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Frontiers in Neurology 2023Juvenile myasthenia gravis (JMG) is a rare autoimmune disease that has so far only been described in small cohort studies. We defined the clinical characteristics,...
BACKGROUND
Juvenile myasthenia gravis (JMG) is a rare autoimmune disease that has so far only been described in small cohort studies. We defined the clinical characteristics, management, and outcomes of JMG patients over the past 22 years.
METHODS
A search of PubMed, EMBASE, and web of science (1/2000-2/2022) identified all English language and human studies of JMG. The population was patients diagnosed with JMG. Outcomes included the history of myasthenic crisis, autoimmune comorbidity, mortality, and treatment outcome. Data extraction was performed by independent reviewers. And we performed a pooled reanalysis of all published data in the included studies and compared with other studies of adult cohorts.
RESULTS
We identified 11 articles describing 1,109 patients diagnosed between 2006 and 2021. JMG occurred in 60.4% of female patients. The mean age at presentation was 7.38 years old, and 60.6% of the patients had ocular symptoms as the first clinical manifestation. The most common initial presentation was ptosis, which occurred in 77.7% patients. AchR-Ab positive accounted for 78.7%. 641 patients received thymus examination, found to have thymic hyperplasia in 64.9% and thymoma in 2.2%. Autoimmune comorbidity was found in 13.6% and the most common one is thyroid disease (61.5%). First-line therapy, including pyridostigmine and steroids, was initiated in 97.8 and 68.6%, respectively. Six patients resolved spontaneously without treatment. Thymectomy was performed in 45.6%. 10.6% of patients had a history of myasthenic crisis. Completely stable remission was achieved in 23.7% and mortality was reported in 2 studies, which reported 8 deaths.
CONCLUSION
JMG is a rare disease with a relatively benign course, and differs from adult MG in some clinical features. The treatment regimen guideline for children is still not well-established. There is a need for prospective studies to properly evaluate treatment regimes.
PubMed: 36970540
DOI: 10.3389/fneur.2023.1119294 -
Frontiers in Oncology 2023According to the principle, thymomas combined with myasthenia gravis (MG) require surgical treatment. However, patients with non-MG thymoma rarely develop MG and early-...
INTRODUCTION
According to the principle, thymomas combined with myasthenia gravis (MG) require surgical treatment. However, patients with non-MG thymoma rarely develop MG and early- or late-onset MG after surgery is called postoperative MG (PMG). Our study used a meta-analysis to examine the incidence of PMG and risk factors.
METHODS
Relevant studies were searched for in the PubMed, EMBASE, Web of Science, CNKI,and Wanfang databases. Investigations that directly or indirectly analyzed the risk factors for PMG development in patients with non-MG thymoma were included in this study. Furthermore, risk ratios (RR) with 95% confidence intervals (CI) were pooled using meta-analysis, and fixed-effects or random-effects models were used depending on the heterogeneity of the included studies.
RESULTS
Thirteen cohorts containing 2,448 patients that met the inclusion criteria were included. Metaanalysis revealed that the incidence of PMG in preoperative patients with non-MG thymoma was 8%. Preoperative seropositive acetylcholine receptor antibody (AChR-Ab) (RR = 5.53, 95% CI 2.36 - 12.96, P<0.001), open thymectomy (RR =1.84, 95% CI 1.39 - 2.43, P<0.001), non-R0 resection (RR = 1.87, 95% CI 1.36 - 2.54, P<0.001), world health organization (WHO) type B (RR =1.80, 95% CI 1.07 - 3.04, P= 0.028), and postoperative inflammation (RR = 1.63, 95% CI 1.26 - 2.12, P<0.001) were the risk factors for PMG in patients with thymoma. Masaoka stage (P = 0.151) and sex (P = 0.777) were not significantly associated with PMG.
DISCUSSION
Patients with thymoma but without MG had a high probability of developing PMG. Although the incidence of PMG was very low, thymectomy could not completely prevent the occurrence of MG. Preoperative seropositive AChR-Ab level, open thymectomy, non-R0 resection, WHO type B, and postoperative inflammation were risk factors for PMG.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/PROSPERO/, identifier CRD42022360002.
PubMed: 36845745
DOI: 10.3389/fonc.2023.1061264 -
Frontiers in Neurology 2022While immune checkpoint inhibitors (ICIs) have been revolutionary in the treatment of cancer, their administration has been associated with a variety of immune-related...
BACKGROUND
While immune checkpoint inhibitors (ICIs) have been revolutionary in the treatment of cancer, their administration has been associated with a variety of immune-related adverse events (irAEs), including myasthenia gravis (MG), and Lambert-Eaton myasthenic syndrome (LEMS).
OBJECTIVE
To provide a comprehensive synthesis of the evidence supporting an etiological role for ICIs in MG and LEMS in patients with no prior history of autoimmune disease.
HYPOTHESIS
ICIs may trigger MG and LEMS in patients with no prior susceptibility to autoimmune disease.
METHODS
Relevant primary research on Medline was interrogated using a series of search algorithms. Search terms were constructed based on the PICOS tool endorsed by the Cochrane Collaboration, which describes population, intervention, comparison, outcomes, and study design. Papers were screened according to inclusion and exclusion criteria. Additional papers were retrieved from the reference lists of screened papers. Each paper included in the qualitative synthesis was assigned an integrated metric of evidence (IME) value, ranging from 0 to 7, based on study design, quality of data, likelihood of a causal link between the immune checkpoint inhibitor(s) and MG/LEMS, confidence of MG/LEMS diagnosis, and the number of patients treated with an ICI prior to MG/LEMS diagnosis.
RESULTS
Ninety-four papers describing at least one patient treated with ICI(s) prior to the onset of MG and/or LEMS were documented. Overall evidence for a causal link between ICI administration and MG/LEMS was low, with a median IME value of 2.88 (range 2.05-6.61).
CONCLUSIONS
There is a paucity of evidence in support of an etiological relationship between ICIs and MG/LEMS, due largely to the lack of mechanistic studies and/or prospective clinical trials with relevant study endpoints. The current literature is dominated by case reports and retrospective cohort studies, which inherently yield only low-level evidence, supporting the need for further work in this area. A role of ICIs in the etiology of MG/LEMS remains plausible, arguing for continued vigilance for irAEs in patients treated with these drugs. We argue that there is a need for future mechanistic, high quality, large-scale studies specifically investigating the possible etiological role of ICIs in MG/LEMS.
PubMed: 36698907
DOI: 10.3389/fneur.2022.1004810 -
The Journal of Investigative Dermatology May 2023Vitiligo has been reported to be associated with a variety of diseases, but it has not been systematically reviewed. Therefore, we aimed to identify prevalent diseases... (Meta-Analysis)
Meta-Analysis
Vitiligo has been reported to be associated with a variety of diseases, but it has not been systematically reviewed. Therefore, we aimed to identify prevalent diseases in patients with vitiligo and quantify their associations compared with those in healthy controls. A comprehensive search of MEDLINE and EMBASE from the inception to June 2022 was conducted. Observational studies on prevalent diseases in patients with vitiligo compared with those in healthy controls were included, whereas studies limited to pediatrics or providing only laboratory results were excluded. A total of 78 studies were eligible for analyses. Patients with vitiligo showed higher risks of having comorbid autoimmune and connective tissue diseases, including alopecia areata (OR = 2.63, 95% confidence interval [CI] = 2.50‒2.78), discoid lupus erythematosus (OR = 2.54, 95% CI = 1.74‒3.72), Sjogren's syndrome (OR = 2.50, 95% CI = 1.98‒3.16), myasthenia gravis (OR = 2.30, 95% CI = 1.74‒3.02), systemic lupus erythematosus (OR = 1.96, 95% CI = 1.52‒2.52), and rheumatoid arthritis (OR = 1.82, 95% CI = 1.55‒2.15). Thyroid diseases, diabetes mellitus, metabolic syndrome, sensorineural hypoacusis, and ophthalmic abnormalities were also more prevalent in patients with vitiligo. In conclusion, vitiligo is associated with various systemic diseases. Physicians should evaluate and manage potential comorbid conditions in patients with vitiligo.
Topics: Humans; Child; Vitiligo; Comorbidity; Sjogren's Syndrome; Lupus Erythematosus, Systemic; Thyroid Diseases; Autoimmune Diseases
PubMed: 36574529
DOI: 10.1016/j.jid.2022.10.021 -
Brain and Behavior Jan 2023Myasthenia gravis (MG) people experience adverse psychiatric outcomes, which may impact on their life and disturb their daily activity. Depression and anxiety are... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Myasthenia gravis (MG) people experience adverse psychiatric outcomes, which may impact on their life and disturb their daily activity. Depression and anxiety are identified as significant psychiatric problems that MG people face. However, there is no sufficient epidemiological information about depression and anxiety-based publication. Due to this limitation, the aim of this study was to review the prevalence of depression and anxiety in MG patients.
METHODS
Original and international databases were searched to find papers about the estimation of anxiety and depression. Random-effects analysis was used for calculating the proportions of anxiety and depression. For estimating anxiety and depression based the severity, instruments, type of studies, and study regions, subgroup analysis was performed.
RESULTS
38 studies met inclusion criteria and entered study. The pooling of the prevalence of depression was found at 36%, (95% CI 28% to 45%). Also, prevalence of anxiety was found at 33%, (95% CI 25% to 42%). Prevalence of depression based on mild, moderate, and severe level was 27%, 14%, and 9%, respectively.
CONCLUSIONS
Anxiety and depression are a major concern among MG individuals. The estimation of both anxiety and depression are high even when compared to other autoimmune diseases. It seems depression and anxiety are important issues and more attention needs to be paid to these psychiatric disorders.
Topics: Humans; Depression; Prevalence; Anxiety; Anxiety Disorders; Myasthenia Gravis
PubMed: 36495116
DOI: 10.1002/brb3.2840 -
Medicine Sep 2022Thymic epithelial tumors (TETs) originate in the thymic epithelial cell, including thymoma and thymic carcinoma. Surgical resection is the first choice for most... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Thymic epithelial tumors (TETs) originate in the thymic epithelial cell, including thymoma and thymic carcinoma. Surgical resection is the first choice for most patients. However, some studies have shown that the factors affecting the prognosis of these patients are not consistent. To evaluate prognostic factors in patients with surgically resected thymic epithelial tumors, we performed a meta-analysis.
METHODS
We searched the Chinese biomedical literature database, Pubmed, Embase, Cochrane Library and other electronic databases. Studies including postoperative overall survival (OS) and predictors of TETs were included. We made a comprehensive analysis the hazard ratios (HRs) through a single proportional combination. HRs were combined using single proportion combinations.
RESULTS
The meta-analysis included 11,695 patients from 26 studies. The pooled OS was 84% at 5 years and 73% at 10 years after TETs operation. The age as continuous-year (HR 1.04, 95% confidence interval (CI) 1.02-1.04), incomplete resection (HR 4.41, 95% CI 3.32-5.85), WHO histologic classification (B2/B3 vs A/AB/B1 HR 2.76, 95% CI 1.25-6.21), Masaoka Stage (stage III/IV vs I/II HR 2.74, 95% CI 2.12-3.55,) were the poor prognostic factors.
CONCLUSIONS
For patients with TETs after surgical resection, advanced age, incomplete resection, WHO classification B2/B3, and higher Masaoka stage are risk factors for poor prognosis.
Topics: Humans; Neoplasm Staging; Neoplasms, Glandular and Epithelial; Prognosis; Retrospective Studies; Thymoma; Thymus Neoplasms
PubMed: 36181069
DOI: 10.1097/MD.0000000000030867 -
The Canadian Journal of Infectious... 2022Myasthenia gravis (MG) is a neuromuscular, autoimmune disease that causes weakness by impairing neuromuscular transmission. According to reports, vaccines can lead to... (Review)
Review
BACKGROUND
Myasthenia gravis (MG) is a neuromuscular, autoimmune disease that causes weakness by impairing neuromuscular transmission. According to reports, vaccines can lead to autoimmunity in different ways, and COVID-19 vaccines are suggested to trigger MG. We conducted this systematic review to assess MG patients after the COVID-19 vaccination.
METHODS
We collected 231 studies from four databases from inception to 26 March 2022.
RESULTS
4 case studies were selected from 231 research studies, and data were extracted based on inclusion criteria. In all cases, MG was reported following COVID-19 vaccination. Symptoms such as muscle weakness, numbness, and ptosis were common. The MG was confirmed through RNST, MRC, NCS, and AchR-binding antibody titer tests.
CONCLUSION
Although all cases of MG were diagnosed following appropriate tests, the sample size was small; therefore, further investigation is required to demonstrate the possible association between MG and COVID-19 vaccination.
PubMed: 36164665
DOI: 10.1155/2022/5009450 -
Therapeutics and Clinical Risk... 2022[This corrects the article DOI: 10.2147/TCRM.S266031.].
Erratum: Practical Management for Use of Eculizumab in the Treatment of Severe, Refractory, Non-Thymomatous, AChR + Generalized Myasthenia Gravis: A Systematic Review [Erratum].
[This corrects the article DOI: 10.2147/TCRM.S266031.].
PubMed: 35958346
DOI: 10.2147/TCRM.S384532 -
Therapeutics and Clinical Risk... 2022Myasthenia gravis (MG) is a rare autoimmune disorder caused by specific autoantibodies at the neuromuscular junction. MG is classified by the antigen specificity of... (Review)
Review
Myasthenia gravis (MG) is a rare autoimmune disorder caused by specific autoantibodies at the neuromuscular junction. MG is classified by the antigen specificity of these antibodies. Acetylcholine receptor (AChR) antibodies are the most common type (74-88%), followed by anti-muscle specific kinase (MuSK) and other antibodies. While all these antibodies lead to neuromuscular transmission failure, the immuno-pathogenic mechanisms are distinct. Complement activation is a primary driver of AChR antibody-positive MG (AChR+ MG) pathogenesis. This leads to the formation of the membrane attack complex and destruction of AChR receptors and the postsynaptic membrane resulting in impaired neurotransmission and muscle weakness characteristic of MG. Broad-based immune-suppressants like corticosteroids are effective in controlling MG; however, their long-term use can be associated with significant adverse effects. Advances in translational research have led to the development of more directed therapeutic agents that are likely to alter the future of MG treatment. Eculizumab is a humanized monoclonal antibody that inhibits the cleavage of complement protein C5 and is approved for use in generalized MG. In this review, we discuss the pathophysiology of MG; the therapeutic efficacy and tolerability of eculizumab, as well as the practical guidelines for its use in MG; future studies exploring the role of eculizumab in different stages and subtypes of MG subtypes; the optimal duration of therapy and its discontinuation; the characterization of non-responder patients; and the use of biomarkers for monitoring therapy are highlighted. Based on the pathophysiologic mechanisms, emerging therapies and new therapeutic targets are also reviewed.
PubMed: 35855752
DOI: 10.2147/TCRM.S266031 -
Obstetric Medicine Jun 2022Myasthenia gravis is an autoimmune disease which can impact pregnancy.
BACKGROUND
Myasthenia gravis is an autoimmune disease which can impact pregnancy.
METHODS
Six databases were systematically searched for studies with at least five subjects reporting pregnancy outcomes for women with myasthenia gravis in pregnancy. Assessment of bias was performed for all included studies. Forty-eight cases from our own centre were also included in the analysis.
RESULTS
In total, 32 publications met inclusion criteria for systematic review, for a total of 33 unique data sets including 48 cases from our institution. Outcome data was available for 824 pregnancies. Spontaneous vaginal delivery occurred in 56.3% of pregnancies. Overall risk of myasthenia gravis exacerbation was 33.8% with a 6.4% risk of myasthenic crisis in pregnancy and 8.2% postpartum. The incidence risk of transient neonatal myasthenia gravis was 13.0%.
CONCLUSIONS
The current systematic review provides the best estimates of risk currently available to aid in counselling women with myasthenia gravis in pregnancy.
PubMed: 35845224
DOI: 10.1177/1753495X211041899