Receptor for Advanced Glycation End Products (RAGE) is a 35 kDa transmembrane receptor belonging to the immunoglobulin superfamily, named after its ability to bind to advanced glycation end products. Due to its innate immune inflammatory function and the ability to recognize a specific class of ligands through a common motif, RAGE is generally considered as a pattern recognition receptor. RAGE is associated with several chronic diseases, such as atherosclerosis, peripheral vascular diseases, myocardial infarction, congestive heart failure, diabetes, Alzheimer’s disease, etc. Compared to other tissues, RAGE has the highest expression in the lungs, especially in type I alveolar epithelial cells, while it is not expressed in idiopathic pulmonary fibrosis (IPF), indicating that the expression and regulation of RAGE in the pulmonary system differ from those in the cardiovascular system. Inhibition or knockout of RAGE leads to impaired cell adhesion, increased cell proliferation and migration.