All patients with monogenic type I interferonopathies, including Aicardi-Goutières syndrome (AGS); proteasome-associated autoinflammatory syndrome (PRAAS); STING-associated vasculopathy with onset in infancy (SAVI); COPA syndrome; spondyloenchondrodysplasia with immune dysregulation (SPENCDI); and other monogenic and polygenic diseases, which are associated with the upregulation of type I IFN signaling, including chronic granulomatous disease (CGD); SLE; and DM; demonstrated high ISs. The gene discussed is STING1; the disease is Aicardi-Goutieres syndrome.