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Goodpasture's syndrome

Goodpasture’s syndrome (also known as Goodpasture’s disease and anti-glomerular basement membrane disease or anti-GBM disease) was first described by Ernest Goodpasture in 1919. It is an rare condition characterised by rapid destruction of the kidneys and haemorrhaging of the lungs. Although many diseases can present with these symptoms, the name Goodpasture’s syndrome is usually reserved for the autoimmune disease produced when the patient’s immune system attacks cells presenting the Goodpasture antigen, which are found in the kidney and lung, causing damage to these organs. more...

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Signs and symptoms

Most patients present with both lung and kidney disease, however, some patients present with one of these diseases alone. The first lung symptoms usually develop days to months before kidney damage is evident.

Lung disease

Lung damage may cause nothing more serious than a dry cough and minor breathlessness and such mild symptoms may last for many years before more severe ones develop. At its most serious, however, lung damage may cause severe impairment of oxygenation so that intensive care is required. Deterioration between the two extremes may occur very rapidly, often at the same time as rapid deterioration in the kidney. The patient often does not seek medical attention until he or she begins coughing up blood. The patient may be anaemic due to loss of blood through lung haemorrhaging over a long period. In Goodpasture’s syndrome, unlike many other conditions that cause similar symptoms, lung haemorrhaging most often occurs in smokers and those with damage from lung infection or exposure to fumes.

Kidney disease

The kidney disease mostly affects the glomeruli causing a form of nephritis. It is usually not detected until a rapid advance of the disease occurs so that kidney function can be completely lost in a matter of days. Blood leaks into the urine causing haematuria, the volume urinated decreases and urea and other products usually excreted by the kidney are retained and build up in the blood. This is acute renal failure. Renal failure does not cause symptoms until more than 80% of kidney function has been lost. Symptoms include loss of appetite and sickness at first and then, when the damage is more advanced, breathlessness, high blood pressure and oedema (swelling caused by fluid retention).

Diagnosis

Because of the vagueness of early symptoms and rapid progression of the disease, diagnosis is often not reached until very late in the course of the disease. Kidney biopsy is often the fastest way to secure the diagnosis and gain information about the extent of the disease and likely effect of treatment. Tests for anti-GBM antibodies may also be useful, combined with tests for antibodies to neutrophil cytoplasmic antigens, which are also directed against the patient’s own proteins.

Pathophysiology

As with many autoimmune conditions, the precise cause of Goodpasture’s Syndrome is not yet known. It is believed to be a type II hypersensitivity reaction to Goodpasture’s antigens on the cells of the glomeruli of the kidneys and the pulmonary alveoli, whereby the immune system wrongly recognises these cells as foreign and attacks and destroys them, as it would an invading pathogen.

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Goodpasture's syndrome
From Gale Encyclopedia of Medicine, 4/6/01 by Ron Gasbarro

Definition

An uncommon and life-threatening hypersensitivity disorder believed to be an autoimmune process related to antibody formation in the body. Goodpasture's syndrome is characterized by renal (kidney) disease and lung hemorrhage.

Description

The disorder is characterized by deposits of antibodies in the membranes of both the lung and kidneys, causing both inflammation of kidney glomerulus (glomerulonephritis) and lung bleeding. It is typically a disease of young males.

Causes & symptoms

The exact cause is unknown. It is an autoimmune disorder; that is, the immune system is fighting the body's own normal tissues. Sometimes the disorder is triggered by a viral infection, or by the inhalation of gasoline or other hydrocarbon solvents. An association also exists between cigarette smoking and the syndrome.

Symptoms include foamy, bloody, or dark colored urine, decreased urine output, cough with bloody sputum, difficulty breathing after exertion, weakness, fatigue, nausea or vomiting, weight loss, nonspecific chest pain and/or pale skin.

Diagnosis

The clinician will perform a battery of tests to confirm a diagnosis. These tests include a complete blood count (CBC) to confirm anemia, iron levels to check for blood loss and blood urea nitrogen (BUN) and creatinine levels to test the kidney function. A urinalysis will be done to check for damage to the kidneys. A sputum test will be done to look for antibodies. A chest x ray will be done to assess the amount of fluid in the lung tissues. A lung needle biopsy and a kidney biopsy will show immune system deposits.

Treatment

Treatment is focused on slowing the progression of the disease. Treatment is most effective when begun early, before kidney function has deteriorated to a point where the kidney is permanently damaged, and dialysis is necessary. Corticosteroids, such as prednisone, or other anti-inflammatory medications may be used to reduce the immune response. Immune suppressants such as cyclophosphamide or azathioprine are used aggressively to reduce immune system effects.

A procedure whereby blood plasma, which contains antibodies, is removed from the body and replaced with fluids or donated plasma (plasmapheresis) may be performed daily for two or more weeks to remove circulating antibodies. It is fairly effective in slowing or reversing the disorder. Dialysis to clean the blood of wastes may be required if kidney function is poor. A kidney transplant may be successful, especially if performed after circulating antibodies have been absent for several months.

Prognosis

The probable outcome is variable. Most cases progress to severe renal failure and end-stage renal disease within months. Early diagnosis and treatment makes the probable outcome more favorable.

Prevention

No known prevention of Goodpasture's syndrome exists. People should avoid glue sniffing and the siphoning gasoline. Stopping smoking, if a family history of renal failure exists, may prevent some cases. Early diagnosis and treatment may slow progression of the disorder.

Key Terms

Antibody
A protein molecule produced by the immune system in response to a protein that is not recognized as belonging in the body.
Autoimmune disorder
An abnormality within the body whereby the immune system incorrectly attacks the body's normal tissues, thereby causing disease or organ dysfunction.
Blood urea nitrogen (BUN)
A test used to measure the blood level of urea nitrogen, a waste that is normally filtered from the kidneys.
Creatinine
A test used to measure the blood level of creatinine, a waste product filtered out of the blood by the kidneys. Higher than usual levels of this substance may indicate kidney disease.
Glomerulus (glomeruli)
A small tuft of blood capillaries in the kidney, responsible for filtering out waste products.

Further Reading

For Your Information

    Books

  • Couser, William G. "Glomerular Disorders." In Cecil Textbook of Medicine, edited by James B. Wyngaarden, et al. Philadelphia: W. B. Saunders Company, 1992.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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