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The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation
BACKGROUND: Myasthenia gravis (MG) is a rare neuromuscular disorder. Symptoms can range from ptosis only to life threatening myasthenic crisis. Thymectomy is recommended for anti-acetylcholine receptor-antibody positive patients with early-onset MG. Here, we investigated prognostic factors shaping t...
Autores principales: | , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10268481/ https://www.ncbi.nlm.nih.gov/pubmed/37316910 http://dx.doi.org/10.1186/s42466-023-00252-w |
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author | Menghesha, Hruy Schroeter, Michael Nelke, Christopher Ruck, Tobias Schlachtenberger, Georg Welskop, Clara Camo, Amina Heldwein, Matthias Bennink, Gerardus Wahlers, Thorsten Bölükbas, Servet Doerr, Fabian Hekmat, Khosro |
author_facet | Menghesha, Hruy Schroeter, Michael Nelke, Christopher Ruck, Tobias Schlachtenberger, Georg Welskop, Clara Camo, Amina Heldwein, Matthias Bennink, Gerardus Wahlers, Thorsten Bölükbas, Servet Doerr, Fabian Hekmat, Khosro |
author_sort | Menghesha, Hruy |
collection | PubMed |
description | BACKGROUND: Myasthenia gravis (MG) is a rare neuromuscular disorder. Symptoms can range from ptosis only to life threatening myasthenic crisis. Thymectomy is recommended for anti-acetylcholine receptor-antibody positive patients with early-onset MG. Here, we investigated prognostic factors shaping therapeutic outcomes of thymectomy to improve patient stratification. METHODS: We retrospectively collected single-center data from a specialized center for MG from all consecutive adult patients that underwent thymectomy from 01/2012 to 12/2020. We selected patients with thymoma-associated and non-thymomatous MG for further investigations. We analyzed the patient collective regarding perioperative parameters in relation to the surgical approach. Furthermore, we investigated the dynamics of the anti-acetylcholine receptor-antibody titers and concurrent immunosuppressive therapies, as well as the therapeutic outcomes in dependence of clinical classifications. RESULTS: Of 137 patients 94 were included for further analysis. We used a minimally invasive approach in 73 patients, whereas 21 patients underwent sternotomy. A total of 45 patients were classified as early-onset MG (EOMG), 28 as late-onset MG (LOMG) and 21 as thymoma-associated MG (TAMG). The groups differed in terms of age at diagnosis (EOMG: 31.1 ± 12.2 years; LOMG: 59.8 ± 13.7 years; TAMG: 58.6 ± 16.7 years; p < 0.001). Patients with EOMG and TAMG were more often female than patients in the LOMG group (EOMG: 75.6%; LOMG: 42.9%; TAMG: 61.9%; p = 0.018). There were no significant differences in outcome scores (quantitative MG; MG activities of daily living; MG Quality of Live) with a median follow-up of 46 months. However, Complete Stable Remission was achieved significantly more frequently in the EOMG group than in the other two groups (p = 0.031). At the same time, symptoms seem to improve similarly in all three groups (p = 0.25). CONCLUSION: Our study confirms the benefit of thymectomy in the therapy of MG. Both, the concentration of acetylcholine receptor antibodies and the necessary dosage of cortisone therapy show a continuous regression after thymectomy in the overall cohort. Beyond EOMG, groups of LOMG and thymomatous MG responded to thymectomy as well, but therapy success was less pronounced and delayed compared to the EOMG subgroup. Thymectomy is a mainstay of MG therapy to be considered in all subgroups of MG patients investigated. |
format | Online Article Text |
id | pubmed-10268481 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-102684812023-06-15 The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation Menghesha, Hruy Schroeter, Michael Nelke, Christopher Ruck, Tobias Schlachtenberger, Georg Welskop, Clara Camo, Amina Heldwein, Matthias Bennink, Gerardus Wahlers, Thorsten Bölükbas, Servet Doerr, Fabian Hekmat, Khosro Neurol Res Pract Research Article BACKGROUND: Myasthenia gravis (MG) is a rare neuromuscular disorder. Symptoms can range from ptosis only to life threatening myasthenic crisis. Thymectomy is recommended for anti-acetylcholine receptor-antibody positive patients with early-onset MG. Here, we investigated prognostic factors shaping therapeutic outcomes of thymectomy to improve patient stratification. METHODS: We retrospectively collected single-center data from a specialized center for MG from all consecutive adult patients that underwent thymectomy from 01/2012 to 12/2020. We selected patients with thymoma-associated and non-thymomatous MG for further investigations. We analyzed the patient collective regarding perioperative parameters in relation to the surgical approach. Furthermore, we investigated the dynamics of the anti-acetylcholine receptor-antibody titers and concurrent immunosuppressive therapies, as well as the therapeutic outcomes in dependence of clinical classifications. RESULTS: Of 137 patients 94 were included for further analysis. We used a minimally invasive approach in 73 patients, whereas 21 patients underwent sternotomy. A total of 45 patients were classified as early-onset MG (EOMG), 28 as late-onset MG (LOMG) and 21 as thymoma-associated MG (TAMG). The groups differed in terms of age at diagnosis (EOMG: 31.1 ± 12.2 years; LOMG: 59.8 ± 13.7 years; TAMG: 58.6 ± 16.7 years; p < 0.001). Patients with EOMG and TAMG were more often female than patients in the LOMG group (EOMG: 75.6%; LOMG: 42.9%; TAMG: 61.9%; p = 0.018). There were no significant differences in outcome scores (quantitative MG; MG activities of daily living; MG Quality of Live) with a median follow-up of 46 months. However, Complete Stable Remission was achieved significantly more frequently in the EOMG group than in the other two groups (p = 0.031). At the same time, symptoms seem to improve similarly in all three groups (p = 0.25). CONCLUSION: Our study confirms the benefit of thymectomy in the therapy of MG. Both, the concentration of acetylcholine receptor antibodies and the necessary dosage of cortisone therapy show a continuous regression after thymectomy in the overall cohort. Beyond EOMG, groups of LOMG and thymomatous MG responded to thymectomy as well, but therapy success was less pronounced and delayed compared to the EOMG subgroup. Thymectomy is a mainstay of MG therapy to be considered in all subgroups of MG patients investigated. BioMed Central 2023-06-15 /pmc/articles/PMC10268481/ /pubmed/37316910 http://dx.doi.org/10.1186/s42466-023-00252-w Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Research Article Menghesha, Hruy Schroeter, Michael Nelke, Christopher Ruck, Tobias Schlachtenberger, Georg Welskop, Clara Camo, Amina Heldwein, Matthias Bennink, Gerardus Wahlers, Thorsten Bölükbas, Servet Doerr, Fabian Hekmat, Khosro The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation |
title | The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation |
title_full | The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation |
title_fullStr | The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation |
title_full_unstemmed | The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation |
title_short | The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation |
title_sort | impact of thymectomy in subgroups of myasthenia gravis patients: a single center longitudinal observation |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10268481/ https://www.ncbi.nlm.nih.gov/pubmed/37316910 http://dx.doi.org/10.1186/s42466-023-00252-w |
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