Various gain-of-function mutants and noncoding SNPs of MITA are found in patients with MITA-associated vasculopathy with onset in infancy (SAVI) and systemic inflammatory or autoimmune diseases characterized by elevated expression of type I IFNs, IFN-stimulated genes (ISGs) and proinflammatory cytokines [24–27]. This evidence concerns the gene STING1 and STING-associated vasculopathy with onset in infancy.