Idiopathic pulmonary fibrosis
- Department of Infection and Immunity, Academic Unit of Respiratory Medicine, University of Sheffield, Sheffield, UK
- Correspondence to Professor Moira Whyte, Department of Infection and Immunity, Academic Unit of Respiratory Medicine, University of Sheffield, Sheffield S10 2RX, UK;
Contributors Both authors were involved in the background reading and writing of this article.
- Received 24 May 2011
- Accepted 27 May 2011
- Published Online First 22 June 2011
Idiopathic pulmonary fibrosis is a progressive lung disease that carries a poor prognosis and for which there are no effective therapies. Although the excessive deposition of extracellular matrix, combined with evidence of recurrent injury to the alveolar epithelium, are well-described there is a pressing need to understand these processes better at a molecular level and thus to identify potential therapeutic targets in this intractable disease. This review considers some recent advances published in Thorax and elsewhere that have improved our understanding of the pathophysiology of idiopathic pulmonary fibrosis, using data both from human cells and tissue and from animal models of pulmonary fibrosis. The studies particularly address the fate of the alveolar epithelial cell and mechanisms of fibrogenesis, and identify mechanistic pathways shared with co-existing conditions such as lung cancer and pulmonary hypertension. The concepts of physiological biomarkers of disease progression and prognosis are also discussed.
Competing interests None.
Provenance and peer review Commissioned; not externally peer reviewed.