Gaspar Pedro, Farinha Filipa, Sayar Zara, Efthymiou Maria, Cohen Hannah, Isenberg David A
Department of Internal Medicine 2, North Lisbon University Hospital Centre, Lisbon, and Instituto de Medicina Molecular João Lobo Antunes, Faculty of Medicine, University of Lisbon, Portugal.
Centre for Rheumatology, Division of Medicine, University College London, UK.
Clin Exp Rheumatol. 2023 Mar;41(3):605-612. doi: 10.55563/clinexprheumatol/0gs167. Epub 2022 Jul 11.
To determine whether early damage and its kinetics measured by the Damage Index for Antiphospholipid Syndrome (DIAPS) predicts mortality.
We carried out a single-centre retrospective analysis of thrombotic APS patients (2006 Sydney criteria), using the DIAPS for damage assessment. Early damage was considered to be at six months after disease onset; early damage increase (delta-DIAPS) was deemed to be at least a one-point rise in DIAPS within the first five years of illness. Groups were compared using appropriate statistical tests. Survival was analysed by the Kaplan-Meier method. Cox regression analysis was performed to investigate predictors of mortality.
A total of 197 patients (71.1% female; 65.9% primary APS; 72.4% Caucasian) were followed for up to 43 years (median 10). Damage developed in 143 (73.6%) patients. Twenty-three patients (12%) died. Secondary APS (HR 3.07, 95%CI 1.32-7.12, p=0.009), male sex (HR 3.14, 95%CI 1.35-7.33, p=0.008) and age at APS onset ≥40 years (HR 5.34, 95%CI 1.96-14.53, p=0.001) were risk factors for death. Early damage (n=69, 35.0%) was not associated with death (p=0.231). Having a first arterial event was associated with early damage (p<0.001), but not with delta-DIAPS (p=0.539) nor with the risk of death (p=0.151). Delta-DIAPS (n=53/181, 29.3%) predicted mortality (HR 5.40, 95%CI 2.33-12.52, p<0.001), even after adjusting individually for APS category (secondary), sex (male), early damage and age at APS onset (≥40 years) (all p<0.005).
Evolving damage in the first five years of illness, but not early damage, predicted mortality regardless of the nature of the first thrombotic event, sex, APS category and age.
确定通过抗磷脂综合征损伤指数（DIAPS）测量的早期损伤及其动力学是否可预测死亡率。
我们对血栓形成性抗磷脂综合征患者（2006年悉尼标准）进行了单中心回顾性分析，使用DIAPS进行损伤评估。早期损伤定义为疾病发作后6个月；早期损伤增加（DIAPS变化值）定义为疾病发病后前5年内DIAPS至少升高1分。使用适当的统计检验对组间进行比较。采用Kaplan-Meier方法分析生存率。进行Cox回归分析以研究死亡率的预测因素。
共纳入197例患者（女性占71.1%；原发性抗磷脂综合征占65.9%；白种人占72.4%），随访时间长达43年（中位数为10年）。143例（73.6%）患者出现损伤。23例（12%）患者死亡。继发性抗磷脂综合征（HR 3.07，95%CI 1.32 - 7.12，p = 0.009）、男性（HR 3.14，95%CI 1.35 - 7.33，p = 0.008）和抗磷脂综合征发病年龄≥40岁（HR 5.34，95%CI 1.96 - 14.53，p = 0.001）是死亡的危险因素。早期损伤（n = 69，35.0%）与死亡无关（p = 0.231）。首次发生动脉事件与早期损伤相关（p < 0.001），但与DIAPS变化值无关（p = 0.539），也与死亡风险无关（p = 0.151）。DIAPS变化值（n = 53/181，29.3%）可预测死亡率（HR 5.40，95%CI 2.33 - 12.52，p < 0.001），即使在分别对抗磷脂综合征类别（继发性）、性别（男性）、早期损伤和抗磷脂综合征发病年龄（≥40岁）进行调整后（所有p < 0.005）。
疾病发病后前5年中不断进展的损伤而非早期损伤可预测死亡率，无论首次血栓形成事件的性质、性别、抗磷脂综合征类别和年龄如何。
