SAVI causes systemic inflammation characterized by vasculopathy, interstitial lung disease, ulcerative skin lesions, and premature death is an autoinflammatory disease caused by gain-of-function mutations in transmembrane protein 173 (TMEM173) gene that encodes STING (139, 140). The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.