Pusey hammersmith hospital and imperial college london, london, united kingdom case presentation a 47yearoldwhite manwho workedas aprofessional musician presented to his general practitioner with a 3week history of malaise, loss of appetite, slight weight loss, and. Collagen is a protein that helps form connective tissue. Goodpasture syndrome is a very rare autoimmune disease thataffects the lungs and or kidneys. Pusey hammersmith hospital and imperial college london, london, united kingdom case presentation a 47yearoldwhite manwho workedas aprofessional musician presented to his general practitioner with a 3week history of malaise, loss of appetite, slight weight loss, and dark urine. Goodpasture syndrome is an autoimmune disease that affects the lungs and kidneys and is characterized by pulmonary alveolar hemorrhage bleeding in the lungs and a kidney disease known as glomerulonephritis. The disease occurs when the bodys immune system mistakenly produces antibodies against collagen in the lungs and kidneys. Goodpasture s disease is defined by the presence of autoantibodies to the alpha3 chain of type iv collagen. This is an uncommon disorder that involves quick worsening of lung disease and kidney failure. Goodpasture syndrome is sometimes called antigbm disease. Goodpasture syndrome antiglomerular basement membrane antibody disease affects persons of all ages and both sexes, but the typical patient is a young male smoker. Goodpasture syndrome national institute of diabetes and. An autoimmune disease means that the immune system, which usually protects the body from infection, attacks healthy parts of the body by mistake.
Lung involvement occurs as a result of lung injury and the exposure of new epitopes to the immune system. It is thought to attack the alpha3 subunit of type iv collagen, which has therefore been referred to as goodpasture s antigen. Goodpasture syndrome is an autoimmune disease where the immune system attacks the basement membrane in the lungs and kidneys. Antiglomerular basement membrane antigbm disease, according to the chapel hill consensus conference nomenclature, is defined as a smallvessel vasculitis affecting glomerular capillaries, pulmonary capillaries or both, with antigbm autoantibody deposition along the gbm. Antigbm disease is a small vessel vasculitis in which circulating antibodies are directed against an antigen intrinsic to the glomerular basement membrane gbm and alveolar basement membrane abm, resulting in rapidly progressive glomerulonephritis andor alveolar hemorrhage. Ernest goodpasture, who first identified the syndrome in 1919. It presents with rapidly progressive renal dysfunction often combined with lung haemorrhage. It presents with rapidly progressive renal dysfunction often combined with lung hemorrhage. Goodpastures disease is an uncommon composite of features including renal failure with pulmonary hemorrhage secondary to an autoimmune response that specifically targets these organ systems.
Goodpastures syndrome causes, symptoms, treatment and. Goodpasture syndrome, or antigbm disease, is an autoimmune pulmonaryrenal syndrome characterized by pulmonary hemorrhage, progressive glomerulonephritis, and circulating antigbm antibodies. Foodborne disease pdf file 2395 kb links to english resource summary. Characterization of absenteeismdisease in nursing workers of a. Goodpasture syndrome, also referred as antiglomerular basement membrane antigbm antibody disease, is an autoimmune disease characterized by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody. Goodpasture syndrome is a rare and potentially lifethreatening autoimmune disease. It was first described by ernest goodpasture in 1919 during an influenza epidemic. Goodpasture syndrome is a pulmonaryrenal syndrome, which is a group of acute illnesses involving the kidneys and lungs. We do not, as a matter of fact, know that the patients described by goodpasture had antigbm disease. It is thought to attack the alpha3 subunit of type iv collagen, which has therefore been referred to as goodpastures antigen. Goodpasture syndrome merck manuals consumer version. Although some patients present with relatively mild renal insufficiency, this disorder is typically associated with severe renal injury that, if untreated, progresses quickly to endstage renal failure. Goodpasture disease case history bmj best practice. Management of occult neck disease in metastatic squamous cell.
Goodpasture syndrome gps also known as goodpastures disease is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and to kidney failure some forms of the disease involve just the lung or the kidney, most times, both. Goodpasture disease is a term used to describe glomerulonephritis, with or without pulmonary hemorrhage, and the presence of circulating antigl. Goodpasture s syndrome consists of a triad of pulmonary hemorrhage, rapidly progressive glomerulonephritis and antiglomerular basement membrane antigbm antibodies, either in circulation or fixed to the kidney. Get a printable copy pdf file of the complete article. Files are available under licenses specified on their description page. Goodpasture syndrome is the combination of glomerulonephritis with alveolar hemorrhage and antigbm antibodies. Media in category goodpasture syndrome the following 3 files are in this category, out of 3 total. It causes buildup of autoimmune proteins in the kidneys and lungs that leads to damage of these organs.
Full text is available as a scanned copy of the original print version. One, goodpasture s disease, is an organspecific autoimmune disease that. The pdf file you selected should load here, if your web browser has a pdf reader. Molecular architecture of the goodpasture autoantigen in. Goodpasture s disease new secrets revealed david j. Goodpastures syndromecessation of pulmonary hemorrhage. The regimens in use have decreased the mortality rate from the original 96% to less than 20%. Goodpasture syndrome is a rare autoimmune disorder that appears to affect males more frequently than females. The antibodies attack the collagen in the lungs and kidneys.
In these people, substances in the environment, such as tobacco smoke and some solvents, or a viral upper respiratory infection or pneumonia less common can cause them to produce proteins antibodies that react and try to destroy certain parts of their own bodies. His proudest accomplishment was discovering in 1934 that mumps, a disease known since the time of hippocrates, was caused by a virus. Goodpasture syndrome nord national organization for. Normally, the immune system makes antibodies to fight off germs. Vasculitis is an autoimmune conditiona disorder in which the bodys immune system attacks the bodys own cells and organsthat involves inlammation in the blood vessels and can cause similar lung and kidney problems. Goodpastures syndrome is an autoimmune disease that affects both children and adults. Simultaneous haemoptysis and renal failure the pulmonary renal syndrome has many causes, but. In its characteristic form goodpasture s syndrome is a disease of young white men, who develop fulminating glomerulonephritis, hemorrhagic pneumonitis, and severe anemia. Goodpasture syndrome initially causes vague symptoms such as fatigue. Suspected resistance of mdtmb in multibacillary leprosy of. Dialysis uses a machine or other equipment to filter the blood.
Goodpasture syndrome an overview sciencedirect topics. The etiology of goodpasture s syndrome is still unknown. This condition can quickly progress to glomerulonephritis and kidney failure. In goodpasture syndrome, immune cells produce antibodies against a specific region of collagen. Antiglomerular basement membrane disease principal discussant. It is a type of pulmonaryrenal syndrome goodpasture syndrome is defined by. Antigbm antibody disease is one of the three major forms of rapidly progressive or crescentic glomerulonephritis. Get a printable copy pdf file of the complete article 107k, or click on a page image below. Goodpastures syndrome and panca associated vasculitis in. Goodpasture s disease, antigbm nephritis and rapidly progressive. It is due to autoantibodies against the nc1 domain of the alpha3 chain of type iv collagen. Goodpasture syndrome requires early and effective treatment if it is not to be fatal.
The pdf file you selected should load here if your web browser has a pdf reader plugin installed for example, a recent version of adobe acrobat reader. Goodpasture syndrome pulmonary disorders merck manuals. Goodpasture s disease is an uncommon composite of features including renal failure with pulmonary hemorrhage secondary to an autoimmune response that specifically targets these organ systems. Goodpasture syndrome is a rare autoimmune disease, with significant morbidity and mortality in young people and otherwise healthy population. Alternatively, the pdf file will download to your computer, where it can also. The variations in the clinical course and the pathology of the disease are discussed. Goodpasture syndrome, also known as antiglomerular basement membrane disease, is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and kidney failure.
Goodpasture syndrome most often manifests as diffuse alveolar hemorrhage and glomerulonephritis together but can occasionally cause glomerulonephritis 10 to 20% or pulmonary disease 10% alone. Rituximab, goodpasture disease, antiglomerular basementmembrane disease, induction therapy background antiglomerular basementmembrane antigbm disease is an autoimmune disorder that is characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis, which usually manifests as acute renal failure. Using immunolocalization techniques, these antibodies can also be. Since that time approximately 600 cases have been noted in the medical literature.
Antiglomerular basement membrane antigbm disease encompasses tissue injury caused by autoantibodies to constituents of the gbm, most commonly the noncollagenous domain nc1 of the alpha3 subunit of type iv collagen 14. The affliction is usually fatal within a few months, the victim dying in uremia or of massive pulmonary hemorrhage. Sattar robbins pathology harrisons principles of internal medicine 18th ed. With goodpasture syndrome, however, the immune system mistakenly makes antibodies that attack the lungs and kidneys. We present a case of biopsy proven antigbm antibody disease with normal renal function, mild urinary abnormalities and positive. Goodpasture syndrome includes all of the following conditions.
Many patients experience an acute or subacute monophasic encephalopathic course that lasts years and is not followed by any further disease. Complete disease remission is possible with prompt diagnosis and treatment. Goodpasture showed that he had mastered the study of disease through controlled experimentation. Goodpastures disease investigations bmj best practice. Age of onset is usually between 20 and 30, but individuals at any age may be affected. If chronic kidney disease is found in the early stages, it can be. Goodpastures disease is characterized by the association of pulmonary hemorrhage, extracapillary glomerulonephritis, and antiglomerular basement membrane antibodies. Causes of foodborne disease food poisoning and how good hygiene helps. Legionnaires disease pdf file 233 kb links to english resource summary. Goodpasture s syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerularalveolar basement membrane. A guide to legionnaires disease, including causes, prevention, symptoms and. Goodpasture disease is defined by the presence of autoantibodies to the alpha3 chain of type iv collagen. Goodpastures syndrome with positive canca and normal.
If the inline pdf is not rendering correctly, you can download the pdf file here. We present a case of particular interest in regards to atypical presentation, and the uncommon treatment that the patient underwent. Goodpasture syndrome is a rare autoimmune disease that affects the lungs and kidneys. Simultaneous hemoptysis and renal failure the pulmonary renal syndrome has many causes, but wher.768 1288 893 1406 1649 423 360 799 702 1211 531 1021 47 1296 1035 1322 806 1456 400 1494 36 1326 553 533 1118 1420 774 1258 606 1082 496 154 1158 1464 194 479 1108 768