Le Guern V, Mahr A, Mouthon L, Jeanneret D, Carzon M, Guillevin L
Department of Internal Medicine, Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, Bobigny, France.
Rheumatology (Oxford). 2004 Sep;43(9):1129-37. doi: 10.1093/rheumatology/keh253. Epub 2004 Jun 22.
To assess the prevalence of systemic sclerosis (SSc) in a French multi-ethnic population and to examine ethnic differences.
This survey was conducted in Seine-Saint-Denis County, a suburb of Paris, home to 1,094,412 adults (>/=15 yr), among whom 26% are of non-European background with mainly northern and sub-Saharan African, Asian and Caribbean ancestries. The study period comprised the entire calendar year 2001. Patients were ascertained through four sources: public and private hospitals, general practitioners and community specialists, the French SSc patient support group, and the National Public Health Insurance System database. Only cases meeting either the 1980 ACR and/or LeRoy and Medsger's classification criteria were included and assigned to three clinical subsets: limited (normal skin) (l), limited cutaneous (lc) or diffuse cutaneous (dc) SSc. Capture-recapture (CR) analyses using log-linear modelling were performed to correct for incomplete case finding.
We retained a total of 119 patients with SSc, including 15 extrapolated from inaccessible files. CR analysis estimated that 54.2 additional cases were missed by all the sources. The overall SSc prevalence (per million adults) was 158.3 (95% confidence interval, 129-187); those of lSSc, lcSSc and dcSSc were, respectively, 32.3 (16-48), 83.1 (66-101) and 42.9 (25-60); and respective values for Europeans and non-Europeans were 140.2 (112-170) and 210.8 (128-293).
Regarding the heterogeneity of previously published estimates, this population-based survey using CR analysis might contribute to obtaining a better appraisal of SSc prevalence. Despite overlapping confidence intervals, the higher prevalence observed for non-Europeans could support potential influences of ethnic origin on the pathogenesis of SSc.
评估法国多民族人群中系统性硬化症（SSc）的患病率，并研究种族差异。
本调查在巴黎郊区塞纳 - 圣但尼省进行，该地区有1,094,412名成年人（≥15岁），其中26% 具有非欧洲背景，主要为北非、撒哈拉以南非洲、亚洲和加勒比海地区血统。研究期为2001年全年。通过四个来源确定患者：公立和私立医院、全科医生和社区专科医生、法国SSc患者支持小组以及国家公共医疗保险系统数据库。仅纳入符合1980年美国风湿病学会（ACR）和/或勒罗伊（LeRoy）及梅兹格（Medsger）分类标准的病例，并分为三个临床亚组：局限性（正常皮肤）（l）、局限性皮肤型（lc）或弥漫性皮肤型（dc）SSc。使用对数线性模型进行捕获 - 再捕获（CR）分析以校正病例发现不完整的情况。
我们共纳入119例SSc患者，包括从无法获取的档案中推断出的15例。CR分析估计所有来源共遗漏了54.2例病例。SSc总体患病率（每百万成年人）为158.3（95%置信区间，129 - 187）；局限性SSc（lSSc）、局限性皮肤型SSc（lcSSc）和弥漫性皮肤型SSc（dcSSc）的患病率分别为32.3（16 - 48）、83.1（66 - 101）和42.9（25 - 60）；欧洲人和非欧洲人的相应患病率分别为140.2（112 - 170）和210.8（128 - 293）。
鉴于先前发表的估计存在异质性，这项基于人群的采用CR分析的调查可能有助于更好地评估SSc患病率。尽管置信区间有重叠，但非欧洲人较高的患病率可能支持种族起源对SSc发病机制的潜在影响。
