title: Vascular involvement in Behçet's disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin.
abstract: OBJECTIVE
  Thrombosis, usually venous, occurs in 10% to 25% of patients with Behçet's disease, but its pathogenesis is poorly understood. We evaluated parameters of hemostasis and their relation with thrombosis in a series of patients with Behçet's disease.
  
  
  METHODS
  We studied 38 patients with Behçet's disease (13 with venous thrombosis), 38 patients with venous thrombosis without thrombophilia, and 100 control subjects. Levels or presence of protein C, protein S, antithrombin, methylenetetrahydrofolate reductase C677T, factor V Leiden, prothrombin gene G20210A, antiphospholipid antibodies, plasminogen, tissue-type plasminogen activator (tPA), type-1 tPA inhibitor (PAI-1), PAI-1 4G/5G polymorphism, prothrombin fragment 1+2, plasmin/alpha(2)-antiplasmin complexes, thrombomodulin, and activated factors VII and XII were determined.
  
  
  RESULTS
  There were no deficiencies in protein C, protein S, antithrombin, or factor V Leiden in the patients with Behçet's disease, nor was there evidence of most other thrombotic abnormalities. Compared with control subjects, however, the Behçet's disease group had elevated mean (+/- SD) levels of prothrombin fragment 1+2 (2091 +/- 1323 pmol/L vs. 804 +/- 398 pmol/L, P <0.001), plasmin/alpha2-antiplasmin complexes (410 +/- 220 microg/L vs. 214 +/- 92 microg/L, P <0.001), and thrombomodulin (37 +/- 24 ng/mL vs. 27 +/- 10 ng/mL, P <0.001). These levels did not differ between patients with or without thrombosis.
  
  
  CONCLUSIONS
  Thrombophilic factors do not seem to explain most thromboses in Behçet's disease. There is increased thrombin generation, fibrinolysis, and thrombomodulin in Behçet's disease, but these abnormalities are not related to thrombosis.
authors.affiliation: Systemic Autoimmune Diseases Unit, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, Barcelona, Spain.
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Josep
authors.initials: J
authors.lastname: Font
authors.forename: Dolors
authors.initials: D
authors.lastname: Tàssies
authors.forename: Antonio
authors.initials: A
authors.lastname: Vidaller
authors.forename: Ramon
authors.initials: R
authors.lastname: Deulofeu
authors.forename: Alfons
authors.initials: A
authors.lastname: López-Soto
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
authors.forename: Antoni
authors.initials: A
authors.lastname: Ordinas
authors.forename: Miguel
authors.initials: M
authors.lastname: Ingelmo
authors.forename: Joan-Carles
authors.initials: JC
authors.lastname: Reverter
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001528:Behcet Syndrome / Q000097:blood* / Q000150:complications*; D001777:Blood Coagulation; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood / Q000378:metabolism*; D001779:Blood Coagulation Factors / Q000378:metabolism*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018180:Thrombomodulin / Q000097:blood; D019851:Thrombophilia / Q000097:blood / Q000150:complications; D020246:Venous Thrombosis / Q000097:blood* / Q000150:complications*
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; D018180:Thrombomodulin