Idiopathic pulmonary fibrosis

DJ Lederer, FJ Martinez - New England Journal of Medicine, 2018 - Mass Medical Soc
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Idiopathic pulmonary fibrosis

L Richeldi, HR Collard, MG Jones - The Lancet, 2017 - thelancet.com
Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease.
Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is
destroyed, which leads to decreased lung compliance, disrupted gas exchange, and
ultimately respiratory failure and death. In less than a decade, understanding of the
pathogenesis and management of this disease has been transformed, and two disease-
modifying therapies have been approved, worldwide. In this Seminar, we summarise the …

Idiopathic pulmonary fibrosis

FJ Martinez, HR Collard, A Pardo, G Raghu… - Nature reviews Disease …, 2017 - nature.com
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by
progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It
is associated with increasing cough and dyspnoea and impaired quality of life. IPF affects∼
3 million people worldwide, with incidence increasing dramatically with age. The diagnostic
approach includes the exclusion of other interstitial lung diseases or overlapping conditions
and depends on the identification of the UIP pattern, usually with high-resolution CT; lung …