New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis.
Idiopathic pulmonary fibrosis is a serious and progressive chronic lung disease that is characterised by altered cellular composition and homoeostasis in the peripheral lung, leading to excessive accumulation of extracellular matrix and, ultimately, loss of lung function. It is the interstitial pneumonia with the worst prognosis--mortality 3-5 years after diagnosis is 50%. During the past decade, researchers have described several novel cellular and molecular mechanisms and signalling pathways implicated in the pathogenesis of idiopathic pulmonary fibrosis, resulting in the identification of new therapeutic targets. These advances will hopefully result in increased survival rates and improved quality of life for patients with this disorder in future.
Comprehensive Pneumology Centre, University Hospital of the Ludwig-Maximilians University Munich, Munich, Germany.
Isis E
IE
Fernandez
Oliver
O
Eickelberg
D004847:Epithelial Cells / Q000473:pathology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000209:etiology / Q000235:genetics / Q000473:pathology*; D055370:Lung Injury / Q000209:etiology / Q000235:genetics / Q000473:pathology*; D035683:MicroRNAs / Q000235:genetics; D058628:Myofibroblasts / Q000473:pathology; D020286:Pericytes / Q000473:pathology; D015398:Signal Transduction / Q000502:physiology
D035683:MicroRNAs