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Goodpasture syndrome

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

 

Goodpasture syndrome is a rare disease that can involve quickly worsening kidney failure and lung disease.

Some forms of the disease involve just the lung or the kidney, but not both.

Causes

 

Goodpasture syndrome is an autoimmune disorder . It occurs when the immune system mistakenly attacks and destroys healthy body tissue. People 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 antiglomerular basement membrane antibodies. The glomerular basement membrane is a part of the kidneys that helps filter waste and extra fluid from the blood. Antiglomerular 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 8 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 often abnormal, and show blood and protein in the urine. Abnormal red blood cells may be seen.

The following tests may also be done:

  • Antiglomerular 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 medicines (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 your intake of salt and fluids to control swelling. In some cases, a low-to-moderate protein diet may be recommended.

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 people will need dialysis or a kidney transplant.

 

Possible Complications

 

Untreated, this condition can lead to any of the following:

  • 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 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 exposes the lungs to hydrocarbon solvents and can cause the disease.

 

 

Open References

References

Phelps RG, Turner AN. Anti-glomerular basement membrane disease and Goodpasture disease. In: Johnson RJ, Feehally J, Floege J, eds. Comprehensive Clinical Nephrology . 5th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 24.

Salama AD, Pusey CD. Goodpasture syndrome and other antiglomerular basement membrane diseases. In: Gilbert SJ, Weiner DE, eds. National Kidney Foundation's Primer on Kidney Diseases . 6th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 21.

 
  • Kidney blood supply

    Kidney blood supply - illustration

    The renal arteries supply the kidneys with blood.

    Kidney blood supply

    illustration

    • Kidney blood supply

      Kidney blood supply - illustration

      The renal arteries supply the kidneys with blood.

      Kidney blood supply

      illustration

    A Closer Look

     

      Talking to your MD

       

        Self Care

         

          Tests for Goodpasture syndrome

           
             

            Review Date: 9/22/2015

            Reviewed By: Charles Silberberg, DO, private practice specializing in nephrology, affiliated with New York Medical College, Division of Nephrology, Valhalla, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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