Center of Neuroimmunology, Laboratory of Advanced Imaging in Neuroimmunological Diseases, Hospital Clinic of Barcelona and Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Department of Preventive Medicine and Epidemiology, Hospital Clinic of Barcelona, University of Barcelona, Barcelona, Spain.
Mult Scler. 2018 Dec;24(14):1843-1851. doi: 10.1177/1352458517735191. Epub 2017 Oct 6.
Population-based studies on neuromyelitis optica spectrum disorders (NMOSD) are limited, and it is unclear whether the rates have changed with the implementation of the new 2015 criteria.
To estimate the incidence and prevalence of NMOSD in Catalonia (Spain), using both the 2006 and the 2015 criteria.
In this clinic-based retrospective study, patients diagnosed with NMOSD between 2006 and 2015 were identified using multiple sources, including direct contact to all Catalan hospitals, identification of cases through the Catalan Health Surveillance System, and registry of antibodies to aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) in a reference laboratory. The incidence rate was calculated for the period 1 January 2006-1 January 2016 and prevalence for the date 1 January 2016.
We identified 74 patients (by the 2015 criteria). Most patients were Caucasian (81%), and female (76%) with a median age at disease onset of 42 years (range, 10-76 years). In total, 54 (73%) patients were positive for AQP4-IgG, 11 (15%) double-seronegative, and 9 (12%) MOG-IgG-positive. Rates of incidence and prevalence (0.63/1,000,000 person-years and 0.89/100,000, respectively) were 1.5-fold higher than those reported by the 2006 criteria. Lowest rates were seen in children and elder people and highest in women and middle-aged people (40-59 years). The female predominance was lost in incident AQP4-IgG-seronegative children and AQP4-IgG-positive elder people. MOG-IgG and double-seronegativity contributed similarly but did not influence the long-term outcome.
The new criteria increase the estimates, but NMOSD remains as a rare disease. The differences in age- and sex-specific estimates highlight the importance of the serologic classification.
基于人群的视神经脊髓炎谱系疾病（NMOSD）研究有限，并且尚不清楚新的 2015 年标准实施后发病率是否发生了变化。
使用 2006 年和 2015 年标准，估计加泰罗尼亚（西班牙）NMOSD 的发病率和患病率。
在这项基于诊所的回顾性研究中，通过多种来源确定了 2006 年至 2015 年间诊断为 NMOSD 的患者，这些来源包括直接联系所有加泰罗尼亚医院、通过加泰罗尼亚健康监测系统识别病例，以及在参考实验室中鉴定水通道蛋白-4（AQP4-IgG）和髓鞘少突胶质细胞糖蛋白（MOG-IgG）抗体。发病率按 2006 年 1 月 1 日至 2016 年 1 月 1 日期间计算，患病率按 2016 年 1 月 1 日计算。
我们确定了 74 名患者（根据 2015 年标准）。大多数患者为白种人（81%），女性（76%），疾病发病中位年龄为 42 岁（范围，10-76 岁）。共有 54 名（73%）患者 AQP4-IgG 阳性，11 名（15%）双阴性，9 名（12%）MOG-IgG 阳性。发病率和患病率（分别为 0.63/100 万人口/年和 0.89/10 万，分别）比 2006 年标准报告的发病率和患病率高 1.5 倍。儿童和老年人的发病率和患病率最低，而女性和中年人群（40-59 岁）发病率和患病率最高。在新发病的 AQP4-IgG 阴性儿童和 AQP4-IgG 阳性老年人中，女性优势丧失。MOG-IgG 和双阴性的作用相似，但对长期预后没有影响。
新标准增加了估计值，但 NMOSD 仍然是一种罕见疾病。年龄和性别特异性估计值的差异突出了血清学分类的重要性。
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