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.
Abstract
Distinct cell populations contribute to the complex pathogenesis of IPF and systemic sclerosis-associated ILD http://ow.ly/AjFaz
Footnotes
This article has supplementary material available from err.ersjournals.com
Conflict of interest: None declared.
Provenance: Submitted article, peer reviewed.
- Received April 27, 2014.
- Accepted June 15, 2014.
- Copyright ©ERS 2015.
ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
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- Article
- Abstract
- Abstract
- Introduction
- Myofibroblasts: the effector cells of fibrosis
- Fibroblasts: resident cells, apoptosis and the transition to myofibroblasts
- Fibrocytes: circulating precursors of fibroblasts, collagen-producing monocytes and recruitment to the site of injury
- AECs: pivotal cells in IPF and their role in epithelial–mesenchymal transition
- Endothelial cells: vascular damage, EMT and progenitors
- Pericytes and pleural mesothelial cells: underestimated contributors to mesenchymal transition
- Macrophages: monocyte recruitment, alternative activation and defective efferocytosis
- Lymphocytes: Th2-polarised response and antibody production
- Mast cells: fibrogenesis and lung infiltration
- Polymorphonuclear cells: inflammation and reactive oxygen species injury
- Conclusion
- Footnotes
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