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  • Featured books on Sjogren's syndrome from Barnes & Noble.com:
  • What is Sjogren’s syndrome?
  • What signs, symptoms, and other conditions are associated with Sjogren's syndrome?
  • What medications can cause dryness?
  • Do the signs and symptoms of Sjogren's syndrome get better?
  • What causes Sjogren's syndrome?
  • How is Sjogren's syndrome classified?
  • At what age do people usually get Sjogren's syndrome?
  • Are women more likely to get Sjogren's syndrome than men?
  • How many people have Sjogren's syndrome?
  • How is Sjogren's syndrome diagnosed?
  • How are problems of the brain, spine, and nerves diagnosed that are associated with Sjogren's syndrome?
  • What type of doctor can diagnose Sjogren's syndrome?
  • How is Sjogren's syndrome treated?
  • What is the prognosis for people with Sjogren's syndrome?
  • What else is Sjogren's syndrome known as?
  • What is the origin of the term, Sjogren's syndrome?

    FEATURED BOOKS ON SJOGREN'S SYNDROME FROM BARNES & NOBLE.COM:

    Sjogren's Syndrome Survival GuideThe New Sjogren's Syndrome Handbook

    WHAT IS SJOGREN’S SYNDROME?

    Sjogren's (pronounced SHOW-grins) syndrome is a disease in which the eyes and mouth become excessively dry. Sjogren's syndrome is a chronic (long-term) disease that progresses slowly.

    WHAT SIGNS, SYMPTOMS, AND OTHER CONDITIONS ARE ASSOCIATED WITH SJOGREN'S SYNDROME?

    The signs and symptoms, of Sjogren's syndrome vary from person to person. These signs and symptoms are discussed below:

    • DRY EYES: The most characteristic feature of Sjogren's syndrome is dry eyes (technically known as keratoconjunctivitis sicca or KCS). The dry eyes can appear red and can cause a feeling of burning and itchiness. The dry eyes can also cause a gritty feeling, as if something foreign is under the eyelid, such as sand. The eyelids may feel stuck together and the eyes may not be able to withstand strong light without it causing discomfort. Blurry vision can result as well. Ulcers (open sores) of the cornea can also occur. In the worst case scenario, Sjogren's syndrome can cause blindness.

    • DRY MOUTH: A dry mouth (technically known as xerostomia) is the other major feature associated with Sjogren's syndrome. A dry mouth can feel like a mouthful of cotton. The lack of saliva in the mouth can lead to difficulty swallowing, chewing, and eating because the food sticks to the tissues. A lack of saliva can also cause tooth decay because saliva contains substances that normally helps rid the mouth of bacteria.

      Recurring mouth infections, such as bacteria infections, virus infections, or fungus infections can occur due to the lack of saliva. A fungus is a simple type of parasite. A parasite is an organism that lives in or on another organism to obtain nourishment. One of the most commonly seen fungal infections in Sjogren's syndrome is known as Candidiasis. More specifically, Candidiasis is a type of yeast infection. Candidiasis appears in the mouth as white patches that can be scraped off. Candidiasis can also present as red, burning areas in the mouth. Mouth infections are treated with the appropriate antibacterial, anti-viral, or anti-fungal medications.

      Dry mouth can cause a burning sensation in the mouth and throat. It can also cause the voice to be weak or hoarse, making it difficult to speak. A lack of saliva can also lead to a loss of taste and smell.

    • DRY SKIN: About half of people with Sjogren's syndrome have dry skin. Some people only experience itching, but the itching can be severe. Scratching itchy skin can cause the skin to break open, leading to infection. Worse, some people develop cracks and splits in their skin that can become infected easily. Infected skin can appear dark, but it will return to normal when the infection goes away.

      Sjogren's syndrome can also cause a dryness in other body areas that need moisture such as the nose, airways, throat, digestive tract, sinuses, and vagina (female reproductive part). Sinuses are openings in the bone that often contain fluid. Dryness of the vagina can cause sex to be painful.

      Mucous membranes become dry in Sjogren's syndrome. A mucous membrane is one of four major types of thin sheets of tissue that line or cover various parts of the body, such as the mouth and passages for breathing. Mucous membranes moisten the structures that they cover. Nosebleeds can also occur in Sjogren's syndrome.

    • FATIGUE & PAIN: Sjogren's syndrome is also known to cause fatigue that can sometimes lead to exhaustion. Joint pain can also occur due to inflammation. A joint is a place where two bones contact each other. Some people with Sjogren's syndrome feel as if they hurt all over their body, as if they have been beat up, and are continuously exhausted. A low-grade fever can also occur in people with Sjogren's syndrome.

    • TISSUE DAMAGE & INFLAMMATION: Sjogren's syndrome can cause tissue damage and inflammation to major organs in the body, such as the stomach, thyroid, intestines, pancreas (a long organ in the back of the belly), and lungs. These, and other body areas are also highlighted below for convenience.

    • THYROID: The thyroid gland is a butterfly-shaped organ located in the front of the neck that plays an important role in metabolism. Metabolism is the chemical actions in cells that release energy from nutrients or use energy to create other substances. The intestine is a tube shaped structure that is part of the digestive tract. It stretches from an opening in the stomach to the anus (the area that poop comes out of) and occupies most of the lower parts of the belly. Many people with Sjogren's syndrome have thyroid disease. The thyroid can be overactive (known as Grave's disease) or underactive (known as Hashimoto's disease).

    • LUNGS: The lungs are two organs in the body that help people breathe. The lungs can be affected by a dry mouth. How? Normally, a person swallows food with the assistance of saliva. People with dry mouth may not have enough saliva to swallow food and wind up breathing it in. This can cause the food to get stuck in the lungs and can result in pneumonia. Pneumonia is inflammation of the lungs due to infection. Another possible cause of pneumonia is infection from bacteria that moves from the mouth to the lungs.

      Another cause of pneumonia is when bacteria gets into the lungs and coughing does not remove it. This can happen to people with Sjogren's syndrome because there may not be enough mucus produced in the lungs to remove the bacteria or the person may be too weak to cough. If pneumonia is not treated, it can cause a hole to develop in the lings.

      If the lungs or lining of the lungs are inflamed, shortness of breath can result. Lung problems can be due to pneumonia, but can also be due to bronchitis, tracheobronchitis, or laryngotracheobronchitis. Bronchitis is inflammation of the bronchial tubes, which are airways that connect that windpipe to the lungs. Tracheobronchitis is inflammation of the windpipe and bronchial tubes. Laryngotracheobronchitis is inflammation of the voice box, windpipe, and bronchial tubes.

    • DIGESTIVE SYSTEM: The digestive system can also become inflamed in Sjogren's syndrome, which can lead to other problems. For example, inflammation of the esophagus can cause difficulty swallowing. The esophagus is a tube-shaped structure that takes food down into the stomach after it is swallowed. Inflammation of the digestive system can also cause heartburn. Heartburn is a burning sensation in the center of the chest that may travel from the tip of the sternum to the heart. The sternum is the flat bone in the middle of the chest.

      Inflammation of the digestive system can cause pain and swelling in the belly. It can cause diarrhea (loose, watery feces or poop), loss of appetite, and weight loss.

    • KIDNEYS AND LIVER: Sjogren's syndrome rarely causes tissue damage and inflammation to the kidneys or liver. The kidneys are two organs located on each side of the spine, behind the stomach. The kidneys filter (remove) wastes from the blood and remove them from the body through urine (pee). Inflammation of tissues around the kidney's tissues, a condition known as interstitial nephritis, is the most common kidney problem in Sjogren's syndrome. Actual inflammation of the kidney filters, a condition known as glomerulonephritis, is less common.

      Some people with Sjogren's syndrome develop renal tubular acidosis, a condition in which not enough acids are released through the urine. This condition causes a decrease in potassium (a type of metallic element) in the blood. Potassium is very important for normal bodily functioning and a decrease of this element can affect the heart, nerves, and muscles.

      The liver is the largest organ in the body and is responsible for filtering (removing) harmful chemical substances, producing important chemicals for the body, and other important functions. Some people with Sjogren's syndrome develop hepatitis, which is inflammation of the liver. Some people with Sjogren's syndrome develop cirrhosis, which is a type of disease that hardens and destroys the liver.

      A type of cirrhosis that some people with Sjogren's syndrome have is known as primary biliary cirrhosis (also known as PBC). PBC is a type of cirrhosis caused by disease to the bile ducts. Bile ducts are tubes that allow bile to flow through it. Bile is a bitter, yellow-green substance released from the liver that carries away waste products. Some symptoms of PBC are fatigue and itchiness.

    • BLOOD VESSELS: In Sjogren's syndrome, permanent widening of certain blood vessels (known as capillaries and venules) can be seen on the skin. The capillaries act as an exchange system that connects the smallest veins (known as venules) with the smallest arteries (known as arterioles). Veins are blood vessels that carry blood to the heart. Arteries are blood vessels that carry blood away from the heart to the rest of the body.

      Sjogren's syndrome can also affect the blood vessels in the skin, which may cause rashes or bruising. Bleeding can occur into the tissues beneath the skin. This can cause bluish discoloration of the skin or tiny purplish red spots on the skin, especially on the face. For more information on how blood vessels can be affected in Sjogren's syndrome, see our discussion of Raynaud's phenomenon below (under the Autoimmune Disorders section).

    • PAROTIC GLAND: The parotid glands on both sides of the body can become enlarged and painful in Sjogren's syndrome. The parotid gland is the largest of three pairs of glands that produce saliva. The parotid gland is located behind the jaw, in front of the ears. A gland is an organ in the body made of special cells that form and release materials such as fluid.

    • NERVES: Sjogren's syndrome can affect the nerves outside the brain and spinal cord as well. For example, pain (that usually gets worse at night) and/or "pins and needle" sensations can be felt in the wrist that is associated with carpal tunnel syndrome. Carpal tunnel syndrome is numbness, tingling, and pain in the thumb, index, and middle fingers, which usually worsens at night. In Sjogren's syndrome, carpal tunnel syndrome is usually due to inflamed tissue in the forearm pressing against a nerve in the hand, causing symptoms. Muscle weakness is sometimes associated with carpal tunnel syndrome.

      Some people with Sjogren's syndrome develop peripheral neuropathy. Peripheral neuropathy is a general term for any disorder of nerves outside the brain or spinal cord. In Sjogren's syndrome, peripheral neuropathy generally happens in the legs and arms. It is usually caused by an attack of the immune system on the nerves in the legs and arms. Blood vessels that supply blood to nerves can become inflamed. This can stop the blood supply to the nerves, causing them to be damaged.

      Another ways in which Sjogren's syndrome can affect nerves outside the brain and spinal cord include cranial neuropathy, trigeminal neuralgia, and sensory neuropathy. Cranial neuropathy is damage to one of or more than one of the nerves that emerges from the brain or spinal cord. Cranial neuropathy can cause loss of feeling in the face, eyes, tongue, ears, or throat, loss of taste or smell, and pain in the face.

      Trigeminal neuralgia is a type of cranial neuropathy that sometimes happens in Sjogren's syndrome. Trigeminal neuralgia is a disorder of the trigeminal nerve in which severe, stabbing pain (like an electric shock) affects the lips, cheek, gums, or chin on one side of the face. There can also be some sensory loss in the face. The trigeminal nerve is one of the nerves that emerges from the brain, and splits into three parts.

      Sensory neuropathy is inflammation or degeneration of sensory nerves. Sensory neuropathy can cause sensory loss, pain, and difficulty walking. Numbness and tingling in the arms and legs can be caused by nerve damage in Sjogren's syndrome.

    • BRAIN & SPINE: Rarely, Sjogren's syndrome affects the brain and/or spinal cord. Brain damage can cause problems with thinking, attention, memory, or other mental abilities. Brain damage can also cause dysfunction in the autonomic nervous system. The autonomic nervous system is an extensive network in the body (with connections to the spinal cord) that is important for involuntary bodily functions that are necessary to maintain life, such as respiration (breathing) and heart rate. Some problems that can be caused by damage to the autonomic nervous syndrome in people with Sjogren's syndrome include sweating abnormalities and sensitivity to temperature changes.

      Brain damage due to Sjogren's syndrome can result in trigeminal neuralgia (see above), fatigue, burning pain, numbness and tingling, difficulty walking, poor balance, difficulty with position sense, and bladder and bowel problems (meaning that there would be a problems related to peeing and pooping). Optic neuritis can also occur in patients with Sjogren's syndrome that have brain damage. Optic neuritis is inflammation of the optic nerve. The optic nerve is a type of nerve located behind the eye that is crucial for vision.

    • AUTOIMMUNE DISORDERS: Sjogren's syndrome tends to occur in people with other autoimmune disorders. An autoimmune disorder is one in which a person's organs or tissues are mistakenly attacked by his/her immune system (defense system). Examples of autoimmune disorders that are associated with Sjogren's's syndrome include rheumatoid arthritis, systemic lupus erythematosus (SLE), scleroderma, dermatomyositis, and polymyositis. These, and other autoimmune disorders are described below in separate sections.

    • RHEUMATOID ARTHRITIS: Rheumatoid arthritis is a disorder in which the body's defense system attacks its own tissues, causing inflammation of bone joints. A joint is a place where two bones contact each other. Bones surrounding the joints can become deformed, such as the fingers and toes. Major organs, blood vessels, and muscles can also be damaged by rheumatoid arthritis.

    • SYSTEMIC LUPUS ERYTHEMATOSUS: SLE is a long-term disease in which the connective tissues throughout the body are inflamed because the body's defense system attacks these tissues as if they were foreign substances. The result is muscle pain, muscle weakness, joint pain, and skin rashes. In severe cases, SLE can even cause problems with the heart, lungs, kidneys, and nerves.

    • SCLERODERMA: Scleroderma (also known as systemic sclerosis) is a rare condition in which the body mistakenly attacks many organs and tissues of the body. Too much collagen builds up in people with scleroderma. Collagen is a protein found in the skin. As a result of the collagen build up, the skin becomes thick and tight. Damage can occur to areas throughout the body such as muscles, joints, the esophagus, kidneys, heart, lungs, intestines, and blood vessels.

    • DERMATOMYOSITIS & POLYMYOSITIS: Dermatomyositis is a rare, sometimes, deadly disease, in which the muscles and skin become inflamed, causing muscle weakness and rash. Polymyositis is a rare disease in which the muscles become weak and inflamed. There is no rash in polymyositis, but it causes pain and difficulty moving. In some cases, it can cause difficulty breathing and swallowing. Polymyositis is treated with corticosteroids and immunosuppressants. Please see the treatment section for a description of corticosteroids and immunosuppressants.

    • MENOPAUSE: Sjogren's syndrome is also associated with menopause. Menopause is the time in a woman's life when periods no longer occur. A period (also known as the menstrual cycle) is the shedding of the lining of the uterus that generally occurs once every 28 days in women who are not pregnant. The uterus is a hollow organ in a female's body where the egg is implanted and the baby develops.

    • RAYNAUD'S PHENOMENON: Sjogren's syndrome is associated with Raynaud's phenomenon. Raynaud's phenomenon, is a disorder of the blood vessels in which cold causes the small arteries that supply blood to the toes and fingers to suddenly constrict (narrow). When the blood vessels narrow, the fingers or toes become white. Afterwards, they become blue. When new blood rushed in, they become red. Raynaud's phenomenon can cause numbness, tingling, and pain in the fingers or toes.

      When the problems in the last paragraph have an unknown cause, the condition is called Raynaud's disease. When it has a known cause, it is called Raynaud's phenomenon. When Raynaud's phenomenon occurs in Sjogren's syndrome, it usually occurs before dry eyes and dry mouth. Medications that widen the blood vessels are used to treat Raynaud's phenomenon.

    • VASCULTIS: Sjogren's syndrome can cause vasculitis (inflammation of blood vessels). The inflamed blood vessels can become scarred, which then can cause them to become too narrow for blood to pass through. If the blood vessels are too narrow, blood cannot reach the organs, causing damage. People with Sjogren's syndrome that have vasculitis tend to have Raynaud's phenomenon or problems with the liver and lungs.

    • LYMPHOMA: About 5% of people with Sjogren's syndrome have lymphoma. Lymphoma is cancer of the lymphatic tissue. The lymphatic system is a system of vessels that drain lymph from all over the body and back into the blood. Lymph is a milky fluid that contains proteins, fats, and white blood cells (which help the body fight off diseases). When lymphoma develops in people with Sjogren's syndrome, it often involves the salivary glands, which causes them to enlarge.

    • MACROGLOBULINEMIA: Sjogren's syndrome is also associated with macroglobulinemia. Macroglobulinemia is the presence of increased levels of macroglobulins in the blood. Macroglobulins are large globulins in the blood. Globulins are types of simple proteins. Proteins are extremely complex, naturally occurring substances made of amino acids that are essential to the body's structure and function.

    WHAT MEDICATIONS CAN CAUSE DRYNESS?

    There are many medications that can possibly cause dryness. Some of these medications include antidepressants, antidiarrhea drugs, and blood pressure medications. Antihistamines, diuretics, decongestants, antipsychotics, and tranquilizers can also cause dryness. A description of these types of medications follows. Substances that block the effects of histamine are known as antihistamines and are used to treat allergies. Histamine is a natural substance in the body that is released during allergic reactions and leads to many allergic symptoms.

    A diuretic is a medication that helps to release fluids from the body in the form of urine (pee). Decongestants are medications that decrease congestion, such as a stuffy nose. Antipsychotic medications are used to treat psychosis. Psychosis is a mental disorder characterize by an impaired ability to understand reality. A tranquilizer is a drug that is designed to have a calming effect on someone.

    DO THE SIGNS AND SYMPTOMS OF SJOGREN'S SYNDROME GET BETTER?

    The signs and symptoms of Sjogren's syndrome sometimes stay the same, sometimes get worse, and sometimes go away. Some people go through periods of good health, only to have the signs and symptoms return later. Some people experience mild signs and symptoms and others experience severe signs and symptoms that greatly impair their quality of life. It all depends on the individual.

    WHAT CAUSES SJOGREN'S SYNDROME?

    In Sjogren's syndrome, the body's immune system (defense system) mistakenly destroys fluid-producing glands that moisten the body, as if they were foreign substances. A gland is an organ in the body made of special cells that form and release materials such as fluid. The mucous membranes also get attacked by the immune system. A mucous membrane is one of four major types of thin sheets of tissue that line or cover various parts of the body, such as the mouth and passages for breathing. Mucous membranes moisten the structures that they cover.

    The way the immune system normally works is that it calls on the services of white blood cells known as lymphocytes, or forms antibodies to fight off foreign substances such as bacteria and viruses. White blood cells are cells that help protect the body against diseases and fight infections. Antibodies are types of proteins that are formed by the body to destroy foreign proteins known as antigens. When an antibody meets an antigen, inflammation results. In Sjogren's syndrome, the immune system mistakenly continues to attack the body. This results in continuous inflammation for as long as antibodies are produced against the body.

    It is not known why the body attacks itself in Sjogren's syndrome, but researchers believe it may be due to a combination of factors, such as infection from a virus, hormonal factors, and genes inherited from one's parents. Genes are tiny structures that contain coded instructions for how proteins should be constructed and how certain bodily characteristics should develop. For example, genes control the natural color of people's eyes and hair, and whether they will be male or female. Genes are passed on from parents to their children.

    There are several different genes that appear to be play a role in Sjogren's syndrome. On type of gene appears to predispose white people to get the disease, whereas other genes appear to predispose black people, Chinese people, and Japanese people. Just having one of these genes, however, is not enough to cause one to develop the disease. It seems that there must be something in the person's environment that acts as a trigger for the immune system to attack the body. The trigger may be a type of bacteria or virus.

    There is decreased fluid production by the lacrimal glands, salivary glands, and other glands, which causes the abnormal dryness of the eyes, mouth, and mucous membranes. The lacrimal glands are small organs in the eye that produce tears. The salivary glands are glands that produce saliva. In Sjogren's syndrome, the lacrimal glands and salivary glands become inflamed, but can eventually decrease in size. The immune system attacks the Bartholins glands in the vagina, causing it to become dry. The Bartholins glands produce mucus (a thick, slippery fluid) that keeps the vagina moist.

    The decreased size of the lacrimal glands causes less tear production. Less tear production leads to dryness of the cornea and conjunctiva, in addition to tissue damage in the eye. The cornea is the clear covering at the front of the eyeball. The conjunctiva is a layer that covers and protects the inside of the eyelids and the front part of the sclera (the white part of the eyes). The decreased size of the salivary glands causes less production of saliva. Less saliva production results in damage to the teeth, loss of taste, and loss of smell.

    HOW IS SJOGREN'S SYNDROME CLASSIFIED?

    There are various ways that Sjogren's syndrome are classified. To begin with, because problems with the immune system occur in Sjogren's syndrome, it is often referred to as an immunological disorder. More specifically, Sjogren's syndrome is called an autoimmune disorder. An autoimmune disorder is one in which a person's organs or tissues are mistakenly attacked by his/her own immune system (defense system).

    If Sjogren's syndrome occurs in the presence of another autoimmune disorder, it is known as secondary Sjogren's syndrome. The other autoimmune disorder typically occurs before Sjogren's syndrome does. People with secondary Sjogren's syndrome usually have more health problems because they have two disorders. If Sjogren's syndrome is present when no other autoimmune disorders are present, it is known as primary Sjogren's syndrome. About 50% of the cases of Sjogren's syndrome occur alone and about 50% occur in the presence of another autoimmune disorder.

    Sjogren's syndrome is also known as a rheumatic disease. A rheumatic disease is a disease that causes inflammation in joints, skin, muscles, or other body tissues. Another way Sjogren's syndrome is classified is to call it a connective tissue disease. Connective tissue is the connective framework of the body that supports organs and tissues (such as skin, joints, and muscles).

    If Sjogren's syndrome mostly affects the eyes and mouth, it is known as localized Sjogren's syndrome. If it affects many different organ systems throughout the body, it is known as systemic Sjogren's syndrome. This is because it affects different systems in the body. When Sjogren's syndrome affects other glands besides the saliva and tear producing glands, this is known as extraglandular involvement.

    AT WHAT AGE DO PEOPLE USUALLY GET SJOGREN'S SYNDROME?

    Sjogren's syndrome mostly affects women over age 40. The disease usually begins in the late 40s, but can occur at any age. However, Sjogren's syndrome rarely occurs in children or teenagers.

    ARE WOMEN MORE LIKELY TO GET SJOGREN'S SYNDROME THAN MEN?

    Yes. In fact, 90% of people with Sjogren's syndrome are women.

    HOW MANY PEOPLE HAVE SJOGREN'S SYNDROME?

    It is estimated that approximately one to four million Americans have Sjogren's syndrome. About 500,000 people in England are estimated to have Sjogren's syndrome.

    HOW IS SJOGREN'S SYNDROME DIAGNOSED?

    Sjogren's syndrome can be difficult to diagnose because it shares signs and symptoms with other conditions. In fact, the average time since the first sign or symptom occurs to the diagnosis of Sjogren's syndrome ranges from 2 to 8 years. Also making Sjogren's syndrome difficult to diagnose is that the signs and symptoms mimic those of many medications. A further complication is that worldwide agreement does not exist on the criteria that should be used to diagnose Sjogren's syndrome. The signs and symptoms of Sjogren's syndrome also vary from person to person. Nevertheless, there are some techniques doctors use to diagnose Sjogren's syndrome that are described below.

    To diagnose Sjogren's syndrome, the doctor will want to get a complete history of the signs and symptoms. The doctor will want to know what the signs and symptoms are and how long they have been present. The doctor will want to know what medications you are using. The doctor will want to know what other diseases or conditions you or your family have now or have had in the past. The doctor may want to know about your diet, such as the type of liquids you drink and how often you drink them. The doctor will want to know if you use alcohol or drugs.

    The eyes and mouth will be examined for dryness to determine how severe the problem is. One technique to assess eye dryness is to use staining tests. Two staining tests are known as Rose Bengal staining and lissamine green staining. Rose Bengal and lissamine green staining involves applying a dye (colored substance) on the surface of the eyeball. Cells in the eye that have been eroded by dryness will absorb this stain whereas healthy cells will not.

    The doctor will examine the eyes with a light to tell if any staining occurred. If the cells of the conjunctiva of the eye become stained with the colored substance, this is consistent with dryness of the eyes. The conjunctiva is a clear layer that covers and protects the inside of the eyelids and the front part of the sclera (the white part of the eyes). The doctor may use a lamp (called a split lamp) that magnifies the eye. This allows the doctor to tell whether the outside of the eye is inflamed and how severe the dryness is.

    Schirmer's tests can be used to measure tear production. The Schirmer I tests involve placing a thin paper strip under the lower eyelid for a certain period of time (usually 5 minutes). The paper strip is then removed and the length of the strip that is wet from tears is measured. The measurement is compared to the length of the strip that should be wet in people with normal tear production. People with Sjogren's syndrome usually produce less than 8 millimeters of tears. The Schirmer II test is similar, but it uses a cotton swab to stimulate a tear reflex inside the nose.

    The doctor will also examine the mouth for dryness and to see if any of the major salivary glands are enlarged. A dry mouth will usually look red and sticky and the saliva is usually thick. The lips may be dry and cracked and the corners of the mouth may have sores. Tooth decay is another sign of a dry mouth. The doctor may take a sample of the saliva to test its quality and to see how much of it is being produced.

    Measurement of the flow of saliva can also be performed when assessing dry mouth. X-rays can be taken of the glands that produce saliva. This type of x-ray is known as a sialogram. A sialogram detects a dye that is injected into the parotid gland (see above). The dye is injected through a small duct in the mouth. A duct is a tube that allows fluids and/or substances to pass through it, especially those produced by glands.

    A small tissue sample of tiny salivary glands from the inside of the lower lip can be performed to look for abnormalities. The tissue sample is examined under a microscope. If inflammation and a particular pattern of lymphocytes are found in the tissue sample during this examination (types of white blood cells), the test is positive for Sjogren's syndrome. This test is the best way to tell if a dry mouth is caused by Sjogren's syndrome. If this test is positive and the patient has a dry mouth and dry eyes, the patient is generally considered to have Sjogren's syndrome. Nevertheless, the doctor may do additional tests to determine what other parts of the body have been affected.

    Blood tests can be performed to determine the presence of antibodies formed in response to two antigens known as Ro and La. See the previous section on causes of Sjogren's syndrome to read more about antibodies and antigens. The antibodies to Ro are known as anti-Ro (also known as SS-A or SS-Ro). The antibodies to La are known as anti-La (also known as SS-B or SS-La). Together, these antibodies are known as Sjogren's antibodies. The presence of these antibodies in pregnant women can cause a rash in the infant that goes away in a few months. In rare cases, these antibodies are associated with heart problems in the newborn.

    People who only have Sjogren's syndrome and no other autoimmune disorders are more likely to have the anti-Ro and anti-La antibodies present in their blood compared to people who have Sjogren's syndrome and another autoimmune disorder. People with Sjogren's syndrome and no other autoimmune disorders are more like to have anti-nuclear antibodies (ANAs) in their blood. Anti-nuclear antibodies are antibodies that are directed against the body. You can have Sjogren's syndrome, however, without having ANAs in the blood.

    Blood tests may be performed to look for other types of antibodies. For example, there are types of antibodies known as gamma globulins and immunoglobulins that everyone has in their blood, but are present in increased levels in people with Sjogren's syndrome. Antibodies formed against the thyroid may be present in the blood of people with Sjogren's syndrome. As was discussed earlier, an inflamed thyroid gland sometimes happens to people with Sjogren's syndrome. The inflamed thyroid is caused by antibodies formed against the thyroid. These antibodies, known as thyroid antibodies, travel from the salivary glands into the thyroid.

    People with Sjogren's syndrome sometimes have a substance known as rheumatoid factor (RF) in their blood. Rheumatoid factor is a type of antibody that is often found in the blood of people with rheumatoid arthritis. Rheumatoid arthritis is a disorder in which the body's defense system attacks its own tissues, causing inflammation of bone joints. Rheumatoid factor can be detected with blood tests. Blood tests may also reveal the presence of abnormal proteins known as cryoglobulins. The presence of cryoglobulins represents a risk of lymphoma (see earlier).

    The doctor may perform a blood test to check the sedimentation rate (also known as the sed rate). The sedimentation rate is the speed at which red blood cells settle to the bottom of a column of blood in a glass tube. Red blood cells are cells that help carry oxygen in the blood. The reason that the doctor will want to check the sedimentation rate is because certain inflammatory conditions can increase the speed at which the red blood cells settle to the bottom of the tube.

    The doctor will likely also perform routine blood tests to check that the liver and kidneys are functioning properly, that the blood sugar level is normal, and that the correct number of various types of blood cells (such as red and white blood cells) are present. A urine test may also be performed to see if Sjogren's syndrome has affected the kidneys. A chest X-ray may be performed to make sure that the lungs are not inflamed.

    HOW ARE PROBLEMS WITH THE BRAIN, SPINE, AND NERVES DIAGNOSED THAT ARE ASSOCIATED WITH SJOGREN'S SYNDROME?

    There are several ways in which problems with the brain, spinal cord, (and nerves outside the brain and spinal cord) can be diagnosed. One way is through an Magnetic Resonance Imaging (also known as MRI) scan of the brain or spine. MRI scans produce extremely detailed pictures of the inside of the body by using very powerful magnets and computer technology.

    A spinal tap (also known as a lumbar puncture) may be performed to diagnose problems with the brain or spine. A spinal tap is a procedure in which a needle is inserted into a space inside the spinal canal for the purpose of removing some of the cerebrospinal fluid. Cerebrospinal fluid is the fluid that cushions the brain and spinal cord. The spinal canal is the space between the spinal cord and the bony structure that surrounds it. Another way to diagnose problems with the nerves is by taking a part of a specific nerve out and performing tests on it. This is known as a nerve biopsy.

    WHAT TYPE OF DOCTOR CAN DIAGNOSE SJOGREN'S SYNDROME?

    There are many different types of doctors that can diagnose Sjogren's syndrome. Usually, a rheumatologist will work with a number of different specialists to make a diagnosis and coordinate treatment. A rheumatologist is a type of doctor that specializes in diseases of the joints, bones, and muscles. Types of doctors that may be involved in the diagnosis of Sjogren's syndrome include allergists, dentists, dermatologists (skin doctors), pulmonologists (lung specialists), otolaryngologists (ear, nose, and throat doctor), and neurologists (doctors specializing in the brain, spine, and nerves).

    Other doctors that may be involved in a diagnosis of Sjogren's syndrome include gastroenterologists, gynecologists, ophthalmologists, and urologists. A gastroenterologist is a doctor that specializes in the digestive system. A gynecologist is a doctor that specializes in the female reproductive system. An ophthalmologist is a medical doctor that specializes in ophthalmology, which is the science that deals with the structure, functions, and diseases of the eye, as well as the diagnosis and treatment of such diseases. A urologist is a doctor that specializes in the urinary tract of males and females and of the male reproductive system. The urinary tract is the part of the body that deals with the formation and release of urine (pee).

    HOW IS SJOGREN'S SYNDROME TREATED?

    Unfortunately, there is no known cure for Sjogren's syndrome. There has also yet to be a treatment found that can restore the release of fluids that moisten the body. Treatment is used to ease the signs and symptoms and differs for each person. Of course, talk to your doctor first to determine what treatment is right for you. We have divided this treatment topic into sections below.

    • DRY EYES: Artificial (fake) teardrops can be used to make the eyes less dry. These drops are available by prescription or over the counter. The tears come in different thicknesses. Thicker drops do not need to be used as often. However, the thicker drops can collect along the eyelids and lead to blurry vision. Some drops contain preservatives so they last on the shelf of the store longer. These preservatives can cause eye irritation and discomfort in some people. Drops without preservatives usually do not bother the eyes. Drops without preservatives come in single dose packages so they do not become contaminated with bacteria.

      Eye ointments are also available, which are drops that are thicker than artificial tears. They moisturize the eyes and protect the eyes for several hours. Eye ointments can cause blurry vision, which is why they tend to provide more relief at night, during sleep.

      Another option for dry eyes is to use Lacriserts (Hydroxypropyl methylcellulose), which are small pellets of chemicals that contain artificial tears to wet the surface of the eye. Lacriserts are tucked under the lower eyelid once a day. Once artificial tears are added to Lacriserts, the pellets dissolve and forms a film over the tears, trapping the moisture.

      Soft contact lenses that can be moistened often are recommended for people that use contact lenses. Wearing glasses throughout the day can also decrease dryness of the eyes. Blinking several times a minute while working on the computer or watching TV is usually helpful as well.

      In severe cases, a procedure called punctal occlusion may be recommended, in which the tear drain of the eye is closed. Normally, the tears leave he eye through two tiny openings (known as lacrimal puncta) in the corner of the eye nearest the nose. Extra tears drain down these openings and empty into the nose. This explains why the nose gets runny when someone cries a lot. By plugging up the tear drains, the extra tears cannot flow into the nose, and stay in the eye longer.

      The plugs can be temporary or "permanent." Temporary plugs are made of collagen (a type of fiber) and slowly dissolve. The "permanent" plugs are made of silicone. Silicone is a compound (combined substance) that is partly made of silicon (a non-metallic element). The word "permanent" is placed in quotes because the doctor can easily remove these plugs or the plugs eventually fall out. For a longer lasting effect, the doctor can perform surgery to seal the tear drains. The surgery can be done with a laser or through cauterization. Cauterization is the application of heated instruments or destructive chemicals to tissues to destroy tissues, promote healing within tissues, or stop bleeding.

    • DRY MOUTH: Drinking a lot of sugar free liquids (especially water) frequently helps to reduce dryness in the mouth. Frequent sips of such drinks throughout the day will be most helpful to people with dry mouth when they are talking or eating. It is important to take sips of liquids rather than to drink large amounts of liquids. Drinking large amounts of liquids will only make you pee more and can cause even more dryness by stripping the mouth of mucus (a thick slippery fluid).

      If the salivary glands are still producing some saliva, it is possible to stimulate them to make more saliva. This can be done by using sugar free candies, sugar free gum, or lemon drops. Consuming foods and drinks that are sugar free is important because they will not cause tooth decay.

      Good dental care is very important since the decreased saliva can lead to tooth decay. This means flossing once a day, avoiding sugar, visiting the dentist three times a year for a teeth cleaning and exam, and rinsing the mouth with water several times a day. The mouth should be checked for redness and sores on a daily basis. The dentist should be contacted if anything unusual is noted or if any bleeding or pain in the mouth occurs.

      Mouthwash with alcohol should be avoided because alcohol is drying. If you cannot avoid sugar, doctors recommend brushing your teeth immediately after you eat or drink something with sugar. Some dentists may recommend placing a protective covering over the teeth to prevent cavities.

      Flouride gels, fluoride supplements, and liquids with minerals in it can be prescribed by a doctor so that the proper chemical balance in the mouth can be restored. Flouride is a mineral that is useful in preventing tooth decay by strengthening the covering of the tooth. Most people that use fluoride gels use them at night. There are toothpastes available that also contain fluoride. Toothpaste that does not foam is less drying. The teeth, gums, and tongue should be brushed gently after each meal and before bedtime.

      There is also artificial saliva that people with dry mouth can use. Artificial saliva is most useful for people that produce very little saliva or none at all. Artificial saliva makes the mouth feel wet. Some artificial salivas contain fluoride, which helps prevent cavities. Salivart, a type of artificial saliva, comes in a spray can and is easy to use. The problem with all artificial salivas is that they all work for a limited time, since they are eventually swallowed. Artificial saliva can last as long as two hours. Those that contain gel in it tend to provide the longest relief.

      The doctor may prescribe Salagen (pilocarpine hydrochloride) to treat dry mouth, because this medication increases the production of saliva. Salagen can cause increased sweating and headaches. Another medication given for dry mouth is Exovac (also known as Cevimeline). Exovac causes certain glands in the mouth to produce more saliva. Exovac also has side effects associated with it, such as sweating, runny/stuffy nose, and nausea. It can less commonly cause itching, difficulty breathing, and fast breathing. Occurrence of any of these less common side effects should lead one to call the doctor immediately. The effects of Salagen and Exovac lasts for a few hours. The products can be used three to four times a day.

      To make it easier to talk, people with Sjogren's syndrome are usually told by doctors not to clear the throat before speaking. Sipping on water, chewing gum, or sucking on a candy helps improve the ability to speak as opposed to clearing the throat. Humming, making an "h" sounds, or laughing gently prior to speaking can help the vocal cords come together so that sounds are produced. Clearing the throat does the same thing, but is rough and irritating to the vocal cords.

      Medications that increase saliva and mucus can decrease dryness in the nose and throat. Dried and/or cracked lips can be treated with lip balm or lipstick that contains oils or petroleum jelly (such as Vaseline). For mouth pain, the doctor can prescribe a mouth rinse that contains medication to control pain and inflammation. The doctor can also prescribe a gel or ointment to rub on sore areas to control pain and inflammation.

    • DRY SKIN: Using heavy moisture creams and ointments when the skin is still damp can help trap moisture and can improve dry skin. Such creams are usually used about three to four times a day. Lotions, which are not as heavy as creams or ointments, are usually not recommended because they evaporate fast, a process that can cause dry skin.

      People with dry skin are usually recommended by their doctor to avoid washing with hot water and to avoid soaking the hands in water or using cleaning solutions. Short showers of less than 5 minutes are generally recommended. Moisturizing soaps can be helpful to clean oneself with. Patting the skin, instead of rubbing it, can be helpful. After patting the skin down is when the moisturizing creams should be used.

      For people that take baths, staying in the tub for 10 to 15 minutes is generally recommended because it gives the body a chance to absorb moisture. Rubber gloves should be used when doing dishes or housecleaning. The doctor can help someone with Sjogren's syndrome avoid medications that can decrease body fluids (see above).

      Avoiding smoking or exposure to smoke can be helpful to people with Sjogren's syndrome because smoke increases dryness. Avoiding windy, breezy, and drafty environments can also help one reduce dryness. Avoiding prolonged sun exposure and use of sunscreen (at least SPF 15) when going outdoors is generally recommended because many people with Sjogren's syndrome (especially those with lupus; see earlier section) get painful burns even with a small amount of sun exposure.

      Use of a humidifier in the bedroom can help moisten the skin. Regarding makeup, it is advised by many doctors to only apply mascara to the tips of the eyelashes so that it does not get in the eyes. Also, eye shadow or eyeliner is often recommended to be placed on the skin above the eyelashes as opposed to the sensitive skin under the eyelashes that is close to the eyes.

      Dryness on the outer part of the vagina can be treated with skin creams and ointments. Due to the risk of not being able to have sexual intercourse without pain because of a dry vagina, a moisturizer can be used inside the vagina to make it wet. This, in turn, would make sex more comfortable. Lubricants, as opposed to moisturizers, do not help the vagina retain moisture. This is why doctors recommend lubricants for sexual intercourse only. There are different types of lubricants. Lubricants that contain oil, such as petroleum jelly, trap moisture, but can cause sores and can interfere with the natural cleaning process of the vagina. Lubricants that dissolve in water are generally better to use.

    • PAIN, INFLAMMATION, & OTHER SYMPTOMS: Other types of treatment would be needed if Sjogren's syndrome caused pain and inflammation. This generally involves the prescription of pain medications. One group of medications that decrease inflammation is known as non-steroidal anti-inflammatories (abbreviated as NSAIDs). An example of such a medication is aspirin. NSAIDs are usually recommended for people with aching and/or swollen joints. NSAIDs should always be taken with food because they can cause stomach bleeding.

      There are types of NSAIDs available by prescription that are more powerful than those purchased over the counter without a prescription. NSAIDS are also used to treat inflammation of the lining of the lungs.

      Antidepressant medications, known as tricyclic antidepressants, are also used to decrease pain in Sjogren's syndrome, as is Neurontin. Neurontin (also known as gabapentin) is a type of antidepressant medication that is also used to treat pain.

      Steroid medications in various forms (such as pills or creams) are also used to treat pain and itchiness associated with Sjogren's syndrome because they decrease inflammation. Steroid medications are drugs that act similarly to certain steroids produced naturally in the body. Steroids are any of a large number of hormone substances with a similar and basic chemical structure. Hormones are natural chemicals produced by the body and released into the blood that have a specific effect on tissues in the body.

      A specific type of steroid medication is sometimes used to treat Sjogren's syndrome. This type of steroid medication is known as a corticosteroid and it helps decrease inflammation and slow down joint damage. Corticosteroids are a group of drugs that act similarly to a natural chemical in the body known as corticosteroid hormone. Corticosteroid hormones control the body's use of nutrients and the amount of water and salts in the urine (pee). This is why corticosteroids are sometimes used to treat kidney problems associated with Sjogren's syndrome.

      Corticosteroids are sometimes used to decrease inflammation of the windpipe, voice box, bronchial tubes, and the lining of the lungs. Bronchial tubes are airways that connect that windpipe to the lungs. If the bronchial tubes have become narrowed, making it difficult to breath, medications are available that can widen them. A humidifier can also help make it breath easier.

      The problem with corticosteroids is that they can become less effective and cause serious side effects when used for months or years. This is why doctors usually prescribe corticosteroids to treat suddenly occurring symptoms, with the goal of slowly taking the patient off the medication.

      Examples of serious side effects from corticosteroids include thinning of the bones, easy bruising, weight gain, round face, and high blood pressure. Corticosteroids can also cause diabetes mellitus. Diabetes mellitus is a complex, long-term disorder in which the body is not able to effectively use a natural chemical called insulin. Insulin's main job is to quickly absorb glucose (a type of sugar) from the blood into cells for their energy needs and into the fat and liver (a large organ that performs many chemical tasks) cells for storage.

      Medications known as alkaline agents are sometimes used to treat kidney problems associated with Sjogren's syndrome. Alkaline agents work by balancing the chemicals in the blood so that too much acid does not build up.

      An anti-malaria medication, Plaquenil (also known as Hydroxychloroquine), is sometimes used to treat Sjogren's syndrome because it seems to decrease tissue damage of the joints, skin, and other organs of the body. However, it is not very good at treating the dryness associated with Sjogren's syndrome. Malaria is a serious disease caused by parasites that is spread by mosquitoes. A parasite is any organism that lives in or on another living being, gains an advantage by doing so, but causes disadvantage to the being it is living on.

      In severe cases, drugs that decrease the response of the immune system are sometimes used. These drugs are known as immunosuppressive drugs. The purpose of using these drugs is to prevent the body from attacking itself. However, by decreasing the response of the immune system, the body is less able to fight off real infections. Examples of immunosuppressive drugs include hydroxychloroquine (Plaquenil), methotrexate (matrix), and cyclophosphamide (Cytoxan). These drugs are most likely to be used if there are problems with the kidneys, blood vessels, nerves, brain, or spine.

      If pneumonia develops, this is treated with various types of antibiotics (medications that kill bacteria) depending on the person and type of infection. Pneumonia is inflammation of the lungs due to infection.

    WHAT IS THE PROGNOSIS FOR PEOPLE WITH SJOGREN'S SYNDROME?

    Sjogren's syndrome is generally not a life-threatening condition. The signs and symptoms are generally more annoying than severe and thus the outlook for most people is good. The dryness may last for the rest of the person's life, however. In addition, some individuals with Sjogren's syndrome do have severe signs and symptoms of this condition.

    WHAT ELSE IS SJOGREN'S SYNDROME KNOWN AS?

    Sjogren's syndrome is also known as Sjogren syndrome, Sjogren's disease, Gougerot-Sjogren's disease, and sicca syndrome.

    WHAT IS THE ORIGIN OF THE TERM, SJOGREN'S SYNDROME?

    Sjogren's syndrome is named after Henrik S.C. Sjogren, a Swedish ophthalmologist who lived from 1899 to 1986. An ophthalmologist is a medical doctor that specializes in ophthalmology, which is the science that deals with the structure, functions, and diseases of the eye, as well as the diagnosis and treatment of such diseases.

    In 1933, Sjogren first described the condition when he called attention to the large number of female patients he saw whose chronic (long-term) arthritis was associated with dry eyes and dry mouth. Arthritis is an inflammatory condition of the joints, which is a place where two bones contact one another. The word "syndrome" comes from the Greek word "syn" meaning "together," and the Greek word "dromos" meaning "course." Put the two words together and you have "course together."

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