Takeda T, Takeda T, Naiki Y, Yonekawa S, Sakaguchi M, Iwamoto I, Tanaka H, Hasegawa H, Imada A, Kanamaru A, Hiruma S, Maekura S, Hashimoto S, Yamazumi T
Third Department of Internal Medicine, Kinki University School of Medicine, Osaka, Japan.
Nihon Jinzo Gakkai Shi. 1998 Nov;40(8):591-6.
A 68-year-old woman was admitted to Kinki University Hospital because of progressive renal failure. She had been well until two months before admission. Laboratory data were as follows: serum creatinine 4.1 mg/dl, BUN 69 mg/dl, MPO-ANCA 33 EU, anti-glomerular basement membrane antibodies (AGBMA) 118 U. Histological findings showed cellular and fibrocellular crescents in many glomeruli. Therefore, we diagnosed rapidly progressive glomerulonephritis (RPGN) due to MPO-ANCA and anti-GBM associated renal disease. The patient was started on prednisolone and double filtration plasmapheresis (DFPP) therapy. Subsequently, the values of MPO-ANCA and AGBMA decreased. However, the patient's condition suddenly worsened and she died of interstitial pneumonia. Autopsy examination revealed crescentic glomerulonephritis and alveolar hemorrhage with linear deposition of IgG along the glomerular and alveolar capillary walls by immunofluorescence studies. We considered this to be a rare case of Goodpasture's syndrome associated with not only anti-GBM antibodies, but also MPO-ANCA.
一名68岁女性因进行性肾衰竭入住近畿大学医院。入院前两个月她身体状况良好。实验室检查数据如下:血清肌酐4.1mg/dl,血尿素氮69mg/dl,髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)33EU,抗肾小球基底膜抗体(AGBMA)118U。组织学检查发现许多肾小球有细胞性和纤维细胞性新月体形成。因此,我们诊断该患者为MPO-ANCA和抗肾小球基底膜相关肾病所致的急进性肾小球肾炎(RPGN)。患者开始接受泼尼松龙和双重滤过血浆置换(DFPP)治疗。随后,MPO-ANCA和AGBMA的值下降。然而,患者病情突然恶化,死于间质性肺炎。尸检显示为新月体性肾小球肾炎,免疫荧光研究显示沿肾小球和肺泡毛细血管壁有IgG线状沉积的肺泡出血。我们认为这是一例罕见的Goodpasture综合征,不仅与抗肾小球基底膜抗体有关,还与MPO-ANCA有关。