Overview

Churg-Strauss syndrome is a disorder marked by blood vessel inflammation. This inflammation can restrict blood flow to organs and tissues, sometimes permanently damaging them. This condition is also known as eosinophilic granulomatosis with polyangiitis (EGPA).

Adult-onset asthma is the most common sign of Churg-Strauss syndrome. The disorder can also cause other problems, such as nasal allergies, sinus problems, rash, gastrointestinal bleeding, and pain and numbness in your hands and feet.

Churg-Strauss syndrome is rare and has no cure. Symptoms can usually be controlled with steroids and other powerful immunosuppressant drugs.

Symptoms

Churg-Strauss syndrome varies greatly from person to person. Some people have only mild symptoms. Others have severe or life-threatening complications.

Also known as EGPA, the syndrome tends to occur in three stages and gets progressively worse. Almost everyone with the condition has asthma, chronic sinusitis and elevated counts of white blood cells called eosinophils.

Other signs and symptoms might include:

  • Loss of appetite and weight loss
  • Joint and muscle pain
  • Abdominal pain and gastrointestinal bleeding
  • Weakness, fatigue or a general feeling of being unwell
  • Rash or skin sores
  • Pain, numbness, and tingling in your hands and feet

When to see a doctor

See your doctor if you develop breathing difficulties or a runny nose that doesn't go away, especially if it's accompanied by persistent facial pain. Also see your doctor if you have asthma or nasal allergies that suddenly worsen.

Churg-Strauss syndrome is rare, and it's more likely that these symptoms have some other cause. But it's important that your doctor evaluate them. Early diagnosis and treatment improve the chances of a good outcome.

Causes

The cause of Churg-Strauss syndrome is largely unknown. It's likely that a combination of genes and environmental factors, such as allergens or certain medications, triggers an overactive immune system response. Instead of protecting against invading bacteria and viruses, the immune system targets healthy tissue, causing widespread inflammation.

Complications

Churg-Strauss syndrome can affect many organs, including the lungs, sinuses, skin, gastrointestinal system, kidneys, muscles, joints and heart. Without treatment, the disease can be fatal.

Complications, which depend on the organs involved, can include:

  • Peripheral nerve damage. Churg-Strauss syndrome can damage the nerves in your hands and feet, leading to numbness, burning and loss of function.
  • Heart disease. Heart-related complications of Churg-Strauss syndrome include inflammation of the membrane surrounding your heart, inflammation of the muscular layer of your heart wall, heart attack and heart failure.
  • Kidney damage. If Churg-Strauss syndrome affects your kidneys, you can develop glomerulonephritis. This disease hampers your kidneys' filtering ability, leading to a buildup of waste products in your bloodstream.

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Sept. 24, 2021
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  8. FDA approves first drug for eosinophilic granulomatosis with polyangiitis, a rare disease formerly known as Churg-Strauss syndrome. U.S. Food and Drug Administration. https://www.fda.gov/news-events/press-announcements/fda-approves-first-drug-eosinophilic-granulomatosis-polyangiitis-rare-disease-formerly-known-churg. Accessed July 14, 2021.
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