title: Clinical and laboratory features dependent on age at onset in Japanese systemic sclerosis.
abstract: OBJECTIVE
  To clarify those clinical characteristics dependent on patient age at onset of Japanese systemic sclerosis (SSc).
  
  
  METHODS
  A total of 329 SSc patients treated at Kanazawa University Hospital were enrolled in the study and subsequently categorized into four subgroups depending on age at SSc onset: childhood-onset, young-onset, normal age-onset, and late-onset. The clinical features at the first visit were compared between groups. Factors that correlated with age at onset were also analyzed.
  
  
  RESULTS
  The childhood-onset subgroup was characterized by a higher male:female ratio, higher anti-topoisomerase I antibody (Ab) and anti-U3 RNP Ab levels, a higher frequency of pitting scars, and a lower frequency of anticentromere Ab and interstitial lung disease (ILD). The young-onset patients had an increased frequency of anti-U1 RNP Ab and anti-U3 RNP Ab, overlap with other connective tissue diseases, digital ulcers, and pitting scars and a reduced frequency of anticentromere Ab. Patients with late-onset SSc showed a shorter disease duration and an increased frequency of anti-RNA polymerase Ab. Multiple regression analysis showed that anti-topoisomerase I Ab, anti-U1 RNP Ab, pitting scars, and long disease duration were associated with onset at a younger age, whereas anticentromere Ab and ILD were associated with onset at an older age.
  
  
  CONCLUSIONS
  The clinical phenotype of SSc in Japanese patients showed a tendency to be dependent on age at onset.
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 13-1 Takaramachi, Kanazawa, Ishikawa, 920-8641, Japan, minoruha@derma.m.kanazawa-u.ac.jp.
authors.forename: Minoru
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Yuri
authors.initials: Y
authors.lastname: Hatta
authors.forename: Takashi
authors.initials: T
authors.lastname: Matsushita
authors.forename: Yasuhito
authors.initials: Y
authors.lastname: Hamaguchi
authors.forename: Manabu
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000974:Antibodies, Antinuclear / Q000276:immunology*; D044466:Asian People; D001323:Autoantibodies / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D004264:DNA Topoisomerases, Type I / Q000276:immunology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007564:Japan; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000276:immunology
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; C538950:anticentromere antibody; D004264:DNA Topoisomerases, Type I; C528909:TOP1 protein, human