Diagnosis and classification of Goodpasture's disease (anti-GBM)
Diagnosis and classification of Goodpasture's disease (anti-GBM)
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DOI：
10.1016/j.jaut.2014.01.024
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发表时间：
2014-02-01
影响因子：
12.8
通讯作者：
Segelmark, Marten
中科院分区：
文献类型：
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作者：
Hellmark, Thomas;Segelmark, Marten
Goodpasture病或抗肾小球基底膜病（anti-GBM-disease）属于免疫复合体小血管病变。抗GBM疾病的定义是一种影响肾小球毛细血管、肺毛细血管或两者的血管炎，伴有GBM自身抗体的沉积。这种疾病是一种自身免疫性疾病的原型，患者产生自身抗体，结合基底膜并激活补体系统的经典途径，从而开始嗜中性粒细胞依赖性炎症。抗gbm疾病的诊断依赖于检测抗gbm抗体并伴有肾小球肾炎和/或肺泡炎。明显的临床症状在肾小球最突出，炎症通常导致严重的快速进展的肾小球肾炎。尽管有现代治疗，但在6个月的随访后，只有不到三分之一的患者存活并保留了肾功能。频率从每年每百万居民0.5例到1例不等，与HLA-DRB1*1501和DRB1*1502有很强的遗传联系。从本质上讲，抗gbm疾病现在是一个更喜欢的术语早期被称为good牧草综合征或good牧草病；抗gbm疾病现在被归类为由原位免疫复合物形成引起的小血管炎；诊断依赖于组织或循环中抗gbm的检测，并伴有肺泡或肾小球疾病；治疗只有在早期发现时才有效，因此必须高度认识以发现这些罕见病例；20-35%的患者同时具有抗gbm和MPO-ANCA，这就需要在肾脏疾病患者进行急性ANCA检测时进行抗gbm检测。(C) 2014 Elsevier Ltd.版权所有。
Goodpasture's disease or anti-glomerular basement membrane disease (anti-GBM-disease) is included among immune complex small vessel vasculitides. The definition of anti-GBM disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM auto-antibodies. The disease is a prototype of autoimmune disease, where the patients develop auto-antibodies that bind to the basement membranes and activate the classical pathway of the complement system, which start a neutrophil dependent inflammation. The diagnosis of anti-GBM disease relies on the detection of anti-GBM antibodies in conjunction with glomerulonephritis and/or alveolitis. Overt clinical symptoms are most prominent in the glomeruli where the inflammation usually results in a severe rapidly progressive glomerulonephritis. Despite modern treatment less than one third of the patients survive with a preserved kidney function after 6 months follow-up. Frequencies vary from 0.5 to 1 cases per million inhabitants per year and there is a strong genetic linkage to HLA-DRB1*1501 and DRB1*1502. Essentially, anti-GBM disease is now a preferred term for what was earlier called Goodpasture's syndrome or Goodpasture's disease; anti-GBM disease is now classified as small vessel vasculitis caused by in situ immune complex formation; the diagnosis relies on the detection of anti-GBM in tissues or circulation in conjunction with alveolar or glomerular disease; therapy is effective only when detected at an early stage, making a high degree of awareness necessary to find these rare cases; 20-35% have anti-GBM and MPO-ANCA simultaneously, which necessitates testing for anti-GBM whenever acute test for ANCA is ordered in patients with renal disease. (C) 2014 Elsevier Ltd. All rights reserved.