Goodpasture's syndrome is an uncommon autoimmune disorder in which bleeding into the lungs and progressive kidney failure occur.

• People usually are short of breath and cough up blood.
• Laboratory tests on samples of blood and urine and chest x-rays are needed to make the diagnosis.
• Corticosteroids, cyclophosphamideSome Trade Names
  LYOPHILIZED CYTOXAN
  (a chemotherapy drug), and plasmapheresis are used to try to prevent permanent lung and kidney damage.

This disease usually affects young men. Some people appear to be genetically susceptible to Goodpasture's syndrome. In these people, substances in the environment, such as tobacco smoke and some solvents, or a viral upper respiratory infection can cause them to produce antibodies against certain parts of their own bodies. Thus, Goodpasture's syndrome is actually an autoimmune rather than an allergic disease. These antibodies usually damage certain structures in the walls of the tiny air sacs (alveoli) and capillaries of the lungs and in the filtering apparatus of the kidneys. The antibodies trigger inflammation that interferes with lung and kidney function. Presumably, they are the direct cause of the disease.

Symptoms and Diagnosis

A person with this disease typically develops shortness of breath and coughs up blood. Symptoms can quickly become severe: Breathing can fail, and large amounts of blood can be lost. At the same time, the kidneys can rapidly fail. There may be small amounts of blood in the urine.

Laboratory tests reveal the characteristic antibodies in the blood. Urine examination reveals blood and protein in the urine. Anemia is often present. A chest x-ray shows abnormal white patches (due to lung bleeding) in both lungs. A needle biopsy specimen of kidney tissue shows microscopic deposits of antibodies in a specific pattern.

Chest X-Ray: Goodpasture's Syndrome

Lung Specimen: Goodpasture's Syndrome

Treatment

The disease may very rapidly lead to severe loss of lung function, a complete loss of kidney function, and death. High doses of corticosteroids (such as prednisone) and cyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
are given intravenously to suppress the activity of the immune system, and the person undergoes plasmapheresis—a procedure in which blood is removed from the circulation, the unwanted antibodies are removed, and the blood cells are returned to the circulation (see Blood Transfusion: Controlling Diseases by Purifying the Blood). The early use of this combination of treatments may help preserve lung and kidney function. Once damage occurs to the kidneys, it is usually permanent.

Many people may need supportive care until the disease runs its course. People may require supplemental oxygen or may need to be on a ventilator for a period of time. Blood transfusions may also be needed. If the kidneys fail, kidney dialysis or a kidney transplant may be required.

Last full review/revision September 2006 by Lee S. Newman, MD, MA