Pauci-immune glomerulonephritis without ANCA
Synonyms: Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis · Pauci-immune glomerulonephritis without antineutrophil cytoplasmic antibody
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A form of pauci-immune glomerulonephritis characterized by rapidly progressive glomerulonephritis and the absence of antineutrophilic cytoplasmic antibodies (ANCA). In comparison with pauci-immune GN with ANCA, patients lacking ANCA may be younger at onset of the disease, 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. The prognosis is generally poorer.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pauci-immune glomerulonephritis without ANCA | Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis · Pauci-immune glomerulonephritis without antineutrophil cytoplasmic antibody |
| fr | Glomérulonéphrite pauci-immune sans ANCA | Glomérulonéphrite pauci-immune sans auto-anticorps anticytoplasme des polynucléaires neutrophiles |
| de | Glomerulonephritis, pauci-immune, ohne ANCA | Pauci-Immun-Glomerulonephritis ohne ANCA · Pauci-immune Glomerulonephritis ohne antineutrophile zytoplasmatische Antikörper |
| es | Glomerulonefritis pauciinmune sin ANCA | Glomerulonefritis pauciinmune negativa para anticuerpos anticitoplasma de neutrófilos · Glomerulonefritis pauciinmune sin anticuerpos anticitoplasma de neutrófilos |
| it | Glomerulonefrite pauci-immune senza ANCA | Glomerulonefrite pauci-immune negativa agli anticorpi anti-citoplasma dei neutrofili · Glomerulonefrite pauci-immune senza anticorpi anti-citoplasma dei neutrofili |
| nl | Pauci-immune glomerulonefritis zonder ANCA | Antineutrofiel cytoplasmatisch antilichaam-negatieve pauci-immune glomerulonefritis · Pauci-immune glomerulonefritis zonder antineutrofiel cytoplasmatisch antilichaam |
| pt | Glomerulonefrite pauci-imune sem ANCAs | |
| pl | Nieme immunologicznie kłębuszkowe zapalenie nerek bez ANCA | = en |
| cs | Pauciimunní glomerulonefritida bez ANCA protilátek | Pauciimunní glomerulonefritida, negativní na antineutrofilní cytoplazmatické protilátky · Pauciimunní glomerulonefritida bez antineutrofilních cytoplazmatických protilátek |
| uk | Pauci-імунний гломерулонефрит без ANCA | Антинейтрофільний цитоплазматичний гломерулонефрит з негативними антинейтрофільними антитілами, паукоімунний гломерулонефрит · Паукоімунний гломерулонефрит без антинейтрофільних цитоплазматичних антитіл |
| zh | ANCA阴性少免疫沉积型肾小球肾炎 | 抗中性粒细胞胞浆抗体阴性少免疫沉积型肾小球肾炎 |
| tr | ANCA-ilişkili olmayan Pauci-immün glomerülonefrit | Antinötrofil sitoplazmik antikor ile seyretmeyen Pauci-immün glomerülonefrit · Antinötrofil sitoplazmik antikor-negatif pauci-immün glomerülonefrit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N05.7 | NTBT | attributed | validated |
| ICD-11 | MF84 | NTBT | attributed | validated |
| MONDO | 0019989 | E | validated | |
| UMLS | C5680253 | 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:93126Pauci-immune glomerulonephritis
rare renal diseases; rare transplant-related disorders
No children.
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97564
- This record as JSON: /data/d/97564.json