Lipids - two sides of the same coin in lung fibrosis.

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive extracellular matrix deposition in the lung parenchyma leading to the destruction of lung structure, respiratory failure and premature death. Recent studies revealed that the pathogenesis of IPF is associated with alterations in the synthesis and the activity of lipids, lipid regulating proteins and cell membrane lipid transporters and receptors in different lung cells. Furthermore, deregulated lipid metabolism was found to contribute to the profibrotic phenotypes of lung fibroblasts and alveolar epithelial cells. Consequently, several pharmacological agents, targeting lipids, lipid mediators, and lipoprotein receptors, was successfully tested in the animal models of lung fibrosis and entered early phase clinical trials. In this review, we highlight new therapeutic options to counteract disturbed lipid hemostasis in the maladaptive lung remodeling.