Angioedema treatment

 

Angioedema treatment mainly aims to control and prevent swelling, or edema, that occurs in the deeper layers of the skin or in mucus membranes. Angioedema swelling frequently affects the face, lips, extremities, and genitals, and can be life-threatening if it occurs in the throat and blocks the patient’s airway.

While there are a number of different treatment options available, most of them medications, their use depends on many factors, such as the type of angioedema and whether the goal is prevention or on-demand treatment.

There are several different types of angioedema. All are marked by swelling, though other symptoms — such as hives, also known as urticaria, and itching — are more common with certain angioedema types. The underlying angioedema causes vary from type to type, so different treatment strategies are required to manage each form of the disease.

Medications for angioedema

In acute allergic angioedema, swelling arises due to an allergic reaction that causes immune cells to release histamine, an inflammatory molecule that widens blood vessels and increases their permeability. Angioedema treatment for this form mainly consists of medications aiming to bring such reactions under control. These include:

• antihistamines
• corticosteroids or other immune-suppressing drugs
• epinephrine (the active agent in EpiPen and related allergy treatments).

In drug-induced nonallergic angioedema that arises as a side effect of certain medications, discontinuing treatment with the medicine that’s causing angioedema and finding a suitable replacement is a key part of treatment. Because this type of angioedema is not driven by an allergic reaction, it will typically not respond to medications used to manage allergic forms of angioedema.

Hereditary angioedema, known as HAE, is a form of angioedema in which swelling attacks occur as a consequence of genetic mutations. Treatments for this angioedema type generally aim to prevent and treat recurrent swelling episodes, or HAE attacks, by addressing their underlying causes. Some of these angioedema medications include:

• Berinert (human C1 esterase inhibitor)
• Cinryze (human C1 esterase inhibitor)
• Haegarda (human C1 esterase inhibitor)
• Ruconest (recombinant C1 esterase inhibitor)
• Firazyr (icatibant)
• Kalbitor (ecallantide)
• Takhzyro (lanadelumab)
• Orladeyo (berotralstat).

In acquired angioedema, swelling develops as a consequence of another underlying health condition, such as an autoimmune disorder or cancer. Long-term treatment for this angioedema type mostly involves medicines to control the underlying condition (e.g., immune-suppressing medications or chemotherapy). Some medications used for HAE, specifically Firazyr and C1 esterase inhibitors, also may be used to help manage acute swelling in acquired angioedema.

There are no approved treatments for idiopathic angioedema, a form of angioedema without a clear cause. However, some reports suggest that certain medications indicated for other forms of angioedema may help to ease swelling episodes in these patients.

 

Alternative treatments for angioedema

Cooling the skin (e.g., with an ice pack applied to a specific area that’s swollen) can be a useful home remedy for reducing swelling. Taking a cold bath or shower also may be an effective strategy to help ease angioedema symptoms.

In forms of angioedema where swelling is accompanied by itching, home remedies for itch like oatmeal baths, aloe vera, or calamine lotion may provide some relief.

There are a few herbal remedies that have been used to manage inflammation, swelling, and other allergy symptoms in traditional medicine practices. These include goldenseal, licorice root, and chamomile. However, there isn’t much modern scientific evidence suggesting these treatments actually are effective.

Similarly, certain supplements have been proposed to help manage some forms of angioedema, including vitamin C, vitamin B12, quercetin, and bromelain — but there’s not much evidence that these are effective. The same is true for homeopathic remedies and acupuncture.

Herbal remedies and supplements should never be used as a replacement for evidence-backed angioedema treatments, and because these substances can cause side effects and interact with other medications, patients are advised to talk with their healthcare team before starting any new supplement or herbal remedy.

Strategies for managing angioedema

In all forms of angioedema, a key aspect in disease management is taking steps to lower the risk of future swelling attacks. Patients who experience an angioedema attack are advised to work with their healthcare team to obtain a correct diagnosis and then figure out an individualized angioedema management plan.

For some patients, preventing future angioedema attacks may include resorting to medications that are taken regularly to reduce the risk of swelling. In cases where angioedema is found or suspected to have been triggered by a medication, stopping that medication — and finding a suitable alternative — is key in preventing future swelling episodes.

Taking precautions to avoid potential attack triggers also is important in angioedema management. For people with acute allergic angioedema, this typically means figuring out which substance(s) an individual is allergic to, and then avoiding those substances.

In hereditary angioedema, swelling attacks may be triggered by physical injury and infections. Thus, disease management often includes steps to minimize the chances of injuries or infections, such as using safety equipment or getting all recommended vaccines. Stress also can act as a trigger for angioedema, so strategies to manage stress, such as meditation or mindfulness, may be helpful for managing angioedema.

Taking steps to avoid damaging or irritating the skin, such as wearing smooth, loose-fitting clothing or applying sunscreen while outdoors, also may be helpful for reducing the risk of swelling attacks in angioedema patients.

Specific triggers that lead to swelling are different for every individual. It’s commonly recommended that patients experiencing angioedema keep a diary or journal to record the details of their swelling attacks, including symptoms and duration, and when they occurred. This may help to identify specific triggers, which can be used to inform a personalized management plan.

Treatment and prevention of HAE attacks

HAE is a genetic disorder that’s characterized by recurrent swelling attacks sometimes accompanied by other symptoms like fatigue or abdominal pain. Because hereditary angioedema affects everyone differently, the U.S. HAE Association recommends that anyone with HAE consult a medical expert with experience in treating the condition to come up with a tailored treatment plan.

The association’s recommendations suggest that every person with HAE should have access to an on-demand treatment to manage swelling attacks if they occur. The guidelines also suggest that patients should consider long-term prophylactic (preventive) angioedema treatment to reduce the risk of swelling attacks. Short-term prophylaxis should always be given before surgeries or other events that may act as a trigger for swelling attacks.

Management of HAE also usually involves steps to identify and minimize exposure to potential swelling triggers, including stress or physical injury.

Treatment for HAE types 1 and 2

HAE types 1 and 2, which account for the vast majority of HAE cases, are caused by mutations in the gene that provides instructions for making a protein called C1 esterase inhibitor (C1-INH). In HAE type 1, not enough C1-INH is made, whereas in HAE type 2, the C1-INH protein doesn’t function correctly. In both cases, low C1-INH protein activity leads to abnormally high levels of bradykinin, a signaling molecule that can trigger swelling.

Prophylactic treatments

Both Cinryze, which is injected directly into the bloodstream, and Haegarda, which is given via a subcutaneous or under-the-skin injection, contain a functional version of the C1-INH protein. Both are approved in the U.S. for routine prophylaxis in HAE patients ages 6 and older. These therapies, which work by providing a functional version of the C1-INH protein patients are missing, are usually the first choice for prophylactic hereditary angioedema treatment.

If these angioedema medications aren’t effective or well tolerated, steroid-based medicines that can boost C1-INH production in the body, such as the attenuated androgen danazol, may be used as a second-line routine preventive treatment.

Takhzyro, an antibody-based treatment administered via a subcutaneous injection, and Orladeyo, an oral small molecule taken once daily, also are available as a prophylactic treatment for HAE. Both work to lower bradykinin levels by inhibiting the activity of an enzyme called kallikrein, but Takhzyro is approved for HAE patients ages 2 and older and Orladeyo for those ages 12 and older.

On-demand treatments

Medications available for on-demand treatment of angioedema swelling attacks in people with HAE types 1 or 2 include Berinert and Ruconest. Both of these contain a functional version of the C1-INH protein that is administered by an injection directly into the bloodstream.

Another approved medication for on-demand treatment of HAE attacks is Kalbitor, which is given by a subcutaneous injection and also works to lower bradykinin levels by inhibiting kallikrein’s activity. It is authorized in the U.S. for the on-demand treatment of swelling attacks in HAE patients ages 12 and older.

Firazyr is another injectable therapy that is available for the on-demand treatment of HAE attacks. In the U.S., Firazyr is authorized to be used in patients ages 18 and older. It works by preventing bradykinin from binding to the receptors it normally interacts with to trigger its effects.

Fresh frozen plasma also may be given to help manage swelling in HAE, particularly in emergency situations in which other treatments aren’t readily available.

Treatment for rarer HAE types

While most cases of HAE are caused by mutations in the gene that encodes the C1-INH protein, there also are rarer cases of HAE caused by mutations in other genes. These forms of HAE fall within the umbrella of HAE type 3.

In some cases, HAE type 3 is caused by mutations in the F12 gene. In individuals with this type of HAE, Berinert is usually used for on-demand treatment to control acute swelling attacks, while treatments to prevent swelling episodes may include danazol, progestins (a type of hormonal therapy), or tranexamic acid, which is an antifibrinolytic agent approved to control bleeding associated with heavy menstrual cycles. Avoiding potential swelling triggers also is an important part of managing F12-associated HAE.

There also are reports of HAE type 3 cases caused by other mutations. However, as few of these cases have been reported, treatment for these HAE patients tends to be more individualized.

HAE treatment in pregnancy and breastfeeding

In people with HAE who are pregnant or breastfeeding, angioedema medications that contain a version of the C1-INH protein are typically the first choice for long-term prophylaxis (Cinryze or Haegarda) and on-demand treatment (Berinert or Ruconest). Attenuated androgens such as danazol are contraindicated, or not recommended, during pregnancy and breastfeeding as they can cross the placenta and pass into breast milk.

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Angioedema News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.