Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjögren's syndrome can be associated with rheumatic disorders such as rheumatoid arthritis, systemic lupus or other connective tissue diseases.
In fact, about 25% of patients with rheumatoid arthritis develop dry eyes and dry mouth problems. Patients with other diseases, such as scleroderma and lupus are susceptible. If there is no underlying rheumatic disease, the condition is called primary Sjögren’s. If there is an underlying problem, the condition is called secondary Sjögren’s.
The hallmark symptoms of the disorder are dry mouth and dry eyes. In addition, Sjögren's syndrome may cause skin, nose and vaginal dryness. It may also affect other organs of the body, including the joints, kidneys, blood vessels, lungs, liver, pancreas and brain.
The glands of the head and neck may swell, including the glands in front of the ears – the parotid glands, and under the jaw, the submandibular glands. The swelling of the glands may even be the first symptom. As the glands become inflamed, so dysfunction of salivary tissue develops, resulting in dryness of the mouth and eyes.
This is a clinical diagnosis. However, blood tests for various antibodies (anti-RO and anti-La) can be done to assess for underlying rheumatic diseases. An eye examination by an ophthalmologist will confirm the dry eye component. Occasionally a lip or salivary gland biopsy is done to assess the glandular component.
Sjögren's can damage vital organs of the body with symptoms that may plateau, worsen or go into remission. Some people may experience only the mild symptoms of dry eyes and mouth, while others go through cycles of good health, followed by severe disease.
Many patients are able to treat problems symptomatically. Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness and difficulty swallowing and eating. Debilitating fatigue and joint pain can seriously impair quality of life.
When the disease affects the organs it may lead to serious symptoms like shortness of breath, kidney failure, strokes and vasculitis of the skin. It can also lead to glandular cancer (lymphoma) in certain cases.
There is no known cure for Sjögren's syndrome, nor is there a specific treatment to restore gland secretion. Treatment is generally symptomatic and supportive. Moisture replacement therapies may ease the symptoms of dryness.
The use of mouth salivary supplements helps the dryness, and eye drops such as artificial tears or gel should be applied frequently. The use of lubricants such as KY jelly may relieve vaginal dryness during sexual intercourse.
Dental hygiene is extremely important, as the teeth are subject to rapid deterioration from dental caries. Saliva is an important natural antibacterial agent and maintains the health of the teeth and mouth. Visit your oral hygienist and dentist regularly to ensure maximum life of your teeth. The use of a daily anti-bacterial mouthwash and fluoride gel on the gums and teeth is advised.
Nonsteroidal anti-inflammatory drugs may be used to treat musculoskeletal symptoms. For individuals with severe complications, corticosteroids or immunosuppressive drugs may be prescribed. Occasionally disease modifying therapies such as antimalarials and methotrexate may be used in aggressive disease. New evidence has shown that the use of a biologic agent, Rituximab, may help to improve systemic involvement and severe sicca syndrome (dry eyes and mouth).
Monitoring of the glands may be required as there may be an increased risk of development of lymphoma.
Previously reviewed by Dr I. C. Louw and Dr A. Halland
Reviewed by Dr Ingrid Louw, MBChB, MMed (Int), rheumatologist/physician, (private practice), August 2011