Japan MG registry: Chronological surveys over 10 years

Clinical and Experimental Neuroimmunology - Tập 14 Số 1 - Trang 5-12 - 2023
Shigeaki Suzuki1, Masayuki Masuda2, Akiyuki Uzawa3, Yuriko Nagane4, Shingo Konno5, Yasushi Suzuki6, Tomoya Kubota7, Takamichi Sugimoto8, Makoto Samukawa9, Genya Watanabe6, Kei Ishizuchi1, Hiroyuki Akamine3, Yosuke Onishi3, Kazuki Yoshizumi10, Takafumi Uchi5, Itaru Amino11, Yuki Ueta2, Naoya Minami11, Naoki Kawaguchi12, Takashi Kimura10, Masanori Takahashi7, Hiroyuki Murai13, Kimiaki Utsugisawa4
1Department of Neurology, Keio University School of Medicine, Tokyo, Japan
2Department of Neurology, Tokyo Medical University, Tokyo, Japan
3Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan
4Department of Neurology, Hanamaki General Hospital, Hanamaki, Japan
5Department of Neurology, Toho University Ohashi Medical Center, Tokyo, Japan
6Department of Neurology, National Hospital Organization Sendai Medical Center, Sendai, Japan
7Department of Clinical Laboratory and Biomedical Sciences, Division of Health Sciences, Osaka University Graduate School of Medicine, Osaka, Japan
8Department of Clinical Neuroscience and Therapeutics, Hiroshima University, Hiroshima, Japan
9Department of Neurology, Kindai University Faculty of Medicine, Sayama, Japan
10Department of Neurology, Hyogo Medical University, Nishinomiya, Japan
11Department of Neurology, National Hospital Organization Hokkaido Medical Center, Sapporo, Japan
12Department of Neurology, Neurology Chiba Clinic, Chiba, Japan
13Department of Neurology, International University of Health and Welfare, Narita, Japan

Tóm tắt

Abstract

The primary purpose of the Japanese myasthenia gravis registry (JAMG‐R) has been to research and promote high‐quality medical care for MG patients in Japan. We reviewed the findings of surveys performed by JAMG‐R over an ~10‐y period. The first goal for favorable quality of life (QOL) is a status of minimal manifestations (MM) or better with an oral prednisolone (PSL) dose of 5 mg/d or less (MM‐5 mg). Early and aggressive use of nonoral fast‐acting treatment together with low‐dose oral PSL (the “EFT strategy”) is recommended to reduce disease severity with minimal oral steroid use so that the MM‐5 mg target can be met as soon as possible. We conducted the fourth largest multicenter survey ever in 2021, obtaining detailed clinical information from 1710 consecutive MG patients all over Japan, and compared the 2021 surveys with those from 2012 and 2015. The frequency of patients treated with EFT strategies showed a gradual increase, reaching 39% of the total MG patients in the 2021 survey. The current and maximum dose of PSL and the number of days at high‐dose (>20 mg/d) PSL showed decreases. Survey results indicate that as EFT strategies have spread, the percentage of patients on MM‐5 mg has increased. We again confirmed that MM‐5 mg was associated with favorable QOL in the 2021 survey. Recent data regarding COVID‐19 suggests that it did not seriously impact the MG population in Japan; unfortunately, refractory MG, observed in 21% of patients, is still an unresolved problem.

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