Mutations in exon 5 of STING lead to functional activation of STING, resulting in the excessive STING-induced IFN signaling, causing a disorder termed SAVI including recurrent fever, ulcerative skin lesions, vasculitis, and interstitial lung disease (Figure 5B) (18, 20). Here, STING1 is linked to STING-associated vasculopathy with onset in infancy.