Clinically, we analyzed peripheral blood and kidney tissues from non-renal SLE and LN patients and confirmed that Dll4 expression correlated with the disease activity of LN.<h4>Conclusions</h4>Our findings suggest that abnormal EC activation may be associated with LN progression, potentially through promoting MC migration and proliferation via the Dll4/Notch3 axis. This evidence concerns the gene NOTCH3 and systemic lupus erythematosus.