In human, loss-of-function mutations in TREX1 trigger autoimmune diseases, such as Aicardi–Goutières syndrome (AGS), systemic lupus erythematosus (SLE), familial chilblain lupus (FCL), and retinal vasculopathy with cerebral leukodystrophy (RVCL) (84, 85). Here, TREX1 is linked to systemic lupus erythematosus.