アブストラクト
Japanese
| Title | 重症筋無力症の治療最近の動向 |
|---|---|
| Subtitle | 第38回日本神経治療学会学術集会特集2 マラソンレクチャー13 |
| Authors | 紺野晋吾** |
| Authors (kana) | |
| Organization | **東邦大学医療センター大橋病院神経内科 |
| Journal | 神経治療学 |
| Volume | 38 |
| Number | 4 |
| Page | 446-448 |
| Year/Month | 2021 / |
| Article | 報告 |
| Publisher | 日本神経治療学会 |
| Abstract | 「はじめに」1990年代以前の重症筋無力症 (myasthenia gravis : MG) は主に胸腺摘除術と高用量の経口prednisolone (PSL) で治療された. 2000年以降に免疫抑制薬や免疫グロブリン療法 (Intravenous immunoglobulin therapy : IVIg) の治療選択肢が増えた. 2016年に非胸腺腫例に対する胸腺摘除の無作為化比較試験で, acetylcholine receptor (AChR) 抗体陽性若年発症MGで有効性が確認され, 胸腺摘除も有効例が明確化された. 2017年には標準治療に抵抗性のAChR抗体陽性例に補体component C5阻害薬であるeculizumabが承認された. 現在, 他の分子標的剤の臨床治験も進行中である. MGの寛解率はいまだ高くなく, 患者quality of life (QOL) 向上を目標に治療が計画される. |
| Practice | 臨床医学：内科系 |
| Keywords | myasthenia gravis (MG) , generalized MG, ocular MG, early fast-acting treatment strategy, molecular target drug |
English
| Title | Recent developments in the treatment of myasthenia gravis |
|---|---|
| Subtitle | |
| Authors | Shingo KONNO |
| Authors (kana) | |
| Organization | Department of Neurology, Toho University Ohashi Medical Center |
| Journal | Neurological Therapeutics |
| Volume | 38 |
| Number | 4 |
| Page | 446-448 |
| Year/Month | 2021 / |
| Article | Report |
| Publisher | Japanese Society of Neurological Therapeutics |
| Abstract | In the 1970s and 1990s, thymectomy and high-dose oral prednisolone (PSL) were the only treatment options for myasthenia gravis (MG) . Immunosuppressive agents and intravenous immunoglobulin therapy for MG were introduced in 2000. In 2016, a randomized controlled trial was conducted to evaluate the efficacy of thymectomy in non-thymoma patients, and the treatment was found to be effective in patients with acetylcholine receptor (AChR) antibody-positive early onset MG. In 2017, eculizumab, a component C5 inhibitor, was introduced for AChR antibody-positive refractory patients who relapse despite standard therapy. New molecularly targeted agents for MG are under development and clinical trials. However, the remission rate of MG remains low, and treatment is aimed at improving patients' quality of life (QOL) . To maximize QOL improvement, PSL use should be minimized. Early treatment with a combination of plasmapheresis, intravenous methylprednisolone therapy, and immunosuppressive drugs is the fastest and most effective way to achieve good QOL and is recommended for the treatment of generalized MG. |
| Practice | Clinical internal medicine |
| Keywords | myasthenia gravis (MG) , generalized MG, ocular MG, early fast-acting treatment strategy, molecular target drug |
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参考文献
- 1) 錫村明生, 本村政勝, 槍沢公明ほか : Clinical Question 4-5後期発症MG(late-onset MG ; 50歳以上発症)の臨床的特徴は, 重症筋無力症診療ガイドライン2014(重症筋無力症診療ガイドライン作成委員会編), 東京, 南光堂, 2014, p38-40
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- 5) Utsugisawa K, Nagane Y, Akaishi T et al : Early fast-acting treatment strategy against generalized myasthenia gravis. Muscle Nerve 55 : 794-801, 2017
残りの7件を表示する
- 6) Suzuki S, Murai H, Imai T et al : Quality of life in purely ocu-lar myasthenia in Japan. BMC Neurol 14 : 142, 2014
- 7) Sussman J, Farrugia ME, Maddison P et al : Myasthenia gravis : Association of British Neurologists' management guidelines. Pract Neurol 15 : 199-206, 2015
- 8) Ozawa Y, Uzawa A, Kanai T et al : Efficacy of high-dose in-travenous methylPSL therapy for ocular myasthenia gravis. J Neurol Sci 402 : 12-15, 2019
- 9) Kanai T, Uzawa A, Sato Y et al : A clinical predictive score for postoperative myasthenic crisis. Ann Neurol 82 : 841-849, 2017
- 10) Wolfe GI, Kaminski HJ, Aban IB et al ; MGTX Study Group : Randomized Trial of Thymectomy in Myasthenia Gravis. N Engl J Med 375 : 511-522, 2016
- 11) Wolfe GI, Kaminski HJ, Aban IB et al ; MGTX Study Group : Long-term effect of thymectomy plus prednisone versus pre-dnisone alone in patients with non-thymomatous myasthe-nia gravis : 2-year extension of the MGTX randomised trial. Lancet Neurol 18 : 259-268, 2019
- 12) Oyama M, Okada K, Masuda M et al : Suitable indications of eculizumab for patients with refractory generalized myasthe-nia gravis. Ther Adv Neurol Disord 13 : 1756286420904207, 2020
