Chamberlain M A
Ann Rheum Dis. 1978 Oct;37(5):459-65. doi: 10.1136/ard.37.5.459.
The first-degree family members of patients with Behcet's syndrome were investigated to determine (1) whether there was a familial aggregation of Behcet's syndrome or of its component features, (2) whether there was an increased incidence of sacro-iliitis in these families, and (3) whether a link exists between Behcet's syndrome and spondarthritides in first-degree relatives. Of the 226 relatives and patients seen, 21 patients with definite Behcet's syndrome and their 71 relatives were analysed in some detail. Orogenital ulceration was found in 2 mothers of probands; 1 patient had psoriasis and Behcet's but neither disease featured in the pedigree. Another family showed numerous manifestations of psoriasis and psoriatic arthritis, 1 member having severe sacro-iliitis but the proband had Behcet's syndrome only. Family trees including HLA pedigrees, where known, are presented. Spouses suffered few of the complaints found in patients, and to a less extent in relatives, but showed a high incidence of backache, perhaps of psychogenic origen.
对白塞氏综合征患者的一级亲属进行了调查,以确定:(1)白塞氏综合征或其组成特征是否存在家族聚集性;(2)这些家族中骶髂关节炎的发病率是否增加;(3)一级亲属中白塞氏综合征与脊柱关节炎之间是否存在联系。在所见的226名亲属和患者中,对21例确诊为白塞氏综合征的患者及其71名亲属进行了较为详细的分析。先证者的2名母亲出现口生殖器溃疡;1例患者患有银屑病和白塞氏病,但家系中均无这两种疾病的特征。另一个家族表现出许多银屑病和银屑病关节炎的症状,1名成员患有严重的骶髂关节炎,但先证者仅患有白塞氏综合征。文中列出了包括已知HLA家系的家族谱系。配偶很少出现患者中发现的症状,亲属出现的症状程度较轻,但背痛的发生率较高,可能源于心理因素。