KEGG   DISEASE: 重症筋無力症
エントリ  
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
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 08 神経系の疾患
  神経筋接合部または筋の疾患
   重症筋無力症または明示された神経筋接合部疾患
    8C60  重症筋無力症
     H01594  重症筋無力症
指定難病 [jp08407.html]
 H01594
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]
ジルコプランナトリウム [DR:D12356] (抗アセチルコリン受容体抗体陽性)
ポリエチレングリコール処理人免疫グロブリン [DR:D08783]
エフガルチギモドアルファ [DR:D11876]
エフガルチギモドアルファ・ボルヒアルロニダーゼアルファ [DR:D12684]
ロザノリキシズマブ [DR:D12182]
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)
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    
  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
文献    
  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
文献    
  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
文献    
  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
文献    
  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
文献    
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
文献    
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
文献    
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
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