• CMAJ · Jul 2004

    Review

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

    • Nasreen Khalil and Robert O'Connor.
    • Respiratory Division, Vancouver Coastal Health Research Institute, Vancouver, BC. nkhalil@interchange.ubc.ca
    • CMAJ. 2004 Jul 20; 171 (2): 153160153-60.

    AbstractIdiopathic 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.

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