What is the prognosis of Rapidly progressive glomerulonephritis (RPGN)?
The prognosis and response to treatment in patients with anti-GBM antibody or Goodpasture disease have not been studied in large trials.
Data from a number of small case series with similar, but not identical, treatment strategies suggest that patient survivals are high (70% to 90%). Overall, only 40% of patients remain off dialysis 1 year after presentation.
However, patients who do not require dialysis and who are treated with immunosuppression and plasma exchange have 1-year kidney survivals of approximately 70% to 75%, even if kidney failure is severe. In contrast, kidney survival is poor in patients with anti-GBM antibody-associated disease, who require dialysis within 72 hours of presentation.
Aggressive therapy with immunosuppressive drugs and plasma exchange may not be appropriate in this subgroup of anti-GBM antibody patients, unless significant acute tubular necrosis, in addition to crescentic nephritis, is demonstrated on kidney biopsy.
Clinical, laboratory, and pathologic parameters do not have sufficient predictive value for kidney outcomes to be used in an individual patient.
Although data on patients with kidney-limited, ANCA-positive RPGN are limited, treatment responses have been recently reported in several cohorts of patients with ANCA-associated necrotizing glomerulonephritis and either GPA or MPA.
Many patients (approximately 75%) achieve remission after induction therapy, but only 40% to 50% remain in long-term remission after 4 to 10 years.
Serum creatinine at presentation is the strongest predictor of kidney survival in patients who are ANCA positive. In contrast to patients with anti-GBM glomerulonephritis, patients with ANCA-associated glomerulonephritis can respond to therapy even if they have already required the initiation of dialysis.