| Idiopathic pulmonary fibrosis. | |
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MedLine Citation:
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PMID: 21719092 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Idiopathic pulmonary fibrosis is a devastating, age-related lung disease of unknown cause that has few treatment options. This disease was once thought to be a chronic inflammatory process, but current evidence indicates that the fibrotic response is driven by abnormally activated alveolar epithelial cells (AECs). These cells produce mediators that induce the formation of fibroblast and myofibroblast foci through the proliferation of resident mesenchymal cells, attraction of circulating fibrocytes, and stimulation of the epithelial to mesenchymal transition. The fibroblast and myofibroblast foci secrete excessive amounts of extracellular matrix, mainly collagens, resulting in scarring and destruction of the lung architecture. The mechanisms that link idiopathic pulmonary fibrosis with ageing and aberrant epithelial activation are unknown; evidence suggests that the abnormal recapitulation of developmental pathways and epigenetic changes have a role. In this Seminar, we review recent data on the clinical course, therapeutic options, and underlying mechanisms thought to be involved in the pathogenesis of idiopathic pulmonary fibrosis. |
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Authors:
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Talmadge E King; Annie Pardo; Moisés Selman |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-6-28 |
Journal Detail:
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Title: Lancet Volume: - ISSN: 1474-547X ISO Abbreviation: - Publication Date: 2011 Jun |
Date Detail:
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Created Date: 2011-7-1 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 2985213R Medline TA: Lancet Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011 Elsevier Ltd. All rights reserved. |
Affiliation:
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Department of Medicine, University of California, San Francisco, CA, USA. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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