Churg-Strauss syndrome — also known as allergic granulomatosis (gran-u-loe-muh-TOE-sis) and allergic angiitis (an-je-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 symptoms — and their similarity to symptoms of other disorders — make 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 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.
Hypereosinophilia can indicate a number of allergic conditions and doesn't necessarily mean you will develop Churg-Strauss syndrome.
Broadly speaking, signs and symptoms of the hypereosinophilia phase may include:
This phase can last months or years, and your symptoms may disappear at times, only to return later. You may also experience symptoms of hypereosinophilia and systemic vasculitis — the third stage of Churg-Strauss syndrome — at the same time.
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 cause of Churg-Strauss syndrome is probably a combination of factors, although those factors haven't yet been identified. It is known that people with Churg-Strauss syndrome have an overactive immune system. Instead of simply protecting against invading organisms such as bacteria and viruses, the immune system overreacts and targets healthy tissue, causing widespread inflammation. Researchers are still trying to learn what triggers this inappropriate immune response.
The role of medications
It's not known precisely how many people have Churg-Strauss syndrome, but the disease is rare. Although may people may have risk factors for the disease, very few actually develop it.
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 improve 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
If you have seen other doctors for your symptoms before this visit, it would help to bring along a letter summarizing their findings. Taking a copy of your previous chest X-ray or sinus X-ray also could be very helpful.
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 six 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 will likely 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 and sometimes devastating 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: 2010-11-13
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