Ricard Laure, Laurent Charlotte, Papo Matthias, Deriaz Sophie, Catano Jennifer, Alamowitch Sonia, Kayem Gilles, Chasset François, De Moreuil Claire, Boffa Jean Jacques, Gerotziafas Grigorios, Elalamy Ismail, Bornes Marie, Maillot François, Audemard-Verger Alexandra, Planche Virginie, Ballot Eric, Fain Olivier, Mekinian Arsène
Service de médecine interne et Inflammation-Immunopathology-Biotherapy Department (DMU i3), hôpital Saint-Antoine, Sorbonne université, AP-HP, 75012 Paris, France.
Médecine interne institut e3m, groupe hospitalier Pitié-Salpêtrière, Paris, France.
Joint Bone Spine. 2022 Mar;89(2):105297. doi: 10.1016/j.jbspin.2021.105297. Epub 2021 Oct 14.
The antiphospholipid syndrome (APS) (1) is defined by the development of vascular thrombosis, or pregnancy morbidity in the presence of persistent antiphospholipid antibodies (aPL). Antinuclear antibodies (ANA) can be detected in primary APS patients without any clinical systemic autoimmune disease. The presence of ANA antibodies could confer a specific phenotype in primary APS.
To evaluate the characteristics of APS patients with antinuclear antibodies without other autoimmune disease (ANA positive APS patients) in comparison with primary APS without ANA or secondary APS patients with associated systemic lupus erythematosus (SLE).
Clinical and biologic data from 195 APS were retrospectively collected and patients were classified as primary APS with positive ANA (ANA-positive APS), primary APS without any ANA (ANA-negative APS), and SLE-associated APS (SLE-APS).
Fourty patients (21%) were classified into ANA-positive APS group, 77 (39%) in ANA-negative APS and 78 (40%) in SLE-APS. In ANA-positive APS patients, 20 patients (51%) had arterial thrombosis, 14 (41%) had veinous thrombosis and 19% had obstetrical complications. There was no difference between the three groups for the frequency of thrombotic manifestations and obstetrical complications. ANA-positive APS patients had more non-criteria manifestations than ANA-negative APS (48% versus 25%; P≤0.01). ANA-positive APS had more triple aPL positivity (59% versus 18%; P<0.001) and more thrombosis and obstetrical recurrences (63% versus 36%; P<0.01) in comparison with ANA-negative APS patients. ANA-positive APS had more triple aPL positivity than SLE-APS patients (54% versus 33%; P<0.05). ANA-positive APS and SLE-APS patients had similar clinical manifestations, and recurrences. Despite a limited follow-up (28 months (11-50)) none of the ANA-positive APS develop SLE. Antiplatelet and anticoagulant therapies were similar for the three groups. SLE-APS patients received more immunomodulatory therapies.
ANA positivity in patients with APS enables to individualize a subset of patients with a more severe phenotype. Whereas the ANA positivity does not seem to be associated with the risk to develop SLE, prospective studies with a longer follow-up are necessary, in particular to evaluate the effect of additional therapies in this subset of APS.
抗磷脂综合征（APS）（1）的定义为在存在持续性抗磷脂抗体（aPL）的情况下发生血管血栓形成或妊娠并发症。在原发性APS患者中可检测到抗核抗体（ANA），且无任何临床系统性自身免疫性疾病。ANA抗体的存在可能赋予原发性APS特定的表型。
评估无其他自身免疫性疾病的抗核抗体阳性APS患者（ANA阳性APS患者）与无ANA的原发性APS患者或合并系统性红斑狼疮（SLE）的继发性APS患者的特征。
回顾性收集195例APS患者的临床和生物学数据，患者被分为ANA阳性的原发性APS（ANA阳性APS）、无任何ANA的原发性APS（ANA阴性APS）和SLE相关的APS（SLE-APS）。
40例患者（21%）被归入ANA阳性APS组，77例（39%）为ANA阴性APS，78例（40%）为SLE-APS。在ANA阳性APS患者中，20例（51%）发生动脉血栓形成，14例（41%）发生静脉血栓形成，19%发生产科并发症。三组之间血栓形成表现和产科并发症的发生率无差异。ANA阳性APS患者的非标准表现比ANA阴性APS患者更多（48%对25%；P≤0.01）。与ANA阴性APS患者相比，ANA阳性APS患者的aPL三联阳性更多（59%对18%；P<0.001），血栓形成和产科复发更多（63%对36%；P<0.01）。ANA阳性APS患者的aPL三联阳性比SLE-APS患者更多（54%对33%；P<0.05）。ANA阳性APS和SLE-APS患者有相似的临床表现和复发情况。尽管随访时间有限（28个月（11 - 50）），但ANA阳性APS患者均未发展为SLE。三组的抗血小板和抗凝治疗相似。SLE-APS患者接受更多的免疫调节治疗。
APS患者中的ANA阳性有助于区分出具有更严重表型的一部分患者。虽然ANA阳性似乎与发展为SLE的风险无关，但需要进行更长随访时间的前瞻性研究，特别是评估针对这部分APS患者额外治疗的效果。
