Mikulicz disease, Sicca syndrome
What is Sjogren's Syndrome?
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 autoimmune rheumatic disease. Recent estimates suggested that there may be as many as half a million sufferers in the UK alone.
The symptoms of Sjogren's Syndrome
There are two categories of Sjogren's Syndrome (SS). It can be localized, affecting mainly the eyes and/or the mouth. Or, it can be systemic, and occur in conjunction with connective tissue diseases such as rheumatoid arthritis, lupus or scleroderma. The dry eyes cause irritation, and feelings of grittiness and, sometimes, burning. The eyelids may stick together and a patient 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 is the cause?
Sjogren's Syndrome is a chronic autoimmune 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 autoimmune diseases it is impossible to get rid of the antigen because it is part of the body.
Patients frequently describe their symptoms as feelings of permanent influenza. The inflammation in Sjogren's Syndrome occurs mainly in the salivary and lachrymal glands, though it can be spread to joints and blood vessels in the skin and may 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. These 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.
Can it be treated?
The symptoms of Sjogren's Syndrome 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 saliva 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 plaquenil, are employed, and in complicated SS, immunosuppressive drugs are sometimes found to be necessary.
- Artificial tears for eye dryness.
- Methylcellulose swab or spray for mouth dryness.
- Normal saline solution drops or aerosolized spray for respiratory dryness.
- K-Y Jelly as a lubricant for vaginal dryness.
- Avoid decongestants and antihistamines. They cause dry mouth.
- Corticosteroids and immunosuppressants may be prescribed for patients with severe symptoms.
- Nystatin may be prescribed for mouth infections.
- Avoid sugar which contributes to dental caries.
- To ease mouth dryness, chew sugarless gum or suck on sugarless candies.
- Drink plenty of fluids, especially at mealtime.
- If mouth soreness prevents eating regular foods, drink highcalorie, high-protein liquid supplements to prevent malnutrition.
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 autoimmunity in an individual already predisposed by genetic or hormonal factors to the disease, may eventually be identified. Research into SS is being conducted in the USA and in several centers in Europe, including England.
Seek Medical Attention
- You or a family member has symptoms of Sjogren's syndrome.
- Symptoms worsen or don't improve with treatment.
Last updated 6 August 2011