Behçet's syndrome is a rare multisystem disorder. The occurrence in families although well recognised is uncommon. The mode of inheritance, however, has not been elucidated. Sixty-one cases of familial disease have been documented to date. There was only one previous report on familial Behçet's syndrome with gastrointestinal involvement. This paper reports two cases of Behçet's syndrome with gastrointestinal involvement in a Malaysian woman of Chinese ethnic origin and her child. The mother satisfied three major criteria (oral ulcers, genital ulcers and erythema nodosum) and one minor criterion (gastrointestinal involvement). Her son satisfied two major criteria (oral and genital ulcers) and three minor criteria (intestinal involvement, central nervous manifestation and a positive family history). The radiological and pathological features of the gastrointestinal lesions in both patients resembled those of Crohn's disease. The literature on Behçet's syndrome is reviewed.
Behçet's disease (BD) is a multifactorial systemic inflammatory disease of unknown aetiology characterised by several clinical manifestations including vascular involvements (i.e., both arterial and venous thrombosis). Antiphospholipid antibodies (aPLs)-including anticardiolipin (aCL), anti-β2-glycoprotein I (β2-GPI) antibodies and lupus anticoagulant (LA) are detected in systemic autoimmune diseases which contribute to thrombosis. The aim of this systematic review and meta-analysis was to evaluate the prevalence of aPLs in patients with BD as compared to controls. A protocol was registered in PROSPERO (Registration No. CRD42018088125) and a systematic literature search was conducted through PubMed, Web of Science, Embase, Scopus and ScienceDirect databases. Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated using random-effects model. Quality assessment was carried out by using the modified 9-star Newcastle-Ottawa Scale (NOS). Publication bias was evaluated via visualisation of contour- enhanced and trim and fill funnel plots along with Begg's and Egger's tests. We included ten case-control studies (a total of 999 participants from 380 BD patients and 619 controls) based on the inclusion criteria. The prevalence of aCL (OR: 12.10, 95% CI: 5.15-28.41, p<0.00001) and anti-β2-GPI antibodies (OR: 23.57, 95% CI: 1.31-423.63, p = 0.03) were statistically significant, however, the prevalence of LA was not significant (OR: 13.77, 95% CI: 0.65-293.59, p = 0.09). The results remained statistically significant from different sensitivity analyses which represented the robustness of this meta-analysis. According to the NOS, 50.0% of the studies were considered as of high methodological quality (low risk of bias). No significant publication bias was detected from contour-enhanced and trim and fill funnel plots or Begg's and Egger's tests. This meta-analysis established that there is a significantly high prevalence of aPLs (i.e., aCL and anti-β2-GPI antibodies) in patients with BD when compared to controls.
This report describes a case of Behcet's syndrome in which there was involvement of both the intracranial and systemic veins. The pathogenesis of Behcet's syndrome is discussed.
A young gentleman presented with a history of multiple pseudoaneurysms of the right carotid artery, left radial artery, right femoral artery as well as deep vein thrombosis involving the right femoral vein. A diagnosis of Behcet's disease was made on the basis of his history of recurrent oral and genital ulceration with characteristic eye lesion.
Despite the high prevalence of oral ulcers little is known about what causes them and how best they can be treated. Recurrent aphthous stomatitis (RAS) is a' chronic inflammatory disease characterized by painful recurring ulcerations of the oral mucosa. RAS can be clinically subdivided into four varieties viz - minor aphthous ulcer, major aphthous ulcer, herpetiform ulcers and Behcet's syndrome. A report on 134 patients with RAS is reported. Minor aphthous ulcer (63%) followed by major aphthous ulcer (29%) were the most frequent. A review of current research on RAS is summarized.
Study site: Department of Stomatology, Institute for Medical Research, Kuala Lumpur, Malaysia
BACKGROUND: We previously reported on a Swedish patient with Behçet's disease (BD) who was an ultra-rapid metaboliser of drugs catalysed by CYP2C9. Was this extreme metabolism caused by the disease?
AIM: This study aims to compare the genotype/phenotype of CYP2C9 in patients with BD and healthy subjects. As the occurrence of BD is high in Turkey, all subjects were recruited from this country.
METHODS: Genotyping of CYP2C9 was performed using standard PCR-RFLP and allele-specific PCR methods. Phenotyping of CYP2C9 was performed by administration of a 50-mg single oral dose of losartan and by calculating the urinary metabolic ratio (MR) of probe drug to its metabolite E-3174. Quantitation was performed by HPLC.
RESULTS: The frequency of CYP2C9*2 and *3 was not significantly different between the Behçet's disease patients (12.5 and 8.7%) and the healthy subjects (8.9 and 8.2%). The geometric mean losartan MR was higher in the 52 patients (1.75) than in the 96 healthy subjects (1.02) (p = 0.002; t-test). Within the genotypes *1/*1, there was a significant difference of MR between patients and healthy subjects (P = 0.006). All but three of the Behçet's disease patients were treated with colchicine. In nine subsequent patients, we found no significant effect of 2 weeks of treatment with colchicine on the CYP2C9 MR.
CONCLUSION: Contrary to expectation, the CYP2C9 activity was lower in Turkish BD patients compared to healthy subjects. As this seems not to be due to colchicine treatment, our hypothesis is that inflammation related to BD might have caused the down-regulation of the CYP2C9 activity due to immune cytokine reactions. The ultra-rapid metabolism of CYP2C9 substrate drugs in the Swedish patient was not due to her BD.