{"orpha":91385,"name":"Acquired angioedema","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["AAE","Acquired C1 inhibitor deficiency","Acquired angioneurotic edema","Acquired bradykinine-induced angioedema","Acquired non histamine-induced angioedema"],"definition":"A rare disease characterized by the occurrence of transitory and recurrent subcutaneous and/or submucosal edemas resulting in swelling and/or abdominal pain due to an acquired C1 inhibitor (C1-INH) deficiency.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-91385/","orphanet_url":"https://www.orpha.net/en/disease/detail/91385","names":{"fr":{"name":"Angio-oedème acquis","synonyms":["Angio-oedème bradykinique acquis","Angio-oedème non histaminique acquis","Déficit acquis en inhibiteur de C1","OANA","Oedème angioneurotique acquis"],"definition":"L'angioedème acquis (AOA) se caractérise par la survenue d'oedèmes sous-cutanés et/ou sous-muqueux transitoires et récidivants avec gonflement localisé et/ou douleur abdominale, dus à un déficit acquis en C1 inhibiteur (C1Inh)."},"de":{"name":"Angioödem, erworbenes","synonyms":["AAE","Angioneurotisches Ödem, erworbenes","Angioödem, erworbenes, Bradykinin-induziertes","Angioödem, erworbenes, nicht-Histamin-induziertes"],"definition":"Das Erworbene Angioödem (AAE) ist gekennzeichnet durch vorübergehende und rezidivierende subkutane und/oder submuköse Ödeme mit Schwellungen und Leibschmerzen, verursacht durch einen erworbenen Mangel des Plasmaprotease-C1-Inhibitors (C1-INH)."},"es":{"name":"Angioedema adquirido","synonyms":["Angioedema adquirido inducido por bradiquinina","Angioedema adquirido no inducido por histamina","Deficiencia adquirida de inhibidor C1","Edema angioneurótico adquirido"],"definition":"Es una enfermedad poco frecuente caracterizada por la aparición recurrente y transitoria de edemas subcutáneos y/o submucosos que provocan inflamación y/o dolor abdominal debido a la deficiencia adquirida de inhibidor C1 (C1-INH)."},"it":{"name":"Angioedema acquisito","synonyms":["AAE","Angioedema acquisito indotto dalla bradichinina","Angioedema acquisito non indotto dall'istamina","Deficit acquisito dell'inibitore C1","Edema angioneurotico acquisito"],"definition":"L'angioedema acquisito (AAE) è caratterizzato dalla comparsa di edemi sottomucosi e/o sottocutanei ricorrenti e transitori, che causano rigonfiamenti e/o dolori addominali da deficit acquisito dell'inibitore C1 (C1-INH)."},"nl":{"name":"Verworven angio-oedeem","synonyms":["AAE","Verworven C1-inhibitordeficiëntie","Verworven angioneurotisch oedeem","Verworven bradykinine-geïnduceerd angio-oedeem","Verworven niet-histamine-geïnduceerd angio-oedeem"],"definition":"Een zeldzame aandoening, gekarakteriseerd door tijdelijke en terugkerende subcutane en/of submucosale oedemen die resulteren in zwelling en/of abdominale pijn, als gevolg van een verworven deficiëntie van C1-inhibitor (C1-INH)."},"pt":{"name":"Angioedema adquirido","definition":"Doença rara caracterizada pela ocorrência de edemas transitórios, subcutâneos e/ou submucosos recorrentes resultando em edema e dor abdominal devido a uma deficiência adquirida do inibidor da C1 (C1-INH)."},"pl":{"name":"Nabyty obrzęk naczynioruchowy","synonyms":["AAE","Nabyty Niedobór inhibitora C1","Nabyty niehistaminowy obrzęk naczynioruchowy","Nabyty obrzęk angioneurotyczny","Nabyty obrzęk naczynioruchowy indukowany bradykininą"]},"cs":{"name":"Získaný angioedém","synonyms":["AAE","Získaný deficit C1 inhibitoru","Získaný angioneurotický edém","Získaný angioedém vyvolaný bradykininem","Získaný angioedém nevyvolaný histaminem"]},"uk":{"name":"Набутий ангіоневротичний набряк","synonyms":["AAE","Набутий ангіоневротичний набряк","Набутий ангіоневротичний набряк, спричинений брадикініном","Набутий дефіцит С1-інгібітору","Набутий негістамінний ангіоневротичний набряк"],"definition":"A rare disease characterized by the occurrence of transitory and recurrent subcutaneous and/or submucosal edemas resulting in swelling and/or abdominal pain due to an acquired C1 inhibitor (C1-INH) deficiency."},"zh":{"name":"获得性血管性水肿","synonyms":["获得性非组胺介导血管性水肿"]},"tr":{"name":"Edinsel anjiyoödem","synonyms":["AAE","Edinsel anjiyonörotik ödem","Edinsel C1 inhibitörü eksikliği","Kazanılmış bradikininin neden olduğu anjiyoödem","Kazanılmış histamin kaynaklı olmayan anjiyoödem"]}},"xrefs":[{"source":"OMIM","code":"300909","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-11","code":"4A00.15","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1078767412"},{"source":"MONDO","code":"0019624","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C538173","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2931758","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8605","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10081035","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":658,"name":"Non-histaminic angioedema","classifications":[195]}],"children":[{"orpha":528663,"name":"Acquired angioedema with C1Inh deficiency","type":"Disease"},{"orpha":100057,"name":"Renin-angiotensin-aldosterone system-blocker-induced angioedema","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.11,"area":"Denmark","status":"Validated","source":"23921495[PMID]"},{"type":"Cases/families","qualification":"Case(s)","class":null,"value":200.0,"area":"Worldwide","status":"Validated","source":"23921495[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}