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Acquired angioedema type 1
Disease definition
A type of acquired angioedema (AAE) characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway.
ORPHA:100056
Classification level: Subtype of disorder- Synonym(s):
- Acquired angioneurotic edema type 1
- Prevalence: -
- Inheritance: Not applicable
- Age of onset: Adult
- ICD-10: T78.3
- ICD-11: 4A00.15
- OMIM: -
- UMLS: C5680380
- MeSH: -
- GARD: -
- MedDRA: -
Summary
Epidemiology
Prevalence is unknown.
Clinical description
Like other forms of AAE it has a later onset than HAE (see this term) and occurs generally in adults over the age of 50 where there is no family history of the disease.
Etiology
The disease occurs due to an acquired C1-INH deficiency. It is often associated with lymphoproliferative or autoimmune diseases which produce immune factors that destroy C1-INH leading to low levels of C1-INH, C1q complement and C4 complement.
Management and treatment
The treatments used for HAE can be effective for AAE but the most effective strategy is the treatment of the associated disease.
A summary on this disease is available in Deutsch (2011) Español (2011) Français (2011) Italiano (2011) Nederlands (2011) Greek (2011, pdf) Polski (2011, pdf)
Detailed information
Guidelines
- Emergency guidelines
- English (2009, pdf) - Orphanet Urgences
- Italiano (2009, pdf) - Orphanet Urgences
- Português (2009, pdf) - Orphanet Urgences
- Español (2017, pdf) - Orphanet Urgences
- Français (2021, pdf) - Orphanet Urgences
produced/endorsed by ERN(s) 
produced/endorsed by FSMR(s)Additional information