These monogeneic disorders, some of which include Aicardi-Goutières syndrome (AGS), monogenetic forms of lupus, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures (CANDLE), stimulator of IFN genes (STING)-associated vasculopathy with onset in infancy (SAVI), are caused by a mutation leading to upregulation of type I IFN, which ultimately is involved in the pathogenesis of the diseases [23]. Here, STING1 is linked to Aicardi-Goutieres syndrome.