Corticosteroids, disease-modifying antirheumatic drugs, intravenous immunoglobulins, and multiple immunosuppressive therapies are not effective in SAVI patients.[6] Because JAKi inhibits the JAK-STAT pathway and blocks the positive feedback loop of IFN synthesis and release, patients with type I interferonopathies are often treated with a JAKi, including some SAVI patients.[24–26] The first JAKi used in SAVI was ruxolitinib, as reported for 3 children in 2016. Here, IFNA1 is linked to STING-associated vasculopathy with onset in infancy.