title: Associated autoimmune diseases in systemic sclerosis define a subset of patients with milder disease: results from 2 large cohorts of European Caucasian patients.
abstract: OBJECTIVE
  To assess the prevalence and potential associations with the systemic sclerosis (SSc) phenotype of additional autoimmune diseases (AID).
  
  
  METHODS
  A multicenter study was performed in France and Italy to recruit consecutive European Caucasian patients with SSc systematically assessed for the coexistence of predefined AID known to occur with connective tissue diseases.
  
  
  RESULTS
  We recruited 585 French and 547 Italian patients with SSc. Specific AID were found in 114/585 (19%) French and 179/547 (33%) Italians with SSc (p < 0.0001). Sjögren's syndrome and thyroiditis were the predominant AID in both cohorts (12% for Sjögren's syndrome and 6% for thyroiditis in the combined populations). The frequency of myositis, primary biliary cirrhosis, rheumatoid arthritis, and systemic lupus erythematosus was low (< 4%) and similar in both cohorts. The coexistence of at least 1 of the AID in the whole cohort was associated in multivariate analysis with the limited cutaneous subtype, the presence of antinuclear antibodies, and a lower prevalence of digital ulcers.
  
  
  CONCLUSIONS
  Our study shows that 21% of this large series of European Caucasian patients with SSc have developed at least 1 AID. This latter condition identified a subset of patients with milder disease. Thus, associations of AID and autoimmune background in SSc have to be considered for further therapeutic and biological investigations in SSc.
authors.affiliation: Rheumatology A Department, Paris Descartes University, Cochin Hospital, Paris, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Avouac
authors.forename: Paolo
authors.initials: P
authors.lastname: Airò
authors.forename: Philippe
authors.initials: P
authors.lastname: Dieude
authors.forename: Paola
authors.initials: P
authors.lastname: Caramaschi
authors.forename: Kiet
authors.initials: K
authors.lastname: Tiev
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Diot
authors.forename: Jean
authors.initials: J
authors.lastname: Sibilia
authors.forename: Susanna
authors.initials: S
authors.lastname: Cappelli
authors.forename: Brigitte
authors.initials: B
authors.lastname: Granel
authors.forename: Alessandra
authors.initials: A
authors.lastname: Vacca
authors.forename: Julien
authors.initials: J
authors.lastname: Wipff
authors.forename: Olivier
authors.initials: O
authors.lastname: Meyer
authors.forename: André
authors.initials: A
authors.lastname: Kahan
authors.forename: Marco
authors.initials: M
authors.lastname: Matucci-Cerinic
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood; D001327:Autoimmune Diseases / Q000453:epidemiology*; D015331:Cohort Studies; D005602:France / Q000453:epidemiology; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008875:Middle Aged; D015999:Multivariate Analysis; D010641:Phenotype; D015995:Prevalence; D012595:Scleroderma, Systemic / Q000150:complications* / Q000208:ethnology* / Q000276:immunology; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000453:epidemiology; D013966:Thyroiditis / Q000453:epidemiology; D044465:White People / Q000208:ethnology*
chemical_list: D000974:Antibodies, Antinuclear