SAVI is an auto-inflammatory disease with the gain-of-function mutations of STING (V147L, V147M, N154S, V155M, C206Y, R281Q, R284G), characterized by early onset systemic inflammation, vasculopathy and interstitial lung disease (ILD) (134, 135). The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.