Møller P, Vinje O, Kåss E, Berg K
Clin Genet. 1982 Oct;22(4):151-9. doi: 10.1111/j.1399-0004.1982.tb01428.x.
One hundred and twenty-two consecutive patients hospitalized for ankylosing spondylitis (AS) were reexamined. The frequency of clinical signs and results of tests for associations are presented. Psoriasis was associated with a distal pattern of peripheral arthropathy. Spinal rigidity was predominantly seen in males. Males with phalangeal arthropathy exhibited preserved spinal mobility. This was the case also when HLA B27 positives and patients who did not have psoriasis were considered separately. HLA B27 positive patients in this group had frequently experienced acute anterior uveitis. It seems possible that the disease in such males is the result of combined predisposition to ankylosing spondylitis and psoriatic arthropathy. Hip arthropathy was frequently present in males with spinal rigidity. The associations observed confirm that AS is a heterogenous group of diseases. The term "syndrome" may be suitable for such a heterogenous group, and we prefer the term "Bechterew's syndrome" as the name of this group. When these new findings are added to the previous observations that acute anterior uveitis probably is a clinical, sex-influenced characteristic of HLA B27 positive Bechterew's syndrome, that HLA B27 negative patients with Bechterew's syndrome frequently had psoriasis and were HLA B13 and B17 negative, and that psoriasis was frequent in HLA B27 positive patients as well, we tentatively conclude that different and interacting genetic mechanisms may be involved in the etiology of Bechterew's syndrome.
对122例因强直性脊柱炎(AS)住院的连续患者进行了复查。列出了临床体征的频率以及相关检查结果。银屑病与外周关节病的远端型相关。脊柱僵硬在男性中更为常见。患有指关节病的男性脊柱活动度保留。在分别考虑HLA B27阳性患者和无银屑病患者时也是如此。该组中HLA B27阳性患者经常发生急性前葡萄膜炎。似乎这类男性的疾病是强直性脊柱炎和银屑病关节炎合并易感性的结果。髋关节病在有脊柱僵硬的男性中很常见。观察到的关联证实AS是一组异质性疾病。“综合征”一词可能适用于这样一个异质性群体,我们更倾向于用“别赫捷列夫综合征”来命名这个群体。当这些新发现与先前的观察结果相结合时,即急性前葡萄膜炎可能是HLA B27阳性别赫捷列夫综合征的一种临床、受性别影响的特征,HLA B27阴性的别赫捷列夫综合征患者经常患有银屑病且HLA B13和B17阴性,以及银屑病在HLA B27阳性患者中也很常见,我们初步得出结论,不同且相互作用的遗传机制可能参与了别赫捷列夫综合征的病因。