発症5年以上の全身型重症筋無力症患者の重症筋無力症－日常生活動作スコアに関連する因子の検討：難治性の診断のために

当科で診療した発症から5年以上の全身型重症筋無力症患者55例を解析対象とし，最終観察時重症筋無力症－日常生活動作（the Myasthenia Gravis Activities of Daily Living，以下MG-ADLと略記）スコアが高い群の特徴を解析した．最終観察時MG-ADLスコアの関連因子は総速効性治療回数とMyasthenia Gravis Foundation of America（MGFA）分類であった．MG-ADLスコア5以上の患者では1）発症時年齢が若年で，2）罹病期間が長く，3）MGFA V症例が多く，4）総FT回数が多く，5）最終観察時PSL量が多かった．MGFA...

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Published in臨床神経学 Vol. 62; no. 12; pp. 915 - 921
Main Authors 野村, 栄一, 山脇, 健盛, 河野, 智之, 郡山, 達男, 丸山, 博文, 越智, 一秀, 内藤, 裕之, 大野, 成美, 儀賀, 麻由実, 杉本, 太路
Format Journal Article
LanguageJapanese
Published 日本神経学会 2022
Subjects
治療効果
速効性治療
難治性
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ISSN0009-918X
1882-0654
DOI10.5692/clinicalneurol.cn-001790

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Abstract 当科で診療した発症から5年以上の全身型重症筋無力症患者55例を解析対象とし，最終観察時重症筋無力症－日常生活動作（the Myasthenia Gravis Activities of Daily Living，以下MG-ADLと略記）スコアが高い群の特徴を解析した．最終観察時MG-ADLスコアの関連因子は総速効性治療回数とMyasthenia Gravis Foundation of America（MGFA）分類であった．MG-ADLスコア5以上の患者では1）発症時年齢が若年で，2）罹病期間が長く，3）MGFA V症例が多く，4）総FT回数が多く，5）最終観察時PSL量が多かった．MGFA V症例，E-L-T分類によらず発症年齢が若年である症例，PSL量の減量が困難な症例はより早期に新規治療の対象になる可能性があり，前向き研究での検証が期待される．
AbstractList 当科で診療した発症から5年以上の全身型重症筋無力症患者55例を解析対象とし，最終観察時重症筋無力症－日常生活動作（the Myasthenia Gravis Activities of Daily Living，以下MG-ADLと略記）スコアが高い群の特徴を解析した．最終観察時MG-ADLスコアの関連因子は総速効性治療回数とMyasthenia Gravis Foundation of America（MGFA）分類であった．MG-ADLスコア5以上の患者では1）発症時年齢が若年で，2）罹病期間が長く，3）MGFA V症例が多く，4）総FT回数が多く，5）最終観察時PSL量が多かった．MGFA V症例，E-L-T分類によらず発症年齢が若年である症例，PSL量の減量が困難な症例はより早期に新規治療の対象になる可能性があり，前向き研究での検証が期待される．
Author 儀賀, 麻由実
丸山, 博文
杉本, 太路
山脇, 健盛
野村, 栄一
越智, 一秀
郡山, 達男
大野, 成美
内藤, 裕之
河野, 智之
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References 5) Howard JF Jr, Nowak RJ, Wolfe GI, et al. Clinical effects of the self-administered subcutaneous complement inhibitor Zilcoplan in patients with moderate to severe generalized myasthenia gravis. JAMA Neurol 2020;77:1-11.
11) 重症筋無力症/ランバート・イートン筋無力症症候群診療ガイドライン作成委員会編．重症筋無力症/ランバート・イートン筋無力症症候群診療ガイドライン2022．東京：南江堂；2022. p. 52.
1) Nagane Y, Suzuki S, Suzuki N, et al. Early aggressive treatment strategy against myasthenia gravis. Eur Neurol 2011;65:16-22.
9) Wolfe GI, Herbelin L, Nations SP, et al. Myasthenia gravis activities of daily living profile. Neurology 1999;52:1487-1489.
12) Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology 2016;87:419-425.
2) Utsugisawa K, Nagane Y, Akaishi T, et al. Early fast-acting treatment strategy against generalized myasthenia gravis. Muscle Nerve 2017;55:794-801.
13) Mantegazza R, Antozzi C. When myasthenia gravis is deemed refractory: clinical signposts and treatment strategies. Ther Adv Neurol Disord 2018;11:1756285617749134.
7) Masaoka A, Yamakawa Y, Niwa H, et al. Extended thymectomy for myasthenia gravis patients: a 20-year review. Ann Thorac Surg 1996;62:853-859.
10) Jaretzki A 3rd, Barohn RJ, Ernstoff RM, et al. Myasthenia gravis: recommendation for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology 2000;55:16-23.
8) Murai H. Japanese clinical guidelines for myasthenia gravis: putting into practice. Clin Exp Neuroimmunol 2014;6:21-31.
14) Oyama M, Okada K, Masuda M, et al. Suitable indications of eculizumab for patients with refractory generalized myasthenia gravis. Ther Adv Neurol Disord 2020;13:1756286420904207.
4) Howard JF Jr, Bril V, Vu T, et al. Safety, efficacy, and tolerability of efgartigimod in patients with generalized myasthenia gravis (ADAPT): a multicentre, randomized, placebo-controlled, phase 3 trial. Lancet Neurol 2021;20:526-536.
6) Bever CT Jr, Aquino AV, Penn AS, et al. Prognosis of ocular myasthenia. Ann Neurol 1983;14:516-519.
3) Howard JF Jr, Utsugisawa K, Benatar M, et al. Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalized myasthenia gravis (REGAIN): a phase 3, randomized, double-blind, placebo-controlled, multi­centre study. Lancet Neurol 2017;16:976-986.
References_xml – reference: 2) Utsugisawa K, Nagane Y, Akaishi T, et al. Early fast-acting treatment strategy against generalized myasthenia gravis. Muscle Nerve 2017;55:794-801.
– reference: 7) Masaoka A, Yamakawa Y, Niwa H, et al. Extended thymectomy for myasthenia gravis patients: a 20-year review. Ann Thorac Surg 1996;62:853-859.
– reference: 12) Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology 2016;87:419-425.
– reference: 11) 重症筋無力症/ランバート・イートン筋無力症症候群診療ガイドライン作成委員会編．重症筋無力症/ランバート・イートン筋無力症症候群診療ガイドライン2022．東京：南江堂；2022. p. 52.
– reference: 5) Howard JF Jr, Nowak RJ, Wolfe GI, et al. Clinical effects of the self-administered subcutaneous complement inhibitor Zilcoplan in patients with moderate to severe generalized myasthenia gravis. JAMA Neurol 2020;77:1-11.
– reference: 1) Nagane Y, Suzuki S, Suzuki N, et al. Early aggressive treatment strategy against myasthenia gravis. Eur Neurol 2011;65:16-22.
– reference: 9) Wolfe GI, Herbelin L, Nations SP, et al. Myasthenia gravis activities of daily living profile. Neurology 1999;52:1487-1489.
– reference: 13) Mantegazza R, Antozzi C. When myasthenia gravis is deemed refractory: clinical signposts and treatment strategies. Ther Adv Neurol Disord 2018;11:1756285617749134.
– reference: 8) Murai H. Japanese clinical guidelines for myasthenia gravis: putting into practice. Clin Exp Neuroimmunol 2014;6:21-31.
– reference: 14) Oyama M, Okada K, Masuda M, et al. Suitable indications of eculizumab for patients with refractory generalized myasthenia gravis. Ther Adv Neurol Disord 2020;13:1756286420904207.
– reference: 4) Howard JF Jr, Bril V, Vu T, et al. Safety, efficacy, and tolerability of efgartigimod in patients with generalized myasthenia gravis (ADAPT): a multicentre, randomized, placebo-controlled, phase 3 trial. Lancet Neurol 2021;20:526-536.
– reference: 10) Jaretzki A 3rd, Barohn RJ, Ernstoff RM, et al. Myasthenia gravis: recommendation for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology 2000;55:16-23.
– reference: 3) Howard JF Jr, Utsugisawa K, Benatar M, et al. Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalized myasthenia gravis (REGAIN): a phase 3, randomized, double-blind, placebo-controlled, multi­centre study. Lancet Neurol 2017;16:976-986.
– reference: 6) Bever CT Jr, Aquino AV, Penn AS, et al. Prognosis of ocular myasthenia. Ann Neurol 1983;14:516-519.
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Snippet 当科で診療した発症から5年以上の全身型重症筋無力症患者55例を解析対象とし，最終観察時重症筋無力症－日常生活動作（the Myasthenia Gravis Activities of Daily...
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SubjectTerms 治療効果
速効性治療
難治性
Title 発症5年以上の全身型重症筋無力症患者の重症筋無力症－日常生活動作スコアに関連する因子の検討：難治性の診断のために
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Volume 62
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