Besides its role in the physiological host defense, several gain-of-function mutations in the STING-encoding TMEM173 gene have been reported as causative mutations in inflammatory diseases, including STING-associated vasculopathy with onset in infancy (SAVI), systemic lupus erythematosus–like syndromes or familial chilblain lupus (177–180). The gene discussed is STING1; the disease is familial chilblain lupus.