6 interferon stimulated genes (ISGs)--IFI127, IFI44L, IFIT1, ISG15, RSAD2 and SIGLEC1-- were greatly expressed in a cohort of patients with Aicardi-Goutieres syndrome (AGS). The median fold change of the six ISGs in relation to the median of 29 controls was used to construct an IS for every patient. This publication supports the theory that upregulated type I IFN signaling is an effective biomarker for inflammatory clinical phenotypes. Additionally, repeat testing of the expression of these novel genes can aid in identification of new ISGs and type I interferonopathies.