Sorbonne Université, Assistance Publique-Hôpitaux de Paris (APHP), Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Autres Maladies Auto-Immunes Systémiques Rares, Paris, France; Sorbonne Université, APHP, Hôpital La Pitié-Salpêtrière, Institut de Cardiométabolisme et Nutrition (ICAN), Service de Médecine Intensive-Réanimation, Paris, France.
Service de Médecine Intensive-Réanimation, Hôpital Lapeyronie, Centre Hospitalier Universitaire (CHU) de Montpellier;, PhyMedExp, Université de Montpellier, INSERM, CNRS, Montpellier, France.
Chest. 2020 May;157(5):1158-1166. doi: 10.1016/j.chest.2019.11.010. Epub 2019 Nov 26.
The antiphospholipid syndrome (APS) is a systemic autoimmune disease defined by thrombotic events that can require ICU admission because of organ dysfunction related to macrovascular and/or microvascular thrombosis. Critically ill patients with thrombosis and APS were studied to gain insight into their prognoses and in-hospital mortality-associated factors.
This French national, multicenter, retrospective study included all patients with APS and any new thrombotic manifestations admitted to 24 ICUs (January 2000-September 2018).
During the study period, 134 patients (male/female ratio, 0.4) with 152 APS episodes were admitted to the ICU (mean age at admission, 46.0 ± 15.1 years). In-hospital mortality of their 134 last episodes was 35 of 134 (26.1%). The Cox multivariable model retained certain factors (hazard ratio [95% CI]: age ≥ 40 years, 11.4 [3.1-41.5], P < .0001; mechanical ventilation, 11.0 [3.3-37], P < .0001; renal replacement therapy, 2.9 [1.3-6.3], P = .007; and in-ICU anticoagulation, 0.1 [0.03-0.3], P < .0001) as independently associated with in-hospital mortality. For the subgroup of definite/probable catastrophic APS, the Cox bivariable model (including the Simplified Acute Physiology Score II score) retained double therapy (corticosteroids + anticoagulant, 0.2 [0.07-0.6]; P = .005) but not triple therapy (corticosteroids + anticoagulant + IV immunoglobulins or plasmapheresis: hazard ratio, 0.3 [0.1-1.1]; P = .07) as independently associated with in-hospital mortality.
In-ICU anticoagulation was the only APS-specific treatment independently associated with survival for all patients. Double therapy was independently associated with better survival of patients with definite/probable catastrophic APS. In these patients, further studies are needed to determine the role of triple therapy.
抗磷脂综合征（APS）是一种系统性自身免疫性疾病，其特征为血栓形成事件，由于大血管和/或微血管血栓形成导致器官功能障碍，可能需要入住重症监护病房（ICU）。本研究旨在探讨重症血栓形成合并 APS 患者的预后和院内死亡相关因素。
这是一项法国全国性、多中心、回顾性研究，纳入了 2000 年 1 月至 2018 年 9 月期间入住 24 家 ICU 的所有 APS 患者和任何新发血栓表现。
在研究期间，共纳入了 134 例（男/女比例为 0.4）患有 152 例 APS 发作的患者（入院时平均年龄为 46.0±15.1 岁）。他们的 134 例最后一次发作中院内死亡率为 35/134（26.1%）。Cox 多变量模型保留了某些因素（年龄≥40 岁，危险比[95%CI]：11.4[3.1-41.5]，P<0.0001；机械通气，11.0[3.3-37]，P<0.0001；肾脏替代治疗，2.9[1.3-6.3]，P=0.007；和 ICU 内抗凝治疗，0.1[0.03-0.3]，P<0.0001）与院内死亡独立相关。对于明确/可能的灾难性 APS 亚组，Cox 双变量模型（包括简化急性生理学评分 II 评分）保留了双药治疗（皮质类固醇+抗凝剂，0.2[0.07-0.6]；P=0.005），但不保留三药治疗（皮质类固醇+抗凝剂+静脉注射免疫球蛋白或血浆置换：危险比，0.3[0.1-1.1]；P=0.07）与院内死亡独立相关。
ICU 内抗凝治疗是唯一与所有患者生存相关的 APS 特异性治疗。双药治疗与明确/可能的灾难性 APS 患者的更好生存相关。在这些患者中，需要进一步研究来确定三药治疗的作用。
