Idiopathic pulmonary fibrosis: pathogenesis and management.

ABSTRACT:

Background

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease characterized by the aberrant accumulation of fibrotic tissue in the lungs parenchyma, associated with significant morbidity and poor prognosis. This review will present the substantial advances achieved in the understanding of IPF pathogenesis and in the therapeutic options that can be offered to patients, and will address the issues regarding diagnosis and management that are still open.

Main body

Over the last two decades much has been clarified about the pathogenic pathways underlying the development and progression of the lung scarring in IPF. Sustained alveolar epithelial micro-injury and activation has been recognised as the trigger of several biological events of disordered repair occurr

SUBMITTER: Sgalla G 

PROVIDER: S-EPMC5824456 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature