scholarly journals Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD

2015 ◽  
Vol 75 (6) ◽  
pp. 958-964 ◽  
Author(s):  
Isabelle Koné-Paut ◽  
Fahrad Shahram ◽  
Martha Darce-Bello ◽  
Luca Cantarini ◽  
Rolando Cimaz ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Disease Onset ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Vascular Involvement ◽  
Data Set ◽  
Behcet's Disease ◽  
Therapeutic Trials

BackgroundWe aimed to describe the main features of Behçet's disease (BD) in children in the largest prospective cohort to date and to propose a classification.MethodsAn international expert consensus group was formed to define a data set of minimal symptoms for the inclusion of patients. Patients were entered prospectively during 66 months. Experts classified patients on a consensus basis. The concordance of two international classifications was analysed in confirmed patients with BD. Comparisons of subgroups of patients helped define consensus criteria. BD-associated clinical manifestations were also investigated in three control diseases extracted from an independent data set (Eurofever).FindingsIn total, 42 centres from 12 countries included 230 patients; data for 219 (M/F ratio=1) could be analysed. The experts classified 156 patients (71.2%) as having confirmed BD. Males more often than females showed cutaneous, ocular and vascular symptoms and females more often genital aphthosis. Age at disease onset and skin and vascular involvement were lower for European than non-European children. Oral aphthosis was the presenting sign for 81% (179/219) of patients. The mean delay to the second symptom was 2.9±2.2 years. International classifications were not concordant with the expert classification. Our paediatric classification contains six categories, a minimum of three signs (each in a distinct category) defining paediatric BD. Three clinical signs discriminated our cohort from the Eurofever cohorts.InterpretationWe present a comprehensive description of a large cohort of patients from both European and non-European countries and propose the first classification of paediatric BD for future therapeutic trials.

Vascular manifestations of Behçet's disease: report of a case and review of the literature

2011 ◽  
Vol 26 (6) ◽  
pp. 249-253 ◽  
Author(s):  
T Kotsis ◽  
K G Moulakakis ◽  
S Mylonas ◽  
V Andrikopoulos
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Inferior Vena ◽  
Recent Literature ◽  
Behçet's Disease ◽  
Vena Cava ◽  
Clinical Manifestations ◽  
Vascular Involvement ◽  
Thrombotic Occlusion ◽  
Behcet's Disease

Adamantiades–Behçet's disease (ABD) is a relapsing vasculitis of unknown aetiology and variable clinical manifestations. The syndrome can be presented in a myriad of ways and can involve nearly every organ. Although vascular involvement is not included among the ABD diagnostic criteria, it is a unique clinical manifestation in adults with a potentially devastating outcome. We report an ABD case, presenting with a thrombotic occlusion of the inferior vena cava. The authors review the recent literature, emphasizing the spectrum of vascular manifestations accompanying Behçet's disease.

Plasma Homocysteine in Behcet's Disease: A Systematic Review and Meta-Analysis

2022 ◽  
Author(s):  
Mira Merashli ◽  
Tommaso Bucci ◽  
Daniele Pastori ◽  
Pasquale Pignatelli ◽  
Alessia Arcaro ◽  
...  
Keyword(s):  
Systematic Review ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Plasma Homocysteine ◽  
Vascular Involvement ◽  
Ocular Involvement ◽  
Female Ratio ◽  
Behcet's Disease

Abstract Aim To evaluate the relevance of plasma homocysteine (HC) in Behcet's disease (BD) and its clinical manifestations. Methods Systematic review of EMBASE and PubMed databases according to PRISMA guidelines from inception to July 2021; random-effects meta-analyses for continuous outcomes. Results The search strategy retrieved 48 case–control (2,669 BD and 2,245 control participants) and 5 cohort studies (708 BD participants). Plasma HC was higher in BD than in controls (p < 0.0001) with wide heterogeneity (I2  = 89.7%) that remained unchanged after sensitivity analysis according to year of article publication, age of BD participants, study size, study quality, method of HC determination, and male/female ratio >1.5; some pooled ethnicities explained a small part of the heterogeneity (I2  = 16.3%). Active BD participants had higher HC than inactive ones (p < 0.0001), with moderate heterogeneity (I2  = 49.2%) that disappeared after removal of an outlier study with very high disease activity. BD participants with any vascular involvement had higher HC than those without (p < 0.0001) with wide heterogeneity (I2  = 89.7%); subgroup analysis on venous thrombosis only changed neither effect size (p < 0.0001) nor heterogeneity (I2  = 72.7%). BD participants with ocular involvement had higher HC than those without (p < 0.0001) with moderate heterogeneity (I2  = 40.3%). Conclusion Although causality cannot be inferred, the consistency of the elevation of plasma HC in BD, particularly in patients with active disease, with vascular and ocular involvement suggests an intrinsic involvement of HC in these clinical manifestations.

scholarly journals Paediatric Behçet’s Disease: Data From A Single Center Experience in Turkey.

2021 ◽  
pp. 101-105
Author(s):  
Esra Bağlan ◽  
Semanur Özdel ◽  
Tülin Güngör ◽  
Deniz Karakaya ◽  
Evra Çelikkaya ◽  
...  
Keyword(s):  
Clinical Features ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Single Center ◽  
Behcet's Disease ◽  
Single Center Experience

Behçet’s disease (BD) is a multisystemic inflammatory disease with unknown etiology. It is characterized by recurrent oral and genital ulcerations, uveitis, and skin lesions, various musculoskeletal, gastrointestinal, central nervous system, and vascular manifestations. The aim of this study was to analyse the demographic characteristics and clinical features, treatment in Turkish paediatric BD from a single center experience. The records of 36 patients with BD who were diagnosed according to the International Study Group criteria between January 2017 and January 2019 in the department of paediatric rheumatology, were retrospectively reviewed. Data on demographic, clinical features and therapy were collected. A total of 36 (19 male) patients were included in this study. Mean age at disease onset was 9.36±4.45 years and mean age at diagnosis 13.99±2.83 years. The frequencies of signs/symptoms were: recurrent oral aphtosis 100%, genital ulcers 80.6%, musculoskeletal 30.6%, ocular 16.7%, neurological 11.1% and vascular involvement 11.1%, gastrointestinal 2.8%. Colchicine and corticosteroids were the main treatments. In this single-center retrospective study, we analyzed the data of paediatric BD and their treatment from a single center in Turkey. The presented small series and the literature review suggest that paediatric BD is a heterogeneous disease with varied clinical manifestations.

scholarly journals Gender Differences in Behçet’s Disease Associated Uveitis

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Didar Ucar-Comlekoglu ◽  
Austin Fox ◽  
H. Nida Sen
Keyword(s):  
Gender Differences ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Vascular Involvement ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Male Patients

Behçet’s disease is a systemic vasculitis of unknown etiology, characterized by oral and genital ulceration, skin lesions, and uveitis as well as vascular, central nervous system, and gastrointestinal system involvement. It is prevalent in the Middle East, Mediterranean, and Eastern Asia. The aim of this review is to evaluate the gender differences in clinical manifestations of Behçet’s disease, treatment responses, mortality, and morbidity. Behçet’s disease has been reported to be more prevalent in males from certain geographic regions and particular ethnic groups; however, recent reports indicate more even gender distribution across the world. There are gender differences in clinical manifestations and severity of the disease. Ocular manifestations, vascular involvement, and neurologic symptoms are more frequently reported in male patients whereas oral and genital ulcers, skin lesions, and arthritis occur more frequently in female patients. The disease can have a more severe course in males, and overall mortality rate is significantly higher among young male patients.

scholarly journals AB0478 CORONARY ATHEROSCLEROSIS IN BEHCET’S DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1537.1-1537
Author(s):  
R. Goloeva ◽  
Z. Alekberova
Keyword(s):  
Behçet’S Disease ◽  
Coronary Atherosclerosis ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Subclinical Atherosclerosis ◽  
Disease Onset ◽  
Atherogenic Index ◽  
Female Ratio ◽  
Behcet's Disease ◽  
The Mean

Background:Behcet’s disease (BD) is systemic vasculitis, which affects all types and sizes of vessels. Increased carotid intima-media thickness (IMT) is parameter associated with subclinical atherosclerosis.Objectives:To determine the prevalence of atherosclerosis in pts with BD.Methods:95 BD pts were evaluated and 45 healthy controls matched for age and gender.IMT was assessed by high-resolution B-mode ultrasonography. Serum concentration of high-sensitivity C-reactive protein (hs CRP) was measured by immunonephelometric assay (BN-100 Analyzer; Dade Behring). Lipid profile evaluation included total cholesterol, TGs, HDL, LDL and atherogenic index.Results:The male-to-female ratio was 3,7:1, the mean age of pts was 29.7 (23-35) yrs, the mean age at the disease onset - 19,9 (14-25) yrs, the mean disease duration - 9,6 (4-15) yrs.Conclusion:Coronary atherosclerosis in BD pts was lower than what we expected. The thinning IMT may be one of the risk factors for aneurysm formation in pts with BD.Disclosure of Interests:None declared

Infliximab treatment in refractory vascular Behcet’s disease: A single-center experience

Vascular ◽  
2020 ◽  
Vol 28 (6) ◽  
pp. 829-833
Author(s):  
Demet Yalçın Kehribar ◽  
Metin Ozgen
Keyword(s):  
Behçet’S Disease ◽  
Oral Anticoagulant ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vascular Involvement ◽  
Infliximab Treatment ◽  
Single Center ◽  
Vascular Events ◽  
Behcet's Disease ◽  
Significant Difference

Objective This study aims to investigate the efficacy and reliability of infliximab treatment in Behcet’s disease with vascular involvement. Methods This single-center retrospective study included a total of 18 patients diagnosed with Behcet’s disease with vascular involvement who were initiated infliximab treatment after exhibiting resistance to conventional immunosuppressive treatments. Results Seventeen patients achieved remission with infliximab treatment. While 18 patients were receiving a median of 50 (IQR: 20–61) mg/day equivalent of methylprednisolone before infliximab treatment, after infliximab treatment, only four patients were receiving 4 mg/day equivalent of methylprednisolone ( p < 0.001). Only 4 patients were receiving oral anticoagulant treatment during infliximab treatment, and compared to the patients who were not receiving oral anticoagulants, there was no significant difference between the two groups according to occurrence of new vascular events. Conclusion Infliximab seems to be an effective and reliable treatment in Behcet’s disease with vascular involvement and may also allow reduced dosage or even the discontinuation of corticosteroids. The results of our study suggest that oral anticoagulant use is unnecessary in Behcet’s disease with vascular involvement. However, further long-term randomized controlled studies are needed to investigate the length of infliximab regimen, whether or not it should be discontinued, and if so, whether or not immunosuppressants should be given as maintenance after discontinuation.

scholarly journals Behcet’s Disease: An In-depth Review About Pathogenesis, Gastrointestinal Manifestations And Management

2021 ◽  
Author(s):  
Anthony Nguyen ◽  
Shubhra Upadhyay ◽  
Muhammad Ali Javaid ◽  
Abdul Moiz Qureshi ◽  
Shahan Haseeb ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Age Groups ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Vascular Disorder ◽  
Robust Algorithms ◽  
Behcet's Disease ◽  
Relapsing Remitting

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.

scholarly journals Behcet's disease with major vascular involvement

2013 ◽  
Vol 2013 (nov08 1) ◽  
pp. bcr2013200893-bcr2013200893 ◽  
Author(s):  
L. N. Geng ◽  
D. Conway ◽  
S. Barnhart ◽  
J. Nowatzky
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vascular Involvement ◽  
Behcet's Disease
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