(Goodpasture Disease; Goodpasture's Syndrome; Anti-GBM Antibody Disease)
People usually have difficulty breathing and may cough up blood.
Laboratory tests on samples of blood and urine and a chest x-ray are needed to make the diagnosis.
Corticosteroids, cyclophosphamide (a chemotherapy drug), and plasma exchange are used to try to prevent permanent lung and kidney damage.
An important function of the immune system is fighting off infections. To do this, the immune system recognizes microorganisms as foreign to the person and produces proteins (antibodies) that join with the microorganisms so they can be removed from the body. In autoimmune disorders, the body mistakenly reacts against a person's own tissues as if they were foreign. In autoimmune disorders that involve the lungs, the immune system attacks and damages lung tissue. Autoimmune disorders that affect the lungs also often affect other organs, particularly the kidneys.
A syndrome is a group of symptoms and other abnormalities that occur together but may be caused by several different disorders (or even by other syndromes). Goodpasture syndrome is an autoimmune disorder that typically causes kidney damage and diffuse alveolar hemorrhage (the pulmonary-renal syndrome). Occasionally Goodpasture syndrome affects only the kidneys or only the lungs.
Goodpasture syndrome usually affects young men. Some people appear to be genetically susceptible. In these people, substances in the environment, such as tobacco smoke and, less commonly, some solvents, or a viral respiratory infection can cause them to produce antibodies that react and try to destroy certain parts of their own bodies. These antibodies usually damage the tiny air sacs (alveoli) and the capillaries of the lungs and the filtering apparatus of the kidneys. The antibodies trigger inflammation that interferes with lung and kidney function.
The first indication of Goodpasture syndrome in most people is a type of kidney problem called rapidly progressive glomerulonephritis, and lung problems are apparent only about half of the time.
Common symptoms of Goodpasture syndrome include
Symptoms can quickly become severe. Occasionally symptoms are severe enough to cause the lungs to fail and people to have severe difficulty breathing, gasping, and bluish discoloration of the skin (cyanosis). When the lungs fail, the tissues of the body do not receive enough oxygen, and the person may die.
Large amounts of blood can be lost. At the same time, the kidneys can rapidly fail.
Symptoms related to bleeding in the lungs may occur weeks or even years before symptoms related to kidney damage.
In many people who are having symptoms related to a lung disorder, a chest x-ray is done. Chest x-rays show abnormal white patches due to lung bleeding in both lungs. If the presence of lung bleeding is not obvious based on symptoms and x-ray results (for example, if the person has not coughed up blood), doctors may need to insert a flexible viewing tube into the lungs (bronchoscopy) and wash out the lungs with fluid (bronchoalveolar lavage).
Urine testing reveals blood and protein. Blood testing often shows anemia.
Laboratory tests reveal the characteristic antibodies in the blood.
Doctors usually remove a small piece of kidney tissue (kidney biopsy) for analysis. The specimen shows microscopic deposits of antibodies in a specific pattern.
Goodpasture syndrome may very rapidly lead to severe loss of lung function, a complete loss of kidney function, and death.
The person undergoes plasma exchange—a procedure in which the unwanted antibodies are removed from the blood.
High doses of corticosteroids and cyclophosphamide are given intravenously to suppress the activity of the immune system. Rituximab, another drug that suppresses the immune system, is sometimes used in people who have severe side effects as a result of cyclophosphamide use.
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, and people may need dialysis or kidney transplantation.
Many people may need supportive care until the disease runs its course. For example, people may be given oxygen or may need help to breathe (using a mechanical ventilator) for a time. Blood or blood product transfusions may also be needed.
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