Mutations in the ABCA3 gene are associated with surfactant dysfunction [120,121], familial lung diseases ranging from respiratory failure in newborns or interstitial lung disease in children [122,123] to idiopathic pulmonary fibrosis (IPF) or diffuse parenchymal lung disease in adults [124,125,126]. This evidence concerns the gene ABCA3 and idiopathic pulmonary fibrosis.