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What is Sjögren’s Syndrome?

Sjögren’s syndrome (also called Mikulicz disease or Sicca syndrome) is a relatively common chronic autoimmune illness, often accompanying other autoimmune conditions such as lupus and rheumatoid arthritis. In this disease immune cells attack healthy cells of the tear-producing glands and salivary glands. The common symptoms are dry eyes and mouth. Symptoms occur primarily due to low production of tears and saliva. In the United States, Sjögren’s syndrome affects nearly 4 million people and it happens to be the most prevalent autoimmune disease.

Fast Facts about Sjögren’s Syndrome

  • Sjögren’s syndrome is an autoimmune disorder causing dry eyes and mouth
  • The disorder causes less production of tears and saliva. Tear and salivary glands are attacked by immune cells affecting their ability to produce tears and saliva
  • The two types of Sjögren’s syndrome are primary and secondary Sjögren’s syndrome
  • Sjögren’s syndrome can be caused due to a genetic factors
  • Women are more prone to developing Sjögren’s syndrome than men. Individuals who have another autoimmune disorder like lupus are more likely to develop Sjögren’s syndrome as well
  • Symptoms include dry mouth, dry eyes, vaginal dryness, dry skin and cough
  • Sjögren’s syndrome can be diagnosed by physical examination, imaging and laboratory tests
  • Medications include over-the-counter eye drops, Restasis, Salagen, Plaquenil, and Evoxac
  • Sjögren’s syndrome can be managed by oral hygiene, using artificial tears and taking care of dry skin

Types of Sjögren’s Syndrome

  • Primary Sjögren’s syndrome is usually seen in people with no other rheumatologic condition
  • Secondary Sjögren’s syndrome occurs in people with other rheumatologic conditions such as rheumatoid arthritis and systemic lupus erythematosus

Causes of Sjögren’s Syndrome

In this autoimmune disorder, healthy cells and tissues are attacked by the immune system. Scientists are yet unable to explain why this autoimmune disorder is seen only in some people. Although there is a genetic component to the disorder, a triggering mechanism is necessary, which can be a bacterial infection. Resulting inflammation then destroys the tear glands and salivary glands. Abnormal proteins can be traced in the blood, indicating that the immune system is acting against healthy cells and tissues.

Risk Factors for Sjögren’s Syndrome

  • Symptoms are generally seen in people in the age group of 45 and 55
  • This condition is seen 10 times more often in women than men
  • People who already have another autoimmune disease like lupus or rheumatoid arthritis are at increased risk for developing Sjögren’s syndrome

Symptoms of Sjögren’s Syndrome

The two major symptoms of Sjögren’s syndrome are dry eyes and dry mouth. Eyes may feel itchy and burning with a gritty feeling. Dry mouth with swollen salivary glands makes it difficult to swallow and speak. Dental decay and gingivitis are associated with dry mouth.

Other symptoms include dry skin, vaginal dryness, joint pain, cough, and fatigue.

Diagnosing Sjögren’s Syndrome

Symptoms of Sjögren’s syndrome are not easy to diagnose because they are similar to symptoms caused by many other diseases. In addition, different people experience different symptoms and with varying degrees of severity. Early diagnosis and treatment prevent complications of the disease.

There is no single test to confirm a diagnosis of Sjögren’s syndrome. A combination of physical examination, laboratory tests and imaging is needed to diagnose this condition.

  • Physical examination consists of eye and dental tests:
  1. An eye test done to measure tear production is called Schirmer test. The surface of the eye is examined by a slit-lamp test
  2. Dental tests include salivary scintigraphy to measure salivary gland function
  • Laboratory tests are done to check for the following:
  1. Blood glucose levels
  2. Erythrocyte sedimentation rate
  3. Problems with liver and kidney
  4. Presence of anti-nuclear antibody
  5. Immunoglobulins
  6. Rheumatoid factor
  • Imaging:
  1. Sialogram – checks the salivary glands for flow of saliva into the mouth
  2. Salivary scintigraphy is a nuclear medicine test using a radioactive isotope. This test measures the functioning of the salivary gland
  3. Chest x-ray is ordered to check for lung inflammation

Other tests include biopsy of the lip to check for inflammatory cells indicating Sjögren’s syndrome. Urine sample may be ordered to check kidney function, i.e. if the kidneys are affected by the condition.

Treatment Options for Sjögren’s Syndrome

Most often, patients are able to manage their symptoms of dry eyes effectively using over-the-counter eye drops. Dry mouth can be overcome by frequent sipping of small quantities of water. Severe cases of Sjögren’s syndrome might sometimes require treatment with prescription medications.

Medications:

  • Salagen and Evoxac are prescribed to increase the production of saliva and tears
  • Arthritic symptoms can be relieved with nonsteroidal anti-inflammatory drugs. Antifungal medications are prescribed for oral yeast infections
  • Plaquenil, an antimalarial drug, is effective in treating Sjögren’s syndrome
  • Restasis is prescribed for dry eyes
  • Immunosuppressants such as methotrexate and cyclosporine are sometimes prescribed to deal with symptoms of Sjögren’s syndrome

Surgery:

Surgical treatment for dry eyes is effective. A minor surgical intervention seals the tear ducts.

Prevention of Sjögren’s Syndrome

Sjögren’s syndrome cannot be prevented but the symptoms can be managed with timely intervention and treatment.

Coping with Sjögren’s Syndrome

Symptoms of Sjögren’s syndrome can be managed well with proper treatment.

  • Dry eyes can be relieved with artificial tears and eye lubricants that are available in the form of eye drops or ointment. Increasing humidity inside the house, office, etc. will benefit your eyes from getting dry and causing discomfort.
  • Dry mouth can cause increased dental cavities and loss of teeth. Oral hygiene is necessary to maintain healthy teeth and mouth. Make sure to brush your teeth after each meal. See your dentist regularly, at least twice a year. Antimicrobial mouthwashes are a safe way to keep your mouth clean and moist.
  • Dry skin can be the result of Sjögren’s syndrome. Use lukewarm water when bathing. Do not rub your skin vigorously after showering; instead pat your skin dry. Hands tend to get rough and dry with constant dishwashing and using housecleaning detergents, therefore, wear protective gloves when engaged in such tasks. Women experiencing vaginal dryness benefit from vaginal moisturizers and lubricants.

Where to Get More Information: Sjögren’s Syndrome Foundation