Sjogren's Syndrome

What Is Sjogren's Syndrome?

In Sjogren's syndrome white blood cells can invade moisture producing
glands as well as joints, muscles, skin and internal organs, often 
damaging them.

Sjogren's (SHOW-grens) syndrome is an autoimmune disease that causes dry
eyes and a dry mouth. An autoimmune disease is a disease In which the 
immune system turns against itself. Your immune system is your body's 
natural defense against antigens, which are substances that your body 
regards as foreign. Bacteria and viruses are examples of antigens. The 
immune system fights off antigens by activating certain white blood 
cells and producing antibodies, which are special types of blood 
protein. These cells and antibodies then attack antigens and render them 
harmless.

Occasionally, the immune system does not function properly and loses the 
ability to distinguish between its own body cells and antigens. Instead 
of fighting antigens, the immune system mistakenly fights the body's own 
cells. This is referred to as an autoimmune response (auto means self 
and this is a response directed against the body's own cells).

Sjogren's syndrome can occur in two forms: primary and secondary.
Primary Sjogren's syndrome occurs by itself and is not associated with
other diseases. Secondary Sjogren's syndrome occurs with rheumatic
diseases such as rheumatoid arthritis, systemic lupus erythematosus 
(lupus), polymyositis and some forms of scleroderma. Rheumatic diseases 
are conditions that affect Joints, bones, muscles, skin and sometimes 
other organs.

Sjogren's syndrome can affect people of any race and any age, although
it is relatively rare in people under age 20. Ninety percent of the 
people affected are women. The tendency to develop Sjogren's syndrome
increases if someone in your family has had the condition. It is 
estimated that Sjogren's syndrome affects more than one million people
in the U.S.

What Happens in Sjogren's Syndrome?

In Sjogren's syndrome, a type of white blood cell called a lymphocyte
invades moisture-producing glands, such as tear and salivary glands in 
the head and Bartholin's glands in the vagina. Lymphocytes can damage 
these glands and prevent them from producing moisture.

Sjogren's syndrome also can cause problems in other parts of the body.
These problems can include joint inflammation as well as inflammation in 
parts of the lungs, kidneys, liver, nerves, thyroid gland and brain. 

Dry Mouth

The mouth normally contains saliva, which aids chewing and swallowing. 
People with Sjogren's syndrome produce much less saliva. This makes
chewing, swallowing and speaking difficult. It also can decrease your 
sense of taste.

Dry Eyes

Your eyes may feel dry, gritty or "sandy" They may burn and look red. 
Excess mucous may accumulate in the corners of your eyes while you 
sleep. Your eyes may be more sensitive to sunlight.

lf not properly treated, Sjogren's syndrome can lead to dry spots on the
eyes and corneal ulcers (ulcers on the clear covering of your eyeball). 
On rare occasions, this can cause loss of vision.

IS IT REALLY SJoGREN'S SYNDROME?

Not every person with dry mouth or dry eyes has Sjogren's syndrome. Dry
mouth or dry eyes can occur because of the aging process. Dryness can be 
a symptom of other diseases. Dryness also can be a side effect of such 
medications as diuretics (water pills), antihistamines or certain high 
blood pressure medications.

Swollen Salivary Glands

Three pairs of major glands produce saliva. These glands are located 
under your tongue, in your cheeks in front of your ears, and under the 
back of your mouth. They may feel or look swollen. Occasionally they may 
become tender and be accompanied by a fever. Swollen glands develop in 
about half of the people with Sjogren's syndrome.

Dental Caries (Cavities)

This is a common problem that becomes worse with dry mouth. Saliva 
contains antibacterial enzymes that fight bacteria and defend against 
dental caries. When there is less saliva, you produce fewer 
antibacterial enzymes. Consequently, your teeth may develop cavities 
more easily, particularly at your gumline.

Oral Yeast Infection

Oral yeast infection (candidiasis - can-di-DYE-ah-sis) is a condition 
that produces redness and burning in the lining of the mouth of many 
people with dry mouth. It is caused by an excess of a common yeast, 
Candida (CAN-di-dah), that usually is present in the mouth in small 
quantities.

Dry Nose, Throat and Lungs

Dryness may make your throat feel dry and tickly. You may have a dry 
cough, hoarseness, a decreased sense of smell and nosebleeds. Dryness 
also can lead to pneumonia, bronchitis and ear problems.

Dryness of the Vagina

Sjogren's syndrome can cause vaginal irritation and make intercourse
painful for women.

Fatigue

Fatigue is a common symptom of Sjogren's syndrome. It can develop either
from the disease itself or as a result of the physical and emotional 
stress of having a chronic illness.

Other Problems

Other problems can include inflamed, achy joints; muscle weakness; dry 
skin; rashes; constipation; inflamed nerves; feelings of numbness and 
tingling; and swollen lymph nodes. (In rare situations, lymph gland 
swelling can result in a type of cancer called lymphoma.) These are 
reasons why medical exams and continued follow-up are important.

What Causes Sjogren's Syndrome?

The cause of this condition is not known. There is some evidence that 
heredity; viral infections and hormones may contribute to Sjogren's
syndrome.

How Is Sjogren's Syndrome Diagnosed?

Your doctor's diagnosis may be based on several sources of information. 
These include: 

Your Medical History

Your doctor will examine your medical records and ask you to describe 
your symptoms.

Your Physical Examination

Your doctor will look or feel for other signs, such as:
changes in your eyes, mouth and/or salivary glands; enlargement of
lymph nodes in your neck; tenderness in your muscles; and inflammation
in your joints. 

Chest X-Ray Results

Your doctor may want you to get a chest X-ray in order to determine if 
any changes have occurred in your lungs.

Laboratory Tests

Your doctor also may have you undergo various lab tests. These include:

Blood Tests

Tests for specific blood markers can help in diagnosing Sjogren's
syndrome. However, not everyone with Sjogren's has these markers, and
not everyone with these markers has Sjogren's syndrome.

Schirmer Test

This test helps determine how dry your eyes are. It involves placing a 
small piece of filter paper beneath your lower eyelid to measure the 
amount of tears your eyes produce.

Slit-Lamp Examination

This is a more accurate way to find out if your eyes are dry. In this 
test, the doctor puts a drop of dye into your eye and examines your eye 
with a special instrument called a slit lamp. The dye will stain the dry 
or eroded areas of your eye. An ophthalmic (eye) specialist usually 
conducts this kind of test.

Lip Biopsy

In this test, the doctor removes a few minor salivary glands from inside 
your lip. The glands are examined under a microscope. Their appearance 
determines if salivary changes you are experiencing are the same ones 
that occur in Sjogren's syndrome.

Salivary Function Tests

These tests measure the actual amount of saliva you produce and help 
determine how dry your mouth is.

Urine Tests

These tests may be done to check your kidney function.

A NOTE ABOUT PREGNANCY

Pregnancy may present special problems for the developing fetus in women 
with Sjogren's syndrome. Your doctor may check you for certain
antibodies (blood proteins) that may help identify potential problems.

If you're a woman who has one of the rheumatic diseases associated with 
secondary Sjogren's syndrome - especially lupus - and you have
experienced kidney problems and/or high blood pressure, be sure to see 
your doctor. If you do become pregnant, you and your doctor can work out 
the best treatment plan.

How Is Sjogren's Syndrome Treated?

As yet, there is no cure for Sjogren's syndrome. But proper treatment
can help relieve symptoms so that you can live a comfortable and 
productive life.

One of the main goals of treatment is to relieve discomfort and lessen 
or prevent the effects of the dryness. Since Sjogren's syndrome affects
everyone differently, your treatment plan needs to be based upon your 
specific needs. Following are some things that can help you cope with 
the symptoms.

For Systemic Problems

Since Sjogren's syndrome is a systemic disorder, which means that it can
affect various internal organs and body parts, several different 
medications can be used to treat it. Aspirin and nonsteroidal 
anti-inflammatory drugs (NSATDs) can help reduce joint pain and 
stiffness, as well as muscle aches. Recent studies have shown that 
hydroxychloroquine (Plaquenil) can help relieve joint pain, rashes and 
fatigue in some people. If you have serious complications, your doctor 
may recommend stronger medicines, such as steroids (cortisone) or 
medications that may alter or modify your immune system response.

Exercise also can help treat systemic problems by keeping joints and 
muscles flexible. Walking, swimming and range-of-motion exercises are 
well suited for people with Sjogren's syndrome.

For Dry Mouth

Sip water throughout the day as needed. Use sugar-free gum or candies
to stimulate saliva production. Seek treatment for oral yeast infection
(candidiasis) from your dentist or physician. Your mouth will feel 
better afterward. However, candidiasis is a condition that can reoccur, 
which means you may need additional treatment. Try saliva substitutes
or mouth-coating gels. They are particularly useful at night and are 
available without a prescription. 

To Prevent Caries (Cavities)

Have frequent dental checkups. Ask your dentist to recommend fluoride-
containing products, particularly for dry mouth. Brush and floss your
teeth effectively and regularly, especially after meals. Eliminate
sugary foods and drinks between meals. 

For Dry Eyes

Use artificial tears to help relieve the discomfort of dry eyes. You
may wish to use preservative-free products if you apply the drops more 
than four times per day. Try lubricating eye ointments at night and
small, long-acting pellets during the day. Your doctor may recommend a
procedure called punctal occlusion. This is a simple operation that 
prevents tears from draining out of your eyes and into your nose. This 
procedure helps retain tears so your eyes can benefit from natural 
moisture. 

For Dry Skin

Use moisturizing lotions for sensitive skin. Avoid drafts from air
conditioners, heaters and radiators whenever possible. Avoid
detergents, deodorant soaps and excessively hot water. Use a humidifier
whenever possible. 

For Vaginal Dryness

Use lubricants made specifically to help vaginal dryness. Don't use
petroleum jelly. 

CHECKUPS ARE IMPORTANT!

Members of your health-care team may include your family doctor, a 
rheumatologist (an arthritis specialist), an eye specialist and your 
dentist. Since Sjogren's syndrome can affect many parts of your body,
regular checkups can help detect and prevent future problems.

The Future

Sjogren's syndrome is generally not life-threatening. The outlook for
people with this condition is usually good. Dryness, however, may last 
for the rest of your life. By using artificial moisture and preventing 
dental caries (cavities), you can help prevent serious problems.