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Renin-angiotensin-aldosterone system-blocker-induced angioedema

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Disease definition

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

ORPHA:100057

Classification level: Disorder

Synonym(s):
  • ACEI-related acquired angioedema
  • Acquired angioedema with normal C1INH
  • Acquired angioedema with normal C1 inhibitor
  • ACE inhibitor-related acquired angioedema
  • RAAS-blocker-induced angioedema
  • RAAS-blocker-induced angioneurotic edema
  • RAE
  • Renin-angiotensin-aldosterone system-blocker-induced angioneurotic edema

Prevalence: Unknown

Inheritance: Multigenic/multifactorial, Not applicable

Age of onset: Adult

ICD-10: T78.3

ICD-11: EH61.1

OMIM: 300909

UMLS: C1268945

Summary
Clinical description

Like other forms of AAE it has a later onset than HAE 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.

Last update: August 2011 - Expert reviewer(s): Pr Laurence BOUILLET
A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

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

Guidelines
Emergency guidelines
Français (2021.pdf) - Orphanet Urgences
Polski (2009.pdf) - Orphanet Urgences
Deutsch (2009.pdf) - Orphanet Urgences
English (2009.pdf) - Orphanet Urgences
Español (2017.pdf) - Orphanet Urgences
Italiano (2009.pdf) - Orphanet Urgences
Português (2009.pdf) - Orphanet Urgences
Diagnostic Keys
Français (2024) - Les clés du diagnostic Logo FSMR
Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Newborn screening

The documents contained in this website 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.