Idiopathic pulmonary fibrosis (IPF) is a deadly lung disease marked by myofibroblasts and extracellular matrix accumulation in the lungs over time. CTGF exacerbates pulmonary fibrosis in radiation-induced lung fibrosis. In this study, researchers demonstrated upregulation of CTGF in a rat lung fibrosis model produced by an adenovirus vector encoding active TGF-1 (AdTGF-1). CTGF was similarly increased in IPF patients, according to the findings. CTGF expression was elevated in fibrotic lungs vascular smooth muscle cells on days 7 and 14 and endothelial cells sorted from fibrotic lungs on days 14 and 28. These findings imply that various cells played a role in maintaining the fibrotic phenotype during the fibrogenesis process.
Treatment of fibroblasts with recombinant CTGF and TGF- enhanced pro-fibrotic markers, indicating that recombinant CTGF and TGF- had a synergistic impact in causing pulmonary fibrosis. In addition, fibrotic extracellular matrix elevated CTGF expression relative to the normal extracellular matrix, implying that fibrogenesis is aided not just by profibrotic mediators but also by a profibrotic environment. Pamrevlumab, a CTGF inhibiting antibody, was similarly shown to reduce fibrosis in the model partially. These findings imply that pamrevlumab may be a viable therapy option for pulmonary fibrosis.
Reference:www.atsjournals.org/doi/abs/10.1165/rcmb.2020-0504OC
