Outcomes of COVID-19 Infection and Vaccination Among Individuals With Myasthenia Gravis.

Importance: There are limited data regarding COVID-19 outcomes and vaccine uptake and safety among people with myasthenia gravis (MG). Objective: To investigate COVID-19-related outcomes and vaccine uptake among a population-based sample of adults with MG. Design, Setting, and Participants: This population-based, matched cohort study in Ontario, Canada, used administrative health data from January 15, 2020, and August 31, 2021. Adults with MG were identified using a validated algorithm. Each patient was matched by age, sex, and geographic area of residence to 5 controls from the general population and from a cohort of individuals with rheumatoid arthritis (RA). Exposure: Patients with MG and matched controls. Main Outcomes and Measures: Main outcomes were COVID-19 infection and related hospitalizations, intensive care unit admissions, and 30-day mortality among patients with MG vs controls. Secondary outcomes were uptake of COVID-19 vaccination among patients with MG vs controls. Results: Among 11 365 233 eligible Ontario residents, 4411 patients with MG (mean [SD] age, 67.7 [15.6] years; 2274 women [51.6%]) were matched to 22 055 general population controls (mean [SD] age, 67.7 [15.6] years; 11 370 women [51.6%]) and 22 055 controls with RA (mean [SD] age, 67.7 [15.6] years; 11 370 women [51.6%]). In the matched cohort, 38 861 of 44 110 individuals (88.1%) were urban residents; in the MG cohort, 3901 (88.4%) were urban residents. Between January 15, 2020, and May 17, 2021, 164 patients with MG (3.7%), 669 general population controls (3.0%), and 668 controls with RA (3.0%) contracted COVID-19. Compared with general population controls and controls with RA, patients with MG had higher rates of COVID-19-associated emergency department visits (36.6% [60 of 164] vs 24.4% [163 of 669] vs 29.9% [200 of 668]), hospital admissions (30.5% [50 of 164] vs 15.1% [101 of 669] vs 20.7% [138 of 668]), and 30-day mortality (14.6% [24 of 164] vs 8.5% [57 of 669] vs 9.9% [66 of 668]). By August 2021, 3540 patients with MG (80.3%) vs 17 913 general population controls (81.2%) had received 2 COVID-19 vaccine doses, and 137 (3.1%) vs 628 (2.8%), respectively had received 1 dose. Of 3461 first vaccine doses for patients with MG, fewer than 6 individuals were hospitalized for MG worsening within 30 days of vaccination. Vaccinated patients with MG had a lower risk than unvaccinated patients with MG of contracting COVID-19 (hazard ratio, 0.43; 95% CI, 0.30-0.60). Conclusions and Relevance: This study suggests that adults with MG who contracted COVID-19 had a higher risk of hospitalization and death compared with matched controls. Vaccine uptake was high, with negligible risk of severe MG exacerbations after vaccination, as well as evidence of effectiveness. The findings support public health policies prioritizing people with MG for vaccination and new COVID-19 therapeutics.

Incidence, Prevalence, Hospitalization Rates, and Treatment Patterns in Myasthenia Gravis: A 10-Year Real-World Data Analysis of German Claims Data.

BACKGROUND: Myasthenia gravis (MG) is a rare chronic autoimmune disease caused by autoantibodies directed against postsynaptic antigens of the neuromuscular junction. Over the last decades, increasing incidence and prevalence rates have been reported. Epidemiological data on prevalence and incidence in Germany are lacking. Furthermore, the MG treatment landscape is rapidly changing due to the continued approval of novel monoclonal antibodies. METHOD: This is a retrospective study assessing incidence, prevalence, and hospitalization rates of MG as well as treatment patterns in Germany over 10 years based on medical claims data covering 6.1 million insured persons. RESULTS: Between 2011 and 2020, the prevalence rate of MG increased from 15.7 to 28.2 per 100,000 person-years. The age-adjusted incidence rate was 2.8 per 100,000 person-years within the study period (95% confidence interval, 2.43-3.22) and decreased dramatically in 2020, the year of the COVID-19 pandemic. Similarly, the hospitalization rate fluctuated within the study period but reached an overall low of 8.3% in 2020 (mean hospitalization rate 11.5%). Treatment patterns showed that most MG patients are treated with base therapy. However, crisis intervention is necessary for 2-5% of MG patients, and therapeutic monoclonal antibodies, including rituximab and eculizumab, are increasingly used. CONCLUSION: This is the first study on MG prevalence and incidence rates in Germany. Data show an increase in prevalence by 1.8-fold over 10 years. Decreasing incidence and hospitalization rates in 2020 hint at the impact of the COVID-19 pandemic. Treatment patterns in MG are changing with the advent of therapeutic monoclonal antibodies in this indication.

The impact of SARS-CoV-2 immunization on COVID-19 disease course in people with myasthenia gravis.

INTRODUCTION/AIMS: There is limited knowledge regarding the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines οn coronavirus disease 2019 (COVID-19) disease course in people with myasthenia gravis. In this study, we aimed to investigate whether SARS-CoV-2 vaccination influences hospitalization and mortality due to COVID-19 in this population. METHODS: This is a retrospective analysis of administrative data extracted from the Greek nationwide database that holds the COVID-19 disease and vaccination registry, as well as all medical prescription records. The study period extended from the onset of the pandemic (February 2020) until the 10th of January 2022. RESULTS: We identified 278 people with myasthenia gravis (mean age 58.1 ± 17.2, 47.5% males) who tested positive for SARS-CoV-2. Of those, 139 (50%) were not vaccinated at the time of infection. Multivariable binary logistic regression analysis showed that the probability of hospitalization increased with age (odds ratio [OR]: 1.058; 95% confidence interval [CI], 1.036-1.080; p < .001) and immunosuppressive treatment (OR: 2.872; 95% CI 1.412-5.839; p = .004), and decreased with vaccination (OR: 0.244; 95% CI 0.132-0.453; p < .001). The probability of a fatal outcome increased with age (OR: 1.085; 95% CI 1.043-1.129; p < .001) and decreased with vaccination (OR: 0.315; 95% CI 0.125-0.791; p = .014). DISCUSSION: SARS-CoV-2 vaccination significantly reduces hospitalization and mortality due to COVID-19 in people with myasthenia gravis. This study regarding the efficacy of these vaccines, together with previous studies regarding their safety, provide evidence to support their use in people with myasthenia gravis.

Clinical course and outcome of Covid-19 in patients with myasthenia gravis.

OBJECTIVES: To analyze the prognosis and outcomes of COVID-19 in patients with MG and to determine factors associated with COVID-19 severity in patients with MG. METHODS: Information concerning COVID-19 occurrence in patients with MG was collected in this single-center observational study. Univariate and multivariate analyses were used to identify factors associated with severe Covid-19. RESULTS: Two hundred seventy-five of the 386 records of MG were included in this study. Eighty-two (29.8%) patients had concurrent COVID-19 . The patients' mean age was 50.3 ± 1.6 years, and the mean duration of MG was 6.7 ± 5.4 years. MG was diagnosed after COVID-19 in five cases. Covid-19 was mild in 45 patients (54.9%), moderate in 23 (28.1%), and severe in 14 (17.07%), while mortality occurred in four of the severe cases (4.9%). Three of the exitus patients were receiving rituximab therapy. Pre-Covid MG Activity of Daily Living (MG-ADL) severity scores were significantly high in severe cases. A history of myasthenic crisis was also higher in severe cases. Similarly, univariate and multivariate analyses revealed an association between severe COVID-19 and myasthenic crisis history and high pre-Covid MG-ADL. The type of MG treatment had no independent effect on COVID-19 severity. CONCLUSION: The vast majority of the MG patients made a good recovery from Covid-19. The risk of severe COVID-19 is high in patients with high MG-ADL severity scores and a history of myasthenic crisis.

Retrospective study on the safety of COVID-19 vaccination in myasthenia gravis.

INTRODUCTION/AIM: Given the lack of information on safety of coronavirus disease 2019 (COVID-19) vaccination in myasthenia gravis (MG) patients, we aimed to review our experience after surveying patients, as part of routine clinical practice, to ensure that advice on safety is accurate. METHODS: We performed a retrospective chart review of MG patients from the Prosserman Family Neuromuscular Clinic at the Toronto General Hospital who received two injections of any COVID-19 vaccine from February to August 2021. Demographic data were abstracted from the patient medical records. We assessed changes in the severity of MG using the virtual Myasthenia Gravis Impairment Index (vMGII), the simple single question (SSQ), and Patient Acceptable Symptom State (PASS). We also assessed adverse effects after vaccination. RESULTS: We included 200 patients with a mean age of 64.3 ± 13.9 y, 51.5% were men, and 82% had generalized MG. The vMGII, SSQ, and PASS scores remained stable after each vaccine dose, and at last follow-up. Of the patients, 60% reported an adverse reaction after the first injection, and 56% after the second. The most common adverse reactions reported were local pain at the injection site, fatigue, headache, and fever. DISCUSSION: COVID-19 vaccinations were well tolerated in MG patients and were not associated with worsening severity of their MG. The prevalence of vaccine-related adverse reactions was the same as in the general population.

Clinical course and outcome of an outpatient clinic population with myasthenia gravis and COVID-19.

INTRODUCTION/AIMS: Coronavirus disease-2019 (COVID-19) may have a more severe course in patients with myasthenia gravis (MG). We aimed to assess severity of the infection and factors contributing to its severity in a group of MG patients, most of whom were not hospitalized. METHODS: One hundred forty outpatients with MG followed between March 2020 and April 2021 were included in our study. Patients were asked to respond to a brief questionnaire in person, by telemedicine, or through electronic messages. RESULTS: Nineteen patients tested positive for COVID-19 by polymerase chain reaction. Two were asymptomatic. Of the 17 symptomatic patients, 11 had mild symptoms. They either had no treatment or received antivirals, antibiotics, and anticoagulants. Their myasthenia was well-controlled before infection and was unaffected by COVID-19. Three patients with moderate COVID-19 required hospitalization, but not intensive care, and had full recovery. Three other patients, the oldest in the cohort, had severe disease: One patient with a postsurgery myasthenic exacerbation before the infection needed intensive care without intubation, but recovered completely; two morbidly obese patients with comorbidities required intubation and died. Corticosteroids were increased in four of the six moderate/severely affected patients. Immunosuppressive (IS) agents were generally continued. Hydroxychloroquine (HCQ) for COVID-19 was used in one patient. DISCUSSION: Most patients had mild COVID-19 and all but two patients recovered. The design of the study made it possible to capture mild cases. Having well-controlled MG before infection and absence of comorbidities likely affected the course of the infection favorably. IS did not influence the progression.

Predictive factors for a severe course of COVID-19 infection in myasthenia gravis patients with an overall impact on myasthenic outcome status and survival.

BACKGROUND AND PURPOSE: Myasthenia gravis (MG) patients could be a vulnerable group in the pandemic era of coronavirus 2019 (COVID-19) mainly due to respiratory muscle weakness, older age and long-term immunosuppressive treatment. We aimed to define factors predicting the severity of COVID-19 in MG patients and risk of MG exacerbation during COVID-19. METHODS: We evaluated clinical features and outcomes after COVID-19 in 93 MG patients. RESULTS: Thirty-five patients (38%) had severe pneumonia and we recorded 10 deaths (11%) due to COVID-19. Higher forced vital capacity (FVC) values tested before COVID-19 were shown to be protective against severe infection (95% CI 0.934-0.98) as well as good control of MG measured by the quantified myasthenia gravis score (95% CI 1.047-1.232). Long-term chronic corticosteroid treatment worsened the course of COVID-19 in MG patients (95% CI 1.784-111.43) and this impact was positively associated with dosage (p = 0.005). Treatment using azathioprine (95% CI 0.448-2.935), mycophenolate mofetil (95% CI 0.91-12.515) and ciclosporin (95% CI 0.029-2.212) did not influence the course of COVID-19. MG patients treated with rituximab had a high risk of death caused by COVID-19 (95% CI 3.216-383.971). Exacerbation of MG during infection was relatively rare (15%) and was not caused by remdesivir, convalescent plasma or favipiravir (95% CI 0.885-10.87). CONCLUSIONS: As the most important predictors of severe COVID-19 in MG patients we identified unsatisfied condition of MG with lower FVC, previous long-term corticosteroid treatment especially in higher doses, older age, the presence of cancer, and recent rituximab treatment.

Predictors of outcome in patients with myasthenic crisis undergoing non-invasive mechanical ventilation: A retrospective 20 year longitudinal cohort study from a single Italian center.

About 20% of patients with myasthenia gravis (MG) may develop myasthenic crisis (MC) requiring ventilation, either invasive (MV) or non-invasive (NIV) and intensive unit care (ICU). NIV failure in patients with MC can occur up to 60% of cases admitted to ICU. Moreover it is not known the outcome of MC receiving NIV. Purpose of this study was to identify predictors of outcome in MC who underwent non-invasive ventilator support outside ICU setting. We enrolled 90 patients, 53 females and 37 males admitted to University Hospital of Modena (Italy) between January 2000 and September 2020. Median age at MC was 65 years. Thirty-four patients (37.8%) required MV. Thymectomy was performed in 45 cases, associated with thymoma in 55%, with hyperplastic thymus in 33%. First-line treatment was plasmaexchange (38.8%) or intravenous immunoglobulins (45.6%). Males exhibited higher risk of MV than females .Patients in MV were treated with plasmaexchange as first-line therapy . Our in-hospital mortality rate was low. Nine patients underwent tracheostomy which was significantly related to male gender. Comorbidities had significant effect on length of ICU .Our study confirms as predictors of prognosis in our patients male gender, older age at onset, infections as trigger, pneumonia.

Myasthenia gravis and covid-19 in pregnancy: a review of the literature and case series report.

OBJECTIVES: The aim of our study was to investigate the interrelations of symptoms, clinical outcomes and treatment regimens in pregnant women, diagnosed with myasthenia gravis and superimposed COVID-19 infection. METHODS: We conducted an observational retrospective study between August, 2020 and July, 2021. Five patients with preexisting MG and superimposed COVID- infection were included in our study. We investigated the duration of MG, the antibody patient status, any present comorbidities, MG baseline treatment and MG severity class prior to the COVID-19 infection, MG severity class and treatment during the COVID-infection, and last but not least, the maternal and fetal clinical outcome. RESULTS: None of the participants were hospitalized as they were treated under quarantine at their homes. The most frequently reported complaints were anosmia, headache and fever, which were observed in 3 out of 5 patients. The MG severity was evaluated twice - before and after the quarantine period. Progression to a more advanced stage was found in 2 of our 5 patients. Three of the patients did not require any changes in the prescribed baseline MG treatment. In 2 patients the pyridostigmine dosage had to be increased. One patient received azithromycin and 4 patients were given LMWH (nadroparin) as specific anti-COVID measures. All patients fully recovered and gave birth to healthy newborns. CONCLUSION: To our knowledge, this is the first study on pregnant MG patients with superimposed COVID-19 infection. Based on our observations in this study it would seem that the coexistence of MG and COVID-19 infection in pregnancy does not elicit exacerbation in neither of those conditions. Further research is needed to confirm or challenge these findings, especially with the prospects of new virus variants emerging in the future.

Myasthenia gravis and telemedicine: a lesson from COVID-19 pandemic.

COVID-19 pandemic has induced an urgent reorganization of the healthcare system to ensure continuity of care for patients affected by chronic neurological diseases including myasthenia gravis (MG). Due to the fluctuating nature of the disease, early detection of disease worsening, adverse events, and possibly life-threatening complications is mandatory. This work analyzes the main unresolved issues in the management of the myasthenic patient, the possibilities offered so far by digital technologies, and proposes an online evaluation protocol based on 4 simple tests to improve MG management. Telemedicine and Digital Technology might help neurologists in the clinical decision-making process of MG management, avoiding unnecessary in presence consultations and allowing a rational use of the time and space reduced by the pandemic.

COVID-19 pandemic year in a sample of Polish myasthenia gravis patients: an observational study.

AIM OF THE STUDY: Coronavirus disease 2019 (COVID-19) incidence, mortality, recovery and hospitalisation rates vary in different countries. This study aimed to present the clinical characteristics of a sample of unvaccinated Polish myasthenia gravis (MG) patients during the first year of the COVID-19 pandemic, taking into account the number of MG exacerbations, a detailed description of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection course, and the need to modify immunosuppressive therapies. Clinical rationale for the study: To assess the impact of the first COVID-19 pandemic year on MG course in a sample of unvaccinated patients. MATERIALS AND METHODS: A retrospective observational study involving 30 unvaccinated Polish MG patients consulted in a neurological outpatient clinic on 11-31 March, 2020 (baseline) and 11-31 March, 2021 (endpoint). RESULTS: During the period of evaluation, exacerbation of MG requiring hospitalisation was reported in 11 patients. Among them, four were treated with intravenous immunoglobulin and another six required plasma exchange. In the study group, COVID-19 was identified in 10 patients. Of them, seven experienced a mild course of SARSCoV-2 infection with spontaneous recovery. In the remaining three patients, both MG exacerbation and SARS-CoV-2 infection were reported. These patients experienced MG exacerbation in the preceding month or concurrently with COVID-19 and were aged over 50 years. Due to the SARS-CoV-2 infection, they required antibiotic and oxygen therapy, and hospitalisation was necessary in the case of two obese patients. None of the patients died due to COVID-19, and nor did any require discontinuation of immunosuppressive therapies during the study period. In total, 12 patients in the study group experienced neither MG exacerbation nor SARS-CoV-2 infection. CONCLUSIONS: In the presented sample of Polish MG patients, favourable outcomes of COVID-19 were observed. Further studies are needed to evaluate the reliable course of COVID-19 taking into account international differences, the types of treatment applied, and the ratio of vaccinated to unvaccinated MG patients.

COVID-19 in patients with myasthenia gravis: Epidemiology and disease course.

INTRODUCTION/AIMS: Coronavirus disease 2019 (COVID-19), a disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, has become a global pandemic. Patients with myasthenia gravis (MG), often treated with immunosuppressants, might be at higher risk of developing COVID-19 and of demonstrating a severe disease course. We aimed to study prevalence and describe features of COVID-19 in MG patients. METHODS: In May 2020, we conducted telephonic interviews with MG patients followed at our referral center. We collected structured data regarding MG and COVID-19, which was diagnosed as probable or confirmed according to the European Centre for Disease Prevention and Control case definition. We compared confirmed-COVID-19 prevalence calculated from the beginning of the pandemic in MG patients with that of the overall Pavia district. RESULTS: We interviewed 162 MG patients (median age, 66 y; interquartile range 41-77; males 59.9%), 88 from the Pavia district. Three patients had SARS-CoV-2-confirmed by polymerase chain reaction and eight had probable-COVID-19. In the Pavia district, the prevalence of confirmed-COVID-19 among MG patients (1/88, 1.14%) and overall population (4777/546 515, 0.87%) did not differ (P = .538). Higher Myasthenia Gravis Foundation of America clinicalclass and the need for recent rescue treatment, but not ongoing immunosuppressive treatments, were associated with COVID-19 risk. Of 11 MG patients with probable/confirmed-COVID-19, 3 required ventilator support, and 2 elderly patients died of COVID-19 respiratory insufficiency. Only 1 of11 patients experienced worsening MG symptoms, which improved after increasing their steroid dose. DISCUSSION: The risk of COVID-19 in MG patients seems to be no higher than that of the general population, regardless of immunosuppressive therapies. In our cohort, COVID-19 barely affected MG course.

Surveillance Study of Acute Neurological Manifestations among 439 Egyptian Patients with COVID-19 in Assiut and Aswan University Hospitals.

BACKGROUND: COVID-19 can be accompanied by acute neurological complications of both central and peripheral nervous systems (CNS and PNS). In this study, we estimate the frequency of such complications among hospital inpatients with COVID-19 in Assiut and Aswan university hospitals. MATERIALS AND METHODS: We screened all patients with suspected COVID-19 admitted from 1 June to 10 August 2020 to the university hospitals of Assiut and Aswan in Upper Egypt. Clinical and laboratory tests, CT/MRI of the chest and brain, and neurophysiology study were performed for each patient if indicated. RESULTS: 439 patients had confirmed/probable COVID-19; neurological manifestations occurred in 222. Of these, 117 had acute neurological disease and the remainder had nonspecific neuropsychiatric symptoms such as headache, vertigo, and depression. The CNS was affected in 75 patients: 55 had stroke and the others had convulsions (5), encephalitis (6), hypoxic encephalopathy (4), cord myelopathy (2), relapse of multiple sclerosis (2), and meningoencephalitis (1). The PNS was affected in 42 patients: the majority had anosmia and ageusia (31) and the others had Guillain-Barré syndrome (4), peripheral neuropathy (3), myasthenia gravis (MG, 2), or myositis (2). Fever, respiratory symptoms, and headache were the most common general symptoms. Hypertension, diabetes mellitus, and ischemic heart disease were the most common comorbidities in patients with CNS affection. CONCLUSION: In COVID-19, both the CNS and PNS are affected. Stroke was the most common complication for CNS, and anosmia and/or ageusia were common for PNS diseases. However, there were 6 cases of encephalitis, 2 cases of spinal cord myelopathy, 2 cases of MG, and 2 cases of myositis.

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.