Sciancalepore Francesco, Lombardi Niccolò, Valdiserra Giulia, Bonaso Marco, Cappello Emiliano, Hyeraci Giulia, Crescioli Giada, Celani Maria Grazia, Cantisani Teresa Anna, Brunori Paola, Vecchi Simona, Bacigalupo Ilaria, Locuratolo Nicoletta, Lacorte Eleonora, Vanacore Nicola, Kirchmayer Ursula
National Center for Disease Prevention and Health Promotion, Italian National Institute of Health, Rome, Italy.
Department of Human Neuroscience, Sapienza University of Rome, Rome, Italy.
Neuroepidemiology. 2024 Oct 9:1-14. doi: 10.1159/000539577.
No systematic reviews were published in the last years investigating epidemiological data, involving myasthenia gravis (MG) and related myasthenic syndromes. This systematic review aimed to estimate the prevalence, incidence, and mortality of all MG types and myasthenic syndromes worldwide.
All literature published up to February 2024 was retrieved by searching the databases "Medline," "Embase," "ISI Web of Science" and "CINAHL" using the following search terms: (epidemiolog* OR frequency OR prevalence OR incidence OR mortality) AND (myasth* OR "anti-acetylcholine receptor antibody" OR "AChR" OR "MuSK" OR "anti-muscle specific kinase antibody" OR "LRP4" OR "seronegative MG").
A total of 94 studies, performed between 1952 and 2022, were included. Prevalence of MG ranged from 20 to 475 cases per million, with a mean prevalence of 173.3 (95% confidence interval [CI]: 129.7-215.5) cases per million and a median prevalence of 129.6 cases per million. Incidence rates ranged from 2.3 to 61.3 cases per million person-years, with a mean incidence of 15.7 (95% CI: 11.5-19.9) and a median of 13.3 cases. Mortality rates showed a mean of 1.4 (95% CI: 0.8-2.1) cases per million person-years. Acetylcholine receptor (AChR)-MG was the clinical subtype more frequent in terms of prevalence and incidence.
The prevalence and incidence of MG have significantly increased over the last years worldwide, probably due to the improvement of epidemiological methodologies and current advances in diagnosis. However, we observed a significant variation in frequencies of MG between and within countries because of methodological biases and complex heterogeneity of the disease characterized by several phenotypes and different clinical responses.
过去几年中，没有发表过关于重症肌无力（MG）及相关肌无力综合征流行病学数据的系统评价。本系统评价旨在估计全球所有类型的MG及肌无力综合征的患病率、发病率和死亡率。
通过检索“Medline”“Embase”“ISI Web of Science”和“CINAHL”数据库，使用以下检索词检索截至2024年2月发表的所有文献：（流行病学或频率或患病率或发病率或死亡率）以及（肌无力或“抗乙酰胆碱受体抗体”或“AChR”或“MuSK”或“抗肌肉特异性激酶抗体”或“LRP4”或“血清阴性MG”）。
共纳入了1952年至2022年间进行的94项研究。MG的患病率为每百万人口20至475例，平均患病率为每百万人口173.3例（95%置信区间[CI]：129.7 - 215.5），中位数患病率为每百万人口129.6例。发病率为每百万人口年2.3至61.3例，平均发病率为15.7例（95% CI：11.5 - 19.9），中位数为13.3例。死亡率平均为每百万人口年1.4例（95% CI：0.8 - 2.1）。乙酰胆碱受体（AChR）-MG是患病率和发病率方面更常见的临床亚型。
在过去几年中，全球范围内MG的患病率和发病率显著增加，这可能归因于流行病学方法的改进和当前诊断技术的进步。然而，由于方法学偏差以及该疾病具有多种表型和不同临床反应的复杂异质性，我们观察到国家之间和国家内部MG的频率存在显著差异。
