1例抗肾小球基底膜肾小球肾炎合并膜性肾病。
A case of antiglomerular basement membrane glomerulonephritis complicated by membranous nephropathy.
作者信息
Iguchi Akira, Ishikawa Tomomi, Yamazaki Hajime, Sakamaki Yuichi, Ito Tomoyuki, Watanabe Yasuo, Saeki Takako, Ito Yumi, Imai Naohumi, Narita Ichiei
机构信息
Department of Internal Medicine, Nagaoka Red Cross Hospital, Sensyu-2 297-1, Nagaoka, Niigata, 940-2085, Japan.
Department of Internal Medicine, Ojiya General Hospital, Niigata, Japan.
出版信息
CEN Case Rep. 2014 May;3(1):94-99. doi: 10.1007/s13730-013-0094-x. Epub 2013 Oct 5.
The sequential or simultaneous presentation of anti-glomerular basement membrane (anti-GBM) glomerulonephritis with membranous nephropathy (MN) has been infrequently reported. Although the mechanism underlying MN superimposed on anti-GBM glomerulonephritis is unknown, the two entities are believed to be interrelated. We report the case of a 75-year-old woman diagnosed with rapidly progressive glomerulonephritis. Renal biopsy revealed crescentic glomerulonephritis with linear and granular staining of immunofluorescent IgG1 and IgG4 granular staining on the capillary loops. Electron microscopy revealed extensive subepithelial deposits. These findings suggested simultaneous development of anti-GBM glomerulonephritis and MN in this case. Serum phospholipase A2 receptor (PLA2R) antibody was negative. The patient was treated with prednisolone and plasma exchange, resulting in resolution of renal insufficiency and a decrease in urinary protein. The rapid decrease in urinary protein and absence of PLA2R antibody suggest that the mechanism of MN associated with anti-GBM glomerulonephritis differs from that of primary MN.
抗肾小球基底膜（anti-GBM）肾小球肾炎与膜性肾病（MN）序贯或同时出现的情况鲜有报道。尽管抗GBM肾小球肾炎叠加MN的潜在机制尚不清楚，但这两种疾病被认为是相互关联的。我们报告了一例75岁女性被诊断为快速进展性肾小球肾炎的病例。肾活检显示新月体性肾小球肾炎，免疫荧光IgG1呈线性和颗粒状染色，毛细血管袢上有IgG4颗粒状染色。电子显微镜显示广泛的上皮下沉积物。这些发现提示该病例中抗GBM肾小球肾炎和MN同时发生。血清磷脂酶A2受体（PLA2R）抗体为阴性。患者接受泼尼松龙和血浆置换治疗，肾功能不全得以缓解，尿蛋白减少。尿蛋白迅速减少且无PLA2R抗体提示与抗GBM肾小球肾炎相关的MN机制与原发性MN不同。
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