Sjogren's Syndrome

from person to person. These signs and symptoms are

from person to person. These signs and symptoms are

The signs and symptoms , of Sjogren's syndrome vary

CONDITIONS ARE ASSOCIATED WITH SJOGREN'S

CONDITIONS ARE ASSOCIATED WITH SJOGREN'S

WHAT SIGNS, SYMPTOMS, AND OTHER

WHAT SIGNS, SYMPTOMS, AND OTHER

disease in which the eyes and mouth become

disease in which the eyes and mouth become

associated with Sjogren's syndrome. A dry mouth can feel like a mouthful of cotton.

associated with Sjogren's syndrome. A dry mouth can feel like a mouthful of cotton.

DRY MOUTH: A dry mouth (technically known as xerostomia) is the other major feature

DRY MOUTH: A dry mouth (technically known as xerostomia) is the other major feature

occur. In the worst case scenario, Sjogren's syndrome can cause blindness.

occur. In the worst case scenario, Sjogren's syndrome can cause blindness.

discomfort. Blurry vision can result as well. Ulcers (open sores) of the cornea can also

stuck together and the eyes may not be able to withstand strong light without it causing

stuck together and the eyes may not be able to withstand strong light without it causing

feeling, as if something foreign is under the eyelid, such as sand. The eyelids may feel

feeling, as if something foreign is under the eyelid, such as sand. The eyelids may feel

and can cause a feeling of burning and itchiness. The dry eyes can also cause a gritty

and can cause a feeling of burning and itchiness. The dry eyes can also cause a gritty

(technically known as keratoconjunctivitis sicca or KCS). The dry eyes can appear red

(technically known as keratoconjunctivitis sicca or KCS). The dry eyes can appear red

DRY EYES: The most characteristic feature of Sjogren's syndrome is dry eyes

DRY EYES: The most characteristic feature of Sjogren's syndrome is dry eyes

"Where Medical Information is Easy to Understand"™

"Where Medical Information is Easy to Understand"™

known as Candidiasis. More specifically, Candidiasis is a type of

known as Candidiasis. More specifically, Candidiasis is a type of

most commonly seen fungal infections in Sjogren's syndrome is

most commonly seen fungal infections in Sjogren's syndrome is

lives in or on another organism to obtain nourishment. One of the

lives in or on another organism to obtain nourishment. One of the

fungus is a simple type of parasite. A parasite is an organism that

fungus is a simple type of parasite. A parasite is an organism that

infections, or fungus infections can occur due to the lack of saliva. A

infections, or fungus infections can occur due to the lack of saliva. A

substances that normally helps rid the mouth of bacteria.

substances that normally helps rid the mouth of bacteria.

of saliva can also cause tooth decay because saliva contains

of saliva can also cause tooth decay because saliva contains

chewing, and eating because the food sticks to the tissues. A lack

chewing, and eating because the food sticks to the tissues. A lack

The lack of saliva in the mouth can lead to difficulty swallowing,

The lack of saliva in the mouth can lead to difficulty swallowing,

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.





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.





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.



