EGPA (formerly Churg-Strauss Syndrome)

Overview

What is eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome)?

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome) is a disease caused by inflammation (swelling) that occurs in certain types of cells in your blood or in your tissues. Everyone who gets EGPA has a history of asthma and/or allergies. It can affect many of your organs.

Almost all people with EGPA have increased numbers of "allergic type" blood cells called eosinophils. Eosinophils are a type of white blood cell that usually make up 5% or less of the total white blood cell count. In EGPA, eosinophils usually make up more than 10% of the total white blood cell count. In addition, most biopsies (tissue samples) contain clusters of cells called "granulomas" that may or may not involve blood vessels.

What is vasculitis?

Vasculitis is a general medical term that means inflammation of the blood vessels. When your blood vessels become inflamed, several things can happen:

  • They can grow weak and stretch, which can cause an aneurysm to develop. (An aneurysm is an abnormal blood-filled enlargement of a blood vessel.)
  • The walls can become thinner. If they do, the walls can rupture, and blood can leak out into tissue.
  • They can narrow to the point of closing. This can cause damage to the organs from the loss of oxygen and nutrients that were being supplied by the blood.

What organs are affected by EGPA?

This inflammation causes injury to your lungs, nose, sinuses, skin, joints, nerves, intestinal tract, heart and/or kidneys and rarely the brain.

How common is EGPA?

It is an extremely rare disease – there are only two to five new cases a year per one million people.

Who is affected by EGPA?

EGPA can occur in people of all ages, from children to the elderly. The average age that someone is diagnosed is between 35 and 50 years old. EGPA appears to affect men and women equally.

Is EGPA contagious?

No.

Symptoms and Causes

What causes eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome)?

The cause of EGPA inflammation is unknown.

Your immune system plays a role in EGPA. The immune system acts to protect the body against “foreign invaders” (germs, bacteria) that cause infections, disease and other injuries to the body. Sometimes your immune system becomes over active and can cause inflammation.

Is EGPA a type of cancer or autoimmune disorder?

No.

Does EGPA run in families?

No, it’s not hereditary.

What are EGPA symptoms?

Because EGPA can affect several different organs, there is a wide range of symptoms.

People who have EGPA may feel generally ill and fatigued or have fevers. They may lose their appetite and lose weight. Other symptoms depend on the organs or diseases involved. For example, you may have:

  • Shortness of breath from asthma or inflammation in the air sacs and blood vessels of the lungs.
  • Chest pain from disease that affects the lungs or heart.
  • Rashes on the skin.
  • Muscle and/or joint pain.
  • Increased nasal discharge (runny nose) or facial pain from sinusitis.
  • Abdominal pain or blood in the stools from intestinal tract involvement.
  • Abnormal feelings, and numbness or loss of strength and feeling from nerve involvement.

You may have any combination of these symptoms.

Kidney disease caused by EGPA often does not have any symptoms. You may not know about inflammation of the kidney until the kidneys begin to stop working. Therefore, if you have any form of vasculitis, you must have regular urinalyses (examinations of the urine).

Diagnosis and Tests

How is eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome) diagnosed?

The healthcare provider may use the following tests to diagnose EGPA:

  • Medical history: to look for EGPA, especially asthma, allergies and other features of the disease.
  • Physical examination: to discover which organs are involved and to rule out other illnesses that may look similar.
  • Blood tests: to look for abnormal blood counts and an increase in eosinophils and special antibody testing called ANCA.
  • Urinalysis: to detect whether there is too much protein, or red blood cells, in the urine.
  • Imaging tests such as x-rays and computed tomography (CT) to look for any abnormalities in areas such as the lungs or sinuses.

Once the diagnosis of EGPA is suspected, a biopsy (tissue sample) is often performed to try to find eosinophils, eosinophilic granulomas and/or vasculitis. Biopsies are not required in all cases, and are only recommended when abnormal findings are seen in the exam, laboratory tests, or imaging tests.

Management and Treatment

How is EGPA treated? What medicines help?

A variety of immunosuppressive drugs (drugs that suppress the immune system) are used to treat EGPA. Your healthcare provider will choose the drug based on how the EGPA is affecting you - whether it is mild (for instance, affecting the skin or joint), or potentially life-threatening.

Glucocorticoids such as prednisone or prednisolone are the most commonly used treatments. People who have a mild disease (those whose disease does not affect the nervous system, heart, kidneys, intestinal tract, or show other features of severe disease) might do extremely well on corticosteroid therapy alone. Once you have a dramatic improvement on this drug, the dosage is reduced to the lowest amount that keeps the disease under control.

People with EGPA that affects critical organs are usually treated with corticosteroids combined with another immunosuppressive drug, such as cyclophosphamide (Cytoxan®), methotrexate, Mycophenolate Mofetil or azathioprine (Imuran®).

These drugs are also used to treat other medical conditions. Azathioprine is used to prevent organ transplant rejection and is also a treatment for lupus and other autoimmune diseases. High doses of cyclophosphamide and methotrexate are used to treat certain types of cancer. In vasculitis, these drugs are given at doses that are 10 to 100 times lower than that used to treat cancer in order to suppress the immune system.

Biologic medications are also used in EGPA such as Rituximab (anti-CD20 monoclonal antibody directed against B cells) and Mepolizumab (anti-interleukin (IL)-5 monoclonal antibodies. Mepolizumab is approved by the FDA for the treatment of EGPA and is indicated in people whose disease is not controlled with oral glucocorticoids alone and who do not have severe disease. Rituximab can be used in people with kidney, skin and nerve involvements.

The goal of treatment is to stop all damage that comes from EGPA. This is known as "remission." Once the disease begins to improve, your healthcare provider may slowly reduce the corticosteroid dose, and eventually stop it completely. Stopping it completely may be difficult if a person has significant asthma, as the airways can be very sensitive to corticosteroids.

If cyclophosphamide is used for severe EGPA, it is often only given until you go into remission (usually around three to six months); you’ll then switch to another immunosuppressive agent, such as methotrexate, Mycophenolate Mofetil, azathioprine or Mepolizumab, to maintain remission.

The duration of maintenance immunosuppressive drugs depends on the person. In most instances, it is given for least one to two years before the healthcare provider considers slowly reducing the dosage and eventually stopping it.

Is surgery needed?

No, surgery is not part of the treatment plan for EGPA.

What can I do to help relieve the symptoms of EGPA?

There are no appropriate at-home treatments. Follow your healthcare provider’s instructions.

Is there anything I can or can’t eat or drink?

Nothing you eat or drink affects EGPA.

Are there side effects of treatment?

All immunosuppressive drugs can have side effects. Each drug can have its own side effects, so it is very important to watch for them. A person with EGPA may tolerate these drugs at first; however, this may not last. This is why it’s so important to watch for side effects, even after you stop taking the drug. Blood tests are also used to evaluate the illness itself. There are many side effects for glucocorticoids, so be sure to stay in close contact with your healthcare provider.

Prevention

Can EGPA be prevented? How can I reduce my risk?

At this time there are no known ways to prevent this disease.

Outlook / Prognosis

What is the outlook for people with EGPA?

Although EGPA can be an increasingly serious illness, many people do extremely well. On average, after five years of illness, more than 80% of people survive the effects of EGPA. How those with EGPA do is strongly related to how severe their illness is.

The best opportunity to limit damage to the organs is when treatment starts right away and is carefully supervised by a healthcare provider who is knowledgeable about EGPA. Even people who have the most severe EGPA can go into remission when they are treated quickly and followed closely.

Can EGPA come back?

After you go into remission, it is possible that the EGPA. can come back (relapse). People with asthma or nasal allergies can often have worsening of these symptoms that is separate from vasculitis. Relapses involving vasculitis occur in about 30% to 50% of people with EGPA. These relapses may be similar to what you experienced at the time of their vasculitis diagnosis.

It’s possible to reduce the likelihood of a severe relapse by reporting any new symptoms to your healthcare provider right away, seeing the healthcare provider on a regular basis, and having regular checkups with laboratory tests and imaging. The treatment approach for relapses is similar to that of newly diagnosed disease, and it’s possible again for these people to go into remission again.

Living With

Can I live a normal life with EGPA?

EGPA shouldn’t stop you from your normal, day-to-day activities, as long as you seek and receive treatment from your healthcare provider.

A note from Cleveland Clinic

EGPA is a serious illness that can affect a variety of organs but, fortunately, more than 80% survive the symptoms (after five years of illness). Stay in close contact with your healthcare provider and be sure to follow his or her instructions regarding your care.

Last reviewed by a Cleveland Clinic medical professional on 01/06/2021.

References

  • Vasculitis Foundation. . Accessed 1/3/2021.Eosinophilic Granulomatosis with Polyangiitis (https://www.vasculitisfoundation.org/education/forms/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome/)
  • Gioffredi A, Maritati F,1 Oliva E,1 and Buzio C. Eosinophilic Granulomatosis with Polyangiitis: An Overview. Front Immunol. 2014;5:549. Accessed 1/3/2021.

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