Clinical Presentation and Outcomes of Antineutrophil Cytoplasmic Autoantibody-Negative Pauci-Immune Glomerulonephritis

The precise pathophysiology and factors contributing to the clinical phenotype of ANCA-negative PIGN remain unclear and potentially represent a distinct disease entity. Adverse outcomes may result from delays in diagnosis, advanced disease at presentation, and less intense immunosuppressive treatment. Current classification criteria inadequately address ANCA-negative disease and collaborative research, which includes ANCA-negative patients in trials is needed.