In addition to a clinical overlap in manifestations between AGS and SLE, mutations in AGS genes like TREX1, RNASEH2B and SAMHD1 were identified in SLE patients, strongly suggesting them as risk factors of polygenic lupus and as drivers of monogenic subtypes like familial chilblain lupus.626 Following these fundamental discoveries, the inflammatory components of many syndromes that are caused by deficiencies in DNA repair enzymes like ATM627 or BLM628 and others have also been shown to be driven by excessive cGAS-STING signaling. The gene discussed is SAMHD1; the disease is systemic lupus erythematosus.