open access

Vol 82, No 1 (2014)
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Submitted: 2013-12-20
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Immunopathogenesis of idiopathic pulmonary fibrosis

Urszula Demkow
DOI: 10.5603/PiAP.2014.0009
·
Pneumonol Alergol Pol 2014;82(1):55-60.

open access

Vol 82, No 1 (2014)
REVIEWS
Submitted: 2013-12-20

Abstract

The paper presents the state of the art in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Both, etiology and pathogenesis of IPF are unclear. The key elements in the pathogenesis of the disease are epithelial destruction and dysregulation of the phenotype of lung fibroblasts. Currently accepted hypothesis claims that IPF is not related to underlying inflammatory state but it is rather a result of pathological interaction between pulmonary epithelium and mesenchyme followed by disturbed healing of damaged alveolar epithelial cells. The function of lung progenitor cells residing in distant lung structures is also impaired. Some scientists claim that genetic defect causes fast shortening of telomeres reducing lung properties of regeneration. According to the current knowledge, IFP is a multifactorial disease resulting from a repeated cycle of injuries followed by pathological regeneration.

Abstract

The paper presents the state of the art in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Both, etiology and pathogenesis of IPF are unclear. The key elements in the pathogenesis of the disease are epithelial destruction and dysregulation of the phenotype of lung fibroblasts. Currently accepted hypothesis claims that IPF is not related to underlying inflammatory state but it is rather a result of pathological interaction between pulmonary epithelium and mesenchyme followed by disturbed healing of damaged alveolar epithelial cells. The function of lung progenitor cells residing in distant lung structures is also impaired. Some scientists claim that genetic defect causes fast shortening of telomeres reducing lung properties of regeneration. According to the current knowledge, IFP is a multifactorial disease resulting from a repeated cycle of injuries followed by pathological regeneration.

Get Citation

Keywords

idiopathic pulmonary fibrosis, pathogenesis, fibroblasts, alveolar epithelial cells

About this article
Title

Immunopathogenesis of idiopathic pulmonary fibrosis

Journal

Advances in Respiratory Medicine

Issue

Vol 82, No 1 (2014)

Pages

55-60

DOI

10.5603/PiAP.2014.0009

Bibliographic record

Pneumonol Alergol Pol 2014;82(1):55-60.

Keywords

idiopathic pulmonary fibrosis
pathogenesis
fibroblasts
alveolar epithelial cells

Authors

Urszula Demkow

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