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

In a multicentre cohort of 259 patients with biopsy-proven pauci-immune glomerulonephritis, those without detectable ANCA presented younger, with worse kidney function and more proteinuria, and reached end-stage kidney disease more often at 1 and 3 years despite relapsing less. They also received standard induction and maintenance immunosuppression less often than ANCA-positive patients.