Clinical Immunology, Angioedema and Allergy Unit, The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Ramat Gan, Israel.
Department of Internal Medicine "C", Shamir Medical Center, Zerifin, Israel.
Front Immunol. 2022 Apr 19;13:843718. doi: 10.3389/fimmu.2022.843718. eCollection 2022.
Antiphospholipid syndrome (APS) is an acquired hypercoagulable condition associated with antiphospholipid antibody (aPL) presence. Data on re-thrombosis following APS-diagnosis are limited.
This is a retrospective analysis of new thrombotic events among primary APS (pAPS) patients followed for up to 15 years in three medical centers in Israel.
Among 312 primary-APS patients, 143 (46%) had new thrombotic event classified to three patterns: (1) associated with heart valve disease (OR 7.24, 95% C.I. 2.26-24.6), hypertension (OR 3, 95% C.I. 1.44-6.25), elevated anti-B2-GPI IgM (OR 1.04, 95% C.I. 0.996-1.08), arterial thrombosis at presentation (OR 1.74 95% C.I. 0.992-3.26), and older age (41 vs. 34 years, p < 0.001). (2) linked with venous thrombosis at presentation (OR 12.9, 95% C.I. 5.27-31.6, p < 0.001), heart valve disease (OR 9.81 95% C.I. 1.82-52.9, p = 0.018), aGAPSS (OR 1.15 95% C.I. 1.02-1.29), and younger age (31 vs. 36.5 years, p = 0.001); and (3) -associated with heart valve disease (OR 40.5 95% C.I. 7.7-212) and pulmonary embolism (OR 7.47 95% C.I. 1.96-28.5). A 4th variant "the Breakthrough pattern" defined by re-thrombosis despite prophylactic therapy was observed in 100/143 (70%) patients and linked with heart valve disease (OR 8. 95% C.I. 2.43-26.3), venous thrombosis at presentation (OR 2.61 95% C.I. 1.47-4.66), leg ulcers (OR 12.2, 95% C.I. 1.4-107), hypertension (OR 1.99, 95% C.I. 0.92-4.34), and higher aGAPSS (OR 1.08, 95% C.I. 0.99-1.18).
In this real-life observation, re-thrombosis was common among pAPS patients including in those recommended to receive prophylactic therapy. Different patterns of recurrence were identified and linked with presenting symptoms, specific serological markers, APS manifestations, and comorbidities. Studies that will address interventions to prevent recurrences of APS-related events are needed.
抗磷脂综合征（APS）是一种与抗磷脂抗体（aPL）存在相关的获得性高凝状态。关于 APS 诊断后再血栓形成的数据有限。
这是在以色列的三个医疗中心对多达 15 年的原发性 APS（pAPS）患者进行的新血栓形成事件的回顾性分析。
在 312 例原发性 APS 患者中，143 例（46%）有新的血栓形成事件，分为三种模式：（1）与心脏瓣膜病相关（OR 7.24，95%CI 2.26-24.6），高血压（OR 3，95%CI 1.44-6.25），升高的抗 B2-GPI IgM（OR 1.04，95%CI 0.996-1.08），初诊时动脉血栓形成（OR 1.74，95%CI 0.992-3.26），年龄较大（41 岁 vs. 34 岁，p<0.001）。（2）与初诊时静脉血栓形成相关（OR 12.9，95%CI 5.27-31.6，p<0.001），心脏瓣膜病（OR 9.81，95%CI 1.82-52.9，p=0.018），aGAPSS（OR 1.15，95%CI 1.02-1.29），年龄较小（31 岁 vs. 36.5 岁，p=0.001）；（3）与心脏瓣膜病相关（OR 40.5，95%CI 7.7-212）和肺栓塞（OR 7.47，95%CI 1.96-28.5）相关。观察到 100/143（70%）患者存在“突破性模式”的第 4 种变体，即尽管进行了预防性治疗仍发生再血栓形成，该变体与心脏瓣膜病（OR 8.95%CI 2.43-26.3），初诊时静脉血栓形成（OR 2.61，95%CI 1.47-4.66），下肢溃疡（OR 12.2，95%CI 1.4-107），高血压（OR 1.99，95%CI 0.92-4.34）和较高的 aGAPSS（OR 1.08，95%CI 0.99-1.18）相关。
在这项真实世界的观察研究中，pAPS 患者包括建议接受预防性治疗的患者中再血栓形成较为常见。确定了不同的复发模式，并与首发症状、特定的血清学标志物、APS 表现和合并症相关。需要开展研究以探讨预防 APS 相关事件复发的干预措施。
