• J. Neurol. Neurosurg. Psychiatr. · Jun 2023

    Effectiveness of early cycles of fast-acting treatment in generalised myasthenia gravis.

    • Akiyuki Uzawa, Shigeaki Suzuki, Satoshi Kuwabara, Hiroyuki Akamine, Yosuke Onishi, Manato Yasuda, Yukiko Ozawa, Naoki Kawaguchi, Tomoya Kubota, TakahashiMasanori PMPDepartment of Clinical Laboratory and Biomedical Sciences, Division of Health Sciences, Osaka University Graduate School of Medicine, Suita, Osaka, Japan., Yasushi Suzuki, Genya Watanabe, Takashi Kimura, Takamichi Sugimoto, Makoto Samukawa, Naoya Minami, Masayuki Masuda, Shingo Konno, Yuriko Nagane, and Kimiaki Utsugisawa.
    • Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan auzawa@chiba-u.jp.
    • J. Neurol. Neurosurg. Psychiatr. 2023 Jun 1; 94 (6): 467473467-473.

    BackgroundEarly fast-acting treatment (EFT) is the aggressive use of fast-acting therapies such as plasmapheresis, intravenous immunoglobulin and/or intravenous high-dose methylprednisolone (IVMP) from the early phases of treatment. EFT is reportedly beneficial for early achievement of minimal manifestations (MM) or better status with ≤5 mg/day prednisolone (MM5mg), a practical therapeutic target for myasthenia gravis (MG).ObjectiveThe current study aimed to clarify which specific EFT regimen is efficacious and the patient characteristics that confer sensitivity to EFT.MethodsWe recruited a total of 1710 consecutive patients with MG who enrolled in the Japan MG Registry for this large-cohort study. Among them, 1066 with generalised MG who had received immunotherapy were analysed. Prognostic background factors were matched in a 1:1 ratio using propensity score matching analysis between patients treated with EFT (n=350) and those treated without EFT (n=350). The clinical course and time to first achieve MM5mg after starting immunotherapy was analysed in relation to treatment combinations and patient characteristics.ResultsKaplan-Meier analyses showed that EFT had a significant effect on the achievement of MM5mg (p<0.0001, log-rank test; HR 1.82, p<0.0001). Notably, EFT was efficacious for any type of MG, and the inclusion of IVMP resulted in earlier and more frequent achievement of MM5mg (p=0.0352, log-rank test; HR 1.46, p=0.0380). In addition, early administration of calcineurin inhibitors also promoted MM5mg achievement.ConclusionEarly cycles of intervention with EFT and early use of calcineurin inhibitors provides long-term benefits in terms of achieving therapeutic targets for generalised MG, regardless of clinical subtype.© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

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