Renin-angiotensin-aldosterone system-blocker-induced angioedema

General Information (adopted from Orphanet):

Synonyms, Signs: AEACEI
RAE
RAAS-blocker-induced angioneurotic edema
RAAS-blocker-induced angioedema
Renin-angiotensin-aldosterone system-blocker-induced angioneurotic edema
Number of Symptoms 1
OrphanetNr: 100057
OMIM Id: 300909
ICD-10: T78.3
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Multifactorial
Not applicable
[Orphanet]
Age of onset: Adult
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Acquired angioedema
 -Rare allergic disease
 -Rare systemic or rheumatologic disease

Symptom Information: Sort by abundance 

1
(HPO:0100665) Angioedema 14 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) Approximately 40 million people take ACE inhibitors (ACEi) to treat hypertension and cardiovascular disease. A small proportion of white patients who take ACEi (0.1-0.7%) develop angioedema (AEACEI) (Israili and Hall, 1992; Vleeming et al., 1998), a potentially life-threatening ...
Clinical Description OMIM Blais et al. (1999) and Adam et al. (2002) reported significantly lower plasma aminopeptidase P (APP) activities in patients with a history of AEACEI. Adam et al. (2002) found significantly decreased plasma activity of aminopeptidase P among 39 ...
Molecular genetics OMIM By genomewide linkage analysis of 8 large pedigrees in which 1 individual had ACEi-associated angioedema, Duan et al. (2005) found significant linkage (lod of 3.75) to a locus on the X chromosome that included the XPNPEP2 candidate gene. ...