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.

IF 1.9 4区 医学 Q3 RHEUMATOLOGY
Lupus Pub Date : 2024-09-01 Epub Date: 2024-08-02 DOI:10.1177/09612033241266989
Prabal Mittal, Marina Pacheco, Laura Trives-Folguera, Joana Rua, Ibrahim Tohidi-Esfahani, Hannah Cohen, Maria Efthymiou, David Isenberg
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引用次数: 0

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; p < 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%, p = 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, p = 0.03) and lower recurrence-free survival after first thrombosis (log-rank test p = 0.01); (ii) lupus anticoagulant (LA)-positivity was associated with higher prevalance of arterial thrombosis (RR 2.69, p = 0.01), and lower prevlance of VTE (RR 0.48, p < 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.

对伴有和不伴有系统性红斑狼疮的血栓性抗磷脂综合征患者的临床和血清学特征进行长达 42 年的随访比较:单中心回顾性研究。
目的评估并发系统性红斑狼疮(SLE)对血栓性抗磷脂综合征(APS)临床病理表现的影响:这项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例为系统性红斑狼疮相关APS(SLE-APS)患者,60例为原发性APS患者:SLE-APS队列和PAPS队列的中位随访时间分别为13.9年(IQR 7.7-19.3)和8.6年(3.5-10.6)。SLE-APS队列中确诊APS的年龄较低(平均35.9岁 vs PAPS:46.7岁;P < 0.05)。各队列的 aPL 亚型分布相似。两个队列中均有略高于一半的患者发生了静脉血栓栓塞(VTE)和动脉血栓。微血管血栓(12.1% 对 0%)和混合型(静脉、动脉和微血管的任意组合)血栓表型(19.0% 对 6.7%,P = 0.05)在系统性红斑狼疮-APS 患者中更为常见。血栓复发率(∼0.5 例/10 患者年)和指数血栓形成后的卡普兰-梅厄无复发生存率相似。在 PAPS 队列中,只有:(i) 三联-aPL 阳性与显著较高的血栓复发率(发病率比 2.22,p = 0.03)和较低的首次血栓后无复发生存率(对数秩检验 p = 0.01);(ii) 狼疮抗凝物(LA)阳性与 LA 阴性相比,动脉血栓形成发生率更高(RR 2.69,P = 0.01),VTE 发生率更低(RR 0.48,P < 0.001):结论:合并系统性红斑狼疮似乎不会改变 APS 患者的长期血栓复发风险或 aPL 表型。与 PAPS 相比,三重 aPL 阳性和 LA 阳性状态对系统性红斑狼疮-APS 患者血栓形成结果的影响可能较小。
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来源期刊
Lupus
Lupus 医学-风湿病学
CiteScore
4.20
自引率
11.50%
发文量
225
审稿时长
1 months
期刊介绍: The only fully peer reviewed international journal devoted exclusively to lupus (and related disease) research. Lupus includes the most promising new clinical and laboratory-based studies from leading specialists in all lupus-related disciplines. Invaluable reading, with extended coverage, lupus-related disciplines include: Rheumatology, Dermatology, Immunology, Obstetrics, Psychiatry and Cardiovascular Research…
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