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Neurologic Clinics May 2018With specialized care, patients with myasthenia gravis can have very good outcomes. The mainstays of treatment are acetylcholinesterase inhibitors, and immunosuppressive... (Review)
Review
With specialized care, patients with myasthenia gravis can have very good outcomes. The mainstays of treatment are acetylcholinesterase inhibitors, and immunosuppressive and immunomodulatory therapies. There is good evidence thymectomy is beneficial in thymomatous and nonthymomatous disease. Nearly all of the drugs used for MG are considered "off-label." The 2 exceptions are acetylcholinesterase inhibitors and complement inhibition with eculizumab, which was recently approved by the US Food and Drug Administration for myasthenia gravis. This article reviews the evidence base and provides a framework for the treatment of myasthenia gravis, highlighting recent additions to the literature.
Topics: Humans; Immunosuppressive Agents; Myasthenia Gravis; Thymectomy
PubMed: 29655452
DOI: 10.1016/j.ncl.2018.01.011 -
The New England Journal of Medicine Aug 2023The function of the thymus in human adults is unclear, and routine removal of the thymus is performed in a variety of surgical procedures. We hypothesized that the adult...
BACKGROUND
The function of the thymus in human adults is unclear, and routine removal of the thymus is performed in a variety of surgical procedures. We hypothesized that the adult thymus is needed to sustain immune competence and overall health.
METHODS
We evaluated the risk of death, cancer, and autoimmune disease among adult patients who had undergone thymectomy as compared with demographically matched controls who had undergone similar cardiothoracic surgery without thymectomy. T-cell production and plasma cytokine levels were also compared in a subgroup of patients.
RESULTS
After exclusions, 1420 patients who had undergone thymectomy and 6021 controls were included in the study; 1146 of the patients who had undergone thymectomy had a matched control and were included in the primary cohort. At 5 years after surgery, all-cause mortality was higher in the thymectomy group than in the control group (8.1% vs. 2.8%; relative risk, 2.9; 95% confidence interval [CI], 1.7 to 4.8), as was the risk of cancer (7.4% vs. 3.7%; relative risk, 2.0; 95% CI, 1.3 to 3.2). Although the risk of autoimmune disease did not differ substantially between the groups in the overall primary cohort (relative risk, 1.1; 95% CI, 0.8 to 1.4), a difference was found when patients with preoperative infection, cancer, or autoimmune disease were excluded from the analysis (12.3% vs. 7.9%; relative risk, 1.5; 95% CI, 1.02 to 2.2). In an analysis involving all patients with more than 5 years of follow-up (with or without a matched control), all-cause mortality was higher in the thymectomy group than in the general U.S. population (9.0% vs. 5.2%), as was mortality due to cancer (2.3% vs. 1.5%). In the subgroup of patients in whom T-cell production and plasma cytokine levels were measured (22 in the thymectomy group and 19 in the control group; mean follow-up, 14.2 postoperative years), those who had undergone thymectomy had less new production of CD4+ and CD8+ lymphocytes than controls (mean CD4+ signal joint T-cell receptor excision circle [sjTREC] count, 1451 vs. 526 per microgram of DNA [P = 0.009]; mean CD8+ sjTREC count, 1466 vs. 447 per microgram of DNA [P<0.001]) and higher levels of proinflammatory cytokines in the blood.
CONCLUSIONS
In this study, all-cause mortality and the risk of cancer were higher among patients who had undergone thymectomy than among controls. Thymectomy also appeared be associated with an increased risk of autoimmune disease when patients with preoperative infection, cancer, or autoimmune disease were excluded from the analysis. (Funded by the Tracey and Craig A. Huff Harvard Stem Cell Institute Research Support Fund and others.).
Topics: Humans; Adult; Thymectomy; Thymus Gland; CD8-Positive T-Lymphocytes; Cytokines; Autoimmune Diseases
PubMed: 37530823
DOI: 10.1056/NEJMoa2302892 -
Continuum (Minneapolis, Minn.) Dec 2022This article reviews updated diagnostic procedures and currently available treatment modalities for myasthenia gravis (MG). (Review)
Review
PURPOSE OF REVIEW
This article reviews updated diagnostic procedures and currently available treatment modalities for myasthenia gravis (MG).
RECENT FINDINGS
Patients with MG can be classified based on antibody status and their clinical presentation; treatment responses may differ based on disease subtypes. Improved diagnostic methods and recognition of new antigenic targets such as lipoprotein-related protein 4 have led to improved diagnostic efficiencies. Corticosteroids remain the first-line immunotherapy, but there is a trend toward minimizing their use at high doses and for long durations. Oral immunosuppressants such as mycophenolate mofetil, azathioprine, and tacrolimus remain useful. An international, multicenter randomized trial comparing thymectomy plus prednisone with prednisone alone demonstrated that thymectomy improves clinical outcomes in selected patients with nonthymomatous MG. Eculizumab, efgartigimod, and ravulizumab have recently been approved by the US Food and Drug Administration (FDA) for adult patients with generalized MG who are acetylcholine receptor-antibody positive. These drugs take advantage of novel mechanisms of action and expand treatment options for patients with MG. Data on rituximab suggest that it can be a good option, especially for patients with MG who are positive for antibodies against muscle-specific tyrosine kinase (MuSK). The number of clinical trials and drugs in development for MG is steadily increasing.
SUMMARY
The diagnosis of MG can generally be made from the patient's history, a neurologic examination, and laboratory and electrodiagnostic testing. Carefully selected treatment improves outcomes in MG. Additional treatment options for MG will likely be available in the near future.
Topics: Adult; Humans; Prednisone; Myasthenia Gravis; Immunosuppressive Agents; Rituximab; Thymectomy; Autoantibodies; Multicenter Studies as Topic
PubMed: 36537972
DOI: 10.1212/CON.0000000000001161 -
Thoracic Surgery Clinics Feb 2023The robotic platform can be viewed as an advanced thoracoscopic instrument and can be utilized for any pathology amenable to thoracoscopic surgery. This ultimately comes... (Review)
Review
The robotic platform can be viewed as an advanced thoracoscopic instrument and can be utilized for any pathology amenable to thoracoscopic surgery. This ultimately comes down to surgeon comfort, but many have demonstrated the robotic approach to be useful in benign and malignant mediastinal disease in all compartments with at least equivalent-if not superior-outcomes compared to sternotomy for many metrics. There are various robotic approaches to the same compartments (such as with thymectomy), and no one robotic approach has proven superior to another. Here we describe our robotic approach to common mediastinal pathology.
Topics: Humans; Robotic Surgical Procedures; Thymectomy; Robotics; Mediastinum; Thoracoscopy
PubMed: 36372537
DOI: 10.1016/j.thorsurg.2022.08.007 -
Kyobu Geka. the Japanese Journal of... Sep 2019Extended thymectomy reported by Masaoka in 1981 is a standard surgical treatment in patients with myasthenia gravis. Thoracoscopic thymectomy, which could be less...
Extended thymectomy reported by Masaoka in 1981 is a standard surgical treatment in patients with myasthenia gravis. Thoracoscopic thymectomy, which could be less invasive, has been widely accepted for an anterior mediastinal lesion in addition to the conventional median sternotomy approach. In thoracoscopic thymectomy, artificial pneumothorax using carbon deoxide(CO2) insufflation or chest wall lifting using rib hook can contribute to make better surgical view with enough working space. Recently, thoracoscopic thymectomy with subxiphoid approach has been reported with its usefulness. Since MGTX trial revealed the significant efficacy of thymectomy in myasthenia gravis patients aged up to 65, the number of patients having operative indication may increase. Therefore, thoracic surgeons should acquire the safe and effective technical skill of thymectomy.
Topics: Humans; Mediastinum; Myasthenia Gravis; Thoracic Surgery, Video-Assisted; Thoracic Wall; Thoracoscopy; Thymectomy
PubMed: 31582710
DOI: No ID Found -
Interactive Cardiovascular and Thoracic... Jul 2022Robot-assisted thymectomy through a subxiphoid scopic approach can provide a good surgical view, similar to that of median sternotomy. We originally used the subxiphoid...
Robot-assisted thymectomy through a subxiphoid scopic approach can provide a good surgical view, similar to that of median sternotomy. We originally used the subxiphoid port only for the robotic scope to avoid instrument collision with the assistant device. This approach, robotic subxiphoid-optical thymectomy, is advantageous for the safe and accurate dissection of the bilateral phrenic nerves and the left brachiocephalic vein, which are especially needed in extended thymectomy for patients with myasthenia gravis.
Topics: Humans; Myasthenia Gravis; Robotic Surgical Procedures; Robotics; Sternotomy; Thymectomy
PubMed: 35416955
DOI: 10.1093/icvts/ivac104 -
Current Opinion in Neurology Oct 2023Thymectomy has long been used in the treatment of patients with myasthenia gravis and antibodies against the acetylcholine receptor. However, its effectiveness has only... (Review)
Review
PURPOSE OF REVIEW
Thymectomy has long been used in the treatment of patients with myasthenia gravis and antibodies against the acetylcholine receptor. However, its effectiveness has only been proven a few years ago in a randomized controlled trial in patients under the age of 65. Here, we review the current literature focusing on patient subgroups, potential biomarkers for outcome prediction and the choice of surgical approach.
RECENT FINDINGS
Long-term follow-up studies after thymectomy confirmed that the benefits regarding clinical outcome parameters and a reduced need for immunosuppressive treatment persist. Nevertheless, a substantial proportion of patients in real-world cohorts do not reach complete stable remission after thymectomy indicating that the underlying autoimmune process is sustained in the periphery. Our understanding of the responsible mechanisms has improved with recent studies. Presently, outcome data after thymectomy in several patient subgroups, such as those aged over 50 years, those with juvenile onset or those with purely ocular symptoms are limited and have been the focus of recent research activities. Similarly, biomarkers guiding an appropriate patient selection for thymectomy are under investigation. A number of cohort studies demonstrated that minimal invasive surgical techniques such as extended robotic thymectomy lead to similar positive outcomes as a transsternal approach with potentially fewer short-term adverse effects.
SUMMARY
Thymectomy is an effective treatment option in adult patients with early onset acetylcholine-receptor positive myasthenia gravis but uncertainty remains with regard to certain patient subgroups.
Topics: Adult; Humans; Middle Aged; Thymectomy; Myasthenia Gravis; Antibodies; Drug-Related Side Effects and Adverse Reactions; Immunosuppressive Agents; Randomized Controlled Trials as Topic
PubMed: 37639450
DOI: 10.1097/WCO.0000000000001189 -
Autoimmunity Reviews Apr 2024
Topics: Humans; Autoimmune Diseases; Splenectomy; Thymectomy; Autoimmunity
PubMed: 38382859
DOI: 10.1016/j.autrev.2024.103518 -
Journal of Immunology (Baltimore, Md. :... Mar 2021
Topics: Aging; Animals; Cell Line, Tumor; Cell Proliferation; Forkhead Transcription Factors; Incidence; Mice; Neoplasms; T-Lymphocytes; Thymectomy; Thymus Gland
PubMed: 33593850
DOI: 10.4049/jimmunol.2190001 -
Thoracic Surgery Clinics May 2019In the early 1900s, chance observations of improved symptoms in several myasthenic patients undergoing thyroidectomy for goiters with concomitant resection of the... (Review)
Review
In the early 1900s, chance observations of improved symptoms in several myasthenic patients undergoing thyroidectomy for goiters with concomitant resection of the adjacent thymus gland, first suggested a possible association between the thymus and myasthenia gravis. With the remarkable progress made in the understanding and treatment of myasthenia and in the anesthetic, surgical, and postoperative management of patients undergoing thoracic procedures, the initial high morbidity and mortality associated with thymectomy have been all but eliminated, and thymectomy is frequently incorporated into the long-term management strategy of this disease.
Topics: History, 20th Century; Humans; Myasthenia Gravis; Thymectomy
PubMed: 30927996
DOI: 10.1016/j.thorsurg.2018.12.011