Takahashi Masaki, Otsubo Shigeru, Takei Takashi, Sugiura Hidekazu, Yoshida Kazuhiko, Tamei Noriko, Koike Minako, Uchida Keiko, Yumura Wako, Kawamura Shunji, Horita Shigeru, Akiba Takashi, Nitta Kosaku
Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo.
Intern Med. 2007;46(6):295-301. doi: 10.2169/internalmedicine.46.6160. Epub 2007 Mar 15.
We present the case of a 56-year-old woman with anti-glomerular basement membrane (anti-GBM) antibody disease accompanied by granulomatous reaction in the kidney. Three months prior to admission to our kidney center, she had suffered from interstitial pneumonia and had a slightly elevated level of MPO-ANCA (13 EU). Her serum level of creatinine was normal (0.72 mg/dl) but proteinuria (1+) and hematuria (2+, 1-4/HF) were present. She was admitted to our hospital because of general fatigue, loss of appetite, high fever (over 38.5 degrees C) and a rapid decline in renal function (creatinine 8.50 mg/dl). Hemodialysis therapy was started immediately after admission. The serological study was negative for MPO-ANCA and PR3-ANCA but positive for anti-GBM antibody (139 EU). Renal biopsy demonstrated necrotizing glomeruli, cellular crescents and grauloma formation with multinucleated giant cells. Immunofluorescence microscopy revealed linear staining of IgG and C3. We diagnosed graulomatous, crescentic and necrotizing glomerulonephritis, patho-logically. She was diagnosed as having anti-GBM antibody disease because alveolar hemorrhage was absent. Steroid therapy including methylprednisolone pulse therapy (500 mg/day, 3 days) and 2 courses of plasma exchange were effective in reducing the fever, anti-GBM antibody titer and C-reactive protein level. Her renal function recovered and she was able to quit hemodialysis therapy 68 days after the start of hemodialysis and she has shown no signs of pulmonary alveolar hemorrhage to date. The present case suggests that intensive therapy may restore renal function in anti-GBM disease even though renal function was sufficiently damaged and required hemodialysis therapy and active pathological changes were observed in renal biopsy specimens.
我们报告一例56岁女性抗肾小球基底膜（anti-GBM）抗体病患者，其肾脏伴有肉芽肿反应。在入住我们肾脏中心前三个月，她曾患间质性肺炎，MPO-ANCA水平略有升高（13 EU）。她的血清肌酐水平正常（0.72 mg/dl），但存在蛋白尿（1+）和血尿（2+，1 - 4/高倍视野）。因全身乏力、食欲不振、高热（超过38.5摄氏度）及肾功能迅速下降（肌酐8.50 mg/dl），她入住我院。入院后立即开始血液透析治疗。血清学检查MPO-ANCA和PR3-ANCA为阴性，但抗GBM抗体阳性（139 EU）。肾活检显示肾小球坏死、细胞性新月体形成以及伴有多核巨细胞的肉芽肿形成。免疫荧光显微镜检查显示IgG和C3呈线性染色。病理诊断为肉芽肿性、新月体性和坏死性肾小球肾炎。由于无肺泡出血，她被诊断为抗GBM抗体病。包括甲泼尼龙冲击治疗（500 mg/天，3天）和2个疗程血浆置换在内的类固醇治疗有效降低了体温、抗GBM抗体滴度和C反应蛋白水平。她的肾功能恢复，血液透析开始68天后能够停止血液透析治疗，且迄今为止未出现肺泡出血迹象。本病例提示，即使肾功能已严重受损且需要血液透析治疗，并且肾活检标本中观察到活跃的病理改变，强化治疗仍可能使抗GBM病患者的肾功能恢复。
