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:
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:
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:
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 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:
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.
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.
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:
Here are some suggestions for coping 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.
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:
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:
A doctor who sees you for possible Churg-Strauss syndrome is likely to ask you questions, such as: