Department of Medicine I, Clinical Division of Hematology and Hemostaseology; and.
Department of Laboratory Medicine, Division of Medical and Chemical Laboratory Diagnostics, Medical University of Vienna, Vienna, Austria.
Blood. 2015 May 28;125(22):3477-83. doi: 10.1182/blood-2014-11-611129. Epub 2015 Mar 25.
Data on the clinical course of lupus anticoagulant (LA)-positive individuals with or without thrombotic manifestations or pregnancy complications are limited. To investigate mortality rates and factors that might influence mortality, we conducted a prospective observational study of LA-positive individuals. In total, 151 patients (82% female) were followed for a median of 8.2 years; 30 of the patients (20%) developed 32 thromboembolic events (15 arterial and 17 venous events) and 20 patients (13%) died. In univariable analysis, new onset of thrombosis (hazard ratio [HR] = 8.76; 95% confidence interval [CI], 3.46-22.16) was associated with adverse survival. Thrombosis remained a strong adverse prognostic factor after multivariable adjustment for age and hypertension (HR = 5.95; 95% CI, 2.43-14.95). Concomitant autoimmune diseases, anticoagulant treatment at baseline, or positivity for anticardiolipin- or anti-β2-glycoprotein I antibodies were not associated with mortality. In a relative survival analysis, our cohort of LA positives showed a persistently worse survival in comparison with an age-, sex-, and study-inclusion-year-matched Austrian reference population. The cumulative relative survival was 95.0% (95% CI, 88.5-98.8) after 5 years and 87.7% (95% CI, 76.3-95.6) after 10 years. We conclude that occurrence of a thrombotic event is associated with higher mortality in patients with LA. Consequently, the prevention of thromboembolic events in LA positives might improve survival.
狼疮抗凝物 (LA) 阳性个体的临床病程数据，无论是伴有血栓表现还是妊娠并发症，都很有限。为了调查死亡率和可能影响死亡率的因素，我们对 LA 阳性个体进行了前瞻性观察研究。共有 151 名患者（82%为女性）接受了中位数为 8.2 年的随访；其中 30 名患者（20%）发生了 32 次血栓栓塞事件（15 次动脉事件和 17 次静脉事件），20 名患者（13%）死亡。在单变量分析中，新发生的血栓形成（风险比 [HR] = 8.76；95%置信区间 [CI]，3.46-22.16）与不良预后相关。在多变量调整年龄和高血压后，血栓形成仍然是一个强烈的不良预后因素（HR = 5.95；95%CI，2.43-14.95）。同时存在自身免疫性疾病、基线时抗凝治疗或抗心磷脂抗体或抗 β2-糖蛋白 I 抗体阳性与死亡率无关。在相对生存分析中，与年龄、性别和研究纳入年份匹配的奥地利参考人群相比，我们的 LA 阳性队列的生存情况持续较差。在 5 年后，累积相对生存率为 95.0%（95%CI，88.5-98.8），在 10 年后为 87.7%（95%CI，76.3-95.6）。我们得出结论，LA 阳性患者发生血栓事件与死亡率升高相关。因此，预防 LA 阳性患者的血栓栓塞事件可能会改善生存。
