Autophagy in pulmonary fibrosis: friend or foe?

ABSTRACT

Autophagy is an evolutionarily conserved process where long-lived and damaged or-ganelles are degraded. Autophagy has been widely associated with several ageing-process as well in diseases such as neurodegeneration, cancer and fibrosis, and is now being utilised as a target in these diseases. Idiopathic pulmonary fibrosis (IPF) is a progressive, interstitial lung disease with limited treatment options available. It is characterised by abnormal extracellular matrix (ECM) deposition by activated myofibroblasts. It is understood that repetitive micro-injuries to aged-alveolar epithelium combined with genetic factors drive the disease. Several groups have demonstrated that autophagy is altered in IPF although whether autophagy has a protective effect or not is yet to be determined. Autophagy has also been shown to influence many other processes including epithelial-mesenchymal transition (EMT) and endothelial-mesenchymal transition (EndMT) which are known to be key in the pathogenesis of IPF. In this review, we summarise the findings of evidence of altered autophagy in IPF lungs, as well as examine its roles within lung fibrosis. Given these findings, together with the growing use of autophagy manipulation in a clinical setting, this is an exciting area for further research in the study of lung fibrosis.