长达 42 年的随访：血栓性抗磷脂综合征患者伴或不伴相关系统性红斑狼疮的临床和血清学特征比较：一项单中心回顾性研究。

Comparison of clinical and serological features in thrombotic antiphospholipid syndrome patients, with and without associated systemic lupus erythematosus, followed for up to 42 years: A single centre retrospective study.

机构信息

Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK.

Department of Haematology, Cancer Institute, University College London, London, United Kingdom.

出版信息

Lupus. 2024 Sep;33(10):1082-1088. doi: 10.1177/09612033241266989. Epub 2024 Aug 2.

DOI:10.1177/09612033241266989

PMID:39092568

Abstract

OBJECTIVE

To assess the impact of concomitant systemic lupus erythematosus (SLE) on the clinicopathological manifestations of thrombotic antiphospholipid syndrome (APS).

METHODS

This single-centre, retrospective study compared clinical and antiphospholipid antibody (aPL) data from 118 patients, 58 with SLE-associated APS (SLE-APS), and 60 with primary APS.

RESULTS

Median follow-up was 13.9 (IQR 7.7-19.3) and 8.6 years (3.5-10.6) for the SLE-APS cohort and PAPS cohort, respectively. Age at diagnosis of APS was lower in the SLE-APS cohort (mean 35.9 vs PAPS: 46.7 years; < 0.05). Distribution of aPL subtypes was similar across cohorts. 198 thrombotic events were identified overall (index plus recurrent), with venous thromboembolism (VTE) and arterial thrombosis each occurring in just over half of patients in both cohorts. Microvascular thrombosis (12.1% vs 0%), and a mixed (any combination of venous, arterial and microvascular) thrombotic phenotype (19.0% vs 6.7%, = 0.05) were more prevalent in SLE-APS patients. Recurrent thrombosis incidence rates (∼0.5 events/10-patient years), and Kaplan-Meier recurrence-free survival after index thrombosis, were similar. In the PAPS cohort, only: (i) triple-aPL-positivity was associated with a significantly higher recurrent thrombosis event rate (incidence rate ratio 2.22, = 0.03) and lower recurrence-free survival after first thrombosis (log-rank test = 0.01); (ii) lupus anticoagulant (LA)-positivity was associated with higher prevalance of arterial thrombosis (RR 2.69, = 0.01), and lower prevlance of VTE (RR 0.48, < 0.001), versus LA-negativity.

CONCLUSION

Concomitant SLE does not appear to modify long-term recurrent thrombosis risk, or aPL phenotypes, in patients with APS. Triple-aPL-positivity and LA-positive status may have less influence on thrombotic outcomes in patients with SLE-APS compared to PAPS.

摘要

目的

评估伴发系统性红斑狼疮（SLE）对血栓性抗磷脂综合征（APS）临床病理表现的影响。

方法

本单中心回顾性研究比较了 118 例患者的临床和抗磷脂抗体（aPL）数据，其中 58 例为伴发 SLE 的 APS（SLE-APS）患者，60 例为原发性 APS 患者。

结果

SLE-APS 队列和 PAPS 队列的中位随访时间分别为 13.9（IQR 7.7-19.3）和 8.6 年（3.5-10.6）。APS 的诊断年龄在 SLE-APS 队列中较低（平均 35.9 岁，而 PAPS 为 46.7 岁；<0.05）。两组患者的 aPL 亚型分布相似。共发现 198 例血栓事件（包括首发和复发），两组患者各有一半以上发生静脉血栓栓塞（VTE）和动脉血栓形成。SLE-APS 患者更常见微血管血栓形成（12.1%比 0%）和混合（任何静脉、动脉和微血管血栓形成的组合）血栓表型（19.0%比 6.7%；=0.05）。SLE-APS 患者的复发血栓形成发生率（~0.5 例/10 患者年）和首发血栓形成后 Kaplan-Meier 无复发生存率相似。在 PAPS 队列中，只有：（i）三抗 aPL 阳性与更高的复发血栓形成事件率显著相关（发生率比 2.22，=0.03）和首发血栓形成后无复发生存率较低（对数秩检验=0.01）；（ii）狼疮抗凝剂（LA）阳性与动脉血栓形成的发生率较高（RR 2.69，=0.01）和静脉血栓栓塞（RR 0.48，<0.001）的发生率较低，与 LA 阴性相比。