The pathogenesis of idiopathic pulmonary fibrosis.

  title={The pathogenesis of idiopathic pulmonary fibrosis.},
  author={William R. Coward and Gauri Saini and Gisli Jenkins},
  journal={Therapeutic advances in respiratory disease},
  volume={4 6},
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an appalling prognosis. The failure of anti-inflammatory therapies coupled with the observation that deranged epithelium overlies proliferative myofibroblasts to form the fibroblastic focus has lead to the emerging concept that IPF is a disease of deregulated epithelial-mesenchymal crosstalk. IPF is triggered by an as yet unidentified alveolar injury that leads to activation of transforming growth factor-β (TGF… CONTINUE READING
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