Goodpasture's syndrome (patient information)

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Editors-in-Chief: C. Michael Gibson, M.S., M.D. Associate Editor-In-Chief: Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]

Overview
Goodpasture syndrome is a rare disease that can involve rapidly progressive kidney failure along with lung disease.However, some forms of the disease involve just the lung or kidney, not both.

What are the symptoms of Goodpasture's syndrome?
Symptoms may occur very slowly over months or even years, but they often develop quickly over days to weeks.

Loss of appetite, fatigue, weakness are often seen at first.

Lung symptoms may include:


 * Dry cough, may cough up blood (bloody sputum)
 * Difficulty breathing after activity

Kidney and other symptoms include:


 * Swelling (edema) in any area of the body
 * Burning sensation when urinating
 * Bloody urine
 * High blood pressure
 * Nausea and vomiting
 * Pale skin

What causes Goodpasture's syndrome?
Goodpasture syndrome is an autoimmune disorder, a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue. Persons with this syndrome develop substances that attack a protein called collagen in the tiny air sacs in the lungs and the filtering units (glomureli) of the kidney.

These substances are called anti-glomerular basement membrane antibodies. Glomerular basement membrane is a part of the kidneys that helps filter waste and extra fluid from the blood. Anti-glomerular basement membrane are antibodies against this membrane. They can lead to kidney damage.

Sometimes the disorder is triggered by a viral respiratory infection or by breathing in hydrocarbon solvents. In such cases, the immune system may attack organs or tissues because it mistakes them for these viruses or foreign chemicals.

The immune system's faulty response causes bleeding in the air sacs and inflammation in the kidney's filtering units.

Who is at highest risk?
Men are eight times more likely to be affected than women. The disease most commonly occurs in early adulthood.

When to seek urgent medical care?
Call for an appointment with your health care provider if the amount of urine you produce drops, or if you have any other symptoms of Goodpasture syndrome.

Never sniff glue or siphon gasoline with your mouth, which expose the lungs to hydrocarbon solvents and can cause the disease

Diagnosis
A physical examination may reveal signs of high blood pressure and fluid overload. The health care provider may hear abnormal heart and lung sounds when listening to the chest with a stethoscope.

Urinalysis results are usually abnormal, and shows blood and protein in the urine. Abnormal red blood cells may be seen.

The following tests may also be done:


 * Anti-glomerular basement membrane test
 * Arterial blood gas
 * BUN
 * Chest x-ray
 * Creatinine
 * Lung biopsy
 * Kidney biopsy

Treatment options
The main goal is to remove the antibodies from the blood. A treatment called plasmapheresis removes blood from the body and replaces it with fluid, protein, or donated plasma. The removal of antibodies may reduce inflammation in the kidney tissues.

Corticosteroids (like prednisone) and other drugs that suppress or quiet the immune system may be used.

Controlling blood pressure is the most important measure to delay kidney damage. Medicines may be given to control high blood pressure. They include angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs).

You may be told to limit salt and fluids to control swelling. A low to moderate protein diet may be recommended in some cases.

If kidney failure becomes severe, dialysis may be needed.

Persons with this condition should be closely watched for signs of kidney failure. Dialysis may be needed.

Kidney transplantation may be need if you have permanent loss of kidney function. The transplant is usually not done until the level of antibodies drop.

Where to find medical care for Goodpasture's syndrome?
Directions to Hospitals Treating Goodpasture's syndrome

What to expect (Outlook/Prognosis)?
An early diagnosis is very important. The patient's outlook is much worse if the kidneys are already severely damaged when treatment begins. Lung damage can range from mild to severe.

Many patients will require dialysis or a kidney transplant.

Possible complications

 * Chronic kidney disease
 * End-stage kidney disease
 * Rapidly progressive glomerulonephritis
 * Lung failure
 * Severe pulmonary hemorrhage (lung bleeding)