Pauci-immune glomerulonephritis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare small vessel vasculitis associated with rapidly progressive glomerulonephritis (GN) and clinically characterized by renal manifestations such as urinary abnormalities (hematuria and/or proteinuria) and hypertension leading to renal failure within days or weeks, and distinguished by the absence of immune deposits on immunofluorescent microscopy. The disease can occur as a renal-limited disease or as a component of systemic necrotizing small-vessel vasculitis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pauci-immune glomerulonephritis | |
| fr | Glomérulonéphrite pauci-immune | |
| de | Glomerulonephritis, pauci-immune | |
| es | Glomerulonefritis pauciinmune | |
| it | Glomerulonefrite pauci-immune | |
| nl | Pauci-immune glomerulonefritis | |
| pt | Glomerulonefrite pauci-imune | |
| pl | Skąpoimmunologiczne zapalenie kłębuszków nerkowych | |
| cs | Pauciimunní glomerulonefritida | |
| uk | Паукоімунний гломерулонефрит | |
| zh | 寡免疫复合物型肾小球肾炎 | |
| tr | Pauci-immün glomerülonefrit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N01.7 | NTBT | attributed | validated |
| ICD-11 | MF84 | E | specific | validated |
| MONDO | 0019641 | E | validated | |
| UMLS | C0343196 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:567560Systemic vasculitis associated with glomerulopathy
rare renal diseases; rare transplant-related disorders
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_Dr Laure-Hélène NOEL[EXPERT] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
37 annotations · validated 2021-03-02| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0000099 | Glomerulonephritis | Diagnostic criterion |
| Very frequent (99-80%) | ||
| HP:0032230 | Cytoplasmic antineutrophil antibody positivity | |
| HP:0012213 | Decreased glomerular filtration rate | |
| HP:0003259 | Elevated circulating creatinine concentration | |
| HP:0002907 | Microscopic hematuria | |
| HP:0000093 | Proteinuria | |
| HP:0000083 | Renal insufficiency | |
| HP:0011944 | Small vessel vasculitis | |
| Frequent (79-30%) | ||
| HP:0002086 | Abnormality of the respiratory system | |
| HP:0000951 | Abnormality of the skin | |
| HP:0008653 | Crescentic glomerulonephritis | |
| HP:0001945 | Fever | |
| HP:0000096 | Glomerulosclerosis | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0000478 | Abnormality of the eye | |
| HP:0004930 | Abnormality of the pulmonary vasculature | |
| HP:0002087 | Abnormality of the upper respiratory tract | |
| HP:0001919 | Acute kidney injury | |
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0012089 | Arteritis | |
| HP:0002829 | Arthralgia | |
| HP:0012735 | Cough | |
| HP:0002094 | Dyspnea | |
| HP:0001609 | Hoarse voice | |
| HP:0012587 | Macroscopic hematuria | |
| HP:0012593 | Nephrotic range proteinuria | |
| HP:0040223 | Pulmonary hemorrhage | |
| HP:0000979 | Purpura | |
| HP:0001970 | Tubulointerstitial nephritis | |
| Very rare (<4-1%) | ||
| HP:0001626 | Abnormality of the cardiovascular system | |
| HP:0011024 | Abnormality of the gastrointestinal tract | |
| HP:0032018 | Multiple mononeuropathy | |
| HP:0000155 | Oral ulcer | |
| HP:0001733 | Pancreatitis | |
| HP:0100532 | Scleritis | |
| Excluded (0%) | ||
| HP:0002955 | Granulomatosis | |
| HP:0032616 | Renal interstitial immunoglobulin deposits | |
Run the differential on the 8 obligate / very frequent terms
Annotation source: 28842398[PMID]_32491362[PMID]_25669759[PMID]_28613478[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/93126
- This record as JSON: /data/d/93126.json