日本第五次全国调查中的多发性硬化症和视神经脊髓炎谱系障碍的患病率及残疾情况。

Prevalence of, and Disability Due to, Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder in Japan by the Fifth Nationwide Survey.

机构信息

From the Department of Neurology (M.W., N.I., T.M., J.K.), Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka; Department of Clinical Research (M.N.), National Hospital Organization Hokkaido Medical Center, Sapporo; Division of Neurology (I.N.), Tohoku Medical and Pharmaceutical University, Sendai; Department of Neurology (T.M.), Kochi Medical School, Kochi University, Nankoku; Department of Pediatrics (Y.S.), Graduate School of Medical Sciences, Kyushu University, Fukuoka; Department of Neurology (J.N.), Keio University School of Medicine, Tokyo; Department of Neurology (I.K.), Brain Research Institute, Niigata University, Niigata; Medical Education Center (I.K.), Niigata University School of Medicine, Niigata; Department of Intractable Disease and Aging Science (H.O.), Ehime University Graduate School of Medicine, Toon; Department of Neurology (Y. Nakatsuji), University of Toyama, Toyama; Jichi Medical University (Y. Nakamura), Shimotsuke Department of Public Health and Epidemiology (K.N.), Graduate School of Medicine, University of the Ryukyus, Nishihara; Department of Hygiene and Preventive Medicine (K.S.), Iwate Medical University, Yahaba; Department of Neurology (M.M.), Kanazawa Medical University, Uchinada; Department of Neurology (S.K.), Graduate School of Medicine, Chiba University, Chiba; Department of Neurology (J.K.), Brain and Nerve Center, Fukuoka Central Hospital, Fukuoka; and Translational Neuroscience Research Center (J.K.), Graduate School of Medicine, and School of Pharmacy at Fukuoka, International University of Health and Welfare, Okawa, Japan.

出版信息

Neurology. 2024 Nov 26;103(10):e209992. doi: 10.1212/WNL.0000000000209992. Epub 2024 Oct 30.

DOI:10.1212/WNL.0000000000209992

PMID:39475688

Abstract

BACKGROUND AND OBJECTIVES

All 4 previous nationwide surveys of multiple sclerosis (MS) in Japan were conducted before the discovery of anti-aquaporin-4 (AQP4) antibodies; thus, neuromyelitis optica spectrum disorder (NMOSD) was included in MS, as optic-spinal MS. We aimed to clarify the epidemiologic features and trends of MS and NMOSD in Japan separately using a fifth nationwide survey.

METHODS

The primary survey, in which a questionnaire was sent to 3,799 selected departments (including neurology/internal medicine, pediatrics, and ophthalmology), explored the estimated number and prevalence of patients with MS or NMOSD in 2017, and the secondary survey collected detailed characteristics of the patients using a second questionnaire.

RESULTS

The response rates for the primary and secondary surveys were 60.1% and 53.9%, respectively. The estimated total number of patients with MS or NMOSD was 24,800, 2.5-fold higher than that in the fourth survey in 2003. The crude prevalence was 19.6 per 100,000 patients (14.2 for MS and 5.4 for NMOSD), compared with 7.7 per 100,000 patients in the fourth survey. Patients with MS showed milder disability (median Expanded Disability Status Scale [EDSS] score: 2.0 [interquartile range 1.0-4.5] vs 2.5 [1.0-6.0]), decreased secondary progression (8.5% vs 15.2%), and increased usage of disease-modifying drugs (63.7% vs 37.2%) compared with those with conventional MS in the fourth survey. The proportions of oligoclonal bands and Barkhof criteria fulfillment on MRI, which are features of classical MS, increased with advancing year of birth. Patients with NMOSD also showed less disability and shorter disease duration than patients with optic-spinal MS in the fourth survey (EDSS score: 3.5 [2.0-5.5] vs 3.8 [2.0-6.0]; disease duration: 8.0 [3.9-14.8] vs 10.0 [5.0-16.0]). Among patients with NMOSD, disability was exacerbated by a history of longitudinally extensive spinal cord lesions and anti-AQP4 antibody positivity, which both decreased with advancing year of birth.

DISCUSSION

The prevalences of MS (particularly with classical features) and NMOSD have been increasing in Japan, suggesting the contribution of environmental factors. However, disabilities in patients with MS and NMOSD have been mitigated. Extensive usage of various disease-modifying drugs could be a factor contributing to this disability mitigation in MS.

摘要

背景与目的

日本此前的 4 项多发性硬化症（MS）全国性调查均在发现水通道蛋白 4（AQP4）抗体之前进行；因此，视神经脊髓炎谱系疾病（NMOSD）被包含在 MS 中，即视神经脊髓 MS。我们旨在使用第 5 次全国性调查，分别阐明日本 MS 和 NMOSD 的流行病学特征和趋势。

方法

首次调查向 3799 个选定科室（包括神经病学/内科、儿科和眼科）发送了问卷，调查了 2017 年 MS 或 NMOSD 患者的估计人数和患病率，第二次调查使用第二份问卷收集了患者的详细特征。

结果

首次和二次调查的回复率分别为 60.1%和 53.9%。MS 或 NMOSD 患者的估计总数为 24800 人，是 2003 年第 4 次调查的 2.5 倍。粗患病率为每 100000 人 19.6 例（MS 为 14.2 例，NMOSD 为 5.4 例），而第 4 次调查为每 100000 人 7.7 例。与第 4 次调查中的传统 MS 患者相比，MS 患者的残疾程度较轻（扩展残疾状态量表[EDSS]评分中位数：2.0 [四分位距 1.0-4.5] vs 2.5 [1.0-6.0]），继发性进展减少（8.5% vs 15.2%），疾病修饰药物使用率增加（63.7% vs 37.2%）。在第 4 次调查中，与经典 MS 相关的寡克隆带和 Barkhof 标准符合率等特征的比例随着出生年份的增加而增加。与第 4 次调查中的视神经脊髓 MS 患者相比，NMOSD 患者的残疾程度和疾病持续时间也较短（EDSS 评分：3.5 [2.0-5.5] vs 3.8 [2.0-6.0]；疾病持续时间：8.0 [3.9-14.8] vs 10.0 [5.0-16.0]）。在 NMOSD 患者中，纵向广泛脊髓病变和抗 AQP4 抗体阳性的病史会导致残疾加重，这两种情况都随着出生年份的增加而减少。