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 rare monogenic disorder caused by biallelic mutations in the autoimmune regulator (AIRE) gene on chromosome 21q22.3 [1,2].