Mendelian autoinflammatory interferonopathies, which are associated with a strong IRG signature, include Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temperature (CANDLE) caused by additive loss-of-function mutations in proteasome components [14–16] and STING-Associated Vasculopathy with onset during Infancy (SAVI), resulting from gain-of-function mutations in the Stimulator of IFN genes (STING) protein [16, 17]. Here, STING1 is linked to vascular disorder.