Randomized Trial of Thymectomy in Myasthenia Gravis

Haran, Michal; Starobin, Daniel; Schlesinger, Ilana

doi:10.1056/nejmc1611704

Cited by 34 publications

(13 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In AChR-MG, a randomized, controlled trial of thymectomy in non-thymomatous acetylcholine receptor patients demonstrated a significant improvement in clinical outcomes after thymectomy, as well as a decreased requirement for immunosuppression (24). Conversely, available studies on thymectomy in MuSK-MG outline a limited improvement in clinical outcomes or immunosuppression management after thymectomy (21)(22)(23)(24). Moreover, it has been reported that the outcome in MuSK-MG after thymectomy may not be beneficial (25).…”

Section: Clinical Featuresmentioning

confidence: 99%

MuSK-Associated Myasthenia Gravis: Clinical Features and Management

et al. 2020

View full text Add to dashboard Cite

Muscle-specific tyrosine kinase (MuSK) myasthenia gravis (MG) is a rare, frequently more severe, subtype of MG with different pathogenesis, and peculiar clinical features. The prevalence varies among countries and ethnic groups, affecting 5-8% of all MG patients. MuSK-MG usually has an acute onset affecting mainly the facial-bulbar muscles. The symptoms usually progress rapidly, within a few weeks. Early respiratory crises are frequent. The disease may lead to generalized muscle weakness up to muscle atrophy. The main bulbar involvement, the absence of significant thymus alterations, and the association with HLA class II DR14, DR16, and DQ5 alleles have been confirmed. Atypical onset, such as ocular involvement, lack of symptom fluctuations, acetylcholinesterase inhibitors failure, and negative results of electrophysiologic testing, if not specifically performed in the mainly involved muscle groups, makes MuSK-MG diagnosis challenging. In most cases, steroids are effective. Conventional immunosuppressants are not commonly able to replace steroids in maintaining a satisfactory long-term control of symptoms. However, the majority of MuSK-MG patients are refractory to treatment. In these cases, the use of rituximab showed promising results, resulting in sustained symptom control.

show abstract

Section: Clinical Featuresmentioning

confidence: 99%

MuSK-Associated Myasthenia Gravis: Clinical Features and Management

et al. 2020

View full text Add to dashboard Cite

show abstract

“…The role of the thymus gland in driving MG has been known for almost a century (35,36). The results from the international thymectomy trial (MGTX) have been crucial in underscoring the role of thymectomy in the management of MG (37). In this trial, non-thymoma MG patients up to the age of 65, with generalized disease and with positive AChR antibodies, were recruited.…”

Section: Thymectomymentioning

confidence: 99%

A Practical Approach to Managing Patients With Myasthenia Gravis—Opinions and a Review of the Literature

Farrugia

Goodfellow

2020

Front. Neurol.

View full text Add to dashboard Cite

When the diagnosis of myasthenia gravis (MG) has been secured, the aim of management should be prompt symptom control and the induction of remission or minimal manifestations. Symptom control, with acetylcholinesterase inhibitors such as pyridostigmine, is commonly employed. This may be sufficient in mild disease. There is no single universally accepted treatment regimen. Corticosteroids are the mainstay of immunosuppressive treatment in patients with more than mild MG to induce remission. Immunosuppressive therapies, such as azathioprine are prescribed in addition to but sometimes instead of corticosteroids when background comorbidities preclude or restrict the use of steroids. Rituximab has a role in refractory MG, while plasmapheresis and immunoglobulin therapy are commonly prescribed to treat MG crisis and in some cases of refractory MG. Data from the MGTX trial showed clear evidence that thymectomy is beneficial in patients with acetylcholine receptor (AChR) antibody positive generalized MG, up to the age of 65 years. Minimally invasive thymectomy surgery including robotic-assisted thymectomy surgery has further revolutionized thymectomy and the management of MG. Ocular MG is not life-threatening but can be significantly disabling when diplopia is persistent. There is evidence to support early treatment with corticosteroids when ocular motility is abnormal and fails to respond to symptomatic treatment. Treatment needs to be individualized in the older age-group depending on specific comorbidities. In the younger age-groups, particularly in women, consideration must be given to the potential teratogenicity of certain therapies. Novel therapies are being developed and trialed, including ones that inhibit complement-induced immunological pathways or interfere with antibody-recycling pathways. Fatigue is common in MG and should be duly identified from fatigable weakness and managed with a combination of physical therapy with or without psychological support. MG patients may also develop dysfunctional breathing and the necessary respiratory physiotherapy techniques need to be implemented to alleviate the patient's symptoms of dyspnoea. In this review, we discuss various facets of myasthenia management in adults with ocular Farrugia and Goodfellow Practical Approaches to Myasthenia Management and generalized disease, including some practical approaches and our personal opinions based on our experience.

show abstract

“…Patients with MG pose a major challenge for anesthesiologists, and the postsurgical risk of respiratory problems is a matter of concern. Yet, surgery is often necessary in MG, especially because thymectomy is a standard treatment of seropositive patients with AChR Abs (Wolfe et al, 2016). A careful selection of anesthetic drugs must be made to avoid complications.…”

Section: Postsynaptic Neuromuscular Junction Effectsmentioning

confidence: 99%

Pathomechanisms and Clinical Implications of Myasthenic Syndromes Exacerbated and Induced by Medical Treatments

Krenn

Grisold

Wohlfarth

et al. 2020

Front. Mol. Neurosci.

View full text Add to dashboard Cite

Myasthenic syndromes are typically characterized by muscle weakness and increased fatigability due to an impaired transmission at the neuromuscular junction (NMJ). Most cases are caused by acquired autoimmune conditions such as myasthenia gravis (MG), typically with antibodies against the acetylcholine receptor (AChR). Different drugs are among the major factors that may complicate pre-existing autoimmune myasthenic conditions by further impairing transmission at the NMJ. Some clinical observations are substantiated by experimental data, indicating that presynaptic, postsynaptic or more complex pathomechanisms at the NMJ may be involved, depending on the individual compound. Most robust data exist for the risks associated with some antibiotics (e.g., aminoglycosides, ketolides, fluoroquinolones) and cardiovascular medications (e.g., class Ia antiarrhythmics, beta blockers). Apart from primarily autoimmune-mediated disorders of the NMJ, de novo myasthenic manifestations may also be triggered by medical treatments that induce an autoimmune reaction. Most notably, there is growing evidence that the immune checkpoint inhibitors (ICI), a modern class of drugs to treat various malignancies, represent a relevant risk factor to develop severe and progressive medication-induced myasthenia via an immune-mediated mechanism. From a clinical perspective, it is of utmost importance for the treating physicians to be aware of such adverse treatment effects and their consequences. In this article, we aim to summarize existing evidence regarding the key molecular and immunological mechanisms as well as the clinical implications of medication-aggravated and medication-induced myasthenic syndromes.

show abstract

Randomized Trial of Thymectomy in Myasthenia Gravis

Cited by 34 publications

References 20 publications

MuSK-Associated Myasthenia Gravis: Clinical Features and Management

MuSK-Associated Myasthenia Gravis: Clinical Features and Management

A Practical Approach to Managing Patients With Myasthenia Gravis—Opinions and a Review of the Literature

Pathomechanisms and Clinical Implications of Myasthenic Syndromes Exacerbated and Induced by Medical Treatments

Contact Info

Product

Resources

About