KEGG   DISEASE: IgA 血管炎
エントリ  
H01584                                                             
名称    
IgA 血管炎;
ヘノッホ・シェーンライン紫斑病
概要    
IgA vasculitis (IgAV), also known as Henoch-Schonlein purpura (HSP), is the most common systemic small vessel vasculitis in childhood with clinical characteristics of non-thrombocytopenic palpable purpura, arthritis, and involvement of internal organs such as gastrointestine (GI) and kidney. IgAV has been associated with a history of preceding infections, especially upper respiratory tract infection. In addition, other characteristics of IgAV include the deposition of IgA and C3 in small vessel walls, polymorphonuclear neutrophil infiltration around the vessel and in vessel walls, and increased serum levels of IgA and proinflammatory cytokines at the acute stage. Combined, IgAV is regarded as a specific immune-mediated entity induced by environmental factors, particularly infections. Clinically, since there are no disease-specific laboratory abnormalities, IgAV is currently diagnosed based on symptoms and signs and histopathological findings. Treatment is supportive because IgAV is usually self-limiting except for serious GI or renal involvement.
カテゴリ  
循環器疾患
階層分類  
ヒト疾患 [BR:jp08402]
 循環器系疾患
  血液疾患
   H01584  IgA 血管炎
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
    H01584  IgA 血管炎
治療薬   
デキサメタゾン [DR:D00292]
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 [DR:D00385]
ベタメタゾン [DR:D00244]
プレドニゾロン [DR:D00472]
メチルプレドニゾロン [DR:D00407]
メチルプレドニゾロン酢酸エステル [DR:D00979]
乾燥濃縮人血液凝固第XIII因子 [DR:D08802]
リンク   
ICD-11: 4A44.92
ICD-10: D69.0
MeSH: D011695
文献    
  著者
Yang YH, Yu HH, Chiang BL
  タイトル
The diagnosis and classification of Henoch-Schonlein purpura: an updated review.
  雑誌
Autoimmun Rev 13:355-8 (2014)
DOI:10.1016/j.autrev.2014.01.031
文献    
  著者
He X, Yu C, Zhao P, Ding Y, Liang X, Zhao Y, Yue X, Wu Y, Yin W
  タイトル
The genetics of Henoch-Schonlein purpura: a systematic review and meta-analysis.
  雑誌
Rheumatol Int 33:1387-95 (2013)
DOI:10.1007/s00296-012-2661-4
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