◽  
Author(s):  
Micaela La Regina ◽  
Armen Yuri Gasparyan ◽  
Francesco Orlandini ◽  
Domenico Prisco
Keyword(s):  
Venous Thrombosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Chronic Inflammatory Disease ◽  
Vascular Lesions ◽  
Behcet's Disease ◽  
Therapeutic Measures ◽  
Arterial Aneurysms

Behçet’s disease (BD) is a chronic inflammatory disease of unknown aetiology characterized by recurrent oral, genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in BD. The frequency of vascular lesions in BD, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. In this review, various factors of thrombogenesis in BD, particularly pro- and antithrombotic endothelial and non-endothelial factors, factors of coagulation, platelet activation and rheological changes are presented and discussed from positions of Virchow’s triad of venous thrombosis. Despite advances in understanding of thrombogenesis in BD, still many issues of diagnosis and targeted preventive and therapeutic measures remain unresolved. Further studies are needed to clarify the pathobiology of BD-related thrombosis and to provide the clinicians with recommendations over the utility, safety and effectiveness of the antithrombotic therapy in BD.

scholarly journals New Evidence-Based Treatment Approach in Behçet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Erkan Alpsoy
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Age Of Onset ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Severe Disease ◽  
Immunosuppressive Agents ◽  
Evidence Based ◽  
Close Monitoring ◽  
Behcet's Disease

Behçet's disease (BD) is a chronic, relapsing, and debilitating systemic vasculitis of unknown aetiology with the clinical features of mucocutaneous lesions, ocular, vascular, articular, neurologic, gastrointestinal, urogenital, and pulmonary involvement. The disease is much more frequent along the ancient “Silk Route” extending from Eastern Asia to the Mediterranean basin, compared with Western countries. The disease usually starts around the third or fourth decade of life. Male sex and a younger age of onset are associated with more severe disease. Although the treatment has become much more effective in recent years, BD is still associated with severe morbidity and considerable mortality. The main aim of the treatment should be the prevention of irreversible organ damage. Therefore, close monitoring, early, and appropriate treatment is mandatory to reduce morbidity and mortality. The treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents. In this paper, current state of knowledge regarding the therapeutic approaches is outlined. To provide a rational framework for selecting the appropriate therapy along the various treatment choices, a stepwise, symptom-based, evidence-based algorithmic approach was developed.

scholarly journals Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis - Case report

2013 ◽  
Vol 88 (3) ◽  
pp. 448-451 ◽  
Author(s):  
Maraya de Jesus Semblano Bittencourt ◽  
Carolina Moraes Dias ◽  
Thaiane Lima Lage ◽  
Renata Silva Barros ◽  
Otavio Augusto Gomes Paz ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vena Cava ◽  
Skin Lesions ◽  
Vascular Lesions ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Behcet's Disease ◽  
Chiari Syndrome

Behçet's disease is a chronic inflammatory disease of unknown aetiology, characterized by recurrent oral and genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in Behçet's disease. The frequency of vascular lesions in Behçet's disease, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. Budd-Chiari syndrome is a rare and serious complication of Behçet's disease and implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. We report a case of a 25-year-old man with Behçet's disease that developed Budd-Chiari syndrome. The correlation of dermatological, pathological and imaging studies confirmed the diagnosis.

scholarly journals Pathologic Features of Behçet's Disease in the Tubuler Gut

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Tuba Kara ◽  
Duygu Düşmez Apa
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Japanese Patients ◽  
Skin Lesions ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Mediterranean Countries ◽  
Pathologic Features ◽  
Urgent Operation

Behçet's disease (BD) is a vasculitic disorder of relapsing acute inflammation characterized by recurrent oral ulcers, genital ulcers, uveitis, and skin lesions. The disease also affects other organs, including joints, the nervous system, blood vessels, and gastrointestinal (GI) system may also be involved and the lower GI tract is the mostly involved part, leading to severe morbidity. The frequency of GI involvement in BD varies among different ethnic groups. Although 50–60% of Japanese patients have GI disease, these manifestations are rare in patients from Mediterranean countries. The gastrointestinal manifestations of BD usually appear 4.5–6 years after the onset of the oral ulcers. The intestinal lesions are usually resistant to medical treatment and recur after surgery. The elementary lesion is apthous ulcer. Deep, round or oval ulcers with a punched-out appearance tend to perforate easily, so that many patients require urgent operation.

scholarly journals Beneficial Effects of Apremilast on Genital Ulcers, Skin Lesions, and Arthritis in Patients With Behçet’s Disease: A Systematic Review and Meta-Analysis

2021 ◽  
Author(s):  
Yuki Iizuka ◽  
Kaoru Takase-Minegishi ◽  
Lisa Hirahara ◽  
Yohei Kirino ◽  
Yutaro Soejima ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Skin Lesions ◽  
Cochrane Library ◽  
Behcet's Disease ◽  
Beneficial Effects ◽  
Oral Ulcers ◽  
Genital Ulcers

Abstract Objective This study aimed to determine the clinical efficacy of apremilast for oral ulcers, extra-oral manifestations, and overall disease activity in patients with Behçet’s disease (BD). Methods A systematic literature search was performed in PubMed, EMBASE, Cochrane Library, and Web of Science Core Collection. Studies assessing the treatment effects of apremilast in BD were included. The odds ratios (ORs) of being symptom free for individual manifestations and mean difference (MD) of Behçet’s Disease Current Activity Form (BDCAF) scores were calculated with 95% confidence intervals (CIs) at 12 and 24 weeks using a random-model meta-analysis. Results Of 259 screened articles, eight were included. After 12 weeks of apremilast treatment the OR of symptom-free was as followings: oral ulcers, 45.76 (95% CI, 13.23–158.31); genital ulcers, 4.56 (95% CI, 2.47–8.44); erythema nodosum, 3.59 (95% CI, 1.11–11.61); pseudofolliculitis, 2.81 (95% CI, 1.29–6.15); and arthritis, 3.55 (95% CI, 1.71–7.40). Furthermore, BDCAF scores at 12 weeks were significantly reduced (MD=−1.38; −1.78 to −0.99). However, the proportion of oral-ulcer free patients increased at 24 weeks (OR=14.88; 4.81 to 46.07). Conclusion The currently accumulated data indicates an improvement in mucocutaneous and articular symptoms by short-term apremilast treatment in patients with BD.

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