title: Brief Report: Spatial Heterogeneity of Systemic Sclerosis in France: High Prevalence in the Northeast Region.
abstract: Alsace is a region in eastern France with a population of ∼2 million. All residents have high access to health care and an accredited referral center for SSc. Seeking care outside of this region is difficult because of the peculiar geography. The aim of this study was to assess the prevalence and spatial variation of systemic sclerosis (SSc) in eastern France.
  
  
  
  Data for SSc patients were obtained from 3 sources (all general practitioners and community specialists, capillaroscopy centers, and all public and private hospital records) and were used to estimate the prevalence of SSc. Surviving patients who resided in Alsace on January 1, 2008 and fulfilled the American College of Rheumatology and/or the LeRoy and Medsger criteria were included in this study. The clinical characteristics of the patients were also assessed. Potentially incomplete case ascertainment was corrected by capture-recapture analyses. Geographic disparities were assessed by spatial cluster analysis and by comparing our results with those for other geographic areas in the world for which data derived using similar methodology were available.
  
  
  
  The review of 499 potential cases identified a total of 244 SSc patients. A trend toward a west-to-east gradient was observed but did not reach statistical significance. According to log-linear modeling, an estimated 83.87 additional cases were missed. Thus, the SSc prevalence was 228.42 cases per million adult inhabitants of Alsace (95% confidence interval 203.70-253.14); this prevalence was significantly higher than that in 2 other regions of France and comparable with the reported prevalence in Detroit, Michigan.
  
  
  
  The stringent methodology used in the current study is very likely to provide an accurate estimation of the prevalence of SSc. Design similarity with 3 other surveys extends the scope of the results by identifying geographic disparities that were previously indistinguishable due to methodologic differences.
authors.affiliation: Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Meyer
authors.affiliation: Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Chifflot
authors.affiliation: Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Chatelus
authors.affiliation: Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Kleinmann
authors.affiliation: Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Ronde-Ousteau
authors.affiliation: Université de Strasbourg, Strasbourg, France.
authors.forename: Delphine
authors.initials: D
authors.lastname: Klein
authors.affiliation: Université de Strasbourg, Strasbourg, France.
authors.forename: Jérémy
authors.initials: J
authors.lastname: Jégu
authors.affiliation: Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Bernard
authors.initials: B
authors.lastname: Geny
authors.affiliation: Centre de Compétence des Maladies Pulmonaires Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Sandrine
authors.initials: S
authors.lastname: Hirshi
authors.affiliation: Centre de Compétence des Maladies Pulmonaires Rares and Centre de Compétences des Hypertensions Pulmonaires Sévères, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Matthieu
authors.initials: M
authors.lastname: Canuet
authors.affiliation: Centre de Compétence des Maladies Auto-immunes Rares, Centre Hospitalier, Colmar, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Blaison
authors.affiliation: Centre Hospitalier, Mulhouse, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Kieffer
authors.affiliation: Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Dan
authors.initials: D
authors.lastname: Lipsker
authors.affiliation: Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Martin
authors.affiliation: Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Erik
authors.initials: E
authors.lastname: Sauleau
authors.affiliation: Université de Strasbourg, Strasbourg, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Velten
authors.affiliation: Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Sibilia
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016000:Cluster Analysis; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012595:Scleroderma, Systemic / Q000453:epidemiology*; D055815:Young Adult