KEGG   DISEASE: 慢性炎症性脱髄性多発神経炎
エントリ  
H01527                                                             
名称    
慢性炎症性脱髄性多発神経炎;
多巣性運動ニューロパチー
概要    
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a polyneuropathy that is often disabling, with more than 50% of patients reported as having temporary disability, and about 10% eventually becoming persistently disabled. The core clinical features are a chronic progressive or relapsing and remitting, symmetrical, and sensory and motor polyradiculoneuropathy causing weakness of proximal and distal muscles. The etiology is suspected to be of autoimmune origin, and the diagnosis is typically based on clinical history, neurologic examination, electrophysiologic studies, cerebrospinal fluid (CSF) studies, and pathologic examination. CIDP often responds to immune therapies including corticosteroids, plasma exchange, and high-dose intravenous immunoglobulin (IVIg).
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
  神経根, 神経叢または末梢神経の疾患
   多発ニューロパチー
    8C01  炎症性多発ニューロパチー
     H01527  慢性炎症性脱髄性多発神経炎
指定難病 [jp08407.html]
 H01527
治療薬   
pH4処理酸性人免疫グロブリン [DR:D08779]
ポリエチレングリコール処理人免疫グロブリン [DR:D08783]
乾燥スルホ化人免疫グロブリン [DR:D08786]
乾燥ポリエチレングリコール処理人免疫グロブリン [DR:D08788]
リンク   
ICD-11: 8C01.3
ICD-10: G61.8
MeSH: D020277
文献    
  著者
Press R, Hiew FL, Rajabally YA
  タイトル
Steroids for chronic inflammatory demyelinating polyradiculoneuropathy: evidence base and clinical practice.
  雑誌
Acta Neurol Scand 133:228-38 (2016)
DOI:10.1111/ane.12519
文献    
  著者
Nobile-Orazio E, Gallia F
  タイトル
Update on the treatment of chronic inflammatory demyelinating polyradiculoneuropathy.
  雑誌
Curr Opin Neurol 28:480-5 (2015)
DOI:10.1097/WCO.0000000000000232
文献    
  著者
Kleyman I, Brannagan TH 3rd
  タイトル
Treatment of chronic inflammatory demyelinating polyneuropathy.
  雑誌
Curr Neurol Neurosci Rep 15:47 (2015)
DOI:10.1007/s11910-015-0563-z
文献    
  著者
Hughes RA, Allen D, Makowska A, Gregson NA
  タイトル
Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy.
  雑誌
J Peripher Nerv Syst 11:30-46 (2006)
DOI:10.1111/j.1085-9489.2006.00061.x
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