| H01721 | |
| H番号 | H01721 |
| 名称 | 抗糸球体基底膜腎炎; グッドパスチャー症候群 |
| 概要 | Goodpasture syndrome (GS), or anti-glomerular basement membrane (anti-GBM) disease, is a rare and organ-specific autoimmune disease defined by anti-GBM antibody-mediated damage (mainly immunoglobulin G-1) resulting in progressive crescentic glomerulonephritis and, frequently, diffuse pulmonary alveolar hemorrhage. Clinically, GS is characterized by pulmonary hemorrhage and renal failure, although the clinical spectrum may range from only mild symptoms to a relentless and finally lethal outcome. This disease accounts for 10-20% of the patients with acute renal failure following a rapidly progressive glomerulonephritis. The disease is caused by an autoimmune response against the non-collagenase domain of the alpha 3 chain of type IV collagen [alpha3(IV)NC1], found in the glomerular and alveolar basement membranes. Without prompt diagnosis and treatment, the disease can lead to bleeding in the lungs, kidney failure, and even death. Early and intensive treatment with plasmapheresis and immunosuppression with systemic corticosteroids pending results of diagnostic testing, and later cyclophosphamide, is often beneficial, with 90% of patients surviving the acute presentation of GS. |
| 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 | - |
| コメント | Autoantigen: COL4A3 [HSA:1285] [KO:K06237] See also H01688 Rapidly progressive glomerulonephritis (RPGN). |
| リンク | ICD-11: MF85 ICD-10: M31.0 N08.5 MeSH: D019867 |
| 文献 | PMID:18502098 著者 Ooi JD, Holdsworth SR, Kitching AR タイトル Advances in the pathogenesis of Goodpasture's disease: from epitopes to autoantibodies to effector T cells. 雑誌 J Autoimmun 31:295-300 (2008) DOI:10.1016/j.jaut.2008.04.005 PMID:21207195 著者 Chan AL, Louie S, Leslie KO, Juarez MM, Albertson TE タイトル Cutting edge issues in Goodpasture's disease. 雑誌 Clin Rev Allergy Immunol 41:151-62 (2011) DOI:10.1007/s12016-010-8222-2 PMID:23806563 著者 Dammacco F, Battaglia S, Gesualdo L, Racanelli V タイトル Goodpasture's disease: a report of ten cases and a review of the literature. 雑誌 Autoimmun Rev 12:1101-8 (2013) DOI:10.1016/j.autrev.2013.06.014 PMID:25462583 著者 Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G, Martellucci S, Turchetta R, Longo L, De Vincentiis M タイトル Goodpasture's syndrome: a clinical update. 雑誌 Autoimmun Rev 14:246-53 (2015) DOI:10.1016/j.autrev.2014.11.006 |