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Respiratory Research

, 3:13

First Online: 28 November 2001Received: 05 September 2001Accepted: 24 September 2001


Treatment of idiopathic pulmonary fibrosis patients has evolved very slowly; the fundamental approach of corticosteroids alone or in combination with other immunosuppressive agents has had little impact on long-term survival. The continued use of corticosteroids is justified because of the lack of a more effective alternative. Current research indicates that the mechanisms driving idiopathic pulmonary fibrosis reflect abnormal, dysregulated wound healing within the lung, involving increased activity and possibly exaggerated responses by a spectrum of profibrogenic growth factors. An understanding of the roles of these growth factors, and the way in which they modulate events at cellular level, could lead to more targeted therapeutic strategies, improving patients- quality of life and survival.

Keywordsalveolar epithelial cell apoptosis growth factor idiopathic pulmonary fibrosis myofibroblast AbbreviationsAECalveolar epithelial cell

AMalveolar macrophage

BALCbronchoalveolar lavage cells

CTGFconnective tissue growth factor

ECE-1endothelin-converting enzyme-1

ECMextracellular matrix


IGF-1insulin-like growth factor-1

IGFBPinsulin-like growth factor-binding protein



IP-10interferon-inducing protein-10

IPFidiopathic pulmonary fibrosis

PDGFplatelet-derived growth factor

PDGF-Rplatelet-derived growth factor receptor

PGE2prostaglandin E2

ThT-cell helper

TGF-βtransforming growth factor-beta

TNF-αtumour necrosis factor-alpha

UIPusual interstitial pneumonia

VEGFvascular endothelial growth factor.

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Autor: Jeremy T Allen - Monica A Spiteri


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