Idiopathic Pulmonary Fibrosis: A Case Study
Introduction
Idiopathic pulmonary fibrosis (IPF) is a chronic and fatal disease characterized by fibrotic remodeling of lung parenchyma followed by progressive decline in lung function [1, 2]. IPF may be initiated by intrinsic and extrinsic cellular stress which lead to activation of fibroblasts, destruction of alveolar structures, and deposition of extracellular matrix proteins, resulting in impaired gas exchange and respiratory failure [3-5]. Although the role of inflammation in the initiation and progression of pulmonary fibrosis remains controversial, immune dysregulation is an important feature of IPF [6].
Idiopathic pulmonary fibrosis (IPF) is a chronic and fatal disease characterized by fibrotic remodeling of lung parenchyma followed by progressive decline in lung function [1, 2]. IPF may be initiated by intrinsic and extrinsic cellular stress which lead to activation of fibroblasts, destruction of alveolar structures, and deposition of extracellular matrix proteins, resulting in impaired gas exchange and respiratory failure [3-5]. Although the role of inflammation in the initiation and progression of pulmonary fibrosis remains controversial, immune dysregulation is an important feature of IPF [6].