DEFINITION

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.

SYMPTOMS

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:

Allergic stage

This is usually the first stage of Churg-Strauss syndrome. It's marked by a number of allergic reactions, including:

·        Asthma. The primary sign of Churg-Strauss syndrome, asthma develops on average three to nine years before other signs and symptoms appear. In people with Churg-Strauss syndrome who already have asthma, symptoms usually become worse and may require steroids for control. Other people develop what is known as late-onset asthma. Developing asthma, even later in life, doesn't necessarily mean that you have Churg-Strauss syndrome, however.

·        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.

Eosinophilic stage

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:

·        Fever
·        Loss of appetite
·        Weight loss
·        Asthma
·        Fatigue
·        Cough
·        Abdominal pain
·        Gastrointestinal bleeding

Vasculitic stage

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:

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

When to see a doctor

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.

CAUSES

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.

RISK FACTORS

Churg-Strauss syndrome is rare, affecting as few as 1 to 3 people per million.
Possible risk factors for Churg-Strauss syndrome include:

·        Age. On average, people with Churg-Strauss syndrome are diagnosed in their mid- to late 40s.

·        History of asthma or nasal problems. Most people diagnosed with Churg-Strauss syndrome have a history of nasal allergies, chronic sinusitis or asthma, which is often severe or hard to control. It's important to note that while almost everyone with Churg-Strauss syndrome has asthma or allergies, most people with those conditions will never develop Churg-Strauss syndrome.

COMPLICATIONS

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:

·        Peripheral nerve damage. Peripheral nerves extend throughout your body, connecting your organs, glands, muscles and skin with your spinal cord and brain. Churg-Strauss syndrome can damage peripheral nerves (peripheral neuropathy), especially those in your hands and feet, leading to numbness, burning and loss of function. Peripheral nerve problems often improve with treatment.

·        Skin scarring. The inflammation may cause sores to develop that can leave scars.

·        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.

·        Kidney (renal) damage. If Churg-Strauss syndrome affects your kidneys, you may develop glomerulonephritis, a type of kidney disease that hampers your kidneys' filtering ability, leading to a buildup of waste products in your bloodstream (uremia). Kidney failure is uncommon.

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

·        Be aware of any pre-appointment restrictions. At the time you make this appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet. Also ask if you need to stay at your doctor's office for observation following your tests.

·        Write down all your symptoms and how long they've been present — even those that seem unrelated to your main problem. Churg-Strauss syndrome can cause symptoms throughout your body and not always in a classic order. It's important that your doctor know exactly what you experienced and when.

·        Gather your key medical information, including other conditions with which you've been diagnosed and the names of all medications, vitamins and supplements you're taking. If possible, take along all of your medications in their original bottles.

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.

·        Write down key personal information, including any recent changes or stressors in your life.

·        Take a family member or friend along. Churg-Strauss syndrome is a complicated disorder, and it can be helpful to have another person who can help remember something that you forgot or missed.

·        Write down questions that you want to ask your doctor.

For signs and symptoms common to Churg-Strauss syndrome, some basic questions to ask include:

·        What's the most likely cause of my condition?
·        Are there any other possible causes for my condition?
·        What diagnostic tests do I need?
·        What treatment do you recommend?
·        How much do you expect my symptoms to improve with treatment?
·        How long will I need to take medications?
·        Am I at risk of complications from this condition, or from the medications used to treat it?
·        What steps can I take to minimize medication side effects?
·        Are there any lifestyle changes I can make to help reduce or manage my symptoms?
·        How often will you see me for follow-up tests?

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

A doctor who sees you for possible Churg-Strauss syndrome is likely to ask a number of questions, such as:

·        What are your symptoms, and when did you first notice them?
·        Have your symptoms gotten worse over time?
·        Do your symptoms include shortness of breath?
·        Do your symptoms include sinus problems?
·        Do your symptoms include any gastrointestinal problems, such as nausea, vomiting or diarrhea?
·        Have you lost weight without trying?
·        Have you been diagnosed with any other medical conditions, including allergies or asthma?
·        If you have allergies or asthma, when were you first diagnosed?
·        What medications have you taken to help manage your other conditions, and for how long?
·        Have your other conditions been getting worse or more difficult to manage?

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

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:

·        Asthma. Most people diagnosed with Churg-Strauss syndrome have chronic, often severe asthma.

·        Higher than normal count of a type of white blood cells called eosinophils (eosinophilia). Eosinophils normally make up 1 to 3 percent of white blood cells. A count higher than 10 percent is considered abnormally high and a strong indicator of Churg-Strauss syndrome.

·        Damage to one or more nerve groups (mononeuropathy or polyneuropathy). Most people with Churg-Strauss syndrome have a type of nerve damage called peripheral neuropathy, which causes numbness or pain in your hands and feet.

·        Migratory spots or lesions on a chest X-ray (pulmonary infiltrates). These lesions typically move from one place to another or come and go. On chest X-ray, the lesions mimic pneumonia.

·        Sinus problems. A history of acute or chronic sinusitis is common in people with Churg-Strauss syndrome.

·        White blood cells present outside your blood vessels (extravascular eosinophils). Your doctor may order a tissue biopsy of either your skin or a removed nasal polyp. A biopsy of a person with Churg-Strauss syndrome may show the presence of eosinophils outside of a blood vessel.

To help determine whether you meet any of these criteria, your doctor is likely to request several tests, including:

·        Blood tests. When your immune system attacks your body's own cells, as happens in Churg-Strauss syndrome, it forms proteins called autoantibodies. A blood test can detect certain autoantibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. A blood test also can measure the level of eosinophils, although an increased number of these cells may be caused by other diseases, including asthma.

·        Imaging tests. X-rays, computerized tomography (CT) scans and magnetic resonance imaging (MRI) look for abnormalities in your lungs and sinuses.

·        Biopsy of affected tissue. If other tests suggest Churg-Strauss syndrome, you may have a small sample of tissue (biopsy) removed for examination under a microscope. The doctor may remove tissue from your lungs or another organ, such as skin or muscle, to confirm or rule out the presence of vasculitis. A biopsy is usually performed only on a site that has shown some abnormality on another test.

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:

·        Corticosteroids. Prednisone is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor may prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control as soon as possible. But because high doses of corticosteroids can cause serious side effects, including bone loss, high blood sugar, weight gain, cataracts and hard-to-treat infections, 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, however.

·        Other immunosuppressive drugs. For people with mild symptoms, a corticosteroid alone may be enough. Other people may require another immunosuppressive drug, such as cyclophosphamide (Cytoxan), azathioprine (Azasan, Imuran) or methotrexate (Rheumatrex, Trexall), to reduce the body's immune reaction still further. Because these drugs impair your body's ability to fight off infection and can cause other serious side effects, your condition will be closely monitored while you're taking them.

·        Immune globulin. Given as a monthly infusion, immune globulin is generally given to people who haven't responded to other treatments. The most common side effects are flu-like symptoms that usually last just a day or so. Immune globulin has two major drawbacks, however: It's very expensive, and it doesn't work for everyone.

·        Biologic medications. Drugs such as rituximab (Rituxan) that alter the immune system's response seem to improve symptoms and decrease the number of eosinophils. However, these medications have only been studied in small trials, and their long-term safety and efficacy is still unknown. These medications may be suggested for those who haven't responded to other treatments.

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.

·        Protect your bones. If you're taking cortisone, it's essential to get adequate amounts of calcium and vitamin D to prevent bone loss and possible fractures. Ask your doctor how much vitamin D and calcium you need in your diet, and discuss whether or not you need to take any supplements.

·        Exercise. Exercise can help you maintain a healthy weight, which is important when you're taking corticosteroid medications that can cause weight gain. Strength training and weight-bearing exercises such as walking and jogging also help improve bone health.

·        Stop smoking. This is one of the most significant things you can do for your overall health. By itself, smoking causes serious health problems. It also makes problems you already have worse and can increase the side effects of medications you're taking.

·        Adopt a healthy diet. Steroids can cause high blood sugar levels and eventually, type 2 diabetes. Emphasizing foods that help keep blood sugar stable, such as fruits, vegetables and whole grains, is helpful.

·        Keep doctor appointments. During therapy for Churg-Strauss syndrome, your doctor will monitor you closely for side effects. This will likely include regular bone scans, eye exams, and tests for blood pressure, blood sugar and cholesterol levels. In addition, your doctor will look for signs of Churg-Strauss syndrome affecting new organs (relapse). Be sure to keep these appointments. If caught in time, it's possible to reverse many of the side effects resulting from steroid use.

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:

·        Educate yourself about Churg-Strauss syndrome. The more you know, the better prepared you may 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 support system. Although family and friends can help tremendously, you sometimes may find the understanding and advice of other people with Churg-Strauss syndrome especially helpful. Your doctor or a medical social worker may be able to put you in touch with a support group. Or you can look for a local or online support group through the Churg-Strauss Syndrome Association.