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Renin-angiotensin-aldosterone system-blocker-induced angioedema

Disease definition

Renin-angiotensin-aldosterone system (RAAS)-blocker induced angioedema (RAE) is a type of acquired angioedema (AAE, see this term) characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway.

ORPHA:100057

Classification level: Disorder
  • Synonym(s):
    • ACE inhibitor-related acquired angioedema
    • ACEI-related acquired angioedema
    • Acquired angioedema with normal C1 inhibitor
    • Acquired angioedema with normal C1INH
    • RAAS-blocker-induced angioedema
    • RAAS-blocker-induced angioneurotic edema
    • RAE
    • Renin-angiotensin-aldosterone system-blocker-induced angioneurotic edema
  • Prevalence: Unknown
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Adult
  • ICD-10: T78.3
  • ICD-11: EH61.1
  • OMIM: 300909
  • UMLS: C1268945
  • MeSH: -
  • GARD: -
  • MedDRA: -

Summary

Clinical description

Like other forms of AAE it has a later onset than HAE (see this term) and occurs generally in adults.

Etiology

The main causative RAAS-blockers are the angiotensin-converting enzyme inhibitors (ACEIs) which increase levels of bradykinin leading to increased vascular permeability and vasodilation. Angioedema develops in 0.1%-0.5% of patients taking these drugs. Angioedema develops more often at an early phase of treatment, but may also occur with long-term treatment. The same side effect appears more rarely with angiotensin II receptor antagonists (ARAIIs) and direct renin inhibitors (DRIs). Co-administration of ACEI and an antidiabetic agent, dipeptidylptidase-4 (DPP-4) inhibitor, significantly increases the risk of angioedema. This reaction imposes the immediate cessation of these drugs.

Management and treatment

The orphan drug icatibant or C1-INH concentrate can be effective for acute attack treatment.

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)

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.