Angioedema

Angioedema

Angioedema is a condition triggered by various factors causing swelling within the tissue beneath the skin’s dermis or the layer under a mucous membrane. It often occurs alongside hives (urticaria) and shares similar underlying causes. Both conditions arise when fluid leaks from small blood vessels into surrounding tissues, leading to swelling.

Typically, angioedema develops rapidly and lasts one to two days, commonly affecting areas like the lips and eyes. Nevertheless, it can become severe and potentially life-threatening if it affects the air passage.

Types of Angioedema

Angioedema is classified into several types, depending on the underlying cause. Although classifications may vary, the main types generally include:

  • Acute Allergic Angioedema

This is the most commonly recognized form. It is caused by an allergic reaction to substances such as foods or beverages, medications, products from natural rubber latex, and insect or spider bites.

Acute allergic angioedema usually occurs within minutes to a couple of hours after exposure to the allergen. In addition to swelling, it is often accompanied by hives.

  • Non-Allergic Drug Reaction

This type isn’t an immediate allergic response but occurs as a reaction to certain medications. Common culprits for this include:

  • ACEIs or ACE inhibitors (angiotensin-converting enzyme inhibitors)- Medications for relaxing blood vessels, treating heart failure, and lowering blood pressure.
  • NSAIDs (nonsteroidal anti-inflammatory drugs)- such as ibuprofen and naproxen.
  • Idiopathic Angioedema

Idiopathic angioedema refers to cases with no identifiable cause. Swelling typically occurs in the face, hands, arms, legs, and trunk. Some individuals may also experience immune system abnormalities or emotional problems.

  • Hereditary Angioedema (HAE)

Hereditary angioedema is a genetic condition passed down from one or both parents or caused by spontaneous genetic mutations. This rare condition affects about 1 in 50,000 people and is linked to abnormalities in the C1 protein or C1 levels of esterase inhibitors in the bloodstream. There are three subtypes of hereditary angioedema, each associated with these proteins.

  • Acquired C1 Inhibitor Deficiency

This type develops later in life rather than being inherited. It is linked to conditions such as B-cell lymphoma and can cause life-threatening swelling in the larynx (voice box), leading to asphyxiation or suffocation if untreated.

  • Vibratory Angioedema

This results from repeated vibrations from activities such as motorcycle riding, jogging, running, vigorous massages, or other similar activities.

Vibratory angioedema is associated with chronic inducible urticaria, where hives are triggered by external stimuli like vibrations, water, cold, heat, or pressure. These symptoms occur recurrently and persist for about 6 weeks.

Signs and Symptoms

  • Swollen or puffy face, particularly around the eyes, lips, tongue, and mouth.
  • Digestive issues due to swelling in the intestines, which may cause abdominal pain, nausea, diarrhea, or vomiting.
  • Swelling in the feet, hands, or genitals.
  • Light-headedness or fainting resulting from changes in blood pressure.
  • Swelling in the throat, mouth, or airway, which can make breathing and speaking difficult. This is a medical emergency that necessitates immediate attention.

Causes

While the underlying reasons for angioedema are based on the type, allergies are among the most common triggers. Key causes include:

  • Food allergies: Common triggers include milk, nuts, eggs, and shellfish.
  • Medication allergies: Medications that may provoke allergic reactions are antibiotics such as penicillin and sulfa drugs, NSAIDs, and contrast media for imaging tests. These reactions are often accompanied by hives.
  • Venom: Caused by stinging insects like bees or wasps, or in rare cases, brown recluse spider bites.
  • Natural rubber latex: Found in gloves, condoms, balloons, and medical catheters. Doctors often ask if you have latex allergies during treatment.

Additional angioedema causes may include:

  • Hereditary or acquired C1 inhibitor protein deficiencies.
  • Non-allergic drug reactions where there are no hives and itchiness.
  • Vibrational triggers

Diagnosis and Tests

Determining the specific type of angioedema can be challenging. Diagnosis typically begins with a physical examination, as the swelling is often visible. Your healthcare provider will also ask detailed questions, such as:

  • When you first notice the swelling.
  • What you ate, touched, or took before the reaction occurred.
  • Medications or supplements you are currently using.
  • Whether you have experienced similar reactions in the past.
  • Any family history of swelling.

To examine the cause further, your doctor may also recommend blood or skin tests to check for allergies or to determine whether you have C1 inhibitor protein-related angioedema.

Treatment and Management

The treatment for angioedema varies depending on its type. You will frequently need to carry injectable epinephrine in case of severe allergic reactions. This needs to be administered while dialing 911.

In addition, your doctor might recommend steroids or antihistamines for allergic angioedema. They can be given intravenously (in the vein) or orally (as a tablet or liquid). For non-allergic drug reactions, the treatment involves finding a different medication to substitute the one triggering swelling.

Home remedies for angioedema include taking chilly showers or applying ice to minimize swelling. These approaches are suitable for cases such as swelling in a single area or throughout your lip. Alternatively, you can use a cool, moist cloth for swollen eyes.

Your provider will most likely refer you to a specialist if you have acquired, idiopathic, or inherited C1 inhibitor deficiency angioedema. Heredity angioedema can be treated or prevented using the following medications:

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

Prevention

By refraining from foods, medicines, and other factors that cause allergic reactions, you can prevent episodes of allergy-related angioedema. In the event that taking ACE inhibitors causes non-allergic angioedema, you will work with your doctor to find an alternative medicine.

Living with Angioedema: Self-Care Tips

You should stay away from allergy triggers if you experience angioedema episodes. It’s also important to follow your doctor’s instructions if they prescribe medicine to stop further attacks. Ensure that you always have the injectors on hand if you must carry injectable epinephrine, and inform your loved ones so they understand how to use them in emergencies.

Conclusion

Angioedema is usually characterized by swelling in the lips, eyes, or hands. You are likely to experience these reactions if you have a history of allergies. It’s thus important to take note of when these episodes occur and try to identify and avoid possible triggers.

If you have family members who have encountered similar reactions, it’s a good idea to discuss this with your healthcare provider. If necessary, tests may be recommended to check for a genetic disorder. It’s essential to always carry injectable epinephrine if your angioedema is allergy-related, as swelling in your air passage can lead to difficulty breathing.

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