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Pathogenesis of Idiopathic Pulmonary Fibrosis

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Guide to Clinical Management of Idiopathic Pulmonary Fibrosis

Abstract

The etiology of idiopathic pulmonary fibrosis (IPF) remains incompletely understood. Historically, IPF was thought to be a condition characterized by inflammation, leading to fibrosis. The current understanding is based on the concept of repetitive injury with an abnormal wound healing response in a genetically susceptible host [1]. The initial injury appears to be to the alveolar epithelial cells (AECs), particularly type II AECs. The current understanding of the pathogenesis of IPF is depicted in Fig. 4.1.

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Nathan, S.D., Brown, A.W., King, C.S. (2016). Pathogenesis of Idiopathic Pulmonary Fibrosis. In: Guide to Clinical Management of Idiopathic Pulmonary Fibrosis. Adis, Cham. https://doi.org/10.1007/978-3-319-32794-5_4

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  • DOI: https://doi.org/10.1007/978-3-319-32794-5_4

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  • Publisher Name: Adis, Cham

  • Print ISBN: 978-3-319-32792-1

  • Online ISBN: 978-3-319-32794-5

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