Thrombosis occurs in 20 to 30 % of patients with Behcet's disease (BD)
. Most of the reported hemostatic abnormalities are related to the inf
lammatory syndrom. We have assessed the activity of antithrombin III,
protein C and protein S (PS), in 30 patients with ED and in 30 healthy
controls Thrombosis antecedents were found in 16 patients. Antithromb
in III and protein C were within the normal range, however free PS and
PSactivity were significantly decreased in patients as compared to co
ntrol group.PS deficiency detected in eight patients, was associated t
o thrombosis in 6 of them. No correlation was found between free PS/to
tal PS ratio and C4bBP levels. Antibodies to PS were screened by ELISA
and were present in 6 patients,associated to PS deficiency in 4, and
to thrombosis antecedents in 5 cases..PS deficiency was transient in t
wo patients, associated to a persistant antiPS in one of them.These fi
ndings suggest that auto-immune acquired PS deficiency may be involved
in the pathogenesis of thrombotic events in ED. (C) 1997 Elsevier Sci
ence Ltd.