Stimulator of interferon genes (STING)-associated vasculitis of infancy (SAVI) arises from sporadic/dominant mutation in the TMEM173 gene and presents early in life with a vasculitic rash affecting the cheeks, nose, and peripheries (Fig. 3) with chronic ulceration; and progressive interstitial pulmonary fibrosis and associated pulmonary hypertension [56, 62]. This evidence concerns the gene STING1 and STING-associated vasculopathy with onset in infancy.