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

Abstract

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.