It has been reported that patients with STING-associated vasculopathy with onset in infancy (SAVI) were characterized by systemic inflammation, severe cutaneous vasculopathy, and interstitial lung disease, which caused by gain-of-function mutations in Tmem173 (also called Sting1), including V147L, N153S, V155M, and V155R (62). Here, STING1 is linked to STING-associated vasculopathy with onset in infancy.