AIRE and autoimmune polyendocrine syndrome type 1: Autoimmune polyendocrine syndrome type 1 (APS-1), also known as autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED), is a monogenetic autoimmune disease with an estimated prevalence of 1:100,000 caused by mutations in the autoimmune regulator (AIRE) gene [1,2,3,4,5].