Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions.

ABSTRACT: Idiopathic pulmonary fibrosis (IPF), the most lethal form of interstitial pneumonia of unknown cause, is associated with a specific radiological and histopathological pattern (the so-called "usual interstitial pneumonia" pattern) and has a median survival estimated to be between 3 and 5 years after diagnosis. However, evidence shows that IPF has different clinical phenotypes, which are characterized by a variable disease course over time. At present, the natural history of IPF is unpredictable for individual patients, although some genetic factors and circulating biomarkers have been associated with different prognoses. Since in its early stages, IPF may be asymptomatic, leading to a delayed diagnosis. Two drugs, pirfenidone and nintedanib, have been shown to modify the disease course by s

SUBMITTER: Stainer A 

PROVIDER: S-EPMC8230407 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature