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Cell and Tissue Research

, Volume 367, Issue 3, pp 607–626 | Cite as

Tissue remodelling in pulmonary fibrosis

  • Lars KnudsenEmail author
  • Clemens Ruppert
  • Matthias Ochs
Review

Abstract

Many lung diseases result in fibrotic remodelling. Fibrotic lung disorders can be divided into diseases with known and unknown aetiology. Among those with unknown aetiology, idiopathic pulmonary fibrosis (IPF) is a common diagnosis. Because of its progressive character leading to a rapid decline in lung function, it is a fatal disease with poor prognosis and limited therapeutic options. Thus, IPF has motivated many studies in the last few decades in order to increase our mechanistic understanding of the pathogenesis of the disease. The current concept suggests an ongoing injury of the alveolar epithelium, an impaired regeneration capacity, alveolar collapse and, finally, a fibroproliferative response. The origin of lung injury remains elusive but a diversity of factors, which will be discussed in this article, has been shown to be associated with IPF. Alveolar epithelial type II (AE2) cells play a key role in lung fibrosis and their crucial role for epithelial regeneration, stabilisation of alveoli and interaction with fibroblasts, all known to be responsible for collagen deposition, will be illustrated. Whereas mechanisms of collagen deposition and fibroproliferation are the focus of many studies in the field, the awareness of other mechanisms in this disease is currently limited to biochemical and imaging studies including quantitative assessments of lung structure in IPF and animal models assigning alveolar collapse and collapse induration crucial roles for the degradation of the lung resulting in de-aeration and loss of surface area. Dysfunctional AE2 cells, instable alveoli and mechanical stress trigger remodelling that consists of collapsed alveoli absorbed by fibrotic tissue (i.e., collapse induration).

Keywords

Idiopathic pulmonary fibrosis Mechanical stress Alveolar collapse Alveolar epithelial type 2 cells Collapse induration 

Notes

Acknowledgments

The authors thank Marita Peter for excellent assistance in preparing Fig. 3. This work was supported by the German Research Foundation (DFG, KN 916 1-1) and via the German Center for Lung Research (DZL) by the Federal Ministry of Education and Research (BMBF).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Lars Knudsen
    • 1
    • 2
    • 3
    Email author
  • Clemens Ruppert
    • 4
    • 5
  • Matthias Ochs
    • 1
    • 2
    • 3
  1. 1.Institute of Functional and Applied AnatomyHannover Medical SchoolHannoverGermany
  2. 2.Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), Member of the German Center for Lung Research (DZL)Hannover Medical SchoolHannoverGermany
  3. 3.REBIRTH, Cluster of ExcellenceHannover Medical SchoolHannoverGermany
  4. 4.Department of Internal MedicineJustus-Liebig-University GiessenGiessenGermany
  5. 5.Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL)Universities of Giessen and MarburgGiessenGermany

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