Abbreviations
heterogeneous disease, respiratory diseases, exhaled breath condensate, IPF
RESPIRATORY DISEASE, IDIOPATHIC PULMONARY FIBROSIS, DISEASE PROGRESSION, LUNG FIBROSIS, DISEASE
percent predictedacute exacerbation in idiopathic pulmonary fibrosisautotaxinCC motif chemokine ligand 17CC motif chemokine ligand 18cartilage oligomeric matrix proteinCXC chemokine ligand 13diffusion capacity of carbon monoxidefatty acidsfalse discovery rateforced vital capacityhigh-resolution computed tomographyidiopathic pulmonary fibrosisliquid chromatography-mass spectrometrylysophosphatidic acidlysophosphatidylcholinematrix metalloproteinase 7phosphatidylcholinephosphatidylethanolamineperiostinquadrupole linear ion trapsurfactant protein-Dsoluble receptor for advanced glycation end productstriglyceridestransforming growth factor-betatype II helper T cellschitinase-3-like protein 1Idiopathic pulmonary fibrosis (IPF) is a heterogeneous disease of unknown etiology and high mortality with a median survival of about 3 years from the time of disease diagnosis (The autotaxin-lysophosphatidic acid (ATX-LPA) signaling pathway has been implicated in lung fibrosis in preclinical models (LPA species vary in length and fatty acid saturation. In patients with IPF, LPA22:4 levels were elevated in the exhaled breath condensate compared to the control (As different LPA and lipid species have been reported in IPF and other respiratory diseases, we performed targeted assays that measured LPA species (LPA16:0, 16:1, 18:0, 18:1, 18:2, 20:4, and 22:4) as well as global lipidomic profiling to identify specific LPA and lipid species that were dysregulated in patients with IPF and assessed the relationship between the dysregulated lipids and disease progression.