Shh signaling is upregulated in animal models and patients with idiopathic pulmonary fibrosis (IPF), other interstitial lung diseases and nonalcoholic fatty liver disease (NAFLD), with nuclear accumulation of Gli1, Gli2 in the fibrotic areas and an increased expression of the Shh downstream target genes. Here, GLI1 is linked to idiopathic pulmonary fibrosis.