Clinical features and outcomes of patients with muscle-specific kinase antibody-positive myasthenia gravis in Japan

被引：4

作者：

Yasuda, Manato ^{[1
]}

Uzawa, Akiyuki ^{[1
,13
]}

Kuwabara, Satoshi ^{[1
]}

Suzuki, Shigeaki ^{[2
]}

Akamine, Hiroyuki ^{[1
]}

Onishi, Yosuke ^{[1
]}

Ozawa, Yukiko ^{[1
]}

Kawaguchi, Naoki ^{[3
]}

Kubota, Tomoya ^{[4
]}

Takahashi, Masanori P. ^{[4
]}

Suzuki, Yasushi ^{[5
]}

Watanabe, Genya ^{[5
]}

Kimura, Takashi ^{[6
]}

Sugimoto, Takamichi ^{[7
]}

Samukawa, Makoto ^{[8
]}

Minami, Naoya ^{[9
]}

Masuda, Masayuki ^{[10
]}

Konno, Shingo ^{[11
]}

Nagane, Yuriko ^{[12
]}

Utsugisawa, Kimiaki ^{[12
]}

机构：

[1] Chiba Univ, Grad Sch Med, Dept Neurol, Chiba, Japan

[2] Keio Univ, Sch Med, Dept Neurol, Tokyo, Japan

[3] Neurol Chiba Clin, Dept Neurol, Chiba, Japan

[4] Osaka Univ, Grad Sch Med, Dept Clin Lab & Biomed Sci, Div Hlth Sci, Osaka, Japan

[5] Natl Hosp Org Sendai Med Ctr, Dept Neurol, Sendai, Japan

[6] Hyogo Med Univ, Dept Neurol, Nishinomiya, Hyogo, Japan

[7] Hiroshima Univ, Dept Clin Neurosci & Therapeut, Hiroshima, Japan

[8] Kindai Univ, Fac Med, Dept Neurol, Osaka, Japan

[9] Natl Hosp Org Hokkaido Med Ctr, Dept Neurol, Sapporo, Hokkaido, Japan

[10] Tokyo Med Univ, Dept Neurol, Tokyo, Japan

[11] Toho Univ, Ohashi Med Ctr, Dept Neurol, Tokyo, Japan

[12] Hanamaki Gen Hosp, Dept Neurol, Hanamaki, Japan

[13] Chiba Univ, Grad Sch Med, Dept Neurol, 1-8-1 Inohana,Chuo Ku, Chiba 2608670, Japan

来源：

JOURNAL OF NEUROIMMUNOLOGY | 2023年 / 385卷

关键词：

Clinical features; MuSK; Myasthenia gravis; Neuroimmunology; Neuromuscular disorder; INTERNATIONAL CONSENSUS GUIDANCE; MUSK; MG; MANAGEMENT;

D O I：

10.1016/j.jneuroim.2023.578241

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

This study included 51 patients with muscle-specific kinase antibody-positive myasthenia gravis (MuSK-MG) from a Japanese multicenter survey to examine clinical features and outcomes. Median onset age was 37 years and female predominance was observed. All patients developed generalized symptoms and almost all (50/51) patients had bulbar symptoms. About half of the patients met the criteria for refractory MG. The refractory group had a lower age of onset, higher severity scores, and higher maximum daily doses of oral prednisolone compared to the nonrefractory group. The outcomes for MuSK-MG patients in Japan are not favorable, indicating the need for more aggressive treatment.

引用

页数：6

共 28 条

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