Brouwer Jan-Leendert P, Bijl Marc, Veeger Nic J G M, Kluin-Nelemans Hanneke C, van der Meer Jan
Division of Haemostasis, Thrombosis and Rheology, Department of Hematology, University Hospital Groningen The Netherlands.
Blood. 2004 Jul 1;104(1):143-8. doi: 10.1182/blood-2003-11-4085. Epub 2004 Mar 16.
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.
系统性红斑狼疮（SLE）与静脉血栓栓塞（VTE）和动脉血栓栓塞（ATE）风险增加相关。狼疮抗凝物（LA）和抗心磷脂抗体（ACA）是已确定的风险因素。我们评估了抗凝血酶、蛋白C、总蛋白S、因子V莱顿、凝血酶原G20210A突变以及活化蛋白C抵抗缺陷单独或与LA和/或ACA以各种组合形式对144例连续SLE患者血栓形成风险的影响。中位随访时间为12.7年。10%的患者发生了VTE，11%的患者发生了ATE。LA、ACA、因子V莱顿和凝血酶原突变被确定为VTE的风险因素。患有这些疾病之一的患者VTE年发病率为2.01（0.74 - 4.37），患有两种疾病的患者为3.05（0.63 - 8.93）。与正常人群相比，VTE风险分别高出20倍和30倍。与LA和ACA不同，易栓症并未影响ATE风险。总之，因子V莱顿和凝血酶原突变会增加SLE患者的VTE风险，当这些易栓缺陷之一与LA和/或ACA同时存在时，会进一步增加这种风险。
