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Goodpasture syndrome

goodpasture syndrome, goodpasture syndrome also known
Goodpasture syndrome GPS 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 It is thought to attack the alpha-3 subunit of type IV collagen, which has therefore been referred to as Goodpasture's antigen1 Goodpasture syndrome may quickly result in permanent lung and kidney damage, often leading to death It is treated with immunosuppressant drugs such as corticosteroids and cyclophosphamide, and with plasmapheresis, in which the antibodies are removed from the blood

The disease was first described by an American pathologist Ernest Goodpasture of Vanderbilt University in 1919 and was later named in his honor23

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Contents

  • 1 Signs and symptoms
  • 2 Cause
  • 3 Pathophysiology
  • 4 Diagnosis
  • 5 Treatment
  • 6 Prognosis
  • 7 Epidemiology
  • 8 See also
  • 9 References
  • 10 External links

Signs and symptomsedit

The antiglomerular basement membrane GBM antibodies primarily attack the kidneys and lungs, although, generalized symptoms like malaise, weight loss, fatigue, fever, and chills are also common, as are joint aches and pains4 60 to 80% of those with the condition experience both lung and kidney involvement; 20-40% have kidney involvement alone, and less than 10% have lung involvement alone4 Lung symptoms usually antedate kidney symptoms and usually include: coughing up blood, chest pain in less than 50% of cases overall, cough, and shortness of breath5 Kidney symptoms usually include blood in the urine, protein in the urine, unexplained swelling of limbs or face, high amounts of urea in the blood, and high blood pressure4

Causeedit

Its precise cause is unknown, but an insult to the blood vessels taking blood from and to the lungs is believed to be required to allow the anti-GBM antibodies to come into contact with the alveoli6 Examples of such an insult include:6

  • Exposure to organic solvents eg chloroform or hydrocarbons
  • Exposure to tobacco smoke
  • Certain gene mutations HLA-DR15
  • Infection, such as influenza A
  • Cocaine inhalation
  • Metal dust inhalation
  • Bacteraemia
  • Sepsis
  • High-oxygen environments
  • Treatment with antilymphocytic treatment especially monoclonal antibodies

Pathophysiologyedit

GPS causes the abnormal production of anti-GBM antibodies, by the plasma cells of the blood6 The anti-GBM antibodies attack the alveoli and glomeruli basement membranes6 These antibodies bind their reactive epitopes to the basement membranes and activate the complement cascade, leading to the death of tagged cells6 T cells are also implicated6 It is generally considered a type II hypersensitivity reaction6

Diagnosisedit

The diagnosis of GPS is often difficult, as numerous other diseases can cause the various manifestations of the condition and the condition itself is rare7 The most accurate means of achieving the diagnosis is testing the affected tissues by means of a biopsy, especially the kidney, as it is the best-studied organ for obtaining a sample for the presence of anti-GBM antibodies7 On top of the anti-GBM antibodies implicated in the disease, about one in three of those affected also has cytoplasmic antineutrophilic antibodies in their bloodstream, which often predates the anti-GBM antibodies by about a few months or even years7 The later the disease is diagnosed, the worse the outcome is for the affected person6

Treatmentedit

The major mainstay of treatment for GPS is plasmapheresis, a procedure in which the affected person's blood is sent through a centrifuge and the various components separated based on weight8 The plasma, clear liquid part of the blood, contains the anti-GBM antibodies that attacks the affected person's lungs and kidneys and is filtered out8 The other parts of the blood, that is, the red blood cells, white blood cells, and platelets, are recycled and given intravenously as a replacement fluid8 Most individuals affected by the disease also need to be treated with immunosuppressant drugs, especially cyclophosphamide, prednisone, and rituximab, to prevent the formation of new anti-GBM antibodies so as to prevent further damage to the kidneys and lungs8 Other, less toxic immunosuppressants such as azathioprine may be used to maintain remission8

Prognosisedit

With treatment the five-year survival rate is >80% and fewer than 30% of affected individuals require long-term dialysis6 A study performed in Australia and New Zealand demonstrated that in patients requiring renal replacement therapy including dialysis the median survival time is 593 years6 Without treatment, virtually every affected person will end up dying from either advanced kidney failure or lung hemorrhages6

Epidemiologyedit

GPS is rare, affecting about 05–18 per million people per year in Europe and Asia6 It is also unusual among autoimmune diseases in that it is more common in males than in females and is also less common in blacks than whites, but more common in the Māori people of New Zealand6 The peak age ranges for the onset of the disease are 20–30 and 60–70 years6

See alsoedit

  • HLA-DR § DR2
  • Pulmonary-renal syndrome

Referencesedit

  1. ^ http://ghrnlmnihgov/gene/COL4A3
  2. ^ Goodpasture EW 1919 "The significance of certain pulmonary lesions in relation to the etiology of influenza" Am J Med Sci 158 6: 863–870 doi:101097/00000441-191911000-00012 
  3. ^ Salama AD, Levy JB, Lightstone L, Pusey CD September 2001 "Goodpasture's disease" Lancet 358 9285: 917–920 PMID 11567730 doi:101016/S0140-67360106077-9 
  4. ^ a b c Kathuria, P; Sanghera, P; Stevenson, FT; Sharma, S; Lederer, E; Lohr, JW; Talavera, F; Verrelli, M 21 May 2013 Batuman, C, ed "Goodpasture Syndrome Clinical Presentation" Medscape Reference WebMD Retrieved 14 March 2014 
  5. ^ Schwarz, MI November 2013 "Goodpasture Syndrome: Diffuse Alveolar Hemorrhage and Pulmonary-Renal Syndrome" Merck Manual Professional Retrieved 14 March 2014 
  6. ^ a b c d e f g h i j k l m n Kathuria, P; Sanghera, P; Stevenson, FT; Sharma, S; Lederer, E; Lohr, JW; Talavera, F; Verrelli, M 21 May 2013 Batuman, C, ed "Goodpasture Syndrome" Medscape Reference WebMD Retrieved 14 March 2014 
  7. ^ a b c Kathuria, P; Sanghera, P; Stevenson, FT; Sharma, S; Lederer, E; Lohr, JW; Talavera, F; Verrelli, M 21 May 2013 Batuman, C, ed "Goodpasture Syndrome Workup" Medscape Reference WebMD Retrieved 14 March 2014 
  8. ^ a b c d e Kathuria, P; Sanghera, P; Stevenson, FT; Sharma, S; Lederer, E; Lohr, JW; Talavera, F; Verrelli, M 21 May 2013 Batuman, C, ed "Goodpasture Syndrome Treatment & Management" Medscape Reference WebMD Retrieved 14 March 2014 

External linksedit

  • GBM antibodies: immunofluorescence image

goodpasture syndrome, goodpasture syndrome also known, goodpasture syndrome electron microscopy, goodpasture syndrome hypersensitivity, goodpasture syndrome mayo clinic, goodpasture syndrome medscape, goodpasture syndrome pathophysiology, goodpasture syndrome prognosis, goodpasture syndrome treatment, goodpasture syndrome wikipedia


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Goodpasture syndrome


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    Goodpasture syndrome beatiful post thanks!

    29.10.2014


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