Fukuda Y, Yamanaka N, Ishizaki M, Suzuki T, Masugi Y, Yajima G, Nagata T
Acta Pathol Jpn. 1982 Mar;32(2):361-70. doi: 10.1111/j.1440-1827.1982.tb02056.x.
An autopsy case of what was clinically considered to be Goodpasture's syndrome was investigated. The lung had hemorrhagic interstitial pneumonia, showing granular patterns of IgG and C3 along the alveoli by the immunofluorescent method and electron-dense subepithelial deposits by electron microscopy. The kidney had crescentic and segmental necrotizing glomerulonephritis associated with membranous nephropathy. Uneven, continuous patterns of immunofluorescent IgG and C3 along the GBM were noted. Electron microscopy showed numerous subepithelial deposits, and immunoelectron microscopy revealed that IgG was not present in the GBM itself but present in the subepithelial deposits. Anti-GBM antibody activity was not detected in the serum or the kidney eluate. It was suggested that renal and pulmonary lesions occurred through the same mechanism and in association with immune deposits. We propose that there is a disease having immune complex-mediated deposits. We propose that there is a disease having immune complex-mediated renal and pulmonary lesions which clinically resembles the conventional Goodpasture's syndrome.
对一例临床上被认为是古德帕斯彻综合征的尸检病例进行了研究。肺部有出血性间质性肺炎，免疫荧光法显示沿肺泡有IgG和C3颗粒状沉积，电子显微镜检查显示有电子致密的上皮下沉积物。肾脏有新月形和节段性坏死性肾小球肾炎伴膜性肾病。沿肾小球基底膜（GBM）观察到IgG和C3免疫荧光不均匀、连续的沉积模式。电子显微镜显示有大量上皮下沉积物，免疫电子显微镜显示GBM本身不存在IgG，而是存在于上皮下沉积物中。血清或肾脏洗脱液中未检测到抗GBM抗体活性。提示肾脏和肺部病变通过相同机制发生并与免疫沉积物有关。我们提出存在一种由免疫复合物介导沉积的疾病。我们提出存在一种临床上类似于传统古德帕斯彻综合征的、由免疫复合物介导肾脏和肺部病变的疾病。
