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Turkish Journal of Medical Sciences Nov 2020We still do not know the cause(s) of Behçet syndrome. Most probably several, separate disease mechanisms are involved. I, like some others, propose we call it not a... (Review)
Review
We still do not know the cause(s) of Behçet syndrome. Most probably several, separate disease mechanisms are involved. I, like some others, propose we call it not a disease but a syndrome, a construct with a list of strong and weak elements. I like to think that this frank admission of our ignorance of its cause(s) will be an important semantic stimulus for more meaningful research.
Topics: Behcet Syndrome; Humans; Male; Syndrome
PubMed: 32222123
DOI: 10.3906/sag-2002-145 -
Clinical and Experimental Rheumatology 2020Ocular involvement in Behçet's syndrome still represents a challenge for both rheumatologists and ophthalmologists; over the past 20 years the availability of new... (Review)
Review
Ocular involvement in Behçet's syndrome still represents a challenge for both rheumatologists and ophthalmologists; over the past 20 years the availability of new diagnostic tools and the concomitant introduction of biologic drugs led to a significant improvement in the management of these patients. The lack of uniform definitions and the diversity of the outcome measures still represent an obstacle for the prompt and correct management of ocular manifestations. The aim of the present review is to summarise the current evidences related to correct diagnosis and proper management of patients with Behçet's syndrome and ocular involvement.
Topics: Behcet Syndrome; Humans
PubMed: 33253088
DOI: No ID Found -
Atencion Primaria Apr 2020
Topics: Adult; Behcet Syndrome; Colchicine; Glucocorticoids; Humans; Male; Prednisone; Stomatitis, Aphthous
PubMed: 31351751
DOI: 10.1016/j.aprim.2019.05.013 -
Journal of the Royal Society of Medicine Oct 1984
Topics: Adult; Behcet Syndrome; Female; Humans; Infant, Newborn; Male
PubMed: 6492037
DOI: 10.1177/014107688407701002 -
JAMA Ophthalmology Apr 2021Although experimental studies support the hypothesis that exposure of infectious agents may trigger an aberrant immune response and contribute to noninfectious uveitis,...
IMPORTANCE
Although experimental studies support the hypothesis that exposure of infectious agents may trigger an aberrant immune response and contribute to noninfectious uveitis, the association of a definite pathogen with human noninfectious uveitis conditions appears not to have been well established in a population.
OBJECTIVE
To evaluate associations of tuberculosis infection with risk of several noninfectious uveitis conditions.
DESIGN, SETTING, AND PARTICIPANTS
These mendelian randomization and observational analyses were conducted with the genetic data of a Chinese cohort enrolled between April 2008 and January 2018 and a Japanese cohort enrolled between January 2002 and June 2009. We recruited participants for T-SPOT.TB (Oxford Immunotec) assays between July and November 2019. The Chinese cohort included patients with uveitis associated with Behçet disease or other uveitis conditions and control participants. The Japanese cohort and the group given T-SPOT.TB assays included individuals with Behçet disease and control participants. Data analyses for this study were completed from July 2019 to January 2020.
EXPOSURES
Genetic variants associated with tuberculosis as natural proxies for tuberculosis exposure.
MAIN OUTCOMES AND MEASURES
The primary outcome was the odds ratio (OR) for Behçet disease, estimated by an inverse variance weighted mean of associations with genetically determined tuberculosis susceptibility. The T-SPOT.TB positivity rate was examined in individuals with Behçet disease and compared with that of control participants.
RESULTS
The Chinese cohort included 999 patients with uveitis associated with Behçet disease, 1585 with other uveitis conditions, and 4417 control participants. The Japanese cohort included 611 individuals with Behçet disease and 737 control participants. The group given T-SPOT.TB assays included 116 individuals with Behçet disease and 121 control participants. Of the Chinese individuals with Behçet disease and control participants, 2257 (41.7%) were female and the mean (SD) age was 35.4 (12.5) years. In the Japanese cohort, 564 (41.8%) were female and the mean (SD) age was 39.1 (12.7) years. Genetically determined tuberculosis susceptibility was associated with an increased risk for Behçet disease. The OR for Behçet disease per 2-fold increase in tuberculosis incidence was 1.26 (95% CI, 1.12-1.43; P = 1.47 × 10-4). Replication using the Japanese cohort yielded similar results (OR, 1.16 [95% CI, 1.08-1.26]). In T-SPOT.TB assays, having a positive result, indicating a history of tuberculosis infection, was found to be an independent risk factor for Behçet disease (OR, 2.26 [95% CI, 1.11-4.60]).
CONCLUSIONS AND RELEVANCE
These human genetic and biomarker data demonstrated that tuberculosis exposure was a risk factor for Behçet disease. This study provides novel evidence linking an infectious agent to the risk of a noninfectious uveitis condition.
Topics: Adult; Behcet Syndrome; Female; Humans; Interferon-gamma Release Tests; Male; Tuberculosis; Tuberculosis, Ocular; Uveitis
PubMed: 33599689
DOI: 10.1001/jamaophthalmol.2020.6985 -
Orphanet Journal of Rare Diseases Apr 2012DEFINITION OF THE DISEASE: Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous... (Review)
Review
UNLABELLED
DEFINITION OF THE DISEASE: Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations.
EPIDEMIOLOGY
BD seems to cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. European cases are often described, not exclusively in the migrant population.
CLINICAL DESCRIPTION
The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations.
ETIOLOGY
The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated.
DIAGNOSTIC METHODS
Diagnosis is only based on clinical criteria. DIFFERRENTIAL DIAGNOSIS: It depends on the clinical presentation of BD, but sarcoidosis, multiple sclerosis, Crohn's disease, Takayasu's arteritis, polychondritis or antiphospholipid syndrome need to be considered.
MANAGEMENT
Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy.
PROGNOSIS
The prognosis is severe due to the ocular, neurological and arterial involvement.
Topics: Adolescent; Adult; Autoimmunity; Behcet Syndrome; Humans; Immunomodulation; Rare Diseases; Steroids; Young Adult
PubMed: 22497990
DOI: 10.1186/1750-1172-7-20 -
Clinical and Experimental Rheumatology 2021This review aims to provide a critical digest of the recent studies that enhance our understanding of Behçet's syndrome by evaluating time trends, differences in... (Review)
Review
This review aims to provide a critical digest of the recent studies that enhance our understanding of Behçet's syndrome by evaluating time trends, differences in disease course between men and women, and between patients with an early and late disease onset, progress in disease assessment, novel findings on immunopathogenesis and genetics, clinical features and differential diagnosis of eye, vascular, nervous system and gastrointestinal system involvement, and new data on treatment modalities including TNF-alpha, IL-17 and IL-6 inhibitors, tofacitinib, and apremilast, as well as surgical interventions.
Topics: Behcet Syndrome; Disease Progression; Female; Humans; Male; Tumor Necrosis Factor Inhibitors
PubMed: 34524077
DOI: 10.55563/clinexprheumatol/lnvc9k -
Frontiers in Immunology 2019Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and... (Review)
Review
Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and gastrointestinal manifestations. Based on this heterogeneity, BS can be hardly considered as a single clinical entity. Growing evidence supports that, within BS, different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, three major BS phenotypes have been reported: (a) the mucocutaneous and articular phenotype, (b) the extra-parenchymal neurological and peripheral vascular phenotype, and (c) the parenchymal neurological and ocular phenotype. To date, guidelines for the management of BS have been focused on the pharmacological treatment of each specific BS manifestation. However, tailoring the treatments on patient's specific phenotype, rather than on single disease manifestation, could represent a valid strategy for a personalized therapeutic approach to BS. In the present literature review, we summarize current evidence on the pharmacological treatments for the first-, second-, and third-line treatment of the major BS phenotypes.
Topics: Behcet Syndrome; Blood Vessels; Cartilage, Articular; Combined Modality Therapy; Disease Management; Humans; Mucous Membrane; Phenotype
PubMed: 31921115
DOI: 10.3389/fimmu.2019.02830 -
Swiss Medical Weekly Jul 2019Behçet's syndrome is a rare systemic autoimmune/autoinflammatory disease affecting mucocutaneous tissues, the skin and the eyes, as well as the joints, the central...
OBJECTIVE
Behçet's syndrome is a rare systemic autoimmune/autoinflammatory disease affecting mucocutaneous tissues, the skin and the eyes, as well as the joints, the central nervous system, the gastrointestinal tract and blood vessels. Because of the lack of clinical data in Switzerland, the aims of this cohort study were to calculate the disease prevalence and to analyse the disease manifestations and the immune-suppressive medication.
METHODS
Data were extracted from 52 patient charts. Thereafter, all patients were interviewed with a questionnaire and 46 had an additional physical examination and laboratory analyses. For calculation of prevalence, data of the national statistical bureau were used.
RESULTS
A disease prevalence of 4.03/100,000 inhabitants was calculated. The mean delay between first disease manifestation and diagnosis was 8 years. It was 2 years longer for Swiss than for non-Swiss individuals (p = 0.45). The time intervals between diagnosis and occurrence of different organ manifestations ranged from +8 to -11 years. There was no difference in organ involvement between different ethnicities. Colchicine was prescribed for 52% of patients only, whereas tumour necrosis factor (TNF) inhibitors and glucocorticoids were most frequently prescribed (80 and 64%, respectively). In almost half of the patients, TNF blockers could be stopped and replaced by conventional immunosuppressive drugs.
CONCLUSION
The data from this cohort of Behçet's syndrome patients, the largest in Switzerland, documents a prevalence higher than anticipated. The diagnostic delay underlines an urgent need to improve awareness of the disease and allow timely treatment.
Topics: Adult; Aged; Behcet Syndrome; Female; Glucocorticoids; Humans; Male; Middle Aged; Pregnancy; Prevalence; Retrospective Studies; Surveys and Questionnaires; Switzerland; Time-to-Treatment
PubMed: 31329265
DOI: 10.4414/smw.2019.20072 -
World Journal of Gastroenterology Feb 2012Adamantiades-Behcet's disease (ABD) is a chronic, relapsing, systemic vasculitis of unknown etiology. It is more prevalent in populations along the ancient Silk Road... (Review)
Review
Adamantiades-Behcet's disease (ABD) is a chronic, relapsing, systemic vasculitis of unknown etiology. It is more prevalent in populations along the ancient Silk Road from Eastern Asia to the Mediterranean Basin, and most frequently affects young adults between the second and fourth decades of life. ABD-complicated gastroenteropathy is a significant cause of morbidity and mortality, with abdominal pain as the most common symptom. The ileocecal region is affected predominantly, with ulcerations that may lead to penetration and/or perforation, whereas other parts of the gastrointestinal system including the esophagus and stomach can also be affected. Endoscopy is useful to locate the site and extent of the lesions, and tissue biopsy is often warranted to examine the histopathology that is often suggestive of underlying vasculitis of small veins/venules or, alternatively in some cases, nonspecific inflammation. Bowel wall thickening is the most common finding on computed tomography scan. Treatment is largely empirical since well-controlled studies are difficult to conduct due to the heterogeneity of the disease, and the unpredictable course with exacerbation and remission. Corticosteroids with or without other immunosuppressive drugs, such as cyclophosphamide, azathioprine, sulfasalazine, tumor necrosis factor α antagonist or thalidomide should be applied before surgery, except in emergency.
Topics: Adrenal Cortex Hormones; Behcet Syndrome; Asia, Eastern; Gastrointestinal Diseases; Humans; Immunosuppressive Agents
PubMed: 22363131
DOI: 10.3748/wjg.v18.i7.609