Mounting evidence suggests that STING, a central adaptor protein in the cGAS-STING signaling pathway, is involved in the pathogenesis of autoinflammatory and autoimmune disorders [9,10] , such as systemic lupus erythematosus, Aicardi-Goutières syndrome (AGS), and STING-associated vasculopathy with onset in infancy (SAVI) [11–13] . This evidence concerns the gene STING1 and STING-associated vasculopathy with onset in infancy.