Furthermore, the up-regulation of the inflammasome-activating DNA sensor absent in melanoma 2 (AIM2) in a mouse model of lupus and in patients with SLE-associated nephritis (Roberts et al., 2009; Kimkong et al., 2009) and the presence of anti-DNA-PK and anti-RNA-Pol III antibodies in patients with SLE and systemic sclerosis (Cavazzana et al., 2008, 2009) makes the link between DNA sensing and autoimmune disorders worthy of further investigation. Here, PRKDC is linked to systemic lupus erythematosus.