Knowledge on rare diseases and orphan drugs
COVID-19 & Rare diseases
Rare Diseases Resources for Refugees/Displaced Persons
Search for a rare disease
Acquired angioedema type 1
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
- Acquired angioneurotic edema type 1
Prevalence: -
Inheritance: Not applicable
Age of onset: Adult
Prevalence is unknown.
Like other forms of AAE it has a later onset than HAE and occurs generally in adults over the age of 50 where there is no family history of the disease.
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.
The treatments used for HAE can be effective for AAE but the most effective strategy is the treatment of the associated disease.
Last update: August 2011 - Expert reviewer(s): Pr Laurence BOUILLET
: produced/endorsed by ERN(s)
: produced/endorsed by FSMR(s)
Guidelines
Further information on this disease
Patient-centred resources for this disease
Research activities on this disease
Newborn screening