Goodpasture syndrome is an uncommon autoimmune disorder in which bleeding into the lungs and progressive kidney failure occur.
People usually have difficulty breathing and may cough up blood.
Laboratory tests on samples of blood and urine and 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 is fighting off infections. To do this, the immune system recognizes microorganisms as foreign to the person and produces proteins (antibodies) that bind to 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 (a pulmonary-renal syndrome). Occasionally Goodpasture syndrome affects only the kidneys or only the lungs.
Goodpasture syndrome affects people who 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 affects the lungs and kidneys.
Dalili za Ugonjwa wa 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
Fatigue and paleness (pallor) may be symptoms of anemia that results from blood loss. Some people may have swelling (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 or pale or grayish 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.
Utambuzi wa Ugonjwa wa 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. 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) 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.
Matibabu ya Ugonjwa wa 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—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 medication 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.
Taarifa Zaidi
The following English-language resource 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
