In addition to the idiopathic rheumatic diseases, dysregulation of IFN has been identified as the driver of genetically-derived interferonopathy syndromes such as Aicardi–Goutières syndrome (AGS) and STING-associated vasculopathy with onset in infancy (SAVI); it is noteworthy that these genetic syndromes present with clinical features that often overlap with those of idiopathic rheumatic diseases (24). Here, STING1 is linked to Aicardi-Goutieres syndrome.