Mutations in TREX1 have been associated with a broad spectrum of autoimmune and inflammatory phenotypes, including Aicardi-Goutières syndrome (AGS), familial chilblain lupus (FCL), systemic lupus erythematosus (SLE), and retinal vasculopathy with cerebral leukodystrophy (RVCL) (Crow and Rehwinkel, 2009). This evidence concerns the gene TREX1 and systemic lupus erythematosus.