- What Is It?
- Life Expectancy
- How to Prevent
What Is Angioedema?
Angioedema is the swelling of tissue under the skin (the deep dermis). Angioedema can affect any part of the body through acute episodes typically affect the lips and mouth, eyelids, face, and tongue. Angioedema can also occur in other parts of the body, including the respiratory and gastrointestinal (GI) mucosa. Laryngeal swelling in the throat or tongue swelling can be life-threatening.
What Are Symptoms of Angioedema?
Angioedema may affect many organ systems and visible swelling is usually the main symptom. Swelling may be accompanied by:
The parts of the body most commonly affected by angioedema include:
- Peripheral swelling
- Affects the skin and urogenital area (e.g., eyelids, lips, tongue, hands, feet, scrotum, etc.)
- Larynx – can be life-threatening
- Throat tightness
- Voice changes
- Difficulty breathing
- Tongue and lip swelling
- Sometimes so severe that tongue protrudes from the mouth
What Causes Angioedema?
Allergies are a common cause of angioedema. Common allergens include:
- Foods - commonly nuts, shellfish, or eggs
- Insect bites
- Something that comes in contact with the skin such as latex, animal saliva or dander, or plants
Angioedema can also be caused by rare genetic diseases such as hereditary angioedema.
How Is Angioedema Diagnosed?
A doctor can usually diagnose angioedema with a medical history and physical exam. Most mild cases of angioedema do not require testing.
Tests can be done to determine allergies that may cause angioedema, and for the rare genetic diseases that cause angioedema.
In chronic or recurrent cases angioedema without a known trigger, doctors may order tests to help determine the cause, such as:
- CBC with differential
- Erythrocyte sedimentation rate (ESR)
- C-reactive protein (CRP) level
- D-dimer level
- Comprehensive metabolic profile
- Antinuclear antibody (ANA) testing
- CH50 level
- Thyroid studies, including levels of thyroid stimulating hormone, free T4, and thyroid autoantibodies
- Stool analysis for ova and parasites
- Helicobacter pylori workup
- Hepatitis B and C virus workup
- Rheumatoid factor
- Cryoglobulin levels
Imaging studies may be indicated if internal organ involvement is suspected during acute attacks:
- Plain abdominal X-rays – may show a “stacked coin” or “thumbprint” appearance of the intestines
- Abdominal ultrasound – may reveal ascites
- Abdominal computed tomography (CT) scan – may show edema of the bowel wall
- Chest X-ray – may show fluid around the lungs (pleural effusion)
- Soft-tissue neck X-ray – may show soft-tissue swelling
What Is the Treatment for Angioedema?
The goal of treatment for angioedema is to reduce and prevent swelling, discomfort, and complications. If swelling is in the face, mouth, or throat, special attention and treatment are needed so the patient’s airway is not closed off.
Medications used to treat hives and severe allergic reactions (anaphylaxis) may also be to treat many types of angioedema such as:
- Alpha- and beta-adrenergic agonist agents such as epinephrine (EpiPen, EpiPen Jr, Auvi-Q, Symjepi, Adrenalin)
- First-generation antihistamines such as diphenhydramine (Benadryl), chlorpheniramine (Chlor-Trimeton), cyproheptadine (Periactin), hydroxyzine hydrochloride (Atarax, Vistaril)
- Second-generation antihistamines such as cetirizine (Zyrtec), desloratadine (Clarinex), fexofenadine (Alleegra), levocetirizine (Xyzal), loratadine (Claritin)
- Histamine H2 antagonists such as ranitidine (Zantac), cimetidine (Tagamet)
- Leukotriene receptor antagonists such as montelukast (Singulair), zafirlukast (Accolate)
- Tricyclic antidepressants such as doxepin (Silenor)
- Corticosteroids such as prednisone (Deltasone, Rayos, Prednisone Intensol, Sterapred, Sterapred DS), methylprednisolone (Medrol, Medrol Dosepak, DepoMedrol, SoluMedrol, A-Methapred), prednisolone (Pediapred, FloPred, Orapred, Millipred, Prelone Syrup, Veripred 20)
- Androgen derivatives such as danazol (Danocrine), oxandrolone (Oxandrin), progesterone-based birth control pills
- Antifibrinolytic agents such as aminocaproic acid (Amicar), tranexamic acid (Cyklokapron, Lysteda)
- Immunomodulators such as cyclosporine (Sandimmune), mycophenolate (CellCept), methotrexate (Rasuvo, Otrexup)
- Agents for treatment or prophylaxis: C1 INH concentrates (Berinert, Cinryze, Haegarda), ecallantide (Kalbitor), lanadelumab (Takhzyro), and icatibant (Firazyr)
- Fresh frozen plasma (FFP)
In severe cases of facial swelling, intubation (placing a tube down the throat to keep the airway and lungs open) may be an emergency necessary. If intubation is unsuccessful, surgery may be required in severe cases of laryngeal edema (throat swelling) to create an airway via cricothyrotomy or tracheotomy.
What Are Complications of Angioedema?
If angioedema affects the larynx and upper airways, it can cause trouble breathing, suffocation, and even death. Symptoms of laryngeal angioedema include throat pain or discomfort, trouble speaking, and difficulty swallowing.
Angioedema may also cause severe pain and disfiguration that can interfere with daily tasks and affect a patient’s quality of life.
What Is the Life Expectancy for Angioedema?
Most patients who have angioedema will have no complications and the symptom is temporary and goes away on its own or with treatment, and life expectancy is not affected.
Ten to 25 percent of cases of angioedema of the larynx and upper airways that enter an emergency department have life-threatening cases, and between 3-40% of patients who have suffocation (asphyxiation) due to laryngeal edema will die.
How Do You Prevent Angioedema?
Patients with known allergies can avoid symptoms of angioedema by avoiding allergic triggers. Blood pressure medications such as ACE inhibitors may need to be changed permanently by your doctor. (Do not stop taking any medication without first talking to your doctor.)Patients who develop angioedema due to exercise may need to restrict activity.