Stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), another Type I interferonopathy, and COPA syndrome have a lot in common in terms of pathophysiology and symptoms—both mendelian disorders that cause dysregulation of STING activity, manifesting as diffuse alveolar hemorrhaging (DAH) and interstitial lung disease (ILD) [3]. The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.