CLINICAL AND PROGNOSTIC ASSOCIATIONS BASED ON SERUM ANTINUCLEAR ANTIBODIES IN JAPANESE PATIENTS WITH SYSTEMIC-SCLEROSIS

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 prognosti c features were retrospectively analyzed in patient groups, categorize d by their serum ANA. Results. Cumulative survival rates at 10 years a fter diagnosis of SSc were 93% in patients with anticentromere antibod ies (ACA), 72% in those with anti-U1 RNP, 66% in those with anti-DNA t opoisomerase I (anti-topo I), and 30% in those with anti-RNA polymeras es I, II, and III (anti-RNAP). Major organ involvement linked to cause of death included biliary cirrhosis in patients with ACA, isolated pu lmonary arterial hypertension and cerebral hemorrhage in those with an ti-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.