SJOGREN'S SYNDROME

What is it?

In 1933. a Swedish physician, Henrik Sjogren, drew attention to the large number of his female patients whose chronic arthritis was accompanied by dry eyes and dry mouth.

Since then patients with this combination of symptoms have been described as having Sjogren's Syndrome. Today it is recognised to be the second most common auto-immune rheumatic disease.

There are two categories of Sjogren's Syndrome (SS). It can be localised, affecting mainly the eyes and/or the mouth. Or. it can be systemic, and occurring in conjunction with connective tissue diseases such as Rheumatoid arthritis, lupus or scleroderma.

Who gets it?

Recent estimates suggest that there may be as many as half a million sufferers in the UK alone.

SS is a chronic auto-immune disease in which the body's immune system reacts against itself, destroying the exocrine (mucous-secreting) glands as though they were foreign bodies. Autoimmunity means making antibodies to your own tissues. Antibodies are normally made to counter infectious agents, called antigens, and are designed to destroy them. When antibody and antigen meet, inflammation results. When this happens, as for example, in influenza, the worst symptoms occur when the antibodies are actually fighting the antigen, in this case their 'flu virus; but when the virus is removed the symptoms vanish.

In auto-immune diseases it is impossible to get rid of the antigen because it is part of the body.

In SS, and so long as the antibody is produced, inflammation continues. Patients frequently describe their own symptoms as being those of permanent influenza. The inflammation in SS is mainly in the salivary and lachrymal glands, though it can spread to joints and blood vessels in the skin where it may cause rashes. In rare cases it can affect the liver and kidneys.

What are the symptoms?

The dry eyes result in feelings of irritation, grittiness and, sometimes, burning. The eyelids may stick together and the patients may not be able to tolerate strong sunlight.

The dry mouth results in a burning sensation in the mouth or throat; the voice may be hoarse or weak; food can be difficult to swallow because it sticks to the tissues; the salivary glands may become enlarged and painful.

Patients frequently experience a "hurt all over" sensation, as if they have been battered, and feel continually exhausted.

What tests will the Doctor want to do?

A number of tests are available for diagnosing SS. They include: examination of the eyes by rose-bengal staining and measurement of tear production (Schirmer test); measurement of saliva production; x-ray examination of certain salivary glands (sialography); blood tests to determine the presence of antibodies to two identifiable antigens (Ro and La); and a lip gland biopsy.

What is the treatment?

The symptoms of SS can be treated depending on the degree of their severity. However, no treatment has yet been found to restore glandular secretions. Artificial tears help to lubricate dry eyes, and some sufferers benefit from using lacriserts; in severe cases punctal occlusion is employed to retain moisture in the eye.

Artificial salivas may provide temporary relief for the dry mouth, and fluoride gels and remineralising solutions are prescribed to restore the proper chemical balance in the mouth. Anti- inflammatory drugs may reduce the swelling of enlarged and inflamed glands. In certain cases, systemic medications, such as corticosteroids or piaquenil, are employed, and in complicated SS, immuno-suppressive drugs are sometimes found to be necessary.