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The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, and in over 80% of cases, antibodies are identified against the nicotinic type of acetylcholine receptor (AChR) on the muscle endplate. Despite the availability of various treatment options, patients wi...

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Autores principales: Ojha, Pawan T., Gaikwad, Abhijeet M., Singh, Rakesh, Jagiasi, Kamlesh, Nagendra, Shashank
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10645268/
https://www.ncbi.nlm.nih.gov/pubmed/37970279
http://dx.doi.org/10.4103/aian.aian_186_23
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author Ojha, Pawan T.
Gaikwad, Abhijeet M.
Singh, Rakesh
Jagiasi, Kamlesh
Nagendra, Shashank
author_facet Ojha, Pawan T.
Gaikwad, Abhijeet M.
Singh, Rakesh
Jagiasi, Kamlesh
Nagendra, Shashank
author_sort Ojha, Pawan T.
collection PubMed
description BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, and in over 80% of cases, antibodies are identified against the nicotinic type of acetylcholine receptor (AChR) on the muscle endplate. Despite the availability of various treatment options, patients with MG experience relapses and remission during the course of the disease. Aims and Objective: To understand the clinical profile, predictors of outcomes in acetyl choline receptor (AChR) antibody positive generalized MG. METHODS: This is a retrospective, single-centre, observational study of 108 patients with AChR positive generalized MG. We collected data on clinical and demographical profiles, treatments received, and treatment responses from those who fulfilled inclusion criteria over a mean follow up period of 33.75 ±7.30 months. Clinical outcomes were studied in terms of the type of remission and crisis or disease exacerbations patients had, considering different variables and treatment received. RESULTS: We found the commonest initial symptoms were ocular or oculo-bulbar, which progressed to generalized MG in the first year of disease onset. 36 (33.3%) patients experienced a crisis requiring mechanical ventilation within a mean period of 9.4 ±4.77 months from the disease onset. Multivariate regression analysis showed late-onset MG (age of onset between 50-70 years) and treatment with rituximab were better correlated with remission, (odd ratio of 4.7; 95 % CI ,1.12 -12.6; P value < 0.05 and odd ratio of 4.56; 95 % CI ,1.2 -10.04; P value < 0.05) respectively. While treatment with Mycofenolate Mofetile (MMF) was associated with a higher number of relapses (odd ratio of 1.8; 95 % CI ,0.08 -0.96; P value < 0.05). Treatment with Rituximab showed a higher rate of remission as compared to treatment refractory (TR) on conventional immunosuppressant therapy (IST). Out of 35(32 %) thymoma patients, 21 patients underwent thymectomy and these patients showed significantly greater rate of remission as compared both thymoma patients who denied thymectomy as a treatment option (N = 10 ;55.60 % vs N = 4; 23.50%). CONCLUSION: In this study of AChR antibody positive generalized MG patients, we found that nearly one-third of them experienced myasthenic crisis despite receiving the best medical care. Rituximab appeared to be effective in the treatment of refractory MG and those who failed thymectomy. Thymectomy was associated with better outcomes in patients, both with or without a thymoma.
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spelling pubmed-106452682023-11-15 The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study Ojha, Pawan T. Gaikwad, Abhijeet M. Singh, Rakesh Jagiasi, Kamlesh Nagendra, Shashank Ann Indian Acad Neurol Original Article BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, and in over 80% of cases, antibodies are identified against the nicotinic type of acetylcholine receptor (AChR) on the muscle endplate. Despite the availability of various treatment options, patients with MG experience relapses and remission during the course of the disease. Aims and Objective: To understand the clinical profile, predictors of outcomes in acetyl choline receptor (AChR) antibody positive generalized MG. METHODS: This is a retrospective, single-centre, observational study of 108 patients with AChR positive generalized MG. We collected data on clinical and demographical profiles, treatments received, and treatment responses from those who fulfilled inclusion criteria over a mean follow up period of 33.75 ±7.30 months. Clinical outcomes were studied in terms of the type of remission and crisis or disease exacerbations patients had, considering different variables and treatment received. RESULTS: We found the commonest initial symptoms were ocular or oculo-bulbar, which progressed to generalized MG in the first year of disease onset. 36 (33.3%) patients experienced a crisis requiring mechanical ventilation within a mean period of 9.4 ±4.77 months from the disease onset. Multivariate regression analysis showed late-onset MG (age of onset between 50-70 years) and treatment with rituximab were better correlated with remission, (odd ratio of 4.7; 95 % CI ,1.12 -12.6; P value < 0.05 and odd ratio of 4.56; 95 % CI ,1.2 -10.04; P value < 0.05) respectively. While treatment with Mycofenolate Mofetile (MMF) was associated with a higher number of relapses (odd ratio of 1.8; 95 % CI ,0.08 -0.96; P value < 0.05). Treatment with Rituximab showed a higher rate of remission as compared to treatment refractory (TR) on conventional immunosuppressant therapy (IST). Out of 35(32 %) thymoma patients, 21 patients underwent thymectomy and these patients showed significantly greater rate of remission as compared both thymoma patients who denied thymectomy as a treatment option (N = 10 ;55.60 % vs N = 4; 23.50%). CONCLUSION: In this study of AChR antibody positive generalized MG patients, we found that nearly one-third of them experienced myasthenic crisis despite receiving the best medical care. Rituximab appeared to be effective in the treatment of refractory MG and those who failed thymectomy. Thymectomy was associated with better outcomes in patients, both with or without a thymoma. Wolters Kluwer - Medknow 2023 2023-08-25 /pmc/articles/PMC10645268/ /pubmed/37970279 http://dx.doi.org/10.4103/aian.aian_186_23 Text en Copyright: © 2023 Annals of Indian Academy of Neurology https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Original Article
Ojha, Pawan T.
Gaikwad, Abhijeet M.
Singh, Rakesh
Jagiasi, Kamlesh
Nagendra, Shashank
The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study
title The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study
title_full The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study
title_fullStr The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study
title_full_unstemmed The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study
title_short The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study
title_sort clinical outcome in achr-positive generalized myasthenia gravis: a retrospective observational study
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10645268/
https://www.ncbi.nlm.nih.gov/pubmed/37970279
http://dx.doi.org/10.4103/aian.aian_186_23
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