What is Sjögren's Syndrome?

[First printed in Lupus UK News & Views, Summer 2000 Number 61]

What is Sjögren's Syndrome?

In 1933, a Swedish physician Henrik Sjögren, drew attention to a large number of his female patients whose chronic arthritis was accompanied by dry eyes and dry mouth. Since then patients have this combination of symptoms have been desribed as having Sjogren's Syndrome. Today it is recognised to be the second most common auto-immune rheumatic disease. Recent estimates suggest there may be as many as half a million sufferers in the Uk alone.

The symptoms of this Syndrome

There are two categories of SS. It can be localised, affecting mainly the eyes and/or mouth. Or, it can be systemic, and occuring in conjunction with connective tissue diseases such as Rheumatoid Arthritis, SLE or scleroderma.

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

What is the cause?

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 the '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 inflamation in SS is mainly in the salivary and lachrymal glands, though it can spread to joints and blood vessels in the skin where it can cause rashes. In rare cases it can affect the liver and kidneys.

How is it diagnosed?

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); measuremememt of saliva production; x-ray examination of certain salivary glands (sialography); blood tests to determine the presence of antibodies to two identiafiable antigens (Ro and La); and a lip biopsy.

Can it be treated?

The symptoms of SS can be treated depending on the degree of their severity. However, no treatment has yet be found to restore glandular secretions. Artificial tears help to lubricate dry eyes, and some sufferers benefit from using lacriserts; in severe cases puntal occlusion is employed to retain moisture in the eye. Artificial salivas may provide temporary relief for the dry mouth and flouideflouride 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 inflammed glands. In certain cases, systemic medicstions, such as corticosteroids or the antimalarial, Plaquenil, are emplyed, and in complicated SS, immuno-suppressive drugs are sometimes found to be necessary.

What hope for a cure?

Far more is known about the genetics and antibodies in SS than in many other rheumatic diseases and there is hope that the external agent (possibly a virus), which induces auto-immunity in an individual already predisposed by genetic or hormonal factors of the disease, may eventually be identified. Research in SS is being conducted in the US and in several centres in Europe, including England.

Helpful Organisations

Raynaud's & Scleroderma Association
112, Crewe Road,
Cheshire ST7 2JA

TEL: 01270 872776

The British Sjögren's Syndrome Association (BSSA)
Unit 1, Manor Workshops,
Nailsea Wall Lane,
West End,
Nailsea BS 48 2DD

TEL: 01275 854215