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Goodpasture Syndrome

(Goodpasture Disease; Goodpasture's Syndrome; Anti-GBM Antibody Disease)

By

Joyce Lee

, MD, MAS, University of Colorado School of Medicine

Last full review/revision Sep 2021| Content last modified Sep 2021
Click here for the Professional Version
  • People usually have difficulty breathing and may cough up blood.

  • Laboratory tests on samples of blood and urine and a chest imaging 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 Overview of the Immune System The immune system is designed to defend the body against foreign or dangerous invaders. Such invaders include Microorganisms (commonly called germs, such as bacteria, viruses, and fungi) Parasites... read more is fighting off infections. To do this, the immune system recognizes microorganisms as foreign to the person and produces proteins (antibodies Antibodies One of the body's lines of defense (immune system) involves white blood cells (leukocytes) that travel through the bloodstream and into tissues, searching for and attacking microorganisms and... read more Antibodies ) that join with the microorganisms so they can be removed from the body. In autoimmune disorders Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers autoimmune disorders is not known. Symptoms vary depending on... read more , 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.

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 Antibodies One of the body's lines of defense (immune system) involves white blood cells (leukocytes) that travel through the bloodstream and into tissues, searching for and attacking microorganisms and... read more 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.

Symptoms of Goodpasture Syndrome

Symptoms of Goodpasture syndrome can include

  • Blood in the urine

  • Cough

  • Coughing up blood (hemoptysis)

  • Fatigue

  • Fever

  • Shortness of breath

  • Unplanned weight loss

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 Cyanosis Cyanosis is a bluish discoloration of the skin resulting from an inadequate amount of oxygen in the blood. Cyanosis occurs when oxygen-depleted (deoxygenated) blood, which is bluish rather than... read more ). 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.

Diagnosis of Goodpasture Syndrome

  • Chest imaging

  • Sometimes insertion of a flexible viewing tube into the lungs (bronchoscopy) with fluid wash out (bronchoalveolar lavage)

  • Blood and urine tests

  • Biopsy of kidney tissue

In many people who are having symptoms related to a lung disorder, chest imaging is done. Chest imaging shows abnormal white patches due to lung bleeding in both lungs. If the presence of lung bleeding is not obvious based on symptoms and chest imaging results (for example, if the person has not coughed up blood), doctors may need to insert a flexible viewing tube into the lungs (bronchoscopy Bronchoscopy Bronchoscopy is a direct visual examination of the voice box (larynx) and airways through a viewing tube (a bronchoscope). A bronchoscope has a camera at the end that allows a doctor to look... read more Bronchoscopy ) and wash out the lungs with fluid (bronchoalveolar lavage Procedures Done With Bronchoscopy Bronchoscopy is a direct visual examination of the voice box (larynx) and airways through a viewing tube (a bronchoscope). A bronchoscope has a camera at the end that allows a doctor to look... read more Procedures Done With Bronchoscopy ).

Urine testing reveals blood and protein. Blood testing often shows anemia.

Laboratory tests reveal the characteristic antibodies in the blood.

Treatment of Goodpasture Syndrome

  • Procedure to remove unwanted antibodies from the blood (plasma exchange)

  • Corticosteroids (such as methylprednisolone) and cyclophosphamide given by vein (intravenously)

  • Sometimes dialysis or kidney transplantation

Goodpasture syndrome may very rapidly lead to severe loss of lung function, a complete loss of kidney function, and death.

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.

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 Mechanical Ventilation Mechanical ventilation is use of a machine to aid the movement of air into and out of the lungs. Some people with respiratory failure need a mechanical ventilator (a machine that helps air get... read more ) for a time. Blood or blood product transfusions may also be needed.

More Information

The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
Click here for the Professional Version
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