The pathogenesis of COPD and IPF: Distinct horns of the same devil?

Respiratory Research

Andrea Rossi³  Venerino Poletti¹  Marco Chilosi² 

[1] Department of Diseases of the Thorax, Morgagni Hospital, Forlì, Italy;Department of Pathology, University of Verona, Italy;Pulmonary Division, Verona General Hospital, Italy

关键词: Caveolin-1;    Notch;    Wnt;    telomere dysfunction;    precursor cell senescence;    IPF;    COPD;   

Others  :  796758
DOI  :  10.1186/1465-9921-13-3

 received in 2011-11-08, accepted in 2012-01-11,  发布年份 2012

【 摘 要 】

New paradigms have been recently proposed in the pathogenesis of both chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF), evidencing surprising similarities between these deadly diseases, despite their obvious clinical, radiological and pathologic differences. There is growing evidence supporting a "double hit" pathogenic model where in both COPD and IPF the cumulative action of an accelerated senescence of pulmonary parenchyma (determined by either telomere dysfunction and/or a variety of genetic predisposing factors), and the noxious activity of cigarette smoke-induced oxidative damage are able to severely compromise the regenerative potential of two pulmonary precursor cell compartments (alveolar epithelial precursors in IPF, mesenchymal precursor cells in COPD/emphysema). The consequent divergent derangement of signalling pathways involved in lung tissue renewal (mainly Wnt and Notch), can eventually lead to the distinct abnormal tissue remodelling and functional impairment that characterise the alveolar parenchyma in these diseases (irreversible fibrosis and bronchiolar honeycombing in IPF, emphysema and airway chronic inflammation in COPD).

【 授权许可】

   
2012 Chilosi et al; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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