If you have angioedema, look to UC San Diego Health for comprehensive services. Our world-class physicians and researchers are committed to improving the lives of people with acute and chronic swelling caused by angioedema.
- A broad range of highly personalized services
- The latest and most advanced therapies and treatments
- Innovative options in angioedema diagnosis and management
- Expert consultation services for physicians
- Clinical trials and groundbreaking basic and translational research
- Consultations on both U.S. and international angioedema cases
See AlsoAllergy & Immunology
What is Angioedema?
Angioedema is swelling that occurs within the skin. While angioedema often involves the face and lips, it can potentially affect any region of the body. Angioedema can also occur in mucous membranes such as the mouth, throat and less commonly, the intestines.
Potential causes of angioedema include:
- Allergic reactions
- Medication side effects
- Genetic (hereditary) conditions
In rare instances, angioedema may be associated with an autoimmune disease, infection or specific type of malignancy. However, angioedema often occurs spontaneously without any identifiable underlying medical problem.
The focus of angioedema management is to safely suppress the symptoms.
What Causes Angioedema?
While there are several types of angioedema, they are usually a result of one of the following factors:
- Histamine-release due to allergies, infections, autoimmune conditions or mast cell activation from undetermined causes.
- Excessive bradykinin production in the skin (usually caused by either a genetic protein deficiency or as a side effect of medication). Bradykinin is a peptide (chemical compound) that causes blood vessels to dilate, leading to a decline in blood pressure.
Our experts develop personalized treatment plans for individuals with all types of angioedema.
- Comprehensive diagnostic testing as indicated
- Tailored medication plan
- Recommended lifestyle modifications
Hereditary Angioedema (HAE)
The Hereditary Angioedema Center at UC San Diego Health is dedicated to diagnosing and treating patients with diverse forms of swelling attacks caused by hereditary angioedema (HAE) – a rare, frequently misdiagnosed and poorly understood genetic condition.
The center was established with support from the U.S. Hereditary Angioedema Association to serve as a national referral center for the 6,000 to 8,000 people in the U.S. with this potentially life-threatening condition.
The center provides:
- Early, reliable diagnosis of HAE due to C1INH protein deficiency
- A treatment plan tailored to your condition and lifestyle
- Education on self-administering treatment at home during a swelling attack
- Education on non-drug approaches to improve quality of life
- Opportunity to participate in clinical trials of new therapies
What is Hereditary Angioedema?
Hereditary angioedema refers to a type of swelling caused by deficient or improper function of C1-inhibitor, a protein that prevents fluid from leaking from blood vessels. When there is not enough C1-inhibitor, or it is dysfunctional, episodic severe swelling of the skin including the hands, feet, face, or genitals can occur.
Swelling of the intestines may also occur, which can cause debilitating abdominal pain.
Swelling of mucous membranes of the mouth, tongue, or throat from HAE can be life-threatening due to obstruction of the airway.
Types of HAE
There are three types of hereditary angioedema:
- HAE Type 1: Associated with insufficient production of C1-inhibitor protein. Most common type, responsible for 85 percent of HAE cases.
- HAE Type 2: Normal or high amounts of C1-inhibitor protein are produced but protein does not function properly. As a result, fluid leaks from blood vessels into surrounding tissue. Responsible for 15 percent of HAE cases.
- HAE with normal C1INH (Type 3): A small group of people, predominantly female, have swelling episodes characteristic of HAE, yet their C1-inhibitor protein levels are normal and appear to be functional. This type of HAE is very rare and the cause is poorly understood.
In about 80 percent of people with hereditary angioedema, the genetic defect is inherited. In the other 20 percent, the disease is caused by a spontaneous mutation of the C1-inhibitor gene during conception.
There are three specific blood tests used to diagnose HAE Type 1 and HAE Type 2.
The first test measures a person’s C4 serum level. C4 levels are decreased during attacks and typically remain low between attacks.
The second and third tests measure the quantity and functionality of C1-inhibitor protein.
There is currently no good diagnostic test for HAE with normal C1INH (Type 3).
People with swelling attacks caused by HAE do not respond to medications that are administered to people with most other forms of angioedema or allergies (such as antihistamines, corticosteroids and epinephrine).
Several medications can help treat and prevent HAE symptoms and swelling attacks.
Our team will help you sift through the growing number of new treatment options and identify an optimal management plan for your condition. This often includes instruction on how to self-administer medication at home during an acute attack.
Why is knowing how to self-administer important?
Self-administered acute care can often be given more quickly and therefore reduce episodes of potentially disabling swelling more rapidly. Ultimately, this means fewer emergency room visits and greater peace of mind.
Do non-drug approaches for HAE work?
In some instances, people with HAE may benefit from non-drug approaches as part of the management plan.
While more research is necessary, addressing the mental health components of HAE may help improve individual quality of life. Of particular interest is helping patients find and practice techniques that can help them lower their stress levels since stress can trigger or exacerbate swelling attacks in some people.
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