title: Ethnicity and association with disease manifestations and mortality in Behçet's disease.
abstract: BACKGROUND
  Behçet's disease (BD) significantly increases morbidity and mortality. BD mainly affects young adults with a peculiar geographical distribution. It has been suggested that BD varies in its phenotypic expression in different ethnic groups.
  
  
  METHODS
  We investigated potential ethnicity-related differences relative to phenotype and prognosis of BD patients in a French multiethnic country. We included 769 consecutive patients fulfilling the international criteria of classification for BD, in the 3 largest ethnic groups of our cohort [European (n = 369), North African (n = 350) and sub Saharan African (n = 50)]. Factors that affect prognosis were assessed by multivariate analysis.
  
  
  RESULTS
  535 (69.6%) patients were male and the median (IQR) age at diagnosis was of 30.9 (24.9-37.2) years. Sub Saharan African BD patients had a higher frequency of CNS involvement (48% vs 32.3% vs 29.5%, p = 0 .035), a higher rate of death (12% vs 6% vs 3.5%, p = 0.029) and a lower frequency of HLA B51 allele (29.4% vs 49.2% vs 55.8%, p = 0.009) compared to those from North Africa and Europe, respectively. Multivariate analysis showed that male gender (HR: 5.01, CI: 1.51-16.65), cardiovascular involvement (HR: 2.24, CI: 1.15-4.36), and sub Saharan African origin (HR 2.62 (0.98-6.97) were independently associated with mortality. The 15-year mortality rate was of 19%, 9% and 6% in sub Saharan African, North African and European BD patients, respectively (p = 0.015).
  
  
  CONCLUSIONS
  We reported ethnicity-related differences with respect to phenotype of BD. Sub Saharan Africans patients exhibited a worse prognosis.
authors.forename: Lea
authors.initials: L
authors.lastname: Savey
authors.forename: Mathieu
authors.initials: M
authors.lastname: Resche-Rigon
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.forename: Cloé
authors.initials: C
authors.lastname: Comarmond
authors.forename: Jean Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.affiliation: Department of Internal medicine and Clinical Immunology, Centre de référence des maladies autoimmunes et systémiques rares, AP-HP, Hôpital Pitié-Salpétrière, Paris, France. david.saadoun@psl.aphp.fr.
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000208:ethnology* / Q000401:mortality / Q000503:physiopathology*; D044383:Black People; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D044465:White People