Background: Over the past 14 years, important advances have been made in diagnosis and treatment of patients with pauci-immune necrotizing glomerulonephritis (PINGN). The present study set out to evaluate the impact of these advances on prognosis by comparing patient survival during the period 1985-1995 with previously reported results for such patients between 1975 and 1982.
Method: A retrospective analysis was carried out at two affiliated inner-city renal units on all patients considered to have PINGN during the period 1985 1995. Details of renal and extra-renal disease at presentation and during follow-up, along with treatment regimes, were noted. Figures for renal and patient survival were compared with those previously reported from one of these units.
Results: A total of 47 patients were diagnosed over the period 1985 1995, with a median age of 57 years. The overall patient survival (+/- standard error) at 1 and 5 years was 72.3 (+/- 0.06) and 51.2% (+/- 0.12) respectively, with corresponding renal survival (alive and independent of renal replacement therapy) at these times of 61.7 (+/- 0.07) and 49.9% (+/- 0.09) respectively. We identified increased age at presentation and advanced renal failure (requiring dialysis or serum creatinine > 300 micromol/l) as predictors of reduced patient and renal survival. When comparing our results with those previously reported (1975-1982), we found no improvement in prognosis for patients with PINGN during the latter period.
Conclusions: These results suggest that the prognosis for patients with PINGN has not improved despite diagnostic and therapeutic advances. Delay in diagnosis and treatment may compromise the therapeutic potential in PINGN.