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$Unique_ID{BRK03779}
$Pretitle{}
$Title{Goodpasture Syndrome}
$Subject{Goodpasture Syndrome Pneumorenal Syndrome}
$Volume{}
$Log{}
Copyright (C) 1986, 1987, 1988, 1989 National Organization for Rare Disorders,
Inc.
279:
Goodpasture Syndrome
** IMPORTANT **
It is possible the main title of the article (Goodpasture Syndrome) is
not the name you expected. Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Pneumorenal Syndrome
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.
Goodpasture Syndrome is a rare inflammatory disorder involving the
membranes of the lungs and kidneys. This disorder can be classified into
three groups: autoimmune or antibody induced disease, systemic vasculitis (a
vessel inflammation which may affect the body as a whole), and idiopathic
Goodpasture (unknown cause). When antibodies cause this inflammation, they
may be deposited in capillary membranes of the lungs and kidneys. Autoimmune
syndromes are caused by the body's natural defenses (antibodies) against
invading organisms which, for unknown reasons, suddenly begin to attack the
body's own tissue.
Symptoms
The major symptoms of Goodpasture Syndrome are hemorrhage of the lungs and
kidney dysfunction.
Respiratory symptoms found in Goodpasture Syndrome may include
expectoration of blood or blood stained sputum (hemoptysis), a rasping sound
in the bronchial tubes similar to snoring (rhonchi), and breathing
difficulties (dyspnea). Less common symptoms may include fatigue, weakness,
coughing, chills and hypertension. Lungs may be affected by increased
fibrous tissue formation or degeneration.
Kidney failure may develop and progress rapidly due to inflammation
(glomerulonephritis). Anemia and pallor may appear as a consequence of
kidney dysfunction. Some blood or protein the urine may also occur.
Symptoms of Goodpasture may recur after treatment. However, continued
treatment can be effective in many patients.
Causes
Goodpasture Syndrome may be due to unknown causes, or it may be caused by
toxins such as hydrocarbon chemical exposure, or infections such as
influenza, etc. It is not known why simple infections can progress to
Goodpasture Syndrome in some people. When infection occurs, the body's
natural defenses (antibodies) fight the invading organisms (e.g., viruses or
bacteria). In autoimmune disorders, antibodies attack healthy tissue for no
apparent reason.
In Goodpasture Syndrome, antiglomerular basement membrane antibodies
appear to circulate throughout the blood and damage membranes of the lungs
and kidneys.
Affected Population
Goodpasture Syndrome seems to be found more frequently in males; it occurs
worldwide.
Related Disorders
Wegener's Granulomatosis is a rare collagen vascular disorder that begins as
a localized inflammation of the upper and lower respiratory tract mucosa, and
usually progresses into generalized inflammation of the blood vessels
(vasculitis) and kidneys (glomerulonephritis). (For more information on this
disorder, choose "Wegener" as your search term in the Rare Disease Database).
Idiopathic Pulmonary Hemosiderosis is a lung disorder similar to
Goodpasture Syndrome, with chronic secondary anemia. It seems to occur
mostly in young children, and does not have the antibody reaction found in
Goodpasture Syndrome.
Bacterial Endocarditis is a lung and kidney disorder which has some
clinical similarities to Goodpasture Syndrome, but also involves the heart.
Caused by bacterial infection, heart murmurs may occur, as well as artery
blockage (embolisms). Skin lesions, spleen enlargement, and intermittent
high fever are other symptoms of this disorder.
Therapies: Standard
Treatment of Goodpasture Syndrome may involve removal of plasma through blood
antibodies (plasmapheresis) along with use of immunosuppressive drugs. This
therapeutic approach has had a high degree of success in treatment of most
patients with Goodpasture Syndrome. Corticosteroids, alone or combined with
azathioprine or mercaptopurine, may be of benefit in some cases. Kidney
(renal) transplant may be helpful, but the long-term benefits of this
operation have not yet been established. Renal dialysis may be useful in
treating kidney failure.
According to recent scientific studies, the mortality rate for this
syndrome has dropped from eighty-six percent to thirteen percent in the years
between 1955 and 1982. Fifty-one percent of Goodpasture patients no longer
require renal dialysis. Treatment of respiratory problems with
plasmapheresis seems to be beneficial in many patients.
Therapies: Investigational
Changes from nonspecific to more selective methods of plasmapheresis are
under investigation in the treatment of Goodpasture Syndrome as well as many
other autoimmune disorders. These methods may include cascade filtration,
cryofiltration, immunoabsorption, enzymatic degradation, and continuous
electrophoresis. These procedures appear to be significant advances in
clinical and experimental immunology therapeutic research.
This disease entry is based upon medical information available through
December 1988. 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 Goodpasture Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Immune Deficiency Foundation
3565 Ellicott Mill Drive, Unit B2
Ellicott City, MD 21043
(800) 296-4433
(410) 461-3127
The National Kidney Foundation
30 East 33rd St.
New York, NY 10016
(212) 689-2210 or (800) 622-9010
American Lung Association
1740 Broadway
New York, NY 10019
(212) 315-8700
National Kidney and Urologic Diseases Information Clearinghouse
Box NKUDIC
Bethesda, MD 20892
(301) 468-6345
References
IMMUNOMODULATION WITH APHERESIS TECHNICS: A. Liebert, et. al.; Allerg
Immunol (Leipz) (1986: issue 32(1)). Pp. 5-18.
GOODPASTURE'S SYNDROME: DEVELOPMENT OF ITS PROGNOSIS FROM 1955 TO 1982: J.
Marcandoro, et. al.; Presse Med (May 28, 1985: issue 12(23)). Pp.
1483-1487.
THE CLINICAL SPECTRUM OF ACUTE GLOMERULONEPHRITIS AND LUNG HAEMORRHAGE
(GOODPASTURE'S SYNDROME): S. Holdsworth, et. al.; Q J Med (April 1985: issue
55(216)). Pp. 75-86.