Ogawara Aoi, Harada Makoto, Ichikawa Tohru, Fujii Kazuaki, Ehara Takashi, Kobayashi Mamoru
Department of Nephrology, Nagano Red Cross Hospital.
Department of Emergency and Critical Care Medicine, Shinshu University School of Medicine.
Tohoku J Exp Med. 2017 Dec;243(4):335-341. doi: 10.1620/tjem.243.335.
Renal prognosis for anti-glomerular basement membrane (GBM) glomerulonephritis is poor. The greater the amount of anti-GBM antibody binding the antigen (type IV collagen of the glomerular basement membrane), the greater the number of crescents that develop in glomeruli, resulting in progression of renal impairment. Immunofluorescence staining reveals linear IgG depositions on glomerular capillary walls. Membranous nephropathy (MN) is one of the most common causes of nephrotic syndrome in middle-aged to elderly patients. Immune complex is deposited in the sub-epithelial space of the glomerulus resulting in the development of a membranous lesion. Immunofluorescence staining reveals granular IgG depositions on glomerular capillary walls. Coexisting anti-GBM glomerulonephritis and MN are rare and, here we report a case of coexisting anti-GBM glomerulonephritis and MN with preserved renal function. There are some cases of coexisting anti-GBM glomerulonephritis and MN do not show severely decreased renal function. A 76-year-old Japanese woman presented with nephrotic syndrome, microscopic hematuria, and was positive for anti-GBM antibody. Kidney biopsy revealed linear and granular IgG depositions in glomerular capillary walls, crescent formations, and electron-dense deposits in the sub-epithelial space. She was diagnosed with anti-GBM glomerulonephritis and MN. Steroid and cyclosporine therapy achieved complete remission, and kidney function was preserved. In conclusion, coexisting anti-GBM glomerulonephritis and MN can have preserved renal function. IgG subclass of deposited anti-GBM antibody may be associated with the severity of anti-GBM glomerulonephritis. In addition, in the case of nephrotic syndrome with hematuria, we should consider the possibility of coexisting anti-GBM glomerulonephritis and MN.
抗肾小球基底膜(GBM)肾小球肾炎的肾脏预后较差。抗GBM抗体与抗原(肾小球基底膜的IV型胶原)结合的量越大,肾小球中形成的新月体数量就越多,从而导致肾功能进展。免疫荧光染色显示肾小球毛细血管壁上有线性IgG沉积。膜性肾病(MN)是中老年患者肾病综合征最常见的病因之一。免疫复合物沉积在肾小球上皮下间隙,导致膜性病变的发生。免疫荧光染色显示肾小球毛细血管壁上有颗粒状IgG沉积。抗GBM肾小球肾炎和MN并存的情况很少见,在此我们报告一例抗GBM肾小球肾炎和MN并存且肾功能保留的病例。有一些抗GBM肾小球肾炎和MN并存的病例并未表现出严重的肾功能下降。一名76岁的日本女性出现肾病综合征、镜下血尿,抗GBM抗体呈阳性。肾活检显示肾小球毛细血管壁有线性和颗粒状IgG沉积、新月体形成以及上皮下间隙有电子致密沉积物。她被诊断为抗GBM肾小球肾炎和MN。类固醇和环孢素治疗实现了完全缓解,肾功能得以保留。总之,抗GBM肾小球肾炎和MN并存时肾功能可能得以保留。沉积的抗GBM抗体的IgG亚类可能与抗GBM肾小球肾炎的严重程度有关。此外,对于伴有血尿的肾病综合征病例,我们应考虑抗GBM肾小球肾炎和MN并存的可能性。