Loss-of-function mutations in TREX1, the DNA exonuclease that is responsible for degrading cytosolic DNA, lead to the accumulation of cytosolic dsDNA and aberrant activation of cGAS-STING pathway, subsequently trigger autoimmune diseases25,26, such as Aicardi-Goutières syndrome (AGS)27,28 or chilblain lupus29 and systemic lupus erythematosus (SLE)30,31. Here, TREX1 is linked to systemic lupus erythematosus.