Thrombus formation is recognized pathologically in the affected arteri
es and is supposed to play a major role in the pathogenesis of Takayas
u's arteritis; however, hemostatic conditions in this disorder have no
t been elucidated fully. We determined plasma levels of molecular mark
ers for platelet activity (platelet factor 4; PF4, beta-thromboglobuli
n; beta TG), thrombotic status (thrombin-antithrombin III complex; TAT
, fibrinopeptide A; FPA), fibrinolytic status (plasmin-alpha(2)-plasmi
n inhibitor complex; PIC, D-dimer), and endothelial injury (von Willeb
rand factor antigen; vWF:Ag, thrombomodulin; TM) in 30 patients with T
akayasu's arteritis and in 20 age-matched control subjects. Plasma lev
els of PF4, beta TG, TAT, FPA and D-dimer, but not PIC, in patients wi
th Takayasu's arteritis were substantially higher than those in normal
control subjects. The levels of these markers were not different betw
een the active and inactive stages of the disease. Plasma levels of vW
F:Ag in patients with Takayasu's arteritis did not differ significantl
y from those in normal subjects, and plasma levels of TM were signific
antly lower than those in normal subjects. In patients with Takayasu's
arteritis, platelet and coagulation activities are significantly incr
eased, leading to hypercoagulable state and thrombus formation, althou
gh there is little, if any, endothelial damage.