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Clinical and Experimental Rheumatology Sep 2022This review highlights publications on different aspects of Behçet's syndrome (BS) that appeared in 2021 and provides a critical view. These publications include works... (Review)
Review
This review highlights publications on different aspects of Behçet's syndrome (BS) that appeared in 2021 and provides a critical view. These publications include works on the epidemiology of BS across different continents, newly developed instruments to assess damage in BS, studies highlighting the immunopathogenesis, genetics and epigenetic factors, histopathology of the pathergy lesion, clinical and imaging aspects of vascular involvement, and safety and efficacy of therapeutic agents including tocilizumab, apremilast and direct oral anticoagulants.
Topics: Anticoagulants; Behcet Syndrome; Humans
PubMed: 35894066
DOI: 10.55563/clinexprheumatol/h4dkrs -
Clinical and Experimental Rheumatology 2020Behçet's syndrome is a variable vessel vasculitis with multi-system involvement that shows important heterogeneity among patients regarding demographic features, organ... (Review)
Review
Behçet's syndrome is a variable vessel vasculitis with multi-system involvement that shows important heterogeneity among patients regarding demographic features, organ manifestations, frequency and severity of relapses, disease course, response to treatment and prognosis. This heterogeneity has made it difficult to interpret and compare the results of studies, to standardise disease assessment and to develop management strategies. Several new studies have been published during the previous year exploring the epidemiology, pathogenesis, clinical manifestations, diagnosis, and management of Behçet's syndrome. The aim of this review is to provide an overview of the most relevant recent research in Behçet's syndrome from a critical perspective.
Topics: Behcet Syndrome; Disease Progression; Humans; Prognosis; Recurrence
PubMed: 33331271
DOI: No ID Found -
Clinical and Experimental Rheumatology Oct 2023This critical review of studies on Behçet's syndrome published during 2022 includes studies on epidemiology, patients' perspective, pathogenesis, diagnosis, clinical... (Review)
Review
This critical review of studies on Behçet's syndrome published during 2022 includes studies on epidemiology, patients' perspective, pathogenesis, diagnosis, clinical features and management. Studies on pathogenesis included potential biomarkers mostly related to macrophages, neutrophil and cytokine balance, new GWAS and polymorphism studies, and studies on miRNAs and long non-coding RNAs. Clinical studies showed that application of pneumococcal vaccine to the prick site increased the sensitivity and specificity of the pathergy test and the prevalence of AA amyloidosis had decreased over the years. Studies on management indicated that more data are needed to understand the effect of apremilast on BS manifestations other than oral ulcers, and new BS manifestations may develop during treatment with infliximab. Other biologics and Jak inhibitors might be an option for patients who are refractory to TNF-α inhibitors. Moreover, endovascular repair of arterial aneurysms might be an alternative to open surgery.
Topics: Humans; Behcet Syndrome; Infliximab; Tumor Necrosis Factor-alpha; Aneurysm; Sensitivity and Specificity; Tumor Necrosis Factor Inhibitors
PubMed: 37877363
DOI: 10.55563/clinexprheumatol/7kdo9x -
World Journal of Gastroenterology Apr 2015Behçet's disease (BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and... (Review)
Review
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.
Topics: Animals; Behcet Syndrome; Diagnosis, Differential; Gastrointestinal Diseases; Humans; Predictive Value of Tests; Prevalence; Risk Factors; Severity of Illness Index; Treatment Outcome
PubMed: 25852265
DOI: 10.3748/wjg.v21.i13.3801 -
Frontiers in Immunology 2019Behcet's disease (BD) is a chronic systemic inflammatory vasculitis of unknown etiology characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin... (Review)
Review
Behcet's disease (BD) is a chronic systemic inflammatory vasculitis of unknown etiology characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, and other manifestations. Although the pathogenesis of BD is unclear, some studies have shown that immunological aberrations play an important role in the development and progression of BD. Infection-related trigger factors, including antigens and autoantigens, are believed to mediate the development of BD in patients with a genetic predisposition and subsequently activate the innate and adaptive immune systems, resulting in the production of numerous cytokines and chemokines to combat the infection-related factors. The study of the immunological mechanism of BD paves the way for the development of innovative therapies. Recently, novel biotherapy approaches, including interferon-α (IFN-α), tumor necrosis factor-α (TNF-α) antagonists, and other agents that target interleukins and their receptors, have shown promising results in the treatment of patients with refractory BD and have improved the prognosis of BD. In this review, we provide the current concepts of BD immunopathogenesis.
Topics: Adaptive Immunity; Autoantigens; Behcet Syndrome; Cytokines; Genetic Predisposition to Disease; Humans; Immunity, Innate
PubMed: 30984205
DOI: 10.3389/fimmu.2019.00665 -
American Journal of Ophthalmology Aug 2021To determine classification criteria for Behçet disease uveitis. (Comparative Study)
Comparative Study
PURPOSE
To determine classification criteria for Behçet disease uveitis.
DESIGN
Machine learning of cases with Behçet disease and 5 other panuveitides.
METHODS
Cases of panuveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set.
RESULTS
One thousand twelve cases of panuveitides, including 194 cases of Behçet disease with uveitis, were evaluated by machine learning. The overall accuracy for panuveitides was 96.3% in the training set and 94.0% in the validation set (95% confidence interval 89.0, 96.8). Key criteria for Behçet disease uveitis were a diagnosis of Behçet disease using the International Study Group for Behçet Disease criteria and a compatible uveitis, including (1) anterior uveitis; (2) anterior chamber and vitreous inflammation; (3) posterior uveitis with retinal vasculitis and/or focal infiltrates; or (4) panuveitis with retinal vasculitis and/or focal infiltrates. The misclassification rates for Behçet disease uveitis were 0.6% in the training set and 0% in the validation set, respectively.
CONCLUSIONS
The criteria for Behçet disease uveitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.
Topics: Adult; Behcet Syndrome; Consensus; Female; Humans; Machine Learning; Male; Retrospective Studies; Uveitis; Young Adult
PubMed: 33845008
DOI: 10.1016/j.ajo.2021.03.058 -
Anais Brasileiros de Dermatologia 2017Behçet's disease is a systemic vasculitis characterized by attacks of acute inflammation, which can affect almost every vascularized area of the body. There is a close... (Review)
Review
Behçet's disease is a systemic vasculitis characterized by attacks of acute inflammation, which can affect almost every vascularized area of the body. There is a close correlation between the geographical distribution of HLA-B51 and its prevalence. In the etiopathogenesis there are indications of genetic susceptibility associated with environmental influence. Among the involved genes are those that encompass innate and adaptive immunities. Polymorphisms and epistatic interactions in several genes are described, as well as the presence of imbalance lineage between HLA-B51 and A (MICA). Herpes simplex and Streptococcus sanguinis may be important extrinsic factors. An increase of Th1 response and of IL-21 is observed. The production of IL-21 is positively related to Th17 cells and negatively to T-regs. The mucocutaneous manifestations are Behcet´s disease markers, and their earlier onset indicates a worse prognosis. Recurrent oral ulcers have varied sizes and arrangements, genital ulcers are recurrent, leaving scars, skin lesions are multivaried, and pathergy, although not so frequent, is important for the diagnosis. There are numerous attempts to validate indexes that can evaluate the disease activity and among them the Mucocutaneous Activity Index. This is a specific score that can help with therapeutic decisions and to reduce morbidity, but still lacks validation. The clinical manifestations of other organs are described as well as treatment options.
Topics: Behcet Syndrome; Diagnosis, Differential; Gene-Environment Interaction; Humans; Immunity, Cellular; Review Literature as Topic
PubMed: 28954091
DOI: 10.1590/abd1806-4841.20177359 -
Journal of Neurology Sep 2014Neuro-Behçet's disease (NBD) is one of the more serious manifestations of Behçet's disease (BD), which is a relapsing inflammatory multisystem disease with an... (Review)
Review
Neuro-Behçet's disease (NBD) is one of the more serious manifestations of Behçet's disease (BD), which is a relapsing inflammatory multisystem disease with an interesting epidemiology. Though NBD is relatively uncommon, being potentially treatable, neurologists need to consider it in the differential diagnosis of inflammatory, infective, or demyelinating CNS disorders. Evidence-based information on key issues of NBD diagnosis and management is scarce, and planning for such studies is challenging. We therefore initiated this project to develop expert consensus recommendations that might be helpful to neurologists and other clinicians, created through an extensive literature review and wide consultations with an international advisory panel, followed by a Delphi exercise. We agreed on consensus criteria for the diagnosis of NBD with two levels of certainty in addition to recommendations on when to consider NBD in a neurological patient, and on the use of various paraclinical tests. The management recommendations included treatment of the parenchymal NBD and cerebral venous thrombosis, the use of disease modifying therapies, prognostic factors, outcome measures, and headache in BD. Future studies are needed to validate the proposed criteria and provide evidence-based treatments.
Topics: Behcet Syndrome; Consensus; Diagnosis, Differential; Evidence-Based Practice; Humans; Intracranial Thrombosis; Venous Thrombosis
PubMed: 24366648
DOI: 10.1007/s00415-013-7209-3 -
Arthritis Research & Therapy Oct 2022Clustering is an important clinical feature of Behçet's syndrome (BS) and may have pathogenetic and therapeutic implications. Recent and previous studies on BS...
Clustering is an important clinical feature of Behçet's syndrome (BS) and may have pathogenetic and therapeutic implications. Recent and previous studies on BS phenotype differ substantially in terms of methodology. Correlation matrices and factor analyses were not efficient enough to uncover clusters. Clustering patterns may change according to demographic factors such as age and sex. Clustering patterns may also be profoundly influenced by the misperception of symptoms that are assumed to be secondary to BS, when, in fact, they represent manifestations of BD mimics. This can give rise to misleading conclusions and should be kept in mind when interpreting data obtained by clustering or other phenotype analyses of BS. A true geographical/racial variability in disease expression could be studied in a multinational consensus cohort. Pathogenetic studies in separate clusters of BS have still been lacking.
Topics: Humans; Behcet Syndrome; Cluster Analysis; Phenotype
PubMed: 36309718
DOI: 10.1186/s13075-022-02937-0 -
Clinical and Experimental Rheumatology Sep 2022
Topics: Behcet Syndrome; Humans
PubMed: 36062752
DOI: 10.55563/clinexprheumatol/2eggnf