Recent research suggests that inflammation in AAAs is predominantly mediated by the NLRP3 inflammasome [19], which is a cytosolic multiprotein complex that activates caspase-1 and controls the production of pro-inflammatory cytokines such as interleukin (IL)-1 and IL-18 as well as the stimulation of lytic cell death, known as pyroptosis, and thereby causes inflammation. Here, IL18 is linked to achalasia-alacrima syndrome.