| H01584 | |
| H番号 | 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 | 免疫系疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 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] |
| コメント | - |
| リンク | ICD-11: 4A44.92 ICD-10: D69.0 MeSH: D011695 |
| 文献 | PMID:24424188 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 PMID:23325094 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 |