CCN2 and idiopathic pulmonary fibrosis: In IPF, however, AECII differentiates and features an abnormally activated phenotype, characterised as cells undergoing hyperplasia, senescence and apoptosis, which contribute as paracrine factors to fibroblast proliferation and their transformation into myofibroblasts through the production and release of profibrotic cytokines and growth factors (amongst them, transforming growth factor beta (TGF-β), connective tissue growth factor (CTGF), platelet-derived growth factor (PDGF), tumour necrosis factor-alpha (TNF-α), and endothelin-1) or other mediators [39,40,41,42].