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:
This is usually the first stage of Churg-Strauss syndrome. It's marked by a number of allergic reactions, including:
An eosinophil is a type of white blood cell that helps your immune system fight certain infections. Normally, eosinophils make up only a small percentage of white blood cells, but in Churg-Strauss syndrome, abnormally high numbers of these cells (hypereosinophilia) are found in your blood or tissues, where they can cause serious damage.
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:
The hallmark of this stage of Churg-Strauss syndrome is severe blood vessel inflammation (vasculitis). By narrowing blood vessels, inflammation reduces blood flow to vital organs and tissues throughout your body, including your skin, heart, peripheral nervous system, muscles, bones and digestive tract. Occasionally, your kidneys may also be affected.
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:
See your doctor anytime 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.
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.
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 disease is generally considered to be present if a person has four of the six criteria, but your doctor may feel confident diagnosing Churg-Strauss syndrome even if you meet only two or three of the criteria, which include:
To help determine whether you meet any of these criteria, your doctor is likely to request several tests, including:
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:
Long-term treatment with prednisone can cause a number of side effects, but you can take steps to help minimize them.
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:
Churg-Strauss syndrome is rare, affecting as few as 1 to 3 people per million.
Possible risk factors for Churg-Strauss syndrome include: