Filed under: Heart & Vascular
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
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
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
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:
* 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. Churg-Strauss occurs only very rarely
as a complication of asthma.
* 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.
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.
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:
* Weight loss
* Night sweats
* Abdominal pain
* Gastrointestinal bleeding
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.
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
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 common to
Churg-Strauss syndrome, 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 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
Some people have developed Churg-Strauss syndrome after using an asthma
and allergy medication called montelukast or when switching from
low-dose oral systemic steroids to inhaled steroid medications. Studies
have not shown a clear link between these medications and Churg-Strauss
syndrome, however, and whether a connection exists remains a point of
controversy among experts. It may be that these medications trigger or
allow the development of more obvious symptoms for previously
undiagnosed Churg-Strauss syndrome.
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:
* Age. On average, people with Churg-Strauss syndrome are diagnosed
between ages 38 and 52. The disorder is rare in older adults and
* 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.
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. In some people, this
damage may be permanent.
* 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). Although kidney failure
isn't common with this disease, it can be fatal when it occurs.
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
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
* Write down key personal information, including any recent changes or stressors in your life.
* Take a family member or friend along. Churg-Strauss 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 is the most likely cause of my condition?
* Are there any other possible causes for my condition?
* What diagnostic tests do I need?
* What treatment approach do you recommend?
* How much do you expect my symptoms to improve with treatment?
* For 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
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?
* What specialists have you already seen, and what did they think about your symptoms?
* 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
The six 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 even if you meet only two or three of the criteria, which
* 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. Doctors tend
to classify this as pulmonary infiltrate with eosinophilia.
* 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
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. Alternately, the number of eosinophils in
your blood could be suppressed by steroid medications.
* Imaging tests. X-rays, computerized tomography (CT) scans and
magnetic resonance imaging (MRI) look for abnormalities in your lungs
* 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
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, 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
* Intravenous immunoglobulin (IVIg). Given as a monthly infusion,
IVIg is the most benign of the treatments used for Churg-Strauss
syndrome. The most common side effects are flu-like symptoms that
usually last just a day or so. IVIg has two major drawbacks, however:
It's very expensive, and it isn't universally effective. IVIg isn't
considered a first-line treatment for Churg-Strauss syndrome, but
studies have shown that it can be helpful for people who don't respond
to other medications.
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.
* Protect your bones. If you're taking cortisone, it's essential to
get adequate amounts of calcium to prevent bone loss and possible
fractures. Getting enough vitamin D is just as important. Not only does
vitamin D improve bone health by helping calcium absorption, but it also
may improve muscle strength. Scientists are continuing to study vitamin
D to determine the optimal daily dose, but it's safe to take up to
2,000 international units (IU) a day. Strength training and
weight-bearing exercises such as walking and jogging also are essential
for bone health.
* Exercise. In addition to keeping your bones strong, exercise can
help you maintain a healthy weight. This is significant because
cortisone tends to cause weight gain, which in turn can contribute to
diabetes — another side effect of cortisone therapy.
* 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, full-blown diabetes. Emphasizing foods that help keep
blood sugar on an even keel, such as fruits, vegetables and whole
grains, is key. So is having your glucose and average blood sugar (A1C)
levels checked regularly. In addition, reaching your ideal weight will
lessen some of the health risks associated with corticosteroids as well
as the risks associated with obesity.
* 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 blood pressure,
glucose and cholesterol checks. 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 associated with steroid use.
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:
* 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 real or virtual support group
through the Churg-Strauss Syndrome Association.