AB0653 The association of autoantibodies with morbidity and mortality of scleroderma renal crisis in Japan

Abstract

BackgroundThe morbidity of scleroderma renal crisis (SRC) and autoantibodies in systemic sclerosis (SSc) vary by races and regions. Anti-RNA polymerase III is associated with SRC in America and European countries. However, the association of autoantibodies with SRC had not been elucidated in Japan.ObjectivesWe aimed to investigate the association of autoantibodies with morbidity and mortality of SRC in Japan.MethodsThe clinical characteristics and mortality of 330 patients with systemic sclerosis (SSc) at Kyoto University Hospital were retrospectively analyzed, focusing on anti-centromere, anti-RNA polymerase III, anti-topoisomerase I, and anti-U1-RNP. Logistic regression analyses were performed to examine the association of autoantibodies with the development and mortality of SRC. Kaplan-Meier survival analysis was performed comparing the groups classified by autoantibodies.ResultsAnti-centromere (n = 177/318, 56%), anti-topoisomerase I (n = 80/311, 26%), anti-RNA polymerase III (n = 27/204, 13%), and anti-U1-RNP (n = 24/305, 8%) were found in SSc patients (n = 330). SRC was observed in 24 out of 330 SSc patients, including anti-topoisomerase I (n = 12/24, 50%), anti-RNA polymerase III (n = 7/24, 29%), anti-U1-RNP (n = 5/24, 21%), and anti-centromere (n = 3/24, 13%). Anti-U1-RNP (odds ratio [95% confidence interval], 3.63 [1.11–10.2]), anti-RNA polymerase III (3.29 [1.16–8.70]), and anti-topoisomerase I (3.22 [1.37–7.57]) were associated with the development of SRC. All patients with SRC were treated with ACE inhibitors and the 1-year survival rate was 54%. Anti-topoisomerase I was associated with the 1-year mortality of SRC (6.00 [1.11–41.1]). When the survival rate was compared between the patients positive for anti-topoisomerase I (n=12) and negative for anti-topoisomerase I (n=12), the 1-year survival rate was 33% vs 75% (p=0.041), respectively (Figure 1A). Furthermore, the 1-year survival of anti-centromere (100%), anti-RNA polymerase III (83%), and others/not detected (50%) were shown in patients negative for anti-topoisomerase I (Figure 1B).Figure 1.Overall survival of patients with SRC according to the type of autoantibodies.(A) The survival rates in SRC patients who were positive for anti-topoisomerase I (solid line, n = 12) and those who were negative for anti-topoisomerase I (dotted line, n = 12).(B) SRC patients negative for anti-topoisomerase I were classified as patients with anti-RNA polymerase III (dotted line, n = 6), anti-centromere (broken line, n = 2), and others/not detected (chain line, n = 4).ConclusionSpecific SSc-related autoantibodies were associated with the morbidity and mortality in SRC.