Non-histaminic angioedema

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:658
Who is this for?
Show terms as
10Specialists8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Non-histaminic angioedema is a group of rare conditions that cause sudden, recurring episodes of swelling beneath the skin. Unlike the more common allergic swelling (which involves a chemical called histamine), this type of swelling is driven by different pathways in the body — most often by a substance called bradykinin. The swelling can affect the face, lips, throat, hands, feet, belly, and genitals. It is also known as bradykinin-mediated angioedema or non-allergic angioedema. The most well-known form is Hereditary Angioedema (HAE), which is caused by a deficiency or malfunction of a protein called C1-inhibitor, or by mutations in other genes like FXII, ANGPT1, PLG, KNG1, MYOF, or HS3ST6. Because histamine is not involved, standard allergy treatments like antihistamines and epinephrine often do not work well, which can make attacks dangerous and difficult to manage. Symptoms come and go unpredictably and can last from hours to several days. Swelling in the throat is a medical emergency because it can block breathing. With the right diagnosis and modern treatments — including medications that target the bradykinin pathway — most people can significantly reduce the number and severity of attacks and live full lives. Early and accurate diagnosis is critical because this condition is often mistaken for allergic reactions for years.

Also known as:

Key symptoms:

Sudden swelling under the skin, especially of the face, lips, hands, feet, or genitalsSwelling inside the throat that can make breathing or swallowing difficultSevere cramping stomach pain caused by swelling in the intestinal wallNausea, vomiting, or diarrhea during abdominal attacksSwelling that does not itch (unlike allergic hives)Episodes that last 2 to 5 days without treatmentSkin tightness or a feeling of pressure in the swollen areaA non-itchy, blotchy rash (erythema marginatum) that sometimes appears before an attackFatigue and discomfort during and after episodesAttacks triggered by stress, minor injury, illness, hormonal changes, or certain medications

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

2 events
Dec 2024Safety and Efficacy of Conestat Alfa for ACE-Induced Angioedema

University of Cincinnati — PHASE3

TrialNOT YET RECRUITING
Oct 2021Bradykinin-degradating Enzymes Activities in Angiotensin-Converting Enzyme Inhibitors-associated Angioedema

University Hospital, Grenoble

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Non-histaminic angioedema.

View clinical trials →

No actively recruiting trials found for Non-histaminic angioedema at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Non-histaminic angioedema community →

Specialists

10 foundView all specialists →
MM
Melody C Carter, M.D.
Specialist
PI on 3 active trials
KM
Kate Connell, Masters
Specialist
PI on 1 active trial
MD
Murat Bas, Dr.
Specialist
PI on 1 active trial
VE
Vanessa espin
Specialist
PI on 1 active trial
JP
Jill A Poole
DENVER, CO
Specialist
PI on 2 active trials
PK
Paneez Khoury
BETHESDA, MD
Specialist
PI on 2 active trials
FD
Federica DEFENDI
Specialist
PI on 1 active trial
JB
Jonathan A. Bernstein
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Non-histaminic angioedema.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Non-histaminic angioedemaForum →

No community posts yet. Be the first to share your experience with Non-histaminic angioedema.

Start the conversation →

Latest news about Non-histaminic angioedema

Disease timeline:

New recruiting trial: Bradykinin-degradating Enzymes Activities in Angiotensin-Converting Enzyme Inhibitors-associated Angioedema

A new clinical trial is recruiting patients for Non-histaminic angioedema

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which specific genetic subtype of non-histaminic angioedema do I have, and what does that mean for my treatment options?,What emergency medication should I carry, and how do I use it if I have a throat attack?,Should I start long-term preventive treatment, and which option is best for my situation?,Are my family members at risk, and should they be tested?,What triggers should I try to avoid, and are there any medications I should never take?,What should I do if I need surgery or dental work — do I need special precautions?,Are there clinical trials or newer treatments I should know about?

Common questions about Non-histaminic angioedema

What is Non-histaminic angioedema?

Non-histaminic angioedema is a group of rare conditions that cause sudden, recurring episodes of swelling beneath the skin. Unlike the more common allergic swelling (which involves a chemical called histamine), this type of swelling is driven by different pathways in the body — most often by a substance called bradykinin. The swelling can affect the face, lips, throat, hands, feet, belly, and genitals. It is also known as bradykinin-mediated angioedema or non-allergic angioedema. The most well-known form is Hereditary Angioedema (HAE), which is caused by a deficiency or malfunction of a prote

Which specialists treat Non-histaminic angioedema?

10 specialists and care centers treating Non-histaminic angioedema are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.