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大西洋岛屿圣米格尔岛多发性硬化症的发病率、患病率及特征。

Incidence, prevalence and characteristics of MS in São Miguel, an island in the Atlantic.

作者信息

Lopes Pedro, Martins Ana, Lopes José

机构信息

Serviço de Neurologia do Hospital do Divino Espírito Santo de Ponta Delgada, EPE, Portugal.

Serviço de Neurologia do Centro Hospitalar e Universitário de Coimbra, Portugal.

出版信息

Mult Scler Relat Disord. 2020 Sep;44:102254. doi: 10.1016/j.msard.2020.102254. Epub 2020 Jun 2.

Abstract

BACKGROUND

Multiple sclerosis (MS) is a chronic inflammatory-demyelinating disease of the central nervous system (CNS). With approximately 2.3 million people estimated to live with MS globally, incidence and prevalence of the disease vary significantly across regions. Generically, the global distribution of MS varies with geographic latitude. However, MS epidemiological data in Southern Europe and Mediterranean countries revealed areas of unexpectedly high prevalence and incidence of MS, thereby demonstrating that clear exceptions to the variation of MS with latitude exist. Epidemiological evidence on MS in Portugal is limited to studies concerning specific regions of the country and therefore not representative of the entire country. More studies form different regions of the country are needed in order to effectively establish MS prevalence and incidence in Portugal. The aim of this study is to determine MS incidence and prevalence, as well as gather information on other clinical data in São Miguel, the biggest island of the Azores, a Portuguese archipelago situated in the North Atlantic.

METHODS

This is a cross-sectional study of MS patients from the only hospital of São Miguel, an island with a population of 137,150 habitants. Only patients from São Miguel were included in the study. 1/07/2019 was established as the prevalence day and demographic characteristics (sex, age at diagnosis and year of diagnosis) and clinical outcome (type of the disease, treatment, clinical severity score [EDSS]) were reported. 5-year Incidence of MS was studied from 1/07/2014 to 01/07/2019.

RESULTS

47 patients were enrolled in the study, 27 (57.4%) were female and 20 (42.6%) were male. 43 patients were classified as having RRMS and 4 patients classified as having SPMS. Prevalence of MS in São Miguel island was 34.3/100.000. The prevalence for females was 38.5/100.000 and for males was 29.9/100.000. Age-adjusted and sex-adjusted (to the population of the European Union in 2019) MS prevalence were 31.1/100.000 and 34.3/100.000 respectively. Incidence was 2.04/100,000 person-years for the overall population, 2.56/100,000 person-years for females and 1.49/100,000 person-years for males. Age-adjusted and sex-adjusted MS incidence were 1.74/100.000 person-years and 2.04/100.000 person-years respectively. Regarding clinical characteristics, the mean EDSS was 2.93 ± 1.85, the mean age at the time of the diagnosis was 31.77 ± 8.47 years and the mean time of evolution of the disease (period between diagnosis and prevalence date) was 9.19 ± 6.55 years. Treatment options were diverse and included first and second-line treatments as well as Immune reconstitution therapies. Interferon-beta was the most prescribed medication. 2 patients were not making any DMT.

CONCLUSIONS

The incidence and prevalence of MS reported by our study is lower than what was registered by other studies. Several reasons may explain these results such as the ancestry of the population of São Miguel, migratory flows and limitations in detecting all MS patients. Incidence and prevalence were higher in females. A marked heterogeneity in clinical characteristics was seen, with great variability in degree of disability, age at diagnosis and time of MS evolution. Treatment options were quite varied in the population, with Interferon-beta being the most prescribed DMT.

摘要

背景

多发性硬化症(MS)是一种中枢神经系统(CNS)的慢性炎症性脱髓鞘疾病。据估计,全球约有230万人患有MS,该病的发病率和患病率在不同地区差异显著。一般来说,MS的全球分布随地理纬度而变化。然而,南欧和地中海国家的MS流行病学数据显示,存在MS患病率和发病率意外高的地区,从而表明MS随纬度变化存在明显例外情况。葡萄牙关于MS的流行病学证据仅限于该国特定地区的研究,因此不代表整个国家。需要开展该国不同地区的更多研究,以便有效确定葡萄牙的MS患病率和发病率。本研究的目的是确定亚速尔群岛最大岛屿圣米格尔岛(位于北大西洋的葡萄牙群岛)的MS发病率和患病率,并收集其他临床数据信息。

方法

这是一项对圣米格尔岛唯一一家医院的MS患者进行的横断面研究,该岛有137,150名居民。本研究仅纳入来自圣米格尔岛的患者。将2019年7月1日确定为患病率统计日,并报告人口统计学特征(性别、诊断时年龄和诊断年份)以及临床结果(疾病类型、治疗、临床严重程度评分[EDSS])。研究了2014年7月1日至2019年7月1日期间MS的5年发病率。

结果

47例患者纳入本研究,27例(57.4%)为女性,20例(42.6%)为男性。43例患者被分类为复发缓解型多发性硬化症(RRMS),4例患者被分类为继发进展型多发性硬化症(SPMS)。圣米格尔岛的MS患病率为34.3/100,000。女性患病率为38.5/100,000,男性患病率为29.9/100,000。年龄调整和性别调整(以2019年欧盟人口为标准)后的MS患病率分别为31.1/100,000和34.3/100,000。总体人群的发病率为2.04/100,000人年,女性为2.56/100,000人年,男性为1.49/100,000人年。年龄调整和性别调整后的MS发病率分别为1.74/100,000人年和2.04/100,000人年。关于临床特征,平均EDSS为2.93±1.85,诊断时的平均年龄为31.77±8.47岁,疾病进展的平均时间(诊断至患病率统计日之间的时间段)为9.19±6.55年。治疗选择多样,包括一线和二线治疗以及免疫重建疗法。β-干扰素是最常开具的药物。2例患者未接受任何疾病修正治疗(DMT)。

结论

我们研究报告的MS发病率和患病率低于其他研究记录的水平。有几个原因可以解释这些结果,如圣米格尔岛居民的血统、移民流动以及检测所有MS患者的局限性。女性的发病率和患病率更高。临床特征存在明显异质性,残疾程度、诊断时年龄和MS进展时间差异很大。该人群的治疗选择相当多样,β-干扰素是最常开具的DMT。

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