A case of anti-GBM-antibody positive rapidly progressive glomerulonephritis who was weaned from hemodialysis after combination therapy with steroid and plasmapheresis

摘要

We report an anti-GBM antibody-positive crescentic glomerulonephritis patient who benefitted from maintenance hemodialysis 4 months after the initial treatment, which included steroid pulse therapy and plasma exchange. A-29-year-old male was referred to our hospital because of high fever, abnormal urinary findings (leukocytes 3+, protein 2+, occult blood 3+) and a moderate degree of azotemia(S-Cr 2.9 mg/dl). C-reactive protein (CRP) was 18.9 mg/dl and antibiotics were administered intravenously for 7 days under the diagnosis of pyelonephritis. High fever persisted, however, and S-Cr increased to 9.2 mg/dl even though a sufficient volume of urine was maintained. Blood and urine cultures were negative for bacteria. A kidney biopsy was performed and cellular crescents were observed around the glomeruli. No abnormal finding was observed in the lung and the nasopharyngeal region. To treat the crescentic glomerulonephritis, steroid and cyclophosphamide were administered while hemodialysis was carried out simultaneously. Although P-ANCA and C-ANCA were negative, anti-GBM antibody was proven to be positive thereafter (169 U) and six sessions of plasmapheresis were additionally performed to remove the antibody. Two months after the last plasmapheresis, the reduced urine volume (300 ml/day) gradually returned to normal. Hemodialysis was terminated because the S-Cr concentration reached a plateau at 4 mg/dl. Repeated biopsy revealed marked glomerulosclerosis, hence hypertension treatment and a low protein diet were ordered. In conclusion, residual renal function might improve even after 4 months of hemodialysis in cases of intensively treated anti-GBM-positive crescentic glomerulonephritis, though consecutive renoprotective therapy is required.