CMAJ • July 20, 2004; 171 (2). doi:10.1503/cmaj.1030055.
© 2004 Canadian Medical Association or its licensors
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Review
Synthèse

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

Nasreen Khalil and Robert O'Connor

From the Respiratory Division, the Vancouver Coastal Health Research Institute (Khalil), and the British Columbia Cancer Agency and the University of British Columbia (O'Connor), Vancouver, BC

Abstract

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.




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are possible ifferent fibroblast subsets in IPF?
Irene Esposito
CMAJ, 1 Oct 2004 [Full text]