Plasma exchange and immunosuppressive drugs in the treatment of glomerulonephritis due to antibodies to the glomerular basement membrane.
Glomerulonephritis due to autoantibodies to the glomerular basement membrane (GBM) usually results in the rapid development of renal failure in untreated patients. We report our experience of the use of plasma exchange and immunosuppressive drugs in the management of this condition. Treatment consisted of daily 4 litre plasma exchanges for plasma protein fraction, together with prednisolone 60 mg daily (reducing to 20 mg daily by 4 weeks), cyclophosphamide 3 mg/kg daily and azathioprine 1 mg/kg daily. It was found that plasma exchange was generally required for 2 weeks, and cytotoxic drugs for 8 weeks. Forty four patients, aged 4 to 72 years, were treated. Anti-GBM antibody production was rapidly controlled, and in 21 of 34 patients antibody was undetectable within 8 weeks. Of 22 oliguric patients, none recovered renal function, 16 remained dialysis-dependent and 6 died; of 5 patients whose initial plasma creatinine was greater than 600 mumol/l, one improved and 4 proceeded to dialysis; and of 17 patients whose creatinine was less than 600 mumol/l, 15 recovered renal function, one became dialysis-dependent and one died. Long term follow-up of those who improved revealed that 2/16 subsequently deteriorated. Our results represent a great improvement in the prognosis of anti-GBM disease, and demonstrate that the majority of patients will recover renal function if treated before irreversible glomerular damage has occurred.
D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis / Q000276:immunology*; D001485:Basement Membrane / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000188:drug therapy / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007678:Kidney Glomerulus / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D003404:Creatinine