Coagulation and fibrinolytic activities were studied in 18 subjects with Behçet's disease and compared with results from 14 matched control patients suffering from sero-negative arthritis. Significantly higher plasma concentrations (median and range) were found in Behçet's patients for the following variables: fibrinogen 3.7 (1.7-6.9) vs 3.0 (2.0-5.1) g/1, p <0.05; von Willebrand factor antigen, 115 (72-344) vs 74 (60-119)%, p <0.002; plasminogen activator activity (106/ECLT2) 219 (94-329) vs 137 (78-197) units, p <0.002; tissue plasminogen activator inhibitor (t-PA-I) activity, 9.1 (5.5-19.3) vs 5.1 (1.8-12.0) IU/ml, p <0.002; and PAI-1 antigen, 13.9 (4.5-20.9) vs 6.4 (2.4-11.1) ng/ml, p <0.002. Protein C antigen was significantly lower: 97 (70-183) vs 126 (96-220)%, p <0.02. No differences were observed in antithrombin III activity or antigen, factor VIII coagulant activity, fibrinopeptides A and Bβ15-42, plasminogen, α-2-antiplasmin, functional and immunological tissue-plasminogen activator, thrombin-antithrombin complexes and D-dimer. Levels of tissue plasminogen activator inhibitor (activity and antigen) correlated with disease activity while fibrinogen and von Willebrand factor concentrations did not. Seven of the 18 subjects with Behçet's disease had suffered thrombotic events but it was not possible to distinguish these from the 11 patients without thrombosis using the assays performed. The results suggest the abnormal fibrinolytic activity in Behçet's disease is due to increased inhibition of tissue plasminogen activator. No abnormality of coagulation or fibrinolytic activity specific to Behçet's disease was detected.
References
1
Chajek T,
Fainaru M.
Behçet's disease. Report of 41 cases and a review of the literature. Medicine 1975; 54: 179-96
5
Kluft C,
Michiels JJ,
Wijngaards G.
Factual or artificial inhibition of fibrinolysis and the occurrence of venous thrombosis in 3 cases of Behçet's disease. Scand J Haematol 1980; 25: 423-30
9
Inaba G.
(ed)
Behçet's Disease. Pathogenic Mechanisms and Clinical Features. Proceedings of an International Conference on Behçet's disease, 1981.. Tokyo University Press, Tokyo: 1982
13
Matsuda T,
Kaneko K,
Hoshi K,
Mizushima Y.
Platelet function in Behçet's disease. In: Recent Advances in Behçet's Disease..
Lehner T,
Barnes CG.
(eds) Royal Society of Medicine Services, London: 1986. p 133
16
Yazici H,
Tunzun Y,
Pazarli H.
et al.
Influence of age of onset and patients sex on the prevalence and severity of manifestations of Behçet's syndrome. Ann Rheum Dis 1984; 43: 783-9
19
Bertina RM,
Broekmans AW,
van der Linden IK,
Mertens K.
Protein C deficiency in a Dutch family with thrombotic disease. Thromb Haemostas 1982; 48: 1-5
22
Verheijen JH,
Chang GTG,
Kluft C.
Evidence for the occurrence of a fast acting inhibitor for tissue type plasminogen activator in human palsma. Thromb Haemostas 1984; 51: 392-5
24
Kudryk B,
Robinson D,
Netre C,
Hessel B,
Blombôck M,
Blombôck B.
Measurement in human blood of fibrinogen/fibrin fragments containing the Bβ15-42 sequence. Thromb Res 1982; 25: 277-91
25
Knos M,
Friberger P.
Methods for plasminogen determination in human plasma and for streptokinase standardisation. Prog Chem Fibr Thromb 1979; 4: 154-8
27
Conard J,
Horellou MH,
Wechsler B,
Blétry O,
Godeau P,
Samama M.
Coagulation and fibrinolysis in 70 patients with Behçet's disease. In: Recent Advances in Behçet's Disease..
Lehner T,
Barnes CG.
(eds) Royal Society of Medicine Services, London: 1986. p 161
28
Efthimiou J,
Cambridge G,
Harris EN,
Snaith ML,
Hughes GVR.
Anticardiolipin antibodies and vascular complications in Behçet's syndrome. In: Recent Advances in Beheçet's Disease..
Lehner T,
Barnes CG.
(eds) Royal Society of Medicine Services, London: 1986. p 151
