What Is Churg-Strauss Syndrome?

Churg-Strauss syndrome is an extremely rare disease that causes inflammation of the blood vessels.

This condition can damage small and medium-sized blood vessels. Doctors also call it Churg-Strauss vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA), and allergic angiitis.

The condition most often affects the nose, sinuses, lungs, heart, intestines, and nerves. In about one-half to two-thirds of people with the disease, inflammation also may affect the kidneys, muscles, or joints.

What Are the Symptoms?

Unlike other types of vasculitis, many people with this condition have asthma or a higher-than-normal number of a type of blood cell called "eosinophils."

Because many different organs may be involved, many different symptoms are possible. People who have the disease may feel ill or also have:

If it affects the nerves, you may feel a burning or prickling sensation in the limbs and other parts of the body, followed by loss of sensation or loss of strength. Doctors call this “paresthesia.”

Who Gets Churg-Strauss Syndrome?

Anyone can get it. On average, people are in their 40s when they get diagnosed.

What Causes It?

Doctors don’t know. Some experts think that allergies play a role in why some people get this disease. But many people with allergies never get it.

How Do Doctors Diagnose It?

There is no single test for it. Your doctor will look at your medical history and give you a physical exam. She will ask you about your symptoms, especially asthma.

You may also get:

If your doctor thinks you have this condition, she may take a biopsy (a small tissue sample) of an affected area to see if the blood vessels in the area are inflamed. For instance, your biopsy might come from an area of your skin that has a suspicious rash.

In some cases, your doctor may recommend doing a kidney or lung biopsy.

Treatment

If you have this disease, you will need to take drugs that target your immune system. The goal is to limit or prevent damage to healthy tissues.

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Corticosteroids, usually called "steroids," are the most common drugs doctors prescribe for this condition. Most people with it take prednisone (by mouth) and prednisolone (by IV). People whose nervous system, heart, kidneys, or intestines are not affected do very well with prednisone alone.

In some cases, doctors also prescribe chemotherapy medications -- such as azathioprene (Imuran), cyclophosphamide (Cytoxan), or methotrexate. Chemo drugs are powerful medicines used to treat cancer. Churg-Strauss syndrome is not cancer, so you would probably take a lower dose than someone who has cancer. While you take these drugs, you will likely get blood tests often to check for any side effects.

Once the disease is under control, doctors slowly lower the dose of prednisone and eventually stop it entirely, if possible. If you also take chemotherapy drugs, your doctor may ease you off of it after your condition is under control. This often takes at least 6 months or up to several years.

What Is the Outlook for People With Churg-Strauss Syndrome?

In most people with the disease (more than 90%), the symptoms will disappear with treatment. This is called remission. The best results tend to happen when your doctor knows a lot about the condition and starts treatment ASAP.

People who have severe kidney, heart, intestinal tract, or brain inflammation usually do not do as well as those with milder forms of the disease that affect only the skin, joints, lungs, nose, sinuses, and ears. But when treated promptly, even people with the most severe forms of CSV can expect to get better.

WebMD Medical Reference Reviewed by David Zelman, MD on October 19, 2014

Sources

SOURCES:

Churg Strauss Syndrome Association.

The Johns Hopkins Vasculitis Center.

Mayo Clinic: "Churg-Strauss syndrome."

Merck Manual: "Eosinophilic Granulomatosis with Polyangiitis (EGPA)."

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