It has been reported that patients with anti-RNAP have the worst survival rate.29,30 However, mortality in patients with anti-RNAP is currently lower than in those with anti-topo I.29 This is because patients with anti-RNAP have a low risk of suffering pulmonary fibrosis, and renal crisis is now more readily treated with angiotensin-converting enzyme inhibitors than pulmonary fibrosis.39 Our data indicate that patients with anti-topo I have the worst survival rate of all subgroups and that the prognosis of other patients with SSc is favourable (Fig. 2b). This evidence concerns the gene ACE and pulmonary fibrosis.