Patients with
systemic sclerosis (SSc) have an increased risk of
malignancy compared with the general population. Recently, SSc patients with anti-
RNA polymerase III antibody have been reported to have an increased risk of
malignancy as compared with those with other disease-specific
autoantibodies in US, European and Australian populations. Therefore, we studied the relationship between disease-specific
autoantibodies and
malignancy in 261 Japanese SSc patients. The prevalence of
malignancy was significantly higher in patients with anti-
RNA polymerase III antibody (7/22, 31.8%) than in those with
anti-topoisomerase I antibody (2/82, 2.4%) and in those with
anticentromere antibody (8/137, 5.8%). Importantly, among seven patients with anti-
RNA polymerase III antibody and
malignancy, three patients (42.9%) developed
malignancy from 6 months before to 12 months after SSc onset. Thus,
malignancy complication in Japanese SSc patients with anti-
RNA polymerase III antibody is as high as that in other races, suggesting that SSc patients with anti-
RNA polymerase III antibody share the same pathological process among different ethnic groups.