KEGG   DISEASE: 封入体筋炎
エントリ  
H01505                                                             
名称    
封入体筋炎
概要    
Inclusion body myositis (IBM) is the most frequent acquired myopathy after age 45. It is distinguished from other inflammatory myopathies by its selective pattern of muscle involvement and slowly progressive course, and by the combination of inflammatory and degenerative muscle pathology and multi-protein deposits in muscle tissue. It typically presents with chronic insidious proximal leg and/or distal arm asymmetric muscle weakness leading to recurrent falls and loss of dexterity. Creatine kinase (CK) is elevated in IBM and needle electromyography (EMG) mostly shows a chronic irritative myopathy. Muscle histopathology demonstrates endomysial inflammatory exudates surrounding and invading non-necrotic muscle fibers often times accompanied by rimmed vacuoles and protein deposits. IBM is refractory to all known immunosuppressive therapies. It has been shown in small short-term trials that individualised exercise programs can lead to improvement or maintenance of muscle strength and aerobic capacity.
カテゴリ  
神経系疾患; 筋骨格疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 04 免疫系の疾患
  非器官特異的全身性自己免疫疾患
   4A41  特発性炎症性ミオパチー
    H01505  封入体筋炎
指定難病 [jp08407.html]
 H01505
リンク   
ICD-11: 4A41.20
ICD-10: G72.4
MeSH: D018979
文献    
  著者
Dimachkie MM, Barohn RJ
  タイトル
Inclusion body myositis.
  雑誌
Neurol Clin 32:629-46, vii (2014)
DOI:10.1016/j.ncl.2014.04.001
文献    
  著者
Needham M, Mastaglia FL
  タイトル
Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment.
  雑誌
Clin Neurophysiol 127:1764-73 (2016)
DOI:10.1016/j.clinph.2015.12.011
文献    
  著者
Gang Q, Bettencourt C, Houlden H, Hanna MG, Machado PM
  タイトル
Genetic advances in sporadic inclusion body myositis.
  雑誌
Curr Opin Rheumatol 27:586-94 (2015)
DOI:10.1097/BOR.0000000000000213

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