Interestingly, numerous studies have reported that patients with LN and antineutrophil cytoplasmic antibodies (ANCA), specifically those who are anti-myeloperoxidase (MPO)/proteinase 3 (PR3)-positive or double positive for anti-MPO and anti-PR3 antibodies, exhibit serologically active SLE (higher dsDNA antibody titers and lower serum C4 concentrations) compared with ANCA-negative LN patients. Here, C4A is linked to systemic lupus erythematosus.