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 with chronic ulceration; and progressive interstitial pulmonary fibrosis and associated pulmonary hypertension [104, 111]. Here, STING1 is linked to pulmonary hypertension.