Furthermore, Abe et al. in a nationwide Japanese cohort, reported that all AGS patients harbouring SAMHD1 mutations developed autoimmune diseases, including systemic lupus erythematosus and, notably, the first described case of Sjögren’s syndrome, highlighting the particularly strong association of SAMHD1 with autoimmunity [18]. Here, SAMHD1 is linked to systemic lupus erythematosus.