Internal and emergency medicine
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Behçet's syndrome (BS) is a complex vasculitis, characterised by peculiar histological, pathogenetic and clinical features. Superficial venous thrombosis (SVT) and deep vein thrombosis (DVT) are the most frequent vascular involvements, affecting altogether 15-40% of BS patients. Atypical thrombosis is also an important clinical feature of BS, involving the vascular districts of the inferior and superior vena cava, suprahepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses and right ventricle. ⋯ More recently, the anti-TNF-α drugs have also been reported as a valid alternative for the treatment also of venous events, especially DVT. An exception to the use of anticoagulant in BS could be represented by cerebral veins thrombosis. In this review, we will depict the main characteristics of the vascular involvement in BS, briefly describing histological and pathogenetic features, while focusing on the clinical and therapeutical approaches of the vascular manifestations of BS.
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Pathergy phenomenon is a non-specific tissue hyperreactivity reaction due to trauma and is a minor diagnostic criterion of Behcet's disease. In this study, 100 patients with a suspicion of Behcet's disease who were referred to Cerrahpasa Medical Faculty Dermatology department between 01.11.2014 and 31.01.2015 are included. Skin pathergy tests were applied to all the patients and results were evaluated by two dermatologists separately at 48th hour, each with naked eye and with dermatoscopy. ⋯ However, naked eye results of doctor number 1 and 2 for the same patients were significantly different from each other (p 0.0372) and with dermatoscopy examination this difference was eliminated (p > 0.05). This study revealed that naked eye evaluation of pathergy test results can yield different results among different interpreters. Use of dermatoscopy during the evaluation process decreases interobserver variation and subjectivity of the test.
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The Endoplasmic reticulum aminopeptidase protein 1 (ERAP1) trims N-terminal amino acids from epitope precursors for Major Histocompatibility Complex class I presentation. Genome-wide association studies demonstrated that ERAP1 gene single nucleotide polymorphisms (SNPs) are associated with Behçet's syndrome (BS). This study was conducted on the two most consistently BS-associated ERAP1 polymorphisms, rs17482078 (NG_027839.1:g.35983G>A) and rs27044 (NG_027839.1:g.35997C>G) to analyse their distribution in 55 Italian BS patients and 65 ethnically matched controls (healthy controls, HC) and to test their association with BS risk. ⋯ No association was found between HLA-B*51 and both ERAP1 SNPs. Although preliminary, our data show a stronger association of rs17482078 with BS compared to rs27044 by means of case-control genetic analysis and bioinformatics prediction of protein structure change. A larger series of patients and controls is required to confirm our preliminary findings.
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Environmental and other triggering factors are suggested to cause the onset and the clinical relapses of Behçet's syndrome (BS), a multi-systemic inflammatory disorder. In this review, environmental factors are discussed according to their interactions with etiopathogenesis, immune response and disease activity. Stress is a common self-triggering factor for most BS patients. ⋯ Infections are associated with BS, and microbial stimuli can activate inflammation in mucosal surfaces with increased Th1/Th17 responses. Fecal and oral microbiome patterns change in diversity and composition in BS. Better oral hygiene applications and anti-microbial interventions might be helpful to suppress oral ulcers in BS.