Goodpasture syndrome

Causes

Goodpasture syndrome is an autoimmune disorder. It 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 (glomeruli) of the kidneys.

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

Sometimes this 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 of the lungs 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 very quickly over days to weeks.

Loss of appetite, fatigue, and weakness are common early symptoms.

Lung symptoms may include:

• Coughing up blood
• Dry cough
• Shortness of breath

Kidney and other symptoms include:

• Bloody urine
• Burning sensation when urinating
• Nausea and vomiting
• Pale skin
• Swelling (edema) in any area of the body, especially in the legs

Exams 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 show 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 (serum)
• Lung biopsy
• Kidney biopsy

Treatment

The main goal is to remove the harmful antibodies from the blood. A treatment called plasmapheresis removes whole blood from the body and replaces the plasma with fluid, protein, or donated plasma. Removing harmful antibodies may reduce inflammation in the kidneys and lungs.

Corticosteroid medications (such as prednisone) and other drugs that suppress or quiet the immune system may be used.

Controlling blood pressure is the most important way to delay kidney damage. You may get medicines to control high blood pressure, such as 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.

You should be closely watched for signs of kidney failure. If kidney failure becomes severe, you may need dialysis.

If you permanently lose kidney function, you may need a kidney transplant. A transplant is not done until the level of harmful antibodies drops.

Outlook (Prognosis)

An early diagnosis is very important. The 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 need dialysis or a kidney transplant.

Possible Complications

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

When to Contact a Medical Professional

Call for an appointment with your health care provider if you are producing less urine, or 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.

Alternative Names

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

References

Appel GB. Glomerular disorders and nephrotic syndromes. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 122.

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

Update Date: 9/19/2011

Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Herbert Y Lin, MD, PhD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.