背景:原发性中枢神经系统血管炎(PACNS)是一种复发缓解型疾病,病程不均。病例系列描述了长期的病程,但没有描述急性结局或其决定因素。PACNS的全美医院负担尚未量化。
方法:分析美国全国再入院数据库(2016-2018)以表征PACNS住院频率,人口特征,住院死亡率,和出院结果。
结果:在3年的研究期间,未加权的1843例(加权3409例)PACNS患者被纳入1078家医疗保健成本和利用项目HCUP参与医院;加权,这个值表明美国医院每年有1136名患者入院,代表每年每10万国家住院0.01例。大多数患者在大都市教学医院住院(81.6%)。入院年龄中位数为54.9(IQR:44.0-66.5)岁,女性占59.4%。38.2%的神经系统表现包括缺血性卒中,短暂性脑缺血发作20.2%,癫痫发作占22.8%,颅内出血13.0%。总的来说,60.0%的病人已出院回家,35.0%的人出院到康复机构或疗养院,5.0%的人在出院前死亡。与出院到另一个机构或死亡独立相关的患者特征包括年龄较大(比值比[OR],1.03[95%CI,[1.03-1.04]]),男性(或,1.22[1.04-1.43]),实质内出血(OR,1.41[1.08-1.84]),缺血性卒中(OR,2.79[2.38-3.28]),和癫痫症(或,1.57[1.31-1.89])。
结论:我们的研究表明,PACNS仍然是一种罕见的中枢神经系统血管炎症性疾病,表明在更多样化和当代的美国人口中,每年每100万人年住院5.1例。总的来说,十分之四的人有不利的出院结果,无法出院回家,20人中有1人在出院前死亡。
BACKGROUND: Primary angiitis of the central nervous system (PACNS) is a relapsing-remitting disease with a heterogeneous course. Case series have delineated the long-term disease course but not acute outcomes or their determinants. The national United States hospital burden of PACNS has not been quantified.
METHODS: Analysis of the United States Nationwide Readmissions Database (2016-2018) to characterize the frequency of PACNS hospitalizations, demographic features, inpatient mortality, and discharge outcomes.
RESULTS: During the 3-year study period, unweighted 1843 (weighted 3409) patients with PACNS were admitted to the 1078 Healthcare Cost and Utilization Project HCUP participating hospitals; with weighting, this value indicates that 1136 patients were admitted each year to US hospitals, representing yearly 0.01 cases per 100 000 national hospitalizations. The majority of patients were hospitalized in metropolitan teaching hospitals (81.6%). The median age at admission was 54.9 (IQR: 44.0-66.5) years and 59.4% were women. Neurologic manifestations included ischemic stroke in 38.2%, transient ischemic attack in 20.2%, seizure disorder in 22.8%, and intracranial hemorrhage in 13.0%. Overall, 60.0% of patients were discharged home, 35.0% discharged to a rehabilitation facility or nursing home and 5.0% died before discharge. Patient features independently associated with the discharge to another facility or death included older age (odds ratio [OR], 1.03 [95% CI, [1.03-1.04]]), male sex (OR, 1.22 [1.04-1.43]), intraparenchymal hemorrhage (OR, 1.41 [1.08-1.84]), ischemic stroke (OR, 2.79 [2.38-3.28]), and seizure disorder (OR, 1.57 [1.31-1.89]).
CONCLUSIONS: Our study showed PACNS is still a rare inflammatory disorder of the blood vessels of the central nervous system suggesting an annual hospitalization of 5.1 cases per 1,000,000 person-years in the more diverse and contemporary US population. Overall, 4 in 10 had unfavorable discharge outcome, being unable to be discharged home, and 1 in 20 died before discharge.