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A high prevalence of Behçet’s disease (BD) among familial M edite rranean fever (FMF) pat ients has been described recently and a weak association of BD and certain MEFV gene mu t at ions, o ri gi nal ly linked to FMF, has been reported in an ethnically mixed population from France. We fu rther inve sti gated the presence of MEFV mutations in BD patients from Turkey, a country with a high prevalence of both disorders. The frequencies of thre e FMF-re l ated MEFV mu t ations (M694V, M680I and V726A) were investigated in BD patients (n= 57) by molecular genetic studies using a poly me ra se chain reaction with the ARMS method. All patients fulfilled the International Study Group Criteria for the diagnosis of BD and patients with FMF-like symptoms or a chronic inflammatory disease were excluded. Fifteen BD patients we re found to carry one single MEFV mutation (26%), compared to 9.1% in the control group (p= 0.003, OR: 3.5, 95% CI: 1.6-7.6). Among 20 BD pat ients with vascular invo l ve ment, 11 (55%) had MEFV mutations compared to 4 p atients (11%) in the non-va scular group (p= 0.001, OR: 10, 95% CI: 2.5-39.3). M694V was the dominant mutation in our study group (11 out of 15 patients with mutated alleles). Six out of 7 female patients with vascular involvement carried MEFV mutations in contrast to 5 out of 13 male patients (85.7% ve rsus 38.4%, p= 0. 0 7, OR: 0.1, 95% CI: 0.009-1.14). No association with other clinical manifestations was observed.