RT Journal Article
SR Electronic
T1 Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment
JF Canadian Medical Association Journal
JO CMAJ
FD Canadian Medical Association
SP 153
OP 160
DO 10.1503/cmaj.1030055
VO 171
IS 2
A1 Nasreen Khalil
A1 Robert O'Connor
YR 2004
UL http://www.cmaj.ca/content/171/2/153.abstract
AB IDIOPATHIC PULMONARY FIBROSIS (IPF) is a progressive and lethal pulmonary 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 fibroblast foci. Pulmonary functions show restricted volumes and capacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomography demonstrates evidence 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.