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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.


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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY

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. G. M. Veeger, Hanneke C. Kluin-Nelemans, and Jan van der Meer

From the Division of Haemostasis, Thrombosis and Rheology, Department of Hematology; the Department of Immunology; and the Trial Coordination Centre, University Hospital Groningen, the Netherlands.

Systemic lupus erythematosus (SLE) is associated with an increased risk of venous (VTE) and arterial thromboembolism (ATE). Lupus anticoagulant (LA) and anticardiolipin antibodies (ACAs) 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 ACAs, to the thrombotic risk in a cohort of 144 consecutive patients with SLE. Median follow-up was 12.7 years. VTE had occurred in 10% and ATE in 11% of patients. LA,ACAs, 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 2 disorders. The risk of VTE was 20- and 30-fold higher, respectively, compared with the normal population. In contrast with LA and ACAs, 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 patients with SLE, and potentiate this risk when one of these thrombophilic defects are combined with LA and/or ACAs.


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