Department of Nephrology, Clinical Sciences in Lund, BMC-B13, Lund University, 221 84 Lund, Sweden.
Division of Drug Research/Nephrology, Department of Medical and Health Sciences, Linköping University, 581 85 Linköping, Sweden.
J Autoimmun. 2014 Feb-Mar;48-49:108-12. doi: 10.1016/j.jaut.2014.01.024. Epub 2014 Jan 21.
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 autoantibodies. The disease is a prototype of autoimmune disease, where the patients develop autoantibodies 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.
Goodpasture 病或抗肾小球基底膜病（抗-GBM 病）属于免疫复合物性小血管血管炎。抗-GBM 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，伴抗-GBM 自身抗体在 GBM 沉积。该疾病是自身免疫疾病的一个范例，患者会产生与基底膜结合并激活补体经典途径的自身抗体，从而引发中性粒细胞依赖的炎症。抗-GBM 病的诊断依赖于抗-GBM 抗体的检测，同时结合肾小球肾炎和/或肺泡炎。明显的临床症状主要出现在肾小球，炎症通常导致严重的快速进行性肾小球肾炎。尽管采用现代治疗方法，在 6 个月的随访后，不到三分之一的患者能够保留肾功能存活。其发病率为每年每百万居民 0.5 至 1 例，与 HLA-DRB1(∗)1501 和 DRB1(∗)1502 存在强烈的遗传关联。从本质上讲，抗-GBM 病现在是以前称为 Goodpasture 综合征或 Goodpasture 病的首选术语；抗-GBM 病现在被归类为原位免疫复合物形成引起的小血管血管炎；诊断依赖于在组织或循环中检测到抗-GBM 与肺泡或肾小球疾病的结合；只有在早期发现时治疗才有效，因此必须高度警惕以发现这些罕见病例；20-35%的患者同时存在抗-GBM 和 MPO-ANCA，因此在对有肾脏疾病的患者进行急性 ANCA 检测时，需要同时检测抗-GBM。
