pauci-immune glomerulonephritis with ANCA
Findings
No curated finding names pauci-immune glomerulonephritis with ANCA yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Pauci-immune glomerulonephritis (GN) with antineutrophil cytoplasmic antibodies (ANCA) is a form of rapidly progressive GN comprising about 90% of pauci-immune glomerulonephritis, and associated with the presence of circulating ANCA (mostly directed against proteinase-3 (PR3) and myeloperoxidase (MPO)). Patients usually present with hematuria and rapidly declining renal function, often leading to dialysis within weeks without treatment. Cutaneous, pulmonary, musculoskeletal and nervous involvement may be observed in case of systemic disease, and the correlation between ANCA titer and disease activity has been demonstrated.
Definition from the Mondo Disease Ontology (MONDO:0019988), read 2026-09-29. CC BY 4.0.
Where it sits
- A kind of
Other names
1 name
Resolves to: pauci-immune glomerulonephritis with ANCA
- Also called
- pauci-immune glomerulonephritis with antineutrophil cytoplasmic antibody