Early prediction of refractory myasthenia gravis based on response to treatment within the first year of diagnosis.

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Title: Early prediction of refractory myasthenia gravis based on response to treatment within the first year of diagnosis.
Authors: Han, Hee Jo (AUTHOR), Kim, Susung (AUTHOR), Park, Hyung Jun (AUTHOR), Shin, Ha Young (AUTHOR), Kim, Seung Woo (AUTHOR)
Source: Neurological Sciences. Aug2025, Vol. 46 Issue 8, p3919-3927. 9p.
Subjects: Myasthenia gravis, Neuromuscular diseases, Cholinergic receptors, Myoneural junction, Immunosuppressive agents
Abstract: Background: The long lead time required to diagnose refractory myasthenia gravis (MG) can prevent early attempts at new treatments. Herein, we aimed to predict refractory MG by comparing treatment responses during the early stages between patients with refractory and non-refractory MG. Methods: We retrospectively investigated the medical records of patients with acetylcholine receptor antibody-positive generalized MG. Refractory MG was defined as (1) inability to lower prednisolone below 20 mg/day, (2) requiring regular intravenous immunoglobulin or plasmapheresis, or (3) Myasthenia Gravis Activities of Daily Living score ≥ 6 after treatment with corticosteroids and ≥ 1 oral immunosuppressant for ≥ 12 months. Results: Of 133 patients, 29 (21.8%) and 104 (78.2%) had refractory and non-refractory MG, respectively. The median duration from the diagnosis of MG to that of refractory MG was 31 months. During the first year following diagnosis, the proportion of patients who achieved a lowest effective prednisolone dose of ≤ 20 mg was lower in the refractory group than in the non-refractory group (69.0% vs. 92.0%). Conversely, the proportions of patients who initiated oral immunosuppressive therapy (58.6% vs. 22.5%), required rescue interventions (48.3% vs. 22.1%), or experienced a myasthenic crisis (34.5% vs. 14.4%) were significantly higher in the refractory group. The risk of refractory MG was 18.7-fold higher in patients who failed to achieve lowest effective prednisolone dose under 20 mg/day and started oral immunosuppressant during the first year than those with neither of these conditions. Conclusions: The refractory group displayed distinct clinical features during the early disease stages, including frequent crises and higher levels of immunosuppressive treatment. These features can be used for early prediction of refractory MG. [ABSTRACT FROM AUTHOR]
Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: Early prediction of refractory myasthenia gravis based on response to treatment within the first year of diagnosis.
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  Data: <searchLink fieldCode="AR" term="%22Han%2C+Hee+Jo%22">Han, Hee Jo</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Kim%2C+Susung%22">Kim, Susung</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Park%2C+Hyung+Jun%22">Park, Hyung Jun</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Shin%2C+Ha+Young%22">Shin, Ha Young</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Kim%2C+Seung+Woo%22">Kim, Seung Woo</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22Neurological+Sciences%22">Neurological Sciences</searchLink>. Aug2025, Vol. 46 Issue 8, p3919-3927. 9p.
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  Data: <searchLink fieldCode="DE" term="%22Myasthenia+gravis%22">Myasthenia gravis</searchLink><br /><searchLink fieldCode="DE" term="%22Neuromuscular+diseases%22">Neuromuscular diseases</searchLink><br /><searchLink fieldCode="DE" term="%22Cholinergic+receptors%22">Cholinergic receptors</searchLink><br /><searchLink fieldCode="DE" term="%22Myoneural+junction%22">Myoneural junction</searchLink><br /><searchLink fieldCode="DE" term="%22Immunosuppressive+agents%22">Immunosuppressive agents</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background: The long lead time required to diagnose refractory myasthenia gravis (MG) can prevent early attempts at new treatments. Herein, we aimed to predict refractory MG by comparing treatment responses during the early stages between patients with refractory and non-refractory MG. Methods: We retrospectively investigated the medical records of patients with acetylcholine receptor antibody-positive generalized MG. Refractory MG was defined as (1) inability to lower prednisolone below 20 mg/day, (2) requiring regular intravenous immunoglobulin or plasmapheresis, or (3) Myasthenia Gravis Activities of Daily Living score ≥ 6 after treatment with corticosteroids and ≥ 1 oral immunosuppressant for ≥ 12 months. Results: Of 133 patients, 29 (21.8%) and 104 (78.2%) had refractory and non-refractory MG, respectively. The median duration from the diagnosis of MG to that of refractory MG was 31 months. During the first year following diagnosis, the proportion of patients who achieved a lowest effective prednisolone dose of ≤ 20 mg was lower in the refractory group than in the non-refractory group (69.0% vs. 92.0%). Conversely, the proportions of patients who initiated oral immunosuppressive therapy (58.6% vs. 22.5%), required rescue interventions (48.3% vs. 22.1%), or experienced a myasthenic crisis (34.5% vs. 14.4%) were significantly higher in the refractory group. The risk of refractory MG was 18.7-fold higher in patients who failed to achieve lowest effective prednisolone dose under 20 mg/day and started oral immunosuppressant during the first year than those with neither of these conditions. Conclusions: The refractory group displayed distinct clinical features during the early disease stages, including frequent crises and higher levels of immunosuppressive treatment. These features can be used for early prediction of refractory MG. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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              Text: Aug2025
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