The efficacy of eculizumab against post-thymectomy exacerbations in thymoma associated myasthenia gravis (MG)
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Abstract:
症例は胸腺腫を合併した重症筋無力症(myasthenia gravis,以下MGと略記)の62歳女性である.嚥下障害が著明で,副腎皮質ステロイド,免疫グロブリン大量静注療法(Intravenous immune globulin,以下IVIGと略記),免疫吸着療法(immunoadsorption plasmapheresis,以下IAPPと略記),免疫抑制剤により一旦改善したが,拡大胸腺摘出術後3週間で嚥下障害が再増悪した.IVIG,IAPPを行うも症状が残存し難渋したが,エクリズマブ開始後に症状は完全に消失し,MGの術後増悪に対して抗補体療法の有効性が示唆された.Keywords:
Thymectomy
Eculizumab
Plasmapheresis
Immunoadsorption
Thymoma is the most common neoplasm of the anterior mediastinum. This neoplasm is composed with neoplastic epithelial cells and non-neoplastic T lymphocytes with varying degrees. In the advanced stage of thymomas, recurrence is not uncommon, and treatment for recurrent lesions— especially disseminated lesions—is not easy. In addition, thymoma is often associated with autoimmune diseases. The representative disorder is myasthenia gravis (MG). Combined treatment for recurrence and complex autoimmune diseases is often difficult. The efficacy of thymectomy for early-onset MG without thymomas was demonstrated in a recent clinical trial, and it may be effective for MG patients associated with thymoma. However, thymectomy or thymomectomy usually does not affect other non-MG autoimmune diseases. Thymectomy has been performed via median sternotomy for many years, and thoracoscopic or mediastinoscopic thymectomy has been reported often. We established a subxiphoid approach to thoracoscopic thymectomy, and its usefulness and efficacy have been reported. In this section, the detailed procedures of thymectomy using this subxiphoid approach are introduced.
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Several cases of thymoma recurrence after resection have been reported. However, thymoma appearance following an extended thymectomy for non-thymomatous myasthenia gravis is very rare. We report a case of thymoma in a 48-year-old woman, 15 years after an extended thymectomy for non-thymomatous myasthenia gravis. The importance of a complete dissection of mediastinal adipose tissue during the extended thymectomy as well as careful follow-up for such patients is also noted.
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We herein report a case of myasthenia gravis (MG) in which thoracoscopic thymectomy was performed for a large thymic cystic lesion using a subxiphoid approach. We have previously suggested the usefulness of the subxiphoid approach in thymectomy. The indications of thoracoscopic thymectomy were recently expanded to include large thymic cystic lesions without intraoperative rupture of the lesions. The pathologic diagnosis of the lesion in the present case was multilocular thymic cyst with type A thymoma and micronodular thymoma. The postoperative complications were minimal without MG crisis. Thoracoscopic thymectomy using a subxiphoid approach seems to be an ideal procedure, even for large thymic cystic lesions. In addition, early-onset MG with a large thymic cystic lesion may suggest the presence of a small thymoma even if the lesion is not detected on a preoperative radiologic examination.
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Background: Thymoma-associated haematological diseases (HDs), such as pure red cell aplasia (PRCA) and Good's syndrome, are extremely rare, and due to the paucity of large-scale studies, the characteristics, remission after thymectomy, and long-term evaluation remain undetermined. Methods: We retrospectively assessed patients with thymoma and associated HDs from Jan 2005 to Dec 2020. All patients received thymectomy and/or additional treatments for HDs. A comparison with thymoma-associated myasthenic gravis (MG), and a systematic review from PubMed/MEDLINE and Embase were conducted. Results: In the median follow-up of 56 months, 130 patients were enrolled. Patients with thymoma-associated MG (n = 46) and HDs [n = 8; PRCA (n = 5), PRCA and Good's syndrome (n = 2) and autoimmune haemolytic anaemia (n = 1)] were evaluated. Patients with MG had a significantly higher remission rate after thymectomy (50 vs. 17%; p = 0.0378) as compared to those with other autoimmune diseases. Two of seven patients with PRCA experienced remission with thymectomy alone, and an additional two patients achieved remission with thymectomy plus immunosuppressive therapy (IST). In the systematic review, 60 studies (case reports, n = 46; case series including the present study, n = 14) were evaluated. Forty-four percent of patients were diagnosed with PRCA after thymoma, and 61% achieved remission with thymectomy plus IST; however, Good's syndrome was unaffected. Conclusions: Our study indicates that patients with thymoma-associated autoimmune diseases other than MG have a lower remission rate than those with MG. Remission of thymoma-associated PRCA can be achieved by thymectomy and IST. This study provides insight into extremely rare but puzzling autoimmune manifestations.
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OBJECTIVE. The authors evaluated the role of antibodies to striated muscle and acetylcholine receptors in diagnostics of myasthenia gravis and thymoma, as well as outcomes of thymectomy and prognosis of myasthenia course. MATERIAL AND METHODS. The study investigated correlations of antibody content to striated muscles and acetylcholine receptors from the presence and size of thymoma, myasthenia in 157 patients with various pathologies of the thymus. The dynamics of antibody concentrations was followed up after thymectomy. RESULTS. Antibody titer to striated muscle depended on the presence and size of thymoma, severity of myasthenia and changed after thymectomy. Concentration of antibodies was associated with the presence of thymoma and it didn’t change after surgical treatment. Thymoma wasn’t revealed in patients who were seronegative to both antibodies. CONCLUSIONS. Seropositivity according to one of antibody could indicate the presence of thymoma, but its absence to both antibodies allowed doctors to eliminate this diagnosis. Antibodies to acetylcholine receptors are important markers of myasthenia. Monitoring of antibody titer dynamics to striated muscles after thymectomy could be useful for assessment of response to surgical treatment and prognosis of course of myasthenia.
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Objective To review video-assisted thoracoscopic thymectomy as a treatment for myasthenia gravis (MG),compare outcomes of thoracoscopic thymectomy for thymoma and non-thymoma MG,and assess the efficacy of Video-assisted Thoracoscopic Extended Thymectomy (VATET) combined with mediastinoscopy.Methods A retrospective review of 500 patientswith MG who underwent VATS thymectomy between 2001 and 2011 has been done.They were divided into three groups:118 cases of thymoma MG group,thoracoscopy for non-thymoma MG group 301cases,and VATET for non-thymoma MG group 81cases.Results There was no mortality.Thoracoscopic thymectomy was successfully performed for 495 cases.In the thoracoscopy group for non-thymoma MG,the operating time is (111.3 ± 31.6) min,11.0％ having post-operative myasthenic crises ; in the VATET group,the operating time is (145.0 ± 71.6) min,9.9％ having post-operative myasthenic crises ;in the thymoma MG group,the operating time is (128.5 ± 77.8)min,24.6％ having post-operative myasthenic crises.During the follow-up,CSR was 37.3％,36.5％ and 28.7％ in the groups of thoracoscopy for non-thymoma MG,VATET and thymoma MG respectively.However,the disease-free survival curve shows that CSR of the thymoma MG group became lower than other two groups 3 years after surgery,and CSR of the VATET group becoming higher than that of thoracoscopy for nou-thymoma MG group 5 years after surgery.CSRs of groups of thoracoscopy for non-thymoma MG,VATET and thymoma MG might reach 50％,60％ and 36％.Conclusion The VATET combined with mediastinoscopy has a better long-term outcome because the more thymus might be removed comparing with non-thymoma MG,thoracoscopic thymectomy for thymoma MG had a worse long-term outcome.
Key words:
Myasthenia gravis; Thymoma ; Surgical procedures, minimally invasive ; Thymectomy
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Thymectomy is required for the treatment of thymoma-associated myasthenia gravis (MG). However, MG may develop only after thymectomy, a condition known as post-thymectomy MG. This study aimed to investigate the risk factors for post-thymectomy MG in patients with thymoma.We retrospectively identified 235 patients with thymoma who underwent thymectomy at a single hospital from January 2008 to December 2017: 44 with preoperatively diagnosed MG were excluded, leaving 191 patients in the final analysis. Univariable survival analyses using Cox proportional hazards regression model and Kaplan-Meier estimate were conducted to identify risk factors for post-thymectomy MG.Post-thymectomy MG developed in 4.2% (8/191) of the patients with thymoma between 18 days and 108 mo after surgery. Hazard ratios (HRs) of pre- and postoperative anti-acetylcholine receptor antibody (AChR-Ab) titers were 2.267 (P = .002) and 1.506 (P < .001), respectively. Patients with extended thymectomy had a low chance of post-thymectomy MG (HR 0.035, P = .007). Larger thymoma (HR, 1.359; P = .005) and type A or AB thymoma according to World Health Organization histological classification (HR, 11.92; P = .021) were associated with higher chances of post-thymectomy MG. Within the subgroup of preoperatively AChR-Ab seropositive patients, post-thymectomy MG developed in 22.2% (6/27).Pre- and postoperative AChR-Ab levels should be measured in patients with thymoma. A large thymoma and partial thymectomy appear to be associated with a higher probability of post-thymectomy MG.
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