Objectives This study focused on the prevalence and impact of antinuclear antibodies (ANA) in antiphospholipid antibody (aPL)-positive patients without concomitant systemic autoimmune rheumatic diseases (SARDs). Methods Data from aPL-positive patients with or without Revised Sapporo APS classification criteria were retrieved from the APS ACTION Registry. Patients with concomitant SARDs were excluded. Results 430 aPL-positive patients were included in the analysis, 56% ANA-positive and 44% negative. ANA positivity was significantly associated with history of hematologic manifestations (persistent autoimmune hemolytic anaemia, thrombocytopenia, leukopenia and/or lymphopenia) (16% of ANA-positive vs 7% of ANA-negative, p= 0.006). Triple aPL-positivity was more frequent in the ANA-positive subgroup (p= 0.02), along with low baseline C3 and C4 levels (p= 0.05 and p= 0.009, respectively), and higher frequency for extractable nuclear antigens (ENA). Among aPL-positive patients with no APS classification, ANA-positive patients showed a higher rate of arthritis (p= 0.006). Among female patients who have experienced at least one pregnancy, 113 were ANA-positive and 96 were ANA-negative; ANA-negative patients had a higher number of pregnancies (p= 0.018), and number of live births (p= 0.014). A wider proportion of ANA-positive patients were treated with hydroxychloroquine (HCQ) (p< 0.001). Conclusion When we analysed aPL-positive patients with no other SARDs, ANA status was not associated with thrombosis or pregnancy morbidity. Interestingly, ANA-positive patients showed higher rates of systemic autoimmune features, including hematologic manifestations, multiple aPL positivity, lower complement levels, ENA positivity, and joint involvement, and were more often treated with HCQ. Finally, aPL-positive subjects who were ANA-negative had a higher rate of pregnancies and live births.

中文翻译：

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ANA 阳性与 ANA 阴性抗磷脂抗体阳性患者：来自 APS ACTION 临床数据库和存储库的结果


目的 本研究重点关注抗磷脂抗体 （aPL） 阳性患者无伴系统性自身免疫性风湿病 （SARDs） 的患病率和影响。方法 从 APS ACTION Registry 中检索有或没有修订的 Sapporo APS 分类标准的 aPL 阳性患者的数据。排除伴有 SARD 的患者。结果 430 例 aPL 阳性患者纳入分析，其中 56% 为 ANA 阳性，44% 为阴性。ANA 阳性与血液学表现史 （持续性自身免疫性溶血性贫血、血小板减少、白细胞减少和/或淋巴细胞减少） 显著相关 （16% 的 ANA 阳性 vs 7% 的 ANA 阴性，p = 0.006）。在 ANA 阳性亚组中 （p= 0.02） 中，三重 aPL 阳性更常见，基线 C3 和 C4 水平较低 （分别为 p= 0.05 和 p= 0.009），可提取核抗原 （ENA） 的频率较高。在无 APS 分类的 aPL 阳性患者中，ANA 阳性患者表现出更高的关节炎发生率 （p= 0.006）。在至少经历过一次妊娠的女性患者中，113 例为 ANA 阳性，96 例为 ANA 阴性;ANA 阴性患者的妊娠数 （p= 0.018） 和活产数 （p= 0.014） 较高。更大比例的 ANA 阳性患者接受羟氯喹 （HCQ） 治疗 （p< 0.001）。结论 当我们分析没有其他 SARD 的 aPL 阳性患者时，ANA 状态与血栓形成或妊娠发病率无关。有趣的是，ANA 阳性患者表现出较高的全身性自身免疫特征发生率，包括血液学表现、多发性 aPL 阳性、较低的补体水平、ENA 阳性和关节受累，并且更常接受 HCQ 治疗。 最后，ANA 阴性的 aPL 阳性受试者的妊娠率和活产率更高。