TITLE

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

AUTHOR(S)
Khalil, Nasreen; O'Connor, Robert
PUB. DATE
July 2004
SOURCE
CMAJ: Canadian Medical Association Journal;7/20/2004, Vol. 171 Issue 2, p153
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
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.
ACCESSION #
13705765

 

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