Angioedema: Causes, Symptoms & Treatment Strategies

What is Angioedema?

Angioedema is a condition characterised by sudden swelling in the deeper layers of the skin and mucous membranes. Unlike hives, which affect the skin's surface, angioedema involves the subcutaneous and submucosal tissues. The swelling most commonly affects the face, particularly the lips, eyelids, and cheeks. However, it can also involve the tongue, throat, hands, feet, and genitals.

The swelling may be asymmetric and can cause discomfort or pain if severe. Angioedema results from fluid accumulation due to increased permeability of blood vessels, which allows fluid to leak into the surrounding tissues.

What are the types of Angioedema?

The meaning of angioedema refers to a condition characterised by sudden swelling beneath the skin or mucous membranes, and it can occur for various reasons. There are several types of angioedema, each with specific causes, symptoms, and treatment approaches.

• Allergic angioedema is the most common type and typically occurs in response to allergens such as certain foods, medications, insect stings, or latex. It often presents alongside hives and other allergic symptoms like itching or difficulty breathing. This type of swelling usually resolves with the use of antihistamines, corticosteroids, or epinephrine in severe cases. Prompt recognition and treatment are important, especially if airway swelling is involved.
• Drug-induced angioedema is frequently linked to medications, particularly ACE inhibitors, which are used to treat high blood pressure and heart conditions. This form of angioedema can appear unexpectedly, even after prolonged use of the drug. The primary treatment involves stopping the medication, which typically leads to resolution of symptoms, though medical supervision is necessary during the acute phase.
• Idiopathic angioedema refers to cases where no clear cause can be identified despite thorough medical evaluation. This angioedema type may be triggered by factors like stress, infections, or immune system irregularities. Symptoms mimic allergic angioedema but tend to recur unpredictably, often making management more complex.
• Hereditary angioedema (HAE) is a rare genetic disorder caused by a deficiency or malfunction of the C1 esterase inhibitor protein. It leads to recurrent swelling episodes that may affect the face, limbs, gastrointestinal tract, and even the airway. HAE can be life-threatening and requires specialised medications to control and prevent attacks.
• Acquired angioedema is a non-genetic variant that typically emerges later in life and may be associated with underlying conditions like lymphoma or autoimmune diseases. It also involves C1 inhibitor deficiency and shares symptoms with HAE.

Correct diagnosis of angioedema types is essential for effective treatment and long-term management.

Who does Angioedema affect?

Angioedema can affect individuals of any age, gender, or ethnicity. However, some forms, such as hereditary angioedema, may have a genetic component and can run in families.

Causes of Angioedema

Angioedema causes vary depending on the type:

• Allergic reactions to foods, medications, insect stings, or latex can trigger allergic angioedema
• Certain drugs, particularly ACE inhibitors, can cause drug-induced angioedema
• Hereditary angioedema is caused by a genetic deficiency or dysfunction of C1 inhibitor protein
• Idiopathic angioedema has no clear cause but may be related to immune system irregularities or triggers like stress or infections
• Acquired angioedema is associated with underlying conditions that affect C1 inhibitor levels

In all cases, angioedema results from the release of chemicals like histamine or bradykinin, which cause fluid leakage and swelling in the affected tissues.

Symptoms of Angioedema

The primary symptom of angioedema is sudden, localised swelling beneath the skin.

Common areas affected include:

• Lips
• Face (eyelids, cheeks)
• Tongue
• Throat
• Hands and feet
• Genitals

The swelling may be accompanied by:

• Pain or a burning sensation
• Redness
• Warmth in the affected area

In some cases, angioedema may occur alongside hives, which are itchy, raised, red welts on the skin's surface.

If angioedema affects the throat or larynx, it can cause:

• Difficulty swallowing or breathing
• Hoarseness
• A feeling of throat tightness

These angioedema symptoms require immediate medical attention, as they can be life-threatening.

Gastrointestinal angioedema may cause:

• Abdominal pain
• Nausea and vomiting
• Diarrhoea

Angioedema episodes often develop quickly and can last from a few hours to several days, depending on the type and severity. Hereditary angioedema attacks may persist for 2-5 days and recur periodically.

Diagnosis

Diagnosing angioedema involves a thorough evaluation of your symptoms, medical history, and potential triggers. Your doctor may:

• Conduct a physical exam to assess the extent and location of swelling
• Ask about your family history of angioedema or allergies
• Review your medication list to identify potential drug-induced causes
• Perform allergy tests (skin prick or blood tests) to determine if an allergic reaction is responsible
• Order blood tests to check C1 inhibitor levels and function, especially if hereditary or acquired angioedema is suspected

In some cases, additional tests or specialist referrals may be necessary to rule out underlying conditions or confirm the specific type of angioedema.

Complications

The most serious complication of angioedema is swelling in the throat or larynx, which can obstruct the airway and lead to difficulty breathing or even asphyxiation. This is a medical emergency requiring immediate treatment.

In some cases, angioedema can affect the gastrointestinal tract, causing intestinal wall swelling, which may lead to severe abdominal pain, nausea, vomiting, and diarrhoea.

Recurrent episodes of angioedema, particularly in hereditary or idiopathic cases, can significantly impact quality of life, causing anxiety, stress, and disruption to daily activities.

Rarely, acquired angioedema may be associated with underlying conditions like lymphoma or autoimmune disorders, which require specific treatment.

Treatment Strategies

The treatment approach for angioedema depends on the underlying cause and severity of symptoms:

• Allergic angioedema:
  • Antihistamines to reduce swelling and itching
  • Corticosteroids to decrease inflammation
  • Epinephrine (adrenaline) for severe reactions affecting breathing
• Drug-induced angioedema:
  • Discontinuation of the offending medication
  • Alternative medications as prescribed by your doctor
• Hereditary angioedema:
  • C1 inhibitor replacement therapy to restore normal levels
  • Bradykinin receptor antagonists to block the effects of bradykinin
  • Kallikrein inhibitors to prevent bradykinin production
  • Androgens (male hormones) to boost C1 inhibitor production
• Idiopathic angioedema:
  • Antihistamines and corticosteroids to manage symptoms
  • Identification and avoidance of triggers, if possible
• Acquired angioedema:
  • Treatment of the underlying condition
  • C1 inhibitor replacement therapy, if necessary

In all cases, your doctor will work with you to develop an individualised angioedema treatment plan based on your specific needs and the severity of your condition.

Prevention Tips

While not all cases of angioedema can be prevented, there are steps you can take to reduce your risk or minimise the frequency and severity of episodes:

• Identify and avoid known triggers, such as specific foods, medications, or environmental factors
• If you have allergies, carry an epinephrine auto-injector and wear a medical alert bracelet
• For drug-induced angioedema, discuss alternative medications with your doctor
• If you have hereditary angioedema, work with your healthcare team to develop a comprehensive management plan, which may include preventive medications and lifestyle modifications
• Manage stress through relaxation techniques, as stress can be a trigger for some forms of angioedema
• Stay informed about your condition and educate family and friends on how to recognise and respond to an angioedema episode

By taking proactive steps and working closely with your healthcare provider, you can effectively manage angioedema and minimise its impact on your daily life.

When to See a Doctor

Seek immediate medical attention if you experience swelling of the lips, tongue, or throat that interferes with breathing or swallowing. This can be life-threatening, especially if accompanied by symptoms of anaphylaxis such as wheezing, dizziness, or fainting.

You should also consult your doctor if you have recurrent episodes of unexplained swelling, a family history of angioedema, or develop symptoms after starting a new medication. Additionally, seek medical advice if the swelling is painful, lasts unusually long, or affects your daily activities. A healthcare provider can help identify the cause and create a treatment plan to manage symptoms and prevent complications.

How do I take care of myself?

If you have been diagnosed with angioedema, it's essential to follow your treatment plan as prescribed by your doctor. This may include taking medications regularly, avoiding triggers, and carrying an epinephrine auto-injector if recommended. Attend follow-up appointments and keep your healthcare team informed of any changes in your symptoms or overall health.

Is angioedema fatal?

In most cases, angioedema is not life-threatening. However, if swelling affects the throat or larynx, it can obstruct the airway and lead to difficulty breathing, which can be fatal if not treated promptly. This is more common in hereditary angioedema but can also occur in severe allergic reactions.

Seeking immediate medical attention for any signs of respiratory distress is crucial. With proper treatment and management, the vast majority of people with angioedema can lead normal, healthy lives.

What can I expect if I have angioedema?

If you have been diagnosed with angioedema lips or swelling in other areas of your face, you can expect to work closely with your healthcare team to develop a personalised management plan. This may include medications, lifestyle modifications, and regular follow-up appointments to monitor your condition and adjust treatment as needed.

Conclusion

Living with angioedema can be challenging, but with the right information and medical guidance, it is possible to manage symptoms effectively and lead a healthy life. Timely and accurate diagnosis plays a crucial role in identifying the underlying cause and guiding appropriate treatment. Metropolis Healthcare offers advanced diagnostic services to help your healthcare provider make informed decisions about your care. Early detection and regular monitoring can make a significant difference in managing angioedema and preventing complications.