| H01655 | |
| H番号 | 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 | 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] 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] アバコパン [DR:D11093] アザチオプリン [DR:D00238] シクロホスファミド水和物 [DR:D00287] リツキシマブ [DR:D02994] |
| コメント | - |
| リンク | ICD-11: 4A44.A1 ICD-10: M31.3 MeSH: D014890 |
| 文献 | PMID:23301777 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 PMID:15890083 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 |