Internal Medicine Department, AP-HP, Referral Center for Rare Autoimmune and Systemic Diseases of Ile de France, Cochin Hospital, Paris, France.
Univ. Lille, Inserm, CHU Lille, Service de Médecine Interne et d'Immunologie Clinique, Centre de référence des maladies autoimmunes systémiques rares du nord et Nord-Ouest de France (CeRAINO), U1167 RID-AGE, F-59000 Lille, France.
J Thromb Haemost. 2023 May;21(5):1258-1265. doi: 10.1016/j.jtha.2023.02.007. Epub 2023 Feb 13.
The prevention of catastrophic antiphospholipid syndrome (CAPS), a rare complication of antiphospholipid syndrome (APS), is a major goal.
We analyzed its precipitating factors, focusing on anticoagulation immediately before CAPS episodes.
We retrospectively analyzed patients in the French multicenter APS/systemic lupus erythematosus database with at least 1 CAPS episode. Then we compared each patient with known APS before CAPS with 2 patients with non-CAPS APS matched for age, sex, center, and APS phenotype.
We included 112 patients with CAPS (70% women; mean age, 43 ± 15 years). At least 1 standard precipitating factor of CAPS was observed for 67 patients (64%), which were mainly infections (n = 28, 27%), pregnancy (n = 23, 22%), and surgery (n = 16, 15%). Before the CAPS episode, 67 (60%) patients already had a diagnosis of APS. Of the 61 treated with anticoagulants, 32 (48%) received vitamin K antagonists (VKAs), 23 (34%) heparin, and 2 (3%) a direct oral anticoagulant. They were less likely than their matched patients with APS without CAPS to receive VKA (48% vs 66%, p = .001). Among those treated with VKA, 72% had a subtherapeutic international normalized ratio (ie, <2) versus 28% in patients with APS without CAPS (p < .001). Finally, excluding pregnant patients (n = 14) for whom we could not differentiate the effect of treatment from that of pregnancy, we were left with 47 cases, 32 (68%) of whom had recently begun a direct oral anticoagulant, planned bridging therapy, or had VKA treatment with international normalized ratio <2.
These results strongly suggest that suboptimal anticoagulation management can trigger CAPS in patients with thrombotic APS.
灾难性抗磷脂综合征（CAPS）是抗磷脂综合征（APS）的一种罕见并发症，其预防是主要目标。
我们分析了其诱发因素，重点关注 CAPS 发作前的抗凝治疗。
我们回顾性分析了法国多中心 APS/系统性红斑狼疮数据库中至少有 1 次 CAPS 发作的患者。然后，我们将每位 CAPS 患者与已知的 CAPS 患者进行比较，并与 2 名非 CAPS APS 患者进行匹配，匹配因素包括年龄、性别、中心和 APS 表型。
我们纳入了 112 例 CAPS 患者（70%为女性；平均年龄 43±15 岁）。67 例（64%）患者至少存在 1 个 CAPS 标准诱发因素，主要包括感染（n=28，27%）、妊娠（n=23，22%）和手术（n=16，15%）。在 CAPS 发作前，67 例（60%）患者已被诊断为 APS。在接受抗凝治疗的 61 例患者中，32 例（48%）接受了维生素 K 拮抗剂（VKA），23 例（34%）接受了肝素，2 例（3%）接受了直接口服抗凝剂。与未发生 CAPS 的 APS 患者相比，他们更不可能接受 VKA 治疗（48%比 66%，p=0.001）。在接受 VKA 治疗的患者中，72%的患者国际标准化比值（INR）低于治疗范围（即<2），而未发生 CAPS 的 APS 患者中这一比例为 28%（p<0.001）。最后，排除妊娠患者（n=14），因为我们无法区分治疗和妊娠的影响，最终纳入 47 例患者，其中 32 例（68%）最近开始使用直接口服抗凝剂、计划桥接治疗或 VKA 治疗，INR 低于 2。
这些结果强烈表明，血栓性 APS 患者的抗凝治疗管理不佳可能会引发 CAPS。
