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QJM Advance Access originally published online on September 6, 2006
QJM 2006 99(10):643-654; doi:10.1093/qjmed/hcl098
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© The Author 2006. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Review

Idiopathic pulmonary fibrosis: an update

O.J. Dempsey1,, K.M. Kerr2, L. Gomersall3, H. Remmen4 and G.P. Currie1

From the Departments of 1Respiratory Medicine, 2Pathology, 3Radiology and 4Cardiothoracic Surgery, Aberdeen Royal Infirmary, Foresterhill, Aberdeen, UK

Address correspondence to Dr O.J. Dempsey, Department of Respiratory Medicine, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZN. email: owen.dempsey{at}nhs.net

Idiopathic pulmonary fibrosis (IPF) is a chronic lung condition of uncertain aetiology that should be considered in the differential diagnosis of patients who experience breathlessness, cough and reduced exercise tolerance. IPF is characterized histologically by the presence of usual interstitial pneumonia, and often has typical radiological appearances. Long-term successful management options are limited and frequently unsuccessful; as the disease progresses, palliation of symptoms becomes the mainstay of treatment. In a minority of patients, lung transplantation provides the only hope of long-term survival. The median survival of patients with IPF is approximately 3 years, which in turn emphasizes the need for further investigation into its pathogenesis and potential disease-modifying pharmacological therapies.


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