$Unique_ID{BRK03605} $Pretitle{} $Title{Churg-Strauss Syndrome} $Subject{Churg-Strauss Syndrome Allergic Angiitis and Granulomatosis Allergic Granulomatosis and Angiitis System Vasculitis with Asthma and Eosinophilia Allergic Granulomatous Angiitis Polyarteritis Nodosa Wegener's Granulomatosis Sarcoidosis Bronchopulmonary Aspergillosis Eosinophilic Pneumonia} $Volume{} $Log{} Copyright (C) 1989, 1990 National Organization for Rare Disorders, Inc. 638: Churg-Strauss Syndrome ** IMPORTANT ** It is possible that the main title of the article (Churg-Strauss Syndrome) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Allergic Angiitis and Granulomatosis Allergic Granulomatosis and Angiitis System Vasculitis with Asthma and Eosinophilia Allergic Granulomatous Angiitis 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 General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Churg-Strauss Syndrome is a lung disorder often occuring as a complication of other disorders. Allergic blood vessel inflammation (angiitis or vasculitis) is accompanied by many inflammatory nodular lesions (granulomatosis) which may be small or granular, and are made up of compactly grouped cells. The age of onset varies from 15 to 70 years of age. Symptoms An allergic reaction or asthma may precede development of Churg-Strauss Syndrome by several years. Asthma tends to subside as widespread blood vessel inflammation (vasculitis) occurs. Lung tissue infiltrations (which may be short term or persistent), fever, and weight loss are often initial signs of the disorder. Interstitial lung disease may also develop. Lesions may be found in the eyes and seizures may occur. Small inflammatory growths (granulomas) may infiltrate any tissue in the body causing deterioration, and may be accompanied by accumulations of certain white blood cells (eosinophils). Later they can join together to form larger lesions. Cells called histiocytes and a variable number of giant cells also may invade tissues, especially in the lungs. When growths center on blood vessels, both deteriorating and infiltrating inflammation can occur. These lesions can heal with or without scar formation. General discomfort (malaise), skin rash, kidney inflammation, nerve disease of the extremities (peripheral neuropathy), pain in many of the joints (asymmetric polyarthralgia), or arthritis may also occur. Causes The exact cause of Churg-Strauss Syndrome is not known. However, some scientists believe that an immune system dysfunction may be involved. Autoimmune disorders are caused when the body's natural defenses (antibodies) against invading organisms suddenly begin to attack healthy tissue. Some cases may be linked to abnormal reactions by blood cells which produce antibodies to a thyroid protein (thyroglobulin), organ wall (parietal) cells, adrenal cells, or thyroid. Affected Population Churg-Strauss Syndrome affects males and females in equal numbers. 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 collagen vascular disorder that begins as a localized inflammation of mucous membranes in the respiratory tract. It usually progresses into generalized inflammation of the blood vessels (vasculitis) and kidney (glomerulonephritis). Onset may be gradual or sudden. A severe common cold, paranasal sinusitis, ulcerations of the mucous membranes of the nose with secondary bacterial infection, middle ear infection (otitis media) with hearing loss, cough, expectoration of blood (hemoptysis), and /or pleuritis are among possible symptoms. (For more information on this disorder, choose "Wegener" as your search term in the Rare Disease Database). Sarcoidosis is a disorder which affects many body systems. It is characterized by small round lesions of granulation tissue. Symptoms vary depending on the severity of the disease and how much of the body is affected. Fever, weight loss and joint pain may be the initial symptoms. Enlarged lymph glands (lymphadenopathy) are common, but usually produce no noticeable symptoms. Lung involvement can follow lymph gland problems. Skin lesions may develop during the long-term generalized phase of the disorder, as well as grainy tissue growths (granulomas) of the nasal mucous membranes and/or eyelids (conjunctivae). (For more information on this disorder, choose "Sarcoidosis" as your search term in the Rare Disease Database). Therapies: Standard Treatment of Churg-Strauss Syndrome involves corticosteroid drugs and/or cyclophosphamide for inflammation and kidney problems. In very severe cases, intravenous methylprednisolone may be effective. An ophthalmologist should be consulted for treatment of eye (conjunctival) complications. Other treatment is symptomatic and supportive. Therapies: Investigational Plasma exchange is being tested as an experimental treatment for Churg- Strauss Syndrome in conjunction with corticosteroid and cyclophosphamide drugs. This is a method for removing unwanted substances such as toxins, metabolic substances and plasma parts from the blood by separation plasma from blood cells. This therapy is still under investigation to analyze side effects and effectiveness. More research is needed before plasma exchange can be recommended for use in all but the most severe cases of Churg-Strauss Syndrome. Studies are being conducted in the use of Sandoglobulin as a treatment for Churg Strauss-Syndrome. Further investigation is needed to determine it's safety and effectiveness. This disease entry is based upon medical information available through March 1990. 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. Resources For more information on Churg-Strauss Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 American Lung Association 1740 Broadway New York NY 10019 (212) 315-8700 NIH/National Heart, Blood & Lung Institute (NHBLI) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 1285-1286. 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; F. Lhote, et al.; Artif Orgins (February, 1988, issue 12 (1)). Pp. 27-33. ALLERGIC ANGIITIS OF CHURG AND STRAUSS SYNDROME. RESPONSE TO PULSE METHYLPREDNISOLONE; R. MacFadyen, et al.; Chest (April, 1987, issue 91 (4)). Pp. 629-631. SYSTEMIC VASCULITIS WITH ASTHMA AND EOSINOPHILIA; A CLINICAL APPROACH TO THE CHURG-STRAUSS SYNDROME; J.G. Lanham, et al.; Medicind (Baltimore), (March, 1984, issue 63 (2)). Pp. 65-81. CONJUNCTIVAL INVOLVEMENT IN CHURG-STRAUSS SYNDROME: C.L. Shields, et al,; Am J Ophthalmol (November 15, 1986, issue 102 (5)). Pp. 601-605.