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.
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.
Authors: A Della Rossa; G Valentini; S Bombardieri; W Bencivelli; A J Silman; S D'Angelo; M M Cerinic; J F Belch; C M Black; R Becvar; P Bruhlman; F Cozzi; L Czirják; A A Drosos; B Dziankowska; C Ferri; A Gabrielli; R Giacomelli; G Hayem; M Inanc; N J McHugh; H Nielsen; R Scorza; E Tirri; F H van den Hoogen; P G Vlachoyiannopoulos Journal: Ann Rheum Dis Date: 2001-06 Impact factor: 19.103
Authors: S Veeraraghavan; E A Renzoni; H Jeal; M Jones; J Hammer; A U Wells; C M Black; K I Welsh; R M du Bois Journal: Ann Rheum Dis Date: 2004-08 Impact factor: 19.103