Defective fibrinolysis in Behcet's syndrome: significance and possible mechanisms.

Abstract

SUMMARY Reduced fibrinolytic activity has frequently been reported in Behqet's syndrome, but both the underlying mechanism and its relation to the development of thrombosis are unclear.The fibrinolytic activity was studied in seven patients with Behqet's syndrome and 12 patients with idiopathic oral ulceration. The patients with Behqet's disease had significantly reduced resting tissue plasminogen activator(t-PA) both by euglobulin fibrin plate lysis and enzyme linked immunosorbent assay (ELISA), but the tissue plasminogen activator inhibitor(t-PAI) was not significantly different from the control value. After stimulation by venous occlusion the euglobulin fibrin plate lysis results showed three good responders and four poor responders, but neither t-PA antigen nor t-PAI concentrations showed any clear difference between the two groups. Interestingly, three out of the four patients with a history of thrombosis were good responders. The group with oral ulceration showed no significant difference from the controls for any of these measurements. These findings suggest that the reduced resting fibrinolytic activity is usually due to decreased production of t-PA, but that the defective response to stimulation may be multifactorial in origin. The fibrinolytic abnormalities appear unrelated to the development of thrombosis.

Plasma Lipoprotein(a) and Its Relationship with Disease Activity in Patients with Behçet’s Disease

“…Moreover, recent reports showed that Lp(a) has a direct action on endothelial function and induces the production of tissue plasminogen activator inhibitor by endothelium cells (24 is not yet understood. On the other hand, it was reported that the reduced fibrinolytic activity caused by decreased production of tissue plasminogen activator and its increased production by endothelium due to vascular damage may contribute to thrombotic events in the patients (3,6). The mean Lp(a) level (0.312 g/1) in patients with Behfefs disease was found to be similar to the cut-off point.…”

“…Sixteen subjects (35.5%) among the patients with Behfet's disease had values higher than the cut-off point, and may therefore be at a much greater risk for thrombo-atherogenesis. It has been previously reported that the thrombotic tendency and the abnormal fibrinolysis is related to the disease activity (6). Also, Lp(a) is known as an acute phase protein and is significantly correlated with acute phase reactants (13,14).…”

“…ι ι rv // / · n / , thought to be association of defective fibrinolysis (6 (3,4). Thrombosis is one ' ô f the vascular manifestations and is seen in about one Lipoprotein(a) [Lp(a)] described by I3et\\> in 1963 const!· third of the patients (5), The basis of thrombosis in the tutcs a newly recognised cardiovascular risk factor with patients is unclear.…”

Response: Is Mean Platelet Volume Increased in Behcet's Disease with Thrombosis?

“…Similarly, investigations on the fibrinolytic parameters yielded contradictory results in the literature. The tissue-type plasminogen activator (tPA) antigen values were reported as normal or reduced (Aitchison et al 1989;Hamton et al 1991;Haznedaroglu et al1996;Demirer et al 2000). Normal and increased tPA activity has also been found (Hamton et al 1991;Haznedaroglu et al 1996;Demirer et al 2000).…”

Behçet's syndrome in pregnancy

“…36,37 Behçet's vasculopathy consists of (1) Superficial and deep vein thrombosis -affecting veins anywhere in the body; (2) Arterial aneurysms -due to adventitial inflammation; (3) Pulmonary vasculitis (since pulmonary arteries are in many ways similar to veins with low O 2 concentrations and low pressures).…”