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Goodpasture Syndrome Goodpasture Syndrome National Kidney and Urologic Diseases Information Clearinghouse What is Goodpasture Goodpasture syndrome is sometimes called anti-GBM disease. However, anti-GBM syndrome? disease is only one cause of pulmonary- Goodpasture syndrome is a pulmonary- renal syndromes, including Goodpasture U.S. Department renal syndrome, which is a group of acute syndrome. of Health and illnesses involving the kidneys and lungs. Human Services Goodpasture syndrome includes all of the Goodpasture syndrome is fatal unless following conditions: quickly diagnosed and treated. NATIONAL INSTITUTES OF HEALTH • glomerulonephritis—inflammation of the glomeruli, which are tiny clusters What causes Goodpasture of looping blood vessels in the kidneys syndrome? that help filter wastes and extra water The causes of Goodpasture syndrome are from the blood not fully understood. People who smoke or • the presence of anti-glomerular base- use hair dyes appear to be at increased risk ment membrane (GBM) antibodies; for this condition. Exposure to hydrocar- the GBM is part of the glomeruli and bon fumes, metallic dust, and certain drugs, is composed of collagen and other such as cocaine, may also raise a person’s proteins risk. Genetics may also play a part, as a small number of cases have been reported • bleeding in the lungs in more than one family member. In Goodpasture syndrome, immune cells produce antibodies against a specific region What are the symptoms of of collagen. The antibodies attack the col- lagen in the lungs and kidneys. Goodpasture syndrome? The symptoms of Goodpasture syndrome Ernest Goodpasture first described the may initially include fatigue, nausea, vomit- syndrome during the influenza pandemic ing, and weakness. The lungs are usually of 1919 when he reported on a patient affected before or at the same time as the who died from bleeding in the lungs and kidneys, and symptoms can include short- kidney failure. Diagnostic tools to confirm ness of breath and coughing, sometimes Goodpasture syndrome were not available with blood. The progression from initial at that time, so it is not known whether the symptoms to the lungs being affected may patient had true Goodpasture syndrome be very rapid. Symptoms that occur when or vasculitis. Vasculitis is an autoimmune the kidneys are affected include blood in the condition—a disorder in which the body’s urine or foamy urine, swelling in the legs, immune system attacks the body’s own cells and high blood pressure. and organs—that involves inflammation in the blood vessels and can cause similar lung and kidney problems. How is Goodpasture to guide the biopsy needle into the kidney. The tissue is examined in a syndrome diagnosed? lab by a pathologist—a doctor who A health care provider may order the specializes in diagnosing diseases. following tests to diagnose Goodpasture The test can show crescent-shaped syndrome: changes in the glomeruli and lines of • Urinalysis. Urinalysis is testing of a antibodies attached to the GBM. urine sample. The urine sample is col- lected in a special container in a health How is Goodpasture care provider’s office or commercial syndrome treated? facility and can be tested in the same Goodpasture syndrome is usually treated location or sent to a lab for analysis. with For the test, a nurse or technician places a strip of chemically treated • immunosuppressive medications, paper, called a dipstick, into the urine. such as cyclophosphamide, to keep Patches on the dipstick change color the immune system from making when protein or blood are present in antibodies urine. A high number of red blood • corticosteroid medications to suppress cells and high levels of protein in the the body’s autoimmune response urine indicate kidney damage. • plasmapheresis—a procedure that uses • Blood test. A blood test involves draw- a machine to remove blood from the ing blood at a health care provider’s body, separate certain cells from the office or commercial facility and send- plasma, and return just the cells to the ing the sample to a lab for analysis. person’s body; the anti-GBM antibod- The blood test can show the presence ies remain in the plasma and are not of anti-GBM antibodies. returned to the person’s body • Chest x ray. An x ray of the chest is Plasmapheresis is usually continued for sev- performed in a health care provider’s eral weeks, and immunosuppressive medi- office, outpatient center, or hospital by cations may be given for 6 to 12 months, an x-ray technician, and the images are depending on the response to therapy. In interpreted by a radiologist—a doctor most cases, bleeding in the lungs stops who specializes in medical imaging. and no permanent lung damage occurs. Abnormalities in the lungs, if present, Damage to the kidneys, however, may be can be seen on the x ray. long lasting. If the kidneys fail, blood- • Biopsy. A biopsy is a procedure that filtering treatments called dialysis or kidney involves taking a piece of kidney tissue transplantation may become necessary. for examination with a microscope. The biopsy is performed by a health care provider in a hospital with light Eating, Diet, and Nutrition sedation and local anesthetic. The Eating, diet, and nutrition have not been health care provider uses imaging shown to play a role in causing or prevent- techniques such as ultrasound or ing Goodpasture syndrome. a computerized tomography scan 2 Goodpasture Syndrome Hope through Research Points to Remember In recent years, researchers have learned • Goodpasture syndrome is a a great deal about kidney disease. The pulmonary-renal syndrome, National Institute of Diabetes and Diges- which is a group of acute illnesses tive and Kidney Diseases (NIDDK) involving the kidneys and lungs. sponsors several programs aimed at Goodpasture syndrome includes understanding rare kidney disorders. The all of the following conditions: NIDDK’s Division of Kidney, Urologic, and Hematologic Diseases supports basic – glomerulonephritis research into normal kidney function and – the presence of anti-glomerular the diseases that impair normal function at basement membrane (GBM) the cellular and molecular levels, including antibodies diabetes, high blood pressure, glomerulone- – bleeding in the lungs phritis, and cystic kidney diseases. • Goodpasture syndrome is fatal Participants in clinical trials can play a unless quickly diagnosed and more active role in their own health care, treated. gain access to new research treatments before they are widely available, and help • People who smoke or use hair others by contributing to medical research. dyes appear to be at increased risk For information about current studies, visit for this condition. Exposure to www.ClinicalTrials.gov. hydrocarbon fumes, metallic dust, and certain drugs may also raise a person’s risk. For More Information • The symptoms of Goodpasture syn- American Kidney Fund drome may initially include fatigue, 6110 Executive Boulevard, Suite 1010 nausea, vomiting, and weakness. Rockville, MD 20852 The lungs are usually affected Phone: 1–800–638–8299 before or at the same time as the Internet: www.kidneyfund.org kidneys, and symptoms can include National Kidney Foundation shortness of breath and coughing, 30 East 33rd Street sometimes with blood. Symptoms New York, NY 10016 that occur when the kidneys are Phone: 1–800–622–9010 or 212–889–2210 affected include blood in the urine Fax: 212–689–9261 or foamy urine, swelling in the legs, Internet: www.kidney.org and high blood pressure. • A urinalysis, blood test, chest x ray, and kidney biopsy are used to diag- nose Goodpasture syndrome. • Goodpasture syndrome is usually treated with immunosuppressive medications, corticosteroid medica- tions, and plasmapheresis. 3 Goodpasture Syndrome Acknowledgments National Kidney and Publications produced by the Clearinghouse Urologic Diseases are carefully reviewed by both NIDDK Information Clearinghouse scientists and outside experts. This publica- tion was reviewed by Fernando C. Fervenza, 3 Information Way M.D., Ph.D., Professor of Medicine, Mayo Bethesda, MD 20892–3580 Clinic. Phone: 1–800–891–5390 TTY: 1–866–569–1162 Fax: 703–738–4929 You may also find additional information about this Email: [email protected] topic by visiting MedlinePlus at www.medlineplus.gov. Internet: www.kidney.niddk.nih.gov This publication may contain information about medications. When prepared, this publication The National Kidney and Urologic Diseases included the most current information available. Information Clearinghouse (NKUDIC) For updates or for questions about any medications, contact the U.S. Food and Drug Administration toll- is a service of the National Institute free at 1–888–INFO–FDA (1–888–463–6332) or visit of Diabetes and Digestive and Kidney www.fda.gov. Consult your health care provider for Diseases (NIDDK). The NIDDK is part of more information. the National Institutes of Health of the U.S. Department of Health and Human Services. Established in 1987, the Clearinghouse provides information about diseases of the kidneys and urologic system to people with kidney and urologic disorders and to their families, health care professionals, and the public. The NKUDIC answers inquiries, develops and distributes publications, and works closely with professional and patient organizations and Government agencies to coordinate resources about kidney and urologic diseases. This publication is not copyrighted. The Clearing- house encourages users of this publication to dupli- cate and distribute as many copies as desired. This publication is available at www.kidney.niddk.nih.gov. U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health NIH Publication No. 12–4558 April 2012 The NIDDK prints on recycled paper with bio-based ink. .
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  • Goodpasture Syndrome
    orphananesthesia Anaesthesia recommendations for Goodpasture syndrome Disease name: Goodpasture syndrome ICD 10: M31.0 Synonyms: Goodpasture’s syndrome (GS), anti-glomerular basement membrane disease, crescentic glomerulonephritis type 1, GPS Disease summary: Goodpasture syndrome is a rare and organ-specific autoimmune disease (Gell and Coombs classification type II). It is mediated by anti-glomerular basement membrane (anti-GBM) antibodies [7]. The disease was first described by Dr. Ernest Goodpasture in 1919 [6], whereby the glomerular basement membrane was first identified as antigen in 1950s. More than one decade later, researchers succeeded in defining the association between antibodies taken from diseased kidneys and nephritis [7]. The disorder is characterised by autoantibodies targeting at the NC1-domain of the α3 chain of type IV collagen in the glomerular and alveolar basement membrane with activation of the complement cascade among other things [7,17]. The exclusive location of this α3 subunit in basement membranes only in lung and kidney is responsible for the unique affection of these two organs in GPS [7]. Nevertheless, the aetiology and the triggering stimuli for anti-GBM production remain unknown. Due to the fact that patients with specific human leukocyte antigen (HLA) types are more susceptible, a genetic predisposition HLA-associated seems possible [4,7]. However, because this strongly associated allele is frequently present, there seem to be additional behavioural or environmental factors influencing immune response and disease expression. The latter may include respiratory infections (e.g., influenza A2), exposition to hydrocarbon fumes, organic solvents, metallic dust, tobacco smoke, certain drugs (i.e., rifampicin, allopurinol, cocaine), physical damage to basement membrane (e.g., lithotripsy or membranous glomerulonephritis) as well as lymphocyte-depletion therapy (such as alemtuzumab), but unequivocal evidence is lacking [4,7,9,17].
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  • Goodpasture's Syndrome
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  • Goodpasture's Syndrome: a Nasty Dose of the 'Flu? J
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  • New Insights Into Goodpasture's Syndrome Glenn Tetsu Nagami Yale University
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