ORPHA:156152
Anti-neutrophil cytoplasmic antibody-associated vasculitis
Synonyms: AAV · ANCA-associated vasculitis · Antineutrophil cytoplasmic antibody-associated vasculitis
- Type
- Clinical group
- Group
- Group of disorders
- Status
- active
This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anti-neutrophil cytoplasmic antibody-associated vasculitis | AAV · ANCA-associated vasculitis · Antineutrophil cytoplasmic antibody-associated vasculitis |
| fr | Vascularite associée aux anticorps antineutrophiles cytoplasmiques | AAV · Vascularite associée aux ANCA · Vascularite pauci-immune à ANCA |
| de | Vaskulitis mit assoziierten antineutrophilen zytoplasmatischen Antikörpern | AAV · ANCA-assoziierte Vaskulitis |
| es | Vasculitis asociada a anticuerpos anticitoplasma de neutrófilo | |
| it | Vasculite associata agli anticorpi anti-citoplasma nei neutrofili | AAV · Vasculite associata ad ANCA |
| nl | Antineutrofiele cytoplasmatische antistoffen-geassocieerde vasculitis | AAV · ANCA-geassocieerde vasculitis |
| pt | Vasculite associada a anticorpos anti-neutrofilo citoplasmáticos (ANCA) | |
| pl | Zapalenie naczyń z przeciwciałami przeciw cytoplazmie neutrofilów | AAV · Zapalenie naczyń związane z ANCA · Zapalenie naczyń związane z cytoplazmatycznymi przeciwciałami antyneutrofilowymi |
| cs | Vaskulitida asociovaná s protilátkami proti cytoplazmě neutrofilů | AAV · ANCA-asociovaná vaskulitida |
| uk | Васкуліт асоційований з антинейтрофільними цитоплазматичними антитілами | AAV · ANCA-асоційований васкуліт · Антинейтрофільні цитоплазматичні антитіла, асоційовані з васкулітом |
| zh | 抗中性粒细胞胞浆抗体血管炎 | 抗中性粒细胞胞浆抗体相关小血管炎 |
| tr | Anti-nötrofil sitoplazmik antikor-ilişkili vaskülit | AAV · ANCA-ilişkili vaskülit · Antineutrofil sitoplazmik antikor-ilişkili vaskülit |
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | 4A44.A | E | specific | validated |
| MONDO | 0015492 | E | validated | |
| MONDO | 15492 | E | validated | |
| MeSH | D056648 | E | validated | |
| UMLS | C2717865 | E | validated | |
| GARD | 13011 | E | validated | |
| MedDRA | 10050894 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:156146Predominantly small-vessel vasculitis
rare systemic and rheumatological diseases; rare circulatory system diseases - ORPHA:280369Rare pediatric vasculitis
rare systemic or rheumatologic diseases of childhood - ORPHA:264973Secondary interstitial lung disease in childhood and adulthood associated with a systemic vasculitis
rare respiratory diseases; rare transplant-related disorders
4
Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
4 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 14.9 / 100 000 | Germany | Value and class | Not yet validated | 24425780[PMID] |
| Point prevalence | 1-5 / 10 000 | — | Europe | Class only | Not yet validated | ORPHANET |
| Annual incidence | 1-9 / 100 000 | 3.3 / 100 000 | United States | Value and class | Validated | 28881446[PMID] |
| Point prevalence | 1-5 / 10 000 | 42.1 / 100 000 | United States | Value and class | Validated | 28881446[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/156152
- This record as JSON: /data/d/156152.json