Pauci-immune glomerulonephritis

Summary

Pauci-immune glomerulonephritis (MONDO:0019641) is a disease. A subtype of glomerulonephritis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide) [Orphanet-validated]
• Phenotypes (HPO): 37

Clinical features

Signs & symptoms

Clinical features (HPO)

37 HPO clinical features (Orphanet curated; top 37 by frequency):

HPO ID	Term	Frequency
HP:0000099	Glomerulonephritis	Obligate (100%)
HP:0000083	Renal insufficiency	Very frequent (80-99%)
HP:0000093	Proteinuria	Very frequent (80-99%)
HP:0002907	Microscopic hematuria	Very frequent (80-99%)
HP:0003259	Elevated circulating creatinine concentration	Very frequent (80-99%)
HP:0011944	Small vessel vasculitis	Very frequent (80-99%)
HP:0012213	Decreased glomerular filtration rate	Very frequent (80-99%)
HP:0032230	Cytoplasmic antineutrophil antibody positivity	Very frequent (80-99%)
HP:0000096	Glomerulosclerosis	Frequent (30-79%)
HP:0000951	Abnormality of the skin	Frequent (30-79%)
HP:0001945	Fever	Frequent (30-79%)
HP:0002086	Abnormality of the respiratory system	Frequent (30-79%)
HP:0008653	Crescentic glomerulonephritis	Frequent (30-79%)
HP:0000478	Abnormality of the eye	Occasional (5-29%)
HP:0000979	Purpura	Occasional (5-29%)
HP:0001609	Hoarse voice	Occasional (5-29%)
HP:0001919	Acute kidney injury	Occasional (5-29%)
HP:0001970	Tubulointerstitial nephritis	Occasional (5-29%)
HP:0002027	Abdominal pain	Occasional (5-29%)
HP:0002087	Abnormality of the upper respiratory tract	Occasional (5-29%)
HP:0002094	Dyspnea	Occasional (5-29%)
HP:0002829	Arthralgia	Occasional (5-29%)
HP:0003493	Antinuclear antibody positivity	Occasional (5-29%)
HP:0004930	Abnormality of the pulmonary vasculature	Occasional (5-29%)
HP:0012089	Arteritis	Occasional (5-29%)
HP:0012587	Macroscopic hematuria	Occasional (5-29%)
HP:0012593	Nephrotic range proteinuria	Occasional (5-29%)
HP:0012735	Cough	Occasional (5-29%)
HP:0040223	Pulmonary hemorrhage	Occasional (5-29%)
HP:0002955	Granulomatosis	Excluded (0%)
HP:0032616	Renal interstitial immunoglobulin deposits	Excluded (0%)
HP:0000155	Oral ulcer	Very rare (<1-4%)
HP:0001626	Abnormality of the cardiovascular system	Very rare (<1-4%)
HP:0001733	Pancreatitis	Very rare (<1-4%)
HP:0011024	Abnormality of the gastrointestinal tract	Very rare (<1-4%)
HP:0032018	Multiple mononeuropathy	Very rare (<1-4%)
HP:0100532	Scleritis	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	Pauci-immune glomerulonephritis
Mondo ID	MONDO:0019641
Orphanet	93126
ICD-11	1359757645
UMLS	C0343196
MedGen	575439
GARD	0019175
Is cancer (heuristic)	no

Disease family

This is a subtype of glomerulonephritis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › urinary system disorder › kidney disorder › nephritis › glomerulonephritis › Pauci-immune glomerulonephritis

Related subtypes (19): acute poststreptococcal glomerulonephritis, membranoproliferative glomerulonephritis, exudative glomerulonephritis, proliferative glomerulonephritis, focal embolic glomerulonephritis, anti-basement membrane glomerulonephritis, diffuse glomerulonephritis, subacute glomerulonephritis, mesangial proliferative glomerulonephritis, immune-complex glomerulonephritis, IgA glomerulonephritis, membranous glomerulonephritis, lupus nephritis, minimal change disease, granulomatosis with polyangiitis, rapidly progressive glomerulonephritis, primary membranoproliferative glomerulonephritis, immunotactoid glomerulopathy, autoimmune glomerulonephritis

Subtypes (2): pauci-immune glomerulonephritis with ANCA, pauci-immune glomerulonephritis without ANCA

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.