{"orpha":100057,"name":"Renin-angiotensin-aldosterone system-blocker-induced angioedema","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ACE inhibitor-related acquired angioedema","ACEI-related acquired angioedema","Acquired angioedema with normal C1 inhibitor","Acquired angioedema with normal C1INH","RAAS-blocker-induced angioedema","RAAS-blocker-induced angioneurotic edema","RAE","Renin-angiotensin-aldosterone system-blocker-induced angioneurotic edema"],"definition":"Renin-angiotensin-aldosterone system (RAAS)-blocker induced angioedema (RAE) is a type of acquired angioedema (AAE) characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-100057/","orphanet_url":"https://www.orpha.net/en/disease/detail/100057","names":{"fr":{"name":"Angio-oedème bradykinique secondaire aux inhibiteurs du système rénine-angiotensine-aldostérone","synonyms":["Angio-oedème acquis associé aux inhibiteurs de ACE","Angio-oedème acquis avec inhibiteur de C1 normal","Angio-oedème bradykinique secondaire aux inhibiteurs du système RAAS","Oedème angioneurotique secondaire aux inhibiteurs du système RAAS","Oedème angioneurotique secondaire aux inhibiteurs du système rénine-angiotensine-aldostérone","RAE"],"definition":"L'angioedème secondaire aux inhibiteurs du système rénine-angiotensine-aldostérone (SRAA) est une forme d'angioedème acquis (AOA) caractérisée par un oedème aigu des tissus sous-cutanés et sous-muqueux des viscères et/ou des voies aériennes supérieures."},"de":{"name":"Angioödem, Renin-Angiotensin-Aldosteron-System Blocker induziertes","synonyms":["Angioödem, erworbenes, mit normalem C1INH","RAE","Angioneurotisches Ödem, RAAS-Blocker-induziertes","Angioneurotisches Ödem, Renin-Angiotensin-Aldosteron-System-Blocker induziertes","Angioödem, ACE-Inhibitor-abhängiges erworbenes","Angioödem, ACEI-abhängiges erworbenes","Angioödem, RAAS-Blocker-induziertes","Angioödem, erworbenes, mit normalem C1-Inhibitor"],"definition":"Das durch Blocker des Renin-Angiotensin-Aldosteron-Systems (RAAS) induzierte Angioödem (RAE), eine Form des Erworbenen Angioödems (EAA; s. dort), ist gekennzeichnet durch akutes Ödem in subkutanen Geweben, inneren Organen und/oder oberen Atemwegen."},"es":{"name":"Angioedema inducido por inhibidores del sistema renina-angiotensina-aldosterona","synonyms":["Angioedema adquirido asociado a IECA","Angioedema adquirido asociado al inhibidor de la ECA","Angioedema adquirido con C1INH normal","Angioedema adquirido con inhibidor C1 normal","Angioedema inducido por el bloqueo del RAAS","Edema angioneurótico inducido por el bloqueo del RAAS","Edema angioneurótico inducido por el bloqueo del sistema renina-angiotensina-aldosterona","RAE"],"definition":"El angioedema inducido por inhibidores del sistema renina-angiotensina-aldosterona (RAAS) es un tipo de angioedema adquirido (AEA, consulte este término) caracterizado por un edema agudo en los tejidos subcutáneos, las vísceras y/o la vía aérea superior."},"it":{"name":"Angioedema da inibitori del sistema renina-angiotensina-aldosterone","synonyms":["Angioedema da inibitori di RAAS","Edema angioneurotico da inibitori del sistema renina-angiotensina-aldosterone","Edema angioneurotico da inibitori di RAAS","RAE"],"definition":"L'angioedema indotto dagli inibitori del sistema renina-angiotensina-renina (RAAS) (RAE) è una forma di angioedema acquisito (si veda questo termine), caratterizzata da edema acuto nel sottocutaneo, nei visceri e/o nelle vie respiratorie superiori."},"nl":{"name":"Angio-oedeem geïnduceerd door renine-angiotensine-aldosteronsysteemblokker","synonyms":["ACE-remmer-gerelateerd verworven angio-oedeem","ACEI-gerelateerd verworven angio-oedeem","Angio-oedeem geïnduceerd door RAAS-blokker","Angioneurotisch oedeem geïnduceerd door RAAS-blokker","Angioneurotisch oedeem geïnduceerd door renine-angiotensine-aldosteronsysteemblokker","RAE","Verworven angio-oedeem met normale C1-remmer","Verworven angio-oedeem met normale C1Inh"],"definition":"Door renine-angiotensine-aldosteronsysteem (RAAS)-blokker geïnduceerd angio-oedeem (RAE) is een type van verworven angio-oedeem (AAE, zie deze term) gekenmerkt door acuut oedeem in subcutane weefsels, inwendige organen en/of de bovenste luchtwegen."},"pt":{"name":"Angioedema, relacionado com o inibidor da ECA","synonyms":["Angioedema adquirido IECA-relacionado","Edema angioneurótico IECA-relacionado","Edema angioneurótico induzido por inibidor do sistema renina-angiotensina-aldosterona"]},"pl":{"name":"Obrzęk naczynioruchowy indukowany blokerem układu renina-angiotensyna-aldosteron","synonyms":["Obrzęk angioneurotyczny indukowany blokerem RAAS","Obrzęk angioneurotyczny indukowany blokerem układu renina-angiotensyna-aldosteron","Obrzęk naczynioruchowy indukowany blokerem RAAS","RAE"]},"cs":{"name":"Angioedém indukovaný blokátory renin-angiotenzin-aldosteronového systému","synonyms":["Angioedém indukovaný RAAS blokátory","RAAS blokovaný angioneurotický edém","RAE","Angioneurotický edém indukovaný blokátory systému renin-angiotensin-aldosteron"]},"uk":{"name":"Ангіоневротичний набряк, спричинений блокаторами ренін-ангіотензин-альдостеронової системи","synonyms":["Набутий ангіоневротичний набряк, повязаний з прийомом інгібіторів АПФ","Набутий ангіоневротичний набряк, повязаний з іАПФ","Набутий ангіоневротичний набряк з нормальним рівнем інгібітору С1","Набутий ангіоневротичний набряк з нормальним C1INH","Ангіоневротичний набряк, спричинений блокатором RAAS","Ангіоневротичний набряк, спричинений блокатором RAAS","RAE","Ангіоневротичний набряк, спричинений блокаторами ренін-ангіотензин-альдостеронової системи"],"definition":"Renin-angiotensin-aldosterone system (RAAS)-blocker induced angioedema (RAE) is a type of acquired angioedema (AAE, see this term) characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway."},"zh":{"name":"肾素血管紧张素醛固酮系统抑制剂诱导的血管性水肿","synonyms":["肾素-血管紧张素-醛固酮系统阻滞剂诱导的血管神经性水肿"],"definition":"肾素-血管紧张素-醛固酮系统(RAAS系统)阻滞剂诱导的血管性水肿(RAE)是获得性血管性水肿(AAE)的一种，以皮下组织、内脏和/或上呼吸道的急性水肿为特征。"},"tr":{"name":"Renin-anjiyotensin-aldosteron sistemi bloker kaynaklı anjiyoödem","synonyms":["RAE","RAAS-blokör kaynaklı anjiyoödem","ACEI-ilişkili edinsel anjiyoödem","Normal C1INH ile seyreden edinsel anjiyoödem","RAAS-bloker kaynaklı anjiyonörotik ödem","ACE inhibitörü-ilişkili edinsel anjiyoödem","Normal C1 inhibitörü ile seyreden edinsel anjiyoödem","Renin-anjiyotensin-aldosteron sistemi-bloker kaynaklı anjiyonörotik ödem"]}},"xrefs":[{"source":"OMIM","code":"300909","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"T78.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EH61.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"729242810"},{"source":"MONDO","code":"0015057","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1268945","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":91385,"name":"Acquired angioedema","classifications":[195]}],"children":[],"genes":[{"symbol":"XPNPEP2","name":"X-prolyl aminopeptidase 2","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Not yet assessed","source":null,"hgnc":"12823","ensembl":"ENSG00000122121","omim":"300145","loci":["Xq26.1"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-11-16","source":"31864442[PMID]_26842379[PMID]_28994183[PMID]_28818177[PMID]_29884969[PMID]","terms":[{"hpo":"HP:0100665","label":"Angioedema","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0025018","label":"Abnormal capillary physiology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000282","label":"Facial edema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100540","label":"Palpebral edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002098","label":"Respiratory distress","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031244","label":"Swollen lip","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040315","label":"Tongue edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012027","label":"Laryngeal edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011855","label":"Pharyngeal edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002781","label":"Upper airway obstruction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Excluded (0%)","criteria":null},{"hpo":"HP:0001025","label":"Urticaria","frequency":"Excluded (0%)","criteria":null}]}}