Role and New Insights of Pirfenidone in Fibrotic Diseases

Abstract

Pirfenidone (PFD) is a non-peptide synthetic molecule issued as a broad-spectrum anti-fibrotic drug with the ability to decrease TGF-β1, TNF-α, PDGF and COL1A1 expression, which is highly related to prevent or remove excessive deposition of scar tissue in several organs. Basic and clinical evidence suggests that PFD may safely slow or inhibit the progressive fibrosis swelling after tissue injuries. Furthermore, a number of evidence suggests that this molecule will have positive effects in the treatment of other inflammatory diseases. This review contains current research in which PFD has been used as the treatment of several diseases, and focus mainly in the outcomes related to improve inflammation and fibrogenesis. Therefore, the main goal of this review is to focus on the novel findings of PFD efficacy rather than deepen in the chemical aspects of the molecule.

Keywords: Pirfenidone; fibrosis; idiopathic pulmonary fibrosis; inflammation.

Figure 1

Pirfenidone targets. Different targets in vivo and in vitro for PFD have been described, the most prominent being inhibition of TGF-β1 and TNF-α. However, it has also been shown that PFD has either direct or indirect action on other molecules such as collagen I, PDGF, IL-6, IL-1β, IL-13, IL-12p40, fibronectin, HSP47 and ICAM-1.