Due to the resemblance of clinical phenotype between AGS and SLE (lupus is considered an interferonopathy), we utilized as control disease a cohort of lupus patients (n = 8), exhibiting active disease, ANA positivity (7/8), anti-dsDNA positivity (6/8) and low complement (7/8). Here, BTG3 is linked to Aicardi-Goutieres syndrome.