| H01594 | |
| H番号 | H01594 |
| 名称 | 重症筋無力症 |
| 概要 | Myasthenia gravis (MG) is an autoimmune disorder characterized by a defective transmission of nerve impulses to muscles leading to muscle weakness and fatigability. Some, but not all, muscles are affected and not necessarily symmetrically. Increased weakness with continued muscle activity represents a diagnostic clue for MG, but these clinical features can vary. MG is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor (AChR), muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction. Patients should be classified into subgroups to help with therapeutic decisions and prognosis. Subgroups based on serum antibodies and clinical features include early-onset, late-onset, thymoma, MUSK, LRP4, antibody-negative, and ocular forms of myasthenia gravis. Agrin-associated MG might emerge as a new entity. The prognosis is good with optimum symptomatic, immunosuppressive, and supportive treatment. The evolution of MG is unpredictable, but it is generally characterized by the occurrence of relapses, sometimes subsequent to remissions and a worsening trend. For 85% of MG patients, the maximum severity is reached within less than 3 years. |
| カテゴリ | 免疫系疾患; 神経系疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | ジスチグミン臭化物 [DR:D01228] アンベノニウム塩化物 [DR:D01001] ネオスチグミン臭化物 [DR:D00995] ネオスチグミンメチル硫酸塩 [DR:D00998] ピリドスチグミン臭化物 [DR:D00487] デキサメタゾン [DR:D00292] デキサメタゾンリン酸エステルナトリウム [DR:D00975] ベタメタゾンリン酸エステルナトリウム [DR:D00972] トリアムシノロンアセトニド [DR:D00983] タクロリムス水和物 [DR:D00107] シクロスポリン [DR:D00184] ヒドロコルチゾンコハク酸エステルナトリウム [DR:D00978] ベタメタゾン [DR:D00244] プレドニゾロン [DR:D00472] プレドニゾロンコハク酸エステルナトリウム [DR:D01239] ポリエチレングリコール処理人免疫グロブリン [DR:D08783] エクリズマブ [DR:D03940] ラブリズマブ [DR:D11054] (抗アセチルコリン受容体抗体陽性) エフガルチギモドアルファ [DR:D11876] |
| コメント | Autoantigen: CHRNA1 [HSA:1134] [KO:K04803] MUSK [HSA:4593] [KO:K05129] LRP4 [HSA:4038] [KO:K20051] |
| リンク | ICD-11: 8C60 ICD-10: G70.0 MeSH: D009157 |
| 文献 | PMID:24530233 著者 Berrih-Aknin S, Frenkian-Cuvelier M, Eymard B タイトル Diagnostic and clinical classification of autoimmune myasthenia gravis. 雑誌 J Autoimmun 48-49:143-8 (2014) DOI:10.1016/j.jaut.2014.01.003 PMID:26376969 著者 Gilhus NE, Verschuuren JJ タイトル Myasthenia gravis: subgroup classification and therapeutic strategies. 雑誌 Lancet Neurol 14:1023-36 (2015) DOI:10.1016/S1474-4422(15)00145-3 PMID:26600875 著者 Dalakas MC タイトル Future perspectives in target-specific immunotherapies of myasthenia gravis. 雑誌 Ther Adv Neurol Disord 8:316-27 (2015) DOI:10.1177/1756285615605700 PMID:27103470 著者 Gilhus NE, Skeie GO, Romi F, Lazaridis K, Zisimopoulou P, Tzartos S タイトル Myasthenia gravis - autoantibody characteristics and their implications for therapy. 雑誌 Nat Rev Neurol 12:259-68 (2016) DOI:10.1038/nrneurol.2016.44 PMID:21180568 著者 Gold R, Hohlfeld R, Toyka KV タイトル Progress in the treatment of myasthenia gravis. 雑誌 Ther Adv Neurol Disord 1:36-51 (2008) DOI:10.1177/1756285608093888 PMID:23252892 (autoantigen) 著者 Lindstrom J, Luo J タイトル Myasthenogenicity of the main immunogenic region. 雑誌 Ann N Y Acad Sci 1274:9-13 (2012) DOI:10.1111/j.1749-6632.2012.06766.x PMID:11231638 (autoantigen) 著者 Hoch W, McConville J, Helms S, Newsom-Davis J, Melms A, Vincent A タイトル Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. 雑誌 Nat Med 7:365-8 (2001) DOI:10.1038/85520 PMID:21387385 (autoantigen) 著者 Higuchi O, Hamuro J, Motomura M, Yamanashi Y タイトル Autoantibodies to low-density lipoprotein receptor-related protein 4 in myasthenia gravis. 雑誌 Ann Neurol 69:418-22 (2011) DOI:10.1002/ana.22312 |