Annexin V has an important role in the regulation of apoptosis and antibodies directed against it have been shown to lead to apoptosis of vascular endothelial cells. To evaluate the role of anti-annexin V antibodies (AA5A) in Takayasu's arteritis (TA), we investigated these antibodies in the sera of 66 TA patients, 50 healthy controls and in the follow-up sera of 12 active TA patients by enzyme-linked immunosorbent assay. The AA5A-positive patients were analysed further for the presence of anti-endothelial cell antibodies (AECA) and anticardiolipin antibodies (ACLA) to determine the relationship of AA5A with these autoantibodies. AA5A were observed in 36% (24/66) of the patients versus 6% (3/50) of the controls (P<0.001) and in 53% (19/36) of patients with active TA versus 17% (5/30) of those with inactive disease (P<0.01). Levels of AA5A were also observed to be significantly higher in patients with TA compared to controls (0.557 +/- 0.362 versus 0.259 +/- 0.069; P<0.0001) and in patients with active disease compared to those with inactive disease (0.700 +/- 0.403 versus 0.385 +/- 0.205; P<0.0001). In the follow-up study, 6/12 patients who became inactive during follow-up also showed normalization of AA5A levels. AECA and ACLA were detected in 54% (13/24) and 12% (3/24) of the AA5A-positive patients, respectively. Our results show that a significant proportion of TA patients have AA5A, which exhibit an association with AECA and because they have a correlation with disease activity thus appear to be involved in the disease pathogenesis.