Villanueva J L, Gonzalez-Dominguez J, Gonzalez-Fernandez R, Prada J L, Peña J, Solana R
Service of Internal Medicine, Hospital Regional Universitario Reina Sofia, Cordoba, Spain.
Ann Rheum Dis. 1993 Feb;52(2):155-7. doi: 10.1136/ard.52.2.155.
Behçet's disease is a multisystemic disease affecting most organs. Although a tendency towards an association with a certain genetic type and with HLA-B51 is suspected, the incidence of several siblings with Behçet's disease in a single family is rare. A family, in which three sisters were affected with Behçet's disease, uveitis being the most severe manifestation, was studied. In this family all siblings were B51 positive. Only the female siblings, however, with a positive identical HLA phenotype: A2, A11, B51, B44, Cw6, Cw5, DR4, DRw13, DRw53, DRw52, DQw7, DQw6, developed the disease symptoms, whereas none of the male siblings was affected.
白塞病是一种累及多数器官的多系统疾病。尽管怀疑其有与特定基因类型及人类白细胞抗原B51(HLA - B51)相关联的倾向,但单个家庭中有数名兄弟姐妹患白塞病的情况较为罕见。我们研究了一个家庭,其中三姐妹患有白塞病,葡萄膜炎是最严重的表现形式。在这个家庭中,所有兄弟姐妹的HLA - B51均呈阳性。然而,只有女性兄弟姐妹具有相同的HLA阳性表型:A2、A11、B51、B44、Cw6、Cw5、DR4、DRw13、DRw53、DRw52、DQw7、DQw6,并出现了疾病症状,而男性兄弟姐妹均未患病。