Objective To evaluate the significance of anti-endothelial cell antibody (AECA) and the correlation between AECA and clinical features of Behcet's disease (BD). Methods AECA was detected in 73 BD, 60 SLE, 35 SSc, 30 RA, 62 systemic vasculitis patients and 40 health controls by indirect immunofluorescence analysis in which the endothelial cells from umbilical vein were used as the substrate. Results Higher frequency of AECA was found in BD (54.8%) with the specificity of 87.2%, compared to those in SLE (8%), SSc (9%), RA (10%), systemic vasculitis (27%) and healthy controls (2%, P