Sjogren's 101
by Thomas A. Medsger, Jr., M.D., University of Pittsburgh
Sjögren’s Syndrome (Show-grens) is an autoimmune disease characterized by malfunction of the tear and saliva-producing glands and the mucous secreting glands of the vagina. The result is the feeling of dryness of the eyes, mouth, and vagina. Sjögren’s Syndrome may be primary (occurring alone, not associated with other diseases), or secondary (occurring in patients who have another autoimmune disease such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma). Over 20% of systemic sclerosis patients have Sjögren’s Syndrome, which occurs most commonly in those with limited skin thickening (old term CREST syndrome).
CAUSES
Sjögren’s Syndrome is caused by accumulation of
lymphocytes in affected glands and the ducts which
drain these glands. Lymphocytes are normally found
in the blood stream, lymph nodes, spleen and bone
marrow. Their appearance in glandular tissues is
abnormal, signifying that organization of the
immune system has become faulty. When they are
present in glandular tissues, lymphocytes interfere
with the production and flow of tears, saliva, and
vaginal secretions.
SYMPTOMS
Dry Eyes
You may have a “gritty” or “sandy” feeling in your
eyes. On awakening in the morning you may notice
increased thick mucus in the corners of your eyes
nearest your nose. Your eyes may be unusually<
sensitive to bright light (photophobia).
Dry Mouth
You will produce less saliva, which makes moving food
in your mouth and speaking more difficult, and
reduces your sense of taste. You will crave water or
other liquids. You may not be able to eat a dry cracker
without water, or eat a meal without frequent sips of
water. You may feel the need to take a bottle of water
with you wherever you go. Your nose and throat may
also be dry, leading to decreased sense of smell,
nosebleeds, hoarseness, and dry cough. Your major
saliva producing glands (parotid glands, just below
and in front of your ears) may become painlessly
enlarged, giving the appearance of mumps.
Dry Vagina
You may experience vaginal irritation, making sexual
intercourse uncomfortable.
(Caution to patients: There are many other causes
of glandular dryness. Increased age, other diseases of
the eyes and mouth, certain medications, and lack of
estrogen in the vagina are common reasons for
dryness which should be considered by your
physicians.)
Other Symptoms
You may suffer from fatigue, which can be severe
enough to interfere with your lifestyle. You may have
stiffness or swelling of the small joints of your hands
(arthritis) and other joints. Joint pain and stiffness
are typically worse in the morning and improve within
one to two hours. Muscle pain or weakness can occur,
leading to difficulty arising from a chair or lifting your
arms over your head. You could experience white or
blue color changes at the tips of your fingers during
cold exposure (Raynaud’s phenomenon).
DIAGNOSIS
This condition is not diagnosed by a blood test. The
most important contributors to a correct diagnosis are
your medical history and physical examination. In
addition, certain tests may be helpful for your
physician.
Eye Tests
The Schirmer test is a screening method to measure
how much you are able to wet a strip of filter paper
placed inside your lower eyelid. If the paper is wet less
than 10 millimeters in five minutes after placement, it
is considered a positive test. Reduced tearing should
be confirmed by an ophthalmologist, who can perform
additional more sophisticated tests and look for other
causes of dry eye.
Mouth Tests
A biopsy of the inside of the lower lip may support the
diagnosis. In Sjögren’s Syndrome, a greatly increased number of lymphocytes is seen surrounding the small
saliva-producing glands. Injection of dye into the
parotid duct (sialogram) can demonstrate
characteristic enlargement (dilatation) and distortion
of these ducts.
Laboratory Tests
Only half of Sjögren patients have anti-SSA and/or
anti-SSB (Sjögren’s Syndrome A and B) antibodies in
their blood. Other tests which may be abnormal
include the white blood cell count (low), total gamma
globulin level (high), blood C3 and C4 complement
levels (low C4), sedimentation rate (high) and
rheumatoid factor (positive).
COMPLICATIONS
Eyes
Eye dryness may cause dry spots or ulcers on the
cornea, leading to inability to wear contact lenses and
in some cases scarring with reduced vision. Affected
eyes are more prone to viral and bacterial infections.
Mouth and Sinuses
An increased number of cavities and infection of the
gums (gingivitis) with loosening of the teeth may
occur. There is an increased frequency of overgrowth
of the common yeast candida, resulting in a mouth
infection termed candidiasis (can-di-DYE-ah-sis) or
“thrush.” There may be slow and painless enlargement
of the parotid (mumps) glands due to blockage of the
ducts through which saliva flows from the glands into
the mouth. If rapid, painful enlargement of one of
these glands occurs, accompanied by redness of the
overlying skin and fever, this may indicate a
secondary bacterial infection of the gland and is a
medical emergency. When lymphocytes interfere with
normal handling of infectious agents such as viruses
and bacteria, sinus infections become more frequent.
Respiratory Tract
Again, because lymphocytes invade normal tissues,
there is an increased risk of developing infections such
as bronchitis and pneumonia. The lung tissue itself
may be the site of lymphocyte accumulation, leading
to shortness of breath and an abnormal chest x-ray (interstitial fibrosis), but this is uncommon.
Nervous System
Lymphocytes or their products may directly injure
nerves in the brain, spinal cord, or extremities. The
results can be disturbances of memory and thought
processes, weakness and abnormal sensation in the
lower extremities, bowel and bladder dysfunction, and
numbness, tingling, “pins and needles” or burning
sensation of the toes and feet. These problems may be
permanent since the ability of nerves to regenerate is
limited.
Vasculitis
A few patients develop vasculitis (inflammation of the
walls of small blood vessels). A red spotted rash on the
legs and numbness and tingling of the feet and toes is
a clue to this complication. Vasculitis can also affect
internal organs such as the heart and intestinal tract
and is a serious complication.
Kidney
Some patients with Sjögren’s Syndrome develop blood
and/or protein in the urine and, in rare cases, mild
kidney malfunction. There are no urinary symptoms
associated with these problems, and thus a periodic
urinalysis and blood creatinine level should be
checked. Excessive loss of potassium in the urine may
lead to a low blood potassium level and muscle
weakness.
Skin
A characteristic rash may appear in skin areas
exposed to ultraviolet light or sunlight (photosensitive
rash). The rash is most often transient. It may be
scaly or appear as circular or oval patches of red skin
with a central white area.
Pregnancy
A woman with Sjögren’s Syndrome and anti-SSA
antibody in her blood may pass this antibody across
the placenta to her developing fetus. One result may
be a transient lupus-like facial rash in the infant after
delivery. A more serious problem is permanent injury
to the fetal heart, leading to a very slow heartbeat
(heart block). This problem can occur as early as the third month of pregnancy and is often fatal. For these
reasons, women with Sjögren’s Syndrome should
consult their rheumatologist and obstetrician before
becoming pregnant.
Lymphoma
Rarely, the lymphocytes in Sjögren patients can
become malignant, resulting in lymphoma. This
complication typically occurs in patients who have had
Sjögren’s Syndrome for many years. Patients may
complain about excessive fatigue and are found to
have multiple painless enlarged lymph nodes or
unusually rapid enlargement of a parotid gland. The
diagnosis is made by lymph node or parotid gland
biopsy. Fortunately, most lymphomas in Sjögren’s
Syndrome patients respond well to chemotherapy.
TREATMENT 
There is no recognized cure for Sjögren’s Syndrome.
Therefore, doctors try to treat the symptoms of the
disease to minimize their effects on your daily life.
The following aids may be recommended:
For dry eyes
- Artificial tears, every two to four hours during the
day, or a long-acting pellet in the morning and a
lubricating ointment at night
- Punctal occlusion, a surgical procedure to retain
moisture by preventing the normal flow of tears
from the inner corner of the eye into the nose. This
can be temporary (by inserting small plastic plugs)
or permanent (by tying off the ducts with a suture)
For dry mouth
- Sips of water throughout the day or over-thecounter
saliva substitutes or gels
- Sugar-free chewing gum or candies to stimulate
saliva flow
- Treatment for oral candidiasis
- One of several oral saliva stimulant medications
containing the active ingredient pilocarpine
- Good oral hygiene to prevent cavities: frequent
dentist visits for teeth cleaning; brushing and
flossing teeth regularly and thoroughly, especially
after meals; avoiding sugar-containing foods and
drinks between meals; using mouth rinses containing fluoride
For vaginal dryness
- Specially designed lubricants, but do not use
petroleum jelly (does not moisturize the vaginal
lining)
For other organs affected
- Common-sense measures: avoid cigarette smoking;
pace activities to avoid fatigue; get adequate
exercise and sleep
- Aspirin or anti-inflammatory drugs for joint pain
and stiffness or muscle pain
- Hydroxychloroquine (Plaquenil®) for arthritis, skin
rash and fatigue
- Cortisone or immune system suppressing drugs for
more serious problems such as involvement of the
lung, kidney, nervous system, or vasculitis
How is Sjögren’s Syndrome Related
to Scleroderma?
Over 20 percent of patients with systemic sclerosis
and a few with localized scleroderma also have
secondary Sjögren’s Syndrome. It is more often
detected in persons with the limited form of systemic
sclerosis. The symptoms and examination findings and
methods of diagnosis and treatment in Sjögren’s
Syndrome secondary to scleroderma are identical to
those in primary Sjögren’s Syndrome. Special
problems encountered by scleroderma patients are
reduced mouth opening, finger-tip ulcers, and
deformities of the fingers, all of which interfere with
maintaining good oral hygiene. Therefore it is
particularly important for scleroderma patients to
consult their dentists and periodontists to make sure
that they use appropriate prophylactic measures.
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