Rare familial cases of SLE segregate with autosomal recessive mutations in DNase1 (deoxyribonuclease 1) or DNASE1L3 (deoxyribonuclease 1 like 3, a homologue of DNAse1), extracellular accumulation of DNA, autoantibody production, complement consumption and early-onset SLE [44, 45]. This evidence concerns the gene DNASE1 and systemic lupus erythematosus.