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Idiopathic Pulmonary Fibrosis: Molecular Mechanisms and Possible Therapeutic Strategies

  • Chapter
Inflammation

Abstract

Idiopathic pulmonary fibrosis (IPF) is a devastating disease with an almost universally terminal outcome. In recent years much insight has been gained into the pathogenesis of IPF from both a bleomycin mice-model as well as ex vivo human tissue studies. Alveolar damage and inflammation of unknown etiology, eventually leading to interstitial fibrosis, characterize IPF. Apoptosis has emerged as an important factor in the pathogenesis of IPF. This review will outline the current understanding of the immunological and molecular mechanisms underlying IPF and discuss new therapeutic strategies.

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Abbreviations

BAL:

broncho-alveolar lavage

EBV:

Epstein-Barr virus

ET-1:

endothelin-1

FasL:

Fas ligand

G-CSF:

granulocyte colony-stimulating factor

IFN-γ:

interferon γ

IL:

interleukin

IPF:

idiopathic pulmonary fibrosis

MAPK:

mitogen-activated protein kinase

MCP:

monocyte-chemoattractant protein

MHC:

major histocompatibility complex

MIP:

macrophage inflammatory protein

TGF-β:

transforming growth factor beta

Th:

T helper

TNF-α:

tumor necrosis factor α

PAI-1:

plasminogen activator inhibitor-1

SCID:

severe combined immunodeficiency

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Authors and Affiliations

  1. Laboratory of Experimental Internal Medicine, Academic Medical Center, G2-130, NL-1105 AZ, Amsterdam, The Netherlands

    Bernt Van Den Blink & Maikel P. Peppelenbosch

  2. Department of Pulmonology, Academic Medical Center, F4-208, Meibergdreef 9, NL-1105 AZ, Amsterdam, The Netherlands

    Henk M. Jansen

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  1. Ludwik Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Wrocław, Poland

    Andrzej Górski , Hubert Krotkiewski  & Michał Zimecki ,  & 

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Van Den Blink, B., Jansen, H.M., Peppelenbosch, M.P. (2001). Idiopathic Pulmonary Fibrosis: Molecular Mechanisms and Possible Therapeutic Strategies. In: Górski, A., Krotkiewski, H., Zimecki, M. (eds) Inflammation. Springer, Dordrecht. https://doi.org/10.1007/978-94-015-9702-9_14

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