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Churg-Strauss syndrome

Churg-Strauss syndrome Definition

Churg-Strauss syndrome is a disorder that causes inflammation in blood vessels (vasculitis), which restricts blood flow to various organs. Although the disease may involve any organ, most commonly it affects your lungs and skin. The restricted blood flow to these organs can cause temporary or permanent damage.

Asthma is a common feature of Churg-Strauss syndrome, which is also known as allergic granulomatosis and allergic angiitis. Besides inflamed blood vessels, Churg-Strauss syndrome produces a type of inflammatory response known as granuloma, which can destroy normal tissue.

Churg-Strauss syndrome, an uncommon disorder, affects males and females equally. The disease can occur at any age, but it's more commonly diagnosed in middle-aged people. People older than 65 are unlikely to develop Churg-Strauss syndrome.

There's no cure for Churg-Strauss syndrome, but, with medication, it's possible to achieve long periods with no signs or symptoms (remission).

Churg-Strauss syndrome Symptoms

There are three stages of Churg-Strauss syndrome, each with its own signs and symptoms, depending on which organs are affected. Churg-Strauss syndrome is progressive, but not everyone develops all three phases, and the phases don't always develop in order.

Allergic
This usually is the first phase (prodromal) of Churg-Strauss syndrome. In this phase, you may develop a number of allergic reactions, including:

• Asthma. If you already have asthma, it may worsen and become harder to treat. Or you may develop late-onset asthma. However, having asthma doesn't mean you have Churg-Strauss syndrome.
• Hay fever (allergic rhinitis). This affects the mucous membranes of your nose, causing runny nose, sneezing and itching.
• Sinus pain and inflammation (sinusitis). You may experience facial pain and develop nasal polyps, which are soft, noncancerous (benign) growths that develop as a result of chronic inflammation.

Hypereosinophilia
An eosinophil is a type of white blood cell that normally helps your immune system fight certain types of infections. In this phase of Churg-Strauss syndrome, your body produces abnormally high numbers of eosinophils.

The signs and symptoms of this phase depend on the area of your body that's affected. Overproduction of eosinophils in your lungs (eosinophilic pneumonia) and overproduction of eosinophils in your digestive tract (eosinophilic gastroenteritis) are common. During this phase, the signs and symptoms may worsen and improve. General signs and symptoms include:

• Fever
• Weight loss
• Fatigue
• Night sweats
• Cough
• Abdominal pain
• Gastrointestinal bleeding

Systemic vasculitis
With treatment in an earlier stage, you may not progress to this stage. The vasculitis commonly affects blood vessels in your skin, heart, lungs, central nervous system, muscles and bones (musculoskeletal system), and gastrointestinal tract. It can also affect your kidneys. Signs and symptoms depend on which organs are affected. General signs and symptoms of this phase include:

• General ill feeling (malaise)
• Swollen lymph nodes
• Weight loss
• Weakness and fatigue, possibly associated with low red blood cell count (anemia)

Depending on which of your organs are affected, you may experience:

• Rash or skin sores
• Joint aches and swelling
• Numbness and tingling in your hands and feet (peripheral neuropathy)
• Severe abdominal pain
• Diarrhea, nausea and vomiting
• Shortness of breath (dyspnea)
• Coughing up blood (hemoptysis)
• Chest pain
• Irregular heartbeat
• Blood in your urine (hematuria)

Churg-Strauss syndrome Causes

Churg-Strauss syndrome is likely due to an overzealous immune system response (autoimmune reaction). In an autoimmune reaction, white blood cells, which normally protect you from unwanted invaders, such as bacteria, viruses and allergens, attack healthy tissue, causing inflammation.

Churg-Strauss syndrome When to seek medical advice

See your doctor if you develop breathing difficulties or have a persistent runny nose that doesn't respond to over-the-counter (OTC) cold medicines, especially if it's accompanied by facial pain other possible signs and symptoms of Churg-Strauss syndrome. If you have asthma or hay fever that worsens, see your doctor. Early diagnosis and effective treatment may keep you from progressing to the vasculitis phase.

Churg-Strauss syndrome Tests and diagnosis

There are no specific tests that confirm Churg-Strauss syndrome, and its signs and symptoms are common to other diseases, as well. So it may be difficult to diagnose. Besides asking you about your signs and symptoms, conducting a physical exam and taking a medical history, your doctor may request several tests, including:

• Blood tests. When your immune system attacks your body's own cells, as happens with Churg-Strauss syndrome, it forms proteins called autoantibodies. A blood test can detect certain autoantibodies in your blood that lend support to a diagnosis of Churg-Strauss syndrome, but not confirm it. Antineutrophil cytoplasmic autoantibodies (ANCAs) appear in the blood of many people with Churg-Strauss syndrome. Your blood test also can measure the level of eosinophils, which your body produces to fight off certain infections; however, an increased number of these cells may be associated with other allergic diseases, such as asthma.
• Biopsy of affected tissue. The only way to confirm a diagnosis of Churg-Strauss syndrome is to remove a small piece of tissue from an affected organ (biopsy) and examine it under a microscope. Your doctor may remove tissue from your lungs or another organ, such as skin or muscle, to confirm or rule out the presence of vasculitis, excessive eosinophils and granulomas. Some biopsies can be performed in an outpatient setting using a numbing medication (local anesthetic). Others, such as a lung biopsy, may require hospitalization.
• Chest X-ray. This test shows cavities or nodes in your lungs. However, it can't distinguish between Churg-Strauss syndrome and other diseases that affect the lungs, such as Wegener's granulomatosis.

Churg-Strauss syndrome Complications

Churg-Strauss may affect multiple organs, including lungs, skin, gastrointestinal system, kidneys, muscles, joints and heart. Without treatment, this disease may be fatal. Complications depend on the organs that are involved, including:

• Peripheral nerve damage. The numbness, tingling and loss of function may be permanent.
• Skin scarring. The inflammation may cause sores to develop, which may lead to severe scarring.
• Heart disease. Heart-related complications of Churg-Strauss syndrome include inflammation of the membrane surrounding your heart (pericarditis), inflammation of the muscular layer of your heart wall (myocarditis), heart attack and heart failure. Most deaths that occur from Churg-Strauss syndrome are a result of heart attack or heart failure.
• Kidney (renal) damage. If Churg-Strauss syndrome affects your kidneys, you may develop glomerulonephritis, a type of kidney disease that hampers your kidneys' ability to remove waste and excess fluid from your body, causing a buildup of waste products in your bloodstream (uremia). Although kidney failure isn't common with this disease, it's possible and may be fatal.

Churg-Strauss syndrome Treatments and drugs

There's no cure for Churg-Strauss syndrome, but some medications may help you achieve remission. Early diagnosis and treatment may prevent the more serious complications of the disease.

Corticosteroids, such as prednisone, are most often prescribed for Churg-Strauss syndrome. Your doctor may prescribe a high dose — 40 milligrams (mg) to 60 mg a day — of corticosteroids to get your disease into remission as soon as possible. However, high doses of corticosteroids can cause serious side effects, such as bone loss and high blood sugar. So after a month or so, your doctor may begin to decrease the dose gradually until you're taking the smallest amount that will keep your disease under control.

For some people, a corticosteroid may be enough. However, other people require another immunosuppressive drug, such as cyclophosphamide (Cytoxan) azathioprine (Imuran) or methotrexate (Rheumatrex), to reduce the body's immune reaction.

Because these drugs suppress your immune system and hamper your body's ability to fight off infection, your doctor must monitor your condition while you're taking them.

Depending on which of your organs the disease affects, your doctor may recommend that you also see a specialist, such as a cardiologist if you have heart involvement.

Churg-Strauss syndrome Coping and support

Treatment may put and keep your Churg-Strauss syndrome in remission. However, you may feel stress about the possibility of recurrence or about any damage the disease may cause, such as to your nerves. Here are some suggestions for coping with the disease:

• Educate yourself about Churg-Strauss syndrome. The more you know, the better prepared you'll be to deal with complications or recurrences. Besides talking to your doctor, you may want to talk to a counselor or medical social worker. Or you may find it helpful to talk to other people with Churg-Strauss syndrome.
• Maintain a strong support system. Family and friends can help you tremendously as you go through this difficult time. Sometimes, though, you may find the concern and understanding of other people with Churg-Strauss syndrome especially comforting. Your doctor or a medical social worker may be able to put you in touch with a support group. Or you may find a real or virtual support group through the Churg-Strauss Syndrome Association.

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