Has the prognosis for patients with pauci-immune necrotizing glomerulonephritis improved?

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.

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.

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.

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.