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ORPHA:100057

Renin-angiotensin-aldosterone system-blocker-induced angioedema

medium confidenceDisorder

Also known as: 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

Publications

10

21.1th percentile

Trials

0

Interventional, condition-specific

Researchers

58

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

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.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (5)

Raas-blocker-induced angioedema · Raas-blocker-induced angioneurotic edema · Raas-blocker-induced angioneurotic oedema · renin-angiotensin-aldosterone system-blocker-induced angioneurotic edema · renin-angiotensin-aldosterone system-blocker-induced angioneurotic oedema

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

3/6 stages with a signal

No specific-condition interventional trial, but broader-category trials exist — discuss eligibility with a clinician.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    10 matched papers (5 in last 10 years) Source

  3. Phenotype characterisedPresent

    13 HPO annotations (e.g. Abnormal capillary physiology; Respiratory distress; Erythema) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPartial

    None under the specific name; 148 for broader category angioedema

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

13

Associated phenotypes · MONDO:0015057

  • Abnormal capillary physiology
  • Respiratory distress
  • Erythema
  • Pruritus
  • Angioedema

Showing 5 of 13 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

10

10 papers have ever been indexed under this name. For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

10 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

5 in the last 10 years · medium confidence · 21.1th percentile (publications denominator)

Phrase hits: 6 · MeSH hits: 4

Open Europe PMC search

Who's working on it?

58

Distinct author names in 10 sampled papers — named people below.

Who's working on it?

No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.

  1. 01
    Bouillet L2 papers · 2020

    National Reference Center for Angioedema (CREAK), Department of Internal Medicine/Clinical Immunology, Grenoble Alpes University Hospital, Grenoble, France.

    Papers in Europe PMC
  2. 02
    Greve J2 papers · 2019

    Department for Otorhinolaryngology, Head and Neck Surgery, Ulm University Hospital, Ulm, Germany.

    Papers in Europe PMC
  3. 03
    Hoffmann TK2 papers · 2019

    Department for Otorhinolaryngology, Head and Neck Surgery, Ulm University Hospital, Ulm, Germany.

    Papers in Europe PMC
  4. 04
    Aerts JM1 paper · 2010
    Papers in Europe PMC
  5. 05
    Ahmadzadeh S1 paper · 2023

    Department of Anesthesiology, Louisiana State University Health Science Center at Shreveport, 1501 Kings Highway, Shreveport, LA, 71103, USA.

    Papers in Europe PMC
  6. 06
    Bas M1 paper · 2010

    Hals-, Nasen- und Ohrenklinik, Klinikum rechts der Isar, Technische Universität München, München. basmurat@web.de

    Papers in Europe PMC
  7. 07
    Belbézier A1 paper · 2020

    National Reference Center for Angioedema (CREAK), Department of Internal Medicine/Clinical Immunology, Grenoble Alpes University Hospital, Grenoble, France.

    Papers in Europe PMC
  8. 08
    Bier H1 paper · 2010
    Papers in Europe PMC
  9. 09
    Boccon-Gibod I1 paper · 2016
    Papers in Europe PMC
  10. 10
    Bocquet A1 paper · 2020

    National Reference Center for Angioedema (CREAK), Department of Internal Medicine/Clinical Immunology, Grenoble Alpes University Hospital, Grenoble, France.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

0

interventional trials for this specific condition

No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present). 148 trials are registered for angioedema, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026 · last trial check 11 September 2026

No matched interventional trials. This is true for 77.2% of diseases in the trials denominator (5501 of 7126). Here are the researchers publishing on it.

medium confidence · 38.6th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.

148 interventional trials matched angioedema, the broader category — listed below. Those studies are not counted in the condition-specific total.

Broader category: angioedema

148

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Renin-angiotensin-aldosterone system-blocker-induced angioedema — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Renin-angiotensin-aldosterone system-blocker-induced angioedema" OR "ACE inhibitor-related acquired angioedema" OR "ACEI-related acquired angioedema" OR "Acquired angioedema with normal C1 inhibitor" OR "Acquired angioedema with normal C1INH" OR "RAAS-blocker-induced angioedema" OR "RAAS-blocker-induced angioneurotic edema" OR "Renin-angiotensin-aldosterone system-blocker-induced angioneurotic edema" OR "Raas-blocker-induced angioneurotic oedema" OR "renin-angiotensin-aldosterone system-blocker-induced angioneurotic oedema"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Reactive angioendotheliomatosis

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Renin-angiotensin-aldosterone system-blocker-induced angioedema" OR "ACE inhibitor-related acquired angioedema" OR "ACEI-related acquired angioedema" OR "Acquired angioedema with normal C1 inhibitor" OR "Acquired angioedema with normal C1INH" OR "RAAS-blocker-induced angioedema" OR "RAAS-blocker-induced angioneurotic edema" OR "Renin-angiotensin-aldosterone system-blocker-induced angioneurotic edema" OR "Raas-blocker-induced angioneurotic oedema" OR "renin-angiotensin-aldosterone system-blocker-induced angioneurotic oedema" OR "Reactive angioendotheliomatosis"

Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"angioedema"

Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: RAE

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T06:58:55.270Z