Sjogren's Syndrome
Medical Author: William C. Shiel Jr., MD, FACP, FACR
Medical Editor: Dennis Lee, MD
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Sjogren's Syndrome Antigen...Getting at the
Cause
Medical Reviewer: William C. Shiel Jr., MD, FACP, FACR
 TOKUSHIMA, JAPAN - Antigens are substances that can
stimulate the immune system to develop a response resulting in
the production of an antibody. Antibodies are proteins that can
bind antigens in the normal immune response. When antigen comes
from the body's own tissues (such as a certain tissue protein)
it is referred to as an autoantigen. The antibody that acts against
it is referred to as an autoantibody. When a disease is characterized
by the production of autoantibodies, it is called an autoimmune
disease.
Sjogren's syndrome is an autoimmune disease, characterized
by the abnormal production of extra antibodies in the blood that
are directed against various tissues of the body. This particular
autoimmune illness is caused by inflammation in the glands of
the body. Inflammation of the glands that produce tears (lacrimal
glands) lead to decreased water production for tears and eye dryness.
Inflammation of the glands that produce the saliva in the mouth
(salivary glands, including the parotid glands) leads to mouth
dryness.
Sjogren's syndrome classically combines dry eyes,
dry mouth, and another disease of the connective tissues, most
commonly rheumatoid arthritis.
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What is Sjogren's syndrome?
Sjogren's syndrome is an autoimmune disease. Autoimmune diseases are characterized by the
abnormal production of extra antibodies in the blood that are directed
against various tissues of the body. This particular autoimmune illness features inflammation in certain glands of the body. Inflammation of the
glands that produce tears (lacrimal glands) leads to decreased water
production for tears and eye dryness. Inflammation of the glands that
produce the saliva in the mouth (salivary glands, including the parotid
glands) leads to dry mouth and dry lips.
Sjogren's syndrome with gland inflammation (resulting dry eyes and mouth, etc.) that is not associated with another connective tissue disease is referred to as primary Sjogren's syndrome.
Sjogren's syndrome that is also associated with a connective tissue disease, such as
rheumatoid arthritis, systemic lupus erythematosus, or scleroderma, is referred to as secondary Sjogren's syndrome.
What causes Sjogren's syndrome?
While the exact
cause of Sjogren's syndrome is not known, there is growing scientific support for
genetic (inherited) factors. The illness is sometimes found in other
family members. It is also found more commonly in families that have
members with other autoimmune illnesses, such as systemic lupus
erythematosus, autoimmune thyroid disease, juvenile diabetes, etc. About 90% of patients with Sjogren's syndrome are female.
What are symptoms of Sjogren's syndrome?
Symptoms of Sjogren's syndrome can involve the glands, as above, but
there are also possible affects of the illness involving other organs of
the body (extraglandular manifestations).
When the tear gland (lacrimal gland) is inflamed from Sjogren's, the
resulting eye dryness can progressively lead to eye irritation, decreased
tear production, "gritty" sensation, infection, and serious
abrasion of the dome of the eye (cornea). Dry eyes can lead to infections of the eyes.
Inflammation of the salivary glands can lead to mouth dryness,
swallowing difficulties, dental decay, gum disease, mouth sores and swelling, stones and/or
infection of parotid gland inside of the cheeks. Dry lips often accompany the mouth dryness.
Other glands that can become inflamed, though less commonly, in Sjogren's
syndrome include those of the lining of the breathing passages
(leading to lung infections) and vagina (sometimes noted as pain during
intercourse recurrent vaginal infections).
Extraglandular (outside of the glands) problems in Sjogren's syndrome
include joint pain or inflammation (arthritis), Raynaud's phenomenon, lung
inflammation, lymph-node enlargement, kidney, nerve, and muscle disease. A
rare serious complication of Sjogren's syndrome is inflammation of the
blood vessels (vasculitis), which can damage the tissues of the body that are supplied by these vessels.
A common disease that is occasionally associated with Sjogren's syndrome
is autoimmune thyroiditis (Hashimoto's thyroiditis), which can lead to abnormal thyroid hormone levels detected by thyroid blood tests. Heartburn and difficulty swallowing can result from gastroesophageal reflux disease (GERD), another common condition associated with Sjogren's syndrome. A rare disease that is
uncommonly associated with Sjogren's syndrome is primary biliary cirrhosis, an immune disease of the liver that leads to scarring of the liver tissue. A small percentage of patients with Sjogren's syndrome develop cancer of the lymph glands (lymphoma). This usually develops only after many years with the illness. Unusual gland swelling should be reported to the physician.
How is Sjogren's syndrome diagnosed?
The diagnosis of Sjogren's syndrome involves detecting the features of
dryness of the eyes and mouth. The dryness of the eyes can be determined
in the doctor's office by testing the eye's ability to wet a small testing
paper strip placed under the eyelid (Schirmer's test using Schirmer tear test strips). More sophisticated
eye testing can be done by an eye specialist (ophthalmologist). Salivary
glands can become larger and harden or become tender. Salivary-gland
inflammation can be detected by radiologic nuclear medicine salivary scans. Also, the
diminished ability of the salivary glands to produce saliva can be
measured with salivary flow testing. The diagnosis is strongly supported
by the abnormal findings of a biopsy of salivary-gland tissue.
The glands of the lower lip are often used to obtain a biopsy sample the salivary-gland tissue in the diagnosis of Sjogren's syndrome. The lower lip salivary-gland biopsy procedure is easily performed under local anesthesia with the surgeon making a tiny incision on the inner part of the lower lip to expose and remove a sample of the tiny salivary glands within.
Patients with Sjogren's syndrome typically produce a myriad of extra
antibodies against a variety of body tissues (autoantibodies). These can
be detected through blood testing and include antinuclear antibodies
(ANAs), which are present in nearly all patients. Typical antibodies that
are found in most, but not all patients, are SS-A and SS-B antibodies, rheumatoid factor, thyroid
antibodies, and others. Low red blood count (anemia) and abnormal blood
testing for inflammation (sedimentation rate) are seen.
Next: How is Sjogren's syndrome treated? »
- prednisone, Deltasone, Liquid Pred - Defines the medication prednisone (Deltasone, Liquid Pred) a drug used for suppressing the immune system and inflammation. Article includes descriptions, uses, drug interaction, and side effects.
- Thyroid Blood Tests - Read about thyroid blood tests, which are used to diagnose disease of the thyroid such as hyperthyroidism and hyperthyroidism, as well as point to other diseases of the thyroid gland.
- Rheumatoid Arthritis - Learn more about rheumatoid arthritis, an autoimmune disease that causes chronic joint inflammation, which has symptoms that include stiffness, fever, muscle and joint aches, loss of appetite, and fatigue. Treatment of rheumatoid arthritis incorporates the use of first-line drugs (aspirin and corticosteroids for pain and inflammation) and second-line drugs (methotrexate and hydroxychloroquine to prevent joint destruction and promote remission).
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