title: Investigation of prognostic factors for skin sclerosis and lung function in Japanese patients with early systemic sclerosis: a multicentre prospective observational study.
abstract: OBJECTIVE
  To clarify the clinical course of SSc in Japanese patients with early-onset disease. It is well known that ethnic variations exist in the clinical features and severity of SSc. However, neither the clinical course nor prognostic factors have been thoroughly investigated in the Japanese population.
  
  
  METHODS
  Ninety-three Japanese patients of early-onset SSc (disease duration: <3 years) with diffuse skin sclerosis and/or interstitial lung disease were registered in a multi-centre observational study. All patients had a physical examination with laboratory tests at their first visit and at each of the three subsequent years. Factors that could predict the severity of skin sclerosis and lung involvement were examined statistically by multiple regression analysis.
  
  
  RESULTS
  Two patients died from SSc-related myocardial involvement and four patients died from other complications during the 3-year study. Among various clinical data assessed, the initial modified Rodnan total skin thickness score (MRSS) and maximal oral aperture were associated positively and negatively with MRSS at Year 3, respectively. Additionally, initial ESR tended to be associated with final MRSS. Pulmonary vital capacity (VC) in the third year was significantly associated with initial %VC. Furthermore, patients with anti-topo I antibody tended to show reduced %VC at Year 3.
  
  
  CONCLUSIONS
  Several possible prognostic factors for skin sclerosis and lung function were detected in Japanese patients with early SSc. Further longitudinal studies of larger populations will be needed to confirm these findings.
authors.affiliation: Department of Dermatology, Kanazawa University Graduate School of Medical Science, 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: Yoshihide
authors.initials: Y
authors.lastname: Asano
authors.forename: Hirahito
authors.initials: H
authors.lastname: Endo
authors.forename: Manabu
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Daisuke
authors.initials: D
authors.lastname: Goto
authors.forename: Hironobu
authors.initials: H
authors.lastname: Ihn
authors.forename: Katsumi
authors.initials: K
authors.lastname: Inoue
authors.forename: Osamu
authors.initials: O
authors.lastname: Ishikawa
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Kawaguchi
authors.forename: Masataka
authors.initials: M
authors.lastname: Kuwana
authors.forename: Yoshinao
authors.initials: Y
authors.lastname: Muro
authors.forename: Fumihide
authors.initials: F
authors.lastname: Ogawa
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Sasaki
authors.forename: Hiroki
authors.initials: H
authors.lastname: Takahashi
authors.forename: Sumiaki
authors.initials: S
authors.lastname: Tanaka
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001799:Blood Sedimentation; D002648:Child; D002675:Child, Preschool; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D018450:Disease Progression; D042241:Early Diagnosis; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D007564:Japan; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009055:Mouth / Q000473:pathology; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D012867:Skin / Q000473:pathology; D014797:Vital Capacity / Q000502:physiology; D055815:Young Adult
chemical_list: D000974:Antibodies, Antinuclear; D015415:Biomarkers; D004264:DNA Topoisomerases, Type I