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Blood, 1 July 2004, Vol. 104, No. 1, pp. 143-148.
Prepublished online as a Blood First Edition Paper on March 16, 2004; DOI 10.1182/blood-2003-11-4085.
Previous Article | Next Article 
Submitted December 1, 2003
Accepted February 28, 2004
The contribution of inherited and acquired thrombophilic defects, alone or combined with antiphospholipid antibodies, to venous and arterial thromboembolism in patients with systemic lupus erythematosus
Jan-Leendert P Brouwer*, Marc Bijl, Nic J Veeger, Hanneke C Kluin-Nelemans, and Jan van der Meer
Division of Haemostasis, Thrombosis and Rheology, Hematology, University Hospital Groningen, Groningen, The Netherlands
Division of Haemostasis, Thrombosis and Rheology, Immunology, University Hospital Groningen, Groningen, The Netherlands
Division of Haemostasis, Thrombosis and Rheology, Trial Coordination Centre, University Hospital Groningen, Groningen, The Netherlands
* Corresponding author; email: j.l.p.brouwer{at}int.azg.nl.
Systemic lupus erythematosus (SLE) is associated with an increased risk of venous (VTE) and arterial thromboembolism (ATE). Lupus anticoagulant (LA) and anticardiolipin antibodies (ACA) are established risk factors. We assessed the contribution of deficiencies of antithrombin, protein C, total protein S, factor V Leiden, the prothrombin G20210A mutation and APC resistance , either alone or in various combinations with LA and/ or ACA, to the thrombotic risk in a cohort of 144 consecutive SLE patients. Median follow-up was 12.7 years. VTE had occurred in 10% and ATE in 11% of patients. LA, ACA, factor V Leiden and the prothrombin mutation were identified as risk factors for VTE. Annual incidences of VTE were 2.01 (0.74-4.37) in patients with one of these disorders and 3.05 (0.63-8.93) in patients with two disorders. The risk of VTE was 20- and 30-fold higher, respectively, compared to the normal population. In contrast with LA and ACA, thrombophilic disorders did not influence the risk of ATE. In conclusion, factor V Leiden and the prothrombin mutation contribute to the risk of VTE in SLE patients, and potentiate this risk when one of these thrombophilic defects are combined with LA and/ or ACA.

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