Churg Strauss Syndrome

Important
It is possible that the main title of the report Churg Strauss Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

• Allergic Angiitis and Granulomatosis
• Allergic Granulomatosis and Angiitis
• Allergic Granulomatosis
• Eosinophilic Granulomatous Vasculitis
• Churg-Strauss Vasculitis

Disorder Subdivisions

• None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

• Polyarteritis Nodosa
• Wegener's Granulomatosis
• Sarcoidosis
• Bronchopulmonary Aspergillosis
• Eosinophilic Pneumonia
• Guillain-Barre Syndrome

General Discussion

Churg-Strauss Syndrome is a rare disorder that may affect multiple organ systems, particularly the lungs. The disorder is characterized by the formation and accumulation of an unusually large number of antibodies, abnormal clustering of certain white blood cells (eosinophilia), inflammation of blood vessels (vasculitis), and the development of inflammatory nodular lesions (granulomatosis).

Many individuals with Churg-Strauss Syndrome have a history of allergy. In addition, asthma and other associated lung (pulmonary) abnormalities (i.e., pulmonary infiltrates) often precede the development of the generalized (systemic) symptoms and findings seen in Churg-Strauss Syndrome by one or more years. Asthma, a chronic respiratory disorder, is characterized by inflammation and narrowing of the lungs' airways, causing difficulties breathing (dyspnea), coughing, the production of a high-pitched whistling sound while breathing (wheezing), and/or other symptoms and findings.

Nonspecific findings associated with Churg-Strauss Syndrome typically include flu-like symptoms, such as fever, a general feeling of weakness and fatigue (malaise), loss of appetite (anorexia), weight loss, and muscle pain (myalgia). Additional symptoms and findings may be variable, depending upon the specific organ systems involved. Without appropriate treatment, serious organ damage and potentially life-threatening complications may result.

Although the exact cause of Churg-Strauss Syndrome is unknown, many researchers indicate that abnormal immunologic and autoimmune factors play an important role.
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Symptoms

Churg-Strauss Syndrome is characterized by an abnormal accumulation of large number of antibodies; increased numbers of certain white blood cells (eosinophilia), indicating an inflammatory or allergic response; inflammation of veins, capillaries, and small- and medium-sized arteries; and the development of inflammatory nodular lesions (granulomatosis) within certain tissues and the walls of blood vessels. Although the lungs are often predominantly affected, multiple organ systems may be involved, including the skin, heart, and nerves outside the brain and spinal cord (peripheral nervous system).

According to reports in the medical literature, many affected individuals may have a history of allergic reactions. In addition, asthma and other pulmonary abnormalities (i.e., pulmonary infiltrates) often precede the occurrence of more systemic symptoms by one or more years. Nonspecific findings typically include flu-like symptoms, such as fever, a general feeling of ill health (malaise), muscle and joint aches (myalgia and arthralgia), loss of appetite (anorexia), and weight loss. Many affected individuals also experience severe, recurrent inflammation of membranes lining the sinuses (sinusitis), which are the cavities within bones surrounding the nose. In addition, individuals with Churg-Strauss Syndrome typically begin to experience severe asthma attacks during which inflammation and narrowing of the lungs' airways cause difficulties breathing (dyspnea); the production of a high-pitched whistling sound while breathing (wheezing); a nonproductive, hacking cough; and/or other symptoms and findings.

Many individuals with the disorder also develop rash-like skin lesions due to bleeding (hemorrhaging) into tissues under the skin (purpura). In some cases, involvement of certain nerves outside the brain and spinal cord (peripheral nerves) may result in associated numbness, tingling, and/or muscle weakness in affected areas (neuropathy). In addition, some individuals may develop cardiac complications due to involvement of the heart. In some cases, other tissues may be affected, such as the kidneys, digestive tract, eyes, or other areas of the body. Associated symptoms and findings are variable, depending upon the specific organ system involved. Without appropriate treatment, Churg-Strauss Syndrome may result in serious organ damage and potentially life-threatening complications.

Causes

The exact cause of Churg-Strauss Syndrome is not known. However, many researchers suggest that the disorder results from excessive immune responses to substances that the body perceives as foreign (hypersensitivity), such as seen in allergies and asthma*, and/or abnormal immune reactions directed against the body's own tissues (autoimmune disorder). In autoimmune disorders, the body's natural defenses (e.g., antibodies) against foreign or invading organisms begin to attack healthy tissue for unknown reasons. (*In individuals with asthma, abnormal sensitivity to certain environmental agents; such as dust or pet dander, or other factors, such as respiratory infections, smoke, cold air, or certain medications or foods, results in inflammation and swelling of the walls of large and small airways in the lungs, causing associated narrowing and obstruction.)

In some cases, Churg-Strauss Syndrome has been associated with use of zafirlukast (Accolate), a nonsteroidal medication that was approved in 1996 as a therapy for the prevention and treatment of asthma in individuals 12 years of age and older. In July 1997, the Food and Drug Administration (FDA) issued a health advisory reporting that six individuals with asthma developed Churg-Strauss Syndrome while receiving zafirlukast therapy. In all reported cases, therapy with steroidal asthma medications was discontinued or was being slowly lowered (tapered) during zafirlukast therapy. According to the FDA, the data do not definitively show that use of zafirlukast caused the condition. In addition, the FDA cautions that individuals who are receiving asthma medication should not discontinue their therapy without consulting with their physicians. New labeling for the medication zafirlukast indicates that physicians should carefully monitor affected individuals as corticosteroid dosages are lowered or such therapy is discontinued.

Zafirlukast belongs to a class of medications known as leukotriene antagonists. Leukotrienes, which occur naturally in certain white blood cells (leukocytes), produce inflammatory or allergic responses and are thought to play some role in causing particular allergies and asthma. Leukotriene antagonists, also known as antileukotrienes, are medications that serve to block the action of leukotrienes. There have been some reports in the medical literature in which therapy with montelukast, another leukotriene antagonist, has been associated with the development of Churg-Strauss Syndrome.

Some researchers suggest that the association of Churg-Strauss Syndrome and therapy with leukotriene antagonists such as zafirlukast or montelukast may be coincidental and may potentially result from withdrawal of steroidal therapy. According to one report, discontinuation of long-term steroidal therapy (i.e., oral prednisone therapy) for asthma appears to have caused or "unmasked" underlying Churg-Strauss Syndrome in an individual who did not receive antileukotriene therapy. However, per another report in the medical literature, two individuals with asthma who did not receive corticosteroid therapy developed Churg-Strauss Syndrome after treatment with zafirlukast. Further research and follow-up is necessary to determine the possible role antileukotriene therapy and discontinuation of steroidal therapy may play in causing Churg-Strauss Syndrome.

Affected Populations

Churg-Strauss Syndrome appears to affect males slightly more often than females. The mean age at onset is approximately 45 years of age, with the disease most commonly becoming apparent from about age 15 to 70.

Related Disorders

Symptoms of the following disorders can be similar to those of Churg-Strauss Syndrome. Comparisons may be useful for a differential diagnosis:

Polyarteritis Nodosa is an inflammatory blood vessel disorder that involves medium-sized and small arteries. This rare condition occurs in approximately one in 100,000 people in the U.S. Males are affected twice as often as females, and the disease may begin at any age. Abnormal immune system processes appear to cause the illness in some cases, although many diverse problems may be the cause. Blood vessel inflammation (vasculitis) is the initial symptom of this disorder. (For more information on this disorder, choose "Polyarteritis Nodosa" as your search term in the Rare Disease Database.)

Wegener's granulomatosis is an uncommon disorder characterized by inflammation of blood vessels (vasculitis) that results in damage to various organ systems of the body, most often the respiratory tract and kidneys. Symptoms may include ulcerations of the mucous membranes in the nose with secondary bacterial infection, a persistent runny nose, sinus pain, and chronic middle ear infection (otitis media) potentially resulting in hearing loss. In some cases, kidney abnormalities may progress to kidney failure, a serious complication. If the lungs are affected, a cough, expectoration of blood (hemoptysis), and inflammation of the thin membrane lining the outside of the lungs and the inside of the lung may be present. The exact cause of Wegener's granulomatosis is not known. (For more information on this disorder, choose "Wegener" as your search term in the Rare Disease Database.)

Guillain-Barré Syndrome (GBS) is a very rare, rapidly progressive disorder that consists of inflammation of the nerves (polyneuritis) and, usually, muscle weakness, often progressing to frank paralysis. Although the precise cause of GBS is unknown, a viral or respiratory infection precedes the onset of the syndrome in about half of the cases. This has led to the theory that GBS may be an autoimmune disease (caused by the body's own immune system). Damage to the covering of nerve cells (myelin) and nerve axons (the extension of the nerve cell that conducts impulses away from the nerve cell body) results in delayed nerve signal transmission. There is a corresponding weakness in the muscles that are supplied or innervated by the damaged nerves. The following variants of GBS (acute inflammatory neuropathy or acute inflammatory demyelinating polyradiculoneuropathy) are recognized: Miller Fisher syndrome, acute motor-sensory axonal polyneuropathy, acute motor axonal polyneuropathy, chronic inflammatory polyneuropathy, (chronic inflammatory demyelinating polyradiculoneuropathy; also called chronic relapsing or recurring inflammatory polyneuropathy), and chronic polyneuropathy with conduction block. (For more information on this disorder, choose "Guillain-Barre" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
The diagnosis of Churg-Strauss Syndrome may be suspected based upon a thorough clinical evaluation, characteristic physical findings, and specialized tests. For example, diagnostic procedures may include the removal (biopsy) and microscopic examination of small samples of lung tissue, demonstrating inflammation of blood vessels and other associated changes. Chest x-rays and other specialized imaging techniques, such as computerized tomography (CT) scanning, typically reveal abnormalities in the lungs (i.e., pulmonary infiltrates). During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of an organ's tissue structure. In addition, laboratory tests may be conducted, including tests that demonstrate elevated levels of certain white blood cells and further suggest the presence of an inflammatory process.

Treatment
The treatment of individuals with Churg-Strauss Syndrome often consists of therapy with agents that suppress the immune system (immunosuppressants), such as particular corticosteroid medications (e.g., oral prednisone), which may also serve to reduce inflammation, and/or drugs that inhibit the proliferation of certain cells (cytotoxic medications, e.g., cyclophosphamide). Most affected individuals respond to treatment with high doses of steroids that may then be gradually reduced. If individuals do not respond to steroid therapy alone, they may require treatment with cytotoxic drugs such as cyclophosphamide or azathioprine. In some cases, other therapies may be recommended. (For more information, please see the "Investigational Therapies" section below.) Other treatment for individuals with Churg-Strauss Syndrome is symptomatic and supportive.

Investigational Therapies

Blood plasma exchange is being tested as a treatment for individuals with Churg-Strauss Syndrome who do not adequately respond to corticosteroid or cytotoxic therapy. This procedure is a method for removing certain substances (e.g., toxins, damaging antibodies, metabolic substances, plasma parts) from the blood. Blood is withdrawn, the fluid portion of the blood (plasma) is removed, and blood cells are mixed with a plasma substitute and then retransfused into the patient. This therapy is still under investigation to analyze side effects and effectiveness. More research is needed before plasma exchange may be recommended for use in all but the most severe cases of Churg-Strauss Syndrome.

Studies are also being conducted to evaluate the use of intravenous infusions with antibodies (immunoglobulins) obtained from plasma (immune globulin [IVIG]) as a treatment for affected individuals who have an incomplete response to corticosteroid or cytotoxic therapy. According to some reports, in some individuals with Churg-Strauss Syndrome, monthly IVIG therapy may result in decreased levels of certain white blood cells (eosinophils), improved lung (pulmonary) function, and additional improvement of other associated symptoms and findings. Further studies are needed to determine the long-term safety and effectiveness of IVIG therapy in individuals with Churg-Strauss Syndrome.

Researchers are studying the uses of intravenous doses of methylprednisolone and cyclophosphamide in the treatment of individuals with Churg-Strauss Syndrome. Initial results were positive. More research is necessary to determine the long-term safety and effectiveness of this potential treatment for individuals with Churg-Strauss Syndrome.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

References

TEXTBOOKS
Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996: .

Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998: 1914.

Fishman AP., ed. Pulmonary Diseases and Disorders, 2nd ed. New York, NY: McGraw-Hill Book Company; 1988:1138-43.

Champion RH, et al., eds. Textbook of Dermatology. 5th ed. Cambridge, MA: Blackwell Scientific Publications; 1992:1956-7.

Magalini SI, et al., eds. Dictionary of Medical Syndromes. 4th ed.New York, NY: Lippincott-Raven Publishers; 1997:766.

Frank MM, et al., Samter's Immunologic Diseases, 5th ed. Boston, MA: Little, Brown and Company; 1995:510-2, 852, 884-5.

JOURNAL ARTICLES
Della Rossa A, et al., Churg-Strauss syndrome: clinical and serological features of 19 patients from an Italian centre. Rheumatology. 2002;41:1286-94.

Vemuri P, et al., Churg-Strauss syndrome: survival for 26 years. Ann Allergy Asthma Immunol. 2002;88:640-3.

Franco J, et al., Pulmonary eosinophilia associated with montelukast. Thorax. 1999;54:558-60.

Green RL, et al., Churg-Strauss syndrome after zafirlukast in two patients not receiving systemic steroid treatment. Lancet. (Feb 27 1999; 353(9154)). Pp. 725-76.

Guillevin L, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine. 1999;78:26-37.

Frosi A, et al., Churg-strauss syndrome and antiasthma therapy. (Letter.) Lancet. 1999;353:1102.

D'Cruz DP, Difficult asthma or Churg-Strauss syndrome? (Editorial.) BMJ. 1999;318:475-6.

Levy Y, et al., Marked improvement of Churg-Strauss vasculitis with intravenous gammaglobulins. South Med J. 1999;92:412-4.

Iwamato Y, et al., A case of allergic granulomatous angitis with beneficial effects of plasma exchange. Rinsho Shinkeigaku. 1997;37:115-8.

Gargulinski RB, et al., Churg-Strauss syndrome. J Am Osteopath Assoc. 1996;96:428-32.

Colucci M, et al., A case of allergic granulomatous angiitis (Churg-Strauss syndrome). Minerva Med. 1995;86:45-7.

Fregoni V, et al., Churg-Strauss syndrome with peripheral polyneuropathy refractory to steroidal and immunosuppressive therapy successfully treated with plasma exchange. Recenti Prog Med. 1995;86:353-4.

Guilevin L, et al., Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum. 1995;38:1638-45.

Lhote F, et al., Allergic angiitis with granulomatosis: the Churg and Strauss syndrome. Rev Med Interne. 1994;15:226s-33s.

Armentia A, et al., Asthma and vasculitis. Response to intravenous immunoglobulins. Allergol Immunopathol. 1993;21:47-52.

Haas C, et al., Allergic angiitis with granulomatosis: Churg-Strauss syndrome. Retrospective study of 16 cases. Ann Med Interne. 1991;142:335-42.

Hamilos DL, et al., Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin. J Allergy Clin Immunol. 1991;88:823-4.

Lhote F, et al., Complications of plasma exchange in the treatment of polyarteritis nodosa and Churg-Strauss angiitis and the contribution of adjuvant immunosuppressive therapy; a randomized trial in 72 patients. Artif Orgins. 1988;12:27-33.

MacFadyen R, et al., Allergic angiitis of Churg and Strauss syndrome. Response to pulse methylprednisolone. Chest. 1987;91:629-31.

Shields CL, et al., Conjunctival involvement in Churg-Strauss syndrome. Am J Ophthalmol. 1986;102:601-5.

Resources

Vasculitis Foundation
P.O. Box 28660
Kansas City, MO 64188
USA
Tel: (816)436-8211
Fax: (816)436-8211
Tel: (800)277-9474
Email: vf@vasculitisfoundation.org
Internet: http://www.vasculitisfoundation.org

American Lung Association
61 Broadway, 6th Floor
New York, NY 10006
USA
Tel: (212)315-8700
Fax: (212)315-8870
Tel: (800)586-4872
Internet: http://www.lungusa.org

NIH/National Heart, Lung and Blood Institute Information Center
P.O. Box 30105
Bethesda, MD 20824-0105
Tel: (301)592-8573
Fax: (301)251-1223
Email: nhlbiinfo@rover.nhlbi.nih.gov

NIH/National Institute of Allergy and Infectious Diseases
6610 Rockledge Drive
MSC 6612
Bethesda, MD 20892-6612
Tel: (301)496-5717
Fax: (301)402-3573
TDD: (800)877-8339
Internet: http://www.niaid.nih.gov/

Vasculitis of the Central Nervous System
vasculitisoftheCNS@yahoogroups.com
Tel: (770)978-5636
Email: SunnyBrook222@aol.com
Internet: http://www.VasculitisoftheCNS@yahoogroups.com

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)519-3194
Fax: (240)632-9164
Tel: (888)205-2311
TDD: (888)205-3223
Email: gardinfo@nih.gov
Internet: http://www.genome.gov/10000409

Churg Strauss Syndrome Association
PO Box 671
Southampton, MA 01073
Tel: (413)862-3636
Fax: (413)862-3636
Email: cssa@cssassociation.org
Internet: http://www.cssassociation.org

Madisons Foundation
PO Box 241956
Los Angeles, CA 90024
Tel: (310)264-0826
Fax: (310)264-4766
Email: getinfo@madisonsfoundation.org
Internet: http://www.madisonsfoundation.org

American Partnership for Eosinophilic Disorders
PO Box 29545
Atlanta, GA 30359
Tel: (713)493-7749
Fax: (713)493-7749
Email: mail@apfed.org
Internet: http://www.apfed.org

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  10/12/2007
Copyright  1989, 1990, 1997, 1999, 2002, 2003, 2007 National Organization for Rare Disorders, Inc.

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