Specker Christof, Fischer-Betz Rebecca, Dörner Thomas
Klinik für Rheumatologie & Klinische Immunologie, Kliniken Essen-Mitte, Pattbergstr. 1-3, 45239, Essen, Deutschland.
Poliklinik für Rheumatologie und Hiller Forschungszentrum, Universitätsklinikum Düsseldorf, Düsseldorf, Deutschland.
Z Rheumatol. 2020 Apr;79(3):255-266. doi: 10.1007/s00393-020-00759-6.
Antiphospholipid syndrome (APS) was first identified in patients with systemic lupus erythematosus (SLE) and frequent occurrence of thromboembolic complications and miscarriages accompanied by detection of anticardiolipin antibodies (aCL). When APS was also later found without an underlying SLE, the so-called primary APS was distinguished from its secondary form with SLE. Even more specific than aCL are the lupus anticoagulant (LA) and antibodies against beta‑2 glycoprotein I (aB2GP I). In recent years, it has become evident that the risk of (further) thromboembolic and obstetric complications is markedly increased if all three serological criteria of APS (aCL, aB2GP I and LA), the so-called triple positivity, are present (high-risk profile). Immunosuppression is not effective in preventing further thromboembolic complications of APS. Low-dose aspirin (LDA), heparin and vitamin K antagonists are used in primary and secondary prophylaxis. The direct oral anticoagulants have an increased risk of complications compared to these treatments and should not be used in cases of high-risk APS.
抗磷脂综合征（APS）最初在系统性红斑狼疮（SLE）患者中被发现，这些患者频繁出现血栓栓塞并发症和流产，并伴有抗心磷脂抗体（aCL）检测阳性。后来发现无潜在SLE的APS患者后，将所谓的原发性APS与继发于SLE的继发性APS区分开来。狼疮抗凝物（LA）和抗β2糖蛋白I抗体（aB2GP I）比aCL更具特异性。近年来，很明显，如果存在APS的所有三项血清学标准（aCL、aB2GP I和LA），即所谓的三联阳性（高危特征），（进一步）发生血栓栓塞和产科并发症的风险会显著增加。免疫抑制对预防APS的进一步血栓栓塞并发症无效。低剂量阿司匹林（LDA）、肝素和维生素K拮抗剂用于一级和二级预防。与这些治疗相比，直接口服抗凝剂的并发症风险增加，不应在高危APS病例中使用。
