The production of high levels of anti–type I IFN auto-Abs may be genetically driven and may occur in early childhood, as in patients with APS-1 carrying germline biallelic or monoallelic rare deleterious variants of AIRE (Levin, 2006; Meager et al., 2006; Meyer et al., 2016; Oftedal et al., 2015). This evidence concerns the gene DDX41 and autoimmune polyendocrine syndrome type 1.