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Short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis

BACKGROUND AND PURPOSE: This study was undertaken to investigate short‐ and long‐term outcome following thymectomy in patients with acetylcholine receptor antibody (AChR‐Ab)‐positive myasthenia gravis (MG). METHODS: Rates of clinical response (defined as minimal manifestation, pharmacological remiss...

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Autores principales: Rath, Jakob, Taborsky, Manuela, Moser, Bernhard, Zulehner, Gudrun, Weng, Rosa, Krenn, Martin, Cetin, Hakan, Matilla, José Ramon, Müllauer, Leonhard, Zimprich, Fritz
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9541265/
https://www.ncbi.nlm.nih.gov/pubmed/35435305
http://dx.doi.org/10.1111/ene.15362
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author Rath, Jakob
Taborsky, Manuela
Moser, Bernhard
Zulehner, Gudrun
Weng, Rosa
Krenn, Martin
Cetin, Hakan
Matilla, José Ramon
Müllauer, Leonhard
Zimprich, Fritz
author_facet Rath, Jakob
Taborsky, Manuela
Moser, Bernhard
Zulehner, Gudrun
Weng, Rosa
Krenn, Martin
Cetin, Hakan
Matilla, José Ramon
Müllauer, Leonhard
Zimprich, Fritz
author_sort Rath, Jakob
collection PubMed
description BACKGROUND AND PURPOSE: This study was undertaken to investigate short‐ and long‐term outcome following thymectomy in patients with acetylcholine receptor antibody (AChR‐Ab)‐positive myasthenia gravis (MG). METHODS: Rates of clinical response (defined as minimal manifestation, pharmacological remission, or complete stable remission) lasting for at least 1 year were retrospectively analyzed using Cox proportional hazard models. The occurrence of relapses was recorded during follow‐up. Clinical factors associated with achieving an initial or a sustained response were analyzed. RESULTS: Ninety‐four patients with a median age of 33 years (interquartile range [IQR] = 22–51), 68% with nonthymomatous MG and 32% with thymoma‐associated MG, were included. An initial clinical response was reached in 72% (68/94). Neither sex, age at onset, thymus histology, delay to surgery after disease onset, surgical approach, corticosteroid treatment, nor clinical severity before thymectomy was significantly associated with achieving this endpoint. During long‐term follow‐up (median = 89.5 months, IQR = 46–189.5), only half of the patients with an initial response (34/68) had a sustained response without relapses. No clinical factors predicted whether the response would become sustained. In patients without immunosuppressive treatment before thymectomy (n = 24), a high AChR‐Ab reduction rate after thymectomy was associated with a higher likelihood of achieving an initial response (p = 0.03). CONCLUSIONS: Sustained long‐term clinical response of MG patients after thymectomy is significantly lower than the initial response rates would suggest. The observation that none of the evaluated clinical factors was associated with a worse outcome supports the current clinical practice of patient selection for thymectomy. The relative decline of AChR‐Abs after surgery appears to be a promising prognostic marker.
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spelling pubmed-95412652022-10-14 Short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis Rath, Jakob Taborsky, Manuela Moser, Bernhard Zulehner, Gudrun Weng, Rosa Krenn, Martin Cetin, Hakan Matilla, José Ramon Müllauer, Leonhard Zimprich, Fritz Eur J Neurol Muscle and NMJ disorders BACKGROUND AND PURPOSE: This study was undertaken to investigate short‐ and long‐term outcome following thymectomy in patients with acetylcholine receptor antibody (AChR‐Ab)‐positive myasthenia gravis (MG). METHODS: Rates of clinical response (defined as minimal manifestation, pharmacological remission, or complete stable remission) lasting for at least 1 year were retrospectively analyzed using Cox proportional hazard models. The occurrence of relapses was recorded during follow‐up. Clinical factors associated with achieving an initial or a sustained response were analyzed. RESULTS: Ninety‐four patients with a median age of 33 years (interquartile range [IQR] = 22–51), 68% with nonthymomatous MG and 32% with thymoma‐associated MG, were included. An initial clinical response was reached in 72% (68/94). Neither sex, age at onset, thymus histology, delay to surgery after disease onset, surgical approach, corticosteroid treatment, nor clinical severity before thymectomy was significantly associated with achieving this endpoint. During long‐term follow‐up (median = 89.5 months, IQR = 46–189.5), only half of the patients with an initial response (34/68) had a sustained response without relapses. No clinical factors predicted whether the response would become sustained. In patients without immunosuppressive treatment before thymectomy (n = 24), a high AChR‐Ab reduction rate after thymectomy was associated with a higher likelihood of achieving an initial response (p = 0.03). CONCLUSIONS: Sustained long‐term clinical response of MG patients after thymectomy is significantly lower than the initial response rates would suggest. The observation that none of the evaluated clinical factors was associated with a worse outcome supports the current clinical practice of patient selection for thymectomy. The relative decline of AChR‐Abs after surgery appears to be a promising prognostic marker. John Wiley and Sons Inc. 2022-04-30 2022-08 /pmc/articles/PMC9541265/ /pubmed/35435305 http://dx.doi.org/10.1111/ene.15362 Text en © 2022 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Muscle and NMJ disorders
Rath, Jakob
Taborsky, Manuela
Moser, Bernhard
Zulehner, Gudrun
Weng, Rosa
Krenn, Martin
Cetin, Hakan
Matilla, José Ramon
Müllauer, Leonhard
Zimprich, Fritz
Short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis
title Short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis
title_full Short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis
title_fullStr Short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis
title_full_unstemmed Short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis
title_short Short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis
title_sort short‐term and sustained clinical response following thymectomy in patients with myasthenia gravis
topic Muscle and NMJ disorders
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9541265/
https://www.ncbi.nlm.nih.gov/pubmed/35435305
http://dx.doi.org/10.1111/ene.15362
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