Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DMU 3iD), Paris, France.
Department of Internal Medicine, AP-HP Nord, Beaujon Hospital, Paris University,Clichy, France.
RMD Open. 2020 Aug;6(2):0. doi: 10.1136/rmdopen-2020-001340.
To compare characteristics, pregnancies and treatments during pregnancies of seronegative and seropositive antiphospholipid syndrome (APS), to analyse factors associated with obstetrical outcome.
Inclusion criteria were: (1) thrombotic and/or obstetrical APS (Sydney criteria); (2) absence of conventional antiphospholipid antibodies (APL); (3) at least one persistent non-conventional APL among IgA anticardiolipin antibodies, IgA anti-B2GPI, anti-vimentin G/M, anti-annexin V G/M, anti-phosphatidylethanolamine G/M and anti-phosphatidylserine/prothrombin G/M antibodies. The exclusion criteria were: (1) systemic lupus erythematosus ( SLE) or SLE-like disease; and (2) other connective tissue disease.
A total of 187 women (mean 33±5 years) with seronegative APS were included from 14 centres in Austria, Spain, Italy, Slovenia and France and compared with 285 patients with seropositive APS. Seronegative APS has more obstetrical rather than thrombotic phenotypes, with only 6% of venous thrombosis in comparison to seropositive APS. Cumulative incidence of adverse obstetrical events was similar in seronegative and seropositive APS patients, although higher rates of intrauterine deaths (15% vs 5%; p=0.03), of preeclampsia (7% vs 16%, p=0.048) and lower live birth term (36±3 vs 38±3 weeks of gestation; p=0.04) were noted in seropositive APS. The cumulative incidence of adverse obstetrical events was significantly improved in treated versus untreated seronegative APS (log rank<0.05), whereas there was no difference between patients who received aspirin or aspirin-low-molecular weighted heparin combination.
Several non-criteria APL can be detected in patients with clinical APS features without any conventional APL, with various rates. The detection of non-criteria APL and thus the diagnosis of seronegative APS could discuss the therapeutic management similar to seropositive APS, but well-designed controlled studies are necessary.
比较抗磷脂抗体（aPL）阴性和阳性抗磷脂综合征（APS）患者的特征、妊娠及妊娠期间的治疗，分析与产科结局相关的因素。
入选标准为：（1）血栓性和/或产科 APS（悉尼标准）；（2）无常规 aPL；（3）至少有一种持续存在的非常规 aPL，包括 IgA 抗心磷脂抗体、IgA 抗β2 糖蛋白 I、抗波形蛋白 G/M、抗膜联蛋白 V G/M、抗磷脂酰乙醇胺 G/M 和抗磷脂酰丝氨酸/凝血酶原 G/M 抗体。排除标准为：（1）系统性红斑狼疮（SLE）或类似 SLE 的疾病；（2）其他结缔组织病。
从奥地利、西班牙、意大利、斯洛文尼亚和法国的 14 个中心共纳入了 187 例抗磷脂抗体阴性 APS 女性（平均年龄 33±5 岁），并与 285 例抗磷脂抗体阳性 APS 患者进行了比较。抗磷脂抗体阴性 APS 患者以产科表现为主，而非血栓形成表现为主，静脉血栓形成的发生率仅为 6%，而抗磷脂抗体阳性 APS 患者则以血栓形成表现为主。抗磷脂抗体阴性和阳性 APS 患者不良产科事件的累积发生率相似，但抗磷脂抗体阳性 APS 患者宫内死亡（15%比 5%；p=0.03）、子痫前期（7%比 16%；p=0.048）和足月活产率（36±3 比 38±3 周；p=0.04）更高。与未经治疗的患者相比，治疗后的抗磷脂抗体阴性 APS 患者不良产科事件的累积发生率显著改善（对数秩检验<0.05），但接受阿司匹林或阿司匹林联合低分子量肝素治疗的患者之间无差异。
在无任何常规 aPL 的情况下，具有临床 APS 特征的患者中可检测到多种非标准 aPL，其发生率各不相同。非标准 aPL 的检测，即抗磷脂抗体阴性 APS 的诊断，可能会使我们对其治疗管理与抗磷脂抗体阳性 APS 相似，但需要进行精心设计的对照研究。
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