When it comes to the upstream and downstream mediators of NETs formation, both NOX2-deficient strain and PAD4-deficient strain exhibited impaired induction of NET formation, yet had elevated levels of antinuclear autoantibodies (ANAs) and exacerbated glomerulonephritis in the pristane-induced model of lupus. Here, PADI4 is linked to systemic lupus erythematosus.