Arginine is a key player in fibroblasts during the course of IPF development

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease characterized by the activation of invasive fibroblasts, which are involved in the uncontrolled production and deposition of extracellular matrix (ECM) in the lung parenchyma. No therapeutic approach is, thus far, available to halt or reverse the process of IPF, although nintedanib and pirfenidone, two US Food and Drug Administration (FDA)-approved drugs, have shown that it is possible to slow the decline of lung function without a noticeable effect upon overall mortality.