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Goodpasture Syndrome

What is Goodpasture Syndrome?

Goodpasture syndrome is a rare autoimmune disorder characterized by the presence of anti-glomerular basement membrane antibodies in the bloodstream. These antibodies attack the collagen and membranes of the lungs and kidneys causing pulmonary hemorrhage (bleeding in the lungs) and glomerulonephritis (inflammation of the glomeruli, or filtering structures, of the kidneys).

 

Synonyms

  • Anti-glomerular basement membrane disease
  • Pneumorenal syndrome

Goodpasture syndrome is a rare autoimmune disorder characterized by the presence of anti-glomerular basement membrane antibodies in the bloodstream. These antibodies attack the collagen and membranes of the lungs and kidneys causing pulmonary hemorrhage (bleeding in the lungs) and glomerulonephritis (inflammation of the glomeruli, or filtering structures, of the kidneys).

Acknowledgement of Goodpasture Syndrome has not been added yet.

Since it was first identified in 1919, there have been approximately 600 cases worldwide. The annual incidence in Europe is estimated to be approximately 0.5-1 individuals per 1,000,000 million people. The disorder appears to affect more males than females and the age of onset is usually between 20 and 30 years old. This disorder most often occurs in Caucasians.

Name Abbreviation
Anti-glomerular basement membrane disease None
Pneumorenal syndrome None

The exact cause of Goodpasture syndrome is unknown. Multiple factors may play a role in the development of the disorder including immunological, environmental and genetic factors.

Goodpasture syndrome is an autoimmune disorder, in which the body’s immune system mistakenly targets health tissue. Antibodies known as anti-glomerular basement membrane (anti-GBM) antibodies are produced by the body and mistakenly target the delicate membranes that line the lung and kidneys or the capillaries (tiny blood vessels) within the lungs or kidneys. The reason these anti-GBM antibodies are produced and attack healthy tissue in not fully understood.

Environmental factors that may contribute to the development of Goodpasture syndrome include hydrocarbon chemical exposure, cigarette smoke, exposure to metallic dust, the use of certain drugs, and infections. Sometimes, a simple infection can progress to Goodpasture syndrome because the immune system, in addition to fighting off the infection, will attack healthy tissue. Pulmonary hemorrhage is frequently linked to smoking.

Genetic factors may also play a role in the development of the disorder. Certain human leukocyte antigens (HLAs) are often found in individuals with Goodpasture syndrome. These specialized proteins can affect an individual’s risk of developing certain diseases.  

The main symptoms are excessive bleeding into the lungs and inflammation of the kidneys. Sometimes, an upper respiratory tract infection can occur just before the onset of Goodpasture syndrome.

The disorder can initially present with nonspecific symptoms such as fever, chills, nausea, vomiting, weakness and fatigue. The lungs are typically affected before or at the same time as the kidneys. Pulmonary hemorrhaging can cause hemoptysis (coughing up blood), which can range from a few specks of blood to a large amount of blood. Lung problems may also include difficulty breathing, fatigue, chest pain, a dry rasping sound from the throat (rhoncus), and/or frequent coughing. The progression from the initial symptoms of this disorder to the involvement of the lungs can be very rapid.

Inflammation of the glomeruli (filtering systems) of the kidneys may lead to impaired kidney function and ultimately kidney failure, in which the kidneys are unable to filter waste and excrete it as urine. As a result of renal failure other symptoms such as bloody or foamy urine, abnormally pale skin (pallor), drowsiness, nausea, and/or vomiting can also develop.

Without proper diagnosis and treatment, Goodpasture syndrome can be extremely serious and develop life-threatening complications because of lung disease and acute kidney failure.

Goodpasture syndrome should be suspected if an individual has symptoms such as pulmonary hemorrhaging (bleeding in the lungs) and glomerulonephritis (inflammation of kidneys). Diagnostic tests may be conducted to look for the presence of anti-GBM antibodies, high levels of protein, high number of red blood cells, abnormalities in the lungs, or changes in the glomeruli of the kidneys.

If a person is suspected of having Goodpasture syndrome, they may examine a sample of urine (urinalysis) since high levels of protein and high numbers of red blood cells in the urine are indicative of kidney damage. A blood test can also be analyzed for the presence of anti-GBM antibodies. It can also be useful to get a chest X-ray, which can reveal damage to the lungs. Another option is a biopsy, in which a small sample of kidney tissue is surgically removed and studied under a microscope. A biopsy sample can be examined for characteristic changes in the kidneys (e.g. damage to the glomeruli or the presence of anti-GBM antibodies).

Drugs that suppress the activity of the immune system, known as immunosuppressive drugs, can be used to reduce the production of harmful antibodies. In addition, corticosteroids (anti-inflammatory medicine) can be given to help to control the autoimmune response.

Many individuals are also treated by plasmapheresis, which is a procedure for removing unwanted substances from the blood. Blood is removed from the patient by a machine to separate the blood cells and plasma. The patient’s plasma is then replaced by other human plasma and the blood is transfused back into the patient. Plasmapheresis removes the harmful antibodies that damage the lungs and kidneys in Goodpasture syndrome.

If the disorder has progressed to affect the function of the kidneys or kidney failure, individuals may require dialysis, in which waste products normally removed by the kidneys are filtered by a machine, or a kidney transplant. In most individuals with proper diagnosis and treatment, damage in the lungs is not permanent, but the damage to the kidneys can be longer lasting.

With plasmapheresis, corticosteroids, and immunosuppressive agents, the prognosis for Goodpasture syndrome has improved dramatically. The 5-year survival rate now exceeds 80% and less than 30% of patients require long-term dialysis. In recent years, there has been significant progress in research on kidney diseases and people with Goodpasture syndrome can participate in clinical trials to access new treatments.

With treatment over a relatively short period of time (from a few weeks to two years), the harmful antibodies should no longer be produced. The lungs may be able to quickly recover; however, the kidneys may be severely affected.  Occasionally, changes in diet may need to be made to protect the kidneys due to high blood pressure and lower kidney function. A physician or dietitian will be able to help clarify these changes in diet. Smoking and secondary smoke are also factors that should be avoided to lower the risks of Goodpasture syndrome.  

Dammacco F, Battaglia S, Gesualdo L, Racanelli V. Goodpasture’s disease: a report of ten cases and a review of the literature. Autoimmune Rev. 2013;12(11):1101-1108.

Fervenza F. Goodpasture Syndrome. Available at: http://www.niddk.nih.gov/health-information/health-topics/kidney-disease/goodpasture-syndrome/pages/facts.aspx

Goodpasture Syndrome. National Organization for Rare Disorders (NORD) website. Available at: https://rarediseases.org/rare-diseases/goodpasture-syndrome/

Goodpasture’s Syndrome. The National Kidney Foundation website. Available at: https://www.kidney.org/atoz/content/goodpasture

Greco A, Rizzo MI, De Virgilio A, et al. Goodpasture’s syndrome: a clinical update. Autoimmun Rev. 2015;14(3)246-253.

Pedchenko V, Bondar O, Fogo AB, et al. Molecular architecture of the Goodpasture autoantigen in anti-GBM nephritis. N Engl J Med. 2010;363(4):343-354.

Robinson J. Goodpasture Syndrome Overview: Diagnosis, Symptoms, and Treatment. WebMD. Available at: http://www.webmd.com/a-to-z-guides/goodpasture-syndrome


Silberberg C. Goodpasture syndrome: MedlinePlus Medical Encyclopedia. US National Library of Medicine. Available at: https://www.nlm.nih.gov/medlineplus/ency/article/000142.htm

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