More recently Xiao et al. evaluated 25 lung biopsies in SSc-ILD and found that the hedgehog pathway activation was increased in the lung tissue of SSc-ILD patients and this was decreased by pirfenidone, Sufu (suppressor of fused) was upregulated in lung fibroblasts after pirfenidone challenge, and pirfenidone inhibited the phosphorylation of GSK-3b signalling [24]. Here, SUFU is linked to interstitial lung disease.