Thymectomy for non-thymomatous myasthenia gravis

Cea, Gabriel; Benatar, Michael; Verdugo, Renato J.; Salinas, Rodrigo

doi:10.1002/14651858.cd008111.pub2

Cited by 41 publications

(21 citation statements)

References 71 publications

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“…6,7 A systematic review 8 of articles describing outcomes in 21 cohorts of patients with myasthenia gravis pointed out numerous methodologic flaws that prevented definite conclusions to be drawn regarding the benefits of thymectomy in patients with nonthymomatous myasthenia gravis.…”

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confidence: 99%

Randomized Trial of Thymectomy in Myasthenia Gravis

et al. 2016

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BACKGROUND Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone. METHODS We compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period. RESULTS A total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, P<0.001); patients in the thymectomy group also had a lower average requirement for alternate-day prednisone (44 mg vs. 60 mg, P<0.001). Fewer patients in the thymectomy group than in the prednisone-only group required immunosuppression with azathioprine (17% vs. 48%, P<0.001) or were hospitalized for exacerbations (9% vs. 37%, P<0.001). The number of patients with treatment-associated complications did not differ significantly between groups (P=0.73), but patients in the thymectomy group had fewer treatment-associated symptoms related to immunosuppressive medications (P<0.001) and lower distress levels related to symptoms (P=0.003). CONCLUSIONS Thymectomy improved clinical outcomes over a 3-year period in patients with nonthymomatous myasthenia gravis. (Funded by the National Institute of Neurological Disorders and Stroke and others; MGTX ClinicalTrials.gov number, NCT00294658.)

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Randomized Trial of Thymectomy in Myasthenia Gravis

et al. 2016

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“…The role of thymectomy in the treatment of AChR‐MG, though still based on retrospective studies (the results of the first randomized trial are not yet available), is supported by a wealth of research data pointing to the relevance of the thymus in the initiation and perpetuation of autoantibody response . In MuSK‐MG, the evidence of a pathogenic link with the thymus is scarce.…”

Section: Diseasesmentioning

confidence: 99%

Characteristics of myasthenia gravis with antibodies to muscle‐specific kinase and low‐density lipoprotein‐related receptor protein 4

Evoli

Iorio

2015

Clinical & Exp Neuroim

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Myasthenia gravis (MG) is characterized by fatigable weakness of voluntary muscles caused by immunoglobulin G autoantibodies binding to post‐synaptic proteins at the neuromuscular junction. Antibodies specific for the acetylcholine receptor are detected in the great majority (85%) of MG patients. Serum immunoglobulin G binding to the muscle‐specific kinase (MuSK) are found in approximately 40% of anti‐acetylcholine receptor‐negative patients. Recently, the low‐density lipoprotein receptor‐related protein 4 (Lrp4), which is the MuSK co‐receptor for agrin, has been recognized as a new antigen in MG. Specific antibodies bind to the extracellular domains of MuSK and Lrp4 and, as experimental models have shown, interfere with the protein function. The present review describes the MuSK‐Lrp4 complex, and summarizes the effects of MuSK and Lrp4 antibodies at the neuromuscular junction. Furthermore, the epidemiological and clinical characteristics, the response to therapy and the controversies in management of the specific diseases are discussed.

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“…Thymectomy has become an indispensable option in the multidisciplinary management of MG since Blalock et al first reported a complete remission of MG after thymectomy about 80 years ago (4). Since then, thymectomy has gained increasing acceptance and many approaches have been described (5,6). As the latest advance of minimally invasive thymectomy, robotic thymectomy has been performed in patients with MG since 2003 (7).…”

Section: Introductionmentioning

confidence: 99%

Results of Robotic Thymectomy Performed in Myasthenia Gravis Patients Older Than 60 Years at Onset

Takahashi

Bauer

et al. 2019

The Annals of Thoracic Surgery

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Background Data are limited on the safety and efficacy of robotic thymectomy in myasthenia gravis (MG) patients older than 60 years at onset. Methods Patients older than 60 years at MG onset who underwent robotic thymectomy in Charite Universitaetsmedizin Berlin between 2003 and 2017 were potentially eligible for inclusion. The main outcomes were perioperative complications and clinical outcome according to the MG Foundation of America Post-Intervention Status (MGFA-PIS). Results Sixty-eight (25 females, 43 males) of 580 MG patients who underwent robotic thymectomy were eligible for perioperative analyses (median age at MG onset 67 years [range 61-85]). The perioperative morbidity rate was 13.2% and the only perioperative mortality was due to aortic dissection. Fifty-one patients were available for further analysis with a median follow-up time of 60 months (range 12-263). The complete stable remission (CSR) rate was 7.8%, the improvement rate was 68.6% and the overall mortality rate was 11.8%. Compared with preoperative use, the average daily dose of corticosteroids was significantly reduced at the last follow-up (17.6±23.6 VS 2.6±6.1 mg, P=0.0001) without increased use of azathioprine (35.9±61.9 VS 42.7±59 mg, P=0.427). After excluding two patients seronegative for the antiacetylcholine receptor (AChR) antibody, 10 out of 49 seropositive patients achieved "good outcome" (including four CSR, three pharmacologic remission and three minimal manifestations 0) which was predicted by being free of concomitant disease (OR: 7.307, 95% CI (1.188-44.937), P=0.032) and MGFA classification I prethymectomy (OR: 6.696, 95% CI (1.259-35.620), P=0.026). Conclusion Robotic thymectomy seems to be safe and effective in MG patients older than 60 years at onset with a significant steroid-sparing effect.

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Thymectomy for non-thymomatous myasthenia gravis

Cited by 41 publications

References 71 publications

Randomized Trial of Thymectomy in Myasthenia Gravis

Randomized Trial of Thymectomy in Myasthenia Gravis

Characteristics of myasthenia gravis with antibodies to muscle‐specific kinase and low‐density lipoprotein‐related receptor protein 4

Results of Robotic Thymectomy Performed in Myasthenia Gravis Patients Older Than 60 Years at Onset

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