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Acquired angioedema type 1

Orpha number ORPHA100056
Synonym(s) Acquired angioneurotic edema type 1
Prevalence -
Inheritance
  • Sporadic
Age of onset Adulthood
ICD-10
  • T78.3
OMIM -
UMLS -
MeSH -
MedDRA -
SNOMED CT -

Summary

Acquired angioedema type 1 (AAE 1) 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 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.

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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