Pauci-immune glomerulonephritis with ANCA
Synonyms: Pauci-immune glomerulonephritis with antineutrophil cytoplasmic antibody
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A form of pauci-immune glomerulonephritis characterized by a rapidly progressive glomerulonephritis in association with the presence of circulating antineutrophilic cytoplasmic antibodies (ANCA), mostly directed against proteinase-3 (PR3) and myeloperoxidase (MPO). Patients usually present with urinary abnormalities and rapidly declining renal function, often leading to dialysis within weeks without treatment. Cutaneous, pulmonary, musculoskeletal and nervous involvement may be observed in case of systemic disease, and the correlation between ANCA titer and disease activity has been demonstrated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pauci-immune glomerulonephritis with ANCA | Pauci-immune glomerulonephritis with antineutrophil cytoplasmic antibody |
| fr | Glomérulonéphrite pauci-immune avec ANCA | Glomérulonéphrite pauci-immune avec auto-anticorps anticytoplasme des polynucléaires neutrophiles |
| de | Glomerulonephritis, pauci-immune, mit ANCA | Pauci-Immun-Glomerulonephritis mit ANCA · Pauci-immune Glomerulonephritis mit antineutrophilen zytoplasmatischen Antikörpern |
| es | Glomerulonefritis pauciinmune con ANCA | Glomerulonefritis pauciinmune con anticuerpos anticitoplasma de neutrófilos |
| it | Glomerulonefrite pauci-immune con ANCA | Glomerulonefrite pauci-immune con anticorpi anti-citoplasma dei neutrofili |
| nl | Pauci-immune glomerulonefritis met ANCA | Pauci-immune glomerulonefritis met antineutrofiel cytoplasmatisch antilichaam |
| pt | Glomerulonefrite pauci-imune com ANCAs | |
| pl | Nieme immunologicznie kłębuszkowe zapalenie nerek z ANCA | = en |
| cs | Pauciimunní glomerulonefritida s ANCA protilátkami | Pauciimunní glomerulonefritida s antineutrofilními cytoplazmatickými protilátkami |
| uk | Pauci-імунний гломерулонефрит з ANCA | Паукоімунний гломерулонефрит з антинейтрофільними цитоплазматичними антитілами |
| zh | ANCA阳性少免疫沉积型肾小球肾炎 | 伴抗中性粒细胞胞浆抗体的少免疫沉积型肾小球肾炎 |
| tr | ANCA-ilişkili Pauci-immün glomerülonefrit | Antinötrofil sitoplazmik antikor ile seyreden 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 | 0019988 | E | validated | |
| UMLS | C5680254 | 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/97563
- This record as JSON: /data/d/97563.json