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 pneu...

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Bibliographic Details
Published in:The Lancet (British edition) 2012-08, Vol.380 (9842), p.680-688
Main Authors: Fernandez, Isis E, MD, Eickelberg, Oliver, Prof
Format: Article
Language:English
Subjects:
Apoptosis
Autophagy
Biological and medical sciences
Epithelial Cells - pathology
extracellular matrix
fibrosis
General aspects
Humans
Hypoxia
Idiopathic Pulmonary Fibrosis - etiology
Idiopathic Pulmonary Fibrosis - genetics
Idiopathic Pulmonary Fibrosis - pathology
Internal Medicine
Lung diseases
lung function
Lung Injury - etiology
Lung Injury - genetics
Lung Injury - pathology
Lungs
Medical sciences
MicroRNAs - genetics
Mortality
Myofibroblasts - pathology
Pathogenesis
patients
Pericytes - pathology
Pneumology
pneumonia
Proteins
Quality of life
researchers
Respiratory function
Respiratory system : syndromes and miscellaneous diseases
Rodents
signal transduction
Signal Transduction - physiology
Survival
survival rate
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Summary: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.
ISSN:0140-6736
1474-547X
DOI:10.1016/S0140-6736(12)61144-1