Figure 2.

Gene expression of selected IFN response genes was determined by Nanostring (NanoString Technologies, Seattle, WA), and an IFN score was calculated for healthy controls, patients with varying autoinflammatory conditions, and our patient (patient O6226). Standardized IFN score (y-axis) is the sum of six Nanostring counts that were standardized by subtracting the mean of healthy controls and dividing by the SD of the healthy controls. IFN response genes assessed in this study were IFI27, IFI44, IFI44L, ISG15, RSAD2, and USP18. Mean and SD of the IFN score are depicted in parentheses for each group of individuals. CANDLE = chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome (mediated by excess IFN); NOMID = neonatal onset multisystem inflammatory disease (mediated by excess IL-1β); SAVI = STING-associated vasculopathy with onset in infancy (mediated by excess IFN).