title: Antineutrophil cytoplasmic autoantibody-associated rapidly progressive glomerulonephritis in a patient with primary biliary cirrhosis.
abstract: 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.
authors.affiliation: Department of Medicine, Shinmatsudo Central General Hospital, Chiba, Japan.
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Nakamura
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kawagoe
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Ueda
authors.forename: Hikaru
authors.initials: H
authors.lastname: Koide
mesh_terms: D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D001706:Biopsy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000276:immunology* / Q000628:therapy; D008775:Methylprednisolone / Q000627:therapeutic use; D009195:Peroxidase / Q000097:blood; D010951:Plasma Exchange; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D009195:Peroxidase; D011241:Prednisone; D008775:Methylprednisolone