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Goodpasture syndrome

Definition

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.

Alternative Names

Anti-glomerular basement membrane antibody disease; Rapidly progressive glomerulonephritis with pulmonary hemorrhage; Pulmonary renal syndrome; Glomerulonephritis - pulmonary hemorrhage

Causes, incidence, and risk factors

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.

Men are eight times more likely to be affected than women. The disease most commonly occurs in early adulthood.

Symptoms

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:

Kidney and other symptoms include:

Signs and tests

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:

Treatment

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.

Expectations (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.

Complications

Calling your health care provider

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.

Prevention

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

References

Appel GB, Radhakrishnan J, D'Agatis V. Secondary Glomerular Disease. In: Brenner BM, ed. Brenner: Brenner and Rector's The Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap.31.


Review Date: 8/13/2009
Reviewed By: Parul Patel, MD, Private Practice specializing in Nephrology and Kidney and Pancreas Transplantation, Affiliated with California Pacific Medical Center, Department of Transplantation, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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