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.