Sjögren's (SHOW-grins) syndrome is an autoimmune disease--that is, a disease in which the immune system turns against the body's own cells. In Sjögren's syndrome, the immune system targets moisture-producing glands and causes dryness in the mouth and eyes. Other parts of the body can be affected as well, resulting in a wide range of possible symptoms.
Normally, the immune system works to protect us from disease by destroying harmful invading organisms like viruses and bacteria. In the case of Sjögren's syndrome, disease-fighting cells attack the glands that produce tears and saliva (the lacrimal and salivary glands). Damage to these glands keeps them from working properly and causes dry eyes and dry mouth. In technical terms, dry eyes are called keratoconjunctivitis sicca, or KCS, and dry mouth is called xerostomia. Your doctor may use these terms when talking to you about Sjögren's syndrome.
The disease can affect other glands too, such as those in the stomach, pancreas, and intestines, and can cause dryness in other places that need moisture, such as the nose, throat, airways, and skin.
You might hear Sjögren's syndrome called a rheumatic disease. A rheumatic disease causes inflammation in joints, muscles, skin, or other body tissue, and Sjögren's can do that. The many forms of arthritis, which often involve inflammation in the joints, among other problems, are examples of rheumatic diseases. Sjögren's is also considered a disorder of connective tissue, which is the framework of the body that supports organs and tissues (joints, muscles, and skin).
Researchers think Sjögren's syndrome is caused by a combination of genetic and environmental factors. Several different genes appear to be involved, but scientists are not certain exactly which ones are linked to the disease since different genes seem to play a role in different people. For example, there is one gene that predisposes Caucasians to the disease. Other genes are linked to Sjögren's in people of Japanese, Chinese, and African American descent. Simply having one of these genes will not cause a person to develop the disease, however. Some sort of trigger must activate the immune system.
Scientists think that the trigger may be a viral or bacterial infection. It might work like this: A person who has a Sjögren's-associated gene gets a viral infection. The virus stimulates the immune system to act, but the gene alters the attack, sending fighter cells (lymphocytes) to the eye and mouth glands. Once there, the lymphocytes attack healthy cells, causing the inflammation that damages the glands and keeps them from working properly. These fighter cells are supposed to die after their attack in a natural process called apoptosis, but in people with Sjögren's syndrome, they continue to attack, causing further damage. Scientists think that resistance to apoptosis may be genetic.
The possibility that the endocrine and nervous systems play a role is also under investigation.
2006-12-22 18:15:56
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answer #1
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answered by sk8er V 2
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SJS is an immune-complex–mediated hypersensitivity disorder that may be caused by many drugs, viral infections, and malignancies. Cocaine recently has been added to the list of drugs capable of producing the syndrome. In up to half of cases, no specific etiology has been identified.
Stevens-Johnson Syndrome is a potentially deadly skin disease that usually results from a drug reaction. Another form of the disease is called Toxic Epidermal Necrolysis, and again this usually results from a drug-related reaction. Both forms of the disease can be deadly as well as very painful and distressing. In most cases, these disorders are caused by a reaction to a drug, and one drug that has come under fire lately is the cox-2 inhibitor Bextra, which is already linked to these disorders.
There are other drugs that have been linked to Stevens-Johnson Syndrome, and these include some other NSAIDS (non-steroid anti-inflammatory drugs), Allopurinol, Phenytoin, Carbamazepine, barbiturates, anticonvulsants, and sulfa antibiotics. The condition can sometimes – although not very often – be attributed to a bacterial infection, and in some cases there is no known cause for the onset of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis. However, the most common cause is through drug related reaction.
2006-12-22 18:36:53
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answer #2
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answered by jamaica 5
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SJS--Steven Johnson syndrome is characterized by a flu-like prodromal period of fever, sore throat, and headache followed by the sudden development of circular mucocutaneous lesions (target lesions) that can cover the majority of the skin. These lesions begin as macules and can develop into papules, vesicles, blisters, or urticarial plaques. Cases involving greater than 30% of body surface area are termed Toxic Epidermal Necrolysis Syndrome (TENS) or Lyell's Syndrome.
SJS can be caused by infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), allergic reactions to drugs, (valdecoxib, penicillins, barbiturate, sulfas, phenytoin, lamotrigine, nevirapine,Ibuprofen, ethosuximide etc..
hope this helps..
2006-12-23 01:33:53
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answer #3
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answered by For peace 3
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Stevens-Johnson syndrome (SJS) is a severe and life-threatening condition. It is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. SJS has been classified as a severe expression of erythema multiforme (EM), and is sometimes referred to as erythema multiforme major. This terminology is not consistent; medical texts often distinguish between causes of SJS, referring to drug induced syndrome as SJS and applying the term erythema multiforme to viral etiology.
Causes:
SJS can be caused by infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), allergic reactions to drugs, (valdecoxib, penicillins, barbiturate, sulfas, phenytoin, lamotrigine, nevirapine,Ibuprofen, ethosuximide), malignancy (carcinomas and lymphomas), or idiopathic factors (up to 50% of the time). SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng. SJS may also be caused by cocaine usage.
2006-12-22 17:47:52
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answer #4
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answered by Som™ 6
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Steven Johnson's Syndrome is a serious systemic disorder in which there are vesicobullous lesions involving the skin and mucous membranes. It can result as an immune response to an antigen or as a drug reaction. Most often it is considered as an allergic reaction. It is a self-limiting condition which responds to immediate management or may result in fluid loss, sepsis and death.
2006-12-22 17:49:11
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answer #5
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answered by Chez 4
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Sjögren's (SHOW-grins) syndrome is an autoimmune disease--that is, a disease in which the immune system turns against the body's own cells. In Sjögren's syndrome, the immune system targets moisture-producing glands and causes dryness in the mouth and eyes. Other parts of the body can be affected as well, resulting in a wide range of possible symptoms.
Normally, the immune system works to protect us from disease by destroying harmful invading organisms like viruses and bacteria. In the case of Sjögren's syndrome, disease-fighting cells attack the glands that produce tears and saliva (the lacrimal and salivary glands). Damage to these glands keeps them from working properly and causes dry eyes and dry mouth. In technical terms, dry eyes are called keratoconjunctivitis sicca, or KCS, and dry mouth is called xerostomia. Your doctor may use these terms when talking to you about Sjögren's syndrome.
The disease can affect other glands too, such as those in the stomach, pancreas, and intestines, and can cause dryness in other places that need moisture, such as the nose, throat, airways, and skin.
You might hear Sjögren's syndrome called a rheumatic disease. A rheumatic disease causes inflammation in joints, muscles, skin, or other body tissue, and Sjögren's can do that. The many forms of arthritis, which often involve inflammation in the joints, among other problems, are examples of rheumatic diseases. Sjögren's is also considered a disorder of connective tissue, which is the framework of the body that supports organs and tissues (joints, muscles, and skin).
Researchers think Sjögren's syndrome is caused by a combination of genetic and environmental factors. Several different genes appear to be involved, but scientists are not certain exactly which ones are linked to the disease since different genes seem to play a role in different people. For example, there is one gene that predisposes Caucasians to the disease. Other genes are linked to Sjögren's in people of Japanese, Chinese, and African American descent. Simply having one of these genes will not cause a person to develop the disease, however. Some sort of trigger must activate the immune system.
Scientists think that the trigger may be a viral or bacterial infection. It might work like this: A person who has a Sjögren's-associated gene gets a viral infection. The virus stimulates the immune system to act, but the gene alters the attack, sending fighter cells (lymphocytes) to the eye and mouth glands. Once there, the lymphocytes attack healthy cells, causing the inflammation that damages the glands and keeps them from working properly. These fighter cells are supposed to die after their attack in a natural process called apoptosis, but in people with Sjögren's syndrome, they continue to attack, causing further damage. Scientists think that resistance to apoptosis may be genetic.
The possibility that the endocrine and nervous systems play a role is also under investigation.
2006-12-22 17:47:19
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answer #6
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answered by debwils_4kids 4
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Steven Johnsons syndrome, everything you need to know: http://en.wikipedia.org/wiki/Stevens_Johnson_syndrome
2006-12-22 17:45:13
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answer #7
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answered by unknowndoe 2
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