1] Renal Division, Department of Medicine, Peking University First Hospital, Beijing, People's Republic of China [2] Institute of Nephrology, Peking University, Beijing, People's Republic of China [3] Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, People's Republic of China [4] Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, People's Republic of China.
1] Renal Division, Department of Medicine, Peking University First Hospital, Beijing, People's Republic of China [2] Institute of Nephrology, Peking University, Beijing, People's Republic of China [3] Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, People's Republic of China [4] Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, People's Republic of China [5] Peking-Tsinghua Center for Life Sciences, Beijing, People's Republic of China.
Kidney Int. 2014 Apr;85(4):945-52. doi: 10.1038/ki.2013.364. Epub 2013 Sep 18.
The association of anti-glomerular basement membrane (GBM) disease, also known as Goodpasture's disease, with membranous nephropathy (MN) has been well documented. However, little is known about the clinical and immunological features of patients with such a combination. This study was designed to investigate the clinical and immunological features of anti-GBM patients with MN and to provide insight into the pathogenesis of this rare entity. Eight patients with combined anti-GBM disease and MN were found to have significantly lower levels of serum creatinine, a significantly lower proportion of oliguria/anuria, and significantly better renal outcomes compared with 30 patients with classical anti-GBM disease. Antibody levels against the EB conformational epitope of anti-α3(IV)NC1 were significantly lower in these patients, as was their levels of anti-α3(IV)NC1 immunoglobulin G1 (IgG1) and IgG3. Serum antibodies against the M-type phospholipase A2 receptor were undetectable in anti-GBM patients with MN but presented in 13 of the 20 patients with primary MN. Thus, patients with combined anti-GBM disease and MN have distinct clinical features and a different immunological profile of MN.
抗肾小球基底膜（GBM）病，也称为Goodpasture 病，与膜性肾病（MN）的关联已有充分的文献记载。然而，对于具有这种组合的患者的临床和免疫学特征知之甚少。本研究旨在探讨伴有 MN 的抗 GBM 病患者的临床和免疫学特征，并深入了解这一罕见实体的发病机制。发现 8 例伴有抗 GBM 疾病和 MN 的患者的血清肌酐水平明显降低，少尿/无尿的比例明显降低，肾脏结局明显好于 30 例经典抗 GBM 疾病患者。与这些患者相比，他们针对抗α3(IV)NC1 的 EB 构象表位的抗体水平明显降低，抗α3(IV)NC1 免疫球蛋白 G1（IgG1）和 IgG3 的水平也明显降低。MN 患者的抗 GBM 患者血清中抗 M 型磷脂酶 A2 受体抗体无法检测到，但在 20 例原发性 MN 患者中的 13 例中存在。因此，伴有抗 GBM 疾病和 MN 的患者具有明显不同的临床特征和 MN 的免疫学特征。
