What are the other Names for this Condition? (Also known as/Synonyms)

• Anti-GBM Disease
• Anti-Glomerular Basement Antibody Disease
• GPS (Goodpasture Syndrome)

What is Goodpasture Syndrome? (Definition/Background Information)

• Goodpasture Syndrome (GPS) is an uncommon autoimmune disorder affecting the kidneys and lungs. The antibodies generated in this disorder are known to specifically target the tiny air sacs in the lungs called alveoli, and filtering units of the kidneys called glomeruli
• The normal function of immune system is to protect from infections and foreign particles by forming antibodies against them. In Goodpasture Syndrome, there is a malfunction of the immune system wherein it starts attacking the body’s own tissue, by forming antibodies against a common protein called collagen
• Collagen is a major component of tissues in the body. There are many types of collagen. In Goodpasture Syndrome, antibodies are formed against a specific type of collagen that is predominantly found in the lungs and kidneys
• Individuals of Caucasian origin are reported to be vulnerable to this condition. Frequently, young adult men develop Goodpasture Syndrome. Some known risk factors for developing this condition include smoking, drug use, and exposure to metallic dust or industrial toxic fumes
• While there may be a genetic cause for this condition, the collagen-specific antibodies (anti-glomerular basement membrane antibodies) that specifically target the protein in the lungs and kidneys are known to develop either sporadically, or as a result of exposure to toxins and respiratory conditions such as pneumonia
• Some common signs and symptoms of the disorder include fatigue, bloody or frothy urine, cough that may contain blood, edema, and high blood pressure. Goodpasture Syndrome can potentially lead to severe complications that include chronic kidney disease, kidney failure, and respiratory failure
• For the diagnosis of Goodpasture Syndrome, information from a physical examination, assessment of the symptoms, diagnostic tests including detection of antibodies, and biopsy of kidney tissue may be required
• This disorder is mainly treated by immunosuppressive medications and through a blood filtration procedure, known as plasmapheresis, to remove the disease-causing antibodies. Apart from this, medications to control blood pressure may be prescribed
• No specific methods or guidelines are available for the prevention of this condition. However, reducing exposure to toxic fumes and avoiding other risk factors are believed to help delay the onset of Goodpasture Syndrome
• With timely intervention and effective treatment, the prognosis of Goodpasture Syndrome is generally good. Although, individuals with extensive kidney damage might require lifelong dialysis

Who gets Goodpasture Syndrome? (Age and Sex Distribution)

• Goodpasture Syndrome is most common in early adulthood, but the condition can affect individuals of all ages
• Although both males and females are affected, the condition is reported to occur more frequently in men
• Caucasians are particularly vulnerable to Goodpasture Syndrome in comparison to individuals of other racial and ethnic groups

What are the Risk Factors for Goodpasture Syndrome? (Predisposing Factors)

The risk factors associated with Goodpasture Syndrome include:

• Smoking
• Cocaine abuse
• Exposure to metallic dusts or industrial fumes
• Viral respiratory infections such as pneumonia

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Goodpasture Syndrome? (Etiology)

Goodpasture Syndrome is caused by a malfunction of the immune system, which results in the formation of antibodies against one’s own tissue. It is an autoimmune disorder.

• The antibodies are called anti-glomerular basement membrane (anti-GBM) antibodies, and these are known to specifically target the collagen protein in the lungs and kidneys
• The development of these antibodies is unpredictable, but it may be associated with pneumonia or inhalation of industrial fumes
• Most cases of Goodpasture Syndrome are sporadic, but there is emerging data to suggest a genetic basis for this disorder

What are the Signs and Symptoms of Goodpasture Syndrome?

Some common signs and symptoms of Goodpasture Syndrome include:

• Fatigue, weakness
• Nausea and vomiting
• Shortness of breath
• Coughing, possibly with blood
• Frequent urination, particularly at night; difficulty in urinating
• Foamy and/or bloody urine
• Back pain
• Edema (swelling of the legs)
• High blood pressure
• Bleeding from the lungs
• Anemia

How is Goodpasture Syndrome Diagnosed?

For an accurate diagnosis of Goodpasture Syndrome, information from the following exams and tests may be required:

• A complete physical examination along with assessment of the individual’s medical history
• An evaluation of symptoms
• Blood tests to detect antibodies such as, anti-GBM antibody or anti-neutrophilic antibody
• Urine test, in order to check for blood or protein
• Chest X-ray to assess the extent of lung damage
• Kidney biopsy: A tissue biopsy refers to a medical procedure that involves the removal of cells or tissues (piece of kidney), which are then examined by a pathologist. This can help establish a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Goodpasture Syndrome?

Potentially, Goodpasture Syndrome may cause the following complications:

• Chronic kidney disease
• Kidney failure
• Lung/respiratory failure

How is Goodpasture Syndrome Treated?

Goodpasture Syndrome is generally treated through the following measures:

• Immunosuppressive medications such as cyclophosphamide or corticosteroids
• Medications to control blood pressure
• Plasmapheresis: A blood-filtering process that involves exchanging the non-cellular components of blood, to remove the disease-causing antibodies
• Exercise and stress reduction may help in some cases
• Some patients may require supplemental oxygen, a ventilator or blood transfusions

How can Goodpasture Syndrome be Prevented?

• There are no specific measures or guidelines available at this time for the prevention of Goodpasture Syndrome, a rare disorder
• Avoidance of risk factors (such as smoking, drug abuse, and industrial exposure to chemicals and toxins) can help reduce the likelihood of onset of the condition. However, in many cases, the cause and risk factors for GPS are unknown

What is the Prognosis of Goodpasture Syndrome? (Outcomes/Resolutions)

• The prognosis of Goodpasture Syndrome depends upon timely and efficient treatment of the disorder. In other words, the earlier the diagnosis, the better is the prognosis
• The extent or severity of lung and kidney damage may vary. If the kidneys suffer extensive damage, then lifelong dialysis may be necessary

Additional and Relevant Useful Information for Goodpasture Syndrome:

Goodpasture Syndrome is named after an American physician/pathologist, Dr. Ernest Goodpasture, who described the condition around 1920.