The Epidemiology of Antiphospholipid Syndrome: A Population-Based Study

The Epidemiology of Antiphospholipid Syndrome: A Population-Based Study
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DOI:
10.1002/art.40901
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发表时间:
2019-08-01
影响因子:
13.3
通讯作者:
Matteson, Eric. L.
Matteson, Eric. L.
中科院分区:
医学1区
文献类型:
--
作者:
Duarte-Garcia, Ali;Pham, Michael M.;Matteson, Eric. L.

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目的估计抗磷脂综合征(APS)的年发病率、患病率和死亡率。方法基于全面的个人病历审查,从地理上明确定义的人群中确定了2000-2015年发生APS的初始队列患者。所有病例均符合2006年悉尼APS标准(主要定义)或经医生共识确认的APS诊断(次要定义)。在中心实验室检测狼疮抗凝剂、IgM和IgG抗心磷脂抗体以及抗β 2-糖蛋白I抗体水平。对2010年美国白色人群的发病率进行年龄和性别调整。患病率估计值是从发病率中获得的,假设没有与APS相关的死亡率增加,并且迁入或迁出该区域与疾病状态无关。结果在2000-2015年的该队列中,确定了33例按照悉尼标准定义的APS事件(患者平均年龄54.2岁; 55%为女性,97%为白色)。年龄≥ 18岁的成人APS年发病率为2.1/100,000(95%置信区间[95% CI] 1.4-2.8)。两性的发病率相似。APS的估计患病率为每100,000人50例(95% CI 42-58),男女患病率相似。6例患者(18%)同时诊断为系统性红斑狼疮。最常见的临床表现是深静脉血栓形成。APS患者的总体死亡率与一般人群无显著差异(标准化死亡率比1.61,95% CI 0.74-3.05)。结论每年每10万人口中约有2人发生APS。估计流行率为每100 000人50人。总体死亡率与在一般人群中观察到的死亡率没有显著差异。
Objective To estimate the annual incidence and prevalence of and frequency of mortality associated with antiphospholipid syndrome (APS). Methods An inception cohort of patients with incident APS in 2000-2015 from a geographically well-defined population was identified based on comprehensive individual medical records review. All cases met the 2006 Sydney criteria for APS (primary definition) or had a diagnosis of APS confirmed by physician consensus (secondary definition). Levels of lupus anticoagulant, IgM and IgG anticardiolipin antibodies, and anti-beta 2-glycoprotein I antibodies were tested in a centralized laboratory. Incidence rates were age- and sex-adjusted to the 2010 US white population. Prevalence estimates were obtained from the incidence rates, assuming that there was no increased mortality associated with APS and that migration in or out of the area was independent of disease status. Results Among this cohort in 2000-2015, 33 cases of incident APS, as defined by the Sydney criteria, were identified (mean age of patients 54.2 years; 55% female, 97% white). The annual incidence of APS in adults ages >= 18 years was 2.1 (95% confidence interval [95% CI] 1.4-2.8) per 100,000 population. Incidence rates were similar in both sexes. The estimated prevalence of APS was 50 (95% CI 42-58) per 100,000 population, and was similar in both sexes. Six patients (18%) had a concurrent diagnosis of systemic lupus erythematosus. The most frequent clinical manifestation was deep vein thrombosis. The overall frequency of mortality among patients with APS was not significantly different from that in the general population (standardized mortality ratio 1.61, 95% CI 0.74-3.05). Conclusion APS occurred in similar to 2 persons per 100,000 population per year. The estimated prevalence was 50 per 100,000 population. Overall mortality was not notably different from that observed in the general population.