In the early 20th century, Swedish physician Henrik Sjögren (SHOW-gren)
first described a group of women whose chronic arthritis was accompanied
by dry eyes and dry mouth. Today rheumatologists know more about the syndrome
that is named for Sjögren and—most significantly for patients—can provide
advice about how to live with it. In most cases, Sjögren's is an occasionally
annoying but treatable condition that should not affect a person's quality
of life.
Fast Facts
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Sjögren's syndrome sometimes develops as a complication
of another autoimmune disorder.
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Symptoms vary in type and intensity, but most people
with Sjögren's are able to live normal lives.
-
Although serious complications are rare, regular medical care
is important.
What Sjögren's syndrome is
Sjögren's syndrome is an inflammatory disease that can affect many different
parts of the body, but most often affects the tear and saliva glands.
Patients with this condition may notice irritation, a gritty feeling,
or painful burning in the eyes. Dry mouth or difficulty eating dry foods,
and swelling of the glands around the face and neck are also common. Some
patients experience dryness of other mucous membranes (such as the nasal
passages, throat, and vagina) and skin.
“Primary” Sjögren's syndrome occurs in people with no other rheumatologic
disease. “Secondary” Sjögren's occurs in people who do have another rheumatologic
disease, most often systemic
lupus erythematosus and rheumatoid
arthritis.
Most of the complications of Sjögren's syndrome occur because of decreased
tears and saliva. Patients with dry eyes are at increased risk for infections
around the eye and may have damage to the cornea. Dry mouth may cause
an increase in dental decay, gingivitis (gum inflammation), and oral yeast
infections (thrush) that may cause pain and burning. Some patients have
episodes of painful swelling in the saliva glands around the face.
Complications in other parts of the body occur rarely in patients with Sjögren's
syndrome. Pain and stiffness in the joints with mild swelling may occur
in some patients, even in those without
rheumatoid
arthritis or
lupus.
Rashes on the arms and legs related to inflammation in small blood vessels
(vasculitis) and inflammation in the lungs, liver, and kidney may occur rarely
and be difficult to diagnose. Neurological complications that cause symptoms
such as numbness, tingling, and weakness have also been described in some
patients.
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The parotid gland is at the edge of the jaw and can become
swollen and inflamed in some people with Sjögren's Syndrome. |
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What causes Sjögren's syndrome
The cause of Sjögren's syndrome is not known, but it is considered an
autoimmune disorder. People with this disease have abnormal proteins in
their blood suggesting that their immune system, which normally functions
to protect the body against cancers and invading infections, is reacting
against their own tissue. The decreased production of tears and saliva
seen in Sjögren's syndrome occurs when the glands that produce these fluids
are damaged by inflammation. Research suggests that genetic factors and
possibly viral infections (as yet unidentified) may predispose people
to developing this condition.
Who gets Sjögren's syndrome
Between 400,000 and 3.1 million adults have Sjögren's syndrome. This condition can affect people of any age, but
symptoms usually appear between the ages of 45 and 55. It affects 10 times
as many women as men. About half of affected patients also have
rheumatoid
arthritis or other connective tissue diseases, such as
systemic
lupus.
How Sjögren's syndrome is diagnosed
Diagnosis depends on a combination of symptoms, physical findings, blood
tests, and sometimes special studies. Dry eyes and mouth may be early
signs of the condition but require further investigation because these
symptoms can be caused by many other conditions or medications. Special
tests may be used to assess any decrease in tear or saliva production
(an example would be the Schirmer test for tear production. An eye examination
is helpful in detecting any eye changes seen in Sjögren's. Blood
tests can determine the presence of antibodies (immune system cells that
help destroy foreign invaders) typical of the disease, including anti-nuclear
antibodies (ANA), anti-SSA and SSB antibodies, or rheumatoid factor. Biopsies
of saliva glands around the face or under the surface of the inner lip
may also sometimes be used to establish a diagnosis.
How Sjögren's syndrome is treated
Treatment is designed to lessen the most bothersome symptoms. Dry eyes
usually respond to the use of artificial tears applied regularly during
the day or to gels applied at night. Other measures, such as plugging
or blocking tear ducts, can be used in more severe cases. Eyedrops that
reduce inflammation in the glands around the eyes (cyclosporine- Restasis)
may be used to increase tear production. Dry mouth can be relieved by
drinking water, chewing gum, or using saliva substitutes. Some patients
benefit from using prescription medications that stimulate saliva flow,
such as pilocarpine (Salagen) or cevimuline (Evoxac).
If patients develop yeast infections, these can be relieved by anti-fungal
therapies. The currently available treatments may help relieve some of
the dryness but usually some dryness persists.
All patients should receive regular dental care in order to prevent cavities
and tooth loss that may occur as a complication of the disorder. Patients
with dry eyes should see an ophthalmologist (eye doctor) regularly for
signs of damage to the cornea. Patients with excessive redness and pain
in the eyes should be evaluated for infections.
Hydroxychloroquine (Plaquinel),
an antimalarial drug used in lupus and
rheumatoid arthritis, may
be helpful in some patients with Sjögren's
syndrome by reducing joint pain and rash experienced by some patients.
Patients with rare but serious systemic symptoms, such as fever, rashes,
abdominal pain, or lung or kidney problems, may require treatment with
corticosteroids such as prednisone (Deltasone and others)
and/or immunosuppressive agents methotrexate (Rheumatrex),
azathioprine (Imuran), mycophenolate (Cellcept), cyclophosphamide (Cytoxan). In addition, rituximab (Rituxan) and other biological therapies (as used in rheumatoid arthritis) are undergoing evaluation for treating patients with severe systemic manifestations of disease.
Broader health impact of Sjögren's syndrome
A vast majority of patients with Sjögren's syndrome remain very healthy,
without any serious complications. Patients should be aware that they
do face an increased risk for infections in and around the eyes and an
increased risk for dental problems—both of which are due to the long-term
reduction in tears and saliva.
Rarely, patients may have complications related to inflammation in other
body systems, including:
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Joint and muscle pain with fatigue
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Lung problems that may mimic pneumonia
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Abnormal liver and kidney function tests
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Skin rashes related to inflammation of small blood vessels
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Neurologic problems causing weakness and numbness
In a small number of people, Sjögren's syndrome may be associated
with lymphoma, a cancer of the lymph glands.
Living with Sjögren's syndrome
People with Sjögren's syndrome are usually able to live normal lives
with very few adjustments. When a diagnosis is made, many patients must
focus a great deal of attention dealing with dry eyes and dry mouth, but
these symptoms tend to subside with time. Any pain or redness in the eyes
should be evaluated promptly, as this may signal an infection. To reduce
risk for cavities and other dental problems, patients must pay close attention
to proper oral hygiene and regular dental care.
Patients should see their physician regularly for general health screening,
and should pay close attention to any abnormal swelling in the glands
around the face or neck, under the arms, or in the groin areas as this
may be a sign of lymphoma.
Points to Remember
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To reduce risk for cavities and other dental problems,
patients must pay close attention to proper oral hygiene and regular
dental care. |
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Sjögren's syndrome is an autoimmune condition that can
occur at any age, but is most common in older women. Many patients
develop Sjögren's syndrome as a complication of another autoimmune
disease, such as rheumatoid arthritis or lupus.
-
Most of the treatment for Sjögren's syndrome is aimed
at relieving symptoms of dry eyes and mouth and preventing and treating
long-term complications such as infection and dental disease. Currently
available treatments often do not completely eliminate the symptoms
of dryness in some patients.
-
Most patients with Sjögren's syndrome remain healthy,
but a number of rare complications have been described, including
an increased risk for cancer of the lymph glands (lymphoma). Thus,
regular medical care and follow up is important for all patients.
The rheumatologist's role in the treatment
of Sjögren's syndrome
Sjögren's syndrome cannot be cured, but in many cases proper treatment helps
to alleviate symptoms. Rheumatologists are specialists in musculoskeletal
disorders and therefore are more likely to make a proper diagnosis. They
can also advise patients about the best treatment options available.
To find a rheumatologist
For more information about rheumatologists, click
here.
For a listing of rheumatologists in your area, click
here.
For more information
The American College of Rheumatology has compiled this list to give you
a starting point for your own additional research. The ACR does not endorse
or maintain these Web sites, and is not responsible for any information
or claims provided on them. It is always best to talk with your rheumatologist
for more information and before making any decisions about your care.
National Osteoporosis Foundation
www.nof.org
National Institute of Health Osteoporosis and Related Bone Diseases Resource
Center
www.osteo.org
The Sjögren's Syndrome Foundation
www.sjogrens.org
Updated April 2004
Written by Christopher Wise, MD, and reviewed by the American College of
Rheumatology Communications and Marketing Committee.
