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 ) 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 an autoimmune disorder is not known. Symptoms vary depending... 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.
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 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 an autoimmune disorder is not known. Symptoms vary depending... read more that typically causes kidney damage and diffuse alveolar hemorrhage Diffuse Alveolar Hemorrhage Diffuse alveolar hemorrhage is a syndrome of recurrent or persistent bleeding into the lungs, caused most often by an autoimmune disorder. Common symptoms are difficulty breathing and coughing... read more (a pulmonary-renal syndrome Pulmonary-Renal Syndrome Pulmonary-renal syndrome combines both diffuse alveolar hemorrhage (recurrent or persistent bleeding into the lungs) and glomerulonephritis (damage to the microscopic blood vessels in the kidneys... read more ). 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 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 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 Glomerulonephritis Glomerulonephritis is a disorder of glomeruli (clusters of microscopic blood vessels in the kidneys with small pores through which blood is filtered). It is characterized by body tissue swelling... read more , and lung problems are apparent only about half of the time.
Symptoms of Goodpasture Syndrome
Symptoms of Goodpasture syndrome can include
Blood in the urine
Coughing up blood (hemoptysis)
Shortness of breath
Unplanned weight loss
Fatigue and paleness (pallor) may be symptoms of anemia Overview of Anemia Anemia is a condition in which the number of red blood cells is low. Red blood cells contain hemoglobin, a protein that enables them to carry oxygen from the lungs and deliver it to all parts... read more that results from blood loss. Some people may have swelling Swelling Swelling is due to excess fluid in the tissues. The fluid is predominantly water. Swelling may be widespread or confined to a single limb or part of a limb. Swelling is often in the feet and... read more (edema) of the legs as a result of kidney damage.
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
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 ) 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 ).
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 Kidney biopsy Site-specific biopsies and cell sampling are also used in the evaluation of people with suspected kidney and urinary tract disorders. (See also Overview of the Urinary Tract.) A kidney biopsy... read more ) for analysis. The specimen shows microscopic deposits of antibodies in a specific pattern.
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.
The person undergoes plasma exchange Plasma exchange In apheresis, blood is removed from a person and then returned after substances are removed from it. Apheresis can be used to Obtain healthy blood components from a donor to transfuse to a person... read more —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 Dialysis Dialysis is an artificial process for removing waste products and excess fluids from the body, a process that is needed when the kidneys are not functioning properly. There are a number of reasons... read more or kidney transplantation Kidney Transplantation Kidney transplantation is the removal of a healthy kidney from a living or recently deceased person and then its transfer into a person with end-stage kidney failure. (See also Overview of Transplantation... read more .
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.
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.
National Kidney Foundation: Goodpasture's Syndrome: General information on Goodpasture syndrome, including symptoms and complications, diagnosis, treatment, and additional self-care information
Drugs Mentioned In This Article
|Generic Name||Select Brand Names|
|Cyclophosphamide, Cytoxan, Neosar|
|A-Methapred, Depmedalone-40, Depmedalone-80 , Depo-Medrol, Medrol, Medrol Dosepak, Solu-Medrol|
|RIABNI, Rituxan, RUXIENCE, truxima|