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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?
from person to person. These signs and symptoms are
discussed below:
Treating dry eye with drops.
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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.
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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.
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.
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.
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."