It has been reported that an uPAR deficiency promotes endothelial dysfunction and fibrosis progression [3,4], and a α2AP deficiency and PAI-1 neutralization attenuate dermal inflammation and fibrosis progression in the bleomycin-induced SSc model mice, and multiple studies suggest that the fibrinolytic factors are associated with the pathology of SSc [5,6,7]. This evidence concerns the gene SERPINE1 and systemic sclerosis.