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Goodpasture’s syndrome (also known as Goodpasture’s disease and anti-glomerular basement membrane disease or anti-GBM disease) was first described by Ernest Goodpasture in 1919. It is an rare condition characterised by rapid destruction of the kidneys and haemorrhaging of the lungs. Although many diseases can present with these symptoms, the name Goodpasture’s syndrome is usually reserved for the autoimmune disease produced when the patient’s immune system attacks cells presenting the Goodpasture antigen, which are found in the kidney and lung, causing damage to these organs. more...
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Signs and symptoms
Most patients present with both lung and kidney disease, however, some patients present with one of these diseases alone. The first lung symptoms usually develop days to months before kidney damage is evident.
Lung damage may cause nothing more serious than a dry cough and minor breathlessness and such mild symptoms may last for many years before more severe ones develop. At its most serious, however, lung damage may cause severe impairment of oxygenation so that intensive care is required. Deterioration between the two extremes may occur very rapidly, often at the same time as rapid deterioration in the kidney. The patient often does not seek medical attention until he or she begins coughing up blood. The patient may be anaemic due to loss of blood through lung haemorrhaging over a long period. In Goodpasture’s syndrome, unlike many other conditions that cause similar symptoms, lung haemorrhaging most often occurs in smokers and those with damage from lung infection or exposure to fumes.
The kidney disease mostly affects the glomeruli causing a form of nephritis. It is usually not detected until a rapid advance of the disease occurs so that kidney function can be completely lost in a matter of days. Blood leaks into the urine causing haematuria, the volume urinated decreases and urea and other products usually excreted by the kidney are retained and build up in the blood. This is acute renal failure. Renal failure does not cause symptoms until more than 80% of kidney function has been lost. Symptoms include loss of appetite and sickness at first and then, when the damage is more advanced, breathlessness, high blood pressure and oedema (swelling caused by fluid retention).
Because of the vagueness of early symptoms and rapid progression of the disease, diagnosis is often not reached until very late in the course of the disease. Kidney biopsy is often the fastest way to secure the diagnosis and gain information about the extent of the disease and likely effect of treatment. Tests for anti-GBM antibodies may also be useful, combined with tests for antibodies to neutrophil cytoplasmic antigens, which are also directed against the patient’s own proteins.
As with many autoimmune conditions, the precise cause of Goodpasture’s Syndrome is not yet known. It is believed to be a type II hypersensitivity reaction to Goodpasture’s antigens on the cells of the glomeruli of the kidneys and the pulmonary alveoli, whereby the immune system wrongly recognises these cells as foreign and attacks and destroys them, as it would an invading pathogen.
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From Gale Encyclopedia of Medicine,
by John Thomas Lohr
Autoimmune disorders are conditions in which a person's immune system attacks the body's own cells, causing tissue destruction.
Autoimmunity is accepted as the cause of a wide range of disorders, and it is suspected to be responsible for many more. Autoimmune diseases are classified as either general, in which the autoimmune reaction takes place simultaneously in a number of tissues, or organ specific, in which the autoimmune reaction targets a single organ.
Autoimmune disorders include the following:
- Systemic lupus erythematosus. A general autoimmune disease in which antibodies attack a number of different tissues. The disease recurs periodically and is seen mainly in young and middle-aged women.
- Rheumatoid arthritis. Occurs when the immune system attacks and destroys the tissues that line bone joints and cartilage. The disease occurs throughout the body, although some joints may be more affected than others.
- Goodpasture's syndrome. Occurs when antibodies are deposited in the membranes of both the lung and kidneys, causing both inflammation of kidney glomerulus (glomerulonephritis) and lung bleeding. It is typically a disease of young males.
- Grave's disease. Caused by an antibody that binds to specific cells in the thyroid gland, causing them to make excessive amounts of thyroid hormone.
- Hashimoto's thyroiditis. Caused by an antibody that binds to cells in the thyroid gland. Unlike in Grave's disease, however, this antibody's action results in less thyroid hormone being made.
- Pemphigus vulgaris. A group of autoimmune disorders that affect the skin.
- Myasthenia gravis. A condition in which the immune system attacks a receptor on the surface of muscle cells, preventing the muscle from receiving nerve impulses and resulting in severe muscle weakness.
- Scleroderma. Also called CREST syndrome or progressive systemic sclerosis, scleroderma affects the connective tissue.
- Autoimmune hemolytic anemia. Occurs when the body produces antibodies that coat red blood cells.
- Autoimmune thrombocytopenic purpura. Disorder in which the immune system targets and destroys blood platelets.
- Polymyositis and Dermatomyositis. Immune disorders that affect the neuromuscular system.
- Pernicious anemia. Disorder in which the immune system attacks the lining of the stomach in such a way that the body cannot metabolize vitamin B12.
- Sjögren's syndrome. Occurs when the exocrine glands are attacked by the immune system, resulting in excessive dryness.
- Ankylosing spondylitis. Immune-system induced degeneration of the joints and soft tissue of the spine.
- Vasculitis. A group of autoimmune disorders in which the immune system attacks and destroys blood vessels.
- Type I diabetes mellitus. May be caused by an antibody that attacks and destroys the islet cells of the pancreas, which produce insulin.
Causes & symptoms
The symptoms of the above disorders include:
- Systemic lupus erythematosus. Symptoms include fever, chills, fatigue, weight loss, skin rashes (particularly the classic "butterfly" rash on the face), vasculitis, polyarthralgia, patchy hair loss, sores in the mouth or nose, lymph-node enlargement, gastric problems, and, in women, irregular periods. About half of those who suffer from lupus develop cardiopulmonary problems, and some may also develop urinary problems. Lupus can also effect the central nervous system, causing seizures, depression, and psychosis.
- Rheumatoid arthritis. Initially may be characterized by a low-grade fever, loss of appetite, weight loss, and a generalized pain in the joints. The joint pain then becomes more specific, usually beginning in the fingers, then spreading to other areas, such as the wrists, elbows, knees, and ankles. As the disease progresses, joint function diminishes sharply and deformities occur, particularly the characteristic "swan's neck" curling of the fingers.
- Goodpasture's syndrome. Symptoms are similar to that of iron deficiency anemia, including fatigue and pallor. Symptoms involving the lungs may range from a cough that produces bloody sputum to outright hemorrhaging. Symptoms involving the urinary system include blood in the urine and/or swelling.
- Grave's disease. This disease is characterized by an enlarged thyroid gland, weight loss without loss of appetite, sweating, heart palpitations, nervousness, and an inability to tolerate heat.
- Hashimoto's thyroiditis. This disorder generally displays no symptoms.
- Pemphigus vulgaris. This disease is characterized by blisters and deep lesions on the skin.
- Myasthenia gravis. Characterized by fatigue and muscle weakness that at first may be confined to certain muscle groups, but then may progress to the point of paralysis. Myasthenia gravis patients often have expressionless faces as well as difficulty chewing and swallowing. If the disease progresses to the respiratory system, artificial respiration may be required.
- Scleroderma. Disorder is usually preceded by Raynaud's phenomenon. Symptoms that follow include pain, swelling, and stiffness of the joints, and the skin takes on a tight, shiny appearance. The digestive system also becomes involved, resulting in weight loss, appetite loss, diarrhea, constipation, and distention of the abdomen. As the disease progresses, the heart, lungs, and kidneys become involved, and malignant hypertension causes death in approximately 30% of cases.
- Autoimmune hemolytic anemia. May be acute or chronic. Symptoms include fatigue and abdominal tenderness due to an enlarged spleen.
- Autoimmune thrombocytopenic purpura. Characterized by pinhead-size red dots on the skin, unexplained bruises, bleeding from the nose and gums, and blood in the stool.
- Polymyositis and Dermatomyositis. In polymyositis, symptoms include muscle weakness, particularly in the shoulders or pelvis, that prevents the patient from performing everyday activities. In dermatomyositis, the same muscle weakness is accompanied by a rash that appears on the upper body, arms, and fingertips. A rash may also appear on the eyelids, and the area around the eyes may become swollen.
- Pernicious anemia. Signs of pernicious anemia include weakness, sore tongue, bleeding gums, and tingling in the extremities. Because the disease causes a decrease in stomach acid, nausea, vomiting, loss of appetite, weight loss, diarrhea, and constipation are possible. Also, because Vitamin B12 is essential for the nervous system function, the deficiency of it brought on by the disease can result in a host of neurological problems, including weakness, lack of coordination, blurred vision, loss of fine motor skills, loss of the sense of taste, ringing in the ears, and loss of bladder control.
- Sjögren's syndrome. Characterized by excessive dryness of the mouth and eyes.
- Ankylosing spondylitis. Generally begins with lower back pain that progresses up the spine. The pain may eventually become crippling.
- Vasculitis. Symptoms depend upon the group of veins affected and can range greatly.
- Type I diabetes mellitus. Characterized by fatigue and an abnormally high level of glucose in the blood (hyperglycemia).
To further understand autoimmune disorders, it is helpful to understand the workings of the immune system. The purpose of the immune system is to defend the body against attack by infectious microbes (germs) and foreign objects. When the immune system attacks an invader, it is very specific--a particular immune system cell will only recognize and target one type of invader. To function properly, the immune system must not only develop this specialized knowledge of individual invaders, but it must also learn how to recognize and not destroy cells that belong to the body itself. Every cell carries protein markers on its surface that identifies it in one of two ways: what kind of cell it is (e.g. nerve cell, muscle cell, blood cell, etc.) and to whom that cell belongs. These markers are called major histocompatability complexes (MHCs). When functioning properly, cells of the immune system will not attack any other cell with markers identifying it as belonging to the body. Conversely, if the immune system cells do not recognize the cell as "self," they attach themselves to it and put out a signal that the body has been invaded, which in turn stimulates the production of substances such as antibodies that engulf and destroy the foreign particles. In case of autoimmune disorders, the immune system cannot distinguish between "self" cells and invader cells. As a result, the same destructive operation is carried out on the body's own cells that would normally be carried out on bacteria, viruses, and other such harmful entities.
The reasons why the immune systems become dysfunctional in this way is not well understood. However, most researchers agree that a combination of genetic, environmental, and hormonal factors play into autoimmunity. Researchers also speculate that certain mechanisms may trigger autoimmunity. First, a substance that is normally restricted to one part of the body, and therefore not usually exposed to the immune system, is released into other areas where it is attacked. Second, the immune system may mistake a component of the body for a similar foreign component. Third, cells of the body may be altered in some way, either by drugs, infection, or some other environmental factor, so that they are no longer recognizable as "self" to the immune system. Fourth, the immune system itself may be damaged, such as by a genetic mutation, and therefore cannot function properly.
A number of tests that can help diagnose autoimmune diseases; however the principle tool used by doctors is antibody testing. Such tests involve measuring the level of antibodies found in the blood and determining if they react with specific antigens that would give rise to an autoimmune reaction. An elevated amount of antibodies indicates that a humoral immune reaction is occurring. Elevated antibody levels are also seen in common infections. These must be ruled out as the cause for the increased antibody levels. The antibodies can also be typed by class. There are five classes of antibodies and they can be separated in the laboratory. The class IgG is usually associated with autoimmune diseases. Unfortunately, IgG class antibodies are also the main class of antibody seen in normal immune responses. The most useful antibody tests involve introducing the patient's antibodies to samples of his or her own tissue--if antibodies bind to the tissue it is diagnostic for an autoimmune disorder. Antibodies from a person without an autoimmune disorder would not reacting to "self" tissue. The tissues used most frequently in this type of testing are thyroid, stomach, liver, and kidney.
Treatment of autoimmune diseases is specific to the disease, and usually concentrates on alleviating symptoms rather than correcting the underlying cause. For example, if a gland involved in an autoimmune reaction is not producing a hormone such as insulin, administration of that hormone is required. Administration of a hormone, however, will restore the function of the gland damaged by the autoimmune disease. The other aspect of treatment is controlling the inflammatory and proliferative nature of the immune response. This is generally accomplished with two types of drugs. Steroid compounds are used to control inflammation. There are many different steroids, each having side effects. The proliferative nature of the immune response is controlled with immunosuppressive drugs. These drugs work by inhibiting the replication of cells and, therefore, also suppress non-immune cells leading to side effects such as anemia.
Prognosis depends upon the pathology of each autoimmune disease.
- An antibody made by a person that reacts with their own tissues.
For Your Information
- Abbas , A.K., A.H. Lichtman, and J.S. Pober. Cellular and Molecular Immunology. Philadelphia: W.B. Saunders Company, 1997.
- Berkow, Robert, ed. Merck Manual of Medical Information. Whitehouse Station, NJ: Merck Research Laboratories, 1997.
- Roit, I.M.Roitt's Essential Immunolgy. Oxford: Blackwell Science Ltd., 1997.
Gale Encyclopedia of Medicine. Gale Research, 1999.
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