Myasthenia Gravis Exacerbation Research Articles

Efficacy and safety of maintenance intravenous immunoglobulin in generalized myasthenia gravis patients with acetylcholine receptor antibodies: A multicenter, double-blind, placebo-controlled trial.

Prospective, randomized, controlled trials of intravenous immunoglobulin (IVIG) maintenance therapy in myasthenia gravis (MG) are lacking. In this trial, we evaluated the safety and efficacy of caprylate/chromatography-purified IVIG; (IGIV-C) in patients with generalized MG undergoing standard care. Sixty-two patients enrolled in this phase 2, multicenter, international, randomized trial (1:1 IGIV-C [2 g/kg loading dose; 1 g/kg every 3 weeks through week 21] or placebo). Efficacy was assessed by changes in Quantitative MG (QMG) score at week 24 versus baseline (primary endpoint) and percentage of patients with clinical improvement in QMG, MG Composite (MGC), and MG-Activities of Daily Living (MG-ADL) scores (secondary endpoints). Safety assessments reported all adverse events (AEs). The change in QMG at 24 weeks was -5.1 for IGIV-C and -3.1 for placebo (p = .187). Seventy percent of patients in the IGIV-C group had improvement in MG-ADL (≥2-point decrease) versus 40.6% in the placebo group (p = .025). Patients showing clinical improvement in QMG and MGC (≥3-point decrease) were 70.0% for IGIV-C versus 59.4% for placebo (p = .442) and 60.0% for IGIV-C versus 53.1% for placebo (p = .610). IGIV-C was well tolerated; serious AEs were similar between arms. Three of four MG exacerbations requiring hospitalizations occurred in the IGIV-C arm with one death. Several efficacy parameters showed numerical results greater than those seen in the placebo group. This was a small study and may have been underpowered to see significant differences. Additional studies may be warranted to fully determine the efficacy of IVIG maintenance therapy in MG.

US Clinical Practice Experience with Eculizumab in Myasthenia Gravis: Acute Clinical Events and Healthcare Resource Utilization.

The terminal complement inhibitor eculizumab is approved in the USA for the treatment of patients with acetylcholine receptor antibody-positive generalized myasthenia gravis (MG). The ELEVATE study aimed to examine clinical-practice outcome data on eculizumab effectiveness in US adults with MG (generalized or ocular). This paper reports the findings on MG exacerbations and crises and associated healthcare resource utilization, and the use of rescue therapy. A retrospective chart review was conducted of US adults with MG who initiated eculizumab. Outcomes assessed for up to 2 years before and after eculizumab initiation included percentages and rates per patient per year (PPPY) of exacerbations and crises (the latter defined as intubation/impending intubation), healthcare resource utilization, and rescue therapy administration. A total of 119 patients diagnosed with MG were enrolled in the study; 92 patients had ≥3 months of data both before and during eculizumab therapy and were included in the analyses. The mean rate of MG exacerbations decreased from 0.385 PPPY before eculizumab initiation to 0.152 PPPY during eculizumab treatment (p = 0.0034); the mean rate of MG crises decreased from 0.411 to 0.056 PPPY (p = 0.0018). Rates of healthcare resource utilization and rescue therapy use also decreased significantly during eculizumab treatment. This retrospective chart review analysis provides evidence for a beneficial impact of eculizumab treatment on the incidence of MG exacerbations and crises and associated healthcare resource utilization in clinical practice, and on rescue therapy use. These data further support the therapeutic benefits of eculizumab in patients with MG.

The Impact of COVID-19 Vaccination and Infection on the Exacerbation of Myasthenia Gravis

Objectives: Myasthenia Gravis (MG) is an autoimmune disorder that can exacerbate for various reasons, including vaccination and infection. This study aimed to investigate the safety of COVID-19 vaccines for MG patients, factors influencing MG exacerbation after COVID-19 infection (MECI), the course and prognosis of MECI, and the impact of COVID-19 vaccine on infected MG patients. Methods: Patients were enrolled from the MG database in the Department of Neurology, Xuanwu Hospital, Capital Medical University. Two questionnaires were administered to collect data concerning COVID-19 vaccination (questionnaire 1, Q1) and infection (questionnaire 2, Q2) during two distinct periods. MG exacerbation was defined as an increase of at least two points in the MG activity of daily living (MG-ADL) score. COVID-19 severity was categorized as “hospitalization” or “home management”; Results: During the first data-collecting period, our database registered 1013 adult patients: 273 (26.9%) had received COVID-19 vaccinations and completed Q1, and 8 (2.9%) experienced MG exacerbation after vaccination. During the second data-collecting period, among the newly registered patients, 366 patients completed Q2. Of these, 244 were infected, with 39 (16.0%) experiencing MECI and 21 (8.6%) requiring hospitalization. Multivariate analysis showed that generalized myasthenia gravis was associated with MECI (OR 3.354, 95% CI: 1.423–7.908, p = 0.006). Among the 244 infected patients, 143 had received COVID-19 vaccinations, including 14 who received their booster dose within 6 months before COVID-19 and 129 who were vaccinated more than 6 months before COVID-19. The remaining 101 were unvaccinated. No significant associations were found between COVID-19 vaccination and COVID-19 severity (p = 0.292) or MECI incidence (p = 0.478); Conclusions: COVID-19 vaccines were found to be safe for MG patients in stable condition. Patients with gMG were more susceptible to experiencing MECI. No significant impact of the vaccine on COVID-19 severity or MECI incidence was observed.

Intensive care due to myasthenia gravis: Risk factors and prognosis.

Exacerbation of myasthenia gravis (MG) with respiratory failure requires intensive care. We aimed to study the risk factors for intensive care unit admission for MG exacerbation and myasthenic crisis (MC) and the prognosis of people with MG (pwMG) thereafter. This retrospective study investigated patients in the Helsinki and Uusimaa hospital district during the years 2008-2021. PwMG (International Classification of Diseases, 10th revision code G70.0) were identified through a data repository search, followed by a chart review of patient records. Risk factors for intensive care due to MG exacerbation were evaluated as compared with the patients only treated in the outpatient clinic and those treated in the neurological ward for MG exacerbation. The outcomes of patients in intensive care for any reason were also compared with those of patients in intensive care for exacerbation of bronchial asthma. Of 577 pwMG, 35 (6.1%) needed intensive care for MG within a median of 5.3 months from diagnosis. The mean (±SD) age at MG diagnosis was higher in the intensive care group (60.5 [±16.1] years) compared to the outpatient (48.3 [±20.9] years; p < 0.001) and neurological ward groups (53.4 [±20.8] years; p = 0.044). Thymoma (odds ratio [OR] 4.8, 95% confidence interval [CI] 1.19-19.43; p = 0.028) and female sex (OR 2.1, 95% CI 1.02-4.48; p = 0.045) were independent risk factors for intensive care. In-hospital mortality was 4% for MC patients. Six-month mortality after intensive care for MG exacerbation (14.3%) was twice that for asthma exacerbation (7.7%). Our study shows an increased risk of intensive care treatment for patients with late-onset MG, female sex or thymoma, occurring usually within 6 months from diagnosis, which emphasises the importance of early treatment choices.

The Risk of Exacerbation of Myasthenia Gravis After COVID-19 Omicron Infection.

The aim of this study is to ascertain whether COVID-19 Omicron infection is associated with exacerbations in these myasthenia gravis (MG) patients. In total, 289 MG patients (comprising 60% females, with an average age of 46 ± 15 years) were enrolled. A total of 80.9% of MG patients reported a COVID-19 infection, with the majority experiencing a benign course (88%). MG patients who experienced COVID-19 infection demonstrated a higher likelihood of MG exacerbation, compared to those without the infection (18.8%vs. 7.3%, p = 0.039). In the survival analysis, after adjusting for confounding factors, the hazard ratio (HR) for exacerbation post-infection was found to be 3.38 (95% CI 1.20-9.53, p = 0.021). Compared to the exacerbation rates observed in JTA21, an increase was noted in DTM23 among COVID-19-infected MG patients (4.4%vs. 17.2%, p < 0.001). The COVID-19 is the risk of MG exacerbation.

Pitfalls in the Evaluation of Respiratory Failure in Myasthenia Gravis Patients: A Case Series

Objectives To highlight the importance of recognizing different presentations of respiratory failure due to myasthenic and non-myasthenic etiologies in myasthenia gravis (MG) patients. Methods We describe 3 patients with different presentations of respiratory failure in MG. Cases Patient 1 is a 49-year-old female with longstanding MG who presented with lethargy and neck weakness without notable respiratory distress. She was found to be in hypercarbic respiratory failure, which improved with plasmapheresis treatment. Patient 2 is a 58-year-old female who presented with ptosis, dysphagia, and dyspnea requiring intubation. Her hypophonia and dyspnea persisted despite escalation in MG treatment, and further workup revealed glottal stenosis secondary to granulomatosis with polyangiitis. Patient 3 is an 85-year-old female with MG presenting with refractory hypoxia, which was secondary to a large patent foramen ovale resulting in right-to-left shunting. Discussion All 3 cases emphasize the role of clinical reasoning and careful analysis based on thorough history taking, detailed neurologic exam and comprehensive laboratory findings to determine the etiologies for respiratory dysfunction in MG and provide appropriate treatment. Conclusion A lack of overt signs of respiratory distress in MG does not rule out the presence of respiratory failure due to the sedating effect of hypercapnia. There is a need to consider alternative etiologies of hypoxia in MG patients if typical symptoms or signs of MG exacerbations are absent.

Clinical features of COVID-19 infection in patients with myasthenia gravis: a real-world retrospective study.

We investigated the risk factors associated with severe or critical Coronavirus disease 2019 (COVID-19) infection due to the Omicron variant in patients with myasthenia gravis (MG) and determined the potential effect of COVID-19 on myasthenic exacerbation during the Omicron pandemic. This retrospective study included 287 patients with MG in Tianjin, China. Clinical data of the patients were collected using electronic questionnaires, databases, and clinical records. The overall infection rate was 84.7%. Advanced age, comorbidities, generalized phenotype, and MG instability were drivers of COVID-19 severity, and post-COVID-19 myasthenic exacerbation. The concurrent use of a steroid-sparing agent did not affect COVID-19 susceptibility or severity. It did lower the risk of myasthenic exacerbation after COVID-19 infection. Patients with severe COVID-19 experienced myasthenic exacerbation earlier than patients with non-severe infection (p < 0.001). The severity of COVID-19 (Hazards Ratio = 3.04, 95% CI: 1.41-6.54, p = 0.004) and the clinical phenotype (Hazards Ratio = 3.29, 95% CI: 1.63-6.63, p < 0.001) emerged as independent risk factors for early MG exacerbation. Generally, patients with MG appear to be susceptible to the Omicron strains. Immunotherapy for MG did not increase COVID-19 susceptibility or severity. We do not advocate an immediate cessation of ongoing immunosuppressive treatments once a COVID-19 infection is diagnosed. Instead, a judicious evaluation of the risks and benefits, tailored to each individual, is recommended.

Frequency and Severity of Myasthenia Gravis Exacerbations Associated with the Use of Ciprofloxacin, Levofloxacin, and Azithromycin (S15.005)

Frequency and Severity of Myasthenia Gravis Exacerbations Associated with the Use of Ciprofloxacin, Levofloxacin, and Azithromycin (S15.005)

Myasthenia Gravis and COVID-19: A Systematic Review and Meta-analysis.

Patients with myasthenia gravis (MG), an autoimmune disease affecting the neuromuscular junction, exhibits varying rates of COVID-19 infection across different studies. This systematic review and meta-analysis aim to estimate the pooled prevalence of COVID-19 infection in individuals with MG. We systematically searched PubMed, Scopus, EMBASE, Web of Science, Google Scholar, and gray literature, including references to the research published before October 2021. The total number of participants, the first author, the publication year, the country of origin, the number of MG patients, their symptoms, hospitalization rates, and deaths were all extracted as study data. Our literature search yielded 253 articles, of which 75 remained after removing duplicates. Finally, 18 articles were included in the meta-analysis. The pooled prevalence of COVID-19 infection in MG cases was found to be 2% (95% CI, 1%, 3%; I2=85%, P<0.001). Additionally, the pooled prevalence of hospitalization among those with COVID-19 infection was 43% (95% CI, 26%, 60%; I2=97.6%; P<0.001), and the pooled prevalence of MG exacerbation was 33% (95% CI, 20%, 46%; I2=92.6%; P<0.001). In summary, this systematic review and meta-analysis reveal that the pooled prevalence of COVID-19 infection in individuals with MG is 2%.

Identification of key genes as potential diagnostic and therapeutic targets for comorbidity of myasthenia gravis and COVID-19.

Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder. Coronavirus disease 2019 (COVID-19) has a significant impact on the health and quality of life of MG patients and may even trigger the onset of MG in some cases. With the worldwide development of the COVID-19 vaccination, several new-onset MG cases and exacerbations following the COVID-19 vaccines have been acknowledged. The potential link between myasthenia gravis (MG) and COVID-19 has prompted the need for further investigation into the underlying molecular mechanism. The differential expression analysis identified six differentially expressed genes (DEGs) shared by myasthenia gravis (MG) and COVID-19, namely SAMD9, PLEK, GZMB, JUNB, NR4A1, and NR1D1. The relationship between the six common genes and immune cells was investigated in the COVID-19 dataset. The predictive value of the shared genes was assessed and a nomogram was constructed using machine learning algorithms. The regulatory miRNAs, transcription factors and small molecular drugs were predicted, and the molecular docking was carried out by AutoDock. We have identified six common DEGs of MG and COVID-19 and explored their immunological effects and regulatory mechanisms. The result may provide new insights for further mechanism research.

Hospitalizations and Mortality From Myasthenia Gravis: Trends From 2 US National Datasets

Myasthenia gravis (MG) is a rare neuromuscular disorder where IgG antibodies damage the communication between nerves and muscles, leading to muscle weakness that can be severe and have a significant impact on patients' lives. MG exacerbations include myasthenic crisis with respiratory failure, the most serious manifestation of MG. Recent studies have found MG prevalence increasing, especially in older patients. This study examined trends in hospital admissions and in-hospital mortality for adult patients with MG and readmissions and postdischarge mortality in older (65 years or older) adults with MG. Data from the Nationwide Inpatient Sample (NIS), an all-payer national database of hospital discharges, were used to characterize trends in hospitalizations and in-hospital mortality related to MG exacerbations and MG crisis among adult patients aged 18 years or older. The Medicare Limited Data Set, a deidentified, longitudinal research database with demographic, enrollment, and claims data was used to assess hospitalizations, length of stay (LOS), readmissions, and 30-day postdischarge mortality among fee-for-service Medicare beneficiaries aged 65 years or older. The study period was 2010-2019. Multinomial logit models and Poisson regression were used to test for significance of trends. Hospitalization rates for 19,715 unique adult patients and 56,822 admissions increased from 2010 to 2019 at an average annualized rate of 4.9% (MG noncrisis: 4.4%; MG crisis: 6.8%; all p < 0.001). Readmission rates were approximately 20% in each study year for both crisis and noncrisis hospitalizations; the in-hospital mortality rate averaged 1.8%. Among patients aged 65 years or older, annualized increases in hospitalizations were estimated at 5.2%, 4.2%, and 7.7% for all, noncrisis, and crisis hospitalizations, respectively (all p < 0.001). The average LOS was stable over the study period, ranging from 11.3 to 13.1 days, but was consistently longer for MG crisis admissions. Mortality among patients aged 65 years or older was higher compared with that in all patients, averaging 5.0% across each of the study years. Increasing hospitalization rates suggest a growing burden associated with MG, especially among older adults. While readmission and mortality rates have remained stable, the increasing hospitalization rates indicate that the raw numbers of readmissions-and deaths-are also increasing. Mortality rates are considerably higher in older patients hospitalized with MG.

Impact of COVID-19 pandemic on the incidence and severity of myasthenia gravis in Korea: using National Health Insurance Service database.

This study investigated the impact of COVID-19 pandemic on the incidence and severity of myasthenia gravis (MG) using the National Health Insurance Service (NHIS) database in Korea. We analyzed data from patients with MG in the NHIS registry from 2015 to 2021. MG was defined as (1) patients aged ≥18 years with the G70.0 code, and (2) patients who visited tertiary hospitals regarldless of department in Korea (outpatient clinics at least twice or hospitalization at least once), and (3) patients who were prescribed pyridostigmine as MG medications at least once. We designated pre-COVID-19 as 2019 and post-COVID-19 as 2021 and analyzed the MG incidence and prevalence in 2019 and 2021. We compared the clinical data of patients with MG between the two years. MG exacerbation was defined as the administration of intravenous immunoglobulin or plasma exchange. Analysis of COVID-19 cases was conducted using an integrated database from the Korea Disease Control and Prevention Agency and NHIS. Patients with MG were divided into two groups according to COVID-19 status to compare their clinical characteristics. A total of 6,888 and 7,439 MG cases were identified in 2019 and 2021, respectively. The standardized incidence was 1.56/100,000 in 2019, decreasing to 1.21/100,000 in 2021. Although the frequency of MG exacerbations was higher in 2019, there were no differences in the number and duration of hospitalizations, duration of ICU stays, hostalization expense, and mortality between 2019 and 2021. Patients with MG and COVID-19 had a higher frequency of MG exacerbations than patients without COVID-19, but there were no differences in the number and duration of hospitalizations, hospitalization expense, and mortality. This study was the first nationwide population-based epidemiological study of MG during COVID-19 pandemic in Korea. The incidence of MG decreased during COVID-19 pandemic, and the severity of MG was not affected by COVID-19.

Efgartigimod Successfully Ameliorated Acute Exacerbation of Myasthenia Gravis with Anti-muscle-specific Kinase Antibodies.

We herein report two patients with anti-muscle-specific kinase (MuSK) antibody-positive myasthenia gravis who experienced rapid deterioration of weakness, particularly respiratory muscle weakness, necessitating non-invasive positive pressure ventilation (NIPPV) and were treated with efgartigimod. After treatment initiation, a rapid reduction in IgG levels and recovery from clinical symptoms were observed. NIPPV was no longer required two to three weeks after the first infusion of efgartigimod. These findings suggest that the reduction of IgG levels using efgartigimod is a good treatment option in patients with myasthenia gravis positive for anti-MuSK antibodies, even during the acute phase of the disease.

Pregnancy planning may impact maternal and neonatal outcomes in people with myasthenia gravis.

Myasthenia Gravis (MG) is an acquired autoimmune condition commonly diagnosed in young people of reproductive age resulting in neuromuscular junction dysfunction. The course of MG during pregnancy and its impact on maternal and neonatal outcomes is vary in the literature. Pregnancy planning is a known strategy and modifiable risk factor in obstetric practice to decrease maternal and neonatal morbidity. We aim to assess if planning a pregnancy impacts maternal and neonatal outcomes, MG exacerbation, and pregnancy-related complications. This study utilized data from an online, North American survey entitled "A Patient Centered study on Pregnancy in People with Myasthenia Gravis", distributed with the assistance of MG advocacy groups in the United States and Canada. It included individuals with MG who had at least one pregnancy in the last 10-years. Key maternal and neonatal outcomes were compared between planned and unplanned pregnancies. Out of 156 survey participants, 58 had a pregnancy following MG diagnosis, totaling 90 reported pregnancies. Of these, 56 (62.2%) were planned and 34 (37.8%) were unplanned pregnancies. The unplanned pregnancies were associated with more MG exacerbations, hospitalizations, and intensive care unit admission (37.7% vs. 13.7%, 26.5% vs. 11%, and 17.6% vs. 8.9%, respectively, p ≤ .05). The neonatal outcomes did not significantly differ between the groups. Planned pregnancies in people with MG may be associated with a reduced gestational and post-partum risk of MG exacerbation, hospitalizations, and ICU admissions. Larger studies are required to confirm this association and account for potential contributing variables.

Healthcare resource utilization, costs and treatment associated with myasthenia gravis exacerbations among patients with myasthenia gravis in the USA: a retrospective analysis of claims data.

Aim: There are limited data on the clinical and economic burden of exacerbations in patients with myasthenia gravis (MG). We assessed patient clinical characteristics, treatmentsand healthcare resource utilization (HCRU) associated with MG exacerbation. Patients & methods: This was a retrospective analysis of adult patients with MG identified by commercial, Medicareor Medicaid insurance claims from the IBM® MarketScan® database. Eligible patients had two or more MG diagnosis codes, without evidence of exacerbation or crisis in the baseline period (12months prior to index [first eligible MG diagnosis]). Clinical characteristics were evaluated at baseline and 12weeks before each exacerbation. Number of exacerbations, MG treatments and HCRU costs associated with exacerbation were described during a 2-year follow-up period. Results: Among 9352 prevalent MG patients, 34.4% (n=3218) experienced ≥1 exacerbation after index: commercial, 53.0% (n=1706); Medicare, 39.4% (n=1269); and Medicaid, 7.6% (n=243). During follow-up, the mean (standard deviation) number of exacerbations per commercial and Medicare patient was 3.7 (7.0) and 2.7 (4.1), respectively. At least two exacerbations were experienced by approximately half of commercial and Medicare patients with ≥1 exacerbation. Mean total MG-related healthcare costs per exacerbation ranged from $26,078 to $51,120, and from $19,903 to $49,967 for commercial and Medicare patients, respectively. AChEI use decreased in patients with multiple exacerbations, while intravenous immunoglobulin use increased with multiple exacerbations. Conclusion: Despite utilization of current treatments for MG, MG exacerbations are associated with a high clinical and economic burden in both commercial and Medicare patients. Additional treatment options and improved disease management may help to reduce exacerbations and disease burden.

COVID-19 and Myasthenia Gravis Exacerbation in an Elderly Patient: a Case Report

Treatment of COVID-19 in elderly patients with myasthenia gravis (MG), an autoimmune disease characterized by increased dynamic muscle weakness, is associated with a number of challenges, such as chronic immunosuppression, exacerbation of MG, and polymorbidity.We present the case of an elderly patient with generalized seropositive MG who contracted COVID-19. Aggravating factors are the patient’s age, the duration of myasthenia gravis, COVID-19, and comorbidity (chronic coronary heart disease, diffuse cardiosclerosis, atrial fibrillation, NYHA class I heart failure, hyper­tension). The patient had a favorable outcome despite the exacerbation. The patient required intravenous injections of immunoglobulin (1 mg/kg) for 2 days, non-invasive ventilatory support, and treatment of respira­tory and urogenital nosocomial infections (Pseudomonas aeruginosa and Klebsiella oxytoca).COVID-19 led to exacerbation of MG, progression of muscle weakness, development of respiratory failure, and hypoxia in an elderly patient. Intravenous immunoglobulin is a possible effective medication in the treatment of this comorbid pathology.During the COVID-19 pandemic patients with MG may be at greater risk of worse outcomes than healthy individuals due to an immunodeficient state associated with immunotherapy and possible respiratory and bulbar muscle weakness. The examination of patients with MG and a COVID-19 may reveal new pathogenetic patterns and change the therapeutic strategy.Knowledge of the essence of the disease, the main links of its pathogenesis, the spectrum of modern therapeutic agents, and algorithms for their use allow us to improve the quality of medical care for patients with MG. The implementation of a comprehensive multidisciplinary approach in the treatment of these patients allows us to significantly reduce the mortality rate, prolong the life of patients, and improve its quality.

Clinical features, treatments, their impact, and quality of life for Myasthenia Gravis patients in Australia

This survey provides an update on the experience of Myasthenia Gravis (MG) patients in Australia. Items were drawn from the 2011 Australian Survey and a 2019 US survey allowing for comparative discussion of survey findings. Patients were recruited through the Myasthenia Alliance Australia. Following consent, patients completed an online survey using REDCap software. Questions included demographics, clinical features, treatment side-effects and quality of life (QOL) scales. Samples for completion of survey sections ranged from N = 242–280 representing a power level of over 80%. Female and seronegative patients reported a significantly greater symptom load, earlier disease onset, longer time to diagnosis, more MG exacerbations, treatment side-effects, and poorer QOL. For exacerbation management there was a higher rate of oral corticosteroid use (66%), a lower use of Intravenous Immunoglobulin (IVIg, 47%) and particularly, Therapeutic Plasma Exchange (TPE, 4.5%) within this sample. Although steroid induced side-effects were rarer (9–34%), a comparatively high use of corticosteroids was reported for current and overall treatments including those for MG crises (52–83%). Common treatment side-effects reported by 57–85% of patients, included fatigue, weight gain, a decrease in the ability to fight infections, gastrointestinal symptoms, and muscle weakness. The impact of MG on daily activities and QOL was considerable, but those who had a thymectomy reported better QOL. The survey identified areas for potential practice improvement in MG treatments (corticosteroids, IVIg, TPE), particularly for exacerbation management, and review is recommended. Further research on gender and antibody status differentials regarding clinical features is required.

Evaluation of Medication Exposure on Exacerbation of Disease in Patients With Myasthenia Gravis.

In patients with myasthenia gravis (MG), worsening of symptoms poses a risk of respiratory failure which can be precipitated by medication use. Although purported, the risks associated with administration of certain medications are not fully elucidated. Thus, clinical decision support involving a best practice alert was executed to caution providers of drug-disease interactions when ordering a potentially harmful medication. We performed an analysis of the alert overrides with subsequent medication exposure to determine the incidence of MG exacerbations. This retrospective chart-review evaluated adult patients with MG at 2 large academic medical centers via electronic health records between November-2019 and November-2021 who received a medication following override of the clinical decision support tool. The primary outcome was proportion of patient encounters complicated by myasthenic exacerbations after potentially harmful medication administration. Secondary outcomes included changes in motor strength, length of stay, discharge disposition, unplanned level-of-care escalations, and changes to immunosuppressant therapy following medication administration. A total of 70 orders were assessed in 38 patients across 55 encounters. Medications administered during these encounters included macrolides, fluoroquinolones, β-blockers, calcium channel blockers, and magnesium sulfate. Exacerbation of disease occurred in 7 patient encounters (12.7%) and occurred after intravenous magnesium or intravenous labetalol. In 5/7 events, at least 1 other risk factor associated with a myasthenic exacerbation was present. Of the medications reported to potentially worsen MG, intravenous labetalol and intravenous magnesium were the 2 agents associated with myasthenic exacerbations with a higher incidence in patients harboring additional risk factors.

Clinical characteristics and outcomes of COVID-19 infection among myasthenia gravis patients in a national referral hospital in Indonesia

Introduction. Due to their immunocompromised state from immunosuppressive therapy and respiratory efficiency, patients with Myasthenia Gravis (MG) are more likely to get infected with COVID-19. Currently, reports on MG patients exposed with COVID-19 in Indonesia are still limited. Therefore, this study was conducted to determine the clinical characteristics and outcomes of MG patients infected with COVID-19, especially in Jakarta, Indonesia. Methods. It is a retrospective study of MG patients in a National Referral Hospital in Indonesia from September 2021 to December 2022. The data obtained includes demographics, medical history, clinical characteristics, and severity of MG assessed by MG composite scale (MGCS), Myasthenia Gravis Foundation of America (MGFA) classification, symptoms, and therapy. Results. Of the 8 patients, 7 patients were female. The range of age at COVID-19 onset was 28-62 years old. On admission to the hospital, 2 patients were mild MG, 2 patients were moderate MG, 3 patients with severe MG, and one patient could not be identified because the patient came in an unconscious state. Five patients were classified as moderate COVID-19, two patients had severe COVID-19, and one patient was classified as critical COVID-19. Most patients experience MG exacerbation or require oxygen support during hospitalization. Only one patient deceased while receiving treatment at the emergency room, while the rest improved and were discharged home. Conclusion. MG patients exposed with COVID-19 tend to experience moderate, severe, and critical COVID-19 symptoms. However, with proper management of the infection and other comorbidities, MG patients with COVID-19 may achieve favorable outcome.

Clinical Outcomes in COVID-19 patients with pre-existing myasthenia gravis:

INTRODUCTION: Myasthenia gravis (MG) presents an additional challenge in managing COVID-19 as outcomes potentially depend on prior disease control and treatment. Yet the role of pre-existing MG in COVID-19 outcomes has not been established.&#x0D; METHODS: We searched PubMed, Scopus, and Web of Science databases for reports of MG patients with confirmed COVID-19 until March 2022. We analyzed data on patient demographics, chronicity, and MG control at baseline pre-COVID, treatment history and outcome following COVID infection.&#x0D; RESULTS: Twenty-nine publications with 119 patients (females n=75, age range 20-93 years, AChR Ab positive n= 65, MuSK Ab positive n= 5, seronegative n=14, unknown n=35) were included. Eighty-three (70%) were hospitalized, more than half with MG exacerbation. There was no significant difference in disease duration or control of MG symptoms at baseline between hospitalized and non-hospitalized. Hospitalization was associated with higher dose of daily prednisone but a comparable proportion of patients were on steroid-sparing agents. Among hospitalized patients, 40% were intubated uncorrelated with MG baseline control. Unfavorable outcome was not always associated with MG exacerbation. Amongst those discharged,75% received intravenous immunoglobulin (IVIG) or Plasmapheresis (PLEX) for MG exacerbation as compared to 67% with a fatal outcome didn’t receive either.&#x0D; CONCLUSION: Preexisting MG does not appear to be associated with severe COVID-19 outcomes. Higher dose of prednisone prior to COVID-19 infection is associated with increased risk of hospitalization but MG control at baseline did not determine worse outcome. IVIG/PLEX appears safe and potentially can reduce fatality in patients with COVID-19 experiencing MG exacerbation.