Petersson Malin, Feresiadou Amalia, Jons Daniel, Ilinca Andreea, Lundin Fredrik, Johansson Rune, Budzianowska Anna, Roos Anna-Karin, Kågström Viktor, Gunnarsson Martin, Sundström Peter, Piehl Fredrik, Brauner Susanna
From the Department of Clinical Neuroscience (M.P., F.P., S.B.), Karolinska Institutet, Stockholm; Department of Neuroscience, Neurology (A.F.), Uppsala University; Department of Clinical Neuroscience, Institute of Neuroscience and Physiology (D.J.), the Sahlgrenska Academy, University of Gothenburg; Department of Neurology (D.J.), Sahlgrenska University Hospital, Gothenburg; Department of Clinical Sciences Lund, Neurology (A.I.), Skåne University Hospital, Lund University, Malmö; Departments of Neurology (F.L.) and Biomedical and Clinical Sciences (F.L., A.B.), Division of Neurobiology, Linköping University; Department of Neurology and Rehabilitation (R.J.), Karlstad Central Hospital; Department of Internal Medicine in Jönköping (A.B.), Section of Neurology, Region Jönköping County; Department of Clinical Science, Neurosciences (A.-K.R.), Unit of Neurology, Umeå University, Östersund; Rehabilitation Clinic (V.K.), Sundsvall Hospital; Department of Neurology, Faculty of Medicine and Health (M.G.), Örebro University; Department of Clinical Science, Neurosciences (P.S.), Umeå University; and Department of Neurology (F.P., S.B.), Karolinska University Hospital, Stockholm, Sweden.
Neurology. 2021 Oct 4;97(14):e1382-e1391. doi: 10.1212/WNL.0000000000012604.
To describe myasthenia gravis activities of daily living (MG-ADL) in relation to clinical characteristics in a large Swedish nationwide cohort.
In a cross-sectional prevalence cohort study, the Genes and Environment in Myasthenia Gravis study, performed from November 2018 through August 2019, patients with myasthenia gravis (MG) were invited to submit an extensive 106-item life environment questionnaire, including the MG-ADL score. Patients were classified into early-onset MG (EOMG, <50 years), late-onset MG (LOMG, ≥50 years), or thymoma-associated MG (TAMG). Comparisons of disease-specific characteristics were made between subgroups, sexes, and different MG-ADL scores.
A total of 1,077 patients were included, yielding a 74% response rate: 505 (47%) were classified as EOMG, 520 (48%) LOMG, and 45 (4%) TAMG. Mean age at inclusion was 64.3 years (SD 15.7) and mean disease duration was 14.6 years (SD 14.0). Complete MG-ADL scores (n = 1,035) ranged from 0p to 18p, where 26% reported a score of 0p. Higher MG-ADL scores were associated with female sex, obesity, and diagnostic delay (odds ratio [OR] 1.62, 1.72, and 1.69; = 0.017, 0.013, and 0.008) and inversely correlated with high educational attainment (OR 0.59; = 0.02), but not with age at inclusion, disease subtype, or disease duration. Almost half of the population (47%) reported MG-ADL ≥3p, corresponding to an unsatisfactory symptom state.
In this nationwide study, comprising more than 40% of the prevalent MG population in Sweden, almost half of the patients reported current disease symptoms associated with an unsatisfactory symptom state, indicating the need for improved treatment options.
在瑞典一项大型全国性队列研究中，描述重症肌无力日常生活活动（MG-ADL）与临床特征的关系。
在2018年11月至2019年8月进行的一项横断面患病率队列研究“重症肌无力的基因与环境研究”中，邀请重症肌无力（MG）患者提交一份包含106个项目的详尽生活环境问卷，其中包括MG-ADL评分。患者被分为早发型MG（EOMG，年龄<50岁）、晚发型MG（LOMG，年龄≥50岁）或胸腺瘤相关MG（TAMG）。对亚组、性别以及不同MG-ADL评分之间的疾病特异性特征进行比较。
共纳入1077例患者，应答率为74%：505例（47%）被归类为EOMG，520例（48%）为LOMG，45例（4%）为TAMG。纳入时的平均年龄为64.3岁（标准差15.7），平均病程为14.6年（标准差14.0）。完整的MG-ADL评分（n = 1035）范围为0分至18分，其中26%报告评分为0分。较高的MG-ADL评分与女性、肥胖和诊断延迟相关（比值比[OR]分别为1.62、1.72和1.69；P = 0.017、0.013和0.008），与高学历呈负相关（OR 0.59；P = 0.02），但与纳入时的年龄、疾病亚型或病程无关。几乎一半的人群（47%）报告MG-ADL≥3分，这对应着不满意的症状状态。
在这项涵盖瑞典40%以上MG现患人群的全国性研究中，几乎一半的患者报告目前的疾病症状与不满意的症状状态相关，这表明需要改进治疗方案。
