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).
Asthma is the most common sign of Churg-Strauss syndrome. The disorder can also cause other problems, such as hay fever, rash, gastrointestinal bleeding, and pain and numbness in your hands and feet.
Churg-Strauss syndrome is rare and has no cure. Your doctor can usually help you control symptoms with steroids and other powerful immunosuppressant drugs.
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. Asthma usually begins five to nine years before the diagnosis of Churg-Strauss syndrome.
Other signs and symptoms might include:
- Hay fever
- 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
- Severe abdominal pain
- Shortness of breath
- Blood in your urine or stools
When to see a doctor
See your doctor if you develop signs and symptoms such as 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 hay fever that suddenly worsens.
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.
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.
Possible risk factors for Churg-Strauss syndrome include:
- Age. On average, people with Churg-Strauss syndrome are diagnosed between 30 and 50 years of age.
- History of asthma or nasal problems. Most people diagnosed with Churg-Strauss syndrome have a history of severe nasal allergies, chronic sinusitis or asthma.
Churg-Strauss syndrome can affect many organs, including your lungs, 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.
- Skin scarring. The inflammation can cause sores that can leave scars.
- 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.
No specific test can confirm Churg-Strauss syndrome. Because signs and symptoms are similar to those of other diseases, it can be difficult to diagnose.
The early signs and symptoms, such as asthma and sinusitis, tend to be fairly commonplace, so a diagnosis might not be made until the inflammation has caused serious damage to organs and nerves.
The 6 criteria
The American College of Rheumatology has established criteria for identifying Churg-Strauss syndrome. You're generally considered to have the condition if you have four of the six criteria. The criteria are:
- Asthma. Most people diagnosed with Churg-Strauss syndrome have chronic, often severe asthma.
- Higher than normal count of a type of white blood cells called eosinophils. Eosinophils normally make up 1% to 3% of white blood cells. A count higher than 10% is considered abnormally high and a strong indicator of Churg-Strauss syndrome.
- Damage to 1 or more nerve groups. Most people with Churg-Strauss syndrome have a type of nerve damage that causes numbness or pain in your hands and feet.
- Migratory spots or lesions on a chest X-ray. These lesions typically move from one place to another or come and go. On chest X-rays, the lesions mimic pneumonia.
- Sinus problems. A history of acute or chronic sinusitis is common in people with Churg-Strauss syndrome.
- White blood cells present outside your blood vessels. Your doctor might order a tissue biopsy of either your skin or a removed nasal polyp to look for eosinophils outside of a blood vessel.
To diagnose Churg-Strauss syndrome, your doctor is likely to request several tests, including:
Blood tests. When your immune system attacks your body's own cells, as happens in Churg-Strauss syndrome, it forms proteins called autoantibodies.
A blood test can detect certain autoantibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. It can also measure the level of eosinophils, although other diseases, including asthma, can increase the number of these cells.
- Imaging tests. X-rays and CT scans look for abnormalities in your lungs and sinuses.
- Biopsy of affected tissue. If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. The tissue can come from your lungs or another organ, such as skin or muscle, to confirm or rule out the presence of vasculitis.
There's no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms.
Medications used to treat Churg-Strauss syndrome include:
Corticosteroids. Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly.
But because high doses of corticosteroids can cause serious side effects, your doctor will decrease the dose gradually until you're taking the smallest amount that will keep your disease under control. Even lower doses taken for extended periods can cause side effects.
Side effects of corticosteroids include bone loss, high blood sugar, weight gain, cataracts and hard-to-treat infections.
Other immunosuppressive drugs. For people with mild symptoms, a corticosteroid alone may be enough. Other people require another immunosuppressive drug, such as cyclophosphamide, azathioprine (Azasan, Imuran) or methotrexate (Trexall), to further reduce the body's immune reaction.
Because these drugs impair your body's ability to fight infection and can cause other serious side effects, your condition will be closely monitored while you're taking them.
- Immune globulin. Given as a monthly infusion, immune globulin is generally given to people who haven't responded to other treatments. The most common side effects are flu-like symptoms that usually last just a day or so. Immune globulin is expensive and doesn't work for everyone.
Biologic medications. Drugs such as the recently Food and Drug Administration-approved mepolizumab (Nucala), as well as benralizumab (Fasenra) and rituximab (Rituxan), alter the immune system's response and seem to improve symptoms and decrease the number of eosinophils.
These medications have been studied only in small trials, and their long-term safety and effectiveness are still unknown.
Drug therapy can relieve symptoms of Churg-Strauss syndrome and send the disease into remission. But relapses are common.
Your doctor likely will conduct blood and other tests regularly to monitor your condition and your reaction to the drugs you're taking.
Long-term treatment with prednisone can cause a number of side effects. You can minimize these problems by taking the following steps:
- Protect your bones. Ask your doctor how much vitamin D and calcium you need in your diet, and discuss whether you should take supplements.
- Exercise. Exercise can help you maintain a healthy weight, which is important when you're taking corticosteroid medications that can cause weight gain. Strength training and weight-bearing exercises such as walking and jogging also help improve bone health.
- Stop smoking. This is one of the most important things you can do for your overall health. Besides causing serious health problems, smoking also worsens problems you have and can increase side effects of medications.
- Eat a healthy diet. Steroids can cause high blood sugar levels and, eventually, type 2 diabetes. Eat foods that help keep blood sugar stable, such as fruits, vegetables and whole grains.
Here are some suggestions for coping with Churg-Strauss syndrome:
- Educate yourself. Knowing about the disease can help prepare you to deal with complications or recurrences.
- Build a support system. Family and friends can help tremendously. You might also want to talk to a counselor or medical social worker who's familiar with the disease. Or you may find it helpful to talk to other people with Churg-Strauss syndrome.
If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improves the outlook of this condition.
If your primary care doctor suspects Churg-Strauss syndrome, he or she will likely refer you to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist. You might also see a pulmonologist since Churg-Strauss affects your respiratory tract.
Here's some information to help you get ready for your appointment.
What you can do
When you make an appointment, ask if you need to do anything in advance, such as restrict your diet. Also ask if you need to stay at your doctor's office for observation following your tests.
Make a list of:
- Your symptoms and when they began, even those that seem unrelated to Churg-Strauss syndrome
- Key medical information, including other conditions with which you've been diagnosed
- All medications, vitamins and other supplements you take, including doses
- Questions to ask your doctor
If you've seen other doctors for your condition, bring a letter summarizing their findings and copies of recent chest X-rays or sinus X-rays. Take a family member or friend along to help you remember the information you receive.
Basic questions to ask your doctor might include:
- What's the most likely cause of my condition?
- What are other possible causes?
- What diagnostic tests do I need?
- What treatment do you recommend?
- What life changes can I make to help reduce or manage my symptoms?
- How often will you see me for follow-up tests?
What to expect from your doctor
A doctor who sees you for possible Churg-Strauss syndrome is likely to ask you questions, such as:
- Have your symptoms, especially asthma-related, worsened over time?
- Do your symptoms include shortness of breath or wheezing?
- Do your symptoms include sinus problems?
- Do your symptoms include gastrointestinal problems, such as nausea, vomiting or diarrhea?
- Have you been having numbness, pain, or weakness in an arm or leg?
- Have you lost weight without trying?
- Have you been diagnosed with any other medical conditions, including allergies or asthma? If so, how long have you had them?