DNASE1L3 and systemic lupus erythematosus: Compared with DNASE 1, DNASE1L3 has the ability to cleave both ‘naked’ DNA and DNA in microparticles of lipids and proteins.2 3 Such an essential function of digesting chromatin microparticles produced by apoptotic cells is important in the elimination of antigenic DNA and autoreactivity.4, 6 The loss of such function has been linked to the development of SLE in both animal models and humans.7, 16 A loss-of-function variant in the DNASE1L3 gene in patients was first reported by Al-Mayouf et al in seven Arab families.9