Section 4. Lung and Airway Disorders

Chapter 51. Allergic Diseases of the Lungs

Topics: Introduction | Hypersensitivity Pneumonitis | Eosinophilic Pneumonia | Allergic Bronchopulmonary Aspergillosis | Goodpasture's Syndrome

Goodpasture's Syndrome

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

This disease usually affects young men. For unknown reasons, people with Goodpasture's syndrome produce antibodies against certain parts of their own bodies, especially certain structures in the filtering apparatus of the kidneys and in the walls of the tiny air sacs (alveoli) and capillaries of the lungs. These antibodies trigger inflammation that interferes with kidney and lung 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 kidney tissue needle biopsy shows microscopic deposits of antibodies in a specific pattern.

Treatment

The disease may very rapidly lead to a complete loss of kidney function or death. High doses of corticosteroids (such as prednisone) and cyclophosphamide may be given intravenously to suppress the activity of the immune system. The person may also undergo plasmapheresis--a procedure in which blood is removed from the circulation, the unwanted antibodies are removed from the blood, and the blood cells are returned to the circulation (see Section 14, Chapter 171). The early use of this combination of treatments may help save kidney and lung function. Once damage occurs to the kidneys, it is usually permanent.

Many people may need supportive care until the disease runs its course. Treatment may require supplemental oxygen or being 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.