TY - JOUR T1 - Pirfenidone and BIBF1120 regulate properties of myofibroblasts JF - European Respiratory Journal JO - Eur Respir J VL - 44 IS - Suppl 58 SP - P3502 AU - Siri Lehtonen AU - Henna Karvonen AU - Elisa Lappi-Blanco AU - Raija Sormunen AU - Ulrika Zagai AU - Magnus Sköld AU - Riitta Kaarteenaho Y1 - 2014/09/01 UR - http://erj.ersjournals.com/content/44/Suppl_58/P3502.abstract N2 - Myofibroblast-type stromal cells are aggregated in fibroblast foci in idiopathic pulmonary fibrosis (IPF). Myofibroblasts produce most of the excess extracellular matrix protein (ECM) and also interact with epithelium in IPF having an essential role in the pathogenesis of the disease. We have previously characterized myofibroblasts from the patients with several types of interstitial lung diseases (Karvonen, H.M. et al. Lab Invest 2012;92:1270-1284).The aim of this study was to evaluate the effect of pirfenidone and BIBF1120 (nintedanib), a triple kinase inhibitor, to the structural and functional properties of myofibroblasts of the patients with IPF.Primary cells were collected from lung tissue and bronchoalveolar lavage samples from the patients with IPF. Cell lines containing of fibroblasts and myofibroblasts were exposed to pirfenidone and BIBF1120 with and without transforming growth factor beta (TGF-beta) induction, and characterized by gel contraction assay, Western blotting, electron (TEM) and immunoelectron microscopy (IEM).Pirfenidone and BIBF1120 inhibited contraction of cells in collagen gel and decreased the amount of alpha smooth muscle actin (alpha-SMA), a marker of myofibroblasts, by Western blotting, which effects were more pronounced with TGF-beta induction. Amount of actin and alpha-SMA was decreased by pirfenidone and BIBF1120 exposition with TGF-beta induction in IEM and TEM.We conclude that both pirfenidone and BIBF1120 affected to the amount of alpha-SMA in myofibroblasts, and moreover, to the collagen gel contraction capacity of the cells regulating both functional and structural phenotype of myofibroblasts. ER -