CFTR and pulmonary fibrosis: 2013), with increased plasma and bronchoalveolar fluid (BALF) TGF‐β concentrations in subjects with more advanced lung disease (Harris et al. 2009, 2011). In addition to the effects of increased TGF‐β signaling on lung fibrosis and remodeling, TGF‐β has direct suppressive actions on CFTR expression in primary differentiated human bronchial epithelial cells (Snodgrass et al. 2013; Sun et al. 2014), with antagonism of recently approved CFTR modulator therapy (Lutful Kabir et al. 2018).