题名 | The description of neuromyelitis optica spectrum disorder: Patient registry in Yangtze River Delta area of China |
作者 | |
发表日期 | 2022-10 |
发表期刊 | MULTIPLE SCLEROSIS AND RELATED DISORDERS 影响因子和分区 |
语种 | 英语 |
原始文献类型 | Article |
关键词 | Neuromyelitis optica spectrum disorder Aquaporin-4 |
其他关键词 | ANTIBODY POSITIVITY ; RISK-FACTORS ; RELAPSES ; MARKER ; SERUM |
摘要 | Objective: To describe the clinical features of neuromyelitis optica spectrum disorder (NMOSD) through patient registry in Yangtze River Delta area of China. Methods: A total of 502 consecutive patients diagnosed with aquaporin-4 antibody (AQP4-ab)-positive NMOSD were registered between December 2018 to January 2021 in multiple tertiary referral centers within the framework of Yangtze River Delta of China. Their baseline data were reviewed, and follow-up clinical information were collected prospectively. Results: The mean age at onset was 37.3 (range 3-80 years) years and the female-to-male ratio was 8.1:1. The median disease duration was 47 months (interquartile range [IQR] 25-84 months). A total of 1372 attacks of the 502 patients were recorded till the last follow-up, with a median annualized relapse rate of 0.4 (IQR 0.3-0.6). Nearly one-fourth (24.5%, 336/1372) of the attacks had prodromic events, including upper respiratory tract infection (36.3%, 122/336), fever (20.2%, 68/336) and pregnancy-related issues (17.9%, 60/336), etc. Myelitis was the most common attack type throughout the disease course (51.4%, 705/1372), followed by optic neuritis (ON, 43.1%, 592/1372). As for onset phenotype, ON (37.3%, 187/502) prevailed over myelitis (28.3%, 142/ 502). The median time to first relapse was 12 months (IQR 5-25 months). Patients with brainstem encephalitis at onset were more likely to have other anatomical region involved in subsequent attacks (p < 0.001), compared to other onset type. The median serum AQP4-ab titer measured by cell-based assays was 1:100 (IQR 1:32-1:320, range 1:10-1:10,000). The baseline AQP4-ab titer in cerebrospinal fluid (r = 0.542, p < 0.001), overall ARR (r = 0.232, p < 0.001) and the EDSS scores at last follow-up (r = 0.119, p = 0.022) significantly correlated with baseline serum AQP4-ab titer. Antinuclear antibodies (48.4%), thyroid peroxidase antibodies (30.7%), and anti -SSA antibodies (26.2%) represented the most frequent concomitant antibodies, while autoimmune thyroid dis-orders (13.1%, 66/502) and Sjogren's syndrome (10.8%, 54/502) were the most common accompanying autoimmune diseases. Till the last follow-up, 403 patients received preventive treatments. Azathioprine represented the most common initial treatment, mycophenolate mofetil and rituximab was the most common second and third-line treatment, respectively. The EDSS score at the last follow-up ranged from 0 to 8.5 with a median of 2 (IQR 1-3). Conclusions: A comprehensive clinical picture of patients with AQP4-ab-positive NMOSD in Yangtze River Delta area of China was presented. More information on disease tragedy and predictive prognostic factors could be generated through long-term observations. |
资助项目 | National Natural Science Foun-dation of China [81771296, 81801196, 82171341]; Shanghai Municipal Science and Technology Major Project [2018SHZDZX01]; ZHANGJIANG LAB; National Key Research and Development Program of China [2016YFC0901504] |
出版者 | ELSEVIER SCI LTD |
出版地 | OXFORD |
ISSN | 2211-0348 |
EISSN | 2211-0356 |
卷号 | 66页码:104023 |
DOI | 10.1016/j.msard.2022.104023 |
页数 | 8 |
WOS类目 | Clinical Neurology |
WOS研究方向 | Neurosciences & Neurology |
WOS记录号 | WOS:000829478300005 |
收录类别 | SCIE ; PUBMED ; SCOPUS |
URL | 查看原文 |
PubMed ID | 35843144 |
SCOPUSEID | 2-s2.0-85134471911 |
通讯作者地址 | [Quan, Chao]Department of Neurology and Rare Disease Center,Huashan Hospital,Shanghai Medical College,Fudan University,12 Wulumuqi Road (Mid),Shanghai,200040,China |
Scopus学科分类 | Neurology;Neurology (clinical) |
引用统计 | |
文献类型 | 期刊论文 |
条目标识符 | https://kms.wmu.edu.cn/handle/3ETUA0LF/154151 |
专题 | 第一临床医学院(信息与工程学院)、附属第一医院_内科学_神经内科 |
通讯作者 | Quan, Chao |
作者单位 | 1.Department of Neurology and Rare Disease Center,Huashan Hospital,Shanghai Medical College,Fudan University,12 Wulumuqi Road (Mid),Shanghai,200040,China; 2.National Center for Neurological Disorders (NCND),China; 3.Department of Neurology,The First Affiliated Hospital of Wenzhou Medical University,Zhejiang,Wenzhou,China; 4.Department of Neurology,Sir Run Shaw Hospital,School of Medicine,Zhejiang University,Zhejiang,Hangzhou,China; 5.Department of Ophthalmology and Vision Science,Eye and ENT Hospital,Fudan University,Shanghai,China |
推荐引用方式 GB/T 7714 | Huang, Wenjuan,Tan, Hongmei,Xia, Junhui,et al. The description of neuromyelitis optica spectrum disorder: Patient registry in Yangtze River Delta area of China[J]. MULTIPLE SCLEROSIS AND RELATED DISORDERS,2022,66:104023. |
APA | Huang, Wenjuan., Tan, Hongmei., Xia, Junhui., Li, Wenyu., Li, Xiang., ... & Quan, Chao. (2022). The description of neuromyelitis optica spectrum disorder: Patient registry in Yangtze River Delta area of China. MULTIPLE SCLEROSIS AND RELATED DISORDERS, 66, 104023. |
MLA | Huang, Wenjuan,et al."The description of neuromyelitis optica spectrum disorder: Patient registry in Yangtze River Delta area of China".MULTIPLE SCLEROSIS AND RELATED DISORDERS 66(2022):104023. |
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