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Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease that presents with a 5-year survival of 20–30%, hence with a mortality rate as high as many aggressive cancer types.1 According to a current analysis by the British Lung Foundation, the incidence of IPF in the UK has long been underestimated (https://www.blf.org.uk/). In addition, a recent systematic review shows that IPF incidence rates have increased worldwide.2 Two recently approved treatments for IPF, pirfenidone and nintedanib, have been shown to decelerate lung function decline in IPF, but do not halt disease progression or cure the disease.3 ,4 Hence, there is a clear need for novel therapeutic strategies, which manifests in (at time of writing) 46 registered interventional clinical studies for IPF (http://clinicaltrials.gov) and a continued quest for novel drug targets by the scientific community, fuelled by an increased understanding of IPF pathogenesis.5 Considering the substantial pathological heterogeneity of IPF and the multitude of dysregulated signalling pathways,6 it is likely that a successful cure of IPF will involve not a single, but several active agents, specifically targeting different pathomechanistic aspects of IPF.
With de novo drug development being an expensive and time-consuming process, it seems highly sensible to examine whether effective treatment for other disease entities with significant pathomechanistic overlap with IPF is suitable for IPF therapy as well. Notably, repositioning of drugs can shorten drug development time by >50% as phases like chemical optimisation, safety assessment or pharmacokinetic (PK) profiling can be bypassed. …