Churg-Strauss syndrome — also known as eosinophilic granulomatosis (gran-u-loe-muh-TOE-sis) with polyangiitis (pol-e-an-jee-I-tis) — is a disorder marked by blood vessel inflammation. This inflammation can restrict blood flow to vital organs and tissues, sometimes permanently damaging them.
Asthma is the most common sign of Churg-Strauss syndrome, but Churg-Strauss syndrome can cause a variety of problems, ranging from hay fever, rash and gastrointestinal bleeding to severe pain and numbness in your hands and feet. The wide range of signs and symptoms and their similarity to those of other disorders makes Churg-Strauss syndrome challenging to diagnose.
Churg-Strauss syndrome is rare and has no cure. But, your doctor can usually help you control symptoms with steroids and other powerful immunosuppressant drugs.
Churg-Strauss syndrome, which used to be known as allergic granulomatosis and allergic granulomatous angiitis, is a highly variable illness. Some people have only mild symptoms, whereas others experience severe or life-threatening complications. There are three stages, or phases, of Churg-Strauss syndrome, each with its own signs and symptoms, but not everyone develops all three phases or in the same order. This is especially true when the disease is caught and treated before the most serious damage occurs.
Churg-Strauss stages may include:
Signs and symptoms of hypereosinophilia depend on which part of your body is affected. Your lungs and digestive tract — including your stomach and esophagus — are involved most often.
Broadly speaking, signs and symptoms of the hypereosinophilia phase may include:
During this phase, you may feel generally unwell and have unintended weight loss, swollen lymph nodes, weakness and fatigue.
Depending on which organs are affected, you may also experience:
When to see a doctor
The exact cause of Churg-Strauss syndrome is unknown. It's likely that an overactive immune system response is triggered by a combination of genes and an environmental trigger, such as allergens or certain medications. Instead of simply protecting against invading organisms such as bacteria and viruses, the immune system overreacts and targets healthy tissue, causing widespread inflammation.
Some people have developed Churg-Strauss syndrome after using an asthma and allergy medication called montelukast or after switching from low-dose oral systemic steroids to inhaled steroid medications. However, no clear connection between Churg-Strauss syndrome and any medication has been proved.
Churg-Strauss syndrome is rare, affecting as few as 1 to 3 people per million.
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 may be fatal. Complications depend on the organs involved and may include:
Preparing for your appointment
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, you will likely be referred to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist.
Here's some information to help you get ready for your appointment, and what to expect from your doctor.
What you can do
For signs and symptoms common to Churg-Strauss syndrome, some basic questions to ask include:
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions at any time that you don't understand something.
What to expect from your doctor
Tests and diagnosis
There are no specific tests to confirm Churg-Strauss syndrome, and signs and symptoms are similar to those of other diseases, so it can be difficult to diagnose. To help make diagnosis easier, the American College of Rheumatology has established criteria for identifying Churg-Strauss syndrome.
The 6 criteria
To help determine whether you meet any of these criteria, your doctor is likely to request several tests, including:
Treatments and drugs
There's no cure for Churg-Strauss syndrome, but certain medications may help even people with serious symptoms achieve remission. A good outcome and a reduced risk of complications from both the disease and its treatment are more likely when Churg-Strauss syndrome is diagnosed and treated early.
Medications used to treat Churg-Strauss syndrome include:
Because of the possible connection between montelukast and Churg-Strauss syndrome, your doctor may take you off this medication to see if your signs and symptoms improve.
Although drug therapy can relieve symptoms of Churg-Strauss syndrome — and send the disease into remission — relapses are common.
Lifestyle and home remedies
Long-term treatment with prednisone can cause a number of side effects, but you can take steps to help minimize them.
Coping and support
Churg-Strauss syndrome is a serious disease. Even when it's in remission, you may worry about the possibility of recurrence or about long-term damage to your heart, lungs and nerves. Here are some suggestions for coping with the disease:
Last Updated: 2013-07-12
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