Previously, Goldbach-Mansky and associates described a vascular and pulmonary syndrome in patients with gain-of-function variants in the STING-encoding gene STING1 (formerly known as TMEM173) and suggested the name STING-associated vasculopathy with onset in infancy (SAVI) for this autoinflammatory interferonopathy [62]. The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.