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Pronunciations arteritis azathioprine corticosteroid cyclophosphamide eosinophil granuloma methotrexate polyp prednisone purpura

Churg-Strauss syndrome is inflammation of small blood vessels that damages organs and that usually occurs in people with a history of asthma, nasal allergies, or both.

• The cause is unknown.
• At first, people may have a runny nose or asthma for months or years or have facial pain, followed by various symptoms, depending on which organs are affected.
• Doctors base the diagnosis on symptoms and results of a physical examination, blood tests, a chest x-ray, and biopsy.
• Corticosteroids are usually effective, but if a vital organ is affected, another drug that suppresses the immune system may be used.

Churg-Strauss syndrome can occur in people of all ages. The average age at the time of diagnosis is 45 to 50. Almost all affected people have a history of asthma, nasal allergies, or both. The cause is unknown.

Inflammation may affect any organ. The nerves, sinuses, skin, joints, lungs, digestive tract, heart, and kidneys are most commonly affected. Collections of immune cells that cause inflammation (called granulomas) may form nodules in affected tissue. Granulomas can destroy normal tissue and interfere with functioning. They may also cause lumps to form under the skin.

Symptoms

At first, asthma, nasal allergies, or both may develop or worsen. People may sneeze and have a persistently runny nose and itchy eyes. Inflammation of the sinuses may cause facial pain, and polyps may develop in the nose.

People may feel generally ill and tired. They may have fevers or night sweats or lose their appetite and lose weight. Other symptoms depend on which organs are affected and may include the following:

• Muscle and joint pain
• Shortness of breath
• Cough, sometimes bringing up blood
• Chest pain
• Rashes
• Abdominal pain
• Blood in the stool
• Abnormal sensations, numbness, or weakness in a limb

Any combination of these symptoms may occur. Symptoms may occur in episodes. In subsequent episodes, people may have the same symptoms as the first episode or different ones.

Inflammation of the kidneys may not cause symptoms until the kidneys malfunction and kidney failure develops. Other complications include heart failure, heart attacks, and heart valve disorders.

Diagnosis

Early diagnosis and treatment helps prevent severe organ damage.

No single test can confirm the diagnosis. The diagnosis is made by recognizing the combination of typical symptoms and results of the physical examination and other tests.

Blood tests are done. Doctors determine how many eosinophils are in the blood. These white blood cells are produced during allergic reactions, and their number increases when Churg-Strauss syndrome is present. Doctors also look for certain antibodies (antineutrophil cytoplasmic antibodies) that may be present. A chest x-ray is done to look for inflammation in the lungs. Urine tests are done to determine whether the kidneys are affected.

A sample of inflamed tissue is taken and examined under a microscope (biopsy). A biopsy can show whether the tissue contains eosinophils or granulomas. If possible, a sample is taken from the skin or muscle because the biopsy can then be done as an outpatient procedure with only a local anesthetic. Sometimes a biopsy of lung tissue is necessary. It may require hospitalization.

Treatment

Corticosteroids (such as prednisone Some Trade Names
DELTASONE
METICORTEN
) are usually used. These drugs can reduce inflammation. If a vital organ is affected, another drug that suppresses the immune system (immunosuppressant) is also used. Azathioprine Some Trade Names
IMURAN
or methotrexate Some Trade Names
RHEUMATREX
may be used. Cyclophosphamide Some Trade Names
CYTOXAN
is used when the symptoms are severe.

After symptoms resolve, the dose of the drugs is gradually reduced, and after a while, the drugs may be stopped. If necessary, they can be started again. These drugs, especially when taken for a long time, can have serious side effects.

People with Churg-Strauss syndrome should learn about their disorder. Then they can recognize any new symptoms and report them immediately to the doctor.

Last full review/revision April 2008 by Carmen E. Gota, MD