Tsukada Yukiko, Wakabayashi Yoshinori, Suetsugu Yasuko, Hamaguchi Kinichi, Fukui Akira, Ogura Makoto, Hosoya Tatsuo
Department of Nephrology, Numazu City Hospital, Shizuoka, Japan.
Nihon Jinzo Gakkai Shi. 2008;50(1):51-8.
Case 1: A 38-year-old female with a history of tonsillitis and sinusitis was admitted to our hospital because of lung edema. On admission, her serum creatinine increased to 5.57 mg/dL. Hypocomplementemia was not found. ASO and MPO-ANCA were 24 U/mL and 12 EU, respectively. She underwent emergency hemodialysis. Renal biopsy showed global sclerosis and fibrocellular crescentic formation, and marked angionecrosis was noted by light microscopy. Granular deposition of C3, IgG and IgM was seen along the capillary walls on immunofluorescence study. Glomerular intramembranous deposits were scattered on electron microscopy. She was treated with intravenous methylprednisolone pulse therapy, and following oral prednisolone administration was decreased gradually. No therapeutic effects were observed, and intermittent hemodialysis was continued and became maintenance hemodialysis therapy. Case 2: A 28-year-old female suffering from both pharyngitis and acute renal failure with systemic edema was admitted to our hospital. On admission, her serum creatinine and ASO were 4.31 mg/dL and 239 U/mL, respectively. MPO-ANCA was negative and CH50 was normal. Hemodialysis was initiated on the 6th hospital day. In renal biopsy, most glomeruli showed cellular crescentic formation, and marked angionecrosis was noted by light microscopy. Global sclerosis was not found in this case. Granular deposition of C3 resembling a starry sky pattern was seen along the capillary walls on immunofluorescence study. Electron microscopy revealed scattered glomerular subepithelial deposits. She was treated with intravenous methylprednisolone pulse therapy and then oral prednisolone administration. Because of the gradual improvement in renal function, hemodialysis was terminated after 53 sessions, however, the patient's chronic renal failure has persisted to date. In these two cases, the pathological findings supported the diagnosis of severe acute post-infectious glomerulonephritis with the characteristic crescentic and necrotizing glomerulonephritis with C3 deposition.
病例1：一名38岁女性，有扁桃体炎和鼻窦炎病史，因肺水肿入住我院。入院时，她的血清肌酐升至5.57mg/dL。未发现补体血症。抗链球菌溶血素O（ASO）和髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA）分别为24U/mL和12EU。她接受了紧急血液透析。肾活检显示全球硬化和纤维细胞新月体形成，光镜下可见明显的血管坏死。免疫荧光研究显示C3、IgG和IgM沿毛细血管壁呈颗粒状沉积。电镜下可见肾小球内皮下散在沉积物。她接受了静脉注射甲泼尼龙冲击治疗，随后口服泼尼松龙剂量逐渐减少。未观察到治疗效果，继续进行间歇性血液透析并转为维持性血液透析治疗。病例2：一名28岁女性，患有咽炎和急性肾衰竭并伴有全身性水肿，入住我院。入院时，她的血清肌酐和ASO分别为4.31mg/dL和239U/mL。MPO-ANCA阴性，总补体活性（CH50）正常。在住院第6天开始进行血液透析。肾活检显示，大多数肾小球呈细胞性新月体形成，光镜下可见明显的血管坏死。该病例未发现全球硬化。免疫荧光研究显示C3沿毛细血管壁呈类似星空图案的颗粒状沉积。电镜显示肾小球上皮下散在沉积物。她接受了静脉注射甲泼尼龙冲击治疗，然后口服泼尼松龙。由于肾功能逐渐改善，53次血液透析后终止透析，然而，患者的慢性肾衰竭至今仍持续存在。在这两个病例中,病理结果支持了重症急性感染后肾小球肾炎伴特征性新月体和坏死性肾小球肾炎及C3沉积的诊断。
