The Burden Patients with Myasthenia Gravis Experience in Terms of Breathing, Fatigue, Sleep, Mental Health, Discomfort and Usual Activities in Comparison to the General Population.

INTRODUCTION: Myasthenia gravis (MG) is a rare neuromuscular disorder marked by a variable combination of weakness of eye, bulbar, respiratory, axial, and limb muscles. This study compared the experience of people with MG regarding breathing, fatigue, sleep, pain/discomfort, mental health, and usual activities with the general population. METHODS: The MyRealWorld-MG digital, multinational study enrolled patients with MG and collected demographics, PROMIS-Dyspnea, PROMIS-Sleep Disturbance, FACIT-Fatigue, EQ-5D-5L, Health Utilities Index (HUI-3), Hospital Anxiety and Depression Scale (HADS), MG-Activities of Daily Living (MG-ADL), and MG-Quality-of-Life (MG-QoL-15r). Comparisons with the general population were based on PROMIS population norms, published literature, or on data from a digital, multinational, observational study which enrolled a representative sample of the general population (POPUP). RESULTS: In MyRealWorld-MG (N = 2074), patients experienced higher intensity, frequency, and duration of PROMIS shortness of breath than a US population (p < 0.0001). Patients with MG had higher PROMIS-Sleep Disturbance scores than POPUP (53.7 vs 50.0, p < 0.0001), and 54.9% of patients had clinically severe FACIT-Fatigue scores vs 6.8% in POPUP (p < 0.0001). Among patients with MG, 69.6% and 18.5% had moderate-to-severe HADS-Anxiety and HADS-Depression compared to 20.3% and 6.9% in POPUP (p < 0.001). Statistically significant and strong associations were found between fatigue, sleep, dyspnea, usual activities, and emotions. All outcomes worsened with more severe disease. CONCLUSION: A considerable burden was observed in this comparison of breathing, sleep, fatigue, mental health, and usual activities between patients with MG and the general population, using data from two international studies and published population norms. Even mildly affected patients had significantly worse outcomes than the general population.

Development and validation of the scale for symptom clusters in patients with myasthenia gravis.

BACKGROUND: Patients with myasthenia gravis(MG)often experience multiple symptoms concurrently, which can have an adverse effect on their quality of life(QOL). However, a specific, systemic and reliable scale for symptom clusters in MG is lacking. AIMS: To develop reliable assessment scale for symptom clusters in patients with MG. DESIGN: A cross-sectional descriptive study. METHODS: Based on the unpleasant symptom theory(TOUS), the first draft of the scale was developed through review literature, qualitative interview, and Delphi expert correspondence, the items of the scale were presented and adjusted through cognitive interviews with 12 patients. To conveniently assess the validity and reliability of the scale, a cross-sectional survey was conducted in 283 patients with MG who were recruited from Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, from June to September 2021. RESULTS: The final symptom cluster scale for patients with MG consisted of 19 items(MGSC-19), with a content validity index ranging from 0.828 to 1.000 for each item and the content validity index was 0.980. Four common variables (ocular muscle weakness, general muscular weakness, treatment-related side effects, and psychiatric problems) were identified by exploratory factor analysis, which explained 70.187% of the total variance. The correlation coefficients between the scale dimension and the overall score ranged from 0.395 to 0.769 (all P < 0.01), while the correlation coefficients between dimensions varied from 0.324 to 0.510 (all P < 0.01). The Cronbach's alpha, retest reliability, and half reliability were 0.932, 0.845, and 0.837, respectively. CONCLUSION: The validity and reliability of MGSC-19 were generally good. This scale can be employed to identify the symptom clusters to help healthcare givers develop individualized symptom management measures for patients with MG.

Developing the health state classification system for a condition-specific preference-based measure for patients with myasthenia gravis.

PURPOSE: This study aimed to generate the health state classification system (HCS) of a condition-specific preference-based measure to capture the health-related quality of life (HRQoL) of patients with myasthenia gravis (MG) from the 15-item Myasthenia Gravis Quality of Life Scale (MG-QoL15r). MATERIALS AND METHODS: An HCS was derived from the MG-QoL15r in a large sample of 1739 patients with MG based on psychometric analysis, including factor analysis, item response theory analysis, and discussions with experts. Reliability, construct and convergent validity, and item fit of the HCS were further assessed using another sample. RESULTS: The HCS has six dimensions: social activity, hobbies and fun activities, meeting family needs, work performance, mobility, and emotion, and it demonstrates good internal consistency reliability. The unidimensionality of the HCS was confirmed using confirmatory factor analysis. Satisfied convergent validity was supported by a significant association with the 12-item Short Form Survey (SF-12). CONCLUSIONS: Based on a solid process of development and consultations with clinical professionals and patients, a valid MG-specific preference-based measure, MGQoL-6D, was developed. Further research will estimate the local preference weight to support the MG-related cost-utility analysis.IMPLICATION FOR REHABILITATIONA new condition-specific health state classification system (HCS) named Myasthenia gravis Quality of Life Scale (MGQoL-6D) is proposed.MGQoL-6D classifies MG health states as a combination of six dimensions with three response levels.The dimensions of the HCS are social activity, hobbies and fun activity, family needs, work performance, mobility, and emotion.The HCS and the upcoming value set of the MGQoL-6D could support the cost-effectiveness analysis of MG-related clinical and rehabilitated interventions.

Physical and mental fatigue in myasthenia gravis and its correlation with other symptoms.

INTRODUCTION: Muscle weakness and easy fatigability are the clinical hallmarks of myasthenia gravis (MG). However, fatigue perception, which can be seen quite often in myasthenic patients, and its effect on the quality of life, irrespective of motor deficit, has not been elucidated yet. The aim is to evaluate the frequency of fatigue in myasthenic patients with nearly full muscle strength and the effect of fatigue on quality of life by assessing its correlation with other symptoms. METHODS: Fifty-three patients with ocular or mild generalized MG in remission or minimal manifestations completed the questionnaires measuring the severity of MG and quality of life (MG Composite Scale and MG-Activities of Daily Living Profile). Both patient group and control group (53 healthy volunteers)completed the scales assessing fatigue [Fatigue Assessment Scale (FAS) and Fatigue Impact Scale (FIS)], depression [Beck Depression Inventory (BDI)] and sleep (Epworth Sleepiness Scale). Disease severity was assessed using MG Foundation of America (MGFA) and MGFA Post-Intervention Status classifications. RESULTS: FAS, FIS physical and BDI scores were significantly higher in patients compared to the control group (p = 0.003, p = 0.001, and p = 0.003, respectively) and fatigue was associated with depression and daytime sleepiness. Inpatient group, depressive symptoms and daytime sleepiness were higher in females (p = 0.019 and p = 0.013). The mean values of FIS total and cognitive scores were higher in patients with generalized MG (p = 0.033 and p = 0.045). Fatigue scores correlated with motor signs. DISCUSSION: Fatigue can be seen in MG independently from muscle weakness and is an important symptom worsening the quality of life.

Burden of disease in myasthenia gravis: taking the patient's perspective.

BACKGROUND: Myasthenia gravis (MG) leads to exertion-dependent muscle weakness, but also psychological and social well-being are limited. We aim to describe the burden of disease in MG including sociodemographic, economical, psychosocial as well as clinical aspects, to compare health-related quality of life (HRQoL) of patients with MG to the general population (genP) and to explore risk factors for a lower HRQoL. METHODS: This case-control study was conducted with MG patients of the German Myasthenia Association. A questionnaire-based survey included sociodemographic and clinical data as well as standardized questionnaires, e.g. the Short Form Health (SF-36). HRQoL was compared to genP in a matched-pairs analysis. Participants of the German Health Interview and Examination Survey for Adults (DEGS1) served as control group. RESULTS: In our study, 1660 MG patients participated and were compared to 2556 controls from the genP. Patients with MG showed lower levels of physical functioning (SF-36 mean 56.0, SD 30.3) compared to the genP (mean 81.8, SD 22.1, adjusted difference: 25, 95% CI 22-29) and lower mental health sub-score (SF-36 mean 67.3, SD 19.8, vs. 74.1, SD 16.7, adjusted difference: 5, 95% CI 2-8). Female gender, higher age, low income, partnership status, lower activities of daily life, symptoms of depression, anxiety and fatigue and self-perceived low social support were associated with a lower HRQoL in MG patients. DISCUSSION: HRQoL is lower in patients with MG compared to genP. The burden of MG on patients includes economic and social aspects as well as their emotional well-being. New therapies must achieve improvements for patients in these areas. TRIAL REGISTRATION INFORMATION: Clinicaltrials.gov, NCT03979521, submitted: June 7, 2019, first patient enrolled: May 1, 2019, https://clinicaltrials.gov/ct2/show/NCT03979521.

Illness perception characteristics and influencing factors in adult patients with myasthenia gravis in China.

OBJECTIVE: We aimed to examine the illness perception characteristics of patients with myasthenia gravis and analyze the associated factors. METHODS: A general information questionnaire, Illness Perception Questionnaire, and Simplified Coping Style Questionnaire were used to survey 90 patients with myasthenia gravis. One-way analysis of variance and multivariate linear regression were used for statistical analysis. RESULTS: The timeline (acute/chronic), consequence, and emotional representation scores of patients with myasthenia gravis were 18.31 ± 4.47, 18.58 ± 3.57, and 20.80 ± 4.56, respectively. Multivariate stepwise linear regression analysis showed that marital status, disease duration, educational level, disease type, and coping style are factors associated with illness perception in patients with myasthenia gravis. CONCLUSION: Patients who have myasthenia gravis have a negative illness perception. Medical professionals should provide targeted intervention measures based on the factors associated with illness perception.

Physical Activity and Sedentary Behaviour in People with Myasthenia Gravis: A Cross-Sectional Study.

BACKGROUND: Despite improvements in the medical management of myasthenia gravis (MG) in recent years, patients continue to report poor health and wellbeing outcomes such as high levels of fatigue, reduced quality of life (QoL), walking limitation and lowered balance confidence. Physical activity has been shown to be associated with these outcomes in other populations, however, there has been limited research in adults with MG. OBJECTIVE: To describe physical activity and sedentary behaviour in adults with MG and to explore associations between these behaviours and fatigue, QoL, balance confidence and walking limitation. METHODS: A self-report online survey was used to assess physical activity, sedentary behaviour, fatigue, QoL, balance confidence and walking limitation in adults with MG. Multiple linear regression was used to examine associations and descriptive statistics were used to analyse participant characteristics, physical activity, and sedentary behaviour. RESULTS: Eighty-five adults with MG were included (mean age 48±16 years). Over half of participants (n = 53, 62.4%) reported sufficient physical activity to meet public health guidelines. Participants reported an average of 9 h/day of sedentary time (mean 9.0±3.5). Physical activity and fatigue (R2 = 0.196), QoL (R2 = 0.330), walking limitation (R2 = 0.305) and balance confidence (R2 = 0.304) were significantly (p < 0.05) and positively correlated, with no associations found for sedentary behaviour. When patterns of physical activity and sedentary behaviour were combined, lower fatigue (R2 = 0.213), higher QoL (R2 = 0.364), reduced walking limitation (R2 = 0.341) and higher balance confidence (R2 = 0.279) was observed in patients who had greater physical activity levels (> 150 mins/week) and lower sedentary time (< 10 h/day). CONCLUSIONS: Higher physical activity and lower sedentary behaviour is associated with favorable health and wellbeing outcomes in adults with MG. Further research is required to ascertain whether these behaviours may be an appropriate target intervention to improve outcomes in this population.

Long-term efficacy of eculizumab in refractory generalized myasthenia gravis: responder analyses.

OBJECTIVE: Generalized myasthenia gravis (gMG) is an autoimmune disease that causes disabling weakness via damage to the neuromuscular junction. In most patients, the disease is mediated by autoantibodies to the acetylcholine receptor, which activate the complement cascade. Our objective was to analyze response profiles in adult patients with anti-acetylcholine receptor antibody-positive refractory gMG treated with eculizumab-a terminal complement inhibitor-in the REGAIN study or its open-label extension (OLE). METHODS: We retrospectively analyzed Myasthenia Gravis-Activities of Daily Living (MG-ADL) and Quantitative Myasthenia Gravis (QMG) scores recorded during REGAIN and its OLE. Early/late responses were defined as improvement in MG-ADL score (≥3 points) or QMG score (≥5 points) at ≤12 or >12 weeks, respectively, after eculizumab initiation. RESULTS: The analysis included 98 patients. By Week 12 and conclusion of the OLE, MG-ADL response had been achieved at some point by 67.3% and 84.7% of patients, respectively, and QMG response by 56.1% and 71.4%, respectively. Response was observed over multiple consecutive assessments for most patients. At Week 130, the least-squares mean percentage changes (95% CI) from baseline in MG-ADL score were -61.9% (-69.9%, -53.9%) and -47.5% (-59.0%, -36.0%) in early and late MG-ADL responders, respectively; the least-squares mean percentage changes from baseline in QMG score were -40.8% (-48.3%, -33.4%) and -55.5% (-68.4%, -42.7%) in early and late QMG responders, respectively. INTERPRETATION: The findings suggest that, although most patients with refractory gMG will achieve clinical response by Week 12 of eculizumab treatment, first responses can be observed with longer-term treatment.

Impact of COVID-19 pandemic and lockdown in a cohort of myasthenia gravis patients in India.

OBJECTIVE: Myasthenia gravis (MG) is characterized by fluctuating muscle weakness due to immune mediated damage of acetylcholine receptor (AchR). COVID-19 infection, mental stress and non-availability of drugs following lockdown may worsen myasthenic symptoms. We report the impact of COVID 19, and lockdown on the physical and mental health, and quality of sleep in a cohort of MG. METHODS: Thirty-eight MG patients were telephonically interviewed 2months after the declaration of lockdown in India. The difficulty in procuring drugs, complications, and worsening in the MG Foundation of America (MGFA) stage were noted. The patients were enquired about MG Quality of Life 15 (MGQOL15), MG Activity of Daily Living (MGADL), Hospital Anxiety and Depression Scale (HADS) and Pittsburgh Sleep Quality Index (PSQI) using a prefixed questionnaire. Their pre-COVID parameters were retrieved from our earlier trial data, which was completed 4months back. The scores of the above mentioned parameters before and after COVID were compared. RESULTS: Their median age was 45 years, and the median duration of treatment for MG was 4.5years. Eleven (28.9 %) patients were hypertensive and 3(7.9 %) diabetic. All were on prednisolone and 18(47.4 %) received azathioprine. None developed COVID, but three had other infections. Two patients needed hospitalization because of wrong medication in one and severe anxiety-insomnia in another. Following COVID19 and lockdown, MG patients had worsening in MGQOL15, MGADL, HADS and PSQI scores. Pittsburgh Sleep Quality Index score correlated with MGQOL15 and dose of acetylcholine esterase inhibitors. CONCLUSION: COVID-19 and lockdown were associated with anxiety, depression, poor MGQOL and sleep especially in severe MG patients.

Fatigue, self-efficacy and psychiatric symptoms influence the quality of life in patients with myasthenia gravis in Tianjin, China.

BACKGROUND: Muscle weakness related to myasthenia gravis (MG) limits the daily functioning of patients. MG patients often experience subjective symptoms including psychiatric disorders, fatigue, and reduction in self-efficacy. All of which ultimately influence their life. The relationship between the subjective symptoms and health-related quality of life (HRQoL) has never been systematically explored among MG patients. OBJECTIVE: This study aimed to evaluate the HRQoL of patients with MG in China, and to assess the impact of potential predictors of HRQoL. METHODS: This was a cross-sectional observational study in patients with confirmed diagnosis of MG. Patients with MG were assessed using the 36-Item Short-Form Health Survey (SF-36) questionnaire, Self-Rating Depression Scale (SDS), Self-Rating Anxiety Scale (SAS), Fatigue Severity Scale (FSS), and Self-Efficacy for Managing Chronic Disease 6 items scale (SES6G). Disease severity was evaluated by two specialists at the same time. RESULTS: Patients had significantly lower mean SF-36 scores for the categories role physical and general health (GH). The mean physical and mental composite scores were 57.76 ± 21.28 and 60.03 ± 23.75. Sex and unemployment influenced the QoL. Financial burden was negatively associated with total SF-36 scores. Compared to the control group, patients with generalized symptoms (MGFA II and III) had lower SF-36 scores, but the patients with pure ocular symptoms (MGFA I) had not significant difference, except GH. The SF-36 scores were highly correlated with the severity of the disease, the states of mood, fatigue, and self-efficacy. CONCLUSIONS: The decrease in the HRQoL of patients with MG was related not only to the gender, severity of disease, and unemployment but also to the subjective experience including depressive and anxiety disorders, fatigue, and self-efficacy. In the course of treatment, the evaluation of HRQoL should be included in the routine assessment of patients with MG. Psychosocial treatment, social support, and health education should be advocated.

Impact of neurological diseases on family planning: A single-center experience.

This cross-sectional study aimed to assess the impact of epilepsy, myasthenia gravis (MG), and multiple sclerosis (MS) on pregnancy and family planning decision-making in a cohort of Saudi women. Women with epilepsy, MG, and MS were recruited consecutively at the time of their follow-up visits at a neurology clinic. Data were collected using 3 standardized questionnaires, and presented using descriptive statistics. A logistic regression was performed to determine variables associated with decisions regarding abstaining from pregnancy and encouraging other women to conceive. A total of 272 (83 epilepsy, 69 MG, and 120 MS) women with a mean age of 29.9 ±â€Š8.0 years participated. The proportion of women who abstained from or postponed pregnancy was 41.2% and 31.4%, respectively. The concerns mentioned most often were disease worsening during pregnancy, peripartum and postpartum, side effects of medications on the unborn child, and inability to care for the child. Older age was independently associated with the decision to abstain from pregnancy (odds ratio [OR] 1.14, 95% confidence interval [CI] 1.04 - 1.25). Higher knowledge levels were independently associated with encouraging other women to have children (OR 1.3, 95% CI 1.11-1.53). Over 50% of women reported that they were not counseled on issues related to pregnancy and childbirth. In conclusion, we identified a major influence of epilepsy, MG, and MS on pregnancy and family planning. Comprehensive counseling programs are needed to help women with these neurological diseases make informed family-planning decisions.

Gender differences in quality of life among patients with myasthenia gravis in China.

BACKGROUND: Myasthenia gravis (MG), a chronic neuromuscular disorder, can adversely affect patients' health-related quality of life (HRQoL), especially in women. The study aimed to evaluate the difference in HRQoL of women and men MG patients and explore the factors that mediate the relationship between gender and HRQoL. METHODS: A cross-sectional study was conducted among 1815 patients with MG in China. The revised 15-item MG quality of life scale (MG-QOL15r) was used to access patients' HRQoL in overall, physical, social and emotional domains. Socio-demographic information, diagnosis and treatment history, comorbidities, social support, active lifestyle and the MG activities of daily living scale (MG-ADL) were recorded and compared between women and men using the Student's t-test and Pearson's Chi-square test. Multivariable regression analyses were conducted to identify independent contributors to HRQoL, especially those affecting different gender. RESULTS: On average, female patients with MG reported a lower MG-QOL15r score than the males (44.49 ± 29.10 vs 49.32 ± 29.18). The association between gender and patients' HRQoL interacted with the number of comorbidities across the overall, physical and social domains of patients. As the number of comorbidities increased, the scores of HRQoL decreased and it was faster among females than the males (p < 0.05). Moreover, unemployment, exacerbation of the disease, and active lifestyle contributed to the patients' HRQoL across all domains. Unemployment (ß = - 4.99 [95%CI, - 7.80 to - 2.18], p < 0.001) and exacerbations (ß = - 8.49 [95%CI, - 11.43 to - 5.54], p < 0.001) were correlated with poorer HRQoL; while an active lifestyle had a positive impact on HRQoL (ß = 0.28 [95%CI, 0.16 to 0.40], p < 0.001). CONCLUSIONS: The results indicate that the HRQoL of women MG patients was lower than that of men. The relationship between gender and HRQoL is modulated by the number of comorbidities. Thus, to improve the HRQoL of women MG patients, symptomatic treatments might not be enough, their comorbid conditions should be considered as well. Additionally, employment status, MG exacerbations, and an active lifestyle have been found as determining factors of the patients' HRQoL, which suggests future interventions should cope with these factors to improve their quality of life.

Prospective study of stress, depression and personality in myasthenia gravis relapses.

BACKGROUND: Psychopathology and personality traits may influence the course of autoimmune disorders. With this prospective longitudinal cohort study, we aimed to assess personality, stress and depression in myasthenia patients who relapse and those who remain stable or improve (non-relapsers). METHOD: We collected data from 155 consecutive adult patients with confirmed MG attending the Neuromuscular Clinic, Toronto General Hospital, between March 2017 and July 2018, for this study. Patients were assessed at baseline and 6 months, or at the time of MG relapse. At both visits, the patients were assessed clinically and were asked to complete self-administered questionnaires for disease severity, chronic stress and depression. Personality type was assessed at baseline only. Relapsing patients were defined as those patients with MGII score increasing by more than 5.5 points from visit 1 to visit 2. RESULTS: Relapsers had higher baseline scores for depression (p = 0.01) and the change in disease severity correlated with the change in depression score (r = 0.2534, p = 0.0015, 95% CI: 0.098 0.3961). Higher levels of stress at baseline and neuroticism predicted higher relapse rates (p = 0.01 and p < .0001, respectively). In the linear regression model, with change of the MGII score as the dependent variable, change in depression scores (p = 0.0004) and age (p = 0.03) predicted change in disease severity. CONCLUSIONS: Since emotional factors and personality type may influence MG, attention to these factors might improve care in MG patients.

Myasthenia gravis patient and physician opinions about immunosuppressant reduction.

INTRODUCTION: To reduce myasthenia gravis (MG) patient risk of immunosuppressant (IS) exposure adverse events (AEs), such as infections and malignancies, and to reduce treatment burden, international guidelines recommend decreasing IS dose in stable MG patients. METHODS: Online surveys were conducted of self-identified MG patients and MG physician experts about the importance of IS dose reduction for MG patients who achieve prolonged periods of disease stability. RESULTS: Eighty-four percent of MG patients (n = 283) and 100% of physicians (n = 45) were concerned about long-term IS-associated AEs. Although both groups favored attempting IS reduction, they raised concerns including MG relapse, hospitalization, and uncertainty about the future. Presented with an estimated 12% significant relapse rate with IS dose reduction, 76% of patients would be willing to enroll in a randomized IS dose reduction trial. DISCUSSION: Patients and physicians favor considering IS dose reduction but are also concerned about potential negative sequelae.

Chronic stress, depression and personality type in patients with myasthenia gravis.

BACKGROUND AND PURPOSE: Stress is a known risk factor for the onset and modulation of disease activity in autoimmune disorders. The aim of this cross-sectional study was to determine any associations between myasthenia gravis (MG) severity and chronic stress, depression and personality type. METHODS: In all, 179 consecutive adult patients with confirmed MG attending the Neuromuscular Clinic between March 2017 and December 2017 were included. At baseline, patients were assessed clinically and they completed self-administered scales for disease severity, perceived stress, depression and personality type. RESULTS: Higher disease severity [Myasthenia Gravis Impairment Index (MGII)] showed a moderate correlation with depression score (Beck's Depression Inventory, Second Edition, r = 0.52, P < 0.001) and a lower correlation with chronic stress (Trier Inventory for Assessment of Chronic Stress, r = 0.28, P = 0.001). Chronic stress scores were different according to personality types (anova, P = 0.02). The linear regression model with MGII score as the dependent variable showed R2 = 0.34, likelihood ratio chi-squared 74.55, with P < 0.0001. The only variables that predicted disease severity were depression scores (P < 0.0001) and female sex (P = 0.003). CONCLUSIONS: A significant association of MG severity with depression and chronic stress was found, as well as with female gender. These findings should raise awareness that the long-term management of MG should address depression and potential stress and consider behavioural management to prevent stress-related immune imbalance.

Severity and depression can impact quality of life in patients with myasthenia gravis.

BACKGROUND: The revised 15-item Myasthenia Gravis (MG) Quality of Life Questionnaire (MGQoL15R) is a validated scale of quality of life in patients with MG. We aimed to study the factors causing the variability within the Arabic version of the MGQoL15R (MGQoL15R-A). METHOD: A standardized questionnaire was completed by 118 patients. Correlations and hierarchical regression analyses were used to assess the contribution of sociodemographic variables, clinical factors, Patient Health Questionnaire-9 (PHQ9-A), and Generalized Anxiety Disorder-7 (GAD7-A) to the variability in the MGQoL15R-A. RESULTS: The MGQoL15R-A was highly correlated with PHQ9-A (r = 0.76), and moderately correlated with GAD7-A (r = 0.52). Clinical factors and PHQ9-A independently explained 30.4% and 34.5% of the variability, respectively. Among the clinical factors, uncontrolled MG status, relapse within the past year, and a higher number of current MG therapies were significantly associated with a higher MGQoL15R-A score. CONCLUSIONS: MG severity and depressive symptoms (measured by PHQ9-A) can affect the MGQoL15R-A score.

Prevalence and Associated Factors of Depressive Symptoms in Patients with Myasthenia Gravis: A Cross-Sectional Study of Two Tertiary Hospitals in Riyadh, Saudi Arabia.

OBJECTIVES: This study is aimed at elucidating the prevalence of depression in patients with myasthenia gravis (MG) and examining the risk factors associated with depression. METHODS: We evaluated adult patients with MG who were recruited from two tertiary hospitals in the central region (Riyadh) of Saudi Arabia. Data were collected with a two-part standardized questionnaire: the first part included data on sociodemographic and clinical features of MG including disease type and duration, therapies, prednisolone dose, time of the last relapse, previous critical care unit admissions, MG status (controlled, partially controlled, or uncontrolled), and comorbid diseases; the second part included items from the previously validated Arabic version of the Patient Health Questionnaire-9 (PHQ-9). RESULTS: In total, 104/150 (69.3%) patients participated (72 females) with a mean age of 38.0 ± 16.0 years. The mean PHQ-9 score was 7.02 ± 6.1. Among all the participants, 27 (26.0%) patients had depression (PHQ-9 ≥ 10). Multiple logistic regression analysis revealed that uncontrolled MG status (OR = 12.31, 95%CI = 1.13-133.8, P = 0.04) was the only factor independently associated with depression. Collectively, the prevalence of depression among patients of the primary care clinics (PCC) as reported by 5 previous studies across multiple regions of the country was 15.8%. The odds of depression among MG patients were twofold higher than those among PCC patients (OR = 2.05, 95%CI = 1.30-3.22, P = 0.002). CONCLUSIONS: Approximately a quarter of MG patients have depression. Achieving a minimal manifestation or better MG status may decrease the depression rate in these patients.

Factores predictores y calidad de vida relacionada con la salud en pacientes con Miastenia Gravis. Revisión sistemática / Predictive factors and health-related quality of life in patients with Myasthenia Gravis. A systematic review

Introducción. La Miastenia Gravis es un trastorno autoinmune que afecta a los componentes de la unión neuromuscular y se caracteriza por grados fluctuantes de debilidad y fatigabilidad de los músculos, influyendo negativamente en la calidad de vida relacionada con la salud. Objetivo. Nuestro objetivo fue describir los factores que afectan a la calidad de vida relacionada con la salud en pacientes diagnosticados de Miastenia Gravis. Metodología. Este estudio es una revisión sistemática elaborada según indicaciones del Manual de Cochrane e informe PRISMA. Se realizó una búsqueda Pubmed, CINAHL, IBECS, IME, SCOPUS y Dialnet, incluyéndo-se artículos publicados entre 2001 y 2018 utilizando las palabras clave «miastenia gravis», «calidad de vida» y «factores predictores». Se aplicaron criterios de selección y exclusión, así como la valoración de calidad metodológica mediante el instrumento Effective Public Health Practice Project. Resultados. Se seleccionaron un total de 27 artículos que hacen referencia a los factores que afectan a la calidad de vida relacionada con la salud en pacientes con Miastenia Gravis. Conclusiones. Los factores fuertes y predictores encontrados que influyen en una peor calidad de vida relacionada con la salud en los pacientes con Miastenia Gravis son: sexo, edad, duración de la patología, presencia del anticuerpo MuSK, severidad de los síntomas, presencia de debilidad muscular y fatiga, presencia de movilidad limitada tras el ejercicio físico, enfermedades comórbidas, trastornos del sueño, situación laboral, nivel educativo, estado civil, apoyo social percibido, ingresos económicos, efectos secundarios de la medicación y salud mental

Introduction. Myasthenia Gravis is an autoimmune disorder that affects the components of the neuromuscular junction and is characterized by fluctuating degrees of weakness and fatigability of the muscles, negatively influencing the health-related quality of life. Objective. We aimed to describe the factors that affect the health-related quality of life in patients diagnosed with Myasthenia Gravis. Methodology. This study is a systematic review prepared according to the Cochrane Handbook and the PRIS-MA report. A search of the Pubmed, CINAHL, IBECS, IME, SCOPUS and Dialnet was conducted for the period from 2001 and 2018 using keywords «myasthenia gravis», «quality of life» and «factors predictors». Selection and exclusion criteria were applied, as well as methodological quality assessment through the Effective Public Health Practice Project instrument. Results. A total of 27 articles were selected, and they refer to the factors that affect the health-related quality of life in patients with Myasthenia Gravis. Conclusions. The strong factors and predictors found that influence a wor-se health-related quality of life with Myasthenia gravis are: sex, age, duration of the disease, presence of the MuSK antibody, severity of the symptoms, presence of muscle weakness and fatigue, presence of limited mobility after physical exercise, comorbid diseases, sleep disorders, employment situation, educational level, ma-rital status, perceived social support, income, side effects of medication and mental health