Goodpasture syndrome

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Goodpasture syndrome
Classification and external resources
Crescentic glomerulonephritis - high mag.jpg
Micrograph of a crescentic glomerulonephritis that was shown to be anti-glomerular basement membrane disease. PAS stain.
ICD-10 M31.0 (ILDS M31.010)
ICD-9 446.21
OMIM 233450
DiseasesDB 5363
MedlinePlus 000142
eMedicine med/923 ped/888
MeSH D019867

Goodpasture syndrome (GPS; also known as Goodpasture’s disease, anti-glomerular basement antibody disease, or anti-GBM disease) is a rare autoimmune disease in which antibodies attack the lungs and kidneys, leading to bleeding from the lungs and to kidney failure. It 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 the American pathologist Ernest Goodpasture of Vanderbilt University, in 1919 and was later named in his honour.[1][2]

Signs and symptoms[edit]

The anti-glomerular basement membrane (GBM) antibodies primarily attack the kidneys and lungs, although, generalised symptoms like malaise, weight loss, fatigue, fever and chills are also common, as are joint aches and pains.[3] 60-80% of those with the condition experience both lung and kidney involvement; 20-40% have kidney involvement alone and less than 10% have lung involvement alone.[3] Lung symptoms usually pre-dates kidney symptoms and usually include: coughing up blood, chest pain (in less than 50% of cases overall), cough and shortness of breath.[4] 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 pressure.[3]

Cause[edit]

Its precise cause is unknown, but it is believed that an insult to the blood vessels taking blood from and to the lungs is required in order to allow the anti-GBM antibodies to come into contact with the alveoli.[5] Examples of such an insult include:[5]

Pathophysiology[edit]

GPS causes the abnormal production of anti-GBM antibodies, by the plasma cells of the blood.[5] The anti-GBM antibodies attack the alveoli and glomeruli basement membranes.[5] These antibodies, in turn, bind their reactive epitopes to the basement membranes and activate the complement cascade, leading to the death of tagged cells.[5] T cells are also implicated.[5] It is generally considered a type II hypersensitivity reaction.[5]

Diagnosis[edit]

The diagnosis of GPS is often difficult, as numerous other diseases can cause the various manifestations of the condition and the condition itself is uncommonly rare.[6] 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 antibodies.[6] On top of the anti-GBM antibodies implicated in the disease about one in three of those affected also have cytoplasmic antineutrophilic antibodies in their bloodstream, which often pre-dates the anti-GBM antibodies by about a few months or even years.[6] The later the disease is diagnosed the worse the outcome is for the affected person.[5]

Treatment[edit]

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 weight.[7] 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 out.[7] The other parts of the blood, that is, the red blood cells, white blood cells and platelets are recycled back and given intravenously as a replacement fluid.[7] On top of this most individuals affected by the disease need to be treated with immunosuppressants (drugs that suppress the functioning of the immune system), especially cyclophosphamide, prednisone and rituximab, to prevent the formation of new anti-GBM antibodies so as to prevent further damage to the kidneys and lungs.[7] Other less toxic immunosuppressants like azathioprine may be used to maintain remission.[7]

Prognosis[edit]

Without treatment virtually every affected person will end up dying from either advanced kidney failure or lung hemorrhages.[5] With treatment the 5-year survival rate is >80% and fewer than 30% of affected individuals require long-term dialysis.[5] Likewise the median survival time is about 5.93 years in Australia and New Zealand.[5]

Epidemiology[edit]

GPS is rare affecting about 0.5-1.8 per million people per year in Europe and Asia.[5] It is also unusual among autoimmune diseases in that it is more common males than in females and is also less common in blacks than whites but more common in the Maori people of New Zealand.[5] The peak age ranges for the onset of the disease are 20-30 and 60-70 years.[5]

See also[edit]

References[edit]

  1. ^ 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:10.1097/00000441-191911000-00012. 
  2. ^ Salama AD, Levy JB, Lightstone L, Pusey CD (September 2001). "Goodpasture's disease". Lancet 358 (9285): 917–920. doi:10.1016/S0140-6736(01)06077-9. PMID 11567730. 
  3. ^ 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. 
  4. ^ Schwarz, MI (November 2013). "Goodpasture Syndrome: Diffuse Alveolar Hemorrhage and Pulmonary-Renal Syndrome". Merck Manual Professional. Retrieved 14 March 2014. 
  5. ^ 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. 
  6. ^ 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. 
  7. ^ 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 links[edit]