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Sjogren's Syndrome is a chronic disorder that causes
insufficient moisture production in certain glands of
the body. It occurs when a person's normally protective
immune system attacks and destroys moisture-producing
glands, including salivary glands and lacrimal
(tear-producing) glands. The lungs, bowel and other
organs are less often affected.
Sjogren's Syndrome is named after the Swedish eye
doctor, Henrik Sjogren, who first described the
condition.
Sjogren's Syndrome may cause the following symptoms:
- Extremely dry eyes
causing a feeling of grit or sand in the eyes or a
burning redness
- Extremely dry mouth and
throat causing difficulty in chewing and swallowing,
decreased sense of taste, difficulty speaking, an
increase in dental cavities, and dry cough or
hoarseness
- Enlarged parotid glands
(located at the angle of jaw) and sometimes
infection of the these glands
- Excessive fatigue
- Aches and pains in
muscles and joints
Less common features of Sjogren's
Syndrome are:
- Irritation of the
nerves in the arms, hands, legs or feet (neuropathy)
- Thyroid gland
abnormalities
- Skin rashes
- Memory loss or
confusion
- Numbness or tingling
- Gastrointestinal
problems
- Inflammation of the
lungs, kidneys, liver or pancreas
- Cancer of the lymphatic
tissue (occurs in less than 1 percent of patients)
What causes Sjogren's Syndrome?
Normally, the immune system (the body's defense against
invaders) protects the body from infection and foreign
substances such as bacteria and viruses.
In autoimmune diseases, such as Sjogren's Syndrome,
the immune system triggers an inflammatory response when
there are no foreign substances to fight off. This
inflammatory response causes the body's white blood
cells to attack and destroy certain moisture-producing
glands.
The exact cause for the abnormal immune response in
Sjogren's Syndrome is unknown. Some theories suggest
that a virus or bacteria may alter the immune system,
causing it to attack the glands. Certain people may have
a genetic or inherited factor that makes them more
likely to develop Sjogren's Syndrome.
What are the forms of the disease?
Sjogren's Syndrome occurs in two basic forms: primary
Sjogren's Syndrome, the disease by itself and not
associated with any other illness; and secondary
Sjogren's Syndrome, disease that develops in the
presence of another autoimmune disease such as
rheumatoid arthritis, systemic lupus erythematosus or
vasculitis.
More than 1 million people in the United States have
Sjogren's Syndrome. More than 90 percent of people
affected by Sjogren's Syndrome are women. The disease
can affect people of any race or age.
How is primary Sjogren's Syndrome diagnosed?
The diagnosis of Sjogren's Syndrome is based on several
factors, including:
- Presence of dry eyes
and mouth. An ophthalmologist can detect dry eyes by
measuring tear production or carefully examining the
cornea (clear part of the eye).
- Certain laboratory
tests also suggest that dry eyes and mouth are
caused by autoimmune mechanisms. Examples include
the presence of autoantibodies in the blood, known
as anti-SSA or anti-SSB (also called anti-Ro or
anti-La).
- Biopsy of the inner lip
(performed in some cases to prove the diagnosis of
primary Sjogren's Syndrome). The biopsy may show
inflammation damaging salivary glands.
How is secondary Sjogren's
Syndrome diagnosed?
Secondary Sjogren's Syndrome is generally diagnosed when
someone with an established autoimmune disease, such as
rheumatoid arthritis or systemic lupus erythematosus,
develops extreme dryness of the eyes and mouth. This
diagnosis only rarely requires a lip biopsy.
Can other problems mimic Sjogren's Syndrome?
Sometimes, the use of certain medications can cause side
effects that mimic the symptoms of Sjogren's Syndrome.
Medications such as tricyclic antidepressants,
antihistamines and radiation treatments to the head and
neck, as well as other autoimmune disorders, can also
cause severely dry eyes and mouth.
How is Sjogren's Syndrome treated?
There is no cure for Sjogren's Syndrome, but it can be
treated and controlled. The goals of treatment are to
decrease discomfort and reduce the harmful effects of
dryness. The type of treatment prescribed will be
tailored to each patient's symptoms and needs. The
following are some of the main methods of treatment for
Sjogren's Syndrome.
- Good oral hygiene:
Good mouth care may not prevent a dry mouth, but it
helps prevent infection. Toothpastes and oral gels
are available for people with dry mouth symptoms.
These products contain low doses of peroxide (high
amounts can cause more severe dryness). These
products may also have antibacterial action to
reduce the severity of dental cavities over a long
period of time.
- Increasing eye
moisture:
Dry eyes are mainly treated with the use of
artificial tears, and a wide variety of products are
available. Artificial tears must be used regularly
and more often in dry environmental conditions such
as on airplanes, in air conditioned buildings and on
windy days.
While artificial tears are
helpful, they often do not last long enough. Thicker
preparations are available that last longer. These are
often used at bedtime because they sometimes cause
blurry vision.
Surgery to slow the
disappearance of tears is another treatment option when
artificial tears are not sufficient.
- Medications:
Medications that tend to deplete body fluids should
be avoided. Mild pain-relieving medications
(analgesics) including acetaminophen (such as
Tylenol) or nonsteroidal antiinflammatory drugs (NSAIDs
such as Motrin and Aleve) can reduce muscle or joint
pain.
In some patients, the
antirheumatic drug hyroxychloroquine has been beneficial
in decreasing pain and salivary gland swelling.
For patients with
generalized symptoms, particularly when the disease
affects internal organs (including the gastrointestinal
system, kidneys or nervous system), high doses of
immunosuppressive medications may be necessary. These
include medicines such as prednisone (a steroid) and,
rarely, chemotherapy-type medications.
- Balance rest and
exercise:
Guided exercise programs can help patients overcome
fatigue, maintain flexibility and overcome joint and
muscle pain.
You can learn more about Sjogren's Syndrome by
contacting the Sjogren's Syndrome Foundation at
800-475-6473 (800-4-SJOGREN).
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