Clinical and prognostic associations based on serum antinuclear antibodies in Japanese patients with systemic sclerosis

Arthritis Rheum. 1994 Jan;37(1):75-83. doi: 10.1002/art.1780370111.


Objective: To clarify the clinical features and prognosis of systemic sclerosis (SSc) based on serum antinuclear antibodies (ANA).

Methods: We studied 275 consecutive Japanese patients newly diagnosed as having SSc, who were first evaluated during the period 1971-1990. Eight SSc-related ANA were identified using indirect immunofluorescence, double immunodiffusion, or immunoprecipitation assays. Clinical and prognostic features were retrospectively analyzed in patient groups, categorized by their serum ANA.

Results: Cumulative survival rates at 10 years after diagnosis of SSc were 93% in patients with anticentromere antibodies (ACA), 72% in those with anti-U1 RNP, 66% in those with anti-DNA topoisomerase I (anti-topo I), and 30% in those with anti-RNA polymerases I, II, and III (anti-RNAP). Major organ involvement linked to cause of death included biliary cirrhosis in patients with ACA, isolated pulmonary arterial hypertension and cerebral hemorrhage in those with anti-U1 RNP, pulmonary interstitial fibrosis in those with anti-topo I, and cardiac and renal involvement in those with anti-RNAP.

Conclusion: Determinations of serum ANA in SSc patients are useful in predicting organ involvement and long-term outcome.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antibodies / analysis
  • Antibodies, Antinuclear / blood*
  • Cause of Death
  • DNA Topoisomerases, Type I / immunology
  • Female
  • Humans
  • Japan / epidemiology
  • Male
  • Middle Aged
  • Retrospective Studies
  • Ribonucleoprotein, U1 Small Nuclear / immunology
  • Scleroderma, Systemic / blood*
  • Scleroderma, Systemic / classification
  • Scleroderma, Systemic / epidemiology*
  • Survival Rate


  • Antibodies
  • Antibodies, Antinuclear
  • Ribonucleoprotein, U1 Small Nuclear
  • DNA Topoisomerases, Type I