AGER and achalasia-alacrima syndrome: The binding of RAGE to its ligands not only stimulates the generation of reactive oxygen species (ROS) and the activation of nuclear factor kappa-B (NF-κB), mitogen-activated protein kinase (MAPK), and protein kinase C (PKC) but also induces cytokine production and inflammatory reactions, all of which are involved in the development and progression of AAAs [9].