Differences in the autoantibody phenotypes and long-term outcomes between juvenile- and adult-onset systemic sclerosis

Objective To investigate differences in autoantibodies, clinical features, and long-term outcomes between juvenile- and adult-onset systemic sclerosis (SSc).Methods Autoantibodies and survival rates over a maximum of 20 years were retrospectively analysed in 504 Japanese patients with SSc (juvenile-onset SSc, n = 17; adult-onset SSc, n = 487) using data from Kyoto University Registry.Results : The autoantibodies observed were anti-topoisomerase-I (71% vs. 26%), anti-centromere (24% vs. 54%), and anti-RNA-polymerase-III (0% vs. 12%). A diffuse type and multiorgan involvement were observed in patients with anti-topoisomerase-I in both juvenile- and adult-onset SSc. In patients with anti-centromere, a diffuse type (juvenile-onset SSc vs. adult-onset SSc, 75% vs. 28%) and pulmonary fibrosis (50% vs. 17%) were more frequently observed in juvenile-onset SSc than in adult-onset SSc. Cox proportional hazard analyses showed that older onset (hazard ratio: 1.06, 95% confidence interval: 1.03-1.09) was associated with death, while autoantibodies were not significantly associated with death. Cumulative survival rates for 20 years were similar between juvenile- and adult-onset SSc when classified based on the presence of anti-centromere (100% vs. 89%, P = .20) and anti-topoisomerase-I (90% vs. 90%, P = .70).Conclusions : Juvenile-onset SSc had more frequent diffuse-type and anti-topoisomerase-I. An older onset was slightly associated with mortality, whereas autoantibodies were not associated with mortality.