A Review of Pirfenidone as an Anti-Fibrotic in Idiopathic Pulmonary Fibrosis and Its Probable Role in Other Diseases.

Parth V Shah,Chelsea Mae N Nobleza,Safeera Khan,Prachi Balani,Angel R Lopez,Mariah Siddiqui

doi:10.7759/cureus.12482

Abstract

Fibrosis is the result of chronic inflammation and is known to pathologically occur in many organs and systems. Pirfenidone (PFD) is an anti-fibrotic known for its use in idiopathic pulmonary fibrosis (IPF). In addition to being an anti-fibrotic, it acts as an anti-inflammatory and antioxidant as well. There have been studies on PFD in other diseases, some clinical and others preclinical. We have compiled and reviewed them to highlight just how widespread PFD use could be. Among many benefits of PFD in IPF, PFD has effectively improved patients' survival in those who had an acute exacerbation of IPF and has reduced respiratory-related hospitalization, among few others. PFD also has shown an improvement in vital capacity in patients with chronic hypersensitive pneumonitis. Also, it has demonstrated anti-fibrotic effects in systemic sclerosis-associated interstitial lung disease. In other diseases outside the lungs, PFD has reversed insulin resistance and proven to be effective in non-alcoholic steatohepatitis (NASH). It has prevented blindness post-alkali injury to the eye and has proven to decrease the proliferation of mesothelioma cells, just to name a few. This review encourages further research in connection with PFD and its use in other diseases and PFD pros in IPF.

Highlights

BackgroundFibrosis is scarring tissue and occurs when excess extracellular matrix material is replaced by collagen and fibronectin in response to cellular damage
In a study done on PFD in malignant mesothelioma, it was shown that PFD reduced the TGF-β-induced extracellular-signal-regulated kinase (ERK) and serine/threonine-specific protein kinase (AKT) pathways [19]
Did PFD reduce the proliferation of cells in mesothelioma, and it reduced the proliferation of fibroblasts derived from Dupuytren's disease in another study done in vitro [19,20]

Summary

Introduction

BackgroundFibrosis is scarring tissue and occurs when excess extracellular matrix material is replaced by collagen and fibronectin in response to cellular damage. Fibroblasts are the producers of a variety of substances found in the extracellular matrix When these cells transform via a range of signaling programs, they differentiate into many types of cells. Myofibroblasts are one of its major phenotypes They play a significant role in excessively synthesizing and secreting the extracellular matrix. These cells are susceptible to chemokines, cytokines, and growth factors [1]. These cells can be generated from other sources and activated by multiple mechanisms, playing an essential role in fibrosis [2]. Any chronic inflammatory state, such as idiopathic pulmonary fibrosis (IPF), rheumatoid arthritis, scleroderma, end-stage renal disease, and myocardial infarction, can lead to fibrosis [3]

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