Abstract
Of the 158 Japanese patients with systemic sclerosis (SSc) who were followed up during the period from 1991 to 1999 in our department, 29.1% had anti-DNA topoisomerase 1 antibody (anti-Topo 1 antibody), 23.4% had anticentromere antibody (ACA), and 8.9% had anti-U 1 RNP antibody. Significant associations between the antinuclear antibody (ANA) and organ involvement were noted: anti-Topo 1 antibody with digital pitting scar, trunkal sclerosis, and lung fibrosis; ACA with esophageal hypomotility, Sjögren’s syndrome, and primary biliary cirrhosis. Cumulative survival rates at 10 years after diagnosis of SSc was lowest in anti-Topo 1 positive diffuse cutaneous SSc (dcSSc) (86.4%). The most frequent cause of death related with SSc was cardiopulmonary disease due to lung fibrosis (9/15, 60%; 2 were anti-Topo 1 antibody positive limited cutaneous SSc (lcSSc), 3 were anti-Topo 1 antibody positive diffuse cutaneus SSc (dcSSc), 2 were U 1 RNP positive lcSSc, 2 were specific antibodies-negative lcSSc). In anti-Topo 1 antibody positive lcSSc and dcSSc, both the lung involvement rates and severe lung disease represented by low vital capacity (<60%) were high. In specific antibodies-negative lcSSc, the severe lung disease rate was high, although the lung involvement rate was low. Careful observation of the progression of pulmonary interstitial fibrosis is needed not only in cases of anti-Topo 1 antibody positive SSc but also in specific antibodies-negative lcSSc with lung fibrosis.
