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.
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