Sjogrens syndrome

by Nathan Wei, MD, FACP, FACR

Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.

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Information in part from the Arthritis Foundation

Sjögren's syndrome is an autoimmune disorder which is a close cousin to systemic lupus erythematosis. The most common symptoms of Sjögren's syndrome are dry eyes or a dry mouth (sometimes both together), fatigue, and achiness.

Although there is no cure for this syndrome, most people can be treated effectively. A few have more serious symptoms which are difficult to manage.

There are two types of Sjögren's syndrome. Secondary Sjögren's syndrome is associated with another rheumatic disease such as rheumatoid arthritis. Primary Sjogren's syndrome is not associated with any underlying rheumatic disease.

Sjögren's syndrome is not the same as sicca syndrome. 'Sicca' simply means dryness. Sicca syndrome describes problems of dry eyes and a dry mouth which are not caused by an autoimmune disorder. Sicca syndrome occurs when the tear glands in the eyes are damaged, or when the glands in the mouth which produce saliva are damaged. This damage can be caused by several illnesses, including sarcoidosis, excessive x-ray irradiation and iron overload disease (hemochromatosis).

Primary Sjögren's syndrome is probably the second most common autoimmune rheumatic disorder after rheumatoid arthritis.

It occurs mostly in women between the ages of 40 and 60. It is much less common in men (1 in 10 of those with Sjögren's syndrome are men), and occurs only rarely in childhood. It affects all races.

The two most common complaints are:

• dry eyes or a dry mouth (sometimes dry eyes and a dry mouth)
• fatigue and achiness.

In Sjögren's syndrome, the immune system mainly attacks the tear glands in the eye and the salivary glands in the mouth. The immune system delievers cells into these glands which cause the glands become inflamed. The body also produces antibodies which damage the glands.

Other parts of the body can be affected, such as the liver and kidneys. Joints can become inflamed.

The cause of Sjögren's syndrome is still unknown but it has been linked to several viruses (e.g. the Epstein–Barr virus, the retroviruses and the HTLV group of viruses).

There may be some hereditary (genetic) factors.

There are generally few problems during or following pregnancy. However, when mothers have primary Sjögren's syndrome a few new-born babies may have a condition called heart block. This is because antibodies known as anti-Ro and anti-La can be passed from mother to baby.

People with Sjögren's syndrome are slightly more likely to develop side-effects to common drugs, e.g. antibiotics.

The primary diagnostic tests for Sjögren's syndrome are:

Schirmer's test
A small piece of blotting paper (sterilized and pre-packaged) is used to measure tear production.

Slit lamp examination
An eye specialist will place eye-drop dye (Rose Bengal dye) in the eye before examining the eye with an instrument called a slit lamp. This is a lamp which shines light through a very narrow slot. The beam produced allows the eye to be examined more effectively.

Measurement of saliva production
This can be measured by arranging for the person with Sjögren's syndrome to spit into a paper cup over a specific period of time. More sophisticated investigations include isotope scanning of the salivary glands or injecting contrast dyes into the salivary glands.

Laboratory blood tests
These are done because two antibodies are found more frequently in primary Sjögren's syndrome than in other disorders. These are the antibodies known as anti-Ro and anti-La, found in about 75% and 60% of people with primary Sjögren's syndrome respectively. Because they occur only rarely in other conditions, these antibodies are useful in diagnosing primary Sjögren's syndrome.

Minor salivary gland (Lip) biopsy
One of the tiny salivary glands can be removed very simply from the lower lip using local anesthetic. The gland is examined under a microscope to check the diagnosis.

Patients with Sjogren 's syndrome have an increased risk of developing certain types of cancer (cancers of the lymphatic tissues – lymphoma). One of the first signs can be enlarged lymph glands.

The treatment for Sjogren’s syndrome is as follows:

Eye dryness:Artificial tears, eye lubricants, avoid contact lenses, use tinted eyeglasses.

Eye stickiness: mucolytic agents such as acetylcysteine eye drops

Very dry eyes: cyclosporine eye drops, eye surgery such as punctual occlusion

Mouth dryness: artificial saliva such as Salivart. Salivary sprays are also available. Chlorhexidine mouth wash. Sugarless gum. Biotene toothpaste and other mouth care products.

A humidifier and avoidance of medicines that can cause dryness is recommended.

Medications such as Salagen and Evoxac may help with tear and saliva flow.

For patients with specific autoimmune problems, treatment of the underlying autoimmune process is helpful. This will include immunosuppressive agents and biologic agents such as those used to treat systemic lupus and rheumatoid arthritis.

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