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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 -
Clinical and Experimental Rheumatology Sep 2022
Topics: Behcet Syndrome; Humans
PubMed: 36062752
DOI: 10.55563/clinexprheumatol/2eggnf -
Journal of Immunology Research 2018Oral signs are frequently the first manifestation of autoimmune diseases. For this reason, dentists play an important role in the detection of emerging autoimmune... (Review)
Review
Oral signs are frequently the first manifestation of autoimmune diseases. For this reason, dentists play an important role in the detection of emerging autoimmune pathologies. Indeed, an early diagnosis can play a decisive role in improving the quality of treatment strategies as well as quality of life. This can be obtained thanks to specific knowledge of oral manifestations of autoimmune diseases. This review is aimed at describing oral presentations, diagnosis, and treatment strategies for systemic lupus erythematosus, Sjögren syndrome, pemphigus vulgaris, mucous membrane pemphigoid, and Behcet disease.
Topics: Autoimmune Diseases; Behcet Syndrome; Diagnosis, Oral; Female; Humans; Lupus Erythematosus, Systemic; Male; Mouth; Mouth Diseases; Pemphigoid, Benign Mucous Membrane; Pemphigus; Prognosis; Sjogren's Syndrome
PubMed: 29977929
DOI: 10.1155/2018/6061825 -
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 -
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 -
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 -
Journal of the European Academy of... Sep 2021
Topics: Behcet Syndrome; COVID-19; Humans; SARS-CoV-2
PubMed: 33914986
DOI: 10.1111/jdv.17325 -
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 -
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 -
Clinical and Experimental Rheumatology Oct 2023The aim of this review was to describe the changes in the microbiota of patients with Behçet's disease (BD) and the mechanisms involved in the relationship between the... (Review)
Review
The aim of this review was to describe the changes in the microbiota of patients with Behçet's disease (BD) and the mechanisms involved in the relationship between the microbiome and immunity in BD. A systematic search for relevant articles was made on PubMed and the Cochrane Library database using the following terms: "microbiota AND Behçet's disease" or "microbiome AND Behçet's disease". Sixteen articles were included in a qualitative synthesis. This systematic review on the microbiome and Behçet's disease underlines the presence of gut dysbiosis in BD patients. This dysbiosis is marked by (i) a decrease in butyrate-producing bacteria, which could affect T cell differentiation and epigenetic regulation of immune-related genes, (ii) a modification of tryptophan-metabolising bacteria, which could be linked to dysregulated IL-22 secretion, and (iii) a decrease in bacteria known to have anti-inflammatory properties. Regarding oral microbiota, this review underlines the possible role of Streptococcus sanguinis through molecular mimicry and NETosis. Clinical studies of BD have shown that (i) need for dentistry is associated with a more severe course in BD, and (ii) antibiotic-supplemented mouthwash reduces pain and ulcers. Fecal transplantation of BD patients' microbiota into mouse models led to decreased SCFA production, neutrophil activation, and Th1/Th17 responses.Recipient mice showed exacerbated experimental autoimmune uveitis (EAU) and experimental autoimmune encephalomyelitis (EAE). In Herpes Virus Simplex-1 (HSV-1) infected mice mimicking BD, administration of butyrateproducing bacteria improved symptoms and immune variables. The microbiome may thus be involved in BD through immunity regulation and epigenetic modifications.
Topics: Humans; Animals; Mice; Behcet Syndrome; Dysbiosis; Epigenesis, Genetic; Uveitis; Microbiota; Bacteria
PubMed: 37382445
DOI: 10.55563/clinexprheumatol/zbt4gx