Churg Strauss Syndrome
What is Churg
Strauss Syndrome?
Churg Strauss
Syndrome (CSS) is a rare systemic disease characterized by asthma, high levels
of eosinophils, and inflammation of small to medium sized blood vessels
(vasculitis). Eosinophils are elevated in the blood and tissues in CSS. The
inflammation can affect various organ systems including the lungs,
gastrointestinal tract, skin, heart and nervous system. CSS is also sometimes
referred to as allergic granulomatosis or allergic angiitis. The cause is
unknown and individuals may be affected very differently by CSS. With early
diagnosis and treatment, CSS can be successfully managed.
Symptoms
Symptoms range from
mild to a wide variety of problems depending on the organ system involved and
the severity of the disease.
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Asthma
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Recurrent sinus infection
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Pneumonia
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Numbness, tingling and/or pain in the feet or hands
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Difficulty breathing
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Chronic cough
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Rashes
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Fevers
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Night sweats
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Muscle aches
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Enlarged lymph nodes
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Weakness
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Severe abdominal pain
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Gastrointestinal bleeding
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Heart problems
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Chest pain
CSS is a progressive disease consisting of three
phases:
1.
Prodromal (Allergic)
2.
Hypereosinophilic
3.
Vasculitis
The first stage, allergic phase, typically consists of asthma, sinusitis, allergic
rhinitis, and/or recurrent respiratory infections. During this phase, the
severity of problems often increases, with the asthma, sinusitis, and rhinitis
becoming more difficult to treat and control.
During the next phase, the hypereosinophilic phase, patients may
develop chronic eosinophilic pneumonia and eosinophilic gastroenteritis
(inflammation of the digestive tract). Symptoms depend on organ involvement,
but may include weight loss, fever, night sweats, cough or abdominal pain.
Symptoms may improve and then recur over a period of months to years. With
treatment, some never experience the third phase.
The third phase is the systemic vasculitis phase during which inflammation of blood vessels throughout the body causes damage to
different organs. A biopsy is helpful in diagnosing this
vasculitis, but not always necessary. Because CSS can affect many different
organs at this stage, symptoms vary widely depending on the organ affected.
Common organs affected by CSS include the skin, heart, lungs, nervous system,
kidneys, musculoskeletal system and the gastrointestinal tract.
Diagnosis of CSS may be suspected in individuals with asthma, eosinophilia > 10% of white blood
cell count, sinusitis, pulmonary infiltrates (hazy shadows on chest x-ray), and
neuropathy. Biopsies showing vasculitis and extra vascular eosinophils make a
definitive diagnosis. Anti-neutrophil cytoplasmic antibodies (ANCA) may be
found in many patients. While a combination of these findings are suggestive of
CSS, these findings can also be seen in other disorders including Wegener’s
granulomatosis, polyarteritis nodosa and eosinophilic pneumonia. Some patients
with eosinophilic gastroenteritis may have high blood levels of eosinophils and
associated allergic diseases, such as asthma, but do not have CSS.
Treatment
There is no cure, but many people achieve
long-term remissions with medication. Treatment consists of reducing
inflammation of the blood vessels and suppressing the immune system. Recent
advances in diagnosis and treatment have greatly improved long-term outcomes.
Medications
1.
Prednisone
Systemic steroids (oral or intravenous) are
usually the initial therapy to reduce inflammation in CSS. Initially, high
doses of oral steroids are given. The steroids are then slowly tapered down to
a lower dose for maintenance. Prednisone is the most common and effective
medication used to treat CSS.
2.
Immunosuppressant
medications
Cyclophosphamide (Cytoxan) is a very powerful
medication that works by suppressing rapidly dividing cells in the immune
system. Azathioprine (Imuran), Cyclosporine and/or Mycophenolate
mofetil are also used when the disease does not respond to steroids alone. Like
cyclophosphamide, they too can cause serious side effects, but the risk is
lower. Regular blood tests are important to monitor dosing and potential side
effects.
3.
Interferon alpha
Interferon
alpha, given by intramuscular injection or under the skin, is one of the
biological drugs used in the treatment of a variety of different diseases,
including leukemia. Interferon is becoming more widely used because it usually
has less toxic side effects but still requires monitoring.
4.
Other drugs used in the treatment of Churg Strauss Syndrome
are not listed here.
More information on
the medications used to treat CSS can be found on the CSSA and APFED websites.
The initial diagnosis of CSS can be
overwhelming and often affects the entire family. Early diagnosis and medical
treatment can control the symptoms in most people. A positive attitude, a good
support system, and proper medical care are important in learning to live with
Churg Strauss Syndrome.
Content
developed in conjunction with the Churg Strauss Syndrome Association.
Updated
8-27-05
Wendy Book mail@apfed.org
About APFED
American
Partnership for Eosinophilic Disorders (APFED) is a non-profit organization
dedicated to patients and their families coping with eosinophilic
disorders. Our mission
is Education, Awareness, Support and Research. All medical information is reviewed for
accuracy by our medical advisory board. HTUwww.apfed.orgUTH
About the Churg Strauss Syndrome Association
The Churg Strauss Syndrome
Association is dedicated to the identification, treatment and cure of Churg
Strauss Syndrome. For more information, visit HTUwww.cssassociation.orgUTHh or The Vasculitis Foundation at www.vasculitisfoundation.org
Dr. Michael Wechsler welcomes
questions about Churg Strauss Syndrome. Please feel free to contact him at
Brigham and Women’s Hospital 75 Francis Street,
Boston
, MA 02115, call 617-732-8202 or by
email HTUmwechsler@rics.bwh.harvard.eduUTH
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