Disease Information for Goodpasture's syndrome: Definition

  • A combination of pulmonary hemorrhage and proliferative glomerulonephritis; It is known also as the lung purpura glomerulonephritis complex; It is considered to be a form of hypersensitivity reaction to basement membrane in alveolae and kidney; x-ray show basilar infiltrates and kidney biopsy shows crescentic nephritis; lethal in six months without treatment [plasmapheresis and immune suppression]----------------------------------

    Goodpasture Syndrome; Pneumorenal Syndrome; Goodpasture syndrome is a rare autoimmune disorder characterized by inflammation glomeruli of the kidneys (glomerulonephritis) and excessive bleeding into the lungs (pulmonary hemorrhaging); Autoimmune syndromes occur when the body"s natural defenses (antibodies) against invading or "foreign" organisms begin to attack the body"s own tissue, often for unknown reasons; Symptoms of Goodpasture syndrome include recurrent episodes of coughing up of blood (hemoptysis), difficulty breathing (dyspnea), fatigue, chest pain, and/or anemia; In many cases, Goodpasture syndrome may result in an inability of the kidneys to process waste products from the blood and excrete them in the urine (acute renal failure); In some cases of Goodpasture syndrome, affected individuals have had an upper respiratory tract infection before the development of the disorder; The exact cause of Goodpasture syndrome is not known---------[NORD 2005]-------

    glomerulonephritis-pulmonary haemorrhage syndrome, haemorrhagic and interstitial pneumonitis with nephritis, haemorrhagic pulmo renal syndrome, pulmonary haemosiderosis with glomerulonephritis, lung purpura with nephritis, pneumorenal syndrome, pulmonary haemosiderosis with glomerulonephritis, progressive glomerulonephritis, renopulmonal syndrome; A progressive, rare, and usually fatal autoimmune disorder affecting primarily the kidney and lung; It is characterized by glomerulonephritis and intra-alveolar haemorrhage;It begins as haemoptysis, anaemia, and dyspnoea, which are followed by hematuria and proteinuria; Haemoptysis is usually accompanied by the ”butterfly-wing” type of pulmonary infiltrates that radiate from each hilar region and are visible on x-rays; Weakness, cough, pallor, low-grade fever, and tachyardia are the additional symptoms; Pathologically, there are changes typical of glomerulonephritis, haemorrhages into the alveolar spaces, deposits of hemosiderin, and thickening of the alveolar septa; It can present at any age and is most common in young men, with onset between 16 and 61 years of age (median 21 years); The glomerulonephritis is produced by antibodies against the glomerular membrane; Death is usually due to renal failure; Etiology unknown; Familial occurrence reported; The increased incidence in HLA-DRw2-carriers (relative risk 30 times higher) indicates the possibility of genetic factors---------[whonamedit_com 2005]-------------