Abstract: Antineutrophil cytoplasmic antibody associated glomerulonephritis (AAGN) is a group of disorders mediated by antineutrophil cytoplasmic antibody (ANCA), pathologically characterized by pauci-immune necrotizing crescentic glomerulonephritis， its main clinical manifestation is rapidly progressive glomerulonephritis, with some patients rapidly progressing to end-stage kidney disease. Timely and accurate identification of prognostic indicators is vital for guiding clinicians- decision-making on treatment and improving patients- prognosis. The recently developed AAGN renal risk score (ARRS) incorporated the percentage of normal glomeruli, percentage of tubular atrophy and interstitial fibrosis and estimated glomerular filtration rate at diagnosis to predict kidney survival.This review focuses on the latest research progress of the ARRS model.

Key words: antineutrophil cytoplasmic antibody associated glomerulonephritis, end-stage kidney disease, renal risk score, risk prediction model