Sjögren's syndrome is a disease in which the body's defense system (immune system) attacks healthy tissues causing dryness of the mouth, eyes and other membranes of the body that secrete mucous. It is often found along with rheumatic disorders such as rheumatoid arthritis, scleroderma and lupus.
Sjögren's syndrome may affect only the eyes or only the mouth or it may be more general. One out of three people who have Sjögren's syndrome also have arthritis. A person may have signs of a rheumatic disease, but not have the dry eyes or mouth associated with some forms of Sjögren's.
The disease damages the glands that supply saliva to the mouth and tears to the eyes. This causes the cornea of the eye and the tissues around the eye to dry out. The eyes may feel scratchy or irritated. In advanced cases, the cornea may be damaged, impairing vision.
Persons with Sjögren's syndrome have little or no saliva. This causes a dry mouth and lips. It also makes chewing and swallowing difficult and promotes tooth decay. The disease may also affect a person's ability to taste or smell food.
Other parts of the body where there are mucous membranes also begin to dry out. This includes the nose, throat, lungs, vulva and vagina. Drying in the lungs can result in lung infections or pneumonia. There may be a loss of hair on the body.
Other symptoms may include:
- Chronic diseases of the liver, gall bladder and pancreas
- Loss of the ability of the nerves to feel sensation
- Kidney disorders
Causes and Risk Factors
Sjögren's syndrome is more common than lupus but less common than rheumatoid arthritis. While its cause is not known, there may be a genetic factor.
A person who has Sjögren's syndrome has a 44 times higher risk of lymphoma compared to other people. Additionally, such individuals are at higher risk of Waldenström's macroglobulinemia.
To test whether a person has Sjögren's syndrome a doctor will do the following tests:
- Schirmer test. This test measures the amount of tears the eye forms when irritated by a filter paper strip placed under each lower eyelid
- Putting a drop of rose of Bengal solution, which stains the areas of the eye that absorb it, can also help identify the dryness of the eye that is characteristic of Sjögren's
- Tests to measure the flow of saliva in the mouth
- A biopsy of salivary glands in the mouth for examination under a microscope
- Blood tests to measure whether the body's immune system is reacting
Currently, treatment focuses on any related rheumatic disease that may be present and treating the symptoms of Sjögren's syndrome. Treatment may include:
- Sipping fluids throughout the day
- Chewing sugarless gum
- Using a saliva substitute as a mouthwash
- Avoiding antihistamines or other drugs that cause mucous membranes to dry up
- Being careful about regular mouth and tooth care and regular visits to the dentist
- A drug, pilocarpine, may be used to make the gland produce more saliva if they haven't been severely shrunken by the disease
- Pain relievers may be prescribed when needed
- Using eye drops
Cedars-Sinai has a range of comprehensive treatment options.