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Acquired angioedema type 2

Disease definition

A type of acquired angioedema (AAE) characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway.

ORPHA:100055

Classification level: Subtype of disorder
  • Synonym(s):
    • AAE 2
    • AAE II
    • Acquired angioneurotic edema type 2
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: T78.3
  • ICD-11: 4A00.15
  • OMIM: -
  • UMLS: C5680381
  • 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 and there is no family history of the disease.

Etiology

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.

Management and treatment

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

  A summary on this disease is available in Deutsch (2011) Español (2011) Français (2011) Italiano (2011) Nederlands (2011) Greek (2011, pdf)

Detailed information

Guidelines

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

Newborn screening

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.