| H01658 | |
| H番号 | H01658 |
| 名称 | 顕微鏡的多発血管炎 |
| 概要 | Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. MPA predominantly affects small-calibre blood vessels and is associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). The association with ANCAs originally defined the group of ANCA-associated vasculitides, comprising granulomatosis with polyangitis (GPA, formerly known as Wegener granulomatosis) [DS:H01655], microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) [DS:H01468], which have different frequencies of ANCA-positivity. ANCA in MPA are predominantly directed against myeloperoxidase (MPO-ANCA) but may, in a minority of patients, be directed against proteinase 3 (PR3-ANCA). Not all patients, however, have ANCA. MPA is clinically characterized by small-vessel vasculitis primarily affecting the kidneys and the lungs but other organs may be involved as well. Renal involvement, which can be the only manifestation, is clinically apparent as rapidly progressive glomerulonephritis and histopathologically as pauci-immune necrotizing and crescentic glomerulonephritis. Diagnosis is mainly established by clinical manifestations, computed tomography (TC), ANCA antibody detection, and renal and pulmonary biopsy. The introduction of aggressive immunosuppressive treatment has substantially improved the prognosis. The standardized therapeutic regimen is based on cyclophosphamide and corticosteroids. Using this regimen, remission can be achieved in most of the patients. Rituximab may represent an important alternative to cyclophosphamide for patients who may not respond adequately to antimetabolite therapies. |
| 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] 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コハク酸エステルナトリウム [DR:D00751] 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.A0 ICD-10: M31.7 MeSH: D055953 |
| 文献 | PMID:25992801 著者 Greco A, De Virgilio A, Rizzo MI, Gallo A, Magliulo G, Fusconi M, Ruoppolo G, Tombolini M, Turchetta R, de Vincentiis M タイトル Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches. 雑誌 Autoimmun Rev 14:837-44 (2015) DOI:10.1016/j.autrev.2015.05.005 PMID:24461388 著者 Kallenberg CG タイトル The diagnosis and classification of microscopic polyangiitis. 雑誌 J Autoimmun 48-49:90-3 (2014) DOI:10.1016/j.jaut.2014.01.023 |