Corroborating our finding of an association between TGF-β1 levels and the diffuse cutaneous subset and pulmonary fibrosis, we observed higher TGF-β1 levels in patients with positive antitopoisomerase I. Furthermore, the presence of scleroderma-associated autoantibodies is correlated with distinct clinical phenotypes of SSc. Here, TGFB1 is linked to systemic sclerosis.