home
***
CD-ROM
|
disk
|
FTP
|
other
***
search
/
CD-ROM Today (UK) (Spanish) 15
/
CDRT.iso
/
dp
/
0360
/
03605.txt
< prev
next >
Wrap
Text File
|
1994-01-17
|
9KB
|
193 lines
$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.