• Siva Mahendran and Tariq Sethi.
• Department of Respiratory Medicine, Kings College London, Denmark Hill Campus, Bessemer Road, SE5 9RS London, UK.
• QJM. 2012 Oct 1;105(10):929-34.

AbstractIdiopathic pulmonary fibrosis (IPF) is a progressive age-related lung disease, the cause of which is not been fully understood. IPF is a devastating disease with mortality worse than many cancers, and treatment options are limited. IPF is thought to occur after recurrent injury to the alveolar epithelium followed by abnormal repair characterized by the formation of fibroblast and myofibroblast foci and excessive deposition of extracellular matrix. An updated classification of the idiopathic interstitial pneumonias has encouraged a large number of clinical trials. On the whole, these have disappointed. Improvements in molecular techniques have developed our understanding of IPF and with it identified new pathways and potential targets for therapeutic intervention. These insights are leading to interest in biomarkers of disease progression and prognosis and to novel anti-fibrotic agents and a more targeted approach to the treatment of IPF.

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