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24 November, 2009

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Sjogren's syndrome

Sjogren’s syndrome is a relatively common autoimmune disease that mainly affects the eyes and salivary glands, but can also affect the sweat glands. Immune system cells, called lymphocytes, and autoantibodies attack the body’s moisture-producing glands. This causes chronic inflammation (pain, heat and swelling), which can eventually destroy the tissues.

The disorder may progress slowly, so the typical symptoms of dry eyes and mouth may take years to show. However, rapid onset can also occur. Symptoms can be mild, moderate or severe and the progression is often unpredictable. Other parts of the body may also be affected.

Around 0.05 per cent of the population is thought to have Sjogren’s syndrome. Women with the disorder outnumber men ten to one. There is no cure, but the disorder can be managed. Sjogren’s syndrome is not usually life-threatening. However, in rare cases, organs such as the liver and kidneys may be involved, which can increase the severity of the condition. The chance of developing lymphoma (cancer of the lymph glands) is also significantly higher in people with Sjogren’s syndrome.

Symptoms
The more common symptoms of Sjogren’s syndrome can include:

Insufficient tear production
Dry, reddened and itchy eyes
Light sensitivity
Dry mouth
Sore and cracked tongue, lips and gums
Dental decay
Swallowing difficulties
A thirst for water while talking
Swollen and tender areas around the ears and below the jaw
Strange taste and smell sensations
Fatigue
Swelling and pain in the joints.

The cause is unknown
No one knows what prompts the immune system to attack the tear and salivary glands. Around half the people who have Sjogren’s syndrome also have rheumatological conditions (such as scleroderma, lupus and rheumatoid arthritis). This is known as secondary Sjogren’s syndrome. In the remaining cases, the autoimmune response occurs in isolation. This is known as primary Sjogren’s syndrome. It is believed to have a genetic basis and to be triggered by an environmental factor – possibly a virus.

Other areas can be affected
All instances of Sjogren’s syndrome are systemic, which means they affect the entire body. Sjogren’s syndrome can be mild, moderate or severe. It can progress unpredictably and some individuals experience a remission of symptoms.

Common complications include dental decay, skin rashes and an increased susceptibility to thrush infections of the mouth. In some people, the disorder affects other areas of the body.. The mucus membranes of the nose and vagina can dry out and internal organs (including kidneys, intestines, lungs, heart and liver) may become inflamed. The structures of the circulatory and nervous systems can also be affected.

Diagnosis
Early diagnosis and treatment are important for preventing complications. Diagnosis of Sjogren’s syndrome can be difficult, since the symptoms can imitate those of other diseases such as lupus, fibromyalgia, multiple sclerosis and senile glandular atrophy. Dry mouth and eyes can also be the side effects of medications for other problems, such as depression and high blood pressure.

Diagnosing Sjogren’s syndrome involves a number of tests, which may include:

Schirmer’s test – special blotting paper held to the eye is used to assess the degree of tear production.
Eye examination – including the use of special dyes.
Biopsy – a small tag of salivary gland tissue is removed (usually from the lip) and examined in a laboratory.
Salivary gland tests – to check the degree of saliva produced.
Blood tests – a series of blood tests may be performed to check the levels of particular immune system cells and immunoglobulins and for autoantibodies in the blood.

Treatment
There is no cure for Sjogren’s syndrome, but the disorder can be managed. Treatment options include:

Artificial tears and lubricating ointments for the eyes
Artificial saliva
Mouth rinses and lozenges
Nasal sprays
Vaginal lubricant lotions
Moisturising lotion for the skin
Non-steroidal anti-inflammatory drugs (NSAIDs)
Corticosteroid medications
Immunosuppressive medications.

Managing the symptoms
Other suggestions to manage the symptoms of Sjogren’s syndrome include:

Avoid dry and dusty environments.
Avoid air drafts or windy weather.
Wear protective glasses when outside in the wind and sun.
Exercise regularly.
Drink water regularly.
Practice good dental hygiene and visit your dentist often.

Where to get help

Your doctor
Rheumatologist
Support groups
Arthritis Victoria Tel. (03) 03 8531 8000 or 1800 011 041

Things to remember

Sjogren’s syndrome is an autoimmune disease that mainly affects the eyes and salivary glands, but can also affect the sweat glands.
Immune system cells attack and destroy the tissue of the tear (lachrymal) and salivary glands.
There is no cure, but the condition can be managed with medications and products such as artificial tears and saliva.

You might also be interested in:
Dry mouth syndrome.
Immune system.
Lupus.
Polymyositis.
Rheumatoid arthritis.
Scleroderma affects the skin.

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Go to More information for support groups, related links and references.

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