Currently, there is scarce of evidence suggesting the relationship between motile cilia impairment and the development of pulmonary fibrosis, while several factors dysregulated in IPF have been reported to interfere ciliogenesis or ciliated cell differentiation, such as MMP7 (Yang et al., 2013; Gharib et al., 2013). This evidence concerns the gene MMP7 and idiopathic pulmonary fibrosis.