Sjögren syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjögren syndrome is also associated with rheumatic disorders such as rheumatoid arthritis or systemic lupus erythematosus. The hallmark symptoms of the disorder are dry mouth and dry eyes. In addition, Sjogren syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain. Treatment is symptomatic and supportive and may include moisture replacement therapies, nonsteroidal anti-inflammatory drugs and, in severe cases, corticosteroids or immunosuppressive drugs.
Signs and symptoms may include:
- Reduced saliva production
- Dry mouth
- Swallowing difficulty due to lack of saliva
- Chewing difficulty due to lack of saliva
- Teeth decay due to lack of saliva
- Gingivitis due to lack of saliva
- Pyorrhea due to lack of saliva
- Atrophy of tear-producing glands
- Reduced tears
- Keratoconjunctivitis sicca
- Gritty eye sensation
- Burning eye sensation
- Dry skin
- Dry nose
- Dry eyes
- Red eyes
- Dry throat
- Dry vagina
- Inflammation of tear-producing glands
- Inflammation of salivary glands
- Mouth sores
- Inflammation of vaginal glands
- • Lung inflammation
- • Lymph node enlargement
Sjogren syndrome likely results from a combination of genetic and environmental factors (multifactorial). Several different genes appear to affect the risk of developing the condition, however, specific genes have yet to be confirmed. Simply having one of these genes does not cause a person to develop the disease; some sort of trigger is also needed. That trigger may be a viral or bacterial infection. The genetic variations that increase susceptibility may reduce the body's ability to turn off the immune response when it is no longer needed. The possibility that the endocrine and nervous systems may play a role in the disease is also under investigation.
A genetic predisposition to Sjogren syndrome has been suggested. Familial clustering of different autoimmune diseases as well as co-association of multiple autoimmune diseases in individuals have frequently been reported. Some studies have shown up to 30% of people with Sjogren syndrome have relatives with autoimmune diseases. While the relatives of people with Sjogren syndrome are at an increased risk of developing autoimmune diseases in general, they are not necessarily more likely to develop Sjogren syndrome.
Sjogren's syndrome can be difficult to diagnose because the signs and symptoms vary from person to person and can be similar to those caused by other diseases. Side effects of a number of medications also mimic some signs and symptoms of Sjogren's syndrome. A variety of tests can help rule out other conditions and help pinpoint a diagnosis of Sjogren's syndrome.
- Levels of different types of blood cells
- Presence of antibodies common in Sjogren's syndrome
- Evidence of inflammatory conditions
- Indications of problems with your liver and kidneys
Your doctor can measure the dryness of your eyes with a test called a Schirmer tear test. In this test, a small piece of filter paper is placed under your lower eyelid to measure your tear production.
A doctor specializing in the treatment of eye disorders (ophthalmologist) may also examine the surface of your eyes with a magnifying device called a slit lamp. He or she may place drops in your eye that make any damage to your cornea easier to see.
Certain imaging tests can check the function of your salivary glands.
- Sialogram. A special X-ray called a sialogram can detect dye that's injected into the salivary glands located in front of your ears. This procedure shows how much saliva flows into your mouth.
- Salivary scintigraphy. This nuclear medicine test involves the intravenous injection of a radioactive isotope, which is tracked over the course of an hour to see how quickly it arrives in all your salivary glands.
Your doctor may also want to do a lip biopsy to detect the presence of clusters of inflammatory cells, which can indicate Sjogren's syndrome. For this test, a small sliver of tissue is removed from salivary glands located in your lip and examined under a microscope.
Most individuals with Sjogren syndrome experience limited symptoms such as dry eyes and dry mouth. Their general health and life expectancy are largely unaffected. Increased mortality may be related to conditions associated with Sjogren syndrome, including systemic lupus erythematous (SLE), rheumatoid arthritis (RA), and primary biliary cirrhosis. Patients who don't develop a lymphoproliferative disorder can generally expect to have a normal life span.
Depending on your symptoms, your doctor may suggest medications that:
- Increase production of saliva. Drugs such as pilocarpine (Salagen) and cevimeline (Evoxac) is indicated to increase the production of saliva, and sometimes tears in patients with Sjogren's syndrome. Side effects may include sweating, abdominal pain, flushing and increased urination.
- Address specific complications. If you develop arthritis symptoms, you may benefit from nonsteroidal anti-inflammatory drugs (NSAIDs) or other arthritis medications. Prescription eyedrops may be needed if over-the-counter drops aren't helpful. Yeast infections in the mouth should be treated with antifungal medications.
- Treat systemwide symptoms. Hydroxychloroquine (Plaquenil), a drug designed to treat malaria, is often helpful in treating Sjogren's syndrome. Drugs that suppress the immune system, such as methotrexate, also may be prescribed.
Refer to Research Publications.