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). Here, DNASE1L3 is linked to systemic lupus erythematosus.