Therefore, the bio-availability of extracellular DNA is dependent mainly on the activity of these two enzymes whose importance is strengthened by the finding that genetic conditions carrying molecular defects in DNASE1 or DNASE1L3 genes are associated with severe forms of pediatric SLE or with other forms of autoimmune disease such as rheumatoid arthritis and sclerodermia (27). This evidence concerns the gene DNASE1 and systemic lupus erythematosus.