$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.