KEGG   DISEASE: 多発血管炎性肉芽腫症
エントリ  
H01655                                                             
名称    
多発血管炎性肉芽腫症;
ウェゲナー肉芽腫症
概要    
Granulomatosis with polyangitis (GPA) is an idiopathic, systemic inflammatory disease characterized by necrotizing granulomatous inflammation and pauci-immune small- vessel vasculitis of upper and lower respiratory tract and kidneys. The discovery of anti-neutrophil cytoplasmic antibodies (ANCAs) as a marker associated with GPA focused attention on the potential pathogenic role of these antibodies. Although it has been described that ANCAs recognise a variety of myeloid antigens, only antibodies that react with proteinase 3 (Pr3) and myeloperoxidase (MPO) have consistently been linked to vasculitis syndromes. Pr3-ANCA is the predominant autoantibody found in patients with GPA. The discovery of ANCA has made the diagnosis of this disease even more possible but there is almost always a need to confirm the diagnosis via histological examination of the lesional tissue.
カテゴリ  
皮膚および結合組織疾患
階層分類  
ヒト疾患 [BR:jp08402]
 皮膚疾患
  皮膚と軟部組織の疾患
   H01655  多発血管炎性肉芽腫症
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
   4A44  血管炎
    H01655  多発血管炎性肉芽腫症
特定疾患 (難病) [jp08407.html]
 H01655
治療薬   
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]
ヒドロコルチゾン [DR:D00088]
トリアムシノロン [DR:D00385]
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]
メチルプレドニゾロン [DR:D00407]
メチルプレドニゾロンコハク酸エステルナトリウム [DR:D00751]
メチルプレドニゾロン酢酸エステル [DR:D00979]
アザチオプリン [DR:D00238]
アバコパン [DR:D11093]
シクロホスファミド水和物 [DR:D00287]
リツキシマブ [DR:D02994]
リンク   
ICD-11: 4A44.A1
ICD-10: M31.3
MeSH: D014890
文献    
  著者
Almouhawis HA, Leao JC, Fedele S, Porter SR
  タイトル
Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment.
  雑誌
J Oral Pathol Med 42:507-16 (2013)
DOI:10.1111/jop.12030
文献    
  著者
Sarraf P, Sneller MC
  タイトル
Pathogenesis of Wegener's granulomatosis: current concepts.
  雑誌
Expert Rev Mol Med 7:1-19 (2005)
DOI:10.1017/S146239940500921X
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