{"orpha":156152,"name":"Anti-neutrophil cytoplasmic antibody-associated vasculitis","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["AAV","ANCA-associated vasculitis","Antineutrophil cytoplasmic antibody-associated vasculitis"],"definition":null,"note":"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.","url":"https://raremap.alftech.space/d/ORPHA-156152/","orphanet_url":"https://www.orpha.net/en/disease/detail/156152","names":{"fr":{"name":"Vascularite associée aux anticorps antineutrophiles cytoplasmiques","synonyms":["AAV","Vascularite associée aux ANCA","Vascularite pauci-immune à ANCA"]},"de":{"name":"Vaskulitis mit assoziierten antineutrophilen zytoplasmatischen Antikörpern","synonyms":["AAV","ANCA-assoziierte Vaskulitis"]},"es":{"name":"Vasculitis asociada a anticuerpos anticitoplasma de neutrófilo"},"it":{"name":"Vasculite associata agli anticorpi anti-citoplasma nei neutrofili","synonyms":["AAV","Vasculite associata ad ANCA"]},"nl":{"name":"Antineutrofiele cytoplasmatische antistoffen-geassocieerde vasculitis","synonyms":["AAV","ANCA-geassocieerde vasculitis"]},"pt":{"name":"Vasculite associada a anticorpos anti-neutrofilo citoplasmáticos (ANCA)"},"pl":{"name":"Zapalenie naczyń z przeciwciałami przeciw cytoplazmie neutrofilów","synonyms":["AAV","Zapalenie naczyń związane z ANCA","Zapalenie naczyń związane z cytoplazmatycznymi przeciwciałami antyneutrofilowymi"]},"cs":{"name":"Vaskulitida asociovaná s protilátkami proti cytoplazmě neutrofilů","synonyms":["AAV","ANCA-asociovaná vaskulitida"]},"uk":{"name":"Васкуліт асоційований з антинейтрофільними цитоплазматичними антитілами","synonyms":["AAV","ANCA-асоційований васкуліт","Антинейтрофільні цитоплазматичні антитіла, асоційовані з васкулітом"]},"zh":{"name":"抗中性粒细胞胞浆抗体血管炎","synonyms":["抗中性粒细胞胞浆抗体相关小血管炎"]},"tr":{"name":"Anti-nötrofil sitoplazmik antikor-ilişkili vaskülit","synonyms":["AAV","ANCA-ilişkili vaskülit","Antineutrofil sitoplazmik antikor-ilişkili vaskülit"]}},"xrefs":[{"source":"ICD-11","code":"4A44.A","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1404622826"},{"source":"MONDO","code":"0015492","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"15492","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D056648","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2717865","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"13011","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10050894","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":156146,"name":"Predominantly small-vessel vasculitis","classifications":[196,198]},{"orpha":280369,"name":"Rare pediatric vasculitis","classifications":[231]},{"orpha":264973,"name":"Secondary interstitial lung disease in childhood and adulthood associated with a systemic vasculitis","classifications":[184,233]}],"children":[{"orpha":183,"name":"Eosinophilic granulomatosis with polyangiitis","type":"Disease"},{"orpha":900,"name":"Granulomatosis with polyangiitis","type":"Disease"},{"orpha":727,"name":"Microscopic polyangiitis","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":14.9,"area":"Germany","status":"Not yet validated","source":"24425780[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"1-5 / 10 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.3,"area":"United States","status":"Validated","source":"28881446[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":42.1,"area":"United States","status":"Validated","source":"28881446[PMID]"}],"phenotypes":null}