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pauci-immune glomerulonephritis without ANCA - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

pauci-immune glomerulonephritis without ANCA

MONDO:0019989

Also known as:antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritispauci-immune glomerulonephritis without antineutrophil cytoplasmic antibody

Overview

Pauci-immune glomerulonephritis (GN) without antineutrophilic cytoplasmic antibodies (ANCA) is a form of rapidly progressive glomerulonephritis comprising 10-43% of pauci-immune glomerulonephritis and characterized by the absence of ANCA. In comparison with pauci-immune GN with ANCA, patients lacking ANCA may be younger at onset of the disease and have a shorter interval from onset of the disease to diagnosis. They have fewer extra renal manifestations (e.g. involvement of lung, eye, ear, nose and throat), fewer constitutional symptoms (e.g. fever, weight loss, muscle pain and arthralgia) and a high prevalence of nephrotic syndrome and chronic renal lesions. Their prognosis is generally poorer.

Diagnosis

Biomarker and diagnostic research for pauci-immune glomerulonephritis without ANCA has been reported in the published literature.

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for pauci-immune glomerulonephritis without ANCA.

109 publications have been identified in PubMed for pauci-immune glomerulonephritis without ANCA. Research spans Case Report / Case Series (64%), Review / Meta-Analysis (16%), and Epidemiology / Natural History (12%).

Research Type	Count	% of Total
Patient case studies	70	64%

Data assembled from 3 of 12 sources · Last updated Sep 19, 2026, 6:28 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:97564

GARD

Genetic and Rare Diseases Info Center

GARD:19380

Questions for Your Care Team

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Kisho

pauci-immune glomerulonephritis without ANCA

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Recent Publications

Latest News & Developments

Pauci-Immune Endocapillary Proliferative Glomerulonephritis With Glomerular M2 Macrophage Infiltration.

pauci-immune glomerulonephritis without ANCA

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Recent Publications

Latest News & Developments

Pauci-Immune Endocapillary Proliferative Glomerulonephritis With Glomerular M2 Macrophage Infiltration.