While limited data are available on the sphingolipidome of lung tissues, plasma, or BAL fluid, metabolomics of IPF lungs showed dysregulated SMPD1, SMPD4, and DEGS1 mRNA expression that point to aberrant ceramide production, whereas reduced mRNA expression of ACER3 suggested dysregulated ceramide metabolism [129]. This evidence concerns the gene SMPD1 and idiopathic pulmonary fibrosis.