Mutations in TREX1 cause a spectrum of type I IFN-dependent autoinflammatory and autoimmune phenotypes including Aicardi–Goutières syndrome (AGS), familial chilblain lupus, retinal vasculopathy with cerebral leukodystrophy (RVCL) and systemic lupus erythematosus (SLE)5, 6, 7, 8, 9. This evidence concerns the gene TREX1 and systemic lupus erythematosus.