Behçet's disease: Clinical and demographic associations


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AIM: To comparatively study the clinical manifestations, sexual and HLA-B51 associations in patients with Behçet's disease (BD) in two ethnic groups/MATERIAL AND METHODS: The authors examined 143 patients with the valid diagnosis of BD who were divided into 2 groups: 1) 85 patients, the dwellers of Dagestan (a multiethnic cohort), 63 men and 22 women (mean age 29±7.4 years); 2) 58 Russian men and women (mean age 33±11.7 years)/RESULTS: Two major criteria for BD, such as aphthous stomatitis and external genital ulcers, were found with the same frequency. Panuveitis and angiitis of the retina were diagnosed more frequently in the Dagestani population with BD than in the Russians. Out of the minor criteria for BD, the incidence of lower limb deep venous thrombosis was 23% for the Dagestanis versus 3% for the Russians. Arterial thromboses and pulmonary artery aneurysms became causes of death in 4 in 5 men aged 19-23 years from their Dagestani ancestry. HLA B51 (B marker) was found in the dwellers of Dagestan: in 70% of the men and 40% of the women who had BD/CONCLUSION: BD runs a more severe course in male patients and is characterized by severe eye diseases and the systematic pattern of the process at young age. Gender-specific and genetic aspects call for further comparative investigations on large ethnic patient cohorts of other ancestries.

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Болезнь Бехчета: клинико-демографические ассоциации. - Резюме. Цель исследования. Сравнительное изучение клинических проявлений, половых и HLA-B51-ассоциаций у больных болезнью Бехчета (ББ) в двух этнических группах. Материалы и методы. Обследовали 143 больных с достоверным диагнозом ББ, которых распределили на 2 группы: в 1-ю группу объединили 85 пациентов - жителей Дагестана (мультиэтническая когорта), 63 мужчин и 22 женщины, средний возраст 29±7,4 года; 2-я группа состояла из 58 русских мужчин и женщин, средний возраст 33±11,7 года. Результаты. В сравниваемых группах с одинаковой частотой встречались 2 больших критерия ББ - афтозный стоматит и язвы наружных половых органов. У дагестанской популяции с ББ достоверно чаще, чем у русских, диагностировались панувеит и ангиит сетчатки. Из "малых" критериев ББ частота развития тромбозов глубоких вен нижних конечностей у дагестанцев составила 23% против 3% у русских. Тромбозы артериальных сосудов и аневризмы легочной артерии стали причиной летальных исходов у 4 из 5 мужчин дагестанского происхождения в возрасте 19-23 лет. HLA-B51 (маркер ББ) выявлен у жителей Дагестана: у 70% мужчин и у 40% женщин с ББ. Заключение. ББ у пациентов мужского пола протекает тяжелее, характеризуется в молодом возрасте тяжелой патологией глаз и системностью процесса. Половые и генетические аспекты нуждаются в дальнейших сравнительных исследованиях на больших этнических когортах у больных других происхождений.
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