title: Anti-glomerular basement membrane antibody disease with granulomatous lesions on renal biopsy.
abstract: We present the case of a 56-year-old woman with anti-glomerular basement membrane (anti-GBM) antibody disease accompanied by granulomatous reaction in the kidney. Three months prior to admission to our kidney center, she had suffered from interstitial pneumonia and had a slightly elevated level of MPO-ANCA (13 EU). Her serum level of creatinine was normal (0.72 mg/dl) but proteinuria (1+) and hematuria (2+, 1-4/HF) were present. She was admitted to our hospital because of general fatigue, loss of appetite, high fever (over 38.5 degrees C) and a rapid decline in renal function (creatinine 8.50 mg/dl). Hemodialysis therapy was started immediately after admission. The serological study was negative for MPO-ANCA and PR3-ANCA but positive for anti-GBM antibody (139 EU). Renal biopsy demonstrated necrotizing glomeruli, cellular crescents and grauloma formation with multinucleated giant cells. Immunofluorescence microscopy revealed linear staining of IgG and C3. We diagnosed graulomatous, crescentic and necrotizing glomerulonephritis, patho-logically. She was diagnosed as having anti-GBM antibody disease because alveolar hemorrhage was absent. Steroid therapy including methylprednisolone pulse therapy (500 mg/day, 3 days) and 2 courses of plasma exchange were effective in reducing the fever, anti-GBM antibody titer and C-reactive protein level. Her renal function recovered and she was able to quit hemodialysis therapy 68 days after the start of hemodialysis and she has shown no signs of pulmonary alveolar hemorrhage to date. The present case suggests that intensive therapy may restore renal function in anti-GBM disease even though renal function was sufficiently damaged and required hemodialysis therapy and active pathological changes were observed in renal biopsy specimens.
authors: Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo.
authors: Masaki
authors: M
authors: Takahashi
authors: Shigeru
authors: S
authors: Otsubo
authors: Takashi
authors: T
authors: Takei
authors: Hidekazu
authors: H
authors: Sugiura
authors: Kazuhiko
authors: K
authors: Yoshida
authors: Noriko
authors: N
authors: Tamei
authors: Minako
authors: M
authors: Koike
authors: Keiko
authors: K
authors: Uchida
authors: Wako
authors: W
authors: Yumura
authors: Shunji
authors: S
authors: Kawamura
authors: Shigeru
authors: S
authors: Horita
authors: Takashi
authors: T
authors: Akiba
authors: Kosaku
authors: K
authors: Nitta
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000473:pathology* / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis; D008875:Middle Aged; D011859:Radiography; D016896:Treatment Outcome