Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China.
Clinical Features of Myasthenia Gravis With Antibodies to MuSK Based on Age at Onset: A Multicenter Retrospective Study in China.
复制标题
DOI：
10.3389/fneur.2022.879261
复制
发表时间：
2022
影响因子：
3.4
通讯作者：
Xi, Jianying
中科院分区：
文献类型：
--
作者：
Zhou, Yufan;Chen, Jialin;Li, Zunbo;Tan, Song;Yan, Chong;Luo, Sushan;Zhou, Lei;Song, Jie;Huan, Xiao;Wang, Ying;Zhao, Chongbo;Zeng, Wenshuang;Xi, Jianying
关键词：
MuSK抗体鉴定了一种罕见的重症肌无力亚型（MuSK-MG）。在西方国家，MuSK-MG的发病年龄高峰在30年代后期，而在中国人群中尚不清楚。在这项回顾性多中心研究中，我们从2016年10月至2021年10月中国五个三级转诊中心的2042例MG患者中筛选了69例MuSK-MG患者，并总结了临床特征和治疗结局。然后将患者分为早发性（<50岁）、晚发性（50 - 64岁）和极晚发性（≥ 65岁）MG，并比较三个亚组之间的虚弱分布、疾病进展和治疗结局的差异。MuSK-MG患者以女性为主（55/69），发病年龄17-81岁，平均44.70 ± 15.84岁。在发病时，29/69例患者被归类为MGFA IIb型，眼球和眼外受累的频率分别为53.6%和69.6%。在弱点分布方面没有差异。与早发型MuSK-MG相比，极晚发型患者在发病后3个月肢体肌肉受累的比例更高（12/15 vs.16/40，p = 0.022）。发病后6个月，极晚发亚组中出现延髓（14/15 vs. 26/39，p = 0.044）和呼吸系统受累（6/15 vs. 0/13，p = 0.013）的患者多于晚发亚组。极晚发亚组的肢体无力发生率最高（86.7%，p <0.001）。发病后1年，极晚发患者的呼吸系统受累频率高于早发患者（4/12 vs. 2/35，p = 0.036）。39/64例患者达到MSE。在46例接受利妥昔单抗治疗的患者中，极晚发患者开始治疗的时间早于晚发患者[6（5.5 - 7.5）个月vs. 18（12 - 65）个月，p = 0.039]，但达到MSE的时间和比率没有差异。MuSK-MG患者多表现为急性起病，以女性为主的延髓和呼吸道受累。极晚发患者在病程中表现出肢体、延髓和呼吸肌的早期受累，这可能促使他们更早使用利妥昔单抗。大多数MuSK-MG患者可以从利妥昔单抗治疗中获益，无论发病年龄如何。
Antibodies 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. In 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. The 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. MuSK-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.
登录
查看更多内容
影响因子：
9.9
作者：
Sanders DB;Wolfe GI;Benatar M;Evoli A;Gilhus NE;Illa I;Kuntz N;Massey JM;Melms A;Murai H;Nicolle M;Palace J;Richman DP;Verschuuren J;Narayanaswami P
通讯作者：
Narayanaswami P
DOI：
10.3324/haematol.2009.018689
发表时间：
2010-06-01
期刊：
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
影响因子：
--
作者：
Caraux, Anouk;Klein, Bernard;Perez-Andres, Martin
通讯作者：
Perez-Andres, Martin
影响因子：
5.1
作者：
Nakata, R.;Motomura, M.;Kawakami, A.
通讯作者：
Kawakami, A.
DOI：
10.1111/j.1749-6632.2012.06781.x
发表时间：
2012-01-01
期刊：
MYASTHENIA GRAVIS AND RELATED DISORDERS I
影响因子：
--
作者：
Evoli, Amelia;Alboini, Paolo E.;Bartocccioni, Emanuela
通讯作者：
Bartocccioni, Emanuela
影响因子：
6
作者：
Leite, Maria I.;Jones, Margaret;Willcox, Nick
通讯作者：
Willcox, Nick