This rare blood vessel disease may cause breathing trouble, face pain and a persistent runny nose. Medications can help.
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.
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:
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.
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.
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:
To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including:
There's no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms.
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.
High doses of corticosteroids can cause serious side effects, so 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.
For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems.
Mepolizumab (Nucala) is currently the only drug approved by the U.S. Food and Drug Administration for treatment of Churg-Strauss syndrome. However, depending on the severity of disease and the organs involved, other medications may be required. Examples include:
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.
Long-term treatment with corticosteroids 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 improve the outlook of this condition.
You may be referred 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: