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Rheumatic Diseases Clinics of North... Aug 2023Behçet's syndrome is a systemic vasculitis affecting arteries and veins of all sizes as well as recurrent oral, genital, and intestinal ulcers, skin lesions,... (Review)
Review
Behçet's syndrome is a systemic vasculitis affecting arteries and veins of all sizes as well as recurrent oral, genital, and intestinal ulcers, skin lesions, predominantly posterior uveitis, and parenchymal brain lesions. These can be present in various combinations and sequences over time and diagnosis is made by recognizing the manifestations, as there are no diagnostic biomarkers or genetic tests. Treatment modalities include immunomodulatory agents, immunosuppressives and biologics, tailored according to prognostic factors, disease activity, severity, and patients' preferences.
Topics: Humans; Behcet Syndrome; Vasculitis
PubMed: 37331734
DOI: 10.1016/j.rdc.2023.03.010 -
Nature Reviews. Disease Primers Sep 2021Behçet syndrome is a systemic vasculitis with an unknown aetiology affecting the small and large vessels of the venous and arterial systems. The presence of symptom... (Review)
Review
Behçet syndrome is a systemic vasculitis with an unknown aetiology affecting the small and large vessels of the venous and arterial systems. The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest that multiple pathological pathways are involved in Behçet syndrome. These disease features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 as a genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bona fide disease, especially in non-endemic regions, suggests that other factors must also be operative in Behçet syndrome. Despite lacking a clear aetiological mechanism and definition, management of manifestations that include major vascular disease, eye disease and central nervous system involvement has improved with the help of new technology. Furthermore, even with our incomplete understanding of disease mechanisms, the prognoses of patients with Behçet syndrome, including those with eye disease, continue to improve. New treatment options and a better understanding of the underlying pathogenesis for various manifestations of this condition are required to further improve the management of the disease, which will improve patient quality of life.
Topics: Behcet Syndrome; Humans; Prognosis; Quality of Life; Risk Factors
PubMed: 34531393
DOI: 10.1038/s41572-021-00301-1 -
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 -
Lancet (London, England) Mar 2024Behçet's syndrome is a rare, chronic multisystemic inflammatory disorder also known as the Silk Route disease due to its geographical distribution. Behçet's syndrome... (Review)
Review
Behçet's syndrome is a rare, chronic multisystemic inflammatory disorder also known as the Silk Route disease due to its geographical distribution. Behçet's syndrome is a multifactorial disease and infectious, genetic, epigenetic, and immunological factors contribute to its pathogenesis. Its heterogeneous spectrum of clinical features include mucocutaneous, articular, ocular, vascular, neurological, and gastrointestinal manifestations that can present with a relapsing and remitting course. Differential diagnosis is often hampered by the non-specific clinical presentation and the absence of laboratory biomarkers or pathognomonic histological features. The therapeutic approach is tailored on the basis of patient-specific manifestations and relies on glucocorticoids, colchicine, and traditional and biological immunosuppressants. Despite progress in the knowledge and management of the disease, unmet needs in diagnostics, monitoring, prediction, and treatment personalisation challenge clinical practice, making Behçet's syndrome a complex disorder associated with an increased risk of morbidity.
Topics: Humans; Behcet Syndrome; Immunosuppressive Agents; Glucocorticoids; Recurrence; Diagnosis, Differential
PubMed: 38402885
DOI: 10.1016/S0140-6736(23)02629-6 -
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 -
The New England Journal of Medicine Feb 2024
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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 -
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 -
Ocular Immunology and Inflammation Dec 2023Uveitis is uncommon in children and its diagnosis and treatment are challenging. Little is known of the epidemiology of pediatric uveitis. Indeed, population-based... (Review)
Review
Uveitis is uncommon in children and its diagnosis and treatment are challenging. Little is known of the epidemiology of pediatric uveitis. Indeed, population-based studies in the literature are rare. However, there are many tertiary referral center reports that describe the patterns of uveitis in childhood, although few are from developed countries, and their comparison presents some issues. Anterior uveitis is the most frequent entity worldwide, especially in Western countries, where juvenile idiopathic arthritis is diffuse. Most cases of intermediate uveitis do not show any association with infectious or noninfectious systemic diseases. In low- and middle-income countries, posterior uveitis and panuveitis are prevalent due to the higher rates of infectious etiologies and systemic diseases such as Behçet disease and Vogt-Koyanagi-Harada disease. In recent decades, idiopathic uveitis rate has decreased thanks to diagnostic improvements.
Topics: Humans; Child; Retrospective Studies; Uveitis; Behcet Syndrome; Uveomeningoencephalitic Syndrome; Uveitis, Posterior
PubMed: 37922466
DOI: 10.1080/09273948.2023.2271988