Department of Medicine II, Hokkaido University Graduate School of Medicine, N15 W7, Kita-ku, Sapporo, 060-8638, Japan.
Curr Rheumatol Rep. 2017 Aug;19(8):51. doi: 10.1007/s11926-017-0674-4.
Antiphospholipid syndrome (APS) is a clinical disorder characterised by thrombosis and/or pregnancy morbidity in the persistence of antiphospholipid (aPL) antibodies that are pathogenic and have pro-coagulant activities. Thrombosis in APS tends to recur and require prophylaxis; however, the stereotypical treatment for APS patients is inadequate and stratification of the thrombotic risks is important as aPL are prevalently observed in various diseases or elderly population.
It is previously known that the multiple positive aPL or high titre aPL correlate to thrombotic events. To progress the stratification of thrombotic risks in APS patients and to quantitatively analyse those risks, antiphospholipid score (aPL-S) and the Global Anti-phospholipid Syndrome Score (GAPSS) were defined. These scores were raised from the large patient cohort data and either aPL profile classified in detail (aPL-S) or simplified aPL profile with classical thrombotic risk factors (GAPSS) was put into a scoring system. Both the aPL-S and GAPSS have shown a degree of accuracy in identifying high-risk APS patients, especially those at a high risk of thrombosis. However, there are several areas requiring improvement, or at least that clinicians should be aware of, before these instruments are applied in clinical practice. One such issue is standardisation of the aPL tests, including general testing of phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT). Additionally, clinicians may need to be aware of the patient's medical history, particularly with respect to the incidence of SLE, which influences the cutoff value for identifying high-risk patients.
抗磷脂综合征（APS）是一种临床疾病，其特征是在持续存在致病性和促凝活性的抗磷脂（aPL）抗体的情况下发生血栓形成和/或妊娠并发症。APS 中的血栓形成倾向于复发，需要预防；然而，APS 患者的典型治疗方法并不充分，对血栓形成风险进行分层很重要，因为 aPL 在各种疾病或老年人群中普遍存在。
先前已知多种阳性 aPL 或高滴度 aPL 与血栓事件相关。为了推进 APS 患者血栓形成风险的分层，并对这些风险进行定量分析，定义了抗磷脂评分（aPL-S）和全球抗磷脂综合征评分（GAPSS）。这些评分是从大量患者队列数据中得出的，要么详细分类 aPL 谱（aPL-S），要么简化包含经典血栓形成危险因素的 aPL 谱（GAPSS），并将其纳入评分系统。aPL-S 和 GAPSS 都在一定程度上显示了识别高危 APS 患者的准确性，尤其是那些有高血栓形成风险的患者。然而，在这些工具应用于临床实践之前，有几个方面需要改进，或者至少临床医生应该意识到。一个这样的问题是 aPL 检测的标准化，包括磷脂酰丝氨酸依赖性抗凝血酶原抗体（aPS/PT）的一般检测。此外，临床医生可能需要了解患者的病史，特别是与 SLE 的发生率有关的病史，这会影响识别高危患者的截止值。
