OBJECTIVE: To investigate the association of autoantibodies with scleroderma renal crisis (SRC) among Japanese patients. METHODS: The clinical characteristics and mortality of 330 patients with systemic sclerosis (SSc) at Kyoto University Hospital were retrospectively analyzed, focusing on anti-topoisomerase I, anti-centromere, anti-RNA polymerase III (RNAPIII), and anti-U1-RNP. Logistic regression analyses were performed to examine the association of autoantibodies with the development and mortality of SRC. RESULTS: SRC was observed in 24 out of 330 SSc patients, including anti-topoisomerase I (n = 12/24, 50%), anti-RNAPIII (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-topoisomerase I (3.22 [1.37-7.57]), and anti-RNAPIII (3.29 [1.16-8.70]) were associated with the development of SRC. Furthermore, anti-topoisomerase I (6.00 [1.11-41.1]) was associated with the 1-year mortality of SRC. The 1-year survival rate after the onset of SRC among all patients and those positive for anti-topoisomerase I was 54% and 33%, respectively. In contrast, the survival rate in patients negative for anti-topoisomerase I was 75%, of which the survival rate of patients positive for anti-RNAPIII and anti-centromere was 83% and 100%, respectively. CONCLUSION: Specific SSc-related autoantibodies were associated with the morbidity and mortality of SRC.