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Acquired angioedema type 2

Orpha number ORPHA100055
Synonym(s) Acquired angioneurotic edema type 2
Prevalence -
Inheritance
  • Sporadic
Age of onset Adulthood
ICD-10
  • T78.3
OMIM -
UMLS -
MeSH -
MedDRA -
SNOMED CT -

Summary

Acquired angioedema type 2 (AAE2) is a type of acquired angioedema (AAE, see this term) characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway. Prevalence is unknown. 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 and there is no family history of the disease. The disease occurs due to an acquired C1-INH deficiency caused by malfunctioning B cells that secrete autoantibodies to the C1-INH molecule that neutralize C1-INH activity. AAE type 2 is often associated with dysglobulinemia of unknown origin. The treatments used for HAE can be effective for AAE but the most effective strategy is to decrease C1-INH antibody level with corticosteroids or rituximab.

Expert reviewer(s)

  • Pr Laurence BOUILLET
Last update: August 2011

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Detailed information
Emergency guidelines
  • EN (2009,pdf)
  • ES (2009,pdf)
  • IT (2009,pdf)
  • FR (2009,pdf)
  • PT (2009,pdf)
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