Public Health Wales, Capital Quarter 2, Tyndall Street, Cardiff, CF10 4BZ, UK.
Congenital Anomaly Register and Information Service, Public Health Wales, Capital Quarter 2, Tyndall Street, Cardiff, CF10 4BZ, UK.
Orphanet J Rare Dis. 2022 Sep 6;17(1):347. doi: 10.1186/s13023-022-02505-4.
Behçet's disease is a rare, chronic, incurable, multisystemic disease. It causes significant morbidity, with patients experiencing symptoms including mucous membrane ulcers, and joint pain and swelling. It is an important cause of avoidable blindness due to ocular involvement. The aetiology is unknown. The aims were to identify population prevalence of Behçet's disease in Wales in comparison to other endemic and non-endemic regions, and provide an epidemiological profile of a case series of adult patients. This is the first analysis of data from the Adult Rare Diseases Surveillance Registry for Wales, established in 2020 as part of the COVID-19 pandemic response.
Between 1995 and 2020, 347 adults and 5 children were recorded in Wales with a diagnosis of Behçet's disease. Population prevalence was calculated as 11.1 per 100,000 population. Of the adult cases, 76.9% were female, and 6.6% died before the end of the study period. When comparing genders, there were no statistically significant differences in age at diagnosis, mortality or socioeconomic status. There was no evidence that the age at which cases were diagnosed had changed over time. Survival analyses showed no significant differences in durations of survival between genders or individuals residing in different WIMD 2019 quintiles. Age at diagnosis was the only factor significantly and independently associated with poorer durations of survival (p < 0.001).
贝切特病是一种罕见的、慢性的、无法治愈的、多系统疾病。它会导致严重的发病，患者会出现黏膜溃疡、关节疼痛和肿胀等症状。由于眼部受累，它是可避免失明的一个重要原因。病因不明。目的是比较威尔士与其他地方性和非地方性地区贝切特病的人群患病率，并提供一系列成年患者的流行病学特征。这是对威尔士 2020 年作为 COVID-19 大流行应对措施的一部分设立的成人罕见疾病监测登记处数据的首次分析。
1995 年至 2020 年间，威尔士记录了 347 名成年患者和 5 名儿童患有贝切特病。人群患病率计算为每 10 万人中有 11.1 人。在成年患者中，76.9%为女性，6.6%在研究结束前死亡。在比较性别时，诊断时的年龄、死亡率或社会经济地位没有统计学上的显著差异。没有证据表明病例诊断的年龄随时间发生了变化。生存分析显示，性别或居住在不同 WIMD 2019 五分位数的个体之间的生存时间没有显著差异。诊断时的年龄是唯一与生存时间较差显著且独立相关的因素（p<0.001）。
