PDF(Pubmed)
Abstract：
A 79-year-old man presented with dyspnea upon exertion, marked renal dysfunction, proteinuria, and hematuria. He was diagnosed with rapidly progressive glomerulonephritis. Serological tests were positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibodies. Since the anti-GBM antibody titer was significantly higher than the ANCA titer and the renal dysfunction was severe, we initially assumed anti-GBM disease and started treatment. Due to poor general condition, a definitive diagnosis could not be made by renal biopsy. Corticosteroid therapy, plasmapheresis, and cyclophosphamide treatment were performed. However, renal function did not improve, and hemodialysis was required. He died of sepsis during treatment. An autopsy was performed with the consent of the family. Renal pathological examination revealed fibrocellular crescent formation in the glomeruli. Immunofluorescence revealed no major deposition in the glomeruli, suggesting ANCA-associated nephritis but not anti-GBM disease. Gross pathological findings of the abdominal aorta showed that a part of the artificial blood vessel had formed a pseudoaneurysm and abscess. There is no evidence of inflammatory cell infiltration or vasculitis in the alveoli. Pathological findings in the other organs did not suggest vasculitis. The renal prognosis of this case could have been improved with appropriate treatment if early diagnosis by renal biopsy had been made. There have been case reports of triple-seropositive rapid progressive glomerulonephritis (RPGN). We report a rare autopsy case of triple-seropositive RPGN.
摘要：
一名79岁的男子在劳累时出现呼吸困难，明显的肾功能障碍，蛋白尿,还有血尿.他被诊断为快速进展性肾小球肾炎。MPO-ANCA血清学检测呈阳性,PR3-ANCA,和抗GBM抗体。由于抗GBM抗体效价明显高于ANCA效价,肾功能严重,我们最初假设抗GBM疾病并开始治疗.由于一般情况较差，肾活检无法做出明确诊断.皮质类固醇治疗,血浆置换,和环磷酰胺治疗。然而,肾功能没有改善,需要血液透析.他在治疗期间死于败血症。经家属同意进行尸检。肾脏病理检查显示肾小球中纤维细胞新月形形成。免疫荧光显示肾小球中没有主要沉积，提示ANCA相关性肾炎，而不是抗GBM疾病。腹主动脉的大体病理发现显示部分人工血管已形成假性动脉瘤和脓肿。在肺泡中没有炎症细胞浸润或血管炎的证据。其他器官的病理发现未提示血管炎。如果通过肾活检进行早期诊断，则可以通过适当的治疗来改善该病例的肾脏预后。已有三联血清阳性快速进展性肾小球肾炎（RPGN）的病例报道。我们报告了罕见的三血清阳性RPGN尸检病例。
