GOODPASTURES-SYNDROME ASSOCIATED WITH P-A NCA

We report a patient with rapidly progressive glomerulonephritis and al veolar hemorrhage. The diagnosis by renal biopsy was extracapillary gl omerulonephritis with linear deposits oi immunoglobulin G. Serological ly, anti-glomerular basement membrane antibodies and anti-neutrophil c ytoplasmic antibodies with perinuclear distribution were present. The patient was treated with steroids, cyclophosphamide, immunoadsorption and plasma exchange. Alveolar hemorrhage ceased but the patient needed dialysis. He died twelve days alter admission to hospital from lung s epsis. This case poses two questions: 1. What is the role oi P-ANCA's in Goodpasture's syndrome! 2. What is the optimum treatment and how sh ould it be monitored?