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Idiopathic Pulmonary Fibrosis

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Treatment of Cystic Fibrosis and Other Rare Lung Diseases

Part of the book series: Milestones in Drug Therapy ((MDT))

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic and fatal disease of unknown cause representing the most common form of idiopathic interstitial pneumonias. It is believed that the development of IPF is influenced by both genetic and environmental factors. The pathogenesis of IPF is complex, and many contributing factors to fibrogenesis are known to date. In recent years there have been important advances in the understanding of the pathogenesis of IPF. A large number of experimental studies have highlighted the importance of epithelial cell injury, fibroblast differentiation and myofibroblast activation, the involvement of inflammatory and progenitor cells, and the effect of genetic and epigenetic factors. IPF is characterized by progressive fibroblast proliferation and differentiation followed by excessive deposition of extracellular matrix (ECM). This results in the damage to the structure of the lung, which eventually causes dyspnea and respiratory failure in patients with IPF. This fibrotic ECM microenvironment is characterized by altered biochemical and biomechanical properties and stores abundant amounts of growth factors, all of which can affect the behavior of structural lung cells and also inflammatory cells. This chapter summarizes the newest insights into the pathogenesis of IPF and tries to describe how this knowledge helps to find new therapies for the patients who are suffering from this devastating disease.

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Authors and Affiliations

  1. Department of Medicine, Firestone Institute for Respiratory Health, McMaster University, Hamilton, ON, Canada

    Chiko Shimbori, Pierre-Simon Bellaye, Philipp Kolb & Martin Kolb

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  1. Chiko Shimbori
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  2. Pierre-Simon Bellaye
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  3. Philipp Kolb
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  4. Martin Kolb
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Correspondence to Martin Kolb .

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Editors and Affiliations

  1. Div of Pulmonary Medicine, Nippon Medical School Div of Pulmonary Medicine, Tokyo, Japan

    Arata Azuma

  2. Children's Hospital of Richmond, Virginia Commonwealth University Children's Hospital of Richmond, Richmond, Virginia, USA

    Michael S. Schechter

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Shimbori, C., Bellaye, PS., Kolb, P., Kolb, M. (2017). Idiopathic Pulmonary Fibrosis. In: Azuma, A., Schechter, M. (eds) Treatment of Cystic Fibrosis and Other Rare Lung Diseases. Milestones in Drug Therapy. Springer, Basel. https://doi.org/10.1007/978-3-0348-0977-1_3

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