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Idiopathic pulmonary fibrosis

Research output: Contribution to journalLiterature reviewpeer-review

Original languageEnglish
Pages (from-to)742-746
Number of pages5
JournalThorax
Volume67
Issue number8
DOIs
Publication statusPublished - Aug 2012

Abstract

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.

    Research areas

  • INTERSTITIAL LUNG-DISEASE, SMOOTH-MUSCLE, SOMATOSTATIN RECEPTORS, COMPUTED-TOMOGRAPHY, PROGNOSTIC VALUE, CONTROLLED-TRIAL, INFLAMMATION, PATHOGENESIS, PNEUMONIA, EXPRESSION

ID: 17164666