Nairong Xie, Qing Liu, Qi Wen, Yaye Wang, Haoran Liu, Yuting Jiang, Yan Lu, Li Di, Min Wang, Wenjia Zhu, Xinmei Wen, Xuxiang Zhang, Xin-Ming Shen, Yuwei Da
{"title":"AChR-Ab阳性极晚发型重症肌无力患者的短期和长期预后","authors":"Nairong Xie, Qing Liu, Qi Wen, Yaye Wang, Haoran Liu, Yuting Jiang, Yan Lu, Li Di, Min Wang, Wenjia Zhu, Xinmei Wen, Xuxiang Zhang, Xin-Ming Shen, Yuwei Da","doi":"10.1177/17562864241309793","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Very-late-onset myasthenia gravis (VLOMG) refers to myasthenia gravis (MG) with onset at age 65 or older. Current research on VLOMG prognosis remains limited, especially regarding factors influencing outcomes.</p><p><strong>Objectives: </strong>To identify the clinical factors that affect the short- and long-term prognosis of MG patients with an onset age ⩾65 years.</p><p><strong>Design: </strong>This was a single-center, retrospective cohort study of AChR-ab positive VLOMG patients, classified into two subgroups based on age of onset: sub-very-late-onset MG (S-VLOMG, onset age ⩾65 and <75 years), and super-late-onset MG (SLOMG, onset age ⩾75 years).</p><p><strong>Methods: </strong>A total of 93 patients were included, including 75 in the S-VLOMG group and 18 in the SLOMG group. Clinical, therapeutic, and prognosis data were reviewed, and the Cox regression model was used to identify factors influencing short- and long-term prognosis.</p><p><strong>Results: </strong>Patient characteristics were well balanced between the groups. Overall, 49.5% of patients achieved minimal symptom expression (MSE) within 6 months and 86% within 24 months. There was no significant difference between the groups in the proportion achieving MSE at 6 months (<i>p</i> = 0.635) or 24 months (<i>p</i> = 0.714). The median time to achieve MSE was also comparable between the S-VLOMG and SLOMG groups (199.0 days vs 280.5 days, <i>p</i> = 0.463). Low baseline MG-ADL score and steroid therapy were associated with better short-term prognosis (<i>p</i> = 0.007 and <i>p</i> = 0.002, respectively). For long-term prognosis, baseline bulbar and limb involvement, time to treatment initiation, and use of immunosuppressants were significant factors (<i>p</i> = 0.025, <i>p</i> = 0.004, <i>p</i> = 0.025, and <i>p</i> < 0.0001, respectively). There were no significant differences in side effects or drug withdrawal rates between two groups.</p><p><strong>Conclusion: </strong>This study demonstrated that AChR-ab positive VLOMG patients have a favorable prognosis and responded well to medication, with age and comorbidities showing no significant impact.</p>","PeriodicalId":22980,"journal":{"name":"Therapeutic Advances in Neurological Disorders","volume":"18 ","pages":"17562864241309793"},"PeriodicalIF":4.7000,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11713957/pdf/","citationCount":"0","resultStr":"{\"title\":\"Short-term and long-term prognoses in AChR-Ab positive very-late-onset myasthenia gravis patients.\",\"authors\":\"Nairong Xie, Qing Liu, Qi Wen, Yaye Wang, Haoran Liu, Yuting Jiang, Yan Lu, Li Di, Min Wang, Wenjia Zhu, Xinmei Wen, Xuxiang Zhang, Xin-Ming Shen, Yuwei Da\",\"doi\":\"10.1177/17562864241309793\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Very-late-onset myasthenia gravis (VLOMG) refers to myasthenia gravis (MG) with onset at age 65 or older. Current research on VLOMG prognosis remains limited, especially regarding factors influencing outcomes.</p><p><strong>Objectives: </strong>To identify the clinical factors that affect the short- and long-term prognosis of MG patients with an onset age ⩾65 years.</p><p><strong>Design: </strong>This was a single-center, retrospective cohort study of AChR-ab positive VLOMG patients, classified into two subgroups based on age of onset: sub-very-late-onset MG (S-VLOMG, onset age ⩾65 and <75 years), and super-late-onset MG (SLOMG, onset age ⩾75 years).</p><p><strong>Methods: </strong>A total of 93 patients were included, including 75 in the S-VLOMG group and 18 in the SLOMG group. Clinical, therapeutic, and prognosis data were reviewed, and the Cox regression model was used to identify factors influencing short- and long-term prognosis.</p><p><strong>Results: </strong>Patient characteristics were well balanced between the groups. Overall, 49.5% of patients achieved minimal symptom expression (MSE) within 6 months and 86% within 24 months. There was no significant difference between the groups in the proportion achieving MSE at 6 months (<i>p</i> = 0.635) or 24 months (<i>p</i> = 0.714). The median time to achieve MSE was also comparable between the S-VLOMG and SLOMG groups (199.0 days vs 280.5 days, <i>p</i> = 0.463). Low baseline MG-ADL score and steroid therapy were associated with better short-term prognosis (<i>p</i> = 0.007 and <i>p</i> = 0.002, respectively). For long-term prognosis, baseline bulbar and limb involvement, time to treatment initiation, and use of immunosuppressants were significant factors (<i>p</i> = 0.025, <i>p</i> = 0.004, <i>p</i> = 0.025, and <i>p</i> < 0.0001, respectively). There were no significant differences in side effects or drug withdrawal rates between two groups.</p><p><strong>Conclusion: </strong>This study demonstrated that AChR-ab positive VLOMG patients have a favorable prognosis and responded well to medication, with age and comorbidities showing no significant impact.</p>\",\"PeriodicalId\":22980,\"journal\":{\"name\":\"Therapeutic Advances in Neurological Disorders\",\"volume\":\"18 \",\"pages\":\"17562864241309793\"},\"PeriodicalIF\":4.7000,\"publicationDate\":\"2025-01-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11713957/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Therapeutic Advances in Neurological Disorders\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1177/17562864241309793\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Therapeutic Advances in Neurological Disorders","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1177/17562864241309793","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Short-term and long-term prognoses in AChR-Ab positive very-late-onset myasthenia gravis patients.
Background: Very-late-onset myasthenia gravis (VLOMG) refers to myasthenia gravis (MG) with onset at age 65 or older. Current research on VLOMG prognosis remains limited, especially regarding factors influencing outcomes.
Objectives: To identify the clinical factors that affect the short- and long-term prognosis of MG patients with an onset age ⩾65 years.
Design: This was a single-center, retrospective cohort study of AChR-ab positive VLOMG patients, classified into two subgroups based on age of onset: sub-very-late-onset MG (S-VLOMG, onset age ⩾65 and <75 years), and super-late-onset MG (SLOMG, onset age ⩾75 years).
Methods: A total of 93 patients were included, including 75 in the S-VLOMG group and 18 in the SLOMG group. Clinical, therapeutic, and prognosis data were reviewed, and the Cox regression model was used to identify factors influencing short- and long-term prognosis.
Results: Patient characteristics were well balanced between the groups. Overall, 49.5% of patients achieved minimal symptom expression (MSE) within 6 months and 86% within 24 months. There was no significant difference between the groups in the proportion achieving MSE at 6 months (p = 0.635) or 24 months (p = 0.714). The median time to achieve MSE was also comparable between the S-VLOMG and SLOMG groups (199.0 days vs 280.5 days, p = 0.463). Low baseline MG-ADL score and steroid therapy were associated with better short-term prognosis (p = 0.007 and p = 0.002, respectively). For long-term prognosis, baseline bulbar and limb involvement, time to treatment initiation, and use of immunosuppressants were significant factors (p = 0.025, p = 0.004, p = 0.025, and p < 0.0001, respectively). There were no significant differences in side effects or drug withdrawal rates between two groups.
Conclusion: This study demonstrated that AChR-ab positive VLOMG patients have a favorable prognosis and responded well to medication, with age and comorbidities showing no significant impact.
期刊介绍:
Therapeutic Advances in Neurological Disorders is a peer-reviewed, open access journal delivering the highest quality articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of neurology. The journal has a strong clinical and pharmacological focus and is aimed at clinicians and researchers in neurology, providing a forum in print and online for publishing the highest quality articles in this area.