Angioedema

What is angioedema?

Angioedema is a reaction to a trigger that causes swelling in the tissue below the inner layer of your skin called the dermis or the layer below a mucous membrane. Angioedema often happens at the same time as hives (urticaria) and for similar reasons. Both angioedema and hives happen when liquid from small blood vessels escapes and fills up tissues, causing swelling.

Usually, angioedema comes on quickly and lasts about a day or two. It most often affects your lips and eyes. However, angioedema can be serious, even fatal, when it affects your airways.

What are the types of angioedema?

There are several types of angioedema. Some organizations may classify angioedema into different types, and the types may vary. In general, though, angioedema types include:

• Acute allergic angioedema
• Non-allergic drug reaction
• Idiopathic angioedema
• Hereditary angioedema
• Acquired C1 inhibitor deficiency
• Vibratory angioedema

Acute allergic angioedema

You might be most familiar with this type of angioedema. It occurs as an allergic reaction to something you’ve come into contact with. This could be something you’ve eaten (a food or beverage), or taken (as in medications) or touched (as in items made with natural rubber latex). In addition, this type of angioedema can occur if an insect or spider bites you.

Acute allergic angioedema happens quickly, usually within minutes to about one to two hours after you’ve made contact with the allergen. You almost always have hives along with the swelling.

Non-allergic drug reaction

This type of angioedema doesn’t necessarily happen as soon as you take the medication. The most common group of medications that cause this non-allergic reaction is angiotensin-converting enzyme inhibitors, often called ACE inhibitors or ACEIs. These medications relax your blood vessels, treat heart failure and may lower blood pressure. Nonsteroidal anti-inflammatory medications, like ibuprofen and naproxen, can also cause angioedema.

Idiopathic angioedema

Idiopathic angioedema is angioedema that has no known cause. The swelling is located in your face, hands, trunk, arms and legs. Some people also have immune system conditions and emotional issues.

Hereditary angioedema

Hereditary angioedema (also called HAE) is something you get genetically from your parents. You can inherit HAE if only one parent carries the gene or has the condition. Some people have spontaneous genetic mutations that cause them to have this type of angioedema. An estimated 1 out of 50,000 people have hereditary angioedema. There are three types of this kind of angioedema, all of them related in some way to the C1 protein and/or C1 esterase inhibitor levels in your blood.

Acquired C1 inhibitor deficiency

Acquired C1 inhibitor deficiency results in angioedema, but it’s not inherited. Acquired means that you weren’t born with this deficiency but you developed it during your lifetime. This type of angioedema, like others, may affect your voice box (larynx) and result in asphyxiation (suffocation). Having B-cell lymphoma may cause acquired C1 inhibitor deficiency.

Vibratory angioedema

In this condition, the swelling is due to repeated vibrations. These can happen while you’re riding a motorcycle, running, jogging, vigorous massage or during other activities. Vibratory angioedema is a form of a similar and rare condition called chronic inducible urticaria, in which hives happen because of outside forces like cold, heat, water, pressure or vibrations. These itchy hives happen repeatedly and last for about six weeks.

Who does angioedema affect?

Angioedema can affect anyone. An estimated 20% to 25% of the U.S. population will have at least one episode of angioedema and/or hives throughout their lives.

What are the signs and symptoms of swelling (angioedema)?

Signs and symptoms of angioedema include:

• Puffy or swollen face, especially your eyes and mouth, including lips and tongue.
• Digestive problems when your intestines are swollen. These problems include abdominal pain, diarrhea or nausea and vomiting.
• Swollen hands, feet or genitals.
• Dizziness or fainting due to blood pressure changes.
• Swelling in your mouth, throat or airway that may make it harder to breathe and talk. When this happens, it’s a medical emergency. Get help right away.

What causes angioedema?

The causes of angioedema depend on what type of angioedema you have. Allergies are probably the main cause of the swelling of angioedema. There are many types of allergies that can cause it, including:

• Food allergies: The main culprits are milk, egg, nuts and shellfish.
• Medication allergies: Some of the drugs that cause these allergies include antibiotics like penicillin and sulfa drugs, nonsteroidal anti-inflammatory drugs (NSAIDS) and contrast media used in imaging tests. You might also get hives with this type of allergy.
• Venom: This is released by stinging insects and, rarely, brown recluse spider bites.
• Natural rubber latex: Latex is used to make gloves, balloons, condoms and catheters (tubes used in medicine). Your healthcare provider has probably asked you if you’re allergic to latex.

Other causes of angioedema include inherited and acquired problems with the C1 inhibitor protein, drug reactions that aren’t standard allergy reactions (there’s no itchiness and no hives) and vibrational movements.

Is angioedema contagious?

No. You can’t catch angioedema or give it to someone else.

What tests will be done to diagnose angioedema?

It may be difficult to tell what kind of angioedema you have. Your provider will begin with a physical examination, though in many cases the swelling will be easy to see. They’ll ask you questions about:

• When the swelling started.
• What you may have eaten, taken or touched that could have caused the reaction.
• What medications and supplements you're taking.
• Whether or not you’ve had this type of reaction before.
• Other family members who may have had swelling.

In addition to the questions, your provider may order blood or skin tests for allergies or blood tests to find out if you have angioedema related to the C1 inhibitor protein.

How is angioedema treated?

Treating angioedema depends on what kind of angioedema you have. For severe allergic reactions, you’ll often have injectable epinephrine to carry. You should administer this while calling 911.

For allergic angioedema, your provider may suggest antihistamines or steroids. You may get them either in oral (pill or liquid) or intravenous (in the vein) form.

If you have a non-allergic drug reaction, your provider will help you find a medication to replace the one that’s causing you to swell.

Home remedies include things like using ice to reduce swelling or taking cool showers. These may work best on things like swelling in one place or all over your lip or a cool wet cloth over your swollen eyes.

If you have hereditary, idiopathic or acquired C1 inhibitor deficiency angioedema, you’ll probably be referred to a specialist. Some medications that treat or prevent heredity angioedema include:

• C1 esterase inhibitor (recombinant) (Ruconest®).
• C1 inhibitor (human) (Berinert®, Cinryze®, Haegarda®).
• Ecallantide (Kalbitor®).
• Icatibant (Firazyr®).
• Lanadelumab (Takhzyro®).
• Berotralstat (Orladeyo®).

What can I expect if I have angioedema?

Most episodes of angioedema won’t last very long. They will probably resolve themselves. For severe episodes, you must have treatment to open the airways.

Is angioedema fatal?

For some people, allergic angioedema can cause anaphylaxis — severe swelling of the airways and lungs. People with this life-threatening condition should carry injectable epinephrine (EpiPen®, Auvi-Q®, Adrenaclick® and other brand names) to treat severe allergic reactions. Angioedema that affects the airways, no matter what the cause, is always a medical emergency and you should seek treatment immediately. Sometimes a tracheostomy, an opening in the windpipe/trachea, is performed to help people breathe.

How can I prevent angioedema?

If you have allergy-related angioedema, you can prevent occurrences by avoiding the food, medication or other triggers that cause allergic reactions. If you have non-allergic angioedema as a drug reaction to taking ACEIs, you’ll need to work with your healthcare provider to find another medication.

How do I take care of myself?

If you have episodes of angioedema, you should avoid allergy triggers. If your healthcare provider suggests medications to prevent further episodes, you should take them as directed. If you need to carry injectable epinephrine, make sure you have the injectors with you at all times. Let your family and friends know how to use them.

When should I see my healthcare provider about angioedema?

You should call 911 if you have a severe allergic reaction and the swelling affects your airways.

In less severe cases, contact your healthcare provider if you have repeated instances of angioedema. You may be able to work out preventive measures. Other treatment options include oral antihistamines and steroid medications. Some providers have used omalizumab (Xolair®), a monoclonal antibody, in difficult-to-treat idiopathic angioedema.