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The Pan African Medical Journal 2017Our study aimed to determine the clinical, therapeutic and prognostic features of ocular involvement in patients with Behçet's disease treated in our Department of...
Our study aimed to determine the clinical, therapeutic and prognostic features of ocular involvement in patients with Behçet's disease treated in our Department of ophthalmology. We conducted a retrospective data collection from medical records of 20 patients treated at the military hospital in Laayoune. All patients underwent complete ophthalmological examination and fluorescein angiography if necessary. OCT exam was performed in two patients. Ten patients had anterior uveitis, complicated in one case by ocular hypertonia; two patients had intermediate uveitis; eight patients had posterior segment involvement complicated in one case by intravitreal hemorrhage. Behcet's Disease (BD) is an systemic idiopathic inflammatory disease currently classified within primary non-necrotizing vasculitis. Ocular involvement is common and severe in Behçet's disease, with the potential to compromise the visual prognosis. Behcet's disease is common in Morocco. It can compromise patient's visual prognosis making the collaboration between ophthalmologists and internists particularly important.
Topics: Adult; Behcet Syndrome; Eye Diseases; Female; Fluorescein Angiography; Hospitals, Military; Humans; Male; Morocco; Prognosis; Retrospective Studies; Severity of Illness Index; Uveitis, Anterior
PubMed: 28690751
DOI: 10.11604/pamj.2017.26.237.1175 -
Journal of Autoimmunity Oct 2022Behçet's disease tends to be more severe in men than women. This study was undertaken to investigate sex-specific genetic effects in Behçet's disease. (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Behçet's disease tends to be more severe in men than women. This study was undertaken to investigate sex-specific genetic effects in Behçet's disease.
METHODS
A total of 1762 male and 1216 female patients with Behçet's disease from six diverse populations were studied, with the majority of patients of Turkish origin. Genotyping was performed using an Infinium ImmunoArray-24 BeadChip, or extracted from available genotyping data. Following imputation and extensive quality control measures, genome-wide association analysis was performed comparing male to female patients in the Turkish cohort, followed by a meta-analysis of significant results in all six populations. In addition, a weighted genetic risk score for Behçet's disease was calculated and compared between male and female patients.
RESULTS
Genetic association analysis comparing male to female patients with Behçet's disease from Turkey revealed an association with male sex in HLA-B/MICA within the HLA region with a GWAS level of significance (rs2848712, OR = 1.46, P = 1.22 × 10). Meta-analysis of the effect in rs2848712 across six populations confirmed these results. Genetic risk score for Behçet's disease was significantly higher in male compared to female patients from Turkey. Higher genetic risk for Behçet's disease was observed in male patients in HLA-B/MICA (rs116799036, OR = 1.45, P = 1.95 × 10), HLA-C (rs12525170, OR = 1.46, P = 5.66 × 10), and KLRC4 (rs2617170, OR = 1.20, P = 0.019). In contrast, IFNGR1 (rs4896243, OR = 0.86, P = 0.011) was shown to confer higher genetic risk in female patients.
CONCLUSIONS
Male patients with Behçet's disease are characterized by higher genetic risk compared to female patients. This genetic difference, primarily derived from our Turkish cohort, is largely explained by risk within the HLA region. These data suggest that genetic factors might contribute to differences in disease presentation between men and women with Behçet's disease.
Topics: Humans; Female; Male; Behcet Syndrome; Genome-Wide Association Study; Risk Factors; HLA-C Antigens; Genetic Testing
PubMed: 35987173
DOI: 10.1016/j.jaut.2022.102882 -
Dermatology (Basel, Switzerland) 2016Adamantiades-Behçet disease (ABD) is a chronic, multisystemic, recurrent, inflammatory vascular disorder of unknown etiology. Patients with symptoms initially appearing... (Review)
Review
Adamantiades-Behçet disease (ABD) is a chronic, multisystemic, recurrent, inflammatory vascular disorder of unknown etiology. Patients with symptoms initially appearing at the age of 16 or less are considered as cases of juvenile-onset ABD (JABD). JABD is relatively rare compared to ABD of adults, and only case reports and case studies have been published regarding this subtype of the disease. Epidemiology, clinical features, diagnosis and treatment of JABD are discussed in this review.
Topics: Adolescent; Arthralgia; Behcet Syndrome; Child; Eye Diseases; Humans; Infant, Newborn; Intestinal Diseases; Nervous System Diseases; Vascular Diseases
PubMed: 26736030
DOI: 10.1159/000442667 -
Internal Medicine (Tokyo, Japan) Feb 2022Behçet disease and its related disorder, Sweet disease, are multifactorial disorders whose susceptibility loci have been identified in the genes of various...
Behçet disease and its related disorder, Sweet disease, are multifactorial disorders whose susceptibility loci have been identified in the genes of various immunological factors aside from human leukocyte antigens. The neurological involvement of these diseases, including encephalitis, myelitis, and meningitis, referred to as neuro-Behçet disease (NBD) and neuro-Sweet disease (NSD) respectively, is sometimes difficult to diagnose, especially when the characteristic mucocutaneous symptoms do not precede neurological symptoms or when characteristics of both diseases are present in a single patient. NBD and NSD constitute a spectrum of diseases that are differentiated according to the combination of risk factors, including the genetic background. Encephalitis, myelitis, and meningitis similar to NBD or NSD can be diagnosed as spectrum disorders, even if the characteristic mucocutaneous symptoms fail to be detected. Understanding these conditions as a disease spectrum may help elucidate the disease pathogenesis and assist in the development of therapeutic agents.
Topics: Behcet Syndrome; Diagnosis, Differential; Encephalitis; Humans; Meningitis; Sweet Syndrome
PubMed: 34615825
DOI: 10.2169/internalmedicine.8227-21 -
Clinics in Dermatology 2023Adamantiades-Behçet disease is an inflammatory, vascular disease of unknown etiology. The disease is named after two physicians, Benediktos Adamantiades and Hulȗsi...
Adamantiades-Behçet disease is an inflammatory, vascular disease of unknown etiology. The disease is named after two physicians, Benediktos Adamantiades and Hulȗsi Behçet, who both made significant contributions to the study of the disease. It was probably first described by Hippocrates in 500 BCE. Adamantiades-Behçet disease is most common in the region encompassing the ancient trade route known as the Silk Road. In Turkey, the disease is estimated to affect 80 to 370 people per 100,000 inhabitants, and it is also the country with the highest incidence rate. The frequency of the disease associated with the clinical picture differs from the origin of the onset. The disease is characterized by recurrent aphthous ulcers of the mouth, genitals, skin lesions, and eye lesions. The disease process can also involve other organs, including the joints, nervous system, large vessels, heart, and gastrointestinal tract. Aphthous oral ulcers appear as the first harbinger of the disease and affect almost all patients (97%-99%). The scientific interest in Adamantiades-Behçet disease has increased exponentially in the past decade.
Topics: Humans; Behcet Syndrome; Ophthalmology; Dermatology; Stomatitis, Aphthous
PubMed: 37572969
DOI: 10.1016/j.clindermatol.2023.08.001 -
Journal of the Royal College of... 2000We report our clinical experience of 230 patients referred to the Hammersmith hospital with a working diagnosis of Behçet's syndrome. The pathogenesis, diagnosis and... (Review)
Review
We report our clinical experience of 230 patients referred to the Hammersmith hospital with a working diagnosis of Behçet's syndrome. The pathogenesis, diagnosis and management of the syndrome are discussed.
Topics: Adolescent; Adult; Aged; Algorithms; Behcet Syndrome; Eye Diseases; Female; Humans; Inflammation; Male; Middle Aged
PubMed: 10816874
DOI: No ID Found -
Dermatology Online Journal Nov 2010We present a 34-year-old man with a two-year history of aphthous stomatitis, who later developed painful, erythematous nodules on his lower extremities. A pathergy test...
We present a 34-year-old man with a two-year history of aphthous stomatitis, who later developed painful, erythematous nodules on his lower extremities. A pathergy test was positive, and the diagnosis of Behçet syndrome (BS) was made. It is important for the dermatologist to recognize the wide variety of cutaneous manifestations of this disorder. A pathergy test is a simple diagnostic tool that may assist in making a diagnosis. Case reports of other unusual skin manifestations in BS also are reviewed.
Topics: Adult; Behcet Syndrome; Dapsone; Dietary Supplements; Glucocorticoids; Humans; Male; Silver Nitrate; Stomatitis, Aphthous; Treatment Outcome; Vitamins
PubMed: 21163169
DOI: No ID Found -
Frontiers in Immunology 2023The symptoms of Behçet's disease (BD), a multisystemic condition with autoimmune and inflammation as hallmarks, include arthritis, recurring oral and vaginal ulcers,... (Review)
Review
The symptoms of Behçet's disease (BD), a multisystemic condition with autoimmune and inflammation as hallmarks, include arthritis, recurring oral and vaginal ulcers, skin rashes and lesions, and involvement of the nervous, gastrointestinal, and vascular systems. Non-coding RNAs (ncRNAs), including microRNAs (miRNAs), long non-coding RNAs (lncRNAs), and circular RNAs (circRNAs), may be important regulators of inflammation and autoimmune disease. These ncRNAs are essential to the physiological and pathophysiological disease course, and miRNA in particular has received significant attention for its role and function in BD and its potential use as a diagnostic biomarker in recent years. Although promising as therapeutic targets, miRNAs must be studied further to fully comprehend how miRNAs in BD act biologically.
Topics: Female; Humans; MicroRNAs; Behcet Syndrome; Inflammation; Autoimmune Diseases; RNA, Long Noncoding
PubMed: 37860009
DOI: 10.3389/fimmu.2023.1249826 -
Turkish Journal of Ophthalmology Jun 2020Behçet's disease is a chronic, multisystem inflammatory disorder characterized by relapsing inflammation. Although its etiopathogenesis has not yet been clarified, both... (Review)
Review
Behçet's disease is a chronic, multisystem inflammatory disorder characterized by relapsing inflammation. Although its etiopathogenesis has not yet been clarified, both the adaptive and innate immune systems, genetic predisposition, and environmental factors have all been implicated. It is more frequent and more severe in males in the third and fourth decades of life. The eye is the most frequently involved organ in the course of the disease. Ocular involvement (Behçet's uveitis) is characterized by bilateral recurrent non-granulomatous panuveitis and occlusive retinal vasculitis. Recurrent inflammatory episodes in the posterior segment may lead to permanent vision loss due to irreversible retinal damage and complications such as macular scarring, macular atrophy, and optic atrophy. Early and aggressive immunomodulatory treatment and the use of biologic agents when needed are crucial for preventing recurrences and improving visual prognosis.
Topics: Behcet Syndrome; Biological Factors; Disease Management; Glucocorticoids; Humans; Immunosuppressive Agents; Prognosis; Recurrence; Retina
PubMed: 32631005
DOI: 10.4274/tjo.galenos.2019.60308 -
The Israel Medical Association Journal... Aug 2002
Topics: Behcet Syndrome; Humans; Mutation; Risk Factors; Vascular Diseases
PubMed: 12183873
DOI: No ID Found