Nancy University Hospital-Clinical Epidemiology and Evaluation, Nancy, France.
Semin Arthritis Rheum. 2013 Apr;42(5):530-8. doi: 10.1016/j.semarthrit.2012.10.001. Epub 2013 Jan 2.
To assess the prevalence of systemic sclerosis (SSc) in the Lorraine region, France.
Data from three sources - general practitioners and community and hospital specialists, medical records departments, and regional and national laboratories-and a capture-recapture method with log-linear models were used to estimate SSc prevalence in the region. Double recording was checked, and reported cases were validated after a review of medical records.
We identified 560 records of suspected SSc cases corresponding to 327 unique suspected SSc cases existing on June 30, 2006, in Lorraine. On the basis of the 193 validated cases (22 [11.4%] with diffuse disease, 136 [70.5%] with limited disease, 31 [16.1%] with limited involvement and 4 unknown), the observed overall crude prevalence of SSc was 105.4 cases per million adult inhabitants (95% confidence interval [CI]: 91.0; 121.4). With the capture-recapture method, the estimated number of SSc cases was 233 (95% CI: 217.3; 260.0), so an estimated 40 cases were not identified by the three sources. The estimated overall prevalence was 132.2 cases per million adult inhabitants (95% CI: 115.8; 154.0).
Our study provides the first estimate of SSc prevalence in the Lorraine region. The capture-recapture method allowed us to estimate an additional 21% of unobserved cases and is a good alternative to the community-based study design for estimating the prevalence of rare diseases.
评估法国洛林地区系统性硬化症（SSc）的患病率。
使用三种来源的数据——全科医生和社区及医院专家、病历部门以及地区和国家实验室，以及对数线性模型的捕获-再捕获方法，来估计该地区的 SSc 患病率。我们检查了双重记录，并在审查病历后对报告的病例进行了验证。
我们共发现了 560 份疑似 SSc 病例的记录，对应于 2006 年 6 月 30 日洛林地区存在的 327 例独特的疑似 SSc 病例。基于 193 例经过验证的病例（22 例弥漫性疾病[11.4%]，136 例局限性疾病[70.5%]，31 例局限性受累[16.1%]和 4 例未知），观察到的 SSc 总粗患病率为每百万成年居民 105.4 例（95%置信区间[CI]：91.0；121.4）。使用捕获-再捕获方法，估计的 SSc 病例数为 233 例（95%CI：217.3；260.0），因此有 40 例估计病例未被三种来源识别。估计的总患病率为每百万成年居民 132.2 例（95%CI：115.8；154.0）。
我们的研究提供了洛林地区 SSc 患病率的首次估计。捕获-再捕获方法使我们能够估计另外 21%的未观察到的病例，这是估计罕见疾病患病率的社区为基础的研究设计的一个很好的替代方法。
