title: Characteristics of patients with juvenile onset systemic sclerosis in an adult single-center cohort.
abstract: OBJECTIVE
  Systemic sclerosis (SSc) is a rare connective tissue disease in childhood. We compared the characteristics of adult patients with juvenile-onset SSc (jSSc) from a single-center cohort to an adult-onset group.
  
  
  METHODS
  Patients with disease onset before the age of 17 years were included in the jSSc cohort, while subjects with SSc onset after age 17 formed the adult-onset cohort.
  
  
  RESULTS
  We identified 52 adult subjects with jSSc and compared them to 954 patients with adult-onset SSc. The mean ± SD age at disease onset of the patients with jSSc was 14 ± 2 years, 39 (75%) of them were women, and 24 (46%) had the diffuse cutaneous subset of SSc (dcSSc). There were no differences between the 2 cohorts in terms of sex and disease subset. Overlaps were significantly more frequent among the jSSc cohort (37%) compared to the adult-onset group (18%; p = 0.002). Autoantibody analysis demonstrated significantly more antitopoisomerase I antibody-positive subjects (33% vs 20%; p = 0.034) and significantly fewer anticentromere antibody-positive subjects (2% vs 25%; p < 0.001) in the jSSc cohort. Compared to the adult-onset group at 10 years from disease onset, survival was significantly higher among the subjects with jSSc (98% vs 75%; p = 0.001), pulmonary arterial hypertension had a significantly lower incidence (2% vs 14%; p = 0.032), and there was no difference in terms of pulmonary fibrosis (22% vs 21%) and cardiac scleroderma (3% vs 2%) between the 2 groups.
  
  
  CONCLUSIONS
  The high survival rates and lower proportion of dcSSc in the adult jSSc cohort may represent a survival bias.
authors.affiliation: Hamburger Zentrum für Kinder- und Jugendrheumatologie, Kompetenz-Zentrum für Sklerodermie und autoimmune Uveitis im Kindesalter, Am Klinikum Eilbek, Dehnhaide 120, D-22081 Hamburg, Germany. sprechstunde@kinderrheumatologie.de
authors.forename: Ivan
authors.initials: I
authors.lastname: Foeldvari
authors.forename: Svetlana I
authors.initials: SI
authors.lastname: Nihtyanova
authors.forename: Angela
authors.initials: A
authors.lastname: Wierk
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Denton
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000974:Antibodies, Antinuclear / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D004264:DNA Topoisomerases, Type I / Q000276:immunology*; D005260:Female; D006801:Humans; D008297:Male; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000276:immunology* / Q000401:mortality; D015996:Survival Rate
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; C538950:anticentromere antibody; D004264:DNA Topoisomerases, Type I