There are further monogenic PIDs with pronounced autoimmunity such as for instance autoimmune polyglandular syndrome type 1, also called autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APS1/APECED) caused by pathogenic variants of AIRE, a transcription factor crucial in the process of the elimination of self-reactive T cells in thymus (4). This evidence concerns the gene AIRE and autoimmune polyendocrine syndrome type 1.