The presence of non-criteria manifestations negatively affects the prognosis of seronegative antiphospholipid syndrome patients: a multicenter study.
The presence of non-criteria manifestations negatively affects the prognosis of seronegative antiphospholipid syndrome patients: a multicenter study.
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DOI：
10.1186/s13075-021-02702-9
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发表时间：
2022-01-03
影响因子：
4.9
通讯作者：
Espinosa G
中科院分区：
文献类型：
--
作者：
da Rosa GP;Sousa-Pinto B;Ferreira E;Araújo O;Barilaro G;Bettencourt P;Cervera R;Espinosa G
血清阴性抗磷脂综合征（SN-APS）通常被定义为存在APS标准表现、阴性抗磷脂抗体（aPL）和APS非标准表现共存。然而，这些非标准特征的影响仍不清楚。另一方面，在APS表现的患者中，单个aPL阳性检测的相关性是另一个证据有限的领域。我们的目的是比较SN-APS和单阳性aPL （SP-aPL）患者的病程与没有非标准特征/aPL阳性的APS患者（对照）的病程。2005年至2020年在欧洲两家医院评估的血栓形成/产科发病率患者回顾性分析患者分为SN-APS组、SP-aPL组和对照组。比较临床特征、合并症和治疗方法。SN-APS组共82例，SP-aPL组88例，对照组185例。在Cox回归模型中，即使校正了遗传性血栓病、系统性红斑狼疮或避孕激素治疗的存在，SN-APS组的血栓复发率也高于对照组（HR 3.8, 95% CI 2.2-6.5, p < 0.001）。SP-aPL组血栓复发率差异无统计学意义（p = 0.078）。不确定抗凝（p < 0.001和p = 0.008）和维生素K拮抗剂（VKA）的使用（两种情况均p < 0.001）在SN-APS/SP-aPL中更为常见。与没有非标准表现的对照组相比，SN-APS表现出更多的血栓复发、无限期抗凝和VKA使用。血栓和aPL阴性患者出现这些特征可能会对其临床病程产生负面影响。
Seronegative antiphospholipid syndrome (SN-APS) is often defined as the presence of APS criteria manifestations, negative antiphospholipid antibodies (aPL), and coexistence of APS non-criteria manifestations. Nevertheless, the impact of these non-criteria features is still unclear. On a different note, the relevance of one single aPL positive determination in patients with APS manifestations is another domain with limited evidence. We aim to compare the course of SN-APS and single-positive aPL (SP-aPL) patients with that of individuals with APS manifestations without non-criteria features/aPL positivity (controls). Retrospective analysis of patients with thrombosis/obstetric morbidity assessed in two European hospitals between 2005 and 2020. Patients were divided into SN-APS, SP-aPL, and control groups. Clinical characteristics, comorbidities, and therapies were compared. A total of 82 patients were included in the SN-APS group, 88 in the SP-aPL group, and 185 in the control group. In Cox regression model, SN-APS displayed more thrombosis recurrence than controls (HR 3.8, 95% CI 2.2–6.5, p < 0.001) even when adjusting for the presence of hereditary thrombophilia, systemic lupus erythematosus, or contraceptive hormonal treatment. In SP-aPL, the difference in thrombosis recurrence did not reach statistical significance (p = 0.078). Indefinite anticoagulation (p < 0.001 and p = 0.008, respectively) and vitamin K antagonist (VKA) use (p < 0.001 in both cases) were more common in SN-APS/SP-aPL. SN-APS displayed more thrombosis recurrence, indefinite anticoagulation, and VKA use than controls without non-criteria manifestations. The presence of such features in patients with thrombosis and negative aPL may negatively impact their clinical course.
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影响因子：
5.9
作者：
Schulman, S;Svenungsson, E;Granqvist, S
通讯作者：
Granqvist, S
影响因子：
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作者：
Francès, C;Niang, S;Piette, JC
通讯作者：
Piette, JC
影响因子：
27.4
作者：
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通讯作者：
Hughes, GRV
影响因子：
3.7
作者：
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通讯作者：
Soares, Marcio
影响因子：
5
作者：
Dlott, Jeffrey S.;Roubey, Robert A. S.
通讯作者：
Roubey, Robert A. S.