Notably, a considerable number of type I interferonopathies, including Aicardi-Goutières syndrome (AGS), proteasome-associated autoinflammatory syndrome (PRAAS) and STING-associated vasculopathy with onset in infancy (SAVI), manifest clinical and laboratory features akin to SLE (Table 1). Here, STING1 is linked to Aicardi-Goutieres syndrome.