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Review
. 2015 Mar;24(135):102-14.
doi: 10.1183/09059180.00003214.

Cellular interactions in the pathogenesis of interstitial lung diseases

Gianluca Bagnato  1 , Sergio Harari  2
Affiliations
Review

Cellular interactions in the pathogenesis of interstitial lung diseases

Gianluca Bagnato et al. Eur Respir Rev. 2015 Mar.

Abstract

Interstitial lung disease (ILD) encompasses a large and diverse group of pathological conditions that share similar clinical, radiological and pathological manifestations, despite potentially having quite different aetiologies and comorbidities. Idiopathic pulmonary fibrosis (IPF) represents probably the most aggressive form of ILD and systemic sclerosis is a multiorgan fibrotic disease frequently associated with ILD. Although the aetiology of these disorders remains unknown, in this review we analyse the pathogenic mechanisms by cell of interest (fibroblast, fibrocyte, myofibroblast, endothelial and alveolar epithelial cells and immune competent cells). New insights into the complex cellular contributions and interactions will be provided, comparing the role of cell subsets in the pathogenesis of IPF and systemic sclerosis.

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Figures

FIGURE 1
FIGURE 1
This schematic view of the morpho-functional unit of the lung (alveolus) depicts the main differences in cellular composition in idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) compared with normal physiological cellular components. Th2: T-helper cell type 2; AEC: alveolar epithelial cell.
FIGURE 2
FIGURE 2
Cellular players and molecules in idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease. Cells inside dashed lines are relevant to IPF pathogenesis only. ROS: reactive oxygen species; TGF: transforming growth factor; IL: interleukin; MCP: monocyte chemotactic protein; PDGF: platelet-derived growth factor; ET: endothelin; PMNC: peripheral blood mononuclear cell; EMT: epithelial–mesenchymal transition; CTGF: connective tissue growth factor; Th2: T-helper cell type 2; AEC: alveolar epithelial cell; PMC: pleural mesothelial cell.
See this image and copyright information in PMC

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