Abstract
Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, and limited to the lungs. Despite the increasing research interest in the pathogenesis of IPF, unfavorable survival rates remain associated with this condition. Recently, novel therapeutic agents have been shown to control the progression of IPF. However, these drugs do not improve lung function and have not been tested prospectively in patients with IPF and coexisting lung cancer, which is a common comorbidity of IPF. Optimal management of patients with IPF and lung cancer requires understanding of pathogenic mechanisms and molecular pathways that are common to both diseases. This review article reflects the current state of knowledge regarding the pathogenesis of pulmonary fibrosis and summarizes the pathways that are common to IPF and lung cancer by focusing on the molecular mechanisms.
Highlights
Idiopathic pulmonary fibrosis is a progressive and usually fatal lung disease characterized by fibroblast proliferation and extracellular matrix remodeling, which results in irreversible distortion of the lung’s architecture
At the time of diagnosis, modifications of lung structure have already been established by the disease and pathological features, such as various stages of epithelial damage, alveolar epithelial cell (AEC) 2s hyperplasia, dense fibrosis, and abnormally proliferating mesenchymal cells, are found
It is not possible to determine the course of events that have led to lung damage; it is accepted that dysfunctional epithelia are key to the pathogenesis of Idiopathic pulmonary fibrosis (IPF) [15]
Summary
Idiopathic pulmonary fibrosis is a progressive and usually fatal lung disease characterized by fibroblast proliferation and extracellular matrix remodeling, which results in irreversible distortion of the lung’s architecture. Based on recent in vitro and in vivo studies of IPF, the novel therapeutic reagents pirfenidone and nintedanib were developed to slow the progression of this complex disease [3,4,5]. These drugs do not improve lung function and patients often remain with poor pulmonary function [6,7]. Primary lung cancer risk is more than 20 times higher in patients who undergo lung transplantation for IPF than in the general population [13,14] These observations warrant efforts to identify pathways that are common to both disorders. This review article summarizes the current knowledge of the pathogenesis of pulmonary fibrosis and outlines the common molecular pathways between IPF and lung cancer
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