A second family with a 5-year-old girl was referred to the Pediatric rheumatology unit, Karolinska University hospital, with suspected vasculitis and lung involvement and was treated with B-cell depleting therapy (anti-CD20, Rituximab), but subsequently was found to have a STING1 (c.463G > A, p.Val155Met) mutation confirming the diagnosis of SAVI (Fig. 1D). This evidence concerns the gene STING1 and STING-associated vasculopathy with onset in infancy.