Furthermore, we have investigated the effect of antifibrotic drugs, namely pirfenidone and nintedanib, on the fibroblastic cell lines cultured from the BAL fluid (n = 4) and lung tissue (n = 3) samples of patients with IPF, showing that both drugs reduced the in vitro proliferation, the amount of α-SMA, and the myofibroblastic appearance of myofibroblasts [21]. Here, ACTA1 is linked to idiopathic pulmonary fibrosis.