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Goodpasture syndrome

Goodpasture syndrome

Kidney blood supply
Kidney blood supply

Definition:

Goodpasture syndrome is a disease that affects the kidneys and lungs. It usually involves rapidly progressive kidney failure that develops in days to weeks along with lung disease (cough, shortness of breath, and blood in the sputum).

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. This means your body makes antibodies that attack your own body tissues. In this case, antibodies form against a certain type of protein called collagen. The collagen is present in the alveoli (tiny air sacs in the lungs) and in the glomeruli (the filtering units of the kidney). These antibodies are called anti-glomerular basement membrane antibodies (or anti-GBM antibodies).

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

The antibody attack leads to bleeding in the air sacs, which causes shortness of breath, cough, and bloody sputum. It also causes inflammation in the glomeruli of the kidney, which causes blood in urine (hematuria), protein in the urine (proteinuria), or kidney failure.

Symptoms:
Signs and tests:

During a physical examination, the health care provider will usually discover that the patient has high blood pressure. The patient usually has signs of fluid overload, such as swelling, gallop rhythms of the heart, and crackle sounds in the lungs. The crackles may also be from blood in the air sacs.

The following are relevant test results:

  • Urinalysis shows blood and protein in the urine. Abnormal red blood cells may be seen.
  • BUN and creatinine levels are elevated.
  • Chest x-ray shows diseased alveoli.
  • Arterial blood gas analysis may show low oxygen in the blood.
  • Lung biopsy shows damaged alveoli.
  • Kidney biopsy shows damaged glomeruli.
  • Anti-GBM antibody levels are elevated.

Review Date: 11/16/2006
Reviewed By: David M. Charytan, MD,Department of Medicine, Division of Nephrology, Brigham and Women's Hospital, Boston, MA. Review provided by VeriMed Healthcare Network.

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