Case Studies
Case 9.4 Anti-glomerular basement membrane glomerulonephritis
A 55-year-old man presented with a 3-week history of malaise, nausea, fever and shivering. Although there were no urinary symptoms, analysis of a mid-stream urine specimen showed microscopic haematuria. There was no cough or haemoptysis and no family history of renal disease or hypertension. On examination, he was mildly pyrexial but there were no vasculitic lesions, oedema or hypertension. Cystoscopy and renal ultrasound showed no cause for his haematuria. Over the next week, his blood urea rose steadily from 10 to 23mmol/l (NR 2.5-7.5) and the serum creatinine from 164 to 515µmol/l (NR 60-120). His haemoglobin was 89g/l with a white cell count of 10.4 x 109/l and a normal differential. His urine contained red cell casts and he rapidly became oliguric. Antinuclear antibodies, including anti-DNA antibodies, were not detected and serum C3 and C4 levels were normal.
A renal biopsy specimen contained seven glomeruli: four showed focal necrotizing glomerulonephritis with epithelial crescents but the remaining three were normal. On immunofluorescence, linear staining with IgG was present along the glomerular capillary basement membrane. The patient's serum contained antibodies to GBM (see Chapter 19). The diagnosis was therefore rapidly progressive glomerulonephritis due to antibodies to GBM. Although oliguric, he was treated with high doses of prednisolone and cyclophosphamide, and underwent daily plasma exchanges for 2 weeks, until anti-GBM antibodies were no longer detectable. However, renal function failed to recover: cytotoxic therapy was stopped and regular haemodialysis started.
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