Xiao H, et al. - This study was conducted in order to examine if pirfenidone attenuated lung fibrosis by interfering with the hedgehog (Hh) signaling pathway in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). Anti-fibrotic effects were illustrated by pirfenidone in SSc-ILD patients via interference with both the Hh signaling pathway and the GSK-3β signaling pathway through the regulation of Sufu expression. Hence, it could be beneficial in other Hh driven lung diseases, for instance, idiopathic pulmonary fibrosis and especially the interstitial lung disease related to connective tissue diseases.