What is Sjogren's Syndrome?
Sjogren's syndrome is a chronic rheumatic disease.
Its characteristic features are inflammation
and destruction of the tear producing and salivary
glands. As a result, people who suffer from
Sjogren's syndrome have progressively less tears
and saliva and experience dryness of the eyes
and mouth. Other moisture producing glands may
also be affected and causing dryness in the
nose, throat irritation, dry skin or loss of
vaginal lubrication. This disease may be mild
so that those affected might not seek medical
advice at all. In others, Sjogren's syndrome
is more severe with arthritis, skin rashes,
blood vessel, lung or kidney involvement.
Sjogren's syndrome may occur on its own (primary
Sjogren's syndrome) or in association with
another autoimmune rheumatic disease such
as rheumatoid
arthritis, scleroderma
or systemic lupus erythematosus (secondary
Sjogren's syndrome).
What Causes
Sjogren's Syndrome?
The cause of Sjogren's syndrome is not known.
Scientists believe it may be hereditary and/or
infectious in origin. Immune cells (lymphocytes)
are found in the glandular tissues causing
a chronic inflammation which leads to the
destruction of the glands.
Who Gets Sjogren's
Syndrome?
Sjogren's syndrome is more common than originally
believed. It affects about 0.05% of the Caucasian
population. This disorder is 10 times more
common in females compared to males. The usual
age of onset is in the fourth to the fifth
decade but it may occur at any age.
Signs &
Symptoms
As mentioned, this disease causes dry eyes
and mouth. When the eyes are dry, the person
may experience tiredness and a gritty sensation
in the eyes. At times, there may be itching,
soreness or even pain of the eyes. When severe,
the sufferers themselves will notice they
have little or no tears and the eyes may be
red. The lack of saliva causes the person
to drink water often especially when talking.
Lips and tongue may feel sore and even crack.
Food tends to stick to the teeth and dental
decay occurs in those with poor oral hygiene.
Some may experience intermittent swelling
of the salivary glands which appear as swellings
at the sides of the face (parotid glands)
and below the chin. Other sites such as the
nose, throat, skin or vagina may (submandibular
glands) may also be dry.
Affected individuals may have joint pain
and swelling, skin rashes, or breathlessness
(if there is lung involvement). They may also
feel tired all the time and complain of a
lack of energy.
In a very small number of patients, Sjogren's
syndrome is associated with lymphoma.
How is it Diagnosed?
If the diagnosis of Sjogren's syndrome is
suspected, blood tests which measure specific
autoantibodies will be done. A simple Schirmer's
test should be performed. This test measures
the amount of tear formation using a strip
of blotting paper. A referral to the ophthalmologist
may also be made. A biopsy of one of the salivary
glands, usually from the lower lip, can be
easily done to demonstrate the presence of
inflammation in the salivary glands. Other
tests may be required to distinguish Sjogren's
Syndrome from other causes of dry eyes and
mouth.
What is the
Treatment?
There is no cure for the disease. However
many patients can be helped with control of
the symptoms. Artificial tears, regular mouth
rinses, lozenges or sips of water, drugs to
stimulate saliva secretion, skin moisturiser
and vaginal lubricant, provide symptomatic
relief. Regular dental visits are important
for those with significant salivary loss.
For those with arthritis, non-steroidal
anti-inflammatory drugs and hydroxychloroquine
are the usual drugs used. Sometimes oral corticosteroids
may be required for the treatment of fatigue,
enlarged salivary glands and some major organ
involvement. Rarely immunosuppressants such
as cyclophosphamide may be required.
What is the
Prognosis?
Sjogren's syndrome is not a life threatening
disease. The severity of the disease varies
from individual to individual and many have
mild problems. The overall outlook for many
with this condition is good.