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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 -
Survey of Ophthalmology 2017The causes of posterior uveitis can be divided into infectious, autoimmune, or masquerade syndromes. Viral infections, a significant cause of sight-threatening ocular... (Review)
Review
The causes of posterior uveitis can be divided into infectious, autoimmune, or masquerade syndromes. Viral infections, a significant cause of sight-threatening ocular diseases in the posterior segment, include human herpesviruses, measles, rubella, and arboviruses such as dengue, West Nile, and chikungunya virus. Viral posterior uveitis may occur as an isolated ocular disease in congenital or acquired infections or as part of a systemic viral illness. Many viruses remain latent in the infected host with a risk of reactivation that depends on various factors, including virulence and host immunity, age, and comorbidities. Although some viral illnesses are self-limiting and have a good visual prognosis, others, such as cytomegalovirus retinitis or acute retinal necrosis, may result in serious complications and profound vision loss. Since some of these infections may respond well to antiviral therapy, it is important to work up all cases of posterior uveitis to rule out an infectious etiology. We review the clinical features, diagnostic tools, treatment regimens, and long-term outcomes for each of these viral posterior uveitides.
Topics: Diagnostic Techniques, Ophthalmological; Eye Infections, Viral; Global Health; Humans; Morbidity; Uveitis, Posterior
PubMed: 28012878
DOI: 10.1016/j.survophthal.2016.12.008 -
Indian Journal of Ophthalmology Jun 2022Sympathetic ophthalmia is a rare, bilateral, granulomatous, panuveitis following penetrating trauma or surgery to one eye. Clinical presentation commonly occurs within... (Review)
Review
Sympathetic ophthalmia is a rare, bilateral, granulomatous, panuveitis following penetrating trauma or surgery to one eye. Clinical presentation commonly occurs within the first year of trauma occurrence but can be delayed by several years. It manifests as acute/chronic granulomatous uveitis with yellowish-white choroidal lesions or Dalen-Fuchs nodules. Initially, patients respond rapidly to corticosteroid therapy, but a majority require long-term use of corticosteroid-sparing agents to prevent recurrences. The purpose of this review is to elaborate on the current understanding of the pathophysiology, the importance of multimodal imaging in early diagnosis, and the role of newer immunomodulatory and biological agents in recalcitrant cases.
Topics: Adrenal Cortex Hormones; Choroid; Humans; Ophthalmia, Sympathetic
PubMed: 35647958
DOI: 10.4103/ijo.IJO_2363_21 -
International Ophthalmology Jan 2022This review aims to summarize the current knowledge concerning the clinical features, diagnostic work-up and therapeutic approach of ocular toxoplasmosis focusing mainly... (Review)
Review
PURPOSE
This review aims to summarize the current knowledge concerning the clinical features, diagnostic work-up and therapeutic approach of ocular toxoplasmosis focusing mainly on the postnatally acquired form of the disease.
METHODS
A meticulous literature search was performed in the PubMed database. A supplementary search was made in Google Scholar to complete the collected items.
RESULTS
Ocular toxoplasmosis is one of the most frequent infectious etiologies of posterior uveitis. It typically presents with retinochoroiditis. Setting an accurate diagnosis depends to a considerable degree on detecting characteristic clinical characteristics. In addition to the evaluation of clinical features, the diagnosis of toxoplasmosis relies at a large degree on serologic testing. The detection of the parasite DNA in the aqueous or vitreous humor can provide evidence for a definitive diagnosis. The current mainstay for the treatment, if necessary, is the use of oral antibiotic with systemic corticosteroids. Recent evidence suggests other therapeutic approaches, such as intravitreal antibiotics can be used.
CONCLUSION
Recent developments in the diagnostic and therapeutic approach have contributed to preventing or limiting vision loss of patients suffering from ocular toxoplasmosis. Further studies are required to provide a better understanding of epidemiology, pathogenesis, diagnosis, and treatment with a significant impact on the management of this challenging clinical entity.
Topics: Chorioretinitis; Eye; Humans; Toxoplasma; Toxoplasmosis, Ocular; Uveitis, Posterior
PubMed: 34370174
DOI: 10.1007/s10792-021-01994-9 -
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 -
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 -
Der Ophthalmologe : Zeitschrift Der... Sep 2021The standardization of uveitis nomenclature (SUN) working group is an international expert committee, which follows the aim to develop a standardized and... (Review)
Review
BACKGROUND
The standardization of uveitis nomenclature (SUN) working group is an international expert committee, which follows the aim to develop a standardized and internationally recognized terminology for the field of uveitis. This appears to be important in view of the demand for evidence-based medicine, especially for relatively rare diseases such as uveitis.
METHODS
A databank of > 4000 uveitis patients was compiled using formal consensus techniques, for whom a majority consensus was previously achieved in the diagnosis. The patient data were analyzed within the subclasses of uveitis and divided into a training set and a validation set. Multinomial logistic regressions with LASSO regularization were carried out on the training set with machine learning (ML). The accuracy of the rules that were developed to express the criteria of ML, were assessed by a masked observer in a random sample of 10%.
RESULTS
The estimations of total accuracy according to the uveitis classes in the validation set were high for all forms of uveitis: anterior uveitis 96.7% (95% confidence interval, CI 92.4-98.6%), intermediate uveitis 99.3% (95% CI 96.1-99.9%), posterior uveitis 98.0% (95% CI 94.3-99.3%), panuveitis 94.0% (95% CI 89.0-96.8%) and infectious posterior uveitis/panuveitis 93.3% (95% CI 89.1-96.3%).
CONCLUSION
Classification criteria are presented, which show a high degree of accuracy (low misclassification rates) and are therefore suitable for future clinical and translational research.
Topics: Humans; Panuveitis; Reference Standards; Uveitis; Uveitis, Anterior; Uveitis, Intermediate
PubMed: 34459962
DOI: 10.1007/s00347-021-01486-2 -
Clinical Medicine (London, England) Feb 2017Behçet's disease (BD) is a chronic relapsing and remitting vasculitis of unknown aetiology. It has the capacity to affect almost all organ systems because of its...
Behçet's disease (BD) is a chronic relapsing and remitting vasculitis of unknown aetiology. It has the capacity to affect almost all organ systems because of its potential to involve both arteries and veins of all sizes, resulting in significant organ-threatening morbidity and mortality. Traditionally known as the 'silk road' disease, it has a worldwide occurrence. The aetiopathological mechanisms of disease development in BD remain poorly understood, but genome wide studies show human leukocyte antigen and non-human leukocyte antigen associations. Environmental influences and genetic factors may have a role in the aetiopathogenetic mechanisms that lead to development of the disease, indicating the autoimmune and auto-inflammatory nature of BD. The evidence base for treatment is limited but new knowledge is emerging and current treatment options range from symptomatic treatment, through to non-biological and biological immunosuppressive drugs, to cover the spectrum of clinical manifestations.
Topics: Behcet Syndrome; Humans
PubMed: 28148585
DOI: 10.7861/clinmedicine.17-1-71 -
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