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Fatigue and associated factors in myasthenia gravis: a nationwide registry study.

ABSTRACT: Fatigue is commonly associated with myasthenia gravis (MG), but factors contributing to fatigue development in MG are incompletely understood. This nationwide cross-sectional registry study included 1464 patients diagnosed with autoimmune MG, recruited between February 2019 and April 2023. Frequency and severity of fatigue was assessed at study inclusion using the patient-reported Chalder Fatigue Questionnaire (CFQ). Frequency of fatigue was 59%. Fatigue severity strongly correlated with both patient-reported and physician-assessed MG outcome measures (MG-ADL, MG-QoL15, QMG and MGFA classes) and was associated with a history of myasthenic exacerbation and/or myasthenic crises and a delay in diagnosis of more than 1 year after symptom onset. Fatigue was more prevalent in women and coincided with symptoms of depression, anxiety, and sleep dissatisfaction. Differences in fatigue severity were observed between antibody (ab) subgroups, with highest fatigue severity in LRP4-ab-positive patients and lowest fatigue severity in AChR-ab-positive patients. Fatigue is a frequent and clinically highly relevant symptom of MG. Early diagnosis and prevention of MG crises may limit the long-term burden of fatigue in patients with MG.

SUBMITTER: Funke M

PROVIDER: S-EPMC11319509 | biostudies-literature | 2024 Aug

REPOSITORIES: biostudies-literature

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Fatigue and associated factors in myasthenia gravis: a nationwide registry study.

Funke Mattea M Eveslage Maria M Zschüntzsch Jana J Hagenacker Tim T Ruck Tobias T Schubert Charlotte C Schroeter Michael M Meisel Andreas A Wiendl Heinz H Hoffmann Sarah S Lünemann Jan D JD

Journal of neurology 20240613 8

Fatigue is commonly associated with myasthenia gravis (MG), but factors contributing to fatigue development in MG are incompletely understood. This nationwide cross-sectional registry study included 1464 patients diagnosed with autoimmune MG, recruited between February 2019 and April 2023. Frequency and severity of fatigue was assessed at study inclusion using the patient-reported Chalder Fatigue Questionnaire (CFQ). Frequency of fatigue was 59%. Fatigue severity strongly correlated with both pa ...[more]

PMID: 38871821

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Project description:ObjectiveTo study the updated prevalence and clinical features of myasthenia gravis (MG) in Japan during 2017.MethodsWe sent survey sheets to the randomly selected medical departments (number = 7,545). First, we asked the number of MG patients who visited medical departments from January 1, 2017, to December 31, 2017. Then, we sent the second survey sheet to the medical departments that answered the first survey to obtain the clinical information of patients who received MG diagnosis between January 1, 2015, and December 31, 2017.ResultsThe received answer to the first survey were 2,708 (recovery rate: 35.9%). After all, the prevalence of the 100,000 population was estimated as 23.1 (95%CI: 20.5-25.6). As a result of the second survey, we obtained 1,464 case records. After checking the duplications and lacking data, we utilized 1,195 data for further analysis. The median [interquartile range (IQR)] from the onset age of total patients was 59 (43-70) years old. The male-female ratio was 1: 1.15. The onset age [median (IQR)] for female patients was 58 (40-72) years old, and that for male patients was 60 (49-69) years old (Wilcoxon-Mann-Whitney test, p = 0.0299). We divided patients into four categories: 1) anti-acetylcholine receptor antibody (AChRAb) (+) thymoma (Tm) (-), 2) AChRAb(+)Tm(+), 3) anti-muscle-specific kinase antibody (MuSKAb) (+), and AChRAb(-)MuSKAb(-) (double negative; DN). The onset age [median (IQR)] of AChRAb(+)Tm(-) was 64 (48-73) years old, and AChRb(+)Tm(+) was 55 (45-66), MuSKAb(+) was 49 (36-64), DN was 47 (35-60) year old. The multivariate logistic regression analysis using sex, initial symptoms, repetitive nerve stimulation test (RNST), and edrophonium test revealed that sex, ocular symptoms, bulbar symptoms, and RNST were factors to distinguish each category. The myasthenia gravis activities of daily living profile at the severest state were significantly higher in MuSKAb(+). MuSKAb(+) frequently received prednisolone, tacrolimus plasmapheresis, and intravenous immunoglobulin; however, they received less acetylcholine esterase inhibitor. 99.2% of AChRAb(+)Tm(+) and 15.4% of AChRAb(+)Tm(-) received thymectomy. MuSKAb(+) did not receive thymectomy, and only 5.7% of DN received thymectomy. The prognosis was favorable in all categories.ConclusionOur result revealed that the prevalence of Japanese MG doubled from the previous study using the same survey method in 2006. We also found that the onset age shifted to the elderly, and the male-female ratio reached almost even. Classification in four categories; AChRAb(+)Tm(-), AChRAb(+)Tm(+), MuSKAb(+), and DN, well describe the specific clinical features of each category and differences in therapeutic approaches.

Project description:BackgroundMyasthenia gravis (MG) is the most common primary disorder of neuromuscular transmission, but the incidence of MG in China is unknown. We conducted the first nationwide study to determine the incidence and mortality rates of MG in all age groups at the national level in China.MethodsThis national population-based registry study is based on the database of the Hospital Quality Monitoring System of National Health Commission, which covers 1665 hospitals providing myasthenia gravis care in 31 provinces and municipalities across China. 94,638 hospital admissions for 59,243 myasthenia gravis patients were identified from January 1st, 2016 to December 31st, 2018. Myasthenia gravis was identified by ICD-10 codes (G70). Incidence of myasthenia gravis was stratified by age, sex, and province.FindingsOf 59,243 patients, 30,503 individuals with myasthenia gravis were newly diagnosed. Age and sex adjusted incidence of myasthenia gravis was 0.68 per 100,000 person-years, with highest in the age group of 70-74 years. The incidence in females was 0.76 per 100,000 and 0.60 per 100,000 in males. The admission mortality rate was 14.69‰. Respiratory failure was the leading cause of death in patients with myasthenic crisis. There were 14,840 patients with thymomas, encompassing 14,636 (26.5%) adults and 204 (7.1%) juveniles. 9453 (63.7%) patients with thymomas underwent thymomectomy. The median length of hospital stay was 8 days (interquartile range (IQR) 4 to 15 days) with median hospitalization cost $1037 (IQR $493 to $2925). The Basic Medical Insurance was the most common payment method, covering 67.4% of patients.InterpretationThe age and sex adjusted incidence of MG was 0.68 per 100,000 person-years in China. The admission mortality rate was 14.69‰. Most cases of new onset MG occurred in the seventh decade of life.FundingNational Science Foundation of China (91642205, and 81830038); Advanced Innovation Center for Human Brain Protection, Capital Medical University, Beijing.

Dataset Information

Fatigue and associated factors in myasthenia gravis: a nationwide registry study.

Publications

Fatigue and associated factors in myasthenia gravis: a nationwide registry study.

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