ORPHA:100057
Renin-angiotensin-aldosterone system-blocker-induced angioedema
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
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
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.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Renin-angiotensin-aldosterone system-blocker-induced angioedema | 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 |
| fr | Angio-oedème bradykinique secondaire aux inhibiteurs du système rénine-angiotensine-aldostérone | 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 |
| de | Angioödem, Renin-Angiotensin-Aldosteron-System Blocker induziertes | 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 |
| es | Angioedema inducido por inhibidores del sistema renina-angiotensina-aldosterona | 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 |
| it | Angioedema da inibitori del sistema renina-angiotensina-aldosterone | Angioedema da inibitori di RAAS · Edema angioneurotico da inibitori del sistema renina-angiotensina-aldosterone · Edema angioneurotico da inibitori di RAAS · RAE |
| nl | Angio-oedeem geïnduceerd door renine-angiotensine-aldosteronsysteemblokker | 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 |
| pt | Angioedema, relacionado com o inibidor da ECA | Angioedema adquirido IECA-relacionado · Edema angioneurótico IECA-relacionado · Edema angioneurótico induzido por inibidor do sistema renina-angiotensina-aldosterona |
| pl | Obrzęk naczynioruchowy indukowany blokerem układu renina-angiotensyna-aldosteron | Obrzęk angioneurotyczny indukowany blokerem RAAS · Obrzęk angioneurotyczny indukowany blokerem układu renina-angiotensyna-aldosteron · Obrzęk naczynioruchowy indukowany blokerem RAAS · RAE |
| cs | Angioedém indukovaný blokátory renin-angiotenzin-aldosteronového systému | Angioedém indukovaný RAAS blokátory · RAAS blokovaný angioneurotický edém · RAE · Angioneurotický edém indukovaný blokátory systému renin-angiotensin-aldosteron |
| uk | Ангіоневротичний набряк, спричинений блокаторами ренін-ангіотензин-альдостеронової системи | 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 |
| zh | 肾素血管紧张素醛固酮系统抑制剂诱导的血管性水肿 | 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 |
| tr | Renin-anjiyotensin-aldosteron sistemi bloker kaynaklı anjiyoödem | 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 |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 300909 | BTNT | validated | |
| ICD-10 | T78.3 | NTBT | attributed | validated |
| ICD-11 | EH61.1 | NTBT | index | validated |
| MONDO | 0015057 | E | validated | |
| UMLS | C1268945 | 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 (1):
- ORPHA:91385Acquired angioedema
rare immunological diseases
No children.
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Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| XPNPEP2 | Major susceptibility factor in | Not yet assessed | HGNC:12823 | ENSG00000122121 | 300145 | Xq26.1 | X-prolyl aminopeptidase 2 |
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Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
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
13 annotations · validated 2021-11-16| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0100665 | Angioedema | Diagnostic criterion |
| Very frequent (99-80%) | ||
| HP:0025018 | Abnormal capillary physiology | |
| HP:0000282 | Facial edema | |
| Frequent (79-30%) | ||
| HP:0100540 | Palpebral edema | |
| HP:0002098 | Respiratory distress | |
| HP:0031244 | Swollen lip | |
| HP:0040315 | Tongue edema | |
| Occasional (29-5%) | ||
| HP:0010783 | Erythema | |
| HP:0012027 | Laryngeal edema | |
| HP:0011855 | Pharyngeal edema | |
| HP:0002781 | Upper airway obstruction | |
| Excluded (0%) | ||
| HP:0000989 | Pruritus | |
| HP:0001025 | Urticaria | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 31864442[PMID]_26842379[PMID]_28994183[PMID]_28818177[PMID]_29884969[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/100057
- This record as JSON: /data/d/100057.json