Pauci-immune glomerulonephritis
Findings
No curated finding names Pauci-immune glomerulonephritis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Pauci-immune glomerulonephritis (GN) is one of the most frequent causes of rapidly progressive GN (RPGN). It is characterized clinically by renal manifestations of RPGN (hematuria, hypertension) leading to renal failure within days or weeks, and may be associated with manifestations of systemic vasculitis (arthralgia, fever, seizures, mono neuritis and lung involvement). Pauci-immune GN is histologically characterized by focal necrotizing and crescentic GN, with mild or absent glomerular staining for immunoglobulin and complement by fluorescence microscopy, which may manifest either as part of a systemic small vessel vasculitis (including microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis), or rarely as part of renal-limited vasculitis (RLV, idiopathic crescentic GN). Immunologic classification is based on the presence or absence of circulating anti-neutrophil cytoplasmic antibodies (ANCAs), namely pauci-immune-GN with ANCA and pauci-immune GN without ANCA.
Definition from the Mondo Disease Ontology (MONDO:0019641), read 2026-09-29. CC BY 4.0.
Features
35 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- GlomerulonephritisHPOHP:0000099
- Obligate (100% of cases)
- Cytoplasmic antineutrophil antibody positivityHPOHP:0032230
- Very frequent (80% to 99% of cases)
- Decreased glomerular filtration rateHPOHP:0012213
- Very frequent (80% to 99% of cases)
- Elevated circulating creatinine concentrationHPOHP:0003259
- Very frequent (80% to 99% of cases)
- Microscopic hematuriaHPOHP:0002907
- Very frequent (80% to 99% of cases)
Where it sits
- A kind of