Renin-angiotensin-aldosterone…

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Renin-angiotensin-aldosterone system-blocker-induced angioedema

ORPHA:100057Disease

Also called 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

What it is

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.

Key facts

Age of onset
Adult
Inheritance
Multigenic/multifactorial, Not applicable
Classified as
Disease

Recorded for the broader condition

Prevalence
1-9 / 1 000 000 (Denmark)Acquired angioedema

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Gene

XPNPEP2Major susceptibility factor

ICD-10 codes

T78.3filed under a broader ICD-10 category — shared with 6 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0015057OMIM 300909UMLS C1268945

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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