Acquired angioedema
Synonyms: AAE · Acquired C1 inhibitor deficiency · Acquired angioneurotic edema · Acquired bradykinine-induced angioedema · Acquired non histamine-induced angioedema
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acquired angioedema | AAE · Acquired C1 inhibitor deficiency · Acquired angioneurotic edema · Acquired bradykinine-induced angioedema · Acquired non histamine-induced angioedema |
| fr | Angio-oedème acquis | Angio-oedème bradykinique acquis · Angio-oedème non histaminique acquis · Déficit acquis en inhibiteur de C1 · OANA · Oedème angioneurotique acquis |
| de | Angioödem, erworbenes | AAE · Angioneurotisches Ödem, erworbenes · Angioödem, erworbenes, Bradykinin-induziertes · Angioödem, erworbenes, nicht-Histamin-induziertes |
| es | Angioedema adquirido | Angioedema adquirido inducido por bradiquinina · Angioedema adquirido no inducido por histamina · Deficiencia adquirida de inhibidor C1 · Edema angioneurótico adquirido |
| it | Angioedema acquisito | AAE · Angioedema acquisito indotto dalla bradichinina · Angioedema acquisito non indotto dall'istamina · Deficit acquisito dell'inibitore C1 · Edema angioneurotico acquisito |
| nl | Verworven angio-oedeem | AAE · Verworven C1-inhibitordeficiëntie · Verworven angioneurotisch oedeem · Verworven bradykinine-geïnduceerd angio-oedeem · Verworven niet-histamine-geïnduceerd angio-oedeem |
| pt | Angioedema adquirido | |
| pl | Nabyty obrzęk naczynioruchowy | AAE · Nabyty Niedobór inhibitora C1 · Nabyty niehistaminowy obrzęk naczynioruchowy · Nabyty obrzęk angioneurotyczny · Nabyty obrzęk naczynioruchowy indukowany bradykininą |
| cs | Získaný angioedém | AAE · Získaný deficit C1 inhibitoru · Získaný angioneurotický edém · Získaný angioedém vyvolaný bradykininem · Získaný angioedém nevyvolaný histaminem |
| uk | Набутий ангіоневротичний набряк | AAE · Набутий ангіоневротичний набряк · Набутий ангіоневротичний набряк, спричинений брадикініном · Набутий дефіцит С1-інгібітору · Набутий негістамінний ангіоневротичний набряк |
| zh | 获得性血管性水肿 | 获得性非组胺介导血管性水肿 |
| tr | Edinsel anjiyoödem | AAE · Edinsel anjiyonörotik ödem · Edinsel C1 inhibitörü eksikliği · Kazanılmış bradikininin neden olduğu anjiyoödem · Kazanılmış histamin kaynaklı olmayan anjiyoödem |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 300909 | BTNT | validated | |
| ICD-11 | 4A00.15 | E | specific | validated |
| MONDO | 0019624 | E | validated | |
| MeSH | C538173 | E | validated | |
| UMLS | C2931758 | E | validated | |
| GARD | 8605 | E | validated | |
| MedDRA | 10081035 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:658Non-histaminic angioedema
rare immunological diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 1 000 000 | 0.11 / 100 000 | Denmark | Value and class | Validated | 23921495[PMID] |
| Cases/families | 200 cases | Worldwide | Case(s) | Validated | 23921495[PMID] | |
| Point prevalence | Unknown | — | Worldwide | Class only | 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/91385
- This record as JSON: /data/d/91385.json