From the Department of Neurology (V.P., Z.I.), Odense University Hospital, University of Southern Denmark; Danish Multiple Sclerosis Center and Danish Multiple Sclerosis Registry (M.M., F.S., J.L.B.), Copenhagen University Hospital-Rigshospitalet, Glostrup; Department of Clinical Medicine (M.M., F.S., J.L.B.), Faculty of Health and Medical Sciences, University of Copenhagen; Open Patient Data Explorative Network (S.M.), Odense University Hospital and Research Unit OPEN, Department of Clinical Research, University of Southern Denmark; Department of Neurology (K.B.S.), Aarhus University Hospital; Danish Multiple Sclerosis Center (H.B.S.), Copenhagen University Hospital-Rigshospitalet, Glostrup; and Department of Clinical Immunology (A.C.N.), Odense University Hospital, Denmark.
Neurology. 2024 Mar 12;102(5):e209147. doi: 10.1212/WNL.0000000000209147. Epub 2024 Feb 6.
We aimed to evaluate the mortality of patients with AQP4 antibody-seropositive (AQP4-Ab+) neuromyelitis optica spectrum disorder (NMOSD) in Denmark compared with that in the general population.
We identified patients with AQP4-Ab+ NMOSD fulfilling the 2015 International Panel for Neuromyelitis Optica Diagnosis (IPND) criteria from multiple sources (laboratories and the Danish Multiple Sclerosis Registry). We obtained detailed information about patients from hospital records and about the general population matched on age, sex, and calendar year from Statistics Denmark. We calculated standardized mortality ratio (SMR), excess number of deaths per 1,000 person-years (EDR), and life expectancies compared with those of the matched general population. We examined predictive factors of mortality and the cause of death.
Of 66 patients with AQP4-Ab+ NMOSD between 2008 and 2020, 15 died. Overall, the SMR was 2.54 (95% CI 1.47-4.09), and the EDR was 16.8 (95% CI 4.6-34.3). The median life expectancy for patients with AQP4-Ab+ NMOSD was 64.08 years (95% CI 53.02-83.9), compared with 83.07 years for the general population. Risk of death over time was increased in the patient population with a hazard ratio (HR) of 2.22 (1.34-3.68; = 0.002). The cause of death was directly related to NMOSD in 93% of the cases. The age at disease onset was an independent predictor of death (HR 1.042; 95% CI 1.006-1.079; = 0.02).
AQP4-Ab+ NMOSD is associated with increased mortality and shorter life expectancy compared with that in the general population, underlining the need for highly effective treatment approaches.
本研究旨在评估丹麦 AQP4 抗体阳性（AQP4-Ab+）视神经脊髓炎谱系疾病（NMOSD）患者的死亡率与普通人群相比的差异。
我们从多个来源（实验室和丹麦多发性硬化症登记处）确定了符合 2015 年国际视神经脊髓炎诊断小组（IPND）标准的 AQP4-Ab+ NMOSD 患者。我们从医院记录中获取有关患者的详细信息，并从丹麦统计局获取与年龄、性别和日历年份相匹配的普通人群的信息。我们计算标准化死亡率比（SMR）、每 1000 人年超额死亡人数（EDR）以及与匹配普通人群相比的预期寿命。我们检查了死亡率的预测因素和死亡原因。
在 2008 年至 2020 年间，66 例 AQP4-Ab+ NMOSD 患者中，有 15 例死亡。总体而言，SMR 为 2.54（95%CI 1.47-4.09），EDR 为 16.8（95%CI 4.6-34.3）。AQP4-Ab+ NMOSD 患者的中位预期寿命为 64.08 岁（95%CI 53.02-83.9），而普通人群的预期寿命为 83.07 岁。患者人群的死亡风险随时间增加，风险比（HR）为 2.22（1.34-3.68； = 0.002）。93%的死亡病例与 NMOSD 直接相关。发病年龄是死亡的独立预测因素（HR 1.042；95%CI 1.006-1.079； = 0.02）。
与普通人群相比，AQP4-Ab+ NMOSD 患者的死亡率更高，预期寿命更短，这突显了需要采取高度有效的治疗方法。
