Gain-of-function mutations in the STING-encoding gene TMEM173 maintains STING in an overactivated state which leads to a persistent “IFN signal”, and drives a systemic and debilitating inflammatory condition known as STING-associated vasculopathy with onset in infancy (SAVI) (192). This evidence concerns the gene STING1 and STING-associated vasculopathy with onset in infancy.