Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China
IntroductionAntibodies to MuSK identify a rare subtype of myasthenia gravis (MuSK-MG). In western countries, the onset age of MuSK-MG peaks in the late 30's while it is unknown in Chinese population.MethodsIn this retrospective multicenter study, we screened 69 MuSK-MG patients from 2042 MG pat...
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Frontiers Media S.A.
2022-04-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2022.879261/full |
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| author | Yufan Zhou Yufan Zhou Yufan Zhou Jialin Chen Zunbo Li Song Tan Song Tan Chong Yan Chong Yan Chong Yan Sushan Luo Sushan Luo Sushan Luo Lei Zhou Lei Zhou Lei Zhou Jie Song Jie Song Jie Song Xiao Huan Xiao Huan Xiao Huan Ying Wang Chongbo Zhao Chongbo Zhao Chongbo Zhao Wenshuang Zeng Jianying Xi Jianying Xi Jianying Xi |
| author_facet | Yufan Zhou Yufan Zhou Yufan Zhou Jialin Chen Zunbo Li Song Tan Song Tan Chong Yan Chong Yan Chong Yan Sushan Luo Sushan Luo Sushan Luo Lei Zhou Lei Zhou Lei Zhou Jie Song Jie Song Jie Song Xiao Huan Xiao Huan Xiao Huan Ying Wang Chongbo Zhao Chongbo Zhao Chongbo Zhao Wenshuang Zeng Jianying Xi Jianying Xi Jianying Xi |
| author_sort | Yufan Zhou |
| collection | DOAJ |
| description | IntroductionAntibodies to MuSK identify a rare subtype of myasthenia gravis (MuSK-MG). In western countries, the onset age of MuSK-MG peaks in the late 30's while it is unknown in Chinese population.MethodsIn this retrospective multicenter study, we screened 69 MuSK-MG patients from 2042 MG patients in five tertiary referral centers in China from October 2016 to October 2021 and summarized the clinical features and treatment outcomes. Then we subgrouped the patients into early-onset (<50 years old), late-onset (50–64 years old), and very-late-onset (≥65 years old) MG and compared the differences in weakness distribution, disease progression and treatment outcomes among three subgroups.ResultsThe patients with MuSK-MG were female-dominant (55/69) and their mean age at onset was 44.70 ± 15.84 years old, with a broad range of 17–81 years old. At disease onset, 29/69 patients were classified as MGFA Type IIb and the frequency of bulbar and extraocular involvement was 53.6 and 69.6%, respectively. There was no difference in weakness distribution. Compared with early-onset MuSK-MG, very-late-onset patients had a higher proportion of limb muscle involvement (12/15 vs.16/40, p = 0.022) 3 months after onset. Six months after onset, more patients with bulbar (14/15 vs. 26/39, p = 0.044) and respiratory involvement (6/15 vs. 0/13, p = 0.013) were seen in very-late-onset than in late-onset subgroup. The very-late-onset subgroup had the highest frequency of limb weakness (86.7%, p < 0.001). One year after onset, very-late-onset patients demonstrated a higher frequency of respiratory involvement than early-onset patients (4/12 vs. 2/35, p = 0.036). 39/64 patients reached MSE. Among 46 patients who received rituximab, very-late-onset patients started earlier than late-onset patients [6 (5.5–7.5) vs. 18 (12–65) months, p = 0.039], but no difference in the time and rate to achieving MSE was identified.ConclusionMuSK-MG patients usually manifested as acute onset and predominant bulbar and respiratory involvement with female dominance. Very-late-onset patients displayed an early involvement of limb, bulbar and respiratory muscles in the disease course, which might prompt their earlier use of rituximab. The majority MuSK-MG patients can benefit from rituximab treatment regardless of age at onset. |
| first_indexed | 2024-12-14T05:50:23Z |
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| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-12-14T05:50:23Z |
| publishDate | 2022-04-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Neurology |
| spelling | doaj.art-c1e04e96b0f1401bbcf46bd50f54dceb2022-12-21T23:14:45ZengFrontiers Media S.A.Frontiers in Neurology1664-22952022-04-011310.3389/fneur.2022.879261879261Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in ChinaYufan Zhou0Yufan Zhou1Yufan Zhou2Jialin Chen3Zunbo Li4Song Tan5Song Tan6Chong Yan7Chong Yan8Chong Yan9Sushan Luo10Sushan Luo11Sushan Luo12Lei Zhou13Lei Zhou14Lei Zhou15Jie Song16Jie Song17Jie Song18Xiao Huan19Xiao Huan20Xiao Huan21Ying Wang22Chongbo Zhao23Chongbo Zhao24Chongbo Zhao25Wenshuang Zeng26Jianying Xi27Jianying Xi28Jianying Xi29Department of Neurology, Huashan Hospital, Fudan University, Shanghai, ChinaHuashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, ChinaNational Center for Neurological Diseases, Shanghai, ChinaDepartment of Neurology, Fujian Medical University Union Hospital, Fuzhou, ChinaDepartment of Neurology, Xi'an Gaoxin Hospital, Xi'an, ChinaDepartment of Neurology, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, ChinaChinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai, ChinaHuashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, ChinaNational Center for Neurological Diseases, Shanghai, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai, ChinaHuashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, ChinaNational Center for Neurological Diseases, Shanghai, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai, ChinaHuashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, ChinaNational Center for Neurological Diseases, Shanghai, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai, ChinaHuashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, ChinaNational Center for Neurological Diseases, Shanghai, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai, ChinaHuashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, ChinaNational Center for Neurological Diseases, Shanghai, ChinaDepartment of Pharmacy, Huashan Hospital, Fudan University, Shanghai, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai, ChinaHuashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, ChinaNational Center for Neurological Diseases, Shanghai, ChinaDepartment of Neurology, The University of Hong Kong-Shenzhen Hospital, Shenzhen, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai, ChinaHuashan Rare Disease Center, Huashan Hospital, Fudan University, Shanghai, ChinaNational Center for Neurological Diseases, Shanghai, ChinaIntroductionAntibodies to MuSK identify a rare subtype of myasthenia gravis (MuSK-MG). In western countries, the onset age of MuSK-MG peaks in the late 30's while it is unknown in Chinese population.MethodsIn this retrospective multicenter study, we screened 69 MuSK-MG patients from 2042 MG patients in five tertiary referral centers in China from October 2016 to October 2021 and summarized the clinical features and treatment outcomes. Then we subgrouped the patients into early-onset (<50 years old), late-onset (50–64 years old), and very-late-onset (≥65 years old) MG and compared the differences in weakness distribution, disease progression and treatment outcomes among three subgroups.ResultsThe patients with MuSK-MG were female-dominant (55/69) and their mean age at onset was 44.70 ± 15.84 years old, with a broad range of 17–81 years old. At disease onset, 29/69 patients were classified as MGFA Type IIb and the frequency of bulbar and extraocular involvement was 53.6 and 69.6%, respectively. There was no difference in weakness distribution. Compared with early-onset MuSK-MG, very-late-onset patients had a higher proportion of limb muscle involvement (12/15 vs.16/40, p = 0.022) 3 months after onset. Six months after onset, more patients with bulbar (14/15 vs. 26/39, p = 0.044) and respiratory involvement (6/15 vs. 0/13, p = 0.013) were seen in very-late-onset than in late-onset subgroup. The very-late-onset subgroup had the highest frequency of limb weakness (86.7%, p < 0.001). One year after onset, very-late-onset patients demonstrated a higher frequency of respiratory involvement than early-onset patients (4/12 vs. 2/35, p = 0.036). 39/64 patients reached MSE. Among 46 patients who received rituximab, very-late-onset patients started earlier than late-onset patients [6 (5.5–7.5) vs. 18 (12–65) months, p = 0.039], but no difference in the time and rate to achieving MSE was identified.ConclusionMuSK-MG patients usually manifested as acute onset and predominant bulbar and respiratory involvement with female dominance. Very-late-onset patients displayed an early involvement of limb, bulbar and respiratory muscles in the disease course, which might prompt their earlier use of rituximab. The majority MuSK-MG patients can benefit from rituximab treatment regardless of age at onset.https://www.frontiersin.org/articles/10.3389/fneur.2022.879261/fullmyasthenia gravismuscle-specific tyrosine kinaseclinical featuresweakness distributionage at onset |
| spellingShingle | Yufan Zhou Yufan Zhou Yufan Zhou Jialin Chen Zunbo Li Song Tan Song Tan Chong Yan Chong Yan Chong Yan Sushan Luo Sushan Luo Sushan Luo Lei Zhou Lei Zhou Lei Zhou Jie Song Jie Song Jie Song Xiao Huan Xiao Huan Xiao Huan Ying Wang Chongbo Zhao Chongbo Zhao Chongbo Zhao Wenshuang Zeng Jianying Xi Jianying Xi Jianying Xi Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China Frontiers in Neurology myasthenia gravis muscle-specific tyrosine kinase clinical features weakness distribution age at onset |
| title | Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China |
| title_full | Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China |
| title_fullStr | Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China |
| title_full_unstemmed | Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China |
| title_short | Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China |
| title_sort | clinical features of myasthenia gravis with antibodies to musk based on age at onset a multicenter retrospective study in china |
| topic | myasthenia gravis muscle-specific tyrosine kinase clinical features weakness distribution age at onset |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2022.879261/full |
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