Genetics in Idiopathic Pulmonary Fibrosis Pathogenesis, Prognosis, and Treatment.

ABSTRACT: Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia (IIP), is characterized by irreversible scarring of the lung parenchyma and progressive decline in lung function leading to eventual respiratory failure. The prognosis of IPF is poor with a median survival of 3-5 years after diagnosis and no curative medical therapies. Although the pathogenesis of IPF is not well understood, there is a growing body of evidence that genetic factors contribute to disease risk. Recent studies have identified common and rare genetic variants associated with both sporadic and familial forms of pulmonary fibrosis, with at least one-third of the risk for developing fibrotic IIP explained by common genetic variants. The IPF-associated genetic loci discovered to date are

SUBMITTER: Kaur A 

PROVIDER: S-EPMC5622313 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature