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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.
Biomarker and diagnostic research for Pauci-immune glomerulonephritis has been reported in the published literature.
Estimated prevalence: Unknown (Unknown prevalence).
No clinical trials have been registered for Pauci-immune glomerulonephritis.
56 publications have been identified in PubMed for Pauci-immune glomerulonephritis. Research spans Case Report / Case Series (63%), Review / Meta-Analysis (13%), and Epidemiology / Natural History (11%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 35 | 63% |
Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 6:21 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about Pauci-immune glomerulonephritis
Research summaries
7 |
13% |
Disease patterns and progression | 6 | 11% |
Other research | 3 | 5% |
Testing and diagnosis research | 2 | 4% |
Clinical study results | 1 | 2% |
Laboratory research | 1 | 2% |
New treatment approaches | 1 | 2% |
Wolfkind IB (2026). [PMID: 41549496](https://pubmed.ncbi.nlm.nih.gov/41549496/). *The American journal of case reports*. [Case Report / Case Series]
Karius AK (2026). [PMID: 42167569](https://pubmed.ncbi.nlm.nih.gov/42167569/). *Am J Med*. [Case Report / Case Series]
Yıldırım S (2026). [PMID: 41719207](https://pubmed.ncbi.nlm.nih.gov/41719207/). *Nephron*. [Diagnostic / Biomarker]
Almaani S (2026). [PMID: 42025332](https://pubmed.ncbi.nlm.nih.gov/42025332/). *Am J Kidney Dis*. [Review / Meta-Analysis]
Berrocal C (2026). [PMID: 41976855](https://pubmed.ncbi.nlm.nih.gov/41976855/). *J Clin Med*. [Case Report / Case Series]
Schreiber A (2026). [PMID: 41927844](https://pubmed.ncbi.nlm.nih.gov/41927844/). *Inn Med (Heidelb)*. [Review / Meta-Analysis]
Rodelo-Ceballos J (2026). [PMID: 41537456](https://pubmed.ncbi.nlm.nih.gov/41537456/). *Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases*. [Other]
Yow BLH (2026). [PMID: 42168832](https://pubmed.ncbi.nlm.nih.gov/42168832/). *Nephrology (Carlton)*. [Case Report / Case Series]
Chebbo L (2026). [PMID: 41446802](https://pubmed.ncbi.nlm.nih.gov/41446802/). *Biomedicine hub*. [Case Report / Case Series]
Kumru G (2026). [PMID: 41654351](https://pubmed.ncbi.nlm.nih.gov/41654351/). *Nefrologia*. [Case Report / Case Series]
AI-curated news mentioning Pauci-immune glomerulonephritis
Updated Jan 21, 2026
A recent study explores pauci-immune endocapillary proliferative glomerulonephritis, highlighting the role of glomerular M2 macrophage infiltration. This research contributes to understanding the disease's pathology and potential therapeutic targets.