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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 [Pā=ā0.009]; 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 -
CMAJ : Canadian Medical Association... Sep 2018
Topics: Diagnostic Techniques, Ophthalmological; Electromyography; Humans; Myasthenia Gravis; Thymectomy
PubMed: 30249760
DOI: 10.1503/cmaj.180656 -
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 -
Proceedings of the National Academy of... Dec 2020
Topics: B-Lymphocytes; Clone Cells; Humans; Myasthenia Gravis; Thymectomy
PubMed: 33273117
DOI: 10.1073/pnas.2022901117 -
The Journal of Thoracic and... Jul 2017
Topics: Biomedical Research; Humans; Myasthenia Gravis; Thymectomy; Treatment Outcome
PubMed: 28479053
DOI: 10.1016/j.jtcvs.2016.12.074 -
PloS One 2022To study the updated prevalence and clinical features of myasthenia gravis (MG) in Japan during 2017.
OBJECTIVE
To study the updated prevalence and clinical features of myasthenia gravis (MG) in Japan during 2017.
METHODS
We sent survey sheets to the randomly selected medical departments (number = 7,545). First, we asked the number of MG patients who visited medical departments from January 1, 2017, to December 31, 2017. Then, we sent the second survey sheet to the medical departments that answered the first survey to obtain the clinical information of patients who received MG diagnosis between January 1, 2015, and December 31, 2017.
RESULTS
The received answer to the first survey were 2,708 (recovery rate: 35.9%). After all, the prevalence of the 100,000 population was estimated as 23.1 (95%CI: 20.5-25.6). As a result of the second survey, we obtained 1,464 case records. After checking the duplications and lacking data, we utilized 1,195 data for further analysis. The median [interquartile range (IQR)] from the onset age of total patients was 59 (43-70) years old. The male-female ratio was 1: 1.15. The onset age [median (IQR)] for female patients was 58 (40-72) years old, and that for male patients was 60 (49-69) years old (Wilcoxon-Mann-Whitney test, p = 0.0299). We divided patients into four categories: 1) anti-acetylcholine receptor antibody (AChRAb) (+) thymoma (Tm) (-), 2) AChRAb(+)Tm(+), 3) anti-muscle-specific kinase antibody (MuSKAb) (+), and AChRAb(-)MuSKAb(-) (double negative; DN). The onset age [median (IQR)] of AChRAb(+)Tm(-) was 64 (48-73) years old, and AChRb(+)Tm(+) was 55 (45-66), MuSKAb(+) was 49 (36-64), DN was 47 (35-60) year old. The multivariate logistic regression analysis using sex, initial symptoms, repetitive nerve stimulation test (RNST), and edrophonium test revealed that sex, ocular symptoms, bulbar symptoms, and RNST were factors to distinguish each category. The myasthenia gravis activities of daily living profile at the severest state were significantly higher in MuSKAb(+). MuSKAb(+) frequently received prednisolone, tacrolimus plasmapheresis, and intravenous immunoglobulin; however, they received less acetylcholine esterase inhibitor. 99.2% of AChRAb(+)Tm(+) and 15.4% of AChRAb(+)Tm(-) received thymectomy. MuSKAb(+) did not receive thymectomy, and only 5.7% of DN received thymectomy. The prognosis was favorable in all categories.
CONCLUSION
Our result revealed that the prevalence of Japanese MG doubled from the previous study using the same survey method in 2006. We also found that the onset age shifted to the elderly, and the male-female ratio reached almost even. Classification in four categories; AChRAb(+)Tm(-), AChRAb(+)Tm(+), MuSKAb(+), and DN, well describe the specific clinical features of each category and differences in therapeutic approaches.
Topics: Activities of Daily Living; Adult; Aged; Autoantibodies; Edrophonium; Esterases; Female; Humans; Immunoglobulins, Intravenous; Japan; Male; Middle Aged; Myasthenia Gravis; Prednisolone; Surveys and Questionnaires; Tacrolimus; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 36129914
DOI: 10.1371/journal.pone.0274161 -
Thoracic Surgery Clinics May 2019Kido and colleagues in 1999 used for the first time the subxiphoid approach. Recently, video-assisted thoracoscopic surgery (VATS) thymectomy has been improved for the... (Review)
Review
Kido and colleagues in 1999 used for the first time the subxiphoid approach. Recently, video-assisted thoracoscopic surgery (VATS) thymectomy has been improved for the advancement of surgical materials and new instruments. The most important aspect regarding the subxiphoid approach is the possibility to avoid the intercostal nerve damage with the consequence of a decreased use of postoperative analgesics, quite short surgical duration, fast discharge from hospital and a guarantee of successful cosmetics results.
Topics: Humans; Myasthenia Gravis; Patient Positioning; Preoperative Care; Thoracic Surgery, Video-Assisted; Thymectomy
PubMed: 30928001
DOI: 10.1016/j.thorsurg.2018.12.010 -
The Journal of Thoracic and... Jul 2020
Topics: Humans; Neoplasm Recurrence, Local; Paraneoplastic Syndromes; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 32067787
DOI: 10.1016/j.jtcvs.2020.01.019 -
Annals of Thoracic and Cardiovascular... 2016Robotic surgeries have developed in the general thoracic field over the past decade, and publications on robotic surgery outcomes have accumulated. However, controversy... (Review)
Review
Robotic surgeries have developed in the general thoracic field over the past decade, and publications on robotic surgery outcomes have accumulated. However, controversy remains about the application of robotic surgery, with a lack of well-established evidence. Robotic surgery has several advantages such as natural movement of the surgeon's hands when manipulating the robotic arms and instruments controlled by computer-assisted systems. Most studies have reported the feasibility and safety of robotic surgery based on acceptable morbidity and mortality compared to open or video-assisted thoracic surgery (VATS). Furthermore, there are accumulated data to indicate longer operation times and shorter hospital stay in robotic surgery. However, randomized controlled trials between robotic and open or VATS procedures are needed to clarify the advantage of robotic surgery. In this review, we focused the literature about robotic surgery used to treat lung cancer and mediastinal tumor.
Topics: Clinical Competence; Humans; Learning Curve; Length of Stay; Lung Neoplasms; Mediastinal Neoplasms; Myasthenia Gravis; Operative Time; Postoperative Complications; Risk Factors; Robotic Surgical Procedures; Thoracic Surgery, Video-Assisted; Thymectomy; Time Factors; Treatment Outcome
PubMed: 26822625
DOI: 10.5761/atcs.ra.15-00344