Idiopathic Pulmonary Fibrosis: Cell Death and Inflammation Revisited

Fibrosis is a common complication and contributor to the morbidity and mortality of a number of acute or chronic lung diseases. Among these pathologies, the most pernicious is idiopathic pulmonary fibrosis (IPF). IPF is characterized by a relentless, progressive accumulation of fibrotic tissue leading to irreversible destruction of lung architecture. As a result, patients with IPF, for whom no curative treatment option is available except for lung transplantation, will die of respiratory failure, with a median survival of 3-5 years from diagnosis. Finally, this study also brings to light some thought-challenging questions related to inflammation and its underlying contribution to IPF. Inflammation has not been considered critical to IPF initiation or progression.