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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
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.
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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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