Antineutrophil cytoplasmic autoantibody-associated rapidly progressive glomerulonephritis in a patient with primary biliary cirrhosis.
Antineutrophil cytoplasmic autoantibody-associated rapidly progressive glomerulonephritis in a patient with primary biliary cirrhosis.
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原发性胆汁性肝硬化患者中抗中性粒细胞胞质自身抗体相关的快速进展性肾小球肾炎。
DOI：
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发表时间：
2004
影响因子：
3.1
通讯作者：
H. Koide
中科院分区：
文献类型：
--
作者：
Tsukasa Nakamura;Y. Kawagoe;Y. Ueda;H. Koide
一个72岁的女性原发性胆汁性肝硬化（PBC）和抗神经细胞胞质自身抗体（ANCA）相关的快速进展性肾小球肾炎的描述。她有6年的PBC病史，经组织学诊断，抗线粒体抗体检测阳性，胆汁酶活性升高。髓过氧化物酶-ANCA检测结果为阴性。患者接受熊去氧胆酸（600 mg/天）治疗，并已稳定6年。她因全身乏力、劳力性呼吸困难和周围性水肿而入院。她的血清肌酐水平升高（4.4 mg/dL），血红蛋白浓度降低（8.0 g/dL）。患者被诊断为快速进展性肾小球肾炎。血清抗核抗体和髓过氧化物酶-ANCA检测结果为阳性。肾活检诊断为坏死性新月体肾炎。给予泼尼松龙，随后给予甲基泼尼松龙冲击治疗和环磷酰胺。患者接受血浆置换，每周两次，持续4周。4周后，她的血清肌酐水平降至1.8 mg/dL，她在没有血液透析的情况下恢复了肾功能。24周后，她的肾功能（血清肌酐水平，1.6 mg/dL）稳定。
A 72-year-old woman with primary biliary cirrhosis (PBC) and antineutrophil cytoplasmic autoantibody (ANCA)-associated rapidly progressive glomerulonephritis is described. She had a 6-year history of PBC diagnosed histologically, with a positive test finding for antimitochondrial antibodies and elevated biliary enzyme activity. The myeloperoxidase-ANCA test result was negative. The patient was treated with ursodeoxycholic acid (600 mg/day) and had been stable for 6 years. She was admitted to our hospital because of general fatigue, exertional dyspnea, and peripheral edema. Her serum level of creatinine was increased (4.4 mg/dL), and her hemoglobin concentration was reduced (8.0 g/dL). The patient was diagnosed as having rapidly progressive glomerulonephritis. Test results for serum antinuclear antibody and myeloperoxidase-ANCA were positive. The diagnosis by renal biopsy was necrotizing crescentic glomerulonephritis. Prednisolone followed by methylprednisolone pulse therapy and cyclophosphamide were administered. The patient underwent plasma exchange twice weekly for 4 weeks. After 4 weeks, her serum creatinine level fell to 1.8 mg/dL, and she recovered renal function without hemodialysis. After 24 weeks, her renal function (serum creatinine level, 1.6 mg/dL) was stable.
影响因子：
39.2
作者：
FALK, RJ;HOGAN, S;JENNETTE, JC
通讯作者：
JENNETTE, JC