Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment

Khalil, Nasreen; O'Connor, Robert
July 2004
CMAJ: Canadian Medical Association Journal;7/20/2004, Vol. 171 Issue 2, p153
Academic Journal
IDIOPATHIC PULMONARY FIBROSIS (IPF) is a progressive and lethal pul-monary fibrotic lung disease. The diagnostic histological changes are called usual interstitial pneumonia and are characterized by histological temporal heterogeneity, whereby normal lung tissue is interspersed with interstitial fibrosis, honeycomb cysts and fibro-blast foci. Pulmonary functions show restricted volumes and ca-pacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomography demonstrates evi-dence of fibrosis and lung remodelling such as honeycomb cysts and traction bronchiectasis. There is no known effective treatment for IPF, but lung transplantation improves survival.


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